[Takayasu's disease: interest in methotrexate treatment]. / Maladie de Takayasu: intérêt du traitement par méthotrexate.

UNLABELLED: Takayasu's disease is a nonspecific aortic arteritis that affects mostly young women and sometimes children. Usual treatment consists of early and prolonged steroids. Some patients do not respond to this treatment, become steroid-dependent or suffer from side effects. We report a case in which methotrexate proved to be effective. CASE REPORT: A 6-year-old girl presented with Takayasu's disease with elevated blood pressure of renovascular origin. Corticosteroids controlled the inflammatory syndrome but not the renal involvement and stopped the growth. Methotrexate (10 mg/m2/week) resulted in the control of the disease, the reduction of steroids and normal growth. CONCLUSION: In Takayasu's disease, steroids should be given as first-line therapy. In case of failure, side effects or steroid dependency, small doses of methotrexate may facilitate the disease's control and weaning from the steroids.

[Results of a protocol for etiologic diagnosis of pneumopathies on immunosuppressed children (author's transl)]. / Résultats d'un protocole d'enquête étiologique des pneumopathies chez les enfants immunodéprimés.

A protocol for etiologic diagnosis has been used on 40 immuno suppressed children with pulmonary infiltrate. The purpose of this protocol is to permit an early etiologic diagnosis, concomitant infections by several pathogen agents being frequently seen. An endobronchial brushing is first performed; the results being obtained between 24 to 48 hours. If the results are negative and the patient's condition worsens we propose an open lung biopsy. Thus pathogenic agent has been established in 24 cases, in 17 cases by endobronchial brushing, in 3 cases by open lung biopsy and in 2 cases by endobronchial brushing and open lung biopsy. In 3 cases several opportunistic organisms were responsible of the pneumopathy. Pneumocystis carinii is the most often established (19 cases); it was also present in one out of 6 post mortem examinations. In 14 cases the pneumopathy occured in children treated for acute leukemia; Pneumocystis was the organism responsible in 6 of them; the evolution was favorable in 13 of the 14 cases. One patient died from a pulmonary infection by the measles virus.

[Acute mercury poisoning in children. Report on four cases (author's transl)]. / Intoxication aiguë par le mercure chez l'enfant. A propos de quatre observations.

The authors report four cases of acute mercury poisoning in children ranged from one week to twelve years of age. All were of favourable course and one of them is particularly well-documented with regard to mercury excretion. Case reports are followed by a clinical study of mercury intoxication with a review of literature about recent therapeutic advances.

Diagnosis of pneumocystis carinii pneumonia using an endobronchial brushing technique. A report on twenty-one cases in immunocompromised children.

By examining the endobronchial brushings from 65 infants and children with interstitial pneumonia--characterized by a severe hypoxia and diffuse lung infiltrates--we have detected the cysts of pneumocystis carinii in 17 of 21 subsequently confirmed cases. The rapid diagnosis and subsequent specific treatment have allowed the recovery of 18 of these children. The particular significance of this article is the youth of the patients, whose ages ranged from 4 months to 15 years.

[Neonatal hernias of the dome of the diaphragm. Why a gloomy prognosis?]. / Les hernies de la coupole diaphragmatique néo-natales. Pourquoi un pronostic si sombre?

The prognosis is neonatal hernias of the dome of the diaphragm remains extremely gloomy. Associated malformations, prematurity or dysmaturity, cannot explain it. The surgeon is now faced with conditions diagnosed in the labour room, which he previously never saw (and which therefore did not figure in his statistics). Those forms in which the lung gas exchange area is reduced are above all those in which there is a syndrome of persistence of foetal circulation. The association of drugs from the alphalytic group (tolazoline) and curarising agents (pancuronium) is often effective without having completely resolved the problem.

[Emergency treatment of inborn amino errors of amino acid metabolism detected in the neonatal period]. / Traitement d'urgence des aminoacidopathies à révélation nónatale.

