Indications for pediatric liver transplantation.

Two hundred fifty pediatric (less than 18 years of age) patients underwent orthotopic liver transplantation because of end-stage liver disease and were given combination therapy with cyclosporine and prednisone. The most common indications for transplantation in decreasing order of frequency were biliary atresia, inborn errors of metabolism, and postnecrotic cirrhosis. The 5-year actuarial survival for the entire group was 69.

Adriamycin resistance in HL60 cells and accompanying modification of a surface membrane protein contained in drug-sensitive cells.

HL60 cells resistant to Adriamycin contain a 32P-labeled, Mr 150,000 surface membrane protein (p150) which is not detected in cells sensitive to drug. The levels of phosphorylation of this protein increase with increasing levels of resistance. Analysis of plasma membranes prepared from cells labeled with [14C]glucosamine shows, however, that both sensitive cells and those exhibiting an 80-fold increase in drug resistance contain essentially identical levels of a highly glycosylated Mr 150,000 protein.

Orthotopic liver transplantation for alpha-1-antitrypsin deficiency: an experience in 29 children and ten adults.

Thirty-nine patients (29 children and ten adults) underwent OLT for liver disease associated with A1AD from March 1980 to March 1986. Thirty of thirty-six patients (83%) with available data were homozygous phenotype PiZZ. The other six were Pi heterozygotes, being either PiMZ or PiSZ.

Sucralfate treatment of nonsteroidal anti-inflammatory drug-induced gastrointestinal symptoms and mucosal damage.

In a randomized, double-blind trial, sucralfate therapy, 1 g four times daily, was compared with placebo in 143 symptomatic patients to assess the treatment of gastrointestinal symptoms and gastric mucosal damage associated with nonsteroidal anti-inflammatory drugs (NSAIDs). All patients followed a fixed regimen of NSAIDs, were assigned to one of two groups based on the presence or absence of gastric erosions at baseline endoscopy, and were then assigned randomly to receive sucralfate or placebo for four weeks. Patients were then followed for up to six months while receiving open-label sucralfate 1 g twice daily to up to 1 g four times daily.

Presacral myelolipoma.

Extramedullary tumors of hematopoietic tissue can be divided into two categories: those associated with a bone marrow disorder, myelolipomas which have no apparent relation to a bone marrow disorder. The etiology of myelolipoma is unknown and the vast majority are adrenal in location. Extramedullary myelolipomas are usually presacral in location and none of the approximately ten reported cases has been diagnosed before surgery.

The first example of autoanti-Kx.

An IgG autoantibody with Kx specificity was found in the blood of a 61-year-old white man. The antibody did not cause hemolysis of his own or transfused Kx-positive red cells. The patient is of common Kell blood type and does not exhibit any clinical or hematologic features of McLeod syndrome.

Liver transplantation before 1 year of age.

Since 1981, 20 infants younger than 1 year of age received 26 orthotopic liver transplants. Immunosuppression was with cyclosporine and corticosteroids. Thirteen (65%) of the recipients were discharged from the hospital.

Acquired loss of red cell Kell antigens.

A 19-year-old patient with a long history of idiopathic thrombocytopenic purpura developed a potent antibody against a high-incidence antigen in the Kell blood group system. The direct antiglobulin test on his red cells was negative. His cells exhibited profound depression of Kell blood group antigens, but antigens of other blood groups were normal.

K23. A low-incidence antigen in the Kell blood group system identified by biochemical characterization.

An antibody in the serum of a gravida 4, para 3 woman reacted with red cells from two of her children, her husband, and his mother, but with none of more than 2100 reference red cell samples and blood samples from donors. The reactive antigen was inactivated by 2-aminoethylisothiouronium bromide or dithiothreitol-papain treatment. The antigen was immunoprecipitated from paternal red cells with maternal antibody and shown to migrate by sodium dodecylsulfate polyacrylamide gel electropheresis as a single protein of approximately 93,000 daltons.

Localized conjunctival amyloidosis associated with extranodal lymphoma.

A 62-year-old man was found to have a large asymptomatic amyloid nodule of the conjunctiva. Within the next year, a diffuse mixed small and large cell lymphoma of his scapula developed with amyloid in its stroma. No abnormal serum or urine protein was found.

Evaluation of red cell volume distribution width (RDW).

