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June 16, 2011
CM takes Food Department by surprise, instruct changes
Kolkata, June16: Chief Minister Mamata Banerjee paid a surprise visit to Khadya Bhavan on Free School Street. Some employees loitering downstairs were taken aback as she entered. After observing the sorry state of the department, Ms. Banerjee sought suggestions within 72 hours about what should be done to improve administration in the Food Department.
Food Minister Jyotipriya Mullick accompanied the Chief Minister as she inspected rooms in the building. Ms. Banerjee was visibly displeased with the way files were stacked. She asked officials to ensure proper file management. The Chief Minister also wanted to check the attendance register to know the time employees present had reported for work. The Chief Minister then held a short meeting with senior food department officials. The Chief Minister also expressed her displeasure over the lack of database in the department and stressed on the formulation of an electronic database. Adopting a tough posture, she asked the officials if they had ever inquired why they cannot reach food to all the needy, since the government provides funds. She stressed on strict monitoring. The Chief Minister also enquired about the system by which the applications for ration cards are sorted out. She also discussed about the various problems faced by the employees. The Chief Minister was unhappy about inadequate staff in the department.
With a vision to improve the administration of the food department, five vigilance committee each consisting of five members are to be formed to monitor the working of the department.


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What You Get in this Plan:
- Three meals a day for a week
- A selection of our 100/120g flavours including protein, veg, fruit & grain (please note this menu changes weekly and includes allergens)
- Option to add an additional protein of your choice where indicated on the meal plan
Please note: If your baby or either parents have known allergies, contact a health professional before trying any of the allergens in this plan. Always consult with a health professional before feeding your baby this plan.
Delivery question? Click here

domain: food


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6 Things I learned from Producing a Webseries
Not too long ago I had a very different mindset. It wasn’t that I didn’t think there was a lack of content out there (not only for people of color but all the different walks and variations of life) It wasn’t that I didn’t sense the need for content that wasn’t a regurgitation of what had been done before or feel the fatigue from watching the same stereotypes after stereotypes be portrayed in the same stereotypical situation after stereotypical situation. So, what had made me so resistant to the idea of creating when I truly felt that it was something I was capable of doing?
Was it laziness? Was it pride (I’m good enough to not have to write stuff for myself to actually work?) Was it fear (If I create it and “they” don’t like it, then what?) Or was it the sheer undertaking and inexperience of it all that made me resolute in my decision to forgo any idea of creating my own content and simply pray and wait for someone to write “me” one day?
Honestly, I’m not sure I know the answer, but truly believe it lies somewhere in “yes” to all of the above. Recently however, I had a revelation about fear and the need to experience something before I could say truly what I did and did not want to do--and I am very glad I did.
Almost two years ago, I was approached by my good friend Jeannette with an idea and the opportunity, and for some reason this time, I said yes…or was pushed to say yes—but i said it. :)
Here’s what I learned:
There is no failure in a try- none. No matter what the end result, the lessons learned while trying to create something from scratch that you've never done before are invaluable.
You can’t please everyone, but you should work to please yourselves. There is no one way to skin a cat, at the end of the process the cat just needs to be skinned. So you begin with trial and error and work from there. As long as you are comfortable with your process and your processes is yielding results…keep experimenting until you’re done—thinking only—“Is this something I am proud of?”
Develop a thicker skin—which for any artist is paramount.
Learn how to communicate—and then forget all of that- and learn how to communicate better. Every relationship survives or dies on the ability to successfully communicate.
You’re idea (while it may not be entirely unique) is valid when it comes from your unique perspective. Your perspective is valuable…to someone. So use your voice…someone is listening.
If you don’t do it at least once for yourself, how will you know how to explain it to someone else to do it for you; better yet, how will you ever truly appreciate it when someone else is doing it for you?
I am still learning, but when you create something you empower yourself. You set goals and check them off one by one. The gratification that comes from those small accomplishments, fuels you to reach higher the next time and work harder. You're proving to yourself what your capabilities are with every step and showing yourself where you may need to improve or grow. You remind yourself the whole way that obstacles are meant to be overcome. Period. There is no such thing as “I don’t know how” You figure it out. You trust your instincts, you take risks, you win some-you lose some, and you grow. You are forced to believe in yourself or investigate why you do not.
Lessons every Artist and Person can benefit from.

domain: literature


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Step back in time to the late 1960s, when Sean Connery resigned from playing James Bond, producers Harry Saltzman and Cubby Broccoli decided to gamble and doubled down with an untested director and an unknown star and came up with the crown jewels: On Her Majesty's Secret Service. Based on years of research, hundreds of interviews, and exclusive access to the archives of aStep back in time to the late 1960s, when Sean Connery resigned from playing James Bond, producers Harry Saltzman and Cubby Broccoli decided to gamble and doubled down with an untested director and an unknown star and came up with the crown jewels: On Her Majesty's Secret Service. Based on years of research, hundreds of interviews, and exclusive access to the archives of author Ian Fleming, screenwriter Richard Maibaum, and director Peter Hunt, this inside look features never-before-published script details, storyboards, production documents, interviews, memos, marketing material, call sheets, and hundreds of rare, behind-the-scenes photographs of the cast and crew, including sequences and entire sets not seen in the film. From novel to script to screen, this book details the incredible journey of making the most unique entry in the James Bond film series, the longest running, most successful film franchise in history. This is not the white-washed "authorized" story, but the real story....
|Title||:||The Making of On Her Majesty's Secret Service|
|Number of Pages||:||290 Pages|
|Status||:||Available For Download|
|Last checked||:||21 Minutes ago!|
The Making of On Her Majesty's Secret Service Reviews
I started to read this book WAAAAAAY back in February 2013, got up to about page 40, then put it down to gather dust for well over a year before I decided to clear down my "currently reading" list and pick it back up again.Rather than try and pick up where I left off, I jumped back to the start and really started to rattle through the pages in the limited spare time I have to read. What surprised me was just how enjoyable and interesting it is. Whilst the simplistic design and the heavy handed plot changes/descriptions between drafts take some getting into, once you're used to the style, it really is a very enjoyable read.Helfenstein's writing style is very conversational and easy to read, he's loaded the books with quotes, comments, anecdotes, facts and figures and has somehow managed to achieve the seemingly impossible, create a unique and niche book about James Bond.That's right. Not one decent 'making of' book exists about the Lazenby starring Bond flick, OHMSS and this book fills that void as an absolute gem of information, behind the scenes pictures and interviews. Charles Helfenstein has created such an in depth and enjoyable book based on one of the lesser known and lesser celebrated James Bond adventures and it has really come up trumps. Recommended for all fans of the Bond movies.
First off, the book suffers from a bit of poor proof-reading, reference control and editing, leading to quite a lot of grammatical and spelling errors, naming a Danish movie poster Swedish and paragraphs cut in mid-sentence by multiple pages of photographs, often with extensive legend text, or even lengthy interviews throughout the entire book.Disregarding this, it is a work of love but even so quite objectively presenting the bad with the good. A great collection of facts and also pictures not seen anywhere else.
Only for those obsessed by this movie, it contains a wealth of information and an amazing collection of stills, posters, and memorabilia.
I decided to read Charles Helfenstein's book on the making of OHMSS because I had used its initial chapter on the Fleming novel in a blog piece I wrote about the Fleming book (which you could find here:https://jackl0073.wordpress.com/2015/...) . I found the information he obtained to be so fascinating that I decided to go ahead and read the rest of the book, which is the most detailed and expansive resource on the 1969 James Bond film available. With interviews and extensive research, Helfenstein created the definitive document covering every aspect of On Her Majesty's Secret Service from Fleming's conception and inspirations behind the novel to every step of pre-production and screen treatments dating back to 1964 (5 years before the film debuted) to the casting, production, marketing, and release of the 1969 film. Everything a Bond fan could possibly want to investigate regarding OHMSS is here. Both the novel and the 1969 film of OHMSS represent some of the highest level of excellence and brilliance within the Bond franchise. Peter Hunt, who directed the film, wanted to not only make "the best Bond film ever," he also wanted to be faithful to Fleming's novel and conception of the Bond character. With George Lazenby making his acting debut as James Bond replacing Sean Connery, Peter Hunt had an enormous challenge ahead of him. Though many Bond fans are divided about this film, I think Peter Hunt's adaptation stands as one of the best film adaptations of Fleming's work on the big screen.
A fantastic, well researched book into the making of the second-best Bond film ever (best of course is From Russia With Love). Full of interesting detail and images, taking one though the whole process from pre-production to post-production and beyond. This book is a life's work from Mr Helfenstein (good name for a Bond villain!), and he's put in an enormous amount of effort. Reading how the film is made from script re-writes to casting and then shooting, you sometimes wonder how any feature film actually gets made. This is a brilliant insight into the making of film and should interest not only Bond fans but anyone interested in modern cinema.

