Efficiency of alteplase in the treatment of venous and arterial thrombosis in neonates.
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Biomedical subjects
Publications and source records attributed to M Fayon.
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BACKGROUND: A chronic intestinal pseudo-obstruction is sometimes seen in premature neonates who are fed early and subsequently suffer from digestive intolerance for several weeks. PATIENTS: Seven premature babies (mean gestational age: 30.5 weeks, mean birthweight: 950 g) suffered from abdominal distension and failure to pass stools at a mean age of 2.5 days (extremes: 1 to 6 days); 2 of them also had vomiting. X-ray examination showed dilated loops of bowel throughout the abdomen without obstructive changes; barium or Gastrografin studies demonstrated inertia of the colon without obstructive changes or abrupt changes in caliber. Histological examination of enteric nerve cells in 2 cases showed normal maturation. Parenteral nutrition was necessary in all patients for 30 to 78 days (mean: 47), followed by continuous enteral feeding for 24 to 48 days (mean: 37). Septicemia complicated parenteral nutrition in 4 babies and was responsible for the death of 1 of them. Normal evacuation of stools occurred between day 27 and day 91 (mean day 46) allowing normal enteral feeding. All 6 patients, now aged 2.5 to 8 years (mean 3.5 years) are in good health, and have no digestive problems. CONCLUSIONS: Immaturity of intestinal motility may occur in some premature neonates suffering from intestinal ileus. Parenteral nutrition may be necessary for several weeks, but spontaneous recovery is usual. This transient intestinal pseudo-obstruction of premature babies is different from the classical chronic pseudo-obstruction for which surgery is often needed.
We report the case of an unusual form of Noonan's syndrome with multisystemic involvement identified in the early neonatal period. Apart from the common association of dysmorphia, cardiopathy and lymphatic vessel anomalies, a tumor-like naevoid cutis verticis gyrata of the scalp was observed. This type of cutaneous lesion has never been described in previous reports of the syndrome.
Near-miss is the term used by English-speaking authors to define a sudden accident suggestive of imminent infant death. This is one of the most worrying problems, due to its frequency and its multiple possible causes, the most common of which are gastro-oesophageal reflux and vagal hypertonia. These accidents occur in the same age-group as the sudden infant death syndrome and in similar circumstances, even though they more often occur during waking. Near-miss therefore may constitute an abortive form of sudden infant death syndrome, which would make its study a good way for understanding the syndrome. Yet one should wait before making this assimilation as it might induce unwarranted medical procedures. The risk of recurrence (about 10%), sometimes lethal, exists, but is must be discussed after full investigation in search of a cause and a possible treatment. Electronic home monitoring is only one of the possible preventive measures; it must be decided upon and applied by a specialized medical team.
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Mitochondrial cytopathies are multisystemic diseases of extremely variable expression caused by a deficiency in oxidative phosphorylation. Only five cases of neonatal liver failure in the context of mitochondrial cytopathy have been reported, with incomplete morphological data of the liver in three. In the case presented here, ascites had been diagnosed prenatally and liver failure was particularly severe (factor V less than 15% with fatal coma the fourth day). Histologically there were incomplete cirrhosis, microvesicular steatosis, major canalicular cholestasis with proliferative neocholangioles, and bile duct thrombi. There were also some iron pigments in the periportal area and partial glycogen depletion. By electron microscopy, mitochondria in numerous hepatocytes appeared abnormal with occasional cristae in a fluffy matrix, some containing dense inclusions. Study of respiratory chain activity showed a defect in cytochrome c oxidase (complex IV), revealed by oxygraphic measurement on fresh muscle biopsy and confirmed by spectrophotometric enzymatic assays performed on muscle and liver homogenates. The association of neonatal liver failure with hyperlactacidemia warrants investigation into a deficiency in oxidative phosphorylation.
The authors report on the case of a 7 week-old boy, in whom a renal mass was discovered after general symptoms were observed. Within 48 h, cardiac failure secondary to systemic arterial hypertension occurred, requiring intensive care. After a few days of mechanical ventilation and alternating elevated and low blood pressure, improvement was obtained with captopril and frusemide enabling further investigations to be carried out which lead to the diagnosis of Wilms tumor. During left-sided nephrectomy, elevated renin from the left renal vein was found. The post surgical course was excellent. Several authors have reported on the association between arterial hypertension and nephroblastoma as being the result of hyperreninism due to hilar compression; however severe hypertension was uncommon. Renin activity determination from the tumoral tissue had led to a different interpretation, ie primary hyperreninism: in the case of mesoblastic nephroma, only the non tumoral but compressed tissue contains a large quantity of renin; in the case of nephroblastomas, only the tumoral tissue contains renin. The question now is whether all or only certain nephroblastomas secrete renin.
Two cases of spontaneous ileal perforation in neonates have been reported. The diagnosis is often difficult due to the lack of clinical or radiological signs. Other possible etiologies are excluded at laparotomy. Hypotheses concerning the etiology of spontaneous intestinal perforations have been discussed.
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The Noonan syndrome is a multiple congenital anomalies syndrome with variable expressivity and autosomal dominant inheritance. We report an observation of a newborn with Noonan syndrome and an unusual molluscoid cutaneous excess over the scalp that might represent a new skin manifestation in Noonan Syndrome rather than a consequence of lymphatic dysplasia.
Eosinophilia is a common finding in premature babies during the neonatal period (75%). It has a variable correlation with several factors that may influence it: gestational age, birth weight, time required to regain birthweight, neonatal stress, caesarean birth, formula feeding, endotracheal intubation, positive pressure ventilation, parenteral nutrition, umbilical catheterization, blood transfusions, antibiotics, phototherapy, sex, neutropenia and sepsis. The possible mechanisms are discussed: resolution of a stress, anabolic state, reaction to antigens, normal granulopoietic maturation, transplacental transfer of a factor stimulating the eosinophil synthesis.