Hyperpigmentation of the distal phalanx in healthy Caucasian neonates.

Transient alterations in pigmentation are frequently observed in black neonates, but to our knowledge, have not previously been reported in Caucasian infants. In 54 Caucasian newborns, we found at least mild periungual hyperpigmentation similar to the variation in coloration in the periungual region of many black newborns. This pigmentation should be added to the transient benign dermatoses of Caucasian infants.

[Small bowel volvulus due to midgut malrotation: value of Doppler sonography. A case report]. / Volvulus du grêle sur mésentère commun: intérêt de l'échographie Doppler. A propos d'un cas.

A case of small bowel volvulus due to midgut malrotation in a 4 years-old child complaining of vomiting and abdominal pain. Diagnosis was made by a Doppler ultrasound showing the volvulus and the intestinal malrotation. We are discussing the interest of this technique.

[Neonatal toxic erythema: 3 atypical cases]. / Erythème toxique néonatal: trois observations atypiques.

UNLABELLED: Diagnosis of pustular dermatosis occurring during the first days of life is based on clinical findings. Erythema toxicum neonatorum (ETN) is the more frequent benign self limiting eruption in the newborn. CASE REPORTS: Three cases of ETN with localized pustules to the genitals and perineal area are described. COMMENT: When encountering a newborn with a localized pustulosis rash, it is important to separate benign condition as ETN from those that require prompt diagnosis and therapy. Atypical ETN and pustular dermatosis due to bacterial or viral infections or inflammatory diseases (e.g., eosinophilic pustulosis) can be differentiated by cytological and bacterial samples.

[Contribution of post mortem skull scanning for the study of sudden infant death]. / Contribution du scanner crânien post-mortem à l'étude de la mort subite du nourrisson.

BACKGROUND: Despite post-mortem examination and autopsy, many cases of sudden infant death (SID) remain unexplained. The aim of this study was to assess usefulness of CT-scan in the Sudden Infant Death Syndrome (SIDS). POPULATION: Twenty-three cases of SIDS had a post-mortem CT-scan evaluation of skull and brain. The pictures were retrospectively reviewed by several independent radiologists who were unaware of the circumstances of death and results of autopsy. RESULTS: Aspects of pneumatocele, probably due to lumbar puncture were found in 6 cases. The subarachnoid spaces appeared inexplically hyperdense, as they were not correlated to the results of lumbar puncture and autopsy. The ventricles were normal in size or density. Density of the dural sinuses (superfical and deep) was often increased, an aspect possibly artefactual, due to post-mortem thrombosis. The cerebral parenchyma was often slighty hypodense; microcalcifications due to congenital toxoplasmosis were found in one case. CONCLUSION: There was no correlation between the CT-scan imaging and the delay of death and lumbar puncture. Infants with or without subarachnoid hemorrage had the same CT scan findings. The CT-scan has a poor value when autopsy is performed; in its absence, it could be useful for diagnosing post-traumatic intracerebral hematoma.

[Clinical heterogeneity of Townes-Brocks syndrome]. / Hétérogénéité clinique du syndrome de Townes-Brocks.

BACKGROUND: Townes-Brocks syndrome (TBS) is a rare autosomal dominant entity mainly characterized by ano-rectal, ear and extremities abnormalities with variable clinical expression. CASE REPORTS: The first case had ear and extremities, but not anorectal, abnormalities; a Pierre-Robin sequence with esophageal atresia was also observed. The second case had the classical triad of abnormalities also associated with tetralogy of Fallot which has been only once reported in the literature. CONCLUSIONS: Both cases are other examples of the frequent clinical variability observed in this syndrome explaining diagnostic difficulties in the absence of a specific marker.

[Spontaneous umbilical cord hematoma, a rare cause of acute fetal distress]. / L'hématome spontané du cordon: une cause rare de souffrance foetale aiguë.

BACKGROUND: Spontaneous umbilical cord hematoma is a rare life-threatening gestational accident. CASE REPORT: A 26 year-old primipara was examined at 38 weeks of gestation for fetal monitoring. There were a number of fetal decelerations with loss of baseline variability. Cesarean section performed for acute fetal distress resulted in a male infant with an Apgar score of 3 at 1 minute and 7 at 5 minutes and neonatal anemia (Hb: 11.6 g/l). Four umbilical cord hematomas were observed. Microscopic examination revealed a ruptured umbilical vein without other abnormality. Post natal evolution was marked by hypoxic-ischemic encephalopathy and death. CONCLUSION: Spontaneous umbilical cord hematoma is rare (1/5,500 births) and often due to rupture of the umbilical vein. Risk factors are shortness or traction of the cord, post-maturity and infection. Umbilical cord hematomas are usually responsible for severe fetal distress or death.

