Biomedical subjects
Publications and source records attributed to B Descos.
In a 12-year-old girl, a duodenojejunitis associated with systemic manifestations led to the diagnosis of Crohn's disease. Continuous enteral alimentation and steroid-therapy has induced remissions of the acute disease.
Three cases of ecchymotic cellulitis in 7 to 11 month old infants are reported. Haemophilus influenzae was isolated from blood in two cases. Ecchymotic cellulitis in infants should, in the first place, evoke an Haemophilus influenzae infection. Bacteremia being frequent in this condition, blood cultures should be performed.
A neonate presented with an inflammatory syndrome with multisystemic manifestations. There was no remission until age 6 years, despite anti-inflammatory treatments. Neonatal onset and neuromeningeal manifestations identified a peculiar rare inflammatory syndrome, whose relationship with juvenile rheumatoid arthritis is discussed. In the case reported, renal, abdominal, deep lymphatic and cranial involvements were present: these have not been previously reported.
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A 3 month-old infant vaccinated with BCG at birth presented with granulomatous hepatitis with BCG isolated in the liver. Splenomegaly, infiltrates in both pulmonary apices and hilar adenopathies were simultaneously present. No immune deficiency could be found. Complete recovery followed specific polychemotherapy. Parenchymal calcifications appeared in the liver, spleen, lungs and mesenteric ganglia.
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The authors report the case of an 8 year-old girl admitted for an erysipelas-like rash of the lower limbs and an episode of Henoch-Schönlein purpura. These manifestations were subsequently attributed to familial mediterranean fever. This case report illustrates the polymorphism of cutaneous manifestations in this disease.
In this paper we report two cases of extended intestinal aganglionosis in siblings, a condition that could be considered a severe variety of Hirschsprung's disease.
Pancreatic, hepatic and intestinal troubles occurred in a 8 year-old acrogeria girl who was treated for Wilm's tumor by cobalt therapy at 2 1/2 years. Complete obstruction of the coeliac trunk and proximal superior mesenteric artery was shown, associated with hypoplasia of the abdominal aorta and its collaterals in the irradiated areas. Acrogeria might enhance the toxic effects of radiotherapy on vessels.
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A 12 year-old boy presented with duodenal hematoma in the hours following small bowel biopsy. Two years later, he presented with abdominal pain and diarrhea. Investigations allowed to find a chronic calcified pancreatitis, which is suggested to be the consequence of the previous duodenal hematoma.
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The authors report a case of the Coffin Siris syndrome which associates a ungueo-digital syndrome (special by the bilateral aplasia or severe hypoplasia of nails and third phalanx of fifth toes and fingers) to other anomalies: facies with thinly fine hairs contrasting with bushy and dense eyebrows and body hypertrichosis, hypotonia and mental retardation. The connections of the Coffin Siris syndrome with the trisomy 9 p+ syndrome and the fetal hydantoïn syndrome are discussed.
A Budd-Chiari syndrome was observed in a 11 year-old girl who was admitted to hospital for abdominal pain and distension, soon followed by severe shock. X-ray investigations, surgery and autopsy showed partial stenosis of the common portion of the left hepatic veins associated with a complete obstruction of other hepatic veins but without alteration of the inferior vena cava.
A retrospective cooperative survey allowed analysis of 58 endoscopically (56) or surgically (2) proven ulcers in children. Drugs or stress induced lesions and ulcers in neonates were excluded from the study. The distribution was as follows: 39 (67%) males, 19 (33%) females, 42 (72%) duodenal, 16 (28%) gastric; 45 children were older than 7 years; a family history of ulcer was present in parents and/or siblings of 12 patients (38%). Ulcer was diagnosed on the occasion of a complication in 39 children (67%). Endoscopy performed in 53 children first detected the ulcer in 51 (in 5 instances at the second examination only). In the remaining 5 children, ulcer was proven endoscopically after radiologic examination was performed. The endoscopic and radiologic data were compared at the time of diagnosis and during follow-up. 31 children were checked endoscopically 3 months after diagnosis and lesions were still present in 21. 10 children were operated on. In these children, evolution is unpredictable and endoscopic check-ups are necessary.
An ileocaecal tuberculosis suspected in an Algerian girl was confirmed only by operative findings and by the presence of caseum at histological examination. Mycobacterium tuberculosis could not be cultured from the lesions. Diagnostic problems between tuberculous enteritis and Crohn's disease are discussed.