[Medical informatics (hospital information systems) in neonatology: reality and insufficiencies]. / Information médicale (programme de médicalisation des systèmes d'information) en nèonatologie: actualités et insuffisances.

The generalized implementation in France of hospital information systems (HIS) is often considered by the medical practitioners as a useless constraint. Nevertheless, they are now largely used by the administrative authorities for their economical evaluation of medical care. In neonatology HIS is applied to the hospitalized sick neonates as well as to the healthy newborn infants during their maternity hospital stay with their mother following birth. This paper focuses on the practical aspects and difficulties of the current French HIS in neonatology.

[Cytomegalovirus pneumopathies in pediatric intensive care units]. / Pneumopathies à cytomégalovirus en réanimation pédiatrique.

BACKGROUND: Cytomegalovirus (CMV) infection can result in major complications in immunocompromised infants and children. CMV pneumonia may be difficult to diagnose and the true pathogenic role of the virus in the disease is not always clear. This report describes a cohort of 20 children who suffered from CMV pneumonia. POPULATIONS AND METHODS: Twenty children aged 1 month to 11 years 10 months were admitted to our intensive care unit between 1981 and 1990 because of pneumonia with evidence of CMV infection. They were classified into three groups: group I (cases 1-10) with hemopathy or cancer, group II (cases 11-14) with AIDS, and group III (cases 15-20): non immunodeficient or immunosuppressed children. CMV infection was diagnosed after isolation of CMV from bronchoalveolar lavage (BAL) fluid (15 patients), lung biopsy revealing intranuclear inclusions or CMV antigens, or CMV-positive cultures (four patients), CMV-positive urine cultures (one patient). RESULTS: Clinical manifestations and X-rays findings were unspecific; interstitial pneumonia was found only in immunodeficient patients. CMV pneumonia was diagnosed only in two patients on post mortem examination. Concomitant pneumocystis carinii was found on BAL in two patients (group I) and two others (group II). Thirteen patients required ventilation. Eleven patients were given ganciclovir for 2 or 3 weeks; one of them was given a single dose. This treatment was well tolerated. Mortality was 90% in group I, 100% in group II and 33% in group III. CONCLUSION: Ganciclovir did not appear to benefit the immunocompromised patients with CMV pneumonia. Future treatment should include hyperimmune CMV immunoglobulins plus ganciclovir. Careful hand washing is important for all those caring for these patients to prevent contamination as is the use of CMV-negative blood products.

[Severe neonatal respiratory distress with linear inspiratory pressure-volume curve]. / Détresses respiratoires graves néonatales à courbe pression-volume inspiratoire linéaire.

Twenty-four newborns with severe respiratory distress, treated by mechanical ventilation, are investigated by inspiratory pressure-volume curve. The curves are obtained by slow continuous inflation technique. Two shapes are described: concave curve, ten newborns (group I), linear curve, fourteen newborns (group II). The gestational age is over 36 weeks in both groups. Determination of respiratory mechanics is indicated when mean AaDO2 is higher than 500 torr in both groups, and when there is a hypercapnia (PaCO2 = 53 +/- 11 torr, with p less than 0.05) in group II. Mechanical ventilation is conducted with individually adjusted PEEP in group I, and without PEEP in group II. Twelve hours after; mean AaDO2 in group I (260 +/- 101 torr) and in group II (420 +/- 188 torr) are significantly different (p less than 0.05). The variance analysis in group II shows that PaCO2 and pH are normalized (p less than 0.001). All the newborns in group I recovered. Three newborns in group II died. Group I can be assimilated to hyaline membrane disease in full-term neonates. Practically, the cases of neonatal respiratory distress in which PEEP is not indicated can be identified by the functional characteristics of group II.

Preliminary report: extracorporeal lung support for neonatal acute respiratory failure.

A technique for ventilatory support of life-threatening neonatal acute respiratory failure by use of apnoeic oxygenation and low-frequency positive-pressure ventilation, with extracorporeal membrane CO2 removal through a single-cannula perfusion circuit, is described. 20 severely ill babies with respiratory failure were treated with this technique, 17 of whom survived with no clinical evidence of pulmonary handicap or neurological deficit at discharge from hospital. All 10 patients followed up at 6 months showed normal growth and development.

[Etiologic diagnosis of severe acute pneumopathies in infants and children]. / Diagnostic étiologique des pneumopathies aiguës graves du nourrisson et de l'enfant.

In infants and children suffering from severe acute lung disease the problem of aetiological diagnosis arises in two sets of circumstances. In cases of diffuse primary pneumonitis, which occurs mainly in immunocompromised subjects, broncho-alveolar lavage is the ideal technique to discover opportunistic infections; besides, the technique is little invasive and well tolerated. It is only when its results are negative that lung biopsy still has a few indications. In hospital-acquired lung diseases occurring in children under mechanical ventilation the results of broncho-alveolar lavage are more difficult to evaluate owing to possible contamination by tracheal organisms. The various ways of solving this problem (bacterial counts, sampling by means of a protected brush operated by remote control) are discussed.