domain: library


By Dara Schindelka
For the students living in the north precambrian shield along the shores of Lac La Ronge, learning to rock is part of the high school curriculum. During the last number of years Churchill Community High School, part of the Northern Lights School Division, has been offering students classes on how to be part of a garage band. That’s right, learning to rock and roll can get you a high school credit and you don’t have to freeze in the family garage.
The program runs under the guidance of educator and music advocate, Colin Jolly. Generally, a lot of students come with little to no music training and within a few short months they are playing everything from bass and drums to keyboards and electric guitars. They meet up with other enthusiastic students and are put into groups that learn basic music skills. By the end of the semester, they put on concerts for the community to come out and watch them play at. More importantly though, many friendships and future bands are created and a love of all things that rock begins to flourish among a new generation.
Just like every other single thing in this day and age, Covid 19 has changed the way this program runs. Gone are the easy-going jam sessions with students on their lunch breaks and after school. No more can groups interact with one another and talk to each other about newly discovered but actually classic bands like Led Zeppelin and Pink Floyd. Now students have to be very careful and are not allowed to mix freely with one another. Classes require masks and lots of virtual rehearsals happen rather than in person.
Jolly has had to find alternative ways to keep the music going in garage band. As many high schools switched to shortened semesters, garage band has become a full afternoon class and that means a lot more time to practice and learn. In that respect, it’s not all bad. “When students were in person before the winter break, they had time to tune up, gather into groups, and have a serious practice. Before that in the previous class schedule they had forty-five minutes so there was little time for actual rehearsal before class ended” Says Jolly.
The changes to being in lockdown presented new challenges for the class that just finished up this past month. The music class students took home their instruments and had daily drill sheets and met each day with their band to discuss their chord charts, where solos are going, and everything else that a band needs to do in order to keep the communication alive. Their hope was that it all came together in the final concert for the year end semester.
For student Xavier Ratte, groups like Black Sabbath, Metallica and Def Leppard are favourite bands. In fact, their music has been key to his mental health and getting through the pandemic. “I love those bands and the music really makes it for me.” Ratte says that he was really looking forward to this Garage Band class and, even though there are changes this year, he couldn’t wait to be a part of it, “I have been singing and playing guitar for a while now”. However, this band opportunity meant that Ratte had a chance to share his talent and enthusiasm and could play together with other students that felt the same.
For Pikaea Groves this has been something that she looked forward to for years and the challenges have not stopped her enthusiasm, “I love this class and love that we are doing all of this music. I took a drum kit home and am getting to meet new people and have lots of new stuff I’m learning” For Groves, who’s current favourite bands include Cavetown and Vampire Weekend, the garage band in a pandemic had interesting challenges, “The concerts were different this year but we were still doing stuff”.
The biggest change was from live to virtual concerts. This year their school created concerts that streamed from the school Facebook page. The students were encouraged to share it on their social media. As Mr. Jolly says, “They actually continue to get a lot of good responses”. After just a few weeks, the views on their concert were already in the thousands. Not bad for a school group that may have had a few hundred people in person if it would have been live at a venue.
With the future of live music so precarious these days, it seems that opportunities like a garage band class seem more important than ever. Without those lazy days of summer working on three chord original songs with teenage angst written all through them, how would we see music continue? We need our youth to continue creating and finding ways to express themselves without the constraints of formal music lessons and note reading. Often it seems there is a misnomer that says in order to be a musician one must have classical or formal lessons. This is simply not the case and never has been.
Music is cultural. Music is universal. Music tells stories in much the same way that the oral folklore was passed down generation to generation. By listening and taking it in, then passing it on again the cycle continues. The idea that music must fit into a box that is created by one group or genre is ridiculous and outdated. Garage band classes bridge the gap between the music of the everyday people and the skills required to pass that gift of music on to others.
With the course that Jolly uses, he focuses on learning basic chords, rhythms, and songs that have great hooks and are familiar to young and old. The purpose is so much more than learning the difference between eighth notes and quarter notes. It is to pass on a way of expressing oneself and sharing that in a group setting.
That is why the garage band program is powerful to many of the youth in this beautiful northern community. They are able to unite together and create something that expresses how they feel and then share that with a larger audience. This kind of creating is empowering. This kind of music speaks louder than any self-confidence ad campaign. This is why we need more programs like this everywhere. Mental health and music go hand in hand and the students that take part in this program are living proof of this.
Jolly finds his role as teacher often extends to a listening ear and encourager at times. He helps students through the rough waves of adolescence and the superpower that he passes on to his students is music. He is not alone. We all may remember teachers that made a difference in our lives. There are many who feel that because of the encouragement of an educator, they took a chance and became something bigger than they first believed they could be. The educator or mentor shared a skill, or a belief in us and our talent, and that made us who we are today.
As we all know, music opportunities in school are an important way to reach a new generation of musicians and future rock stars. It is also a way to continue positive mental health and cultural practices. The northern students of Churchill Community High School are grateful to have the chance to keep rockin’ in the Covid world. If ever there was a time to keep the music alive, it is now. Even in a pandemic, the show, and the garage band class, must go on.
*pictures courtesy of Colin Jolly


As the title says. I have verified that the images are in the selected Kustom folder of the device, however, Kustom is not able to load the images when formula to specify the local path. This issue only appeared after updating to the newest beta version
Edit: added screenshot
The latest beta removed storage permissions, you have to either manually load the image or use flows to pic them for it to work, it broke my setup too
Wow that’s dumb. I mean shouldn’t I be able to still access the files in the Kustom folder?
Kustom can access the Kustom folder BUT it cannot access it via the standard filesystem API, so if you use file:/// it might not work, please try replacing /storage/emulated… with “file:///sdcard/Kustom/…”, doing this will trigger a workaround and Kustom will try to access icons via the new system. Otherwise please use flows and the new storage APIs. I plan to add support to new storage APIs to globals too.
This topic was automatically closed 25 days after the last reply. New replies are no longer allowed.


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Susan Wilson, host and producer of the weekly public affairs program, "KC Currents," recently visited the Postal Service's Stamp Fulfillment Services (SFS) Center in Kansas City, MO.
The SFS Center is located 150 feet below the surface, inside a massive underground business park called the Hunt Midwest SubTropolis. Because the limestone caverns maintain a year-round temperature of about 70 degrees, Fahrenheit, and low humidity, it's a great place to house the Postal Service's Stamp Fulfillment operations.
SFS Manager Khalid Hussain and members of his staff provided Wilson a tour of the facility, which includes more than 326,000 square-feet of workroom floor and storage space -- and millions of dollars worth of commemorative and definitive postage stamps. Wilson learned about the work of the SFS's 178 career USPS employees, who fill stamp orders from collectors located around the country and around the globe.
To listen to the program, go to
http://www.kcur.org/ or click here .
|Stamp Fulfillment Services (SFS) Manager Khalid Hussain |
explains the inner -- and underground --
workings of the SFS to KCUR-FM's Susan Wilson.
I've visited the cave once. It was extremely interesting and worth the time. However, it was a little unnerving. The walls are solid rock and even though the ceiling is pretty high you could still get a little claustrophobic. One of the more interesting facts: customers can send in an envelop with original artwork (a one of kind piece) and get a first day of issue stamp applied. That makes a very personal and collectible piece.
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domain: geography


If you are worried about your motor vehicle, you’ve come to the right place, and yes, through this page we plan to support you in dealing with the little problems that your Jaguar F Pace can cause you. We will now check out your Jaguar F Pace that doesn’t idle effectively , which can be almost invisible and sometimes signify the failure of essential components, but it is not a issue that can go unseen. It is consequently very essential that you pay attention to it and you did the right thing in starting to look for a solution to the problem. To answer this question, we will first check out some of the points to look at for an Jaguar F Pace that doesn’t idle and finally, some parts that may be to blame for your Jaguar F Pace that doesn’t idle.
Jaguar F Pace THAT WON’T Idle effectively: RISKS AND THE PRELIMINARY CHECKS TO BE CARRIED OUT
First of all, it is essential to know that your Jaguar F Pace iddle is not holding correctly , if it is troubled you will risk prematurely damaging parts of your motor vehicle. Generally, it is expected that the idle speed of your Jaguar F Pace should be between 750 and 900 rpm. If your Jaguar F Pace does not idle, or if your idle speed is unstable, this can be noticed by moderate variations in the idle speed of your Jaguar F Pace from 100 rpm up to the engine unit idle speed.
We will now take a closer look at the checkpoints that need to be evaluated to get an idea of the motive of your Jaguar F Pace not holding idle:
- Electronic Problem: Whether it is an ECU or sensor issue, you may have an engine unit defect in your ECU, inspect it with a diagnostic tool and if it is the case, concentrate on the electrical malfunction, it could be that your Jaguar F Pace is not idling and is caused by one of these problems.
- Exhaust analysis: If you notice black smoke, it should be a signal of a combustion or intake issue. If you notice white fumes, it’s probably because of water inside your cylinders, be careful with your cylinder head gasket!
- Engine unit block examination: Ensure you have a good check out all the hoses of your Jaguar F Pace, your electrical connectors, your sensors, a very easy hole in a hose or a disconnected cable can be the cause of your concerns.
Jaguar F Pace THAT WON’T IDLE: THE COMPONENTS THAT CAN CAUSE IT
In this section, we will now review the parts that can produce your Jaguar F Pace go into slow motion. First we’ll focus on the worries of electric source, and then on those related to the admission of your Jaguar F Pace.
Jaguar F Pace IDLING UNSTABLE BECAUSE OF ELECTRICAL MALFUNCTION
- One of the most general factors that your Jaguar F Pace will not idle is if one of your sensors is broken, dirty or simply has a bad connection to the power supply. On this website is a list of the main things you should check out if you have ever had a fault displayed on your ECU. Verify the temperature sensor, your air flow meter and the lambda sensors (2 in total).
- Sensor hassle: Just as with sensors, it’s more than likely that a very easy faulty sensor is the origin of your idle that doesn’t fit on the Jaguar F Pace. Consequently, verify the camshaft sensor and the top dead center sensor.
- ECU trouble: At last, your ECU may be the cause of your problems with your Jaguar F Pace. It can be because of an electrical malfunction or a software issue, in any case go to your garage area for a reprogramming or a verify of the connections.
- Check your alternator which, if it is weak, can be the cause of the issue.
Jaguar F Pace SLOWED DOWN UNSTABLE BECAUSE OF INTAKE
Finally, in this article are the possible motives of your Jaguar F Pace not keeping up when idle caused by an intake issue.
- Fuel Intake Problem: Your fuel injection pump or injectors may be clogged. As well verify your pressure sensors which are basic motives of this sort of issue on the Jaguar F Pace.
- Throttle body issue: Nevertheless generally found on gasoline engines, the throttle body can be the cause of an Jaguar F Pace not holding idle, try to check out it.
If you have any further questions about the Jaguar F Pace, do not hesitate to consult our Jaguar F Pace category.


