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Biomedical subjects

D Feist

Publications and source records attributed to D Feist.

At least 19 recordsLinked to original sources
PubMed1991

Alteration of the lung parenchyma associated with autoimmune hepatitis.

The clinical history, radiological and histomorphological alterations of the lung parenchyma associated with chronic active autoimmune hepatitis are described. A 6-month-old female infant developed chronic active autoimmune hepatitis associated with autoimmune haemolytic anaemia. She was treated with immunosuppressive drugs, including steroids, for more than 6 years and developed symptoms and radiological signs of interstitial pneumonitis 4 years after onset of the autoimmune hepatitis. Associated bronchiectasis was detected 1 year later. No abnormalities of lung defence mechanisms could be demonstrated. Resection of the sixth left segment and of the basal parts of the left lower lobe revealed honeycombing with changes in the lung parenchyma which included chronic interstitial pneumonitis with multinucleate giant cells, seen predominantly in the distal airways, marked diffuse interstitial mononuclear infiltrates and mild diffuse interstitial fibrosis as well as bronchiectasis and organizing pneumonia. Granulomatous lesions, angiitis and necrotic areas were absent. Immunohistochemistry for immunoglobulins was negative for IgA, IgG and IgM and positive for IgD in the multinucleate giant cells. A strong positive reaction to HLA-DR-specific monoclonal antibody was noted, whereas no specific sugar receptors (endogenous lectins) could be detected by use of biotinylated glyconeoproteins.

Autoimmune Diseases↗
PubMed1991

[Fibrosing alveolitis in a 7-year-old girl with autoimmune hemolytic anemia and autoimmune hepatitis].

A Coombs-positive haemolytic anaemia with proof of heat autoagglutinins and autoantibodies against cell nuclei was diagnosed in a girl, now 7 1/2 years of age, in the sixth month of her life as a sequel to jaundice. Shortly afterwards liver biopsy yielded a confirmed diagnosis of giant cell hepatitis. The signs of the disease receded under treatment with decortin and imurek. An attempt to discontinue the medication at 5 1/2 years of age resulted in a histologically confirmed recurrence of autoimmune hepatitis. When the patient was 5 years old, the x-ray revealed a mainly interstitial pneumonia; at 6 years of age, bronchiectases in the region of the left lower lobe were confirmed by bronchography. Resection of the lower lobe was performed after occurrence of atelectasis. Histopathological findings confirmed fibrotic alveolitis associated with giant cells. Based on the overall clinical course and the histological and immunological findings, autoimmune etiology of fibrotic alveolitis must be discussed. Progression of the disease could be avoided during the past 6 months by intensifying the immunosuppressive prednisolone therapy.

Anemia, Hemolytic, Autoimmune↗
PubMed1990

[Clinical aspects, results of diagnostic pulmonary function tests and biochemical parameters in children with homozygote alpha-1-antitrypsin deficiency].

The clinical course and pulmonary function tests of individuals with severe Alpha-1-Antitrypsin (Alpha-1-AT) deficiency reveal a marked interindividual variability. 4 patients with PI type ZZ and 2 patients with PI type SZ had been identified by neonatal cholestasis. None had pulmonary symptoms at a mean age of 13 (range 9-16) years. Lung function tests disclosed signs of incipient pulmonary emphysema in one girl. Bronchial hyperreactivity had led to the diagnosis of Alpha-1 AT-deficiency (PI type ZZ) in another girl at the age of 7 years. The biochemical analysis of the serum of three children (two PI ZZ and one PI SZ) revealed additional protease inhibitors that may be effective as compensatory mechanisms. The lack of those protective factors as well as the presence of environmental hazards may contribute to the increased risk of emphysema in individual patients.

Adolescent↗
PubMed1987

Fanconi-Bickel syndrome.

Clinical, biochemical, functional and morphological data are presented in nine infants, children and adults, with Fanconi-Bickel syndrome. Long-term follow-up studies show severe growth retardation, partly compensated for by late onset of puberty. Glomerular filtration rate is normal or slightly decreased. Renal tubular dysfunction is characterized by a specific pattern of impaired proximal tubular transport mechanisms, with marked impairment of glucose transport. The utilization of glucose and galactose is defective, whereas fructose metabolism seems to be normal. Glycogenosis of the liver may be an epiphenomenon. Glycogen accumulation in the kidney is limited to the proximal tubule, with maximal levels in the straight part. The Fanconi-Bickel syndrome is a defined clinical entity which is distinguished from other inherited metabolic diseases by complex defects of renal tubular transport and other forms of glycogenosis.

