text:
The quartet went into Rudy Van Gelder's studio on April 27, 1964, and performed all five of the songs on this album as well as a short version of "Song of Praise"—which was recut in May 1965 and compiled on The John Coltrane Quartet Plays. They returned to the studio on June 1, 1964, and recorded versions of the title track and "Bessie's Blues", which ended up on the album. The three rejected recordings from April 27 are compiled on The Classic Quartet: The Complete Impulse! Recordings.
The music represents Coltrane's return to meticulous form and structure, and post-bop modality, after several years of free-form experimentation alternating with traditional balladeering. The album's closing track is an improvisational feature for Jones' drums (with spare melodic accompaniment from Coltrane's tenor sax and Garrison's bass at the song's beginning and end): Coltrane would continue to explore drum/saxophone conversations both in live performances with this group and on subsequent recordings such as the posthumously released Interstellar Space (with Rashied Ali). Furthermore, Coltrane does not solo at all on side two of the original LP; the ballad "Lonnie's Lament" instead features a long bass solo by Garrison.
The album's liner notes are written by Nat Hentoff, and the original LP's inner gatefold profile photograph of Coltrane was also featured on the cover of Coltrane's next Impulse! album release, A Love Supreme.

domain: entertainment


SOCIAL SECURITY ACT, Payment of Benefits when due
CITE AS: California Human Resources Department v Java, 402 U.S. 121 (1971).
Appeal pending: No
Claimant: Judith Java
Docket No: U.S. S.Ct. 507 (1970)
UNITED STATES SUPREME COURT HOLDING: Benefits due a claimant after an initial finding of eligibility may not be held in abeyance pending the employer's appeal.
FACTS: Claimants were discharged from employment. They applied for benefits. They were given an eligibility interview which the employer could have, but did not, attend. As a result of the interview both claimants were found eligible for benefits and received benefits. The employer then appealed. At that point payments automatically stopped in accordance with California law and practice. At the Referee level, Hudson was ruled eligible but Java was found to be ineligible.
The procedure used by California in stopping payment of benefits upon employer protest resulted in a median 7 week delay in payments to eligible claimants. Employers were successful in less than 50% of appeals.
DECISION: Procedure used by California was not in compliance with the Social Security Acts' directive to pay unemployment compensation "when due".
RATIONALE: The Social Security Act requires administration of the Unemployment Compensation Fund in a manner reasonably calculated to insure full payment of benefits when due. The objective of Congress was to provide for benefit payments on the nearest pay day following the termination of employment to the extent administratively possible in order to provide the unemployed worker with cash at a time when he/she would otherwise have nothing to spend. "When due" as contained in Section 303(a)(1) of the Social Security Act is construed to mean when benefits are allowed after a hearing of which both parties have notice and have an opportunity to present their respective positions.


text:
According to the Broward County Supervisor of Elections, eligible voters will be removed from the voting rolls as a result of the massive voter purge ordered by Governor Rick Scott. “It will happen,” Mary Cooney, a spokeswoman for the Broward County Supervisor of Elections, told ThinkProgress.
Late last year, Governor Scott ordered his Secretary of State, Kurt Browning to “to identify and remove non-U.S. citizens from the voter rolls.” Browning could not get access to reliable citizenship data. So Scott urged election officials to identify non-U.S. citizens by comparing data from the state motor vehicle administration with the voting file.
That process produced a massive list of 182,000 names, which Browning considered unreliable. The Fair Elections Legal Network, which is challenging the purge, noted that database matching is “notoriously unreliable” and “data entry errors, similar-sounding names, and changing information can all produce false matches.” Further, some voters may have naturalized since their driver’s license information was collected.
Browning resigned in February. But Scott has pressed forward with his efforts to purge voters from the rolls based on the dubious list. Here’s the letter Maureen Russo, a U.S. citizen and registered voter in Florida for the last 40 years, received two weeks ago:
In Broward County 259 people recieved letters just like the one addressed to Maureen above, according to the Broward County Supervisor of Elections. So far only 7 (including Maureen) have responded to the ominous and legalistic letter. Five of the responses included proof of citizenship.
If the other 252 people don’t respond within 30 of recieving the letter — a deadline that is rapidly approaching — they will be summarily removed from the voting roles. Cooney, the Supervisor of Elections spokeswoman, says some of those who are purged under this “very new” process will “be eligible” but will have to be removed from the rolls anyway.
Rep. Ted Deutch (D-FL) and other Democratic members of the Florida Congressional delegation — as well as a coalition of voter protection groups — have called on Scott to “immediately suspend” the voting purge since the lists of ineligible voters has proven extremely unreliable.

domain: sociology


text:
Opposition March Scheduled for Thursday, May 9, is Post-Poned Due to International Community Pressure
[NOTE: Apologies for the sparse amount of news posted to this site over the last week. After fixing some computer problems, we plan to be back in full swing.]
Today, the opposition met with international dialogue facilitator, Said Djinnit, who made a last ditch effort to get the opposition to suspend its plans for a march tomorrow. Evidently, Djinnit was able to make the case to the opposition that Alpha Conde was open to making concessions in a dialogue framework in a variety of areas including the most controversial, getting rid of Waymark. While the opposition knows not to trust Conde’s new-found conciliatory attitude, the opposition will have to determine how duplicitous the international community’s need is to keep the opposition off the streets and behind closed doors. If the opposition intends to keep the confidence of the people of Guinea, it must withdraw from the talks at the first “whiff” of Alpha Conde and/or international community maneuvering. In a dialogue framework, facilitators and the Conde government have significant control over the opposition. When the opposition is in the streets, it has both facilitators and Conde off-kilter and, therefore, more leverage.
The dialogue facilitators issued the following press release posted on the aminata.com site. Translated into English via Google.
We facilitators of the Guinea dialogue:
Considering the joint statement dated 23 April 2013, by which the parties to the political process in Guinea, including the Government, the political parties of the opposition and the presidential majority, are committed to exercise restraint, to avoid any form of violence, and to peacefully resolve their differences;
Taking into account the urgency to work together for political, inclusive, peaceful process, democratic and non-violent in the upcoming elections, we express our deep concern at the escalation of violence on the occasion of public events and the risk that such a situation is for peace, security and social cohesion;
Reiterate in a solemn manner that all parties concerned contribute to call an end to incidents of violence;
Encourage all forces of the nation including institutional managers, community leaders and religious authorities and the media, to work tirelessly to preserve peace and social cohesion, as well as return Guinea to a peaceful atmosphere;
Reiterate that dialogue is the only alternative to resolving political differences;
Reiterate our call to all signatories of the Declaration of nonviolence April 23 to scrupulously respect their engagement with parties;
Urge everyone in his case, to show responsibility and citizenship in order to preserve peace and social harmony;
Take this purpose for the parties to resume dialogue without delay, and come in a spirit of opening to find the necessary compromises to create a climate conducive to the holding of elections in a peaceful environment and under conditions of transparency requirements;
To help restore a climate of appeasement necessary to restore the dialogue, we call on all parties to refrain from any action in the coming days that bears the risk of hindering the momentum towards peace.
In this regard, we welcome the decision of the opposition to postpone the event, which was scheduled for this Thursday, May 9
Tomorrow, facilitators will undertake consultations with stakeholders to discuss ways and means for a return to dialogue as soon as possible.
Made in Conakry, May 8, 2013

domain: news


text:
|www.BedandBreakfast-Directory.co.uk - The Bed and Breakfast Directory Website.|
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domain: geography


On Friday, April 15, 2016, a new policy published by the Texas Department of Criminal Justice (TDCJ) took effect. It prohibits Texas inmates from "maintaining active social media accounts for the purposes of soliciting, updating, or engaging others, through a third party or otherwise" -- and raises serious First Amendment concerns.
The first problem is obvious: Texas inmates don't have access to the Internet. Instead, inmates are allowed to communicate with family and friends through written letters and in-person visits, all of which TDCJ already monitors.
For Texas inmates to "maintain active social media accounts," they must ask family or friends to post messages on their behalf. And they do so for a variety of reasons. Inmates sometimes use social media to generate public support for a challenge to a verdict or sentence, for example. Other inmates may use social media to express their feelings about incarcerated life, a social outlet that often helps prepare inmates to reenter society successfully.
Whatever the reason, inmates' speech on social media occurs outside prison walls, facilitated by non-prisoners. That is what Texas seeks to prohibit, with blatant disregard for the First Amendment. The government has absolutely no authority to silence chunks of the population because they associate with individuals who are incarcerated.
Texas claims that social media provides a way for inmates to "sell items over the Internet based on the notoriety of their crime, harass victims or victim's families, and continue their criminal activity." But this position cannot justify the burdens the new policy imposes on First Amendment rights.
To start -- because it bears repeating -- this policy will largely impact the First Amendment rights of people who are not in prison. Plus, TDCJ already has a means of regulating the information it purportedly seeks to prohibit through its ability to exercise control over the flow of information in and out of prison. It's not clear what this new social media policy adds.
The policy already has had a chilling effect on the speech of those who are afraid of running afoul of its elusive mandates. Many inmates' supporters have begun taking down websites and social media accounts they've established on behalf of -- or even in support of -- Texas inmates for fear TDCJ will discipline those inmates under the new policy.
Which brings us to another concern: The policy seemingly gives TDCJ the power to discipline inmates for third-party actions. TDCJ has an array of punishments for rules violations. For example, TDCJ can take away an inmate's visitation and phone call privileges, or place him in more restrictive housing.
Finally, the policy is remarkably vague. Since learning of TDCJ's new policy, concerned friends and family members have been seeking clarification of its scope. What if a visiting mother updates her Facebook page with what her incarcerated son ate for breakfast that morning? Can a son take a photo with his incarcerated father and post it to Instagram? Would this reach the type of reporting and advocacy seen in the hit podcast "Serial"?
Social media sites created to advocate against the death penalty for an individual inmate could easily fall within the policy's scope, particularly if it's unclear whether the inmate initiated or has any control over the content posted on the site. In this way, the policy could inhibit pure political speech, an arena in which First Amendment protection is at its zenith.
To quote Benjamin Franklin, "[f]reedom of speech is a principal pillar of a free government; when this support is taken away, the constitution of a free society is dissolved, and tyranny is erected on its ruins." By suppressing the voices of inmates and their supporters, this new policy is certain to have the kind of effect that Franklin so feared -- TDCJ will be freed from the public oversight necessary to hold the state accountable.
We cannot tolerate such governmental overreach. This vague, overly broad policy must be invalidated.


Figure below shows ZTS-320R:
Functions of keyboards and the display of information:
Microprocessor unit in reflectorless total station processes the data collected to compute:
Average of multiple angles measured.
Average of multiple distance measured.
Distance between any two points.
Elevation of objects and
All the three coordinates of the observed points.
ZTS-320R data format:
MEA= Measurement File, working for storing data
COO= Coordinate File, being used when transferring coordinate
COD= Code File, being used when transferring code
Select File >File(.MEA) A.MEA File(.COO) File(.LH) Save Enter
LH= LS =Defined line File, working for staking out roadway
How to aim at a target (only for reference)
1.Aim at the bright sky with the telescope and adjust the eyepiece to focus until a sharp image of the cross wire forms.
2.Aim at the target with the cross center in the coarse sighting device on the top of the lens. The eye should keep a proper distance (about 200mm) away from the sighting device.
3.Obtain a sharp image of the target on the reticule with the focusing screw of the telescope. If optical parallax appears when angle of view changed, the focus or the diopter of the eyepiece may be unadjusted. For precision concerns, please adjust the eyepiece focus to eliminate the optical parallax.
Field of View
1°30′ (2.7m at 100m)
Minimum Focusing Distance
Distance Measurement (ZTS-320 with Reflector)
3000m (9,842ft.) under good condition
6000m (19,685ft.) under good condition
800m (2,624 ft.)
Measuring Time (Fine/Quick/Tracking)
Distance Measurement(ZTS-320R Reflectorless)
Single-axis liquid tilt sensor/Dual-axis (optional)


text:
*It was more of a challenge than I thought it would be. Writing something every day that would be interesting to readers was difficult, because I do not blog regularly.
*The "theme" thing. I did not do this, instead I wrote a different piece every day, but if you wanted to know something about me, read my A-Z blogs. I have an grandson who has autism, am first generation Russian, have written book in a lions's voice, etc.
*The "ageism" thing. I am aware and self-conscious about my age and time in life and hesitated to say that I had grandchildren, as it would turn off some who would follow my blog.
*I like the idea of being able to dip into other interests and themes and there were many: astronomy; YA Fiction; Swedish; French; short-wave radio; movies; poetry; and more. I found that I am interested in other bloggers and finding out more about them. I do have one favorite among all my favorite, The Clay Baboons. How she manages to write and then illustrate her stories with those figures is her secret, and I want to see what she creates every day.
*My goal is to become more adept at the manipulation of the technical aspects of blogging, illustrations, etc., I know there is more to learn, and being part of this writing challenge introduced me to a new world, the blogging world. Thank you!
*Please don't emphasize the "popularity" aspect of the blogs. I am thrilled with 76 followers and hope I can maintain contact with many of them. I don't want to feel that my contribution is any less because I don't have hundreds of followers. I appreciate everyone who commented on my blogs and who became followers.
*Favorite part: meeting with all those bloggers--it was like a big party: "So, what do you do?" Or to quote Cole Porter:
" Have you heard? It's in the stars,
Next July we collide with Mars!
Well, did you evah?
What a swell party this is!
What a swellagent, elegant blogging party this was!"
*Blogging is a reflection of who we are and I am grateful to the editors for the effort and time they gave so that we could be part of this "Moveable Feast."
Attribution: Photo from: "Vanity Fair" May 2012, Ad for Ritz Carleton

domain: artistic


text:
MIAMI — By recent Miami Heat standards, Monday night's win counts as close.
The Heat trailed early and waited until the end of the third quarter to make their move, winning by double figures for the ninth game in a row by beating New Orleans 96-84.
Dwyane Wade scored 32 points, topping 30 for the third consecutive game, and Chris Bosh added 23 points and 11 rebounds. The Heat blew the game open with an 18-2 spurt that put them up 94-77.
LeBron James had 20 points and seven assists for Miami, which became the sixth team in NBA history to win nine straight games by at least 10 points or more. And James has had a hand in two of those runs — Cleveland was the last team to do it, between Nov. 22 and Dec. 9, 2008.
Only three teams — the 2007-08 Houston Rockets, 2003-04 New Jersey Nets and 1946-47 Washington Capitols — have won 10 straight by at least 10 points, and the Heat will try to match the NBA record Wednesday against James' former team, the Cleveland Cavaliers.
David West had 26 points and 12 rebounds for the Hornets, who are 3-9 since an 11-1 start.
BULLS 92, PACERS 73: At Chicago, Carlos Boozer had 22 points and 18 rebounds, and Derrick Rose added 17 points and 12 assists as Chicago got its first six-game winning streak since Nov. 25-Dec. 8, 2006.
The 73 points was a season-low for Indiana and it was the third straight game Chicago held its opponent to a season-worst total. The Bulls held the Los Angeles Lakers to 84 points on Friday and the Minnesota Timberwolves to 82 points on Saturday.
Brandon Rush and T.J. Ford both had 13 points for Indiana.
GRIZZLIES 86, TRAIL BLAZERS 73: At Memphis, Tenn., Zach Randolph had 25 points and 20 rebounds, and Rudy Gay added 16 points to lead Memphis to its third straight win.
The victory snapped an eight-game winning streak for Portland in Memphis. The Grizzlies hadn't won a home game in the series in almost five years.Comment on this story
Wesley Matthews scored 18 points and Andre Miller had 14 points and nine assists for Portland, which lost its second straight after a four-game winning streak.
BUCKS 103, MAVERICKS 99: At Dallas, Brandon Jennings had 23 points and 10 assists, and Milwaukee rallied from a 20-point second-quarter deficit to end Dallas' 12-game winning streak. Andrew Bogut had 21 points and 14 rebounds, and Keyon Dooling added 16 points for the Bucks, who won their fourth in five games. Dirk Nowitzki scored 30 points and Shawn Marion had 14 for the Mavericks, who hadn't lost since Nov. 19 to Chicago. Dallas had also won eight straight at home over the Bucks.