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Wallis (Photo courtesy Michael J. Media Group LLC)
15 year-old Wallis, a heretofore unknown young singer/songwriter from Philadelphia, has continued to create and perform — despite the challenges of 2020 — by writing songs with her father.
“Lonely Christmas” is Wallis’ very first single, and the perfect tune for this unprecedented holiday season.
The Districts perform at 9:30 Club on March 10, 2020. (Photo by Casey Vock.)
Sometimes an album comes to life when a band needs it most, and the tour to support it is how the group actually reaps the spiritual, and financial, benefits of their work.
The Districts’ latest release — You Know I’m Not Going Anywhere — is said to have almost never happened: the band had nearly exhausted itself after an intense few years on the road and seemed to be searching for direction when founder and head singer Rob Grote isolated himself and put pen to pad to nurture the words that would comprise the newest LP.
The Districts (Photo by Shervin Lainez)
Philadelphia’s The Districts hit 9:30 Club on Tuesday, March 10, in support of You Know I’m Not Going Anywhere, their new album available on March 13 via Fat Possum.
Slaughter Beach, Dog performs at Baltimore Soundstage on Feb. 8, 2020. (Photo by Casey Vock)
Musicians naturally morph over the course of their careers — their tastes evolve, they’re shaped by their experiences, influences can come and go.
That change tends to be gradual and isn’t easily identified or documented. You can dissect only what the artist offers up for public consumption.
G. Love and Special Sauce perform at The Hamilton Live, Jan. 30, 2020. (Photo by Ari Strauss)
Eclectic blues/hip-hop artist G. Love was in DC recently with his band, Special Sauce, to play two shows at The Hamilton Live. The performances were part of a winter tour in support of his new album The Juice, which was released Jan. 17 via Philadelphonic/ Thirty Tigers.
G. Love (Photo by Sarah Ewalt)
G. Love released The Juice, his latest studio album, on Jan. 17 via Philadelphonic/Thirty Tigers. G. Love & Special Sauce performs at The Hamilton Live on Thursday, Jan. 30.
Son Little performs at Rock and Roll Hotel on Nov. 30, 2019. (Photo by David LaMason)
Philadelphia artist Son Little (the alias of Aaron Livingston), a sometime collaborator with The Roots and alternative hip-hop artist Rjd2, combines blues, soul, and hip-hop in his unique singer-songwriter style. Playing solo on acoustic and electric guitar, Son Little entertained a post-Thanksgiving crowd of music lovers at the Rock and Roll Hotel on Saturday.
David Bromberg (Photo by Joe del Tufo)
Noted roots artist David Bromberg appeared in a big band format at the Sixth & I Historic Synagogue recently. His band included a horn section—trumpet, trombone, and sax — and strings — fiddle and banjo. But David honored his roots in the blues — he’s a protégé of the Rev. Gary Davis — growling and howling through numbers like his opener, which I think, was Robert Johnson’s “Walkin’ Blues.”
David Schelzel fronts The Ocean Blue at Union Stage on Oct. 4, 2019. (Photo by Mickey McCarter)
Here’s how I like to imagine it happened: Teenaged David Schelzel stood on an overlook above Hershey Park in Pennsylvania and pondered the decade that was coming to a close. “I’m going to take the very best of the ’80s and synthesize it into one band,” he said, reaching deep into England, where the very best new wave and psychedelia had emerged, and scooping all of those sounds into one impossibly sweet band.
And so was born The Ocean Blue, which published its first self-titled album in 1989, and captured the zeitgeist of cool alternative music in one quartet (or so we say!). Quite active in recent years, The Ocean Blue again reminded us just how cool they are in a sold-out show at Union Stage last week.
Daya performs at The Anthem on Sept. 18, 2019. (Photo by Katherine Gaines/ ambienteye.com)
Singer Grace Martine Tandon, better known as Daya, dropped by The Anthem to open Marina recently, and the rising young pop star promised more to come from her soon. Katherine Gaines photographed Daya in action!

