[Adams-Oliver syndrome and cutis marmorata telangiectatica congenita]. / Syndrome d'Adams-Oliver et cutis marmorata telangiectatica congenita.

Adams-Oliver syndrome (AOS) is a congenital condition characterized by congenital aplasia cutis and transverse limb defects. Herein we report a case of an infant with severe intra-uterine growth restriction presenting AOS associated with cutis marmorata telangiectatica but with no other organ complications. The outcome was complicated by hemorrhagic and septic shock, which resulted in the death of the infant in a setting of multiorgan failure.

[Seizures revealing phosphocalcic metabolism abnormalities]. / Crises épileptiques révélant des anomalies du métabolisme phosphocalcique.

Hypocalcemia due to hypoparathyroidism produces a broad spectrum of clinical manifestations, but overt symptoms may be sparse. One unusual presentation is onset or aggravation of epilepsy in adolescence revealing hypoparathyroidism. This situation can lead to delayed diagnosis, with inefficacity of the antiepileptic drugs. We report five cases of adolescence-onset epilepsy with unsuccessful antiepileptic therapy, even with gradually increasing dose. Physical examination revealed signs of hypocalcemia, confirmed biologically. Full testing disclosed the origin of the seizures: hypoparathyroidism in three patients and pseudohypoparathyroidism in the other two. In four of five patients, computed tomography showed calcification of the basal ganglia, defining Fahr's syndrome. The patients were treated with oral calcium and active vitamin D (1-alphahydroxy vitamin D3). Seizure frequency progressively decreased and serum calcium levels returned to normal. These cases illustrate the importance of the physical examination and of routine serum calcium assay in patients with new-onset epileptic seizures in order to detect hypocalcemia secondary to hypoparathyroidism.

Cerebral Ischemia after Stenting of Coarctation of the Aorta.

Percutaneous stenting angioplasty of native coarctation of the aorta is considered a low-risk procedure with high success rate. The incidence of cerebral complications, especially ischemic complications, is very low. We report a case of a 15-year-old boy who underwent a percutaneous stenting angioplasty for a coarctation of the aorta and developed a cerebral infraction 4 hours after the procedure.

An unusual cause of cardiac failure in a neonate.

We report the case of a newborn admitted with signs of congestive cardiac failure with prominent and pulsatile cervical veins. Echocardiography showed a structurally normal heart, right-to-left ductal flow, and reversed diastolic flow in the proximal descending aorta. A computed-tomography scan of the head showed a vein of Galen arteriovenous malformation. This highlights the importance of considering an intracranial cause in the differential diagnosis of neonatal congestive heart failure.

[Capnocytophaga sputigena pleuropneumonitis: a case report]. / Pleuropneumopathie àCapnocytophaga sputigena : à propos d'un cas.

Community-acquired pneumonia is complicated by purulent pleural effusion in approximately 28% of affected children. Its incidence has increased over the last decade. Streptococcus pneumoniae is the most frequent causal microorganism, encountered in roughly 80% of cases. We report the case of an immunocompetent 13-year-old teenager wearing orthodontic braces who suffered from pleuropneumonia due to Capnocytophaga sputigena. To date, this Gram-negative bacillus, a commensal of the buccal cavity, had never been reported to be responsible for pleural or lung infection. The clinical presentation is similar to that observed with usual bacteria, while bacterial sensitivity to betalactam antibiotics is excellent.

[Infantile herpes zoster]. / Zona chez un nourrisson.

Herpes zoster occurs seldom in infants, especially in the absence of exposure to maternal varicella either intrauterine or postnatal. We report on a case in a 3-month-old infant admitted for herpes zoster in the sciatic nerve territory. No cutaneous eruption was found in the mother or in people who were in contact with the patient. This rare clinical situation is here reviewed, showing that the absence of antenatal or postnatal exposure to herpes viruses does not preclude the occurrence of herpes zoster infection in early infancy.

[Hypercalcemia complicating BCG lymphadenitis: case report]. / Hypercalcémie compliquant une bécégite locorégionale : à propos d'un cas.

One of the side effects of the BCG vaccine is a local infection that may spread to the regional lymph nodes causing lymphadenitis, which can resolve spontaneously without treatment. We report the case of an immunocompetent infant who developed lymphadenitis after administration of the BCG vaccine, complicated with persistent symptomatic hypercalcemia in spite of the usual treatment including corticotherapy. Antituberculous treatment was necessary to reduce this hypercalcemia.

Indications and results of renal biopsy in children: A single-center experience from Morocco.