It outperforms conventional motor oil, reduces friction and wear at start-up and protects your engine against performance robbing sludge and varnish deposits. Petromerica Full Synthetic ULTRA Motor Oil is recommended for turbo-charged and high-powered passenger car, truck, sport utility vehicle and other mobile or stationary engines.
Petromerica Full Synthetic ULTRA SAE 10W-30 Motor Oil meets or exceeds ILSAC GF-6A and American Petroleum Institute (API) Resource Conserving SP service classifications and is compatible with all prior API categories. Petromerica Full Synthetic ULTRA SAE 10W-30 motor oil has been field tested to be comparable to most American, Asian and European manufacturers’ standards including GM 6094M, GM4718M, Chrysler MS6395M, Ford WSS-M2C205A, and other OEM’s specifications where a full synthetic SP motor oil is recommended.
GRADE: ILSAC GF-6A / API SP
FIELD TESTED: GM 6094M, GM4718M, Chrysler MS6395M, Ford WSS-M2C205A


New London Awards Honours King's Cross Station and Olympic Masterplan
The King's Cross Station redevelopment and the Olympic Masterplan share this year's prestigious NLA 'Overall Winner' Award. Both projects were commended for their outstanding contribution to London.
NLA Chairman and Chairman of the Awards Jury Peter Murray, announcing the awards, said: "Our jury of overseas experts was impressed by the dramatic new space created by the King's Cross Station Concourse as well as the painstaking work on the heritage of the Grade I listed station. This balance between old and new typifies the complexity of development in London."
JMP Chairman John McAslan said: "We are delighted to be sharing this honour with the London Olympics, as well as winning the Transport and Conservation categories. This project has been an extraordinary, collaborative effort with Network Rail as client, Arup as engineers and Vinci as contractors."
Entries were judged by a panel of international experts in the fields of architecture and urban design including:
David J Burney, Commissioner of New York City's Department of Design and Construction
Dominique Alba, Director of Apur, Paris
Monica von Schmalensee, CEO/ VD, partner, White
Riccardo Marini, City Design Leader of Edinburgh City Council


Estimated read time: 1-2 minutes
This archived news story is available only for your personal, non-commercial use. Information in the story may be outdated or superseded by additional information. Reading or replaying the story in its archived form does not constitute a republication of the story.
WASHINGTON (AP) — The International Monetary Fund said Wednesday it is preparing $127 million in zero-interest loans to the three African countries hardest hit by the Ebola outbreak — Guinea, Liberia and Sierra Leone.
The money would help the three governments deal with rising health and security expenses at a time when Ebola has damaged their economies and reduced tax revenue.
The loans would cover "a sizeable share" of the $300 million the governments need over the next six to nine months, the IMF said in a statement. The IMF's staff recommended the loans and the fund's board will consider the request early next month.
The $127 million would be on top of existing IMF loans to the three countries.
The World Bank on Tuesday approved a $105 million grant to finance efforts to contain Ebola. The disease is believed to have killed at least 2,400 people in the biggest outbreak ever.
The outbreak is also doing considerable economic damage. As Ebola spreads, farmers leave their fields, shops close and transportation is disrupted.
The World Bank said Ebola will reduce economic growth in Guinea this year to 2.4 percent from previous expectations of 4.5 percent growth. Liberia's growth is forecast to slip to 2.5 percent from 5.9 percent and Sierra Leone's to 8 percent from 11.3 percent.
Copyright © The Associated Press. All rights reserved. This material may not be published, broadcast, rewritten or redistributed.


1.69 acres...directly across from Foam and Fabrics Outlet.
Clean environmental report on file.
Ready for re-development:
Corner of Hendersonville Road and South Baldwin Circle. 1 block south of signalized intersection at Hendersonville Road and St. Johns Road and 2 blocks south of the Fletcher Ingles (at Old Airport and Hendersonville Roads).
* #9653-63-7418 is 1.3 acres (.87 acres zoned C-1; .43 acres zoned R-2 in rear).
No value given to structures (2;891 SF block main building with attached garage; converted single-wide in rear)
* #9653-63-4535 is .39 acres (zoned C-1 -- hard corner of Hendersonville and S. Baldwin Circle). Also falls within the Heart of Fletcher Zoning District (Town Center Business Overlay).
No value given to structures (992 SF converted single-wide residence; detatched 570 SF garage). Also falls within the Heart of Fletcher Zoning District (Town Center Gateway Overlay).
The Heart of Fletcher Overlay District does not affect permitted uses; but rather is in place for develoment standards (height/design; setback; ingress/egress; etc -- via approval of the Town of Fletcher Planning Board).
- Price: $589,000
- Square Feet: 0
- Acres: 1.690
- County: Henderson County
- Zoning: C-1 and R-2; Fletcher Ove
- Price per SqFt: $348,521
- Price per Acre: $348,521
- Listed By: Trusilo Commercial