domain: entertainment


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In continental Europe, there have been registers of rare lung diseases for many years. The term ‘orphan’ lung disease was coined because of the feeling that these diseases have, in the past, tended to be neglected because of their rarity; hence, there is limited knowledge of the conditions and limited available data on which to base clinical practice.
The previous British reporting system (BOLD) has now closed. The BTS Specialist Advisory Group on interstitial and rare lung diseases may continue this initiative in due course.
This is a rare ILD, characterized by the accumulation of numerous and diffuse calcified microliths (round calcium and phosphate hydroxyapatite bodies) in the alveolar space. There is no identifiable abnormality of calcium metabolism. A mutation in the SLC34A2 gene that encodes a type IIb sodium-phosphate co-transporter in alveolar type II cells, resulting in the accumulation and formation of microliths rich in calcium phosphate (due to impaired clearance), is considered to be the cause of the disease. Microliths are occasionally identified in the sputum. At post-mortem, the lungs are heavy and rock hard, often needing a saw to cut them. Fewer than 200 cases are reported.
• Typically presents in young adults, most commonly in the third and fourth decades of life
• May be an incidental CXR finding in asymptomatic patients
• Familial tendency—probable autosomal recessive inheritance
• Equal sex distribution in sporadic cases, 2:1 ♀ preponderance in familial cases
• Usually slowly progressive, with progressive breathlessness, hypoxia, respiratory failure, and death
• CXR and chest CT show fine micronodular lung calcification, predominantly basally or around the hila. It may produce complete radiographic opacification. There is no associated lymph node enlargement. Progressive lung infiltration causes restriction of lung movement and impairs gas exchange, leading to progressive respiratory failure.
Amyloidosis is the extracellular deposition of low molecular weight protein molecules as insoluble fibrils. More than 20 such proteins have been described in different diseases and circumstances. For classification, see Box 49.1.
The disease is one of abnormal protein folding and is classified by the origin of the precursor proteins that form the amyloid. For example, AL amyloid forms from the light chains of immunoglobulins. In familial forms, genetic missense mutations produce abnormal folding of the protein. Little is known of the specific genetic and environmental factors that lead to the development of this abnormal folding. Despite their different origins, these protein molecules fold into alternative forms that are very similar to each other; in the classic ‘β-pleated sheet’ structure, fibrils form in an ordered fashion, with uniformity of fibril structure within the sheet. Substitutions of particular amino acids at specific positions in the light chain variable region lead to destabilization of the light chains, increasing the chance of fibrillogenesis. In certain models, this abnormal folding can be initiated by the addition of ‘amyloid-enhancing factor’, rather like the initiation of crystal formation in a supersaturated solution. Amyloid deposits accumulate in the extracellular space, disrupting normal tissue architecture and leading to organ dysfunction, both directly, and having space-occupying effects. The fibrils may be directly cytotoxic (possibly by promoting apoptosis). The subdivisions of amyloid are largely based on the origin of the amyloid protein and shown in Box 49.1.
The epidemiology is difficult to define accurately, as the disease is often un- or misdiagnosed. The age-adjusted incidence is estimated to be 5–13 per million person years.
Clinically significant respiratory tract disease is almost always AL in type, though the presence of a strong family history or chronic inflammatory disease may suggest other types.
Amyloid causes up to 1% of benign laryngeal disease. May present as discrete nodules or diffuse infiltration and is usually localized, though can be a rare manifestation of systemic (AL) amyloid. Deposits are seen most commonly in the supraglottic larynx (presenting with hoarse voice or stridor). May present with choking and exertional dyspnoea that can be progressive or recurrent.
is rare (67 worldwide cases reported by the mid 1980s). Macroscopically, is either diffusely infiltrative or ‘tumour-like’. It is associated with tracheobronchopathia osteoplastica (a disorder characterized by the deposition of calcified submucosal airway nodules). It presents after the fifth decade with dyspnoea, cough, and rarely haemoptysis. Airway narrowing can lead to atelectasis or recurrent pneumonia; solitary nodules may lead to investigation for presumed lung cancer. Symptomatic disease is usually localized.
is the most frequently diagnosed amyloid respiratory disease. It is usually divided radiologically into solitary/multiple pulmonary nodules (usually localized AL amyloid) or a diffuse alveolar pattern (usually a manifestation of systemic AL amyloid). Parenchymal amyloid lung nodules are usually peripheral and subpleural, may be bilateral, and are more common in the lower lobes, ranging in diameter from 0.4 to 15cm. They may cavitate or calcify. Clinical signs are non-diagnostic; PFTs may show a restrictive defect with reduced transfer factor. The differential diagnosis usually includes fibrosis. Cardiac amyloid may coexist, and distinguishing the contribution to the symptoms of the pulmonary and cardiac disease can be difficult. Median survival with clinically overt lung disease is about 16 months (similar to that of systemic amyloid).
Mediastinal and hilar amyloidosis
are rarely associated with localized pulmonary amyloidosis, and their diagnosis should lead to a search for a systemic cause of amyloid. Amyloid lymphadenopathy can also represent localized AL deposition, in association with B-cell lymphoma.
Rare reports of:
• Ventilatory failure due to diaphragm or other respiratory muscle involvement
• Sleep apnoea from macroglossia due to amyloid
• Exudative pleural effusions.
• Dyspnoea and cough
• None—parenchymal disease may be an incidental finding on routine radiography
• Consider the diagnosis particularly in patients with odd upper airway symptoms and parenchymal involvement or those with unexplained CCF or nephrotic syndrome.
Histological confirmation is usually required. Congo red stain producing ‘apple green’ birefringence in crossed polarized light is the gold standard. Positive histology must lead to immunohistochemistry to determine the fibril type.
• Histology TBB or occasionally open or VATS biopsy (more likely if investigation for solitary pulmonary nodule)
• 123I-labelled scintigraphy Radiolabelled serum amyloid P (SAP) localizes to amyloid deposits in proportion to the quantity of amyloid present, therefore allowing identification of the distribution and burden of disease. It is most sensitive for solid organ disease though, in lung disease, is useful for determining the extent of disease in other organs. It is, however, expensive and carries an infection risk, as the SAP component is currently obtained from blood donors
• HRCT may show nodules or parenchymal disease
• Laryngoscopy and bronchoscopy may be needed to obtain samples for histology, depending on the clinical presentation
• PFTs to assess the effect of disease on respiratory function. May show reduced transfer factor and a restrictive pattern. Tracheobronchial involvement may lead to abnormal flow–volume loops due to larger airway obstruction
• Systemic disease:
• FBC, biochemistry, and urinalysis (?renal involvement)
• Investigate for underlying blood cell dyscrasia, e.g. myeloma, Waldenström’s macroglobulinaemia (bone marrow examination, and search for urine and serum monoclonal protein by immunofixation—the clonal proliferation underlying systemic AL amyloid is usually very subtle, and its identification may be difficult)
• Echo for associated cardiac involvement (when CCF is present; survival is 4–6 months)
• Thyroid/adrenal function is impaired in up to 10%.
There are limited clinical trials with which to guide management of respiratory tract amyloid. Management decisions are therefore often made empirically.
• No treatment may be needed
• Local measures may be warranted for endobronchial disease, e.g. symptomatic laryngeal disease—endoscopic excision, CO2 laser evaporation (useful for small recurrent lesions), stenting. Steroids have no effect on laryngeal amyloid
• Tracheobronchial amyloid—management depends on symptoms, and treatment may involve repeated endoscopic resection, YAG (yttrium–aluminium–garnet) laser therapy, and surgical resection. Repeated endoscopic procedures are thought to be safer than repeated open surgery
• Chemotherapy may be warranted for diffuse parenchymal amyloid if there is objectively measurable disease (prednisolone and melphalan to suppress the underlying blood cell dyscrasia). More intensive chemotherapy has a better clinical response, but there are few trials.
Hereditary haemorrhagic telangiectasia
An autosomal dominant disorder, >80% of all cases of HHT are due to mutations in either ENG or ACVRL1 (endoglin and activin, both TGF-β1 receptors). A total of over 600 different mutations is known. It is characterized by the development of abnormal dilated vessels in the systemic circulation, which may bleed, leading to:
• Recurrent epistaxis
• GI bleeding
• Iron deficiency anaemia
• Other organ involvement, e.g. hepatic (in 30%, commonly asymptomatic), renal, pulmonary, and spinal AVMs.
Careful questioning of family members (does anyone in the family have frequent nose bleeds?) and examination for telangiectasia should reveal those in whom screening should occur.
All those with HHT should be screened for pulmonary AVMs (PAVMs; see pp. [link]–[link]), and all of their offspring post-puberty and pre-pregnancy. There is increasing penetrance with increasing age (62% at age 16, 95% at age 40). Similarly, the detection of PAVMs in a patient should lead to screening for HHT in family members.
There is no consensus regarding the best screening method, but a combination of the following tests may be used:
• Supine and erect oximetry
• CT chest
• Shunt quantification techniques, e.g. contrast echo, 100% O2 rebreathing.
Screening should continue throughout life (every 5–10y) and during times of enlargement or development of AVMs—post-puberty and pre-pregnancy.
• Usually involves liaison with ENT and gastroenterology colleagues for symptomatic treatment
• Iron replacement, transfusions
• Asymptomatic hepatic AVMs—no treatment usually required
• Cerebral AVMs (in 15% of HHT patients)—some specialists argue these should be treated prophylactically due to the risk of rupture and bleeding (2%/y, often fatal).
A rare disease of undetermined aetiology, characterized by recurrent episodes of alveolar haemorrhage and haemoptysis (in the absence of renal disease), usually leading to iron deficiency anaemia.
The alveolar space and interstitium contain haemosiderin-laden macrophages, with variable degrees of interstitial fibrosis and degeneration of alveolar, interstitial, and vascular elastic fibres, depending on the chronicity of the condition. Electron microscopy shows damage to the endothelial and basement membranes, but no consistent or diagnostic features have been recognized.
No antibodies have been identified, though serum IgA levels are sometimes raised. With recurrent alveolar haemorrhage, the alveolar blood provokes a fibrotic reaction, with the development of diffuse pulmonary fibrosis.
Iron turnover studies show that the accompanying iron deficiency anaemia is due to loss of iron into the lung through haemorrhage.
is uncertain but likely to be multifactorial. Possible associations include toxic insecticides (epidemiological studies in rural Greece), premature birth, and fungal toxin exposure. The disease has an equal sex incidence in childhood, with twice as many men affected in adulthood.
Most patients present in childhood, with 85% of cases having onset of symptoms before 16y. The actual prevalence is unknown, but a cohort study of Swedish children in the 1960s described an incidence of 0.24 per million children. Familial clustering is reported.
Pulmonary haemosiderosis is associated with RA, thyrotoxicosis, coeliac disease, and autoimmune haemolytic anaemia, suggesting a potential autoimmune mechanism.
The clinical course is very variable and ranges from continuous low-level bleeding to massive pulmonary haemorrhage. The latter may be fatal but is fortunately rare.
• Continuous mild pulmonary haemorrhage leads to a chronic non-productive cough with haemoptysis, malaise, lethargy, and failure to thrive in children
• Iron deficiency anaemia is common, as are positive faecal occult blood tests (due to swallowed blood)
• Generalized lymphadenopathy and hepatosplenomegaly are recognized
• With an acute bleed, cough and haemoptysis may worsen, and dyspnoea, chest tightness, and pyrexia may develop
• Chronic bleeding leads to chronic disabling dyspnoea, chronic anaemia, and clubbing (in 25%). Cor pulmonale 2° to pulmonary fibrosis and hypoxaemia may develop.
may be normal. Clubbing, basal crepitations, and cor pulmonale are all recognized, depending on the severity of the resulting lung disease.
The diagnosis is one of exclusion, with no evidence of other organ involvement. The main differential diagnosis is Goodpasture’s syndrome, GPA, SLE, and microscopic polyarteritis.
• Blood tests Microcytic, hypochromic anaemia, with low iron levels. ANCA, dsDNA, and anti-GBM antibodies should be negative
• CXR May show transient patchy infiltrates, which worsen during an acute bleed. The apices are usually spared. Progressive disease leads to the development of reticulonodular infiltrates and a ground-glass appearance that is typically perihilar or in the lower zones. Hilar lymphadenopathy may be seen
• PFTs kCO is transiently elevated during bleeding episodes (≥130% is abnormal), but this is only useful acutely. A restrictive defect with reduced kCO may develop with chronic disease
• CT chest The changes seen are fairly non-specific, showing a diffuse bilateral infiltrate, with patchy ground-glass change
• BAL (if done) contains haemosiderin-laden macrophages.
There is no specific treatment.
• Steroids and immunosuppressive drugs (e.g. cyclophosphamide) may be of benefit during acute bleeding episodes but do not appear to affect the long-term outcome. There are no published data to guide the optimal timing of treatment during the course of disease
• The iron deficiency anaemia responds to replacement therapy, and blood transfusion may be needed in severe bleeds
• Lung transplant has been tried.
At routine clinic appointments
• Check spirometry
• Measure Hb and serum iron levels
• Ask about increases in SOB or haemoptysis.
The prognosis is very variable, with some patients showing spontaneous remission, others progressing to death. The duration of disease in the literature ranges from death within days, following an acute severe illness, to survival with cor pulmonale associated with chronic disease after 20y.
Pulmonary Langerhans cell histiocytosis (LCH; previously termed pulmonary histiocytosis X or pulmonary eosinophilic granuloma) is a rare condition characterized by infiltration of the lung with histiocytes (Langerhans cells). Pulmonary LCH overlaps with a number of other conditions with similar pathological findings but diverse clinical features. These range from localized infiltration of a single organ (e.g. eosinophilic granuloma of bone) to systemic diseases affecting multiple organs (Letterer–Siwe disease, a multi-organ disease affecting infants and elderly, associated with poor prognosis; also Hand–Schueller–Christian syndrome). Although the isolated pulmonary form most commonly presents to chest physicians, pulmonary manifestations also commonly occur in the systemic forms of the disease.
Rare, it tends to affect young adults aged 20–40y. The vast majority of cases occur in current smokers, usually heavy smokers (tobacco and cannabis). May be more common in men, who tend to present at a younger age than women.
Langerhans cells are involved in antigen presentation and are characterized by the presence of well-demarcated cytoplasmic organelles called Birbeck granules on electron microscopy. The Langerhans cells seen in LCH appear to be monoclonal, although it is unclear if this represents a true neoplastic process. The antigen stimulus for activating Langerhans cells in the lung is unknown, although cigarette smoke is a possible candidate. Langerhans cells are typically organized into granulomata that are located in bronchiolar walls and subsequently enlarge and invade adjacent structures. This results in the radiological appearance of nodules that, at first, cavitate and then become cystic.
Typically exertional breathlessness and cough, sometimes with systemic symptoms (e.g. fever, weight loss). Pneumothorax occurs in at least 10% of patients and may be the presenting feature. Rib lesions may also give rise to chest pain. Around 25% of patients are asymptomatic. Examination is usually normal.
• CXR Typically diffuse reticulonodular shadowing, sometimes with cystic change; upper and middle lobe predominance. May be normal
• HRCT Diffuse centrilobular nodules, sometimes with cavitation, and thin- and thick-walled cystic lesions, reflecting lesions of varying age. These are interspersed with normal lung. Upper and middle lobe predominance; costophrenic angles are typically spared. Purely nodular or purely cystic appearances may occur. Unusual manifestations, such as single nodules or large airways involvement, are also described
• PFTs Variable, ranging from normal to obstructive, restricted, or mixed patterns. Reduced gas transfer and exertional hypoxia are common
• TBB may yield diagnostic material although is often unhelpful; risk of pneumothorax is unknown although may be increased. Surgical lung biopsy is often preferable
• BAL Increased total cell counts and pigmented macrophages, reflecting simply the presence of cigarette smoking. Use of antibodies (e.g. OKT6) to detect Langerhans cells in BAL fluid is limited by poor sensitivity
• Extrathoracic biopsy of involved sites (e.g. bone) may be diagnostic.
Usually based on the combination of clinical and HRCT findings: typically a young adult smoker with cysts and nodules on HRCT. Confirmation by surgical lung biopsy may be considered in atypical presentations such as the finding of solely nodular or cystic disease on HRCT. The appearance of purely cystic disease on HRCT may be confused with emphysema (where cysts lack walls) or LAM (where cysts are present uniformly in all regions of lung, including the costophrenic angles).
• Severe PHT—may be seen in the absence of significant parenchymal lung involvement; direct disease involvement of pulmonary vessels has been described
• Manifestations of systemic LCH—particularly diabetes insipidus from pituitary disease, skin involvement, lytic bony lesions, and rarely cardiac or GI disease
• Lymphoma—may precede, complicate, or coexist with pulmonary LCH
• Lung cancer—more common, probably as a result of cigarette smoking.
Treatment, other than smoking cessation, is often not required and may be entirely successful with resolution of radiographic abnormalities. Oral corticosteroids may be tried in symptomatic disease, although there is little evidence to support their use; they are usually administered for at least 6 months. Lung transplantation should be considered in patients with severe respiratory failure or PHT. Pulmonary LCH may recur in transplanted lungs. Experimental treatments, such as the use of IL2 and anti-TNF-α, may be of benefit in the systemic forms of LCH seen in children.
