g children’s hospitalization affects the level of agreement between reports on their HRQOL.An amendment to this paper has been published and can be accessed via the original article.

Melnick-Needles syndrome (MNS) is an extremely rare osteochondrodysplasia caused by a mutation of FLNA, the gene encoding filamin A. MNS is inherited in an X-linked dominant manner. In this study, we describe three members of the same family with MNS, who exhibited different phenotypic severity despite having an identical FLNA gene mutation.

The patient was 16 months old, with a history of delayed physical development, multiple upper respiratory infections and otitis media episodes. She was referred to our orthopedic clinic because of bowed legs and an abnormal plain chest radiograph. Both upper and lower extremities were bowed. Plain X-rays showed thoracolumbar kyphoscoliosis, with anterior and posterior vertebral scalloping, and thin, wavy ribs. Hypoplasia of the pubis and ischium, with bilateral coxa valga, were also noted. Target exome sequencing revealed a heterozygous mutation of FLNA, c.3578 T > C, p.Lys1193Pro, which confirmed the diagnosis of MNS. Her older sister and mother had minimal deformities of the axial and extremity skeleton, but genetic analyses revealed the same FLNA mutation as the patient. The mutation identified in this family has not been previously reported.

This report illustrates the potential inherited nature of MNS and the phenotypic variability of clinicoradiologic characteristics. In patients with traits suggestive of MNS, a careful medical and family history should be obtained, and genetic testing should be performed for the patient, as well as all family members.

This report illustrates the potential inherited nature of MNS and the phenotypic variability of clinicoradiologic characteristics. In patients with traits suggestive of MNS, a careful medical and family history should be obtained, and genetic testing should be performed for the patient, as well as all family members.

Few studies have quantitatively estimated the income elasticity of demand of voluntary private health insurance (VPHI) in countries with a universal National Health Service. Most studies to date have uses cross-sectional data.

In this paper we used a longitudinal database from the Bank of Spain to analyse the financial behaviour of approximately six thousand families per wave. We used three waves (2008, 2011 and 2014). We estimated income and wealth semi-elasticities of VPHI in Spain considering personal and family characteristics (age, sex, level of health, education, composition of the household), i.e. changes in the probability of buying VPHI as result of 1% change in income or wealth. We estimated cross-sectional models for each wave and longitudinal models for families remaining for at least two waves, taking account of possible selection bias due to attrition.

Cross-sectional models suggest that the income effect on the probability of buying a VPHI increased from 2008 to 2014. The positive impact lusions are 1) Cross-sectional estimates of semi-elasticities of VPHI might be biased upwards; 2) Wealth is alongside income are economic determinants, of the decision to buy VPHI in high-income countries; 3) The effects of income and wealth on the probability of buying VHPI are neither linear nor log-linear. There are no significant differences among 60% of the most disadvantaged families, while the families of the two upper wealth quintiles show clearly differentiated behaviour with a higher probability of insurance.

Klippel-Trénaunay syndrome (KTS) is a complex congenital vascular disorder, typically accompanied by port-wine stains, varicose veins, and limb hypertrophy. This paper reports a rare and unusual clinical condition of periosteal reaction in a pediatric case of KTS. Although periosteal new bone formation is not rare in children, as is KTS, their dual occurrence or the presentation of the former due to KTS has not been previously documented. Our objective in this study is to highlight the potential association between periosteal new bone formation and KTS, as well as to help physicians consider this association when bone neoplasm has been ruled out.

A 7-year old girl, initially presented with a persistent mild swelling in her left shank, with no abnormalities in the X-ray of the tibiofibular. However, after a few consults and examinations, 7 weeks later, a 17cm-long periosteal new bone formation along the left tibia and diffused dilated vessels in the left shank were revealed by the radiological examination. Not knowing the true nature of the fast-growing lesion in a typical case of KTS was worrying. Therefore, a core needle biopsy was performed. The test demonstrated a possible parosteal hemangioma. Following further investigation through an excisional biopsy, and a pathological analysis, hyperplasia of the bone tissues with no tumor cells was revealed. Thereafter, an elastic stocking treatment was prescribed. During the first two-year follow-up, recurrence of the mass or sign of progression of KTS was not observed.

Periosteal new bone formation is a potential manifestation of KTS. compound library chemical Based on the conclusive pathological results of the excisional biopsy, invasive examinations and surgeries could be avoided in future KTS-subperiosteal lesion manifestations.

Periosteal new bone formation is a potential manifestation of KTS. Based on the conclusive pathological results of the excisional biopsy, invasive examinations and surgeries could be avoided in future KTS-subperiosteal lesion manifestations.

Skiing and snowboarding are popular activities among Canadian youth and these sports have evolved to include certain risk behaviours such as listening to music, using terrain parks, and video recording yourself or others. The objective of this study was to determine the prevalence of these risk behaviours and identify factors that are associated with the risk behaviours.

Using focus group methodology, a questionnaire was developed to capture aspects of the Theory of Planned Behaviour. A cross-sectional study was conducted where the questionnaire was administered to youth aged 13-18 during two winter seasons attwo ski hills in Manitoba, Canada.

The sample was comprised of 735 youth (mean age 14.9; 82.1% male, 83.6% snowboarding). The most common behavior was using the TP (83.1%), followed by listening to music that day (36.9%), and video recording that day (34.5%). Youth had significantly higher odds of listening to music that day if they planned to next time (OR 19.13; 95% CI 10.62, 34.44), were skiing or snowboarding alone (OR 2.