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Biomedical subjects

J B Otte

Publications and source records attributed to J B Otte.

At least 127 records · Page 7Linked to original sources
PubMed1992

Progressive cardiac failure following orthotopic liver transplantation for type IV glycogenosis.

Orthotopic liver transplantation (OLT) has been proposed to treat patients with type IV glycogenosis because of early progressive cirrhosis. Reports have shown absence of disease progression in other organs after OLT and even regression of cardiac amylopectin infiltration in one case. We describe a 15-month-old child in whom a liver transplant was performed for type IV glycogenosis. There were no clinical signs of extrahepatic disease before OLT. Nine months later, the patient developed progressive cardiac insufficiency and died from cardiac failure. Because of massive amylopectin deposits, decreased myofibrils in cardiac cells, and exclusion of other causes of cardiac failure, death was attributed to amylopectionosis. Our observation contrasts with the Pittsburgh experience and suggests that cardiac amylopectionosis may progress after OLT.

Amylopectin↗
PubMed1992

Upper gastro-intestinal tract bleeding in cirrhotic children candidates for liver transplantation.

Signs of portal hypertension, history of upper gastro-intestinal tract bleeding episodes and outcome of the latter were recorded in 76 cirrhotic children evaluated for liver transplantation. Fifty-three (70%) had varices and 22 (29%) had experienced upper gastro-intestinal tract bleeding. Of these 22, 19 bled from varices and 3 from ulcers. Non bleeding ulcers were also found in five patients bleeding from varices. Iterative sclerotherapy controlled acute variceal bleeding in all but one patient in whom emergency transplantation was performed. Six of the eight patients with ulcers were successfully treated by the H2 histamine receptor antagonist ranitidine. We conclude that iterative sclerotherapy is efficient to control acute variceal bleeding and prevents recurrent bleeding in children with end-stage liver diseases awaiting liver replacement. Bleeding asymptomatic ulcers are frequent and respond to H2 histamine receptor antagonists.

Acute Disease↗
PubMed1992

Acute adenovirus hepatitis in liver transplant recipients.

An acute or fulminant adenovirus hepatitis developed in 5 of 224 pediatric patients who were recipients of orthotopic liver transplants. All had received prednisolone, azathioprine, and cyclosporine as basal immunosuppression, and four received monoclonal (OKT3) or polyclonal (antithymocyte globulin) antibodies for steroid-resistant rejection episodes. These patients initially had high fever and a worsening condition for a mean of 73 days after transplantation (range 44 to 140 days). Results of biochemical tests showed very high serum levels of lactate dehydrogenase. Aspartate aminotransferase values were always markedly more elevated than those of alanine aminotransferase. Two patients had severe leukopenia. Results of histologic studies of the liver showed extensive areas of confluent necrosis and targetlike hepatocyte nuclei. Typical intranuclear viral inclusions were observed on electron microscopy. Adenovirus was cultured in all patients and in two relatives. Two patients died of liver failure; others recovered after cessation of immunosuppression. We conclude that adenovirus hepatitis can be fatal in liver transplant recipients. There is no specific treatment, and immunosuppression must be discontinued.

Adenovirus Infections, Human↗
PubMed1992

Hepatic localization of a fibrosarcoma in a child with a liver transplant.

Two years after an orthotopic liver transplantation, a multifocal hepatic tumor with lymphonodular metastases, identified as a fibrosarcoma, developed in a 4-year-old girl being treated with cyclosporine. On a needle biopsy sample, genetic typing of the HLA-DR group revealed that tumoral cells were from the recipient.

Child, Preschool↗
PubMed1992

Quality of life three years or more after esophagectomy for cancer.