is variable. Spontaneous improvement is common, although later reactivation of disease may occur. A minority of patients deteriorate rapidly, with respiratory failure and death within months. Overall life expectancy is reduced, with median survival 12–13y from diagnosis. Death is most commonly due to respiratory failure. Poor prognostic factors include reduced FEV1, increased RV, and reduced gas transfer.
Definition and aetiology
A rare disorder characterized by abnormal proliferation of smooth muscle cells, affecting women of childbearing age, usually in their 30s. The disease is hormone-dependent so can occur in post-menopausal women on oestrogen replacement therapy.
• Incidence of 1 in 1.1 million population
• 40% of adult women with tuberous sclerosis (learning difficulties, subungual fibromas, seizures, facial angiofibromas, autosomal dominant inheritance or spontaneous mutation) develop pulmonary changes identical to those of LAM.
Abnormal proliferation of atypical smooth muscle cells (LAM cells) throughout the lung, airways, blood vessels, and lymphatics. There is nodular infiltration, which is initially subtle. Progressive growth causes lymphatic and airway obstruction, leading to cyst formation throughout the lungs. The infiltrating cells stain with antibodies to smooth muscle actin and desmin with HMB-45, an antibody that recognizes an epitope within the protein gp-100 in the melanogenesis pathway. LAM is caused by mutations in the tuberous sclerosis (TSC) genes, resulting in activation of the mTOR complex 1 signalling network. Inactivation of both alleles of TSC2 seems to be necessary.
• 2° pneumothorax (in two-thirds of patients; occurs due to lung cystic change; recurrence is common)
• Dyspnoea (in 42%) and cough (in 20%)
• Haemoptysis (in 14%)
• Chylothorax (in 12%, thoracic duct leakage as a result of lymphatic obstruction by LAM cells, may be bilateral).
Other organs affected
Angiomyolipoma, a benign tumour, occurs in 50% of LAM patients. Usually diagnosed on CT, these are mostly small and single but can be multiple and larger in tuberous sclerosis. Smaller tumours are usually asymptomatic, but larger ones can cause flank pain and bleeding into the renal tract. Treatment options include tumour resection or embolization. Nephrectomy is not usually required. Screening for these lesions is important, as it allows careful treatment planning in case they become symptomatic.
Lymphadenopathy due to lymphatic obstruction. Occurs in one-third of patients and is usually asymptomatic.
Lymphangioleiomyoma—a cystic mass that enlarges during the day and causes fullness and bloating.
May be normal. There may be pulmonary crepitations or signs of pleural effusion. Palpable abdominal masses may be present.
• PFTs may be normal or show a predominantly obstructive pattern. Rarely restrictive. Decreased TLCO, with a normal or increased TLC
• CXR may be normal. Lungs may appear hyperinflated, with reticular shadowing and septal lines due to obstructed lymphatics. There may also be a diffuse interstitial infiltrate
• HRCT shows a characteristic appearance, with multiple cysts throughout the lung of varying size, which are usually small (<1cm) and thin-walled. The adjoining lung parenchyma is normal. There may be pleural effusions
• CT abdomen to examine for presence of angiomyolipomas and other lymphatic involvement.
Consider particularly in young or middle-aged women with:
• Recurrent pneumothoraces, especially those with pre-existing dyspnoea or haemoptysis
• Cystic lung disease, airflow obstruction, or chylous pleural effusions
• Angiomyolipomas or other retroperitoneal tumours
• Tuberous sclerosis and respiratory symptoms.
The disease is easily missed in its early stages. The diagnosis can be made on the characteristic CT appearances or with open lung biopsy. TBBs may not be diagnostic. Large retroperitoneal abdominal lymph nodes can also be biopsied.
The course of LAM is variable. Treatment should be aimed at those who are symptomatic and declining.
• Refer to a specialist centre (Nottingham City Hospital in the UK).
• Diet Low-fat diet with medium-chain triglyceride supplementation may prevent chylothorax recurrence; no strong evidence for this, difficult diet to follow
• Bronchodilators may improve airflow obstruction
• Sirolimus shown to stabilize lung function and improve QoL and is recommended treatment
• Hormonal manipulation with progesterone has been tried. It may be beneficial in reducing the decline in FEV1 and TLCO, particularly in patients with progressive disease, but there are no large studies. Tamoxifen and oophorectomy have also been tried
• Avoid oestrogens, i.e. the OCP and HRT
• Contraception An increase in symptoms and accelerated disease decline are reported in pregnancy. Use the progesterone-only pill
• Pleural aspiration when required for pleural effusions. For recurrent effusions or chylothoraces, thoracic duct ligation or pleurectomy may be effective. Pleurodesis can be performed, but this is relatively contraindicated if future lung transplant is an option
• Recurrent pneumothoraces Advise regarding flying and diving. Thoracic surgery may be necessary
• Avoid air travel, if possible, due to risk of pneumothorax
• Transplant Single (usually) or double lung, or heart-lung. LAM can recur in the transplanted lung
• Stop smoking, as this accelerates the rate of decline
• Influenza vaccine.
is very variable. The condition usually slowly progresses to respiratory failure. At 10y, 55% of patients have MRC grade 3 dyspnoea, 23% are on LTOT, and 10% are housebound. Survival: 70% of patients are alive at 10y, 33% are alive at 15y, and 25% are alive at 20y.
A rare genetic cause of chronic respiratory disease, usually encountered in adult respiratory clinics as a cause of bronchiectasis. Cilia are found in:
• The whole length of the upper respiratory tract
• Brain ventricles
• Fallopian tube/ductus epididymis.
They are made up of dynein arms, with outer and inner connecting rings, and beat at 14 beats/s. Many gene defects have been identified in PCD, causing a number of cilial abnormalities.
Abnormal cilia do not beat normally, leading to reduced mucociliary clearance, microbiological colonization (which further inhibits cilial action), chronic infection, and the development of bronchiectasis.
The main aim following diagnosis in childhood is the prevention of chronic respiratory disease and bronchiectasis.
• Autosomal recessive, >200 phenotypes
• May present with neonatal respiratory distress
• Situs inversus (in about 30%, as cilia determine the side of the organs. Random organ siting occurs with cilial dysfunction, hence the situs inversus of Kartagener’s syndrome)
• Nasal blockage/rhinitis
• Persistent wet cough in childhood
• Hearing problems/history of glue ear/grommets in childhood
• Clubbing and signs of chest disease are rare in childhood
• Wheeze in 20%
• Infertility due to immotile sperm (sperm tails have same morphological defect as the cilia and do not beat correctly)
• In adults, the disease usually presents with the clinical signs of bronchiectasis: cough productive of purulent sputum, recurrent chest infections, intermittent haemoptysis.
Saccharin test (see pp. [link]–[link]). Nasal NO is very low in PCD (possibly because NO mediates ciliary function); this is the most sensitive and specific screening test. Cilial biopsy via the nasal route. Cilia are examined by high-speed digital video where their beat frequency and pattern can be assessed, confirming the diagnosis. Most cases of PCD are diagnosed in childhood. There is an increased frequency in the children of consanguineous marriages.
Consider the diagnosis in:
• Situs inversus
• Persistent upper and lower respiratory infection from early childhood
• Infertility—♂ may present in infertility clinics.
A national service for the diagnosis of PCD was set up in 2007, with three centres—London (Royal Brompton), Southampton, and Leicester.
• Management of haemoptysis.
Bush A, Hogg C. Primary ciliary dyskinesia: recent advances in epidemiology, diagnosis, management and relationship with the expanding spectrum of ciliopathy. Expert Rev Respir Med 2012;6:663–82.Find this resource:
Patient support group. http://www.pcdsupport.org.uk.
PAP, also referred to as alveolar lipoproteinosis, is a rare alveolar filling defect affecting around 3 per million people. There is a limited published literature: five reported case series of ≥10 cases, and only 410 total cases reported.
PAP is due to failure of alveolar macrophages to clear spent surfactant, leading to the filling of alveoli with a phospholipid proteinaceous material. It is thought that the defect has an autoimmune basis and, in the idiopathic form, is due to the presence of antibodies to granulocyte-macrophage colony-stimulating factor (GM-CSF), which cause inhibition of normal alveolar macrophage function, leading to abnormalities of surfactant homeostasis. Defects in GM-CSF signalling have been identified in animal models. Congenital disease is thought to be due to mutations in surfactant gene proteins. Other mechanisms for surfactant accumulation have also been identified:
• Heavy dust exposure leads to surfactant hypersecretion, which exceeds the lungs’ normal clearance mechanism. Animal models have shown that this condition develops from endogenous lipoid pneumonia, with the accumulation of lipid-laden macrophages, which break down to release surfactant
• Amphiphilic drugs, e.g. amiodarone, chlorphentermine
• Lymphoma, leukaemia, and immunosuppression The mechanism is uncertain, but it is thought that the lipoprotein may be generated from degenerating alveolar cells.
Appearances similar to alveolar lipoproteinosis may also be seen in endogenous lipoid pneumonia resulting from bronchial obstruction and are described in surfactant-secreting alveolar cell carcinoma.
The alveoli are filled with a granular acellular eosinophilic PAS (periodic acid–Schiff)-positive deposit. Cholesterol clefts and large foamy macrophages may also be seen. The alveolar architecture is usually well preserved. Surfactant protein can be identified using immunohistochemistry. Electron microscopy shows multiple osmiophilic bodies, consistent with denatured surfactant.
• Presents aged 30–50 (case reports in children and the elderly)
• ♂:♀ ≈ 4:1
• Increased incidence in smokers
• Rare familial cases reported.
• Typically presents with breathlessness and a non-productive cough. Examination may be normal, or crackles may be heard on auscultation. Clubbing in one-third
• May present with superadded infection, causing an apparent acute onset of symptoms, in association with fever
• Median duration of symptoms before diagnosis is 7 months
• Opportunistic infection is the major complication, most commonly Nocardia species, fungi, and mycobacteria. This occurs due to impaired macrophage function and impaired host defence due to surfactant accumulation.
is usually made on the basis of a characteristic CT appearance, although other tests may also be useful.
• Raised serum LDH
• ABGs Hypoxia and increased A–a gradient
• PFTs Restrictive defect, with reduced lung volumes and transfer factor
• CXR Bilateral consolidation with thickened interlobular septa. Usually bilateral. The pattern is very variable and, in up to 50%, may be perihilar (bat-wing appearance)
• CT appearance is characteristic, with airspace shadowing in a geographical distribution, alternating with areas of normal lung, the so-called ‘crazy paving’ pattern. This CT appearance is not specific to alveolar proteinosis but is also seen in lipoid pneumonia and bronchoalveolar cell carcinoma
• BAL reveals milky washings. Identification of antibodies to GM-CSF in BAL washings is diagnostic. Cytological examination shows a granular extracellular deposit with foamy macrophages and cellular debris
• Transbronchial/open lung biopsies when CT is not characteristic.
of choice is repeated therapeutic whole lung lavage, which should be performed at a specialist centre. There are no RCTs of this treatment, but there is evidence of efficacy in terms of subsequent improvement of symptoms, physiology, and radiology.
• The indication for whole lung lavage is usually breathlessness, limiting activities of daily living
• The procedure is done under general anaesthesia using 100% O2 and one-lung ventilation using a double-lumen tube. Repeated warm saline lavage using a closed circuit continues until the bronchial washing returns are clear—this may take up to 40L lavage. One or both lungs may be treated at a time
• The response is variable—some patients need only one treatment; others may need multiple treatments, and about 10% fail to respond
• May be done on bypass if the patient is very hypoxic
• Characteristic milky lavage fluid is obtained
• Granulocyte colony-stimulating factor (SC injections) is a novel treatment option (only phase II studies, no RCT yet), which may prevent progression of disease
• There is no benefit from treatment with steroids, and they may exacerbate opportunistic infections.
with whole lung lavage is generally good. Spontaneous remission occurs in one-third; one-third remains stable, and one-third progresses to respiratory failure and death. There are reports of progression to pulmonary fibrosis (which may be a coincidental occurrence).
• PAVMs are abnormal blood vessels replacing normal capillaries, making a direct low-resistance connection between the pulmonary arterial and systemic venous circulations. They vary in size, from tiny clusters of vessels (telangiectasia) to larger, more complex aneurysmal-type sacs
• The disorder is rare, affecting 1 in 15, 000–24, 000
• Several genetic susceptibility loci have been identified on Chr 9 and Chr 12. One identified mutation is in the endoglin gene. This modulates signalling via the TGF-β family of growth factors. This gene is also implicated in the development of PPH
• Subjects with significant PAVMs have low pulmonary vascular resistance, a low mean PAP, and a high cardiac output—due to long-standing adaptive mechanisms to the effects of the shunt, in addition to vascular remodelling effects
• Most patients present post-puberty, as AVMs probably develop at this time. They probably grow throughout life, especially during puberty and in pregnancy. They may rarely regress spontaneously.
• Most patients present with an abnormal CXR, classically showing a smooth, rounded intrapulmonary mass, with draining or feeding vessels
• Mild hypoxaemia An AVM is a direct communication between the pulmonary artery and pulmonary vein. Blood therefore bypasses the pulmonary capillary bed, with reduced oxygenation, which poorly corrects with supplementary O2
• Orthodeoxia is desaturation on standing, due to an increase in blood flow in the dependent lung areas. 70% of PAVMs are basal, hence the desaturation seen
• CT identifies all AVMs and can determine those suitable for embolization. Contrast is not required
• Patients may present with the complications of a PAVM, particularly bleeding or peripheral abscess formation. The absence of a normal filtering capillary bed means small particles can reach the systemic circulation, leading to sequelae, particularly in the cerebral circulation—strokes and cerebral abscesses. These abnormal vessels are also at risk of rupture.
• 100% O2 rebreathing study, a non-invasive method of shunt quantification
• 99mTC perfusion scan, a tracer study; the size of the shunt can be assessed from the proportion of radiolabelled macro-aggregates reaching the systemic circulation, compared with the total number injected. In a normal study, aggregates accumulate in the kidneys
• Contrast echo to measure the circulatory transit time of injected echo contrast
• In normal individuals, the anatomical shunt is <2–3.5% of the cardiac output (due to post-pulmonary drainage of bronchial veins into pulmonary vein and drainage into the left atrium).
• Asymptomatic (50%)
• Haemoptysis (10%), probably due to additional bronchial telangiectasia, which can also cause haemorrhage into bronchi or the pleural cavity
• Chest pain (12%); aetiology is uncertain
• Vascular bruits
• Telangiectasia; 80% of PAVM patients have HHT, and their families should be screened because of the risk of stroke (see p. [link])
• May present with acute stroke, with focal neurological signs.
is usually done with coils, which generate local thrombin, leading to cessation of blood flow in AVM feeding vessels. This results in a reduction in the right-to-left shunt and improvement in hypoxaemia and should be done by an expert in a specialist centre only. The small risk of neurological sequelae and angina/arrhythmias is reduced with operator experience.
60–70% of patients are left with a small persisting shunt following treatment and retain a small risk of abscess formation. Patients are therefore given prophylactic antibiotics for dental and surgical procedures (ensure the patient has a MedicAlert card).
the majority of patients with PAVMs have HHT, and so screening of family members is important.
all patients need regular follow-up, with shunt assessment post-surgical resection or embolization, as removal of one shunt may unmask or provoke the development of others.
• PAVM patients never die of respiratory failure in the absence of additional respiratory disease
• All patients are at risk of stroke and cerebral abscesses
• Transient ischaemic attack/stroke (in 25%) due to rupture of abnormal capillaries in aneurysms
• Abscess (in 10%) due to paradoxical emboli, through the right-to-left shunt, and the absence of a filtering capillary bed.
is associated with an increase in size of AVMs, and new ones may develop, with potentially catastrophic consequences. Careful shunt assessment is therefore needed prior to pregnancy, with contraceptive advice prior to specialist assessment. Close liaison between the specialist centre and obstetric team is paramount. AVMs may need embolization in the third trimester to allow safe delivery.
These are essentially warts of the upper respiratory tract, caused by the human papillomavirus (HPV 6 or 11). The virus infects epithelial cells and mucous membranes, similar to that seen in cutaneous and anogenital infection. The infection is most commonly acquired during ororespiratory exposure from the mother during vaginal delivery and typically presents in childhood from 6 months onwards, with signs and symptoms of URTI. It may also present for the first time in adulthood. It is associated with HLA-DR3 and with sexual transmission in adults. Recurrent respiratory papillomatosis is rare (2 per 100, 000), but oral HPV infection is common.
This is variable.
• May remit spontaneously
• Progressive voice loss and airway obstruction
• Most cases are confined to the larynx, although up to 25% of patients subsequently develop extralaryngeal spread to the bronchial tree
• 1% have malignant change to squamous cell carcinomas.
• Surgical excision to maintain airway patency
• Laser therapy—but potential problems of thermal injury, stricture formation, and spread of papillomas
• PDT reduces recurrence rate, using oral or IV photosensitizing agent, then a laser to destroy photosensitive tissue
• Microdebrider is now used more commonly
• Medical treatment—interferon, aciclovir, ribavirin, isotretinoin, and methotrexate have all been tried
• Interferon alfa as a daily SC injection leads to complete remission in 30–50%, and partial resolution in 30%. One-third recur when treatment is stopped. Adverse reactions are common: flu-like symptoms, deranged LFTs, leucopenia, and alopecia
• Cidofovir is a nucleoside monophosphate analogue and inhibits viral polymerase. It is given as an intralesional injection. Theoretical side effects include nephrotoxicity and neutropenia but have not been seen in practice.


