Biomedical subjects

D Alagille

Publications and source records attributed to D Alagille.

168 records · Page 10Linked to original sources

PubMedDate not supplied

[Medical aspects of children with portal hypertension at Bicêtre hospital. A study of 282 cases (1954-1981) (author's transl)].

282 children with portal hypertension (PHT) were studied. PHT was due to extra-hepatic portal venous obstruction in 40% of children, to intra-hepatic disease in 56% and to post-sinusoídal lesions in 4%. Presenting symptoms, and risk of gastro-intestinal bleeding were analyzed in each etiologic group, as well as the follow-up in children in whom a portal-systemic shunt was not carried out. In portal venous obstruction, gastro-intestinal bleeding occurred frequently and early in life; liver failure and portal systemic encephalopathy did not occur; none of these children died except those with severe congenital heart disease. In children with cirrhosis, gastro-intestinal bleeding occurred twice less frequently and its date depended upon the etiology of cirrhosis; in this group, death was due twice more often to the underlying liver disease than to gastro-intestinal bleeding. In supra-hepatic PHT, the risks of PHT proper are limited except for ascites.

Adolescent↗

PubMedDate not supplied

[Portal hypertension in children. Hemorrhagic risk and measures for prevention (author's transl)].

The retrospective analysis of 201 children who presented with portal hypertension and were investigated by esophageal endoscopy provides the following results : 1) esophageal varices were present in 90% of the children examined; 2) the hemorrhagic risk is much higher in children with extra hepatic portal obstruction (gastrointestinal bleeding occurred in 80% of these children) than in children with intrahepatic or postsinusoidal portal hypertension (29%); 3) endoscopic signs of major portal hypertension such as tension of varices and/or congestion of the esophageal mucosa were present in virtually all children who experienced one or more episodes of gastrointestinal bleeding; 4) esophageal or gastric lesions not related to varices were responsible for bleeding in 6 children; 5) gastric varices were present in 21 of 105 children in whom a thorough gastric endoscopy was carried out; 6) partial or complete regression of varices occurred in 11 of 27 children in whom several endoscopies were performed with a follow-up of 9 months to 6 years; complete regression occurred only when varices were of small size; 7) measures used to prevent gastrointestinal bleeding in children with varices often failed when endoscopic signs of major portal hypertension were present. These results confirm the major importance of eso-gastric endoscopy in the investigation of children with portal hypertension and suggest that porto-systemic surgical shunts should be considered in children who experience at least one spontaneous episode of gastrointestinal bleeding provided the responsibility of varices is ascertained by endoscopy.

Child↗

PubMedDate not supplied

[Early gastro-intestinal bleeding in children with portal hypertension (author's transl)].

From the retrospective study of 47 children who presented with portal hypertension and gastro-intestinal bleeding before 3 years of age, the following conclusions can be drawn: 1) The cause of portal hypertension was extrahepatic portal obstruction in 35 children, cirrhosis in 10 and congenital hepatic fibrosis in 2. 2) Endoscopic signs of major portal hypertension were present in 26 of 27 children in whom a thorough endoscopic examination was carried out. 3) Most children experienced several episodes of bleeding. One or several hematemeses occurred in 36 children, one or several episodes of melaena in 23, one or several proctorrhagia in 5 and anemia alone in 2. Two children with liver failure died during a gastrointestinal bleeding episode. 4) Transthoracic direct ligation of esophageal varices was performed in 8 children ; all but one experienced one or more episodes of bleeding within one year after surgery. One child died during one of these episodes. 5) A surgical portosystemic shunt was carried out in 37 rated on before the age of 3. Shunts were successful in 7 of 11 children who were ope children. Patency of the shunt was demonstrated in 32. These results show that early shunt surgery can provide a permanent solution to the life-threatening problems of gastrointestinal bleeding in young children with portal hypertensions.

Age Factors↗

PubMedDate not supplied

[Portal hypertension and biliary atresia (author's transl)].

The study of portal pressure during surgical exploration for biliary atresia in the series of 260 children operated at the Hospital Saint-Vincent de Paul since 1968 showed that portal hypertension could be demonstrated in 61.8% of the cases before the age of 3 months. Among the 54 children surviving without jaundice, splenomegaly was found in 35 cases; esophageal varices were seen in 14 children, more frequently after 5 years of age. Bleeding from esophageal varices occurred only in cases with recurrence of jaundice or moderate biliary retention, i.e. for 6 children. A portocaval shunt was constructed in 7 cases; all had bled except one. One child died two and a half years later from pneumococcal sepsis after a splenorenal shunt with splenectomy; another child had numerous recurrence of bleeding episodes. Five children have a functional shunt and have not rebled. When biliary atresia is cured by hepatoportoenterostomy, with complete disappearance of biliary retention, the risk of bleeding from esophageal varices is unlikely, in spite of persistent cirrhotic alterations of the liver; in these children, there should very seldom be an indication for a portocaval shunt.

Bile Ducts↗

PubMedDate not supplied

[Surgical treatment of portal hypertension in children. Retrospective study of 157 cases (author's transl)].

From 1959 until 1981, 157 children were treated for portal hypertension by esophageal varices ligation in 13 cases and by portosystemic shunt in 144 cases. The age of the patients at operation was correlated with the cause of portal hypertension : mean age was six and a half years for cases with extra-hepatic blockage, and ten years for cases with cirrhosis. In 73% of cases, the shunt was undertaken following a bleeding episode from esophageal varices; at the present time, the decision to undertake a prophylactic type of shunt would be much more questionable. Central splenorenal shunt and mesocaval shunt were the operations most frequently performed by the different surgical teams in charge of these children (respectively 69 and 47 cases). Among the postoperative complications, three cases of venous stasis in lower limbs occurred after a mesocaval shunt; one child died two and a half years after a central splenorenal shunt from pneumococcal sepsis. During the last two years, there is a tendency in our group to perform a Warren shunt for intrahepatic portal hypertension, and a mesocaval shunt with jugular vein interposition in the case of extrahepatic portal hypertension. Recurrence of bleeding from esophageal varices after simple ligation has been observed in 64% of the cases; after portosystemic shunts, the anastomosis was a success in 89.3% of the cases. Whereas a significant fall in portal pressure after completion of the anastomosis is of good prognostic value, the fact that in some cases intraoperative measurement of pressure before and after shunting may show no difference does not imply a secondary thrombosis of the anastomosis, since this complication was seen in only 13% of the cases in these conditions.

Adolescent↗

PubMedDate not supplied

[Portal hypertension in children. Follow-up after portal systemic shunts (author's transl)].

The retrospective study of 115 children in whom a successful portosystemic shunt was carried out for portal hypertension, provides the following conclusions: 1) Patency of the shunt must be checked by esophageal endoscopy, six months postoperatively. A patent shunt can be expected when the size of the spleen and/or thrombocytopenia improve in the early post-operative period. Early ultrasound examination is also very useful in that respect. 2) None of the children with extrahepatic portal vein obstruction or congenital hepatic fibrosis presented with clinical signs of portal systemic encephalopathy (PSE). 3) Eight of 30 children with cirrhosis presented with one or more clinical episodes of PSE. Some were transient allowing for a normal diet to be resumed later on. 4) It is thus advisable to give children with cirrhosis a low protein diet in the months after surgery. Protein content of the diet can usually be increased progressively over a period of one to two years. 5) Whenever splenectomy is necessary, it is mandatory to prevent pneumococcal infections with the pneumococcal vaccine and daily treatment with oral penicillin.

Adolescent↗