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Objective: The aim of this paper is to describe the surgical technique, originally devised by Dr. Renier which is currently used to treat children with scaphocephaly under 6 months of age at the Craniofacial Unit of Hopital Necker Enfants Malades (French National Referral Center for Faciocraniosynostosis), focusing on its advantages and limitations.

Objective: To investigate the determinants of postural imbalance after stroke in geriatric patients admitted for low-intensity rehabilitation in skilled nursing facilities (SNFs), particularly the role of multimorbidity., Design: Cross-sectional study design., Setting: Fifteen SNFs., Participants: All patients who were admitted for rehabilitation after stroke in one of the participating SNFs (N=378) were eligible., Interventions: Not applicable., Main Outcome Measures: The Berg Balance Scale (BBS) was selected as a measure of standing balance and the Functional Ambulation Categories (FAC) as a measure of walking balance., Results: Multimorbidity was present in 34% of the patients. The patients with multimorbidity differed from the patients without multimorbidity with respect to age, proprioception, and vibration sense, but not for any of the cognitive tests, muscle strength, or sitting balance. Patients with multimorbidity had, on average, lower scores on both outcome measures. In linear regression analyses, both the BBS and FAC were best explained by multimorbidity, muscle strength, and the interaction between muscle strength and static sitting balance (overall explained variance 66% and 67%, respectively), while proprioception added only to the variance of the FAC., Conclusions: Multimorbidity was independently related to postural imbalance after stroke in patients admitted for rehabilitation in SNFs. Muscle strength and the interaction of muscle strength with static sitting balance were important determinants of both standing and walking balance, indicating these factors as essential targets for rehabilitation., (Copyright © 2012 American Congress of Rehabilitation Medicine. Published by Elsevier Inc. All rights reserved.)

Quadricuspid aortic valves are rare congenital anomalies, usually diagnosed in adult patients affected by severe aortic regurgitation. We have now encountered three such valves in children undergoing transthoracic echocardiography. All children were asymptomatic. The first child possessed a valve with two larger leaflets, and smaller leaflets of equal size. In the second child, 3 leaflets were of equal size, with 1 small accessory leaflet. The third child had a valve with four leaflets of approximately equal size. In one child, the aortic valve was functioning normally, and came to attention because of mitral valvar prolapse causing mild regurgitation. In the remaining two children, central mild aortic regurgitation was detected using colour flow analysis, and associated dilation of the aortic root was revealed by measurements of the cross-sectional images.

Variations in individual TGF-beta receptors (TbetaRs) may modify TGF-beta activity and significantly alter its effects on connective tissue growth or repair. Differences in the amount of TbetaR type III (TbetaRIII) relative to signal transducing TbetaRI occur on bone cells during differentiation or in response to other growth regulators. Here we investigated prostaglandin (PG) E2, a potent effector during trauma, inflammation, or mechanical load, on TbetaR expression in primary osteoblast-enriched cultures. PGE2 rapidly increased TbetaRIII mRNA and protein expression and enhanced TbetaRIII gene promoter activity through a discrete region within 0.4 kb of the transcription start site. PGE2 alters osteoblast function through multiple signal-inducing pathways. In this regard, protein kinase A (PKA) activators, PGE1 and forskolin, also enhanced gene expression through the TbetaRIII gene promoter, whereas protein kinase C activators, PGF2alpha and phorbol myristate acetate, did not. The stimulatory effect of PGE2 on TbetaRIII promoter activity was suppressed by a dominant negative PKA-regulatory subunit, but not by dominant negative protein kinase C. PGE2 specifically increased nuclear factor CCAAT enhancer-binding protein delta (C/EBPdelta) binding to a half-binding site upstream of the basal TbetaRIII promoter region, and promoter activity was sensitive to C/EBPdelta overexpression and to dominant-negative C/EBPdelta competition. In parallel with their effect on TbetaRIII expression, activators of PKA decreased TGF-beta-induced activity. In summary, high levels of PGE2 that occur with inflammation or trauma may, through PKA-activated C/EBPdelta, preferentially increase TbetaRIII expression and in this way delay TGF-beta-dependent activation of osteoblasts during the early stabilization phase of bone repair.

We report the case of an infant affected by frequent episodes of loss of consciousness with the clinical features of pallid breath-holding attacks. Prolonged asystole, up to 26 seconds, was demonstrated by Holter monitoring. The patient was treated with permanent pacemaker implantation, followed by complete symptom resolution during a 26-month follow-up.

Background: Pathogenesis of nonalcoholic fatty liver disease (NAFLD) remains incompletely known, and oxidative stress is one of the mechanisms incriminated. The aim of this study was to evaluate the role of liver oxidative stress in NAFLD affecting morbidly obese patients., Methods: 39 consecutive patients with BMI >40 kg/m2 submitted to Roux-en-Y gastric bypass were enrolled, and wedge liver biopsy was obtained during operation. Oxidative stress was measured by concentration of hydroperoxides (CEOOH) in liver tissue., Results: Female gender was dominant (89.7%) and median age was 43.6 +/- 11.1 years. Histology showed fatty liver in 92.3%, including 43.6% with nonalcoholic steatohepatitis (NASH), 48.7% with isolated steatosis and just 7.7% with normal liver. Liver cirrhosis was present in 11.7% of those with nonalcoholic steatohepatitis. Concentration of CEOOH was increased in the liver of patients with NASH when compared to isolated steatosis and normal liver (0.26+/- 0.17, 0.20+/- 0.01 and 0.14+/- 0.00 nmol/mg protein, respectively) (P < 0.01). Liver biochemical variables were normal in 92.3% of all cases, and no difference between NASH and isolated steatosis could be demonstrated., Conclusions: 1) Nonalcoholic steatosis, steatohepatitis and cirrhosis were identified in substantial numbers of morbidly obese patients; 2) Concentration of hydroperoxides was increased in steatohepatitis, consistent with a pathogenetic role for oxidative stress in this condition.

Several causes of acute respiratory insufficiency have been reported in the literature. We describe a case in which it was caused by a rare combination of a giant right coronary artery aneurysm with fistula that occurred in a 56-year-old woman with concomitant congenital hypothyroidism. Diagnostic tools, differential diagnosis, surgical techniques, and follow-up at 1 year are discussed.

Transposition of the great arteries with intact ventricular septum and aortopulmonary window is an extremely rare anatomic combination, having been reported just twice previously. Other authors performed a physiologic repair, because the combination was considered unsuitable for an anatomic repair. We describe the case of a 26-day-old baby with such anatomy who was successfully treated with an arterial switch operation. A 4 mm fenestration at atrial level was made for a smoother postoperative course.

Congenital coronary artery fistulas are very rare. The most common connect one of the two coronary arteries with the right cardiac chambers, in particular the right ventricle. So, a left to right shunt appears; furthermore ischemic problems are possible. Notwithstanding in the pediatric age subset the patients are usually free of symptoms. The most important clinical sign is a continuous murmur in the precordium. We report a case of a newborn with a fistula between the right coronary artery and the right ventricle. He presented with the typical murmur; ECG and chest-ray were considered as normal. Echocardiography did not do the diagnosis, and the baby was submitted to a cardiac catheterization. There was a mild left to right shunt (QP/QS = 1.5/1), without pulmonary hypertension. The diagnosis was done by selective coronary angiography. We decided not to close the fistula, and the baby was discharged. Three months later he is still well. In this discussion we consider indications for surgery, and hypothesize a wider application in the future of transcutaneous embolization.

The right pulmonary artery arising from the proximal ascending aorta is a rare and severe malformation. This retrospective study of 11 children with this condition was undertaken to determine the conditions of diagnosis, to analyse the results of surgery, and, above all, to clarify the mechanism of the left pulmonary arterial hypertension which was always present. Ten of these patients were 4 to 90 days old. All had severe congestive cardiac failure with iso- or suprasystemic left pulmonary arterial hypertension. The only associated lesions were ventricular septal defect (1 case) and patent ductus arteriosus (7 cases). None of the patients had significant left-to-right shunts and only one had left atrial hypertension: this patient died before surgery could be performed. The other 9 patients underwent surgical correction and the pulmonary pressures immediately fell to normal or almost normal values. The child with the ventricular septal defect died of infection 6 weeks after surgery. The 8 survivors are doing well 1 month to 12 years later and left pulmonary pressures are normal in all, including those (5 cases) with a stenosed (4 cases) or completely occluded right pulmonary arterial circulation (1 case) and in 1 patient with obstructive vascular disease. The eleventh patient was very different: she had no signs or symptoms until 2 years of age, when a right pulmonary obstructive arterial disease but with normal left pulmonary pressures was documented. She was not operated on and remains well nine years later.(ABSTRACT TRUNCATED AT 250 WORDS)

Active infective endocarditis (AIE) involving native and especially prosthetic aortic valve is often complicated by conduction abnormalities. These conduction disturbances are considered to represent extension of infection from the valve to the annulus and surrounding myocardium. The authors report their experience of conduction disorders in 6/8 patients in whom the aortic prosthetic valve infection was complicated by periprosthetic abscess. They underline the importance of conduction abnormalities as early markers of severe complication in patients with AIE. In fact, their detection is a useful tool in revealing severe complications since clinical, laboratory and other noninvasive examinations do not always allow early diagnosis of the extension of infection to the surrounding myocardium.