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CBN hikes interest rate to 16.5% to tackle inflation
In an ongoing effort to curb inflation and relieve pressure on the naira, the Central Bank of Nigeria on Tuesday increased its benchmark lending rate to 16.5 per cent.
This was announced by CBN Governor Godwin Emefiele at the conclusion of the Monetary Policy Committee meeting in Abuja.
The apex bank explained that more tightening was necessary as prior increases were starting to show results.
Along with maintaining the MPR’s asymmetrical corridor at +100 and -700 basis points, the committee opted to keep the Cash Reserve Ratio (CRR) at 32.5 percent.
The apex bank also retained the 30 per cent liquidity ratio.
The central bank has maintained its strong attempts in the foreign exchange market to support the country’s plunging currency, even as inflation rate continued to rise.
In October, Nigeria’s inflation rate reached a 17-year high of 21.09 percent.
Peoples Gazette reported in October that the apex bank had announced intentions to redesign naira notes as part of efforts to stabilise the country’s sinking currency.
In order to absorb liquidity, the CBN boosted the cash reserve requirement (CRR) early this year to a minimum of 32.5 per cent.
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Man dead after shooting at gas station on Wrightsboro Road
One man is dead after a shooting Friday morning at a Wrightsboro Road gas station.
About half a dozen Richmond County Sheriff's Office patrol cars, a fire truck and an ambulance were parked outside of the Smart Gas Station on Wrightsboro Road around 11:30 a.m.
A witness on scene told The Augusta Chronicle she heard gunshots and then saw a man laying on the ground. Reporters observed a man being lifted off the ground and loaded into an ambulance as police raised caution tape.
Just after 1:30 p.m. the sheriff's office reported a male victim was shot at least once and was transported to the hospital. He later died from his injuries.
Investigators have two subjects in custody, but have not released their identities, according to the sheriff's office.
Breaking news. This story will be updated as new information becomes available.