text:
Serpentine fibula polycystic kidney syndrome (SFPKS; MIM600330) is a rare skeletal dysplasia that has polycystic kidneys and dysmorphic facies as additional defining phenotypic components. The nosological classification of this disease has been debated as the condition shares features common to other skeletal dysplasias such as Melnick Needles syndrome (MNS; MIM309350) and Hajdu–Cheney Syndrome (HCS; MIM102500). Here, two previously reported cases of SFPKS are presented with emphasis on their phenotypic evolution. With the recent discovery that HCS is caused by mutations in NOTCH2, DNA from the both cases was examined and both were found to have truncating mutations in exon 34 of NOTCH2. The phenotypic evolution of SFPKS and this molecular analysis strongly suggest that SFPKS is part of the phenotypic spectrum of HCS and should no longer be classified as a distinct disease entity.
Serpentine fibula polycystic kidney syndrome (SFPKS) is a rare skeletal dysplasia that primarily affects bone and kidney development. SFPKS was first described in a 5-year-old female with a distinct facial appearance, proportional short stature, hirsutism, polycystic kidneys, vertebral abnormalities, elongated ‘serpentine’ fibulae,1 acro-osteolysis of the terminal phalanges and toes,2 bilateral cystic kidneys, progressive hearing loss, epilepsy and hypertension.3 She died at 21 years of age from grand-mal seizures and renal failure.3 A second case of SFPKS was reported in 1993 in another female patient who had a more extreme skeletal manifestation of the disease.4 The only reported familial case of SFPKS consisted of an unequivocally affected female and her two deceased brothers who may have had some manifestations of the disorder. Only the female proband had serpentine fibulae.5 The pronounced bowing of the long bones in SFPKS evoked comparisons with Melnick Needles syndrome in one report5 and forms the basis of the current classification of the condition alongside the filaminopathy disorders in the 2010 Nosology and Classification of Genetic Skeletal Disorders.6 Mutations in FLNA, the locus mutated in Melnick Needles syndrome, have been excluded in one individual with SFPCKS.7
In the report of the familial instance of SFPKS, it was noted that the three siblings had significant phenotypic overlap with the autosomal dominant condition known as Hajdu-Cheney syndrome (HCS).5 The presentation of HCS is variable but typically includes acro-osteolysis of the distal phalanges, generalised osteoporosis, craniofacial anomalies, short stature, premature tooth loss and cystic kidneys.8 Simpson et al9 and Isidor et al10 have recently identified truncating mutations in exon 34 of NOTCH2 in several families and sporadic individuals with HCS. All individuals were heterozygous for these mutations, consistent with the established autosomal dominant inheritance pattern for this disease. Here data are presented to show that mutations in exon 34 of NOTCH2 also underlie some instances of SFPKS and that some individuals with this condition evolve a phenotype over time that closely approximates that of HCS.
Case one has been described previously at the age of 4 months as the only survivor of three affected siblings.5 She had persistent ductus arteriosus and intestinal malrotation (which were repaired), an atrial septal defect, splenomegaly, bilateral sensorineural hearing loss, recurrent respiratory infections and corneal diameters of 11–12 mm without glaucoma. Facial dysmorphism was evident with high-arched eyebrows, hypertelorism, full checks, left ptosis and micrognathia. Radiological findings included short terminal phalanges, short bowed humeri, short dysplastic radii and metacarpal heads, subluxed elbows, bowed femora and serpentine fibulae.
On follow-up at the age of 8 years mild developmental delay was evident, as well as short stature, dental malocclusion, a narrow hirsute forehead, low-set posteriorly rotated ears, bilateral epicanthic folds and reduced supination at the elbow (Figure 1). At 9 years of age her lung function had deteriorated so that she required supplemental oxygen, and radiographs confirmed acro-osteolysis of the terminal phalanges. By 10 years of age she required corticosteroid treatment for her pulmonary disease, which had also necessitated a partial lobectomy. Her height was 123.3 cm (0.4th–2nd centile), weight 33.6 kg (50th centile) and OFC 53.5 cm (>50th centile). Her facial dysmorphism resembled HCS with a hirsute forehead, low posterior hairline, shallow supra-orbital ridges, horizontal palpebral fissures, a convergent squint, a pinched nasal bridge with a wide nose, a small mouth, low-set posteriorly rotated ears and prominent maxillae. Her acro-osteolysis had progressed. At 12 years of age she had osteoporosis and had sustained stress fractures of the metatarsals bilaterally. She is negative for a mutation in FLNA.
Case two (Figure 2) was described at 8 years of age as a white female with a persistent ductus arteriosus, ventricular septal defect and dysmorphism, which consisted of a thin upper lip, downturned mouth, wide nasal tip, long and flat philtrum, dysplastic and posteriorly rotated ears.7 She had bilateral sensorineural hearing loss. Radiographs of the patient showed wormian bones, vertebral abnormalities and serpentine fibulae. Ultrasound examination demonstrated polycystic kidneys, but she had normal renal function. At 18 years of age she weighs 51 kg (25th centile) and has a height of 151.5 cm (<5th centile). Since her previous review she had developed hypothyroidism, bathrocephaly and irregular tooth positioning. Radiographs of her hands and feet did not show significant acro-osteolysis, but mild thinning of the distal phalanges was observed (Figure 2b). MRI scan of her head and neck showed basilar invagination and abnormal curvature of the cervical spine without cord compression (Figure 2c). Her intelligence was unimpaired.
NOTCH2 exon 34 was amplified by PCR as described9 from genomic DNA. Automated sequencing was performed on the 3730xl DNA Analyzer (Applied Biosystems, Foster City, CA, USA). All traces were analysed in Sequencher v4.9 (GeneCodes, Ann Arbor, MI, USA). Both patients with SFPKS were heterozygous for truncating mutations in NOTCH2 exon 34. Case one, as reported,9 was heterozygous for c.6895G>T (p.Glu2299X) and case two has a novel truncating mutation, c.7165C>T (Gln2389X). Despite parental samples not being available for either case, the truncating nature of these variants and their predicted functional consequences were identical to previously reported NOTCH2 mutations causative of HCS. Consequently, these mutations are highly likely to be causative of the observed phenotypes.
Both individuals described here demonstrate phenotypic features that are shared between SFPKS and HCS. Previously the delineation of these two disorders has primarily been through the presence of serpentine fibulae in SFPKS, while individuals with HCS have acro-osteolysis and coarse hirsute facial features. However, both conditions have cystic renal disease as a phenotypic feature and serpentine fibulae have also been documented in HCS cases8, 11 casting doubt on these conditions being distinct. Case one5 was originally reported as an example of familial SFPKS, but her phenotype has evolved into a more typical HCS presentation complete with acrosteolysis. Case two7 presented with marked serpentine fibulae and lacks acro-osteolysis, but many of her other phenotypic features were typical of the HCS phenotype. The phenotypic overlap between HCS and SFPKS was first noted in a description of a cohort of individuals with HCS and polycystic kidney syndrome.12 Ramos et al11 compared the phenotype of SFPKS with HCS and concluded that ascertainment bias had led to the delineation of SFPKS as a distinct condition. Two additional cases of SFPKS were subsequently reported in female patients7, 13 and both authors concluded that HCS and SFPKS are likely to be allelic. Both individuals described here are heterozygous for truncating mutations in exon 34 of NOTCH2, similar to those identified in individuals with HCS.9, 10 The truncating mutations in these two SFPKS patients are located in the same region of the gene where mutations causative of HCS are found. The recently identified HCS mutations lead to truncation of the terminal exon of NOTCH2, which produces a protein product missing the proteolytic proline–glutamate–serine–threonine-rich (PEST) domain. PEST domains mediate the internalisation and recycling of proteins in which they reside and therefore the absence of a PEST sequence in this protein is proposed to lead to an elevated level of NOTCH signalling activity in multiple tissues, an observation that highlights its essential role in skeletal development and homeostasis.9 Two cases of Alagille syndrome (MIM#118450) have been reported with mutations in NOTCH2, one of which is a splice acceptor mutation of exon 33 that not only leads to the loss of the PEST domain but also the nuclear localisation sequence and three ankyrin repeats14 (Figure 2d). The ankyrin repeat domains facilitate protein–protein interactions and are required for NOTCH signalling.15 In contrast, the gain-of-function mutations that cause HCS are restricted to the terminal exon that encodes the PEST domain only.
Given the strong phenotypic overlap between SFPKS and HCS and the observation that the two current cases have mutations in NOTCH2, there is strong evidence that SFPKS is not only allelic to HCS but also should be considered one end of the spectrum of the disorder. On the basis of these observations the diagnosis of SFPCKS appears to be redundant, and patients falling into this phenotypic category are best considered to have HCS.
Exner GU : Serpentine fibula – polycystic kidney syndrome. A variant of the Melnick-Needles syndrome or a distinct entity? Eur J Pediatr 1988; 147: 544–546.
Dereymaeker AM, Christens J, Eeckels R, Heremans G, Fryns JP : Melnick-Needles syndrome (osteodysplasty). Clinical and radiological heterogeneity. Helv Paediatr Acta 1986; 41: 339–351.
Fryns JP : Serpentine fibula syndrome: a variant clinical presentation of Hajdu-Cheney syndrome? Clin Dysmorphol 1997; 6: 287–288.
Majewski F, Enders H, Ranke MB, Voit T : Serpentine fibula – polycystic kidney syndrome and Melnick-Needles syndrome are different disorders. Eur J Pediatr 1993; 152: 916–921.
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We thank the families for their willing participation in this study. This work was supported by Curekids New Zealand.
The authors declare no conflict of interest.
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Cite this article
Gray, M., Kim, C., Bertola, D. et al. Serpentine fibula polycystic kidney syndrome is part of the phenotypic spectrum of Hajdu–Cheney syndrome. Eur J Hum Genet 20, 122–124 (2012). https://doi.org/10.1038/ejhg.2011.125
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domain: literature