The quality of life and alimentary comfort of 17 patients with esophageal cancer who were disease free more than 3 years after an esophageal resection were evaluated by analyzing responses to a follow-up questionnaire. Fourteen patients had subtotal esophagectomy and gastric pull-up to the neck. Three patients underwent a total esophagopharyngolaryngectomy, the digestive continuity being restored by means of an isoperistaltic colon segment interposed between the base of the tongue and the stomach. Current body weight, when compared with that existing postoperatively, was increased in 13 patients and unchanged in four. The number of meals per day was an average of 2.8, but 12 patients took additional snacks between main meals (2.3 as a mean). The major long-term complaints were a sensation of early fullness during eating in 11 patients, dysphagia in three, diarrhea in two, cough-induced vomiting in two, and postprandial sweating in two. Ratings given by self-evaluation of current alimentary comfort in comparison with that predating the initial esophageal symptoms ranged from 3 of 10 to 10 of 10 (mean 7.1/10). Thirteen patients led active lives, seven at home and six employed outside the home. The present survey suggests that most disease-free patients may obtain a satisfactory quality of life after esophagectomy and gastric or colonic pull-up; long-term alimentary comfort is conditioned mainly by the small capacity of the esophageal substitute.

Adult↗
PubMed1992

Retroperitoneal laparostomy: a surgical treatment of pancreatic abscesses after an acute necrotizing pancreatitis.

BACKGROUND: From 1982 to 1988, 20 patients with pancreatic abscesses after an acute necrotizing pancreatitis underwent a retroperitoneal laparostomy (RPL). METHODS: The severity of the disease was assessed by Ranson's bioclinical and Hill's computed tomographic scoring systems. The RPL, guided by the results of repeated computed tomographic scans (high frequency of peripancreatic necrotic extension through the anterior pararenal space) consists of a left or right lateral incision under the twelfth rib, allowing direct access to the pancreas and peripancreatic spaces. RESULTS: Four patients (20%) had local complications: colonic fistula (one patient), gastric and colonic fistula (one patient), jejunal fistula (one patient), and local hemorrhage (one patient). Only one complication was lethal (gastric and colonic fistula). Four patients died (mortality rate 20%). In two of the cases death was related directly to a persistent sepsis after the RPL, whereas the two other patients died despite a complete surgical drainage. CONCLUSIONS: RPL (left or right, sometimes bilateral) allows a total exploration of the pancreas and peripancreatic spaces in most cases, as well as a complete manual removal of the necrotic infected masses. Furthermore, several second-look removals of newly formed necrotic masses can be performed without the risk of peritoneal contamination and with a low rate of digestive fistula.

Abscess↗
PubMed1991

Platelet aggregates in small lung vessels and death during liver transplantation.

10 children who died suddenly during liver transplantation were found at necropsy to have extensive obstruction of small lung vessels by platelet aggregates. In 7 of these patients pulmonary artery pressure changes before death were consistent with acute obstruction of the pulmonary vascular bed. Platelet aggregates were not strikingly increased in blood vessels in other tissues. No single obvious cause for these unusual histological findings could be identified, although the presence of intravascular catheters, perioperative blood and platelet concentrate transfusions, and cellular debris from the liver forced into the circulation during surgery might predispose to platelet aggregation.

Capillaries↗
PubMed1991

Effectiveness and toxicity of cisplatin and doxorubicin (PLADO) in childhood hepatoblastoma and hepatocellular carcinoma: a SIOP pilot study.

Sixteen children, aged 16 days to 13 years with hepatoblastoma (HB) (13 patients) or hepatocellular carcinoma (HCC) (3 patients), were given a total of 89 courses of cisplatin and doxorubicin (PLADO) as IV continuous infusion. All tumors were confined to the liver except for 1 hepatoblastoma patient with pulmonary metastases at presentation. Tumor response to PLADO was evaluable in 10 children (8 HB, 2 HCC) treated with preoperative chemotherapy and in another 2 HB patients treated when they developed pulmonary metastases after initial treatment with surgery alone. There were 2 complete responses (2 HB with pulmonary recurrences), 7 very good partial responses (6 HB and 1 HCC), 2 partial responses (1 HB, 1 HCC), and 1 stable disease (HB). The last patient underwent orthotopic liver transplantation whereas all the other patients had their tumor completely excised at delayed surgery. Documented toxicity was BM depression (16 patients), infection (11), vomiting (11), mucositis (3), hearing loss (1), and cardiotoxicity (1). These data indicate that PLADO in continuous infusion is effective in the treatment of malignant epithelial liver tumors with acceptable toxicity.