domain: news


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Two Engineering Programs Earn State Recognition
The Kentucky Engineering Technology Education Association (KETEA) has chosen the engineering programs at Leestown Middle and Bryan Station High schools as models for the state. They will receive 2023 Program of the Year honors at the KETEA fall conference in mid-November.
The awards committee noted Leestown’s commitment to engaging students in project-based learning experiences that empower them for life-long success. The program also illustrates community collaboration through a Family Engagement Night featuring students’ work.
Leestown’s Hayley Bromagen said Pre-Engineering provides opportunities for a diverse group of students to test out STEM careers. “Our program encourages them to be critical thinkers and creative problem solvers as they explore engineering concepts,” she added. “We believe that students who have a strong foundation in technology skills, an ability to collaborate with people from diverse walks of life, and experiences in the engineering process will be rewarded with opportunities and choice in how they will create fulfilling and meaningful lives.”
The KETEA committee picked Bryan Station High because of the huge lift in rebuilding the program over the past six years, including strong collaboration with English, Math, and Science teachers. In addition, the panel found that program leaders see the value of the Career & Technical Student Organization, and they have grown the Technology Student Association membership.
Rusty Willhoite, an instructor in the school’s Academy of Engineering, Manufacturing, and Robotics, also noted how their students have an outstanding success rate in industry certifications.


(Bismarck, ND) -- Senator Kevin Cramer helped announce Wednesday the U.S. Department of Agriculture's awarding of the following grants and loan totaling $6,058,020:
- $2,644,000 loan and a $747,020 grant to the City of Ellendale to replace sewer pipe and manholes for the city;
- $2,000,000 to the Turtle Mountain Band of Chippewa Indians to construct sewer improvements, replace sewer mains and sagging pipes with new PVC pipe, and install new manholes; and
- $667,000 to the Turtle Mountain Band of Chippewa Indians to construct a water reservoir and booster station and provide a reliable supply of water to the water treatment plant.
The funds come through the Water and Waste Disposal Loan and Grant Program, which provides funding for clean and reliable drinking water systems, sanitary sewage disposal, sanitary solid waste disposal, and storm water drainage for households and businesses in eligible rural areas with populations of 10,000 or less.


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Unprecedented climate and temperature whiplash scenarios are wreaking havoc on agricultural production all over the world. How long till the grocery store shelves are empty? Though countless forms of human activity have contributed to the decimation of our once thriving planet, geoengineering programs are the most destructive of all. The combined and cumulative consequences of ongoing global climate engineering operations are rapidly eroding the planet's remaining life support systems. The converging cataclysms we collectively face are all connected, this includes the unfolding and expanding CV-19 scenario. All are needed in the critical battle to wake populations to what is coming, we must make every day count.
Just a few days ago, I wrote an article (The Supply Chain Is Broken and Food Shortages Are HERE) detailing the disruptions to the US supply chain and explained how food shortages are coming to the United States. In fact, I pointed out how they are already here, but many Americans simply don’t know it yet. After all, mainstream media hasn’t been telling them about the disruptions to the supply chain as a result of COVID lockdowns and questionable strikes nor is it telling them about how many crops have been destroyed as a result of wildfires in the West of the country, straight-line winds in the middle, or flooding and hurricanes in the south. As I wrote in my last article, the food supply chain is broken. I asked the readers if they have experienced shortages in their areas. Take a look at the comments. People are seeing shortages all over. And they are seeing those shortages now, not three months from now. If you’re concerned about the broken supply chain, now is the time to start stocking up on food. You do not have much time left before the items you can grab now are gone and gone for good.
Wonder why there is a canned food shortage at your local grocery store? Washington State has been stocking away millions of dollars of non-perishable food — so have other US states, and the federal government — in anticipation of “the need ahead.” If states are preparing, so too must you be today. And spread the word. SHARE THIS!


Dance, Dance: Florida State earns No. 2 seed in NCAA Tournament
Ah, March – gentle breezes, springtime warmth and the NCAA Tournament. Florida State learned of its fate Monday, as the women's basketball team earned their roadmap to Tampa Bay, the journey beginning Saturday at the Tucker Center as a No. 2 seed, taking on 15-seed Alabama State 1:30 p.m.
It was a season unlike any in their history. Florida State, with its influx of new talent, raced through the regular season, piling up 27 wins. It saw two nine-game win streaks, an undefeated home record, head coach Sue Semrau bring home her third ACC Coach of the Year award and the Seminoles earn their highest ever seed in the NCAA tournament.
FSU finds itself behind only South Carolina in the Greensboro Region. The Gamecocks played 22 games before their first loss and held down the top spot in the AP ranking for 11 weeks, before stepping down to No. 2. South Carolina will be hosting its first round matchup against Savannah State.
Tallahassee and Columbia will both host the first two rounds of the tournament, offering home-court advantage to two teams with undefeated home records.
The winner of FSU and Alabama State will play the winner of (10) Oklahoma State and (7) Florida Gulf Coast. The high-flying Eagles are riding a 25-game win streak and have only lost twice this season, both before December, one by three points and the other in double overtime. Oklahoma State, on the other hand, stumbled to a 9-9 conference record and zero top-25 wins. The second round matchup will take place on March 23.
The Seminoles are a confident bunch, and rightfully so. Florida State (29-4, 14-2) has already played three teams in their bracket, beating Savannah State and Syracuse before losing at North Carolina. In their 13 games against NCAA tournament opponents, the Seminoles are 9-4, with losses to UNC, Washington, and two to Notre Dame.
Florida State is coming off an impressive run in the ACC Tournament. After finishing the regular season on a five-game win streak, FSU easily dispatched Virginia Tech and hung on to beat Louisville for the second time this season. Awaiting them, however, was Notre Dame.
The two teams met on January 2 in South Bend and the Irish prevailed, 74-68. "We were still so young and finding our identity," Semrau said about their first meeting.
The identity crisis didn't last much longer, though, as FSU rattled off nine-straight wins, including wins over Duke, Louisville, and Syracuse. The emergence of guard Leticia Romero, along with Maegan Conwright and Ivey Slaughter, gave this team a stifling defense and unrelenting rebounding.
The Seminoles outrebounded each and every one of their opponents until Notre Dame brought down 39 boards to FSU's 27. Predictably enough, FSU couldn't match up and ultimately fell 71-58.
"Our confidence is built on reality… They've reaped the benefit of their hard work and so having reaped some of that you go in believing you're going to reap it again," Semrau said of the upcoming NCAA tournament.
While the loss to Notre Dame stung, this team knows how good it can be. This team is more than just the talent of the starting five; it's built on the depth of players like Emiah Bingley, Shakayla Thomas, Shakena Richardson.
The other two matchups in Tallahassee this week will be (6) Texas A&M vs. (11) Arkansas – Little Rock and (3) Arizona State vs. (14) Ohio. The winners of those two games will meet on March 23.
Columbia's side of the bracket includes (8) Syracuse vs. (9) Nebraska, (5) Ohio State vs. (12) James Madison, and (4) North Carolina vs. (13) Liberty. The winner of South Carolina and Savannah State will play the winner of Syracuse and Nebraska. The winner of Ohio State and JMU will take on the winner of UNC and Liberty.
All opening round games will be televised on ESPN2. General admission to the Tucker Center will be $7, just $5 for students and faculty.


