[Motor skills as the basis of sensorimotor development, an essential dimension according to André Bullinger]. / La motricité comme base du développement sensorimoteur, une dimension essentielle selon André Bullinger.

In neonatology and pediatric intensive care units, as in pediatric units, development support care practiced according to the approach of psychologist André Bullinger takes into account sensorimotor and psychological aspects. Considering the quality and quantity of movements, from the antenatal period, allows for individualized and early care. Sensitive periods of sensorimotor development, during which brain plasticity is optimal, are decisive for psychomotor and cognitive development. Observation and evaluation of movements, as well as postural support to enable active motor skills, constitute essential elements of the developmental support provided to vulnerable children of all ages.

Renovascular hypertension: results in adulthood of renal autotransplantation performed in children.

BACKGROUND: This study describes the long-term results of renal autotransplantation for renovascular hypertension performed in children who are now 21 years of age or older. METHODS: Sixteen children (4 boys, 12 girls) with a mean age of 11.2 years at the time of the procedure underwent ex-vivo surgery at the university hospital of Saint-Etienne between 1992 and 2008. Acetylsalicylic acid was used for antiplatelet therapy in the postoperative period, without routine anticoagulation. The mean follow-up period was 15 years. The clinical course of these patients was retrospectively reviewed in adulthood and the results analyzed. RESULTS: The children were treated with a mean of 2.37 drugs per patient, and the mean preoperative blood pressure of the entire patient population was 151/89 mmHg. Mean preoperative creatinine clearance was 80 ml/min/1.73 m2. There was no postoperative death. One patient experienced a thrombosis immediately after the surgery, leading to a redo surgery. In this patient diuresis was restarted, but without efficient concentration and filtration, ultimately leading the patient to have a renal transplant after 1 year. At the end of the follow-up period, eight of the 16 patients (50%) were cured and the others were improved. At the last follow-up the mean blood pressure was 127/70 mmHg, and the mean number of drugs per patient was 0.68. The mean creatinine clearance at last follow-up was 104.3 ml/min/1.73 m2. Three patients had secondary procedures, with two undergoing percutaneous angioplasty (at postoperative months 9 and 12, respectively) and one having an hepatorenal bypass at postoperative year 4. Primary patency was 12/16 (75%); primary assisted patency was 15/16 (94%); secondary patency was 16/16 (100%). CONCLUSION: This study shows that renal autotransplantation has good and stable long-term results and is an effective conservative strategy for treating renovascular hypertension in children, thus avoiding nephrectomy.

Renal function can be impaired in children with primary hyperoxaluria type 3.

BACKGROUND: Primary hyperoxaluria type 3 (PH3) is characterized by mutations in the 4-hydroxy-2-oxoglutarate aldolase (HOGA1) gene. PH3 patients are believed to present with a less severe phenotype than those with PH1 and PH2, but the clinical characteristics of PH3 patients have yet to be defined in sufficient detail. The aim of this study was to report our experience with PH3. METHODS: Genetic analysis of HOGA1 was performed in patients with a high clinical suspicion of PH after the presence of mutations in the alanine-glyoxylate aminotransferase gene had been ruled out. Clinical, biochemical and genetic data of the seven patients identified with HOGA1 mutations were subsequently retrospectively reviewed. RESULTS: Among the seven patients identified with HOGA1 mutations the median onset of clinical symptoms was 1.8 (range 0.4-9.8) years. Five patients initially presented with urolithiasis, and two other patients presented with urinary tract infection. All patients experienced persistent hyperoxaluria. Seven mutations were found in HOGA1, including two previously unreported ones, c.834 + 1G > T and c.3G > A. At last follow-up, two patients had impaired renal function based on estimated glomerular filtration rates (GFRs) of 77 and 83 mL/min per 1.73 m(2), respectively. CONCLUSIONS: We found that the GFR was significantly impaired in two of our seven patients with PH3 diagnosed during childhood. This finding is in contrast to the early-impaired renal function in PH1 and PH2 and appears to refute to preliminary reassuring data on renal function in PH3.

Inaugural cerebral sinovenous thrombosis revealing homocystinuria in a 2-year-old boy.

Cerebral sinovenous thrombosis is unusual during childhood and requires early and accurate management because of its detrimental consequences. We report on the case of a 2-year-old boy with mild psychomotor delay, who presented with nonfebrile acute ataxia. A brain computed tomographic (CT) scan showed complete thrombosis of the superior sagittal sinus, confirmed by magnetic resonance angiography and associated with a right frontal hemorrhagic infarction. Systematic screening for thrombophilia revealed homocystinuria linked to cystathionine ß-synthase deficiency with underlying compound heterozygosity. The evolution was favorable after anticoagulant therapy, specific diet, and vitamin supplementation. This case is of interest because of the unusual clinical presentation as a pediatric cerebral sinovenous thrombosis. Furthermore, homocystinuria is rarely revealed by cerebral sinovenous thrombosis at the onset of the disease and should systematically be ruled out in pediatric stroke.

