Clinical and biochemical features in a Congolese infant with congenital disorder of glycosylation (CDG)-IIx.

We describe an infant girl with psychomotor retardation, growth retardation, mild facial dysmorphy, evidence of liver involvement and a type 2 pattern of serum sialotransferrins. Serum transferrin glycan analysis with MALDI-TOF showed an extremely altered N-glycan pattern with a large number of truncated asialoglycans pointing to a severely defective N-glycan processing. The basic defect in this patient with CDG-IIx has not yet been identified.

L'encephalopathie associee a l'infection VIH chez l'enfant : A propos de 19 cas observes aux Cliniques de Universitaires de Kinshasa

De 1989 a 1995; 29 enfants ont presente une encephalopathie liee a l'infection a VIH et leur moyenne d'age est de 5 ans (extremes 9 mois et 13 ans). La transmission est materno-foetale dans 23 cas; secondaire a une transfusion dans 2 cas et le mode de contamination est non etabli chez 4 malades. Les manifestations en sont principalement les troubles du tonus (50 pour cent de cas); les troubles du comportement 30 pour cent) et l'alteration de la conscience. L'encephalopatie observee est statique et elle est irreversible

Les epanchements sous-duraux idiopathiques du nourrisson

L'article porte sur le diagnostic sous-dural idiopathique chez 6 nourrissons dans un centre de 1990 a 1995 apres exclusion de toute cause connue. L'analyse d'elements cliniques et paracliniques a permis de faire les constatations suivantes: les antecedents d'epanchement sous-dural ont ete retrouves dans la fratrie; le perimetre cranien n'a pas excede les valeurs moyennes pour l'age et le sexe; l'echographie transfontanellaire a ete l'examen le plus precis; Ces collections liquidiennes souvent bilaterales etaient susceptibles de se reconstituer meme apres un traitement chirurgical et enfin; un retard psycho-moteur a ete releve au cours de l'evolution chez un enfant. La survenue possible des complications et sequelles peut faire reconsiderer le caractere benin de cette pathologie

La dysenterie bacillaire de l'enfant aux Cliniques universitaires de Kinshasa

Cette etude effectuee de juin 1994 a decembre 1995 montre que des 148 enfants admis dans le Departement de Pediatrie des Cliniques universitaires de Kinshasa pour diarrhee sanguinolente; 26 etaient suspects de dysenterie bacillaire. Huit cas ont ete confirmes par la coproculture. Les germes les plus rencontres sont les shigellas dysenteriae et le shigella flexneri. Pour le traitement; l'acide nalidixique et la norfloxaxine sont des antibiotiques de choix

Gastrites et infection a helicobacter pylori a Kinshasa (Zaire)

La prevalence de la gastrite chronique associee a l'infection a H. Pylori est inconnue dans notre milieu. Le but de ce travail est de determiner cette prevalence dans un echantillon de la population zairoise. Tous les patients consultant pour epigastralgies diverses entre le 1er janvier et le 30 juin ont subi une endoscopie digestive haute avec prelevement biopsique pour recherche de H. pylori. 46 patients souffraient d'epigastralgies sans ulcere; 3 avaient un ulcere gastrique et 9 un ulcere duodenal. Une gastrite chronique etait presente dans 82;8 pour cent des cas. L'infection a H. Pylori etait associee a la gastrite chronique dans 68;75 pour cent des cas; a l'ulcere gastrique dans 100 pour cent des cas et a l'ulcere duodenal dans 66;6 pour cent des cas. Au total; la presence de l'infection a H. Pylori etait etroitement en correlation avec l'activite de la gastrite

Paludisme en Afrique: donnees epidemiologiques; cliniques et therapeutiques

En Afrique; le paludisme occupe le premier rang parmi les causes de morbidite et de mortalite chez l'enfant de moins de 5 ans demuni de toute premunition. Actuellement; sur le continent africain; la resistance du plasmodium falciparum aux medicaments antipaludiques a ete demontree. Plusieurs facteurs contribuent encore a l'apparition et l'aggravation du phenomene de resistance du plasmodium falciparum vis-a-vis des medicaments antipaludiques; notamment la mobilite de la population; la transmission intense du paludisme et l'administration des produits antimalariens et le non respect de la posologie et/ou de la duree du traitement

Place de l'echographie dans l'evaluation diagnostique et pronostique de la cardiomyopathie congestive

L'echographie pourrait etre utilisee dans l'evaluation de la survie des patients souffrant de cardiomyopathie congestive. Les criteres les plus signicatifs sont les dimensions ventriculaires; la fraction d'ejection et le changement de la geometrie ventriculaire exprime par la perte de sa forme ellipsoide