The indications and effects of exchange transfusion, peritoneal dialysis, osmotic diuresis and early feeding have been studied in 28 children with inborn errors of aminoacid metabolism presenting in the neonatal period. Exchange-transfusion has only a transitory and incomplete effect but it is simple and quick. Peritoneal dialysis has a remarkable and often life-saving effect because the blood levels of toxic metabolites are reduced very effectively in organic acidaemias. However the rate of removal may decline if plasma concentrations fall below a critical level (1.2 mmol/L foor leucine). If the dialysis is prolonged for more than 36 hours it may cause hypoprotidaemia. Osmotic diuresis increases the 45 ml/min. However it is of little elimination of methylmalonic acid because the renal clearance is between 15 and 45 ml/min. However it is of little value in maple syrup urine disease, propionic acidaemia or isovaleric acidaemia because the renal clearance of the toxic metabolites is so low. The early re-introduction of low protein high calorie of a low protein and high calorie diet by continuous intragastric feeding is very important. The authors propose a protocol for the treatment of babies presenting inborn errors of aminoacid metabolism in the neonatal period. Peritoneal dialysis should be started as soon as the diagnosis is considered and continued for 24 to 36 hours. An exchange of transfusion shold be undertaken before and after the dialysis, together with an osmotic diuresis if appropriate. Continuous enteral feeding should be given, the quantity being adjusted to the baby's requirements.

[Pneumocystis carinii pneumonia in chronically immunosuppressed patients. Apropos of 33 cases]. / Pneumonie à Pneumocystis carinii dans les maladies chroniques immunodéprimantes. A propos de 33 observations.

The availability of drugs that are active against Pneumocystis carinii has renewed interest in and underlined the difficulties of the early diagnosis pneumocystis pneumonia. A retrospective study of 33 cases was undertaken to define the optimal management. It is necessary to take into consideration the high mortality of the untreated condition and the risks of investigation and of treatment. Pneumocystis pneumonia affects only those patients who are immunologicaly incompetent, particularly those with defects of cellular immunity. Early symptoms are common but they are insidious. Indirect immunofluorescent tests were positive in 75% of our cases and were useful as screening tests. However it is necessary to identify the parasite (in our cases it was detected in 29 patients) and this requires invasive techniques such as lung puncture, lung biopsy or bronchial brushing. The best method for the rapid diagnosis of pneumocystis is endobronchial brushing, because it is simple, effect and has few complications.

[Serum creatine kinase isoenzyme as a reflection of neonatal cerebral distress (author's transl)]. / Isoenzyme BB de la créatine kinase sérique témoin de la souffrance cérébrale néo-natale.

The BB fraction of serum creatine kinase was estimated in 3 groups of newborn : 30 healthy newborn at term, 20 newborn ill but without cerebral distress, 17 newborn with acute perinatal or foetal distress. In the first two groups, BB fraction was negligeable or nil, in respectively 26 and 16 cases, and between 1 and 4% in 4 cases. In the third group, it was greater than 4% in 10 cases, nil in 6 and 4% in one. The lability of this isoenzyme over periods of time, and in relation to temperature, requires the use of clearly defined techniques of sampling, storage and estimation, which may explain the false negatives seen. The determination of this isoenzyme, virtually exclusively cerebral, would thus appear to be a reliable biological indicator of neonatal cerebral distress.

[Acute tuberculous meningitis in children. A report on 4 cases (author's transl)]. / Méningite tuberculeuse grave de l'enfant. A propos de quatre observations dans un service de réanimation.

Four children were treated for acute tuberculous meningitis. One child died and 2 others were left with severe neurological sequellae. The diagnostic and clinical signs of tuberculous meningitis are reviewed. Treatment includes the administration of an association of INH-rifampicine and ethambutol orally, or INH-ethambutol-ethionamide intravenously when oral administration is impossible. Intrathecal injections of rifamycine SV can be given for acute forms. Corticoids have only one indication: intracranial hypertension with cerebral edema, which requires surgical decompression if no improvement is obtained. The fact that cases of tuberculous meningitis are still notified, is a justification for early BCG vaccination and regular control of the tuberculin allergic reaction.

[Principles underlying the use of antituberculosis medication in children (author's transl)]. / Principes d'emploi des médicaments antituberculeux chez l'enfant.

The authors review the principles underlying antituberculosis therapy in children. Basing their comment on the published literature, they analyze the properties, pharmacology, toxicity, and side-effects of each medication, and define their dosage and modes of administration.

[Accidental ingestion of caustic in childhood (author's transl)]. / Les intoxications par caustique chez l'enfant.

Accidental ingestion of caustic in childhood is not frequent but severe. It result in oesophagal burns progressing to stricture formation. 18 children with severe oesophagal burns were admitted during the six past years in the Intensive Care Unit of "Hôpital des Enfants-Malades" in Paris. The authors report mainly two cases. They recommend oesophagoscopy and they propose a protocol of treating applied since 1974 in France and foreing countries. The present problem consists mainly in a preventive program to avoid kind of accidents and their sequels in children and their families and finally in the community.