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Effects of propranolol and metoprolol on the peripheral circulation.Br Med J 1978; 2 doi: https://doi.org/10.1136/bmj.2.6152.1598 (Published 09 December 1978) Cite this as: Br Med J 1978;2:1598
- P D McSorley,
- D J Warren
The effects of single doses of propranolol and metoprolol on skin temperature and skin and muscle blood flow were compared in 10 normal subjects and four patients with essential hypertension. In normal subjects the mean skin temperature fell by 1.30 +/- 0.62 degrees C 90 minutes after 80 mg propranolol and 0.15 +/- 0.05 degrees C after 100 mg metoprolol. Skin blood flow and resting muscle blood flow were not affected by metoprolol but fell significantly after propranolol. Both drugs reduced post-exercise muscle hyperaemia, propranolol by more than metoprolol. Similar changes were seen in the hypertensive patients. Propranolol should be used with care in patients with known vascular disease.


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* Retailer information may vary - to confirm pricing, shipping and return information, please contact Chico's.
These mixed-metal drop-hoop earrings take chic to the next level with double goldtone hoops and circlet embellishments. Drop-hoop design. Dimensions: 1.625" x .75". Zinc, brass, steel. Imported. Part of the Delight collection. Custom designed exclusively for Chico's. Handcrafted with nickel-free metal.

domain: artistic


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A HAMMIER HORROR DOUBLE BILL
HAMMIER HORROR 2
This is a fully independent review of the books written by Phillip J Alexander, obtained on behalf of the publishers, New Millennium. Should anybody wish to purchase copies, they are available through all good bookstores in the UK.
A Hammier Horror Double Bill
by Phillip J Alexander
“The Curse of the Blood Monster”
This is an immensely funny read presented somewhat like a film script, which is appropriate as it is a general send-up of the film genre and it works very well. It is black humour with lots of tongue-in-cheek sex and violence. The main protagonist, Professor Winkel, who features in both stories, plays the role of a detective of the occult and supernatural.
The settings for the two stories are based on classic Hammer Horror films with remote village in darkest Europe or ancient archaeological sites and temples, suspicious inhabitants, who speak with Cornish accents, woods, mist, buxom ladies, and the baron in the castle on top of the hill where no man dares to go.
In the first story, Professor Winkel is called in when newly-weds Gerald and Alice want to investigate the death of Gerald’s brother. Gerald discovers that there have been 240 deaths where the victims are left as flat as pancakes.
After almost everybody close to Gerald and Alice have been flattened, Professor Winkel’s research has solved the mystery. We find out that the Baron’s daughter was given to a remote tribe of Indians in exchange for his life. After some weird ritual she is able to metamorphose into a giant spider that stuns her victims and sucks out the contents of the bodies, leaving only the skin. Spider-woman wants Gerald as her consort but Professor Winkel discovers that South American water snake venom will defeat the monster and sets off to obtain the venom. He returns just in time to find the villagers storming the castle, which is then blown to bits as Winkel administers the antidote.
“The Curse of the Mummy’s Boy”
The second story is equally comic and involves stiff -upper-lip British Egyptologists in a mummy curse adventure set among ancient tombs. This is full of sexual innuendo with a transvestite son of a high priest and a giant penis artefact. When victims are later found torn apart, it is time to call in Professor Winkel. The now familiar occult doctor solves the mystery after some hilarious, highly imaginative, scenes. There are lots of funny lines and situations in these stories and I believe most people would find the book hugely entertaining. They would make very good film comedies. The writing is of good standard and the style is amenable to easy reading.
Hammier Horror 2
by Phillip J Alexander
“The Curse of the Zombies”
This is a witty, amusing and sometimes deliciously gory turn-of-the-century tale of voodoo curses, zombies and other supernatural goings-on in the West Indian island of Haiti and its Scottish namesake, Hightae.
The scene is skilfully set at the beginning with Nosbert’s pledge to bury his father in his birthplace, and while the confusion between the two places is a rather obvious joke, as are most in this book, this very obviousness is part of the tongue-in-cheek approach, and generally it works well.
The author’s eminently readable style, well-constructed plotline and the light-hearted pleasure he clearly got from writing the novel are a winning combination, and the reader can settle back to enjoy what is essentially a good literary laugh from a writer who does not take himself or his genre too seriously, and understands the secret of ham.


The IRIS instrument can measure up to 50 different types of instruments, including string instruments and percussion instruments.
It’s an inexpensive, simple way to measure musical instruments.
IRI uses a laser to shoot a beam of light into the instrument, which then bounces off the instruments.
IRIS will also measure how many times each instrument is hit.
The IRISE instrument is also designed to be portable.
It can be carried around the house, put in a backpack, or carried in a purse.
And IRIS can also measure the instruments that come with a guitar, piano, or other instrument.
The new IRIS instruments will be used to measure and record sounds, not just instruments.
In addition to measuring the instruments, IRIS is also used to record information about them.
The instruments will include a microphone, speaker, microphone jack, and power supply.
The instrument can also be connected to a microphone for sound measurement.
IRISE can also record sound waves for analysis.
IRIION, the instrument that will measure strings, is a different instrument altogether.
IRIV, the IRIS Instrument, is the instrument IRIS uses to measure sound waves.
IRIIS is a low-cost, portable instrument that has been developed with the help of $500,000 in federal funding from the Defense Department and the Department of Homeland Security.
IRICON, which is IRIS’ first instrument, is designed for military use.
IRIT, the infrared instrument, was designed by IRIS and is a cheap, portable, and portable instrument.
IRIA, the Interdisciplinary Instrument, was created by the National Instruments Research Center.
IRIO, the Instrument of Inertia, is intended to be used for biomedical research.
IRON, or Instrument of Iron, is IRI’s most expensive instrument, costing about $100,000.
IRIRIS is currently available for $99, the same price as IRIICON.IRIS was designed to make it easier for people to measure the properties of the instruments they are working with, according to the IRI website.
It has an IRIS mic and IRIS speaker, which are included with IRI.
IRSI also sells IRIS-based instruments in the United States and Europe.
The IRIS team, which has been working on IRIS for nearly a decade, is based in the lab of Dr. Mark G. O’Brien, IRI CEO and president.
O-Brien said that IRIS has the potential to be a “very powerful instrument.”
He said that in the future, IRICon and IRIAon could be built into IRIS.
O-Brien also told Recode that IRICons and IRI-based IRIS Instruments will become more affordable as technology improves.
IRi has partnered with a variety of manufacturers and distributors to help the instrument reach the marketplace.
For example, the company recently partnered with the National Science Foundation to develop IRIS hardware for commercial use.
It is currently building IRIS products for the military and commercial clients.
IRIBon is the company that manufactures IRICones, which IRICony uses for testing.
IRIDON is the IRII Instrument, which uses IRI for measurements.
IRIEon is a handheld IRICone instrument.IRI, IRi, and IRICono are all owned by O-Briens.
OBrians said that when the instruments first came out, he was surprised by how many people were using them.
I really did not expect the volume of people who were using it.
And so, I’m really pleased to say that we have gotten quite a lot of people using it over the last few years.
I’m also really pleased that people have started using it, I believe that it has given the world some really wonderful instruments.
O’Brien said IRIConic, IRIAonic, and iridonic instruments are the new IRIControl Instruments.
IRICEonic is an IRI instrument for measuring strings and instruments.
OBIOControl is an instrument for analyzing and monitoring electrical signals.
IRICAontrol, IRIEontrol and IRICAonic instruments measure sounds.IRi, IRII, and the IRICond instrument is a portable instrument designed for personal use, according the IRi website.
IRISAontrol for measuring sound waves, is also an IRII instrument.
A handheld IRIocontrol instrument that measures sounds.


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Saying goodbye to our beloved pets is incredibly difficult. Sometimes it is sudden; other times we have a little time to prepare.
But it's never enough time.
CELEBRATION OF LIFE/RAINBOW BRIDGE SESSIONS
This session is designed to help with the transition, because even though we know our fur babies will be waiting for us at the bridge -- we need something now to hold and cherish.
Your dog's comfort is our priority while we capture your relationship and what has made your dog's life so important and beautiful. For this reason, we come to your home or meet you at your dog's favorite place(s).
Our Celebration of Life/Rainbow Bridge Session includes:
A custom-made session tailored to highlight your dog's wonderful life and his or her special relationship with you.
Onsite image review and selection immediately following the session. We feel reviewing images with your sweet pupper in your arms is the least painful way to make your selections.
(Please note: The Celebration of Life/Rainbow Bridge Session is for the session only: prints and products are ordered a la carte to afford you the greatest flexibility.)
Most clients enjoy the flexibility of choosing their products from our a la carte menu. Many of our clients create an album of their images or ave us design and create an album for them. Also popular are our folio boxes with fine art, matted prints that can be displayed in the box itself, in a frame of your choice, or in one of our modern, acrylic mounts or heavy blocks.
Celebration of Life Dog Portraits | Rainbow Bridge Dog Portraits | Jacksonville Dog Photographer | Florida Pet Photographer | Jacksonville Beach Dog Photographer| Award-winning Dog Photographer | Neptune Beach Dog Photographer | Atlantic Beach Dog Photographer | St Augustine Pet Photographer | Joan H Walker PAWtography

domain: pet