Adolescent↗
PubMed1986

[Liver cirrhosis in childhood--etiology, diagnosis and conservative therapy].

Liver cirrhosis is relatively rare in children as compared to adults; frequently it is diagnosed too late. Biliary cirrhosis of early childhood is often the result of neonatal cholestatic syndromes. Beyond infancy, cirrhosis as a consequence of chronic active autoimmune hepatitis or of Wilson's disease may be prevented, if causal therapy is begun in time. Hence paediatricians should exclude both diseases in all children with elevated transaminases and clinical features of a liver disorder.

Autoimmune Diseases↗
PubMed1979

[Pathogenesis of the neonatal cholestasis syndrome (author's transl)].

Cholestasis means reduced flow of bile between hepatocytes and duodenum. Functional as well as mechanical factors may be important in the pathogenesis of cholestatic syndromes. Extra- and intrahepatic cholestasis can be distinguished. Intrahepatic bile ducts use to be dilated in extrahepatic cholestasis after early infancy; they are however not dilated in obstructive jaundice of the newborn. Neonatal cholestatic syndromes caused by liver cell diseases may in addition cause an obstruction of extrahepatic bile ducts. At the present time most workers in the field do believe that extrahepatic cholestasis in the newborn is rather due to inflammation than to congenital malformation.

Bile Ducts↗
PubMed1979

[Wilson's liver disease in children and adolescents (author's transl)].

Hepatic symptoms are usually the first in Wilson's disease of children and adolescents, while neurologic symptoms and the corneal ring are still missing. Liver lesions due to copper accumulation may develop throughout years without clinical symptoms or biochemical abnormalities. Hemolytic jaundice or gastrointestinal bleeding are the presenting symptoms in some cases. In spite of being a rare syndrom Wilson's disease ought to be considered after hepatitis B or autoimmune liver disease have been excluded as causes of juvenile cirrhosis of the liver. If life-long treatment with D-penicillamin is started in an early stage of Wilson's disease, prognosis is rather good.

Adolescent↗
PubMed1978

Quantitative determination of single serum proteins during acute hepatitis in childhood.

Fifteen serum proteins were estimated by linear immunodiffusion in blood samples from children with acute hepatitis. Blood was drawn at the beginning of the disease and three weeks later. The results were compared with results obtained from a group of age-matched normal children. At the beginning of the disease prealbumin and beta-2-glycoprotein I were depressed, whereas alpha-1-acid-glycoprotein, alpha-1-antitrypsin, cerloplasmin and alpha-2-HS-glycoprotein were found to be elevated. Alpha-2-macroglobulin, transferrin and beta-lipoprotein showed a significant elevation after three weeks. Beta-1-A/C, IgM and IgG remain elevated during time of observation. Albumin, haptoglobin and IgA were similar in patients and controls and did not change during the period of observation.

Acute Disease↗
PubMed1978

[Early diagnosis of Wilson's disease in childhood (author's transl)].

Most of the time Wilson's disease becomes clinically evident in childhood by atypical abdominal symptoms. Therefore early diagnosis is very difficult. In four out of eight patients with Wilson's disease, diagnosed relatively early in the Children's Hospital of Heidelberg University, we could demonstrate that each case of liver disease, which cannot be classified, may be suspicious of Wilson's disease. Even normal levels of ceruloplasmin in serum and copper in urine are not inconsistent with Wilson's disease. There is no screening method in infancy. Fatty liver in school children is very suspicious of Wilson's disease.

Adolescent↗
PubMed1976

[Hypermethioninemia in the differential diagnosis of infantile obstructive jaundice (author's transl)].

In 7 infants suffering from obstructive jaundice we found transient high levels of methionine in serum. All cases had only intrahepatic cholestasis, especially with intrahepatic biliary hypoplasia, whereas other patients with extrahepatic biliary atresia and/or combination of extra- and intrahepatic obstructive jaundice always showed normal levels of methionine. Therefore hypermethioninemia seems to be helpful in differentiating the various causes of infantile obstructive jaundice.

Bile Ducts↗
PubMed1975

[Studies on juvenile chronic hepatitis].

Follow-up study of 40 children suffering from chronic hepatitis. The diagnosis was made by liver needle biopsy with the Menghini method, when clinