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Biomedical subjects

M Burdelski

Publications and source records attributed to M Burdelski.

At least 127 records · Page 7Linked to original sources
PubMed1986

[Liver transplantation in childhood].

From 1977 to 1985 altogether 143 children were referred to our hospital for liver transplantation. These children were aged 6 months to 15 years. According to the results of a defined examination protocol liver transplantation was indicated in 102 of these children. Contraindications were observed in 17 patients. In 14 children liver transplantation was not yet indicated. Parents of 8 children refused transplantation. Only 30 children have been transplanted so far. Out of these, 21 actually survive. The cumulative 5-year survival rate after transplantation is calculated to be 60.5%.

Adolescent↗
PubMed1986

[Primary polyuric kidney failure and acute yellow liver dystrophy following infusion of glucose substitutes in children].

Four children (aged 2 1/2-14 years) were given infusions of fructose, sorbitol and xylitol after sustaining head trauma (n = 3) or after attempted suicide with carbromal (n = 1). After transitory polyuria renal failure of varying severity set in three to five days after onset of the infusion treatment. Serum osmolality fell to 265-274 mosm/kg, haematocrit to 0.25-0.31, and hyponatraemia developed. Serum creatinine rose to maximally 256-930 mumol/l. Liver damage developed in parallel to the renal failure, two children dying with acute yellow liver atrophy. Two children--given symptomatic treatment with balanced equalization of the hyponatraemia, administration of frusemide and adjusted carbohydrate substitution--were discharged after four to eight weeks with normal renal and hepatic functions. Dialysis was not required. The hepatic and renal abnormalities must have been due to the high amounts of fructose, sorbitol and xylitol, to a total of 7.1-23.0 g/kg on the first day, well above recommended levels.

Acute Kidney Injury↗
PubMed1986

Selective determination of C-terminal (70-84) hPTH: elevated concentrations in cholestatic liver disease.

In order to obtain a better resolution of circulating PTH-fragments, we developed a radioimmunoassay with an antiserum raised against the synthetic 70-84 hPTH molecule in a sheep. [125I](69 Tyr) 70-84 hPTH was used as the radioactive tracer with the antiserum at a final dilution of 1:200 000. Displacement curves showed that only the 70-84 hPTH, (69 Tyr) 70-84 hPTH and 53-84 hPTH fragments were able to compete with the tracer. Synthetic 28-48 hPTH, 44-68 hPTH, (61 Tyr)62-68 hPTH, purified 1-84 hPTH and 53-84 bPTH showed no ability to displace the tracer indicating that the idiotype of the antibody is directed against the 70-84 portion that is usually hidden within the globular structure of the intact molecule. Healthy adults revealed PTH-peptide levels of 153 +/- 86 pg/ml (mean +/- 2 SD); 2 SD range: 67-239 pg/ml. C-terminal PTH-peptide (70-84 hPTH) concentration was normal in patients with primary hyperparathyroidism and chronic renal failure, thus excluding that neither the kidney nor the parathyroid gland is involved in the generation of this C-terminal PTH-peptide sequence. Increased levels of 70-84 hPTH immunoreactivity were found in most individuals with severe cholestatic liver disease. Since the liver appears to be responsible for part of the peripheral PTH metabolism, this assay system might be used as an indicator of altered liver function.

Adult↗
PubMed1985

[Hereditary chronic calcifying pancreatitis].

Four family members covering three generations presented with chronic calcifying pancreatitis. A tumor at the pancreas head was detected intraoperatively in a fifth elderly member of the family. Two of the four cases of chronic pancreatitis were diagnosed in childhood and one in adolescence. The fourth patient had typical symptoms during adolescence but the disease was not recognized at that time. Hereditary chronic pancreatitis has an autosomal-dominant inheritance with incomplete penetrance. The pathogenesis is not known. The course of the disease differed between the family members. Duodenal stenosis with gastrointestinal bleeding was observed, but also a more mild development with recurrent pain and long complaint-free intervals. The occurrence of complications and pain appears to decrease with increasing age. The extent of calcification, widening of the duct, exocrine and endocrine pancreatic insufficiency varies. The question of conservative or operative therapy depends on the course of the disease. A Whipple operation was vitally necessary in one child at the age of six. A pancreo-jejunostomy had to be introduced in a further member of the family at the age of 20 years due to an occlusion of the duct. Two patients receive only conservative treatment.

