Biomedical subjects
Publications and source records attributed to C Fromentin.
Through the case report of severe digitalis intoxication in a premature born after 27 weeks of gestation with a birthweight of 800 g, the authors show that anti-digoxin antibodies constitute a very efficacious therapeutic tool which, up to the present, has not been used in prematures.
A literature review was conducted in relation to a case of chronic diarrhea associated with a VIP (vasoactive intestinal polypeptide) producing ganglioneuroblastoma (GNB), in an 18-month old female baby. This is a rare entity characterized by premonitory, persisting diarrhea, causing fluid and electrolyte changes typical of the WDHA syndrome, associating watery diarrhea, hypokalemia, and achlorhydia. Elevated VIP plasma levels are an indication for an echographic and/or CT-scan search for the causal secreting tumor. Although the prognosis of this condition seems favorable, the recommended treatment is surgery. The VIP substance represents an excellent biological monitoring marker. Ganglioneuroblastomas are tumors of the sympathetic nervous system, which, according to Pearse's cell and embryologic theory (1966), have to be linked to the APUD system tumors (paraneuromas). VIP-producing forms are rare in children, and only 29 case studies have been compiled in the literature since 1970, when the VIP substance was discovered. The case reported in this study illustrates the diagnostic problems raised by such lesions, and allows us to confirm VIP's imputability for the occurrence of the chronic diarrhea condition in this child.
A 3,200 g birth weight, 38-week gestational age neonate developed very severe persistent pulmonary hypertension following surgical repair of a left diaphragmatic hernia. Despite administration of dopamine, dobutamine, and tolazoline, a significant alveolar-arterial oxygen difference (660 mmHg) was found at age 41 hours. Nifedipine was then administered sublingually in a dose of 1 mg/kg and produced an immediate and dramatic improvement in the right radial artery pO2 that was sustained despite persistence of the right-to-left shunt. We discuss the mode of action of this vasodilating agent that, when added to major therapeutic agents, proved capable of completely reversing a hazardous neonatal situation.
On the 4th day of acyclovir treatment for Herpes simplex pneumonia, a 28 month-old girl who had received allogenic marrow transplant for stage IV neuroblastoma presented with severe neurologic disorders including coma and choreic movements. These symptoms disappeared 9 days after acyclovir was stopped. The disturbance in acyclovir kinetics because of acute renal failure and/or a cerebral cortex atrophy might explain the poor neurologic tolerance of acyclovir. This reversible neurologic involvement on a prone patient should be known as a differential diagnosis of Herpes simplex encephalitis.
A 4 month-old premature infant having received prolonged intensive care for necrotizing enterocolitis and Pseudomonas infection, developed anuria with intrapyelic fungus ball developed in a congenital single kidney. Review of the literature found 19 similar cases and emphasized the difficulty of an early diagnosis and the usefulness of renal ultrasonography for its detection. Moreover the opportunity to treat premature neonates carrying candida albicans with IV amphotericin B and flucytosine before the occurrence of an uneasy treatable urological obstacle in discussed.
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The preliminary results of our experience with the "inverted" subxiphoid approach for 2D echocardiographic visualisation of patent ductus arteriosus (PDA) in premature neonates and infants are reported. Eight premature ventilated neonates weighting 870 to 1,200 g with an isolated PDA were examined by this technique using a mechanical sector scanner and a 7.5 MHz transducer; three views were obtained (left ventricular outflow tract or "long axis"; an oblique view through the two atria and aortic arch; short axis view of the ventricles). A PDA was directly visualised in 6 of the 8 patients. It was possible to measure its diameter and observe its tortuous or rectilinear trajectory; the outcome of Indomethacin therapy or surgical ligature could also be evaluated. In the 2 patients in whom the PDA was not visualised, there were no clinical symptoms or echocardiographic signs of shunt: these PDA were therefore probably extremely small. In the neonate and infant, it is relatively easy to demonstrate a PDA by this approach. The PDA can also be followed up in "ductus dependent" congenital heart disease during treatment with Prostaglandin E. In conclusion, the subxiphoid approach represents an interesting alternative for the visualisation of PDA, especially in premature neonates in whom the suprasternal and parasternal views are often difficult to obtain.
Eight children with steroid-response nephrotic syndrome were investigated to study the relation-ship between steroid responsive syndrome, allergy and HLA-antigens. Six of them had clinical and/or biological allergic symptoms. The allergen was identified in four out of six cases (grass-pollen 2 cases, house dust 1 case, frullania 1 case). Six children had DR7 HLA-antigen, five had B12 antigen. Three of the four children with cortico-dependent nephrotic syndrome have associated B12-DR7 HLA antigens. Search of an allergen seems to be of interest in children with steroid-responsive nephrotic syndrome, since eviction of the allergen may be proposed as a complement to cortico-therapy.
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The locked in syndrome seldom occurs in children, is rarely due to trauma and only in exceptional cases has a favourable outcome. The case reported below is unusual in all these respects, and its evolution might be an example of successful axonal regeneration in the central nervous system.
INTRODUCTION: Leucinosis (maple syrup urine disease) is a metabolic disorder caused by an enzymatic deficiency involved in the degradative pathways of the three branched-chain amino acids. We report an observation of acrodermatitis enteropathica-like syndrome induced by essential amino acid deficiency in a child with leucinosis. CASE REPORT: A child with leucinosis was referred to our hospital for exfoliative dermatitis of the perioral and anogenital regions associated with diarrhea and pancytopenia. The diagnosis of iatrogenic acrodermatitis enteropathica-like syndrome was confirmed after screening showing isoleucine deficiency. Rapid response was observed after adequate isoleucine supplementation. DISCUSSION: The acrodermatitis enteropathica-like eruption in our patient was due to an iatrogenic amino acid nutritional imbalance. Our observation underlines the risk of using a branched-chain amino acid-free formula without adequate supplementation of deficient amino acids. In addition, dietary insufficiency of isoleucine, associated with the treatment of organic aciduria should be added to the causes of acrodermatitis enteropathica-like syndrome.