Pseudohypoaldosteronisms, report on a 10-patient series.

BACKGROUND: Type 1 pseudohypoaldosteronism (PHA1) is a salt-wasting syndrome caused by mineralocorticoid resistance. Autosomal recessive and dominant hereditary forms are caused by Epithelial Na Channel and Mineralocorticoid Receptor mutation respectively, while secondary PHA1 is usually associated with urological problems. METHODS: Ten patients were studied in four French pediatric units in order to characterize PHA1 spectrum in infants. Patients were selected by chart review. Genetic, clinical and biochemistry data were collected and analyzed. RESULTS: Autosomal recessive PHA1 (n = 3) was diagnosed at 6 and 7 days of life in three patients presenting with severe hyperkalaemia and weight loss. After 8 months, 3 and 5 years on follow-up, neurological development and longitudinal growth was normal with high sodium supplementation. Autosomal dominant PHA1 (n = 4) was revealed at 15, 19, 22 and 30 days of life because of failure to thrive. At 8 months, 3 and 21 years of age, longitudinal growth was normal in three patients who were given salt supplementation; no significant catch-up growth was obtained in the last patient at 20 months of age. Secondary PHA1 (n = 3) was diagnosed at 11, 26 days and 5 months of life concomitantly with acute pyelonephritis in three children with either renal hypoplasia, urinary duplication or bilateral megaureter. The outcome was favourable and salt supplementation was discontinued after 3, 11 and 13 months. CONCLUSIONS: PHA1 should be suspected in case of severe hyperkalemia and weight loss in infants and need careful management. Pathogenesis of secondary PHA1 is still challenging and further studies are mandatory to highlight the link between infection, developing urinary tract and pseudohypoaldosteronism.

Surgery of the abdominal aorta and its branches in children: late follow-up.

BACKGROUND: The aim of this study was to evaluate the late results in adult patients who underwent surgery of the abdominal aorta as children. METHODS: During a 17-year period, eight children underwent surgery for lesions of the abdominal aorta. There were 6 boys and 2 girls, with an average age of 10 years. The presenting symptom that led to diagnosis of abdominal aortic lesions was hypertension in five cases and lower-limb claudication in three. The underlying disease was middle aortic syndrome in three cases, infrarenal aortic hypoplasia in two, infrarenal aortic aneurysm in two, and Takayasu's disease in one. Five children had associated renal artery lesions, including four with bilateral lesions and one with a unilateral lesion. Aortic bypass was used in all cases. A straight tube graft was placed between the distal descending thoracic or supraceliac aorta and the infrarenal aorta in six cases, and a bifurcated bypass was placed between the infrarenal aorta and the iliac arteries in two. Renal artery revascularization procedures (n = 9) included ex vivo repair with renal autotransplantation in five cases, direct reimplantation on the arch of Riolan in two, and direct reimplantation of the renal artery onto the aortic graft in two. RESULTS: One patient died on postoperative day 1. The remaining seven patients recovered uneventfully. Mean follow-up was 10.2 years. No patient was lost to follow-up. Further surgical intervention was required in three patients. The indications for additional surgery were fibrosis of a renal artery reimplanted onto the graft at 3 years, deterioration of the aortoaortic graft at 5 years, and false iliac aneurysm at 20 years. All seven patients had normal physical development. The average increase in height and weight were 28.5 cm and 26.2 kg, respectively. All patients had normal sexual function, and two are parents. All patients are currently asymptomatic. Short Form 36 scores for quality-of-life parameters were 78% to 83%. CONCLUSIONS: Late results of abdominal aortic surgery in children, in our experience, are encouraging. Quality of life in adulthood was excellent. Insofar as possible, correction should be deferred until the child is 8 to 10 years old so that a prosthesis of sufficient diameter can be used.

Implication of a healthcare worker with chronic skin disease in the transmission of an epidemic strain of methicillin-resistant Staphylococcus aureus in a pediatric intensive care unit.

This outbreak of colonization of neonates in a 10-bed pediatric intensive care unit illustrates the probable role of a healthcare worker (HCW) in the transmission of methicillin-resistant Staphylococcus aureus, despite good hygienic practices. It raises the issue of preventive exclusion of HCWs affected by chronic skin disease from high-risk units.