Adolescent↗
PubMed1985

Developments in liver transplantation.

Liver transplantation has gained increasing interest. While liver grafting for tumor is successful over prolonged periods only in its early course, liver grafting for end-stage cirrhosis may lead to a long survival. Liver grafting in children is the most successful indication; in adults the results depend largely on timing and indication. Actual developments are mainly seen in the following points: a. Improvement in immunosuppression by use of Cyclosporin A. The resorption and metabolism of the drug, in relation to liver function, have to be carefully observed. b. The tendency to perform liver grafting electively instead of in emergency. c. Improvement in operative management, particularly the use of veno-venous bypass. d. The best possible anaesthesiological and intensive care management for the patients. It can be expected, that these developments will enable continuous improvement of results, particularly in an elective situation. One hundred and forty liver grafts have been done in our institution and the results are discussed herein. Progress in liver transplantation is marked by steadily growing numbers of liver grafts performed, and of centers performing grafts, as well as by improved success rates and the recommendation of the U.S. National Institutes of Health, based on discussions at a liver transplantation consent meeting, held in June 1983. This interest is also reflected in discussions among the medical and non-medical community. The first section of this paper will deal with the present state and results of liver grafting particularly, at our own institution and some actual developments in this field will be discussed.

Bile Ducts↗
PubMed1985

Indications for liver transplantation in childhood.

Retrospective analysis of 97 children aged 3 months to 15 years presented for liver transplantation in our clinic indicates that children with extrahepatic biliary atresia (BA) show a cumulative survival of only 27% after 2.5 years of observation without transplantation. Children with cirrhosis of the liver of other origin (C) have an even worse cumulative survival rate of only 10% after the same time and treatment. Liver transplantation seems to be very urgent if there is a parallel drop of activity of pseudocholinesterase (CHE) below 1100 U/l, a drop of prothrombin test (PT) below 60% and a concomitant increase of concentration of bilirubin (Bili) and total serum bile acids (TBA) to 380 and 120 mumol/l respectively. In order to improve medical care of children with endstage liver disease it is mandatory to use all potential donor offers and to develop new surgical techniques such as transplantation of liver segments.

Adolescent↗
PubMed1984

Acrofacial dysplasia resembling geleophysic dysplasia.

We report on a 12-year-old girl with acrofacial dysplasia, growth retardation, joint contractures, mitral valve incompetence and focal hepatic storage of material reacting histochemically as glycoprotein. The patient's phenotype resembles that of patients with geleophysic dysplasia but differs with respect to facial appearance, milder changes of hand bones and normal capital femoral epiphyses. It is undecided if her disorder is part of a wider phenotypic spectrum of geleophysic dysplasia or a different entity.

Abnormalities, Multiple↗
PubMed1983

Liver transplantation in children.

Liver transplantation in children is still rarely performed although the prognosis for children with benign and final-stage liver disease and individual cases with unresectable hepatomas is acceptable. This report is based on the cases of eight children who underwent liver transplantation, in five cases for end-stage cirrhosis and in three cases for hepatoma. There was early mortality in only one case while in all the others development and rehabilitation were remarkable. For patients with malignancies, tumour recurrence is a limiting factor. Transplantation in biliary atresia is complicated when carried out at late stages of the disease. In cases where the time of indication is sufficiently early, a long waiting period for a suitable donor organ must be expected, during which several potential candidates may succumb to the underlying disease. Increasing experience and improved immunosuppressive therapy signify that liver transplantation can be considered more often in end-stage liver diseases.