A Southeast Kelowna resident says he is frustrated and confused after two teens decided to kick in his newly renovated door.
The man partially caught the teens on his front door camera.
"We replaced our front door and then the other night we hear this big bang. So I came out and it was these two teenagers," Don said.
Don tells Castanet the same thing happened to his neighbour back in August.
"They come up to the door, put their backs to the door and then they kick backwards at the same time, bash the door in and run. What we are hearing now is this is quite common now going around Kelowna."
"We started a police report, but they aren't getting any traction," the resident added.
Don says the damage will cost him a few thousand dollars.
"When they kicked the door in it busted the jamb which pushed the trim away from the drywall and pulled some drywall. The whole wall needs to be repainted, the trim needs to be redone, the doorframe needs to be replaced and probably the door at the same time," he said.
The incident is under investigation. Castanet has requested information from the RCMP.
Newsweek reported last year that strangers doors were being kicked randomly across the states due to a TikTok challenge.


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In our last blog we spoke about caring about complaints. On the same theme of care our clinical lead Joanne Percival wanted to discuss as importantly about caring for yourself. Joanne has many years experience in helping and managing people so understands the pressures they face in health and social care.
As registered managers, care support and healthcare workers you are on the frontline of caring for people. Care staff are often the difference between care services running smoothly or having real staffing issues because of their own health related problems.
For this reason, and many others, it is important that your look after yourself so you can look after others. A resilient care workforce is vital for care services, and your own well-being.

domain: medical


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Banker and Tradesman is reporting that Bristol, Plymouth and Norfolk County Registrars of Deeds plan to file a class action suit against Mortgage Electronic Registration System (MERS), aiming to recoup land recording fees they believe they are owed. B&T reporter Colleen Sullivan reports that:
The counties are being represented by Bernstein Liebhard LLP, a New York firm specializing in class actions which has already brought a similar suit on behalf of all the counties of Ohio. John Mitchell, a Bristol County commissioner, said the board considered pursuing a claim last year, but decided to hold off until the national mortgage settlement between the banks and the states’ attorneys general was resolved. But as it became clear that the vast bulk of the funds in that settlement would go towards foreclosures and loan modifications, he said the county decided to pursue the matter. Bristol County officials estimate the county may have lost out on millions of dollars in fees over the past decade because of the alleged use of MERS as a kind of private registry among large banks. A rough calculation prepared by county officials last year came up with a figure of between $3.1 million and $6.5 million lost, using a conservative estimate of one or two additional non-recorded assignments per MERS- registered property.
“Over the last month, we were approached by [Bernstein Liebhard] and other firms….they already had Norfolk and Plymouth, and we thought it made sense to get as many counties together,” Mitchell told Banker & Tradesman. Mitchell said he wasn’t sure if the remaining Massachusetts counties with county-level governance would join the suit. The relatively small size of counties like Nantucket and Dukes would mean far smaller sums at stake.
County-level governance was abolished in Massachusetts in eight of the state’s 14 counties around the turn of the century. Only Barnstable, Bristol, Norfolk, Plymouth, and Dukes retain county boards; Nantucket has a combined city-county government. The remaining boards retain the right to bring independent actions in court.
“We’re familiar with their claims, and there’s no merit to them,” said Janis Smith, spokeswoman for MERS. Smith said that by registering under the MERS name, banks fulfill the purpose of having a registry, that is, alerting the public of any existing leins on a property. “MERS does not eliminate or replace county records, and the recording fees are paid,” she said. “The MERS business model is legal in all 50 states and has been affirmed by Massachusetts courts.”
“I commend the counties,” said John O’Brien, the registrar of deeds in Essex County, who has been an active critic of MERS for the past two years. O’Brien was the first public official in Massachusetts to calculate how much the MERS system may have cost the state in allegedly lost recording fees, coming up with a figure of $22 million for his county alone. “If I had the authority, I would have filed this suit two years ago.”
The other registries fall under Secretary of State William Galvin’s jurisdiction. O’Brien said he plans to petition the legislature to recover his ability to bring suit on behalf of Essex County as one of its elected officials.
The Registrars are reportedly incensed that the MERS private recording system has deprived them of millions of recording fees. We will keep tabs on this important case.