Etude la malnutrition neonatale dans la malnutrition maternelle au cours de la grossesse

L'influence de la nutrition maternelle sur le bien-etre perinatal sera favorable ou non selon que l'apport nutritionnel correspond aux recommandations. Ce travail montre que les 2000 kcal/jour recommandes a la gestante africaine n'est pas une surestimation; mais surtout qu'en dessous de 1700kcal/jour le risque de malnutrition neonatale est tres eleve. Le seuil de 1700kcal apparait donc comme le niveau en dessous duquel il ne faut absolument pas descendre

Le diagnostic et le traitement actuel de la fievre typhoide au Zaire

La fievre typhoide demeure encore aujourd'hui un probleme d'actualite au Zaire; infection specifique purement humaine et due a des bacilles du genre salmonelle; elle est favorisee par des conditions d'hygiene toujours precaire; particulierement par le peril fecal. Le diagnostic positif par hemoculture et coproculture ne peut etre realise que dans des laboratoires de biologie bien equipes. Le traitement specifique par les antibiotiques et les sulfamides pose de serieux problemes du fait de phenomenes de resistance de plus en plus grande de certaines souches de salmonelle

Les occlusions intestinales neonatales aux Cliniques Universitaires de Kinshasa

Revue de 63 observations d'occlusions intestinales neo-natales colligees sur une periode de 22 ans (2;8 cas; moyenne par an). La prise en charge rendue inadequate par l'existence de quelques facteurs locaux specifiques tels que la carence en surveillance systematique de la grossesse (echographie); l'insuffisance de moyens d'investigation et la penurie de moyens therapeutiques appropries et des systemes d'assistance socio-financiere des maladies. Devant le taux eleve de mortalite (80;9 pour cent); les auteurs en appellent a l'eveil de la conscience des cliniciens et a une meilleure promotion socio-sanitaire pour prendre en charge efficacement ces cas d'urgence neo-natale

The effect of artificial feeding on cholestasis, gallbladder sludge and lithiasis in infants: correlation with plasma cholecystokinin levels.

The occurrence of hepatic cholestatis (judged by fasting serum bile acid levels), gallbladder sludge formation and lithiasis (ultrasonography) and their correlation with plasma cholecystokinin (CCK) levels was studied in a group of children on continuous total parenteral nutrition (TPN) (n = 95), and later in 40 of these children on cyclic TPN (cTPN). After resumption of oral feeding, 75 were studied on partial oral feeding (2-4 meals) and 40 on constant rate enteral nutrition (CREN) then 45 on total oral feeding (4-6 meals). Gallbladder sludge occurred in 23% of the children on TPN for 1 month and 32% of those on cTPN for 3 months. On CREN, the sludge rate was unchanged, but dropped significantly (17%) on partial oral feeding, and disappeared in children on total oral feeding. Serum bile acids were abnormal in 80% of children on TPN or cTPN and diminished significantly on total oral feeding only. Plasma CCK levels on TPN, cTPN and CREN were identical to fasting levels of children on total oral feeding. Plasma CCK levels increased significantly 1 h post-prandially during both partial (p < 0.02) and total oral feeding (p < 0.001). There was a significant negative correlation between the gallbladder sludge rate and CCK levels for all methods of feeding. This study demonstrates the frequent occurrence of hepatic cholestasis in infants, and the much lower frequency of gallbladder sludge in children compared to adults on TPN. Plasma CCK levels obtained during the different methods of feeding could explain the reduction and eventual disappearance of sludge following stimulation of CCK secretion by discontinuous feeding.

[Salmonella infections in Kinshasa: the species involved and sensitivity to antibiotics]. / Les infections à Salmonella à Kinshasa: espèces en cause et sensibilité aux antibiotiques.

292 Salmonella strains were isolated between 1986-1988 in the bacteriological unit of the Cliniques Universitaires de Kinshasa (Zaïre). One hundred of these strains were isolated from pediatric samples. The most frequent strains were S typhi, S enteritidis and S typhimurium. S typhi strains were sensitive to all antibiotics tested. The other salmonella serotypes had an increasing resistance to several antibiotics especially ampicillin, streptomycin, tetracyclin and kanamycin. A significant decline was observed with chloramphenicol and sulfamethoxazol-trimetoprim. Stool-isolated strains from children were found to be resistant to all antibiotics in common use in 5/6 cases. In systemic Salmonella infection, treatment with chloramphenicol or sulfamethoxazole-trimetoprim plus gentamycin is probably preferable to any other antibiotic recommended for Salmonella infection.