Age Factors↗
PubMed1979

Endoscopic therapy of a congenital oesophageal stricture.

A sixteen months old girl with Down's-Syndrome suffered from congenital oesophageal membrane and segmental oesophageal stricture. In the neonatal period, two other associated malformations of the gastrointestinal tract (malrotation of gut and duodenal web) had been operated successfully. The first endoscopic examination of the eosophagus was performed at the age of sixteen months because of increasing dysphagia. A solid membrane in the lower part of the oesophagus was incised by diathermy. The associated segmental stenosis necessitated repeated bougienages. After this treatment the child developed well without clinical signs of dysphagia.

Down Syndrome↗
PubMed1979

[Peritoneoscopy in infancy and childhood, indications, technique and diagnostic value (author's transl)].

Indications, technique and diagnostic value of peritoneoscopy in infancy and childhood are reviewed from the literature; own results are discussed as well. Peritoneoscopy is an important procedure in the diagnosis of chronic inflammatory liver disease and gonadal dysgenesis in this age group. If other diseases are suspected, non-invasive examination techniques may give sufficient diagnostic information.

Biopsy↗
PubMed1979

[Ectodermal dysplasia and exocrine pancreatic insufficiency--a familial syndrome].

A syndrome was observed in two sisters which was characterized by a median defect of the hair of the head, hypoplasia of the ala nasi, absence of the permanent teeth, microdontia of the deciduous teeth, and exocrine insufficiency of the pancreas. This syndrome is probably autosomal regressive; the symptoms are similar to those of Johanson-Blizzard syndrome.

Child↗
PubMed1979

[Endoscopic retrograde cholangiopancreaticography (ERCP) and percutaneous transhepatic cholangiography (PTC) in cholestatic syndromes of children and adolescents (author's transl)].

Conventional cholangiography had failed to establish a diagnosis in 5 children aged 6 weeks to 14 years and in 6 adolescents aged 14 to 18 years suffering from cholestasis; therefore endoscopic retrograde cholangiopancreaticography (ERCP) was performed, allowing to establish congenital biliary tract anomalies, cholelithiasis and benign obstruction of the extrahepatic biliary tract as causes of the cholestatic syndromes. Thus ERCP again turned out to be a valuable diagnostic procedure. Percutaneous transhepatic cholangiography (PTC) using the Chiba technique has not yet been applied in infancy to a great extent. Application of PTC however should be considered as well in cholestasis. It cannot yet be decided, if ERCP or PTC is to be prefered in neonatal cholestasis; further investigations are needed to clear up this point.

Adolescent↗
PubMed1979

[Diagnostic value of laboratory and scintigraphic investigations in young infants with cholestatic jaundice (author's transl)].

The diagnostic value of laboratory and scintigraphic examination techniques in young infants with cholestatic jaundice will be discussed. The correct diagnosis of neonatal hepatitis or extrahepatic biliary atresia cannot be derived from such investigations as determination of bilirubin, enzyme activities, immunologic or serologic parameters or scintigraphy of the liver. Only quantitative changes of serum LP-X before and after administration of cholestyramin and the modified rose-bengal test may help to establish a correct diagnosis in cholestatic jaundice during the first 6 weeks of life.

Cholestasis↗
PubMed1978

Endoscopy in pediatric gastroenterology.

Seventy two endoscopies of the upper gastrointestinal tract and one hundred and fifteen colonoscopies were performed in children aged nine months to seventeen years. The experience gained with different types of endoscopes suggests that endoscopy of the upper gastrointestinal tract should be only performed with pediatric instruments. These are: GIF-P2(1) (max. outer diameter 9 mm) and TX-7(2) (max. outer diameter 10mm). For colonoscopy, the ideal pediatric instrument is not yet available. Sigmoidocoloscopes like the CFMB1 (length 1115 mm) and the F-91-S2 (length 650 mm) are suitable for older children.

Adolescent↗