[Neonatal seizures revealing incontinentia pigmenti]. / Convulsions néonatales révélant une incontinentia pigmenti.

Incontinentia pigmenti (IP) is a rare affection inherited as X-linked dominant disease. It is usually lethal in male infants. IP can affect ectodermal tissues such as the skin, teeth, eyes, bones, and the central nervous system. Skin lesions occur mostly during the neonatal period and are characterized by a classic progression in 4 stages leading to hyperpigmentation. We report on the case of a female neonate presenting on the 3rd day of life with seizures without obvious cause, in which the diagnosis of IP was made 1 week later when skin lesions appeared.

[Systemic congenital candidiasis, a rare condition in neonates: case report in a premature infant]. / Candidose congénitale systémique, forme rare de candidose néonatale : à propos d'une observation chez un nouveau-né prématuré

UNLABELLED: Congenital cutaneous candidiasis presenting at birth is very uncommon and is due to intra-uterine infection. The systemic form has to be considered when cutaneous signs are associated with sepsis symptoms. CASE REPORT: A female infant was born by vaginal delivery at 35 weeks' gestation to a mother whose pregnancy had been complicated by urinary tract infection treated 3 days before delivery. The infant was admitted because of respiratory distress. Clinical features consisted of respiratory retraction signs associated with hepatomegaly and rash on the trunk. The white blood cell (WBC) count was 50 × 10(9)/L and C-reactive protein was negative. Maternofetal bacterial infection was suspected and intravenous antibiotics were prescribed. Over the next 6h, macules appeared on the trunk, back, and limbs, which changed after 24h into papulovesicular lesions over the trunk, back, limbs, palms, and scalp. Congenital candidiasis was suspected, confirmed by cultures from vesicle swabs and maternal vaginal discharge. The systemic form was considered because of respiratory distress requiring oxygen therapy for 4 days, hepatomegaly, elevated WBC count, and chest X-ray infiltrates. The infant was started on intravenous systemic antifungal therapy (fluconazole, 6 mg/day). Treatment was continued for 3 weeks. The rash resolved by desquamation after about 1 week and hepatomegaly disappeared. The infant remained well at follow-up.

[Acute renal failure in full term neonates with perinatal asphyxia. Prospective study of 87 cases]. / L'insuffisance rénale aiguë au cours de l'asphyxie périnatale du nouveau-né à terme. Etude prospective de 87 cas.

BACKGROUND: Renal involvement is frequent in neonates with perinatal asphyxia. It is correlated with the severity of neurological damage and seems to worsen the long-term neurological outcome. PURPOSE: The aim of this study was to determine the incidence of renal failure after perinatal asphyxia, to precise the relationship between severity of cerebral damage and renal failure and to evaluate the place of renal damage in the short- and middle-term neurological outcome. POPULATION AND METHODS: We conducted a prospective study including 87 full-term neonates admitted in the neonatology department of F. Hached university hospital in Sousse (Tunisia) and suffering from hypoxic ischemic encephalopathy from 1st January 2003 to 30 June 2005. Renal function was assessed by measuring plasma urea and creatinine at age 48 h. Renal failure was defined by a level of creatinine above 90 micromol/l. Neurologic examination was performed on day 7. The survivors were followed up by the same senior after discharge. RESULTS: During the study period, 87 full-term neonates were admitted for hypoxic ischemic encephalopathy. The degree of neurological impairment was determined according to Sarnat classification: 1st stage 9 neonates (10,3%), 2nd stage 67 (77%) and 3rd stage 11(12,6%). Renal failure involved 15 neonates (17,2%) of whom 10 belonging to the 2nd stage group. Renal function outcome was favorable in all survivors with normalisation of plasma creatinine level between day 5 and day 15. Eight neonates died, of whom 3 with renal failure. Neurologic examination was abnormal in 36 out of 72 (50%) neonates without renal failure and in 9 of the 12 (75%) survivors with renal failure. Among the 12 neonates with renal failure, 7 had abnormal neurologic features at discharge. Neurologic assessment between 6 and 18 months was abnormal in 4/12 (33%) of neonates with renal failure versus 8/72(11%) of neonates without renal failure. CONCLUSION: Transient renal failure is commonly observed in perinatal asphyxia. Renal failure is correlated with neurologic severity. Renal function assessment using creatinine plasma level seems to be correlated with neurologic outcome. However, other tools appreciating renal function, namely tubular function, should be determined earlier in order to predict neurologic outcome after hypoxic ischemic encephalopathy.

[Major neonatal aortic thrombosis: a case report]. / Thrombose aortique néonatale majeure: à propos d'une observation.

Aortic thrombosis is a rare and severe condition in neonates which often is revealed by ischemia of lower limbs. We report a case of major aortic thrombosis revealed by renal failure, hematuria and dehydration in a 10-day-old girl. Clinical features consisted in hypertension at upper limbs without ischemic signs of the lower limbs. Diagnosis was made using renal Doppler ultrasound. Thrombosis resolved after thrombolytic treatment and anticoagulation using heparin. The case report is followed by a review of the literature dealing with clinical, etiological and therapeutic aspects of neonatal arterial thrombosis.