[Halofantrine hydrochloride in the treatment of Plasmodium falciparum malaria in a region of resistance. Analysis of 54 pediatric cases]. / Traitement par le chlorhydrate d'halofantrine du paludisme à Plasmodium falciparum dans une zone de résistance. Analyse de 54 cas pédiatriques.

54 children (mean age: 43 months), with initial parasitemia up to 1,000 Plasmodium falciparum isolates per mm3, were treated with halofantrine hydrochloride 8 mg/kg twice daily at 6 hours intervals. 35 of these 54 children had received unsuccessful chloroquine and/or quinine treatment during 15 days before admission to the trial. Fever, which was observed in 52 of the children, and parasitemia disappeared respectively 18 hours and 37 hours after initial administration of the drug (mean values). Clinical and biological tolerance was excellent for all patients. Halofantrine is a new precious alternative for the treatment of malaria in regions with extensive multi-drug resistant strains of Plasmodium.

Microscopic colitis: a new cause of chronic diarrhea in children?

From a retrospective study on children who underwent colonoscopy or rectosigmoidoscopy with multiple level biopsies, we selected five patients whose rectocolonic endoscopic aspect was normal and contrasting with the presence of a microscopic colitis on biopsies. These five children had chronic diarrhea (mean duration of 14 months), associated with vomiting (three cases), abdominal pain (two cases), anorexia (two cases), abdominal distension (two cases), and weight loss (four cases). Symptomatic treatment was used in all children: loperamide (one case), trimebutine (three cases), and aluminium and magnesium silicate (two cases). One child received sulfasalazine for 2 months. After 1 year, all patients had normal stools. Rectosigmoidoscopy was performed in four patients and was normal. Biopsies obtained in three cases were normal in two cases and showed a persistent microscopic colitis in one case. Microscopic colitis may be a distinct cause of chronic diarrhea in children.

Crohn's disease lesions in the upper gastrointestinal tract: correlation between clinical, radiological, endoscopic, and histological features in adolescents and children.

The incidence of Crohn's disease (CD) lesions in the upper gastrointestinal (GI) tract of both adults and children is frequently underestimated. In this prospective study, a total of 31 children suspected of having Crohn's disease were systematically examined to identify upper digestive tract lesions. They all underwent barium transit endoscopy with multiple-level biopsies. Typical clinical symptoms suggestive of upper GI tract involvement were found in 5 children (16%), radiological signs in only one child (3%), endoscopic lesions in 13 children (42%), and specific granulomas in 12 children (39%). In eight of these 12 children, the biopsies were taken from macroscopically normal areas of the esophagogastroduodenal mucosa. One of the 31 children had no abnormal radiological and endoscopic features suggestive of CD on the distal small bowel and the colon. There was no correlation between the clinical, radiological, and histological data. Endoscopy plus biopsy provided a positive diagnosis in 39% of cases and a confirmation of the diagnosis in 87% of cases. Endoscopic and histological evidence of CD of the upper GI tract is often present despite an absence of clinical symptoms or radiological changes. Upper GI tract endoscopy with multiple biopsies may be important in the evaluation of this condition and even in some cases for the establishment of the diagnosis.

Effect of total parenteral nutrition, constant rate enteral nutrition, and discontinuous oral feeding on plasma cholecystokinin immunoreactivity in children.

Plasma cholecystokinin (CCK) levels were measured by radioimmunoassay in 100 children (mean age: 20 months) on various types of artificial nutrition. Of the 81 children given total parenteral nutrition (TPN), 32 were studied while on cyclic TPN (cTPN), 66 while on partial fractioned feeding completed by parenteral nutrition, 25 while on constant rate enteral nutrition, and 18 while on total discontinuous oral feeding. The remaining 19 control cases were on normal alimentation. Plasma CCK levels during TPN (21.4 +/- 1.6 pg/ml), cTPN (21.8 +/- 2.7 pg/ml), and constant rate enteral nutrition (26.4 +/- 2.8 pg/ml) were not significantly different from each other and were similar to preprandial total discontinuous feeding (21 +/- 2 pg/ml) and control (22.6 +/- 3.5 pg/ml) levels. The postprandial CCK level increased significantly in partial fractionated feeding (33.6 +/- 3.3 pg/ml, p less than 0.02) but remained half that of postprandial total discontinuous oral feeding (75.6 +/- 6.6 pg/ml, p less than 0.001) and postprandial controls (75 +/- 7 pg/ml, p less than 0.001). Thus, basal and stimulated CCK levels are similar in children and adults, and the use of long-term artificial nutritional techniques does not modify the feeding stimulation of CCK. Plasma CCK levels during TPN and constant rate enteral nutrition are similar to fasting values, indicating a possible role for CCK in the biliary sludge.