[Focal dermal hypoplasia: description of three cases].

Background: Focal dermal hypoplasia syndrome is mainly defined by the association of abnormalities of extremities, atrophy and linear hyperpigmentation of the skin, localized deposits of superficial fat, anomalies of the eyes and of the nails. Neonates are often small for their age.

Case Reports: Three sporadic cases are reported.

[Localized neonatal convulsions and cerebral arterial infarction].

Background: Stroke is a rare cause of neonatal seizures.

Case Reports: During a 5-year period, eight full-term infants were admitted to hospital for seizures due to a stroke. Seizures began shortly after birth and were always one-sided.

Enterovirus-associated haemophagocytic syndrome in a neonate.

A 3-d-old neonate presented with fever, hepatosplenomegaly, coagulopathy, thrombopenia and anaemia. Secondary haemophagocytic lymphohistiocytosis was suspected, as persistent cytopenias were associated with hypofibrinogenaemia, haemophagocytosis in bone marrow and decreased NK cell. There was no positive family lymphohistiocytosis history or parental consanguinity.

Prenatal ultrasound abnormalities in a patient with generalized neurofibromatosis type 1.

We describe a patient with an unusual neonatal disseminated form of neurofibromatosis (NF1). Prenatal ultrasound studies, at 35 weeks of gestation, revealed ambiguous external genitalia, an increased biparietal diameter and a decreased growth of long bones. Postnatal examination displayed generalized neurofibromatosis, with perineal, thoracic and spinal cord invasion by tumors.

Prognostic value of neonatal electroencephalography in premature newborns less than 33 weeks of gestational age.

In a prospective study of 417 premature neonates born before 33 weeks' gestational age, neonatal tracings were reviewed to evaluate the use of EEG in prognosis of neurological injuries. The population was divided into two groups: Group 1, infants who died before the age of 1, and Group 2, survivors in which two categories of motor development were considered. Category A, were abnormal, and Category B, were always normal.

[Fetal growth in premature infants in Haute-Normandie].

Background: Fetal growth standards of preterm infants are different from one study to another, especially for extremely preterm babies.

Population And Methods: Between 1976 and 1990, a cross-sectional study of the resulting intrauterine growth of premature newborns from Haute-Normandie (France) was conducted by collecting data of the compulsory health certificate set up in the first week after birth. In spite of exclusions, curves for obstetrical terms ranging from 28 to 36 weeks of gestational age were settled.

Expression and effects of hyaluronan and of the hyaluronan-binding protein hyaluronectin in newborn rat brain glial cell cultures.

Hyaluronan (HA) is a polymerized nonsulfated extracellular matrix glycosaminoglycan that may be involved in brain development. We have tested the expression of HA and the HA-binding protein hyaluronectin (HN) in glial cell cultures from newborn rat brain. HA was secreted into the culture medium by type 1 astrocytes in the first stages of the primary cultures.

Caffeine decreases glial cell number and increases hyaluronan secretion in newborn rat brain cultures.

Newborn rat brain astrocytes (type 1 astrocytes, O-2A progenitor cells, and O-2A progenitor-derived cells, i.e. oligodendrocytes and type 2 astrocytes) were cultivated to investigate the effect of addition of caffeine to the culture medium on glial cell development and secretion of hyaluronan (hyaluronic acid, HA).

Herpes simplex hepatitis with chronic cholestasis in a newborn.

In the case of the central nervous system or hepatic involvement, the prognosis of neonatal herpes simplex infection remains poor, despite antiviral drugs, presumably effective if given early. We report the case of a neonate with herpes simplex hepatitis, where the course of the illness was unusual with chronic, ultimately fatal, cholestasis. The treatment was not effective, because its administration was delayed, because of high infant C reactive protein level and the absence of clinical maternal genital infection, and because it was interrupted due to misleading information: clinical improvement, negative viral tests and raised herpes IgG antibody titer.

Positive rolandic sharp waves in the EEG of the premature newborn: a five year prospective study.

In a prospective study of 301 premature newborn infants, neonatal tracings were done to evaluate the use of the electroencephalogram (EEG) and positive rolandic sharp waves (PRSW) in the diagnosis and prognosis of periventricular leucomalacia. Each infant had ultrasonographic studies and standardised neurological examinations at 1 year of age or later. Two hundred and eighty infants were followed up at 1 year.

GTP-dependent protein(Gs) activity in preterm infants.

In a previous study, we showed a renal resistance to PTH in preterm infants during their 1st week of life. We proposed it could explain early neonatal hypocalcemia. Such renal resistance is well known in type 1 pseudohypoparathyroid patients and is explained by a defect of stimulatory GTP-dependent protein (Gs).

[Sudden severe hypoxemia: a cause of severe fainting attacks in infants].

Six infants with life-threatening fainting fits are reported; five had bronchopulmonary dysplasia and one Pierre Robin anomaly. Clinically unsuspected episodes of hypoxaemia, related to an underlying respiratory pathology, appear to be the origin of these fits.

[Positive rolandic sharp waves, periventricular ischemia and neurologic outcome. Prospective study in 66 premature infants].

A prospective study concerning 66 prematures born after 32 weeks of gestation showed a 33% incidence of positive rolandic sharp waves (PRSW) on EEGs. The results showed a good sensitivity (Se = 95%) and specificity (Sp = 93%) of PRSW for the diagnosis of periventricular ischemia with motor sequelae. A PRSW frequency higher than one per minute and its persistence on 2 successive EEGs constitutes an indication of severity of the lesions.

[Bilateral diaphragmatic hernia. Apropos of 2 cases].

Two cases of bilateral posterolateral diaphragmatic hernia are reported, due to their rarity. Both rapidly led to death. One of them was associated with severe malformations of the upper limbs.