Adolescent↗
PubMed1991

Intrathoracic Nissen fundoplication: long-term clinical and pH-monitoring evaluation.

From 1976 until April 1989, 31 intrathoracic total fundoplications were performed for reflux esophagitis and irreducible hiatus hernia. In the first 16 patients (group 1) the operation was complicated with acute perforation of the wrap in 4 cases, bronchogastric fistula in 1, and herniation of the wrap higher in the chest in 1. Technical modifications were applied to 15 more recent patients (group 2). These are enlargement of the hiatus, looseness of the wrap and its appropriate anchorage, avoidance of forceps when handling the stomach, care with the vagi, and efficient gastric decompression in the postoperative period. The postoperative course was always uneventful in group 2. Twenty-six patients, who still have their initial wrap, were considered for clinical evaluation: 11 from group 1 (mean follow-up, 81.5 months) and 15 from group 2 (mean follow-up, 32.8 months). All are free from any symptom of reflux; gas-bloat syndrome is infrequent and dysphagia is relieved. Twenty-four-hour pH monitoring, performed in 14 patients (3 from group 1 and 11 from group 2) (mean follow-up, 42 months), was normal in 13; a pathological upright reflux (time pH less than 4, 8.4%) was demonstrated in one symptom-free woman in whom endoscopy was unremarkable. Mechanisms of complications experienced in group 1 are analyzed in the light of the technical evolution of the procedure, and the place of the intrathoracic total fundoplication in the management of short esophagus is defined, considering the other available surgical techniques.

Cardia↗
PubMed1991

Patterns of hepatitis delta virus reinfection and disease in liver transplantation.

Twenty-seven carriers of the hepatitis B surface antigen who underwent liver transplantation in Italy and Belgium for terminal Hepatitis delta virus (HDV) cirrhosis were investigated. In 22 of the patients, HDV infection recurred. Two patients died of coexisting HDV and hepatitis B virus (HBV) reactivation. Four patients who died of unrelated causes were found to have HDV without signs of HBV reactivation. Five patients (18%) cleared both HBV and HDV after transplantation with no evidence of hepatitis (mean follow-up, 29 months). In many surviving patients. HDV infection recurred early without signs of HBV reactivation. Disease returned in the 11 HDV-infected patients in whom HBV also recurred. Histological hepatitis did not recur during an interim of 12-33 months in the 5 HDV-infected patients in whom HBV did not return. The overall medium-term survival in patients with HDV who underwent transplantation was 77.7%. Liver transplantation offers patients with HDV a hope of cure from disease despite a high risk of reinfection. In the transplantation setting. HDV can cause subclinical infections without any apparent assistance from HBV; these infections become symptomatic only if and when HBV reactivates. Thus, HDV may not be in itself pathogenic but requires cooperation from HBV to cause the appearance of the disease.

Adolescent↗
PubMed1991

Liver transplantation in children with biliary atresia and polysplenia syndrome.

Biliary atresia is the most common indication for orthotopic liver transplantation (OLT) in children. The polysplenia syndrome anomalies, which occur in approximately 10% of children with biliary atresia, may represent special difficulties at liver transplantation. We have reviewed our experience with this syndrome in 116 children with biliary atresia who underwent liver transplantation between March 1984 and December 1989. The main features of the polysplenia syndrome, which included absence of the inferior vena cava, preduodenal portal vein, midgut malrotation, aberrant hepatic artery, and situs inversus, were encountered in 12 of the 116 children (10.3%). Severe portal vein hypoplasia (3.5 mm or smaller) was also present in 7 of these children. Eight patients received a complete and four received a reduced liver graft. The vascular anomalies increased the technical difficulty of OLT but could be surmounted, although they did contribute to the peroperative death of one child. The 1-month survival rate was 83% for the 12 children with features of the polysplenia syndrome and 88% for the other 92 children with biliary atresia alone.

Abnormalities, Multiple↗