[Utilization of an equimolar mixture of oxygen-nitrous oxide in a general pediatric ward]. / Utilisation du mélange équimolaire oxygène-protoxyde d'azote dans un service de pédiatrie générale.

UNLABELLED: We report our experience of the utilization of the 50% oxygen-nitrous oxide mixture (nitrous oxide 50%) in our general pediatric ward after one year of use. PATIENTS AND METHODS: Between 1st April 1997 and 31st March 1998, children who had to undergo a painful procedure were proposed to inhale 50% nitrous oxide before the procedure. We evaluate pain, restlessness and adverse effects. RESULTS: The procedures (127 of them) were carried out in 90 children (61 boys). They were aged from 5 months to 15 years (mean: 5.7 years; median: 4.1 years). Indications were: lumbar puncture (n = 45), burning dressing (n = 29), venous cannulation (n = 12), minor surgery (n = 27), and miscellaneous (n = 14). Inhalation time was between 2 to 70 min (mean: 14.4 min; median: 11 min). Pain was absent or low in 106 cases (83.4%). Restlessness was absent or low in 100 cases (78.8%). Averse events were observed 12 times, but they were always minor and quickly reversible. CONCLUSION: Nitrous oxide (50%) can be used successfully in a general pediatric ward. Other studies are necessary to define the best conditions.

[Self-induced cutaneous lesions in Prader-Willi syndrome]. / Lésions cutanées auto-induites au cours du syndrome de Prader-Willi.

INTRODUCTION: The Prader-Labhart-Willi syndrome was first described in 1956. Prader-Labhart-Willi syndrome is the most common genetic form of human obesity and the incidence of Prader-Labhart-Willi syndrome has been estimated to 1 in 10,000 or 25,000 live births. Skin-picking was frequently reported in Prader-Labhart-Willi syndrome and two patients who displayed repetitive skin picking are described. OBSERVATIONS: Two childrens (6 year-old girl and 7 year-old boy) were examined and noted superficial ulcers of their arms and legs. This cutaneous lesions were induced by children themselves. Skin-picking, in our cases, were associated with behavior problems (temper tantrums, violence). CONCLUSIONS: Skin-picking appears to occur in the great majority of patients with Prader-Labhart-Willi syndrome and constitutes a minor criteria of diagnosis. Hypopigmentation in Prader-Labhart-Willi syndrome appears to be as common as previously features. Significant differences in hair color, sun sensitivity and complexion were found between those patients with chromosome 15 deletion and those with normal chromosome. Association between obesity (onset before 6 years) and skin picking constitute a sign for diagnosis of Prader-Labhart-Willi syndrome.

[Lethargy in the course of treatment with ranitidine in a newborn infant]. / Léthargie au cours d'un traitement de ranitidine chez un nouveau-né.

The neuropsychiatric complications of histamine H2 receptor antagonists are poorly described in children. In this case report, lethargy occurred in a neonate treated with ranitidine. We urge caution when a histamine H2 receptor antagonist needs to be prescribed in such cases.

gamma-Aminobutyric acid concentrations in the cerebrospinal fluid of newborn infants determined by high performance liquid chromatography.

gamma-Aminobutyric acid (GABA), a major inhibitory amino acid, has a central role in cardiorespiratory regulation. Its measurement in the cerebrospinal fluid (CSF) complements the study of neurotransmission systems. Forty-one children were studied (postnatal age < 1 year). For each child, date of birth, date of sampling and current treatments were collated and their postnatal (days) and postconception (weeks) ages were calculated. CSF samples were studied using reverse phase high performance liquid chromatography (HPLC) with o-phthaldialdehyde derivation and spectro-fluorimetric measurement. A clear increase in levels of GABA was observed around 41 weeks postconception, followed by a progressive decrease, with levels stabilizing after 57 weeks postconception. GABA-regulated neuromodulation therefore appears to be mature at 41 weeks postconception and not at birth. The data could be used in further studies investigating amino acid metabolism in relation to brain function in various neurological disorders.