[A rare cause of neonatal ulcero-necrotizing enterocolitis: aortic coarctation syndrome]. / Une cause rare d'entérocolite ulcéro-nécrosante néo-natale: le syndrome de coarctation aortique.

The authors report a case of necrotizing enterocolitis which appeared in the first hours of life of a full-term neonate without signs of sepsis. This neonate presented with a severe hypoplasia of the horizontal aorta and very tight coarctation responsible for hepatic, renal and mesenteric ischemia. Reports of enterocolitis as a complication of congenital heart disease are rare and related most often to hypoplastic left heart than to coarctation of the aorta.

Use of total inspiratory pressure-volume curves for determination of appropriate positive end-expiratory pressure in newborns with hyaline membrane disease.

Thirty newborns with hyaline membrane disease were treated by mechanical ventilation with individualized appropriate positive end-expiratory pressure (APEEP) from inspiratory pressure-volume curves of the total respiratory system. APEEP was started before H24 in group 1 (19 patients), and after H24 in group 2 (11 patients). Until APEEP, the 2 groups had classical PEEP levels (lower than 0.8 kPa) either without or with incomplete improvement in arterial hypoxia. The mean APEEP of each group was greater than classical PEEP (p less than 0.001). In group 1 the time of exposure to FiO2 greater than 0.4 was shorter (23.8 +/- 13.7 h) than in group 2 (88.6 +/- 56.9 h) (p less than 0.001) and rapid improvement in blood gas exchanges was seen in group 1 compared to group 2 that was independent of the severity of the disease. Tolerance was excellent. APEEP ventilation started before H24 is of special interest in the management of newborns with severe alveolar injury.

[Hemodialysis in a newborn infant with acute renal failure]. / Hémodialyse chez un nouveau-né en insuffisance rénale aiguë.

A 10-day old newborn with post-operative acute renal failure and in whom peritoneal dialysis was impossible was successfully treated by haemodialysis. The technique requires close monitoring and has some particular features, such as difficult vascular approaches, filling of the extracorporeal circuit prior to dialysis, low blood flow rate, sodium bicarbonate in the dialysis bath and high glucose intake. No major complication occurred in the 14 sessions which made it possible to overcome the acute phase of renal failure. Renal function was almost normal at the age of 9 months.

[Extensive cerebral infarction in a case of hemolytic-uremic syndrome]. / Ramollissement cérébral étendu au cours d'un syndrome hémolytique et urémique.

A hemolytic-uremic syndrome is reported in a 9 month-old girl. It was remarkable because of the severity of the renal lesions, which ended in terminal renal failure; there were also neurologic changes, responsible for a coma of 3 month-duration and for right-sided hemiplegia. Two CT scan examinations showed a left hemispherical hypodensity, resulting from a largely extended infarction in the sylvian area. After a 3 year's follow-up, the magnitude of the clinical improvement shows the possibility of neurologic recovery in children.

[Familial form of total digestive aganglionosis with absence of nerve fibers]. / Forme familiale d'aganglionose digestive totale avec absence de fibres nerveuses.

Two familial cases of intestinal aganglionosis with lack of innervation concerning the whole digestive tract from the rectum to the esophagus are reported. The relationships between this histologic picture and that found in classical Hirschsprung's disease are discussed. The lack of obstructive symptom makes the diagnosis difficult; however, it should be suspected in the presence of intraluminal calcifications in the small bowel. The familial incidence of the condition seems to be high. A recessive autosomal transmission is likely. Neurologic signs resembling those in dysautonomia lead to suspect an extensive disorder of the development of the neural crest.

[Segmental dilatation of the ileum in a neonate. Study of the myenteric plexus with a silver staining preparation (author's transl)]. / Dilatation segmentaire du grêle à révélation néonatale. Etude par imprégnation argentique des plexus myentériques.

A case of segmental dilatation of the ileum in a neonate is reported. Conventional staining methods failed to show any abnormality, but a study of the myenteric plexus with a silver preparation demonstrated reduction in the number of agyrophil neurons and irregular and altered neuron cell bodies. Occurring in a neonate, therefore without previous clinical history, this case, together with various published data suggests that in some cases segmental dilatation of the ileum is due to congenital damage to the myenteric plexus.

[Severe allergic cutaneovisceral vasculitis in children. Apropos of 6 cases]. / Vascularites allergiques cutaneo-viscérales graves de l'enfant. A propos de six observations.

Six children aged between 10 months and 13 years with allergic vasculitis are described. The features they had in common were necrotic skin lesions, involvement of various viscera and signs of hypersensitivity reactions. Histological changes of allergic vasculitis were present in the small vessels of the skin. This condition has features in common with both polyarteritis nodosa and with anaphylactoid purpura. Severe cases that do not respond to corticosteroids may respond to immuno-suppression with cyclophosphamide.