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Biomedical subjects

J Sarles

Publications and source records attributed to J Sarles.

At least 19 recordsLinked to original sources
PubMed1998

Renovascular hypertension and vascular anomalies in Alagille syndrome.

Alagille syndrome (AS) is characterized by the association of at least three of the following five abnormalities: chronic cholestasis, peripheral pulmonary artery stenosis, vertebral arch defects, embryotoxon, and typical facies. In addition to urological abnormalities, tubulointerstitial nephritis, renal tubular acidosis, and mesangiolipidosis have been noted in AS. The usual manifestations of such renal pathologies rarely include hypertension. We report five patients with at least four of the five major features of AS who developed secondary hypertension of renovascular origin 3.5-28 years after the initial diagnosis of AS. Angiography demonstrated uni- or bilateral renal artery stenosis and various other abnormalities of the main arteries in all five patients: aorta (3 cases), celiac artery (4 cases), superior mesenteric artery (1 case), subclavian artery (1 case). Our findings underscore the value of arterial blood pressure monitoring in patients with AS. If hypertension occurs, a renovascular origin should be sought. The diffuse vascular abnormalities which appeared to be a feature of AS in these patients should prompt larger studies of vascular abnormalities in AS.

Acidosis↗
PubMed1998

Interferon alfa therapy for chronic hepatitis B in children: a multinational randomized controlled trial.

BACKGROUND & AIMS: Treatment of chronic hepatitis B with interferon alfa is not approved in children. The aim of this study was to evaluate the safety and efficacy of interferon alfa (IFN-alpha) in children with chronic hepatitis B and increased transaminase levels. METHODS: Children were given either IFN-alpha2b (6 megaunits/m2 thrice weekly for 24 weeks) or no treatment. Clearance of markers of viral replication was evaluated 24 weeks after therapy and after 48 weeks of observation in controls. RESULTS: Of 149 children enrolled, 144 were evaluable (70 treated and 74 controls). Serum hepatitis B e antigen and viral DNA became negative in 26% of treated children and 11% of controls (P < 0.05). Serum aminotransferase levels normalized and liver histology improved among responders. Hepatitis B surface antigen became undetectable in 10% of treated patients and 1% of controls. Female gender and interferon treatment were the only significant predictors of response. Ethnic origin, baseline aminotransferase level, initial DNA levels, and histology did not correlate with response. Most adverse reactions were mild or moderate, and dose was reduced in 24% of children. CONCLUSIONS: In children with chronic hepatitis B, INF-alpha promotes loss of viral replication markers and surface antigen and improves aminotransferases and histology.

Adolescent↗
PubMed1996

[Sleeping position, prevention of sudden death syndrome and gastroesophageal reflux].

Based on results of epidemiological studies, dorsal or lateral sleeping positions are now recommanded in the prevention of sudden infant death syndrome (SIDS). This raises an ethical question about the attitude towards the ventral positioning therapy for gastroesophageal reflux (GOR). The consensus conference considers that the ventral position should only be recommanded in GOR when the benefit appears to outweigh the risk of SIDS that it induces. The conference proposes that for infants with simple uncomplicated reflux, sleeping in the prone position should not be introduced in the first line treatment. Prone positioning should be restricted to complicated cases resistant to dietary and medical measures.

Gastroesophageal Reflux↗
PubMed1996

Successful medical treatment of severely decompensated Wilson disease.

Delayed response to medical treatment sometimes leads to unnecessary liver transplantation in patients with severely decompensated Wilson disease. We report the course of five patients (mean age 13.4 years, range 11 to 15 years) with severely decompensated Wilson disease who were successfully treated medically. Prothrombin time improved after a minimum of 1 month and returned to normal within 3 months to 1 year or more.

Adolescent↗
PubMed1994

The pancreatitis-associated protein (PAP). A new candidate for neonatal screening of cystic fibrosis.

Neonatal screening of cystic fibrosis (CF) is presently based on immunoreactive trypsin (IRT) assay in blood spots, whose low specificity is a matter of concern. Because the pancreatitis-associated protein (PAP) proved to be a better serum marker of pancreatic alteration than exocrine enzymes, it might be an interesting alternative for CF screening. We report here a preliminary evaluation of the PAP test, conducted in retrospect on blood spots from groups of neonates already screened for CF with the IRT. Neonates with elevated IRT were submitted to subsequent analysis of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene. Neonates with normal IRT (n = 990) or high IRT but normal genotype (n = 28) had normal PAP. Elevated PAP was observed in all CF neonates (n = 11). False-positives for the PAP test were found only among neonates with high IRT and heterozygotes for a mutation in the CFTR gene (6 out of 17 cases). That group represents less than 0.2% of newborns. These results therefore suggest that PAP discriminates CF neonates with a significantly better specificity than IRT.

Antigens, Neoplasm↗
PubMed1994

[Antigliadin, antireticulin, antiendomysium antibodies: value in the diagnosis and follow-up of celiac disease in children].

In recent years, the clinical spectrum of coeliac disease has changed and forms with mild aspecific symptoms are today frequent. Therefore many infants are submitted to jejunal biopsy in order to exclude coeliac disease or to allow an early diagnosis. This has led to a search for a simple and reliable diagnostic test of coeliac disease in order to limit the use of jejunal biopsy. Recent data suggest that the study of serum antigliadin, antireticulin and antiendomysium antibodies may possibly play the role. In this paper the working group on coeliac disease of the Groupe Francophone de Gastroentérologie et Nutrition Pédiatriques expresses its view on the place of the dosages of these antibodies in the diagnosis and follow up procedures of coeliac disease in infants and children. At the present time, although it allows a simplification of the procedures, these dosages are presently not sufficiently reliable to serve as a substitute of jejunal biopsy.

Autoantibodies↗
PubMed1993

Pancreatic function and congenital duodenal anomalies.

Six children operated on for congenital anomalies of the duodenum were investigated to find out if pancreatic dysfunction was associated with the duodenal malformation, even in the absence of clinical evidence of pancreatic insufficiency. None of the children had diarrhea and none requested nutritional support. Pancreatic function was assessed by enzyme activities (lipase, trypsin, and chymotrypsin) bicarbonate and calcium measurements in pancreatic juice obtained through a nasoduodenal tube under stimulation by secretin and cerulein. Results showed no significant modification in hydro-electrolytic secretion, but impairment of enzymatic secretion was seen. The physiopathological relationship between duodenal anomalies and pancreatic dysfunction is discussed.

Child, Preschool↗