The purpose of this study was to evaluate red cell distribution width (RDW) in the differential diagnosis of patients with well defined iron deficiency (ID), anemia of chronic disease (ACD), and thalassemia trait. Mean RDW in 100 "normals" was 13.1%.

Computer-assisted stereotactic biopsy of intracranial lesions.

The use of a computer program that allows the integration of stereotactically gathered CT, MRI and digital angiographic data in the planning of a biopsy trajectory is described. This system has been used to perform 447 stereotactic biopsies in 439 patients. Intracranial hemorrhages occurred in three patients; combined morbidity and mortality was less than 1%.

Visualization of c-Ki-ras-2 oncogene sequences in human pancreas, a chemically induced transplantable carcinoma, and carcinomas of pancreas by in situ hybridization.

c-Ki-ras-2 sequences were visualized in paraffin-embedded sections from normal fetal and adult human pancreases, a chemically induced transplantable human pancreas carcinoma (PT-1) and three carcinomas of pancreas by in situ hybridization technique. A biotinylated 1-kilobase-pair (kb) EcoRI fragment of pHiHi3 DNA was used as probe and the oncogene was visualized as one or two large grains of reaction products produced by streptavidin-peroxidase complex and diaminobenzidine tetrachloride in more than 9% of normal pancreas nuclei. Its amplification in the chemically induced cell line was detected as one or more large grains in 72% of the nuclei and numerous cytoplasmic grains.

Effect of hyperglycemia on brain pH levels in areas of focal incomplete cerebral ischemia in monkeys.

The adverse effect of a minimal cerebral blood flow (CBF) in models of global ischemia has been noted by many investigators. One factor believed important in this situation is the level of blood glucose, since a continued supply of this metabolite results in increased tissue lactate, decreased brain pH, and increased cell damage. The authors have extended these observations to a model of focal incomplete ischemia.

Saphenous vein bypass grafts for giant aneurysms and intracranial occlusive disease.

The authors report their experience with the use of saphenous vein bypass grafts for treating advanced occlusive disease in the posterior circulation (77 patients, all of whom had failed medical management and showed severe ischemic symptoms), deteriorating patients with giant aneurysms of the posterior circulation (nine patients), progressive ischemia in the anterior circulation (26 patients, none of whom had a normal examination), and giant aneurysms in the anterior circulation (20 patients, all of whom presented with mass effect or subarachnoid hemorrhage). Graft patency in the first 65 cases treated was 74%. However, after significant technical changes of vein-graft preparation and construction of the proximal anastomosis, patency in the following 67 cases was 94%.

Estrogen-progesterone therapy for bleeding gastrointestinal telangiectasias in chronic renal failure. An uncontrolled trial.

Gastrointestinal telangiectasias cause hemorrhage in patients with chronic renal failure. Therapies using vasoconstrictors, endoscopic application of heat, and surgery have had limited efficacy. Because several reports have suggested that estrogen or estrogen-progesterone therapy may control mucosal bleeding from telangiectasias in patients with hereditary hemorrhagic telangiectasia, we treated seven patients with chronic renal failure and bleeding gastrointestinal telangiectasias with systemic estrogen or estrogen-progesterone in an uncontrolled trial.

Castleman's disease in children.

A three and one-half-year-old girl and a 12-year-old boy presented with features of the two clinical presentations of Castleman's disease or giant lymph node hyperplasia. The girl presented with anemia, fever, night sweats, hypergammaglobulinemia, and a palpable abdominal mass. Her symptoms were consistent with those seen in the plasma-cell type of this disease.

Ventricular ectopy associated with peroral colonic lavage.

Peroral colonic lavage solutions are widely used to prepare patients for colonoscopy. The effects of peroral colonic lavage on cardiac rhythm have not been evaluated previously. Using continuous electrocardiographic monitoring, the authors evaluated cardiac rhythm in 22 patients undergoing 24 colonoscopic examinations.

Isolation and characterization of adriamycin-resistant HL-60 cells which are not defective in the initial intracellular accumulation of drug.

Two human leukemia cell lines (Molt-4 and HL-60) have been used for establishing cells which exhibit a low level resistance to Adriamycin. Analysis of drug uptake patterns shows that the Molt-4 resistant cells are defective in the initial intracellular accumulation of drug. In contrast to Molt-4 the levels of drug which accumulate in the sensitive and resistant HL-60 cells during a 60-min incubation period are essentially the same.