Changes in life-style after liver transplantation

Sixty-five pediatric patients who received liver transplants between May 1981 and May 1984 were observed for as many as 5 years and examined for changes in life-style. Children were less frequently hospitalized, spent less time hospitalized, required fewer medications, and generally had excellent liver and renal function after hepatic transplantation as compared with their pretransplantation status. Most children were in age-appropriate and standard school classes or were only 1 year behind. Cognitive abilities remained unchanged. Children improved in gross motor function and patients' behavior significantly improved according to parents' perceptions. Enuresis was more prevalent, however, than in the population of children who had not received liver transplants. Parental divorce rates were no greater than those reported for other families with chronically ill children. Overall, objective changes in life-style as well as parents' perceptions of behavior of children appear to be improved after liver transplantation

Distal Splenorenal Shunt for Portal Vein Thrombosis after Liver Transplantation

A 17‐yr‐oId female received a liver transplant for type I glycogen storage disease. A year later, when she experienced variceal gastrointestinal hemorrhage, an angiogram revealed thrombosis of the portal vein with hepatopetal collateral channels. A distal splenorenal shunt was performed because of failure of sclerotherapy to control subsequent bleeding episodes and the fact that the liver function was normal. This patient continues to have normal hepatic function with a patent splenorenal shunt 4 yr after the shunting procedure. This case illustrates the feasibility of a distal splenorenal shunt to alleviate portal hypertension in cases of thrombosis of the portal vein following hepatic transplantation if the liver function is normal. Copyright © 1989, Wiley Blackwell. All rights reserve

CONSENSUS CONFERENCE REPORT ON LIVER* TRANSPLANTATION.

Liver transplantation has been developed to the point of a service operation, the exploitation of which depends upon the establishment of multiple regional centers. The increased use of this procedure will permit the delivery of optimum health care to victims of end stage liver disease

Percutaneous transhepatic cholangiography and biliary drainage after liver transplantation: A five-year experience

Evaluation of the biliary tract by percutaneous transhepatic cholangiography (PTC) is often required in liver transplant patients with an abnormal postoperative course. Indications for PTC include failure of liver enzyme levels to return to normal postoperatively, an elevation of serum bilirubin or liver enzyme levels, suspected bile leak, biliary obstructive symptoms, cholangitis, and sepsis. Over a 5-year period 625 liver transplants in 477 patients were performed at the University Health Center of Pittsburgh. Fifty-three patients (56 transplants) underwent 70 PTCs. Complications diagnosed by PTC included biliary strictures, bile leaks, bilomas, liver abscesses, stones, and problems associated with internal biliary stents. Thirty-two percutaneous transhepatic biliary drainage procedures were performed. Ten transplantation patients underwent balloon dilatation of postoperative biliary strictures. Interventional radiologic techniques were important in treating other complications and avoiding additional surgery in many of these patients. © 1987 Springer-Verlag New York Inc

Liver and kidney transplantation in children receiving cyclosporin A and steroids

The new immunosuppressive agent, cyclosporin A, was used with low doses of steroids to treat eight patients undergoing hepatic transplantation and three patients undergoing cadaveric renal transplantation. Seven of the eight liver recipients are well, including one who was given two livers. The three kidney recipients, who had developed cytotoxic antibodies after previously rejecting grafts with conventional immunosuppressive therapy, have had good results despite conditions which usually preclude attempts at transplantation. The ability to control rejection effectively and safely without chronic high-dose steroid therapy may make the described therapeutic regimen valuable for pediatric recipients of whole organs. © 1982 The C. V. Mosby Company

Choosing a pediatric recipient for orthotopic liver transplantation

Between March 3, 1981, and June 1, 1984, 216 children were evaluated for orthotopic liver transplantation. Of the 216 patients, 117 (55%) had recelved at least one liver transplant by June 1, 1985. Fifty-five (25%) died before transplantation. The 117 patients who received transplants were grouped according to severity of disease and degree of general decompensation at the time of transplantation. The severity of a patient's medical condition with the possible exception of deep hepatic coma, did not predict outcome following orthotopic liver transplantation. Seventy variables were assessed at the time of the evaluation. Twenty-three of the 70 variables were found to have prognostic significance with regard to death from progressive liver disease before transplantation. These 23 variables were Incorporated into a multivariate model to provide a means of determining the relative risk of death among pediatric patients with end-stage liver disease. This information may allow more informed selection of candidates awaiting liver transplantation. © 1987 The C. V. Mosby Company

ALPK1 missense pathogenic variant in five families leads to ROSAH syndrome, an ocular multisystem autosomal dominant disorder

Purpose: To identify the molecular cause in five unrelated families with a distinct autosomal dominant ocular systemic disorder we called ROSAH syndrome due to clinical features of retinal dystrophy, optic nerve edema, splenomegaly, anhidrosis, and migraine headache. Methods: Independent discovery exome and genome sequencing in families 1, 2, and 3, and confirmation in families 4 and 5. Expression of wild-type messenger RNA and protein in human and mouse tissues and cell lines. Ciliary assays in fibroblasts from affected and unaffected family members. Results: We found the heterozygous missense variant in the ɑkinase gene, ALPK1, (c.710C>T, [p.Thr237Met]), segregated with disease in all five families. All patients shared the ROSAH phenotype with additional low-grade ocular inflammation, pancytopenia, recurrent infections, and mild renal impairment in some. ALPK1 was notably expressed in retina, retinal pigment epithelium, and optic nerve, with immunofluorescence indicating localization to the basal body of the connecting cilium of the photoreceptors, and presence in the sweat glands. Immunocytofluorescence revealed expression at the centrioles and spindle poles during metaphase, and at the base of the primary cilium. Affected family member fibroblasts demonstrated defective ciliogenesis. Conclusion: Heterozygosity for ALPK1, p.Thr237Met leads to ROSAH syndrome, an autosomal dominant ocular systemic disorder