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Support workers to prevent incidents, correct errors, solve safety-related problems and encourage learning from each other.
Involve workers in work health and safety (WHS) discussions to encourage positive safety behaviours, including speaking up when they observe unsafe behaviours. A culture of silence can contribute to problems being hidden, and errors and mistakes being ignored. This creates the potential for injury and illness to other workers unaware of the risk.
The Work Health and Safety Act 2011 states, ‘Workers have a duty to take reasonable care for their own health and safety and to not adversely affect the health and safety of other persons.’
Encourage workers to challenge unsafe behaviour of others, by reinforcing safety values, beliefs, and attitudes such as:
- our workmate’s lives and wellbeing are important. People should go home in the same condition they came to work in
- wellbeing and personal safety in the workplace are more important than money
- promote and support workers who advocate and model good WHS behaviours and practices, especially when safety standards come under pressure.
Once an unsafe behaviour has been identified, check to make sure it has been stopped, and use it as a learning opportunity for everyone.
Promote the idea that differences in ideas and opinions is a positive. Support workers who challenge long held beliefs about how work should be done when they are trying to improve safety for everyone.
Effective consultation includes communication systems and processes that encourage workers to express their views and to raise WHS issues.
If WHS concerns are raised in a group setting such as a toolbox meeting, it is possible not everyone will agree or view the issue as a risk. In this situation:
- acknowledge the concerns raised
- invite questions to clarify understanding
- ask for suggestions to address the concerns
- manage expectations by explaining next steps.
Where suitable, consider temporary solutions, to manage a risk while a more comprehensive assessment is arranged. This may involve modifying a work procedure.
The Safety fundamentals toolkit has resources and information including an Incident/investigation form (DOC, 0.07 MB) and Tips for investigating workplace incidents (PDF, 0.48 MB) templates you can use to involve workers in responding to WHS concerns.
Reduce the likelihood of incidents by encouraging workers to share their experience when a risk is identified. Support your workers to learn from each other for everyone’s benefit.
Establish a WHS committee. This could provide your workers the opportunity to share their experiences, learn from each other and collectively address WHS issues.
Refresh workers’ knowledge of good safety practices and risk management. Workers will often identify risks that you may overlook.
Allow time for workers to talk through errors, what happened, and how it has been fixed. This will help build your organisation’s safety capability.
- Build safety capability by encouraging workers to share WHS error learnings with others.
- Ensure workers have the knowledge, skills, and training to identity when work is being performed unsafely.
- Educate workers about their WHS responsibilities, including speaking up about unsafe behaviours of others.
- Encourage your workers to see mistakes as a learning opportunity.
- Implement a program to acknowledge proactive WHS worker behaviours.
View the Resources page for information and tips on how to improve your systems and processes to build your safety capability and understand your legal obligations.


Sony Television Entertainment and King World Prods, are said to be near a deal to form a strategic alliance that will include Columbia TriStar Television, the studio’s international TV distribution arm, domestic sales barter unit and Sony’s Game Show Network, according to industry sources.
Although talks are still at a delicate stage, it is expected that as part of the alliance King World will take an ownership stake in the Game Show Network, marking the syndication giant’s official entry into the cable business. A final pact could be reached any day.
Spokesmen for King World and Sony Television Entertainment declined to comment.
Talks between King World and Sony started several months ago when King World announced plans to launch a new version of the “Planet Hollywood Squares” gameshow in a partnership with the Planet Hollywood restaurant chain.
However, Sony – which produces “Wheel of Fortune” and “Jeopardy!” for King World – has a deal with the syndicator that prohibits King World from introducing new gameshows without approval from the studio.
As part of the deal, Sony TV’s Columbia TriStar Television will produce “Planet Hollywood Squares” with King World and Planet Hollywood Inc., according to sources familiar with the talks.
King World will distribute “Planet Hollywood Squares” domestically, as well as distribute and co-produce what will likely be a remake of one of three Chuck Barris gameshows
– “The Dating Game,” “The Newlywed Game” or “The Gong Show” – with one of Sony Television Entertainment’s production arms. Columbia will have international distribution rights to the shows. Both syndie series will be sold on a cash-plus-barter basis. King World’s Camelot sales arm is expected to sell time for “Planet Hollywood Squares.” Columbia TriStar Advertiser Sales will handle ad inventory for the Barris show it decides to take out.
King World has a strong distribution track record with gameshows, and the partners probably figured it would be better to have one company handle both programs for the station market. It’s often easier to sell an hour block these days than just one 30-minute strip.
Gameshows are making a comeback this year with other syndicators. Tribune Entertainment and Spelling’s World-vision Enterprises have teamed up for “Swaps,” for instance.
Columbia TriStar Television Distribution also has other development projects in the works for next fall that are not part of this alliance, including a latenight project starring rapper/actor Kid of Kid ‘ n Play.
Ready to grow
For the fledgling Game Show Network, an ownership stake by King World might signal to the cable industry that the studio wants to grow the service. It currently reaches about 3 million homes.
King World brings strong sales resources to the Game Show Network, and its name as a distributor of top gameshows, although it does not have a library of shows.
But King World, which has been looking to get into the cable business for several years, could become a producer for the Game Show Network. The syndicator recently hired former CNBC programming chief Andy Friendly, who has been beefing up the syndicator’s programming staff with the intention of increasing its reach.
Exact terms of King World’s investment in the Game Show Network have not been settled yet. The syndicator could make an equity investment in the network or take a revenue-sharing position when the network hits black ink.