The contribution of renal biopsy (RB) is of major importance in the management of many renal diseases in children. Specific indications for performing biopsy in children include steroid-resistant nephrotic syndrome (NS) and secondary nephropathies. The aim of our study was to report the common histological varieties of kidney diseases in children in Morocco. In this retrospective and descriptive study, we included all renal biopsies performed in patients under 16 years in the Department of Pediatrics of Hassan II University Hospital, Fez, Morocco from July 2009 to December 2013. Biopsy samples without glomeruli and those with less than five glomeruli or repeat biopsies on the same patient were excluded from our study. We performed 112 RBs during this period; the average age at the time of RB was 10.05 ± 4 years and the sex-ratio was 1.07. The indications for RB were NS with hematuria and/or renal failure (RF) in 32.1%, active urinary sediment in 21.4%, isolated NS in 15.2%, RF in 13.4% and steroid-resistant NS in 10.7% of cases. Primary nephropathies represented 59.8% of cases, with a predominance of minimal change disease (MCD) seen in 40.2% of the cases. Secondary nephropathies accounted for 27.7% of the cases, with a predominance of lupus nephritis (11.6%), followed by Henoch-Schonlein purpura nephritis (6.2% of cases) and post-streptococcal glomerulonephritis (3.6%). There was one case of hepatitis B virus-associated membranous glomerulonephritis. Chronic glomerulonephritis accounted for 12.5% of the cases. Vascular and tubulo-interstitial nephritis were rare. Our study confirmed that primary glomerular nephropathy was the most common renal disease in children. The most common lesion was MCD. Secondary nephropathies were less frequent, with a predominance of lupus nephritis.

Repair of isolated double-chambered right ventricle.

The finding of a double-chambered right ventricle (DCRV) is exceptionally rare as an isolated anomaly. It is a congenital cardiac anomaly in which the right ventricle is separated into two chambers, a proximal high-pressure chamber and a distal low-pressure chamber, by anomalous muscles or fibrous tissues in the right ventricular cavity. We report the case of a 6-year-old infant who was admitted for growth retardation. The patient was diagnosed with an isolated DCRV without any other associated congenital anomalies. The patient underwent a successful cardiac surgical procedure of enlargement repair; he was discharged in good clinical condition with a normal cardiac function.

[Pseudothrombotic microangiopathy related to vitamin B(12) deficiency]. / Pseudo-microangiopathie thrombotique liée à un déficit en vitamine B12.

Vitamin B(12), or cobalamin, deficiency is often unrecognized because the clinical manifestations are subtle; they are also potentially serious. We report a case of pseudothrombotic microangiopathy related to cobalamin deficiency. Vitamin B(12) deficiency, which is more commonly recognized in the context of malnutrition, should be considered in the context of microangiopathy.

Left Ventricle Diverticulum with Partial Cantrell's Syndrome.

CANTRELL SYNDROME IS A VERY RARE CONGENITAL DISEASE ASSOCIATING FIVE FEATURES: a midline, upper abdominal wall disorder, lower sternal abnormality, anterior diaphragmatic defect, diaphragmatic pericardial abnormality, and congenital abnormalities of the heart. In this paper, we report a case of partial Cantrell's syndrome with left ventricular diverticulum, triatrial situs solitus, ventricular septal defect, dextrorotation of the heart, an anterior pericardial diaphragmatic defect, and a midline supraumbilical abdominal wall defect with umbilical hernia. The 5-month-old patient underwent a successful cardiac surgical procedure. A PTFE membrane was placed on the apex of the heart to facilitate reopening of the patient's chest. Postoperative course was uneventful. The patient was discharged with good clinical condition and with a normal cardiac function.

Epidemiological and clinical characteristics of scorpion stings in children in Fez, Morocco

Scorpion stings are a public health problem in Morocco, especially among children, who experience the most severe cases. Epidemiological and clinical findings on scorpion stings in Fez, Morocco, were evaluated in this investigation. Of 163 cases that required medical attention, 62.6 percent were male children. The mean age of patients was 4.8 ± 3.4 years. The mean time between stings and first medical attention was 3.36 ± 2.5 hours. Almost all cases occurred in the summer (94 percent) and extremities represented the most frequent sting sites (86.5 percent). Local pain, hyperemia, scarification, vomiting, sweating, restlessness, tachycardia and tachypnea were the observed clinical symptoms. Regarding severity, 55.2 percent of patients belonged to class III, followed by class II (26.4 percent) and class I (18.4 percent). None of our patients received antivenom; however, all of them were treated symptomatically depending on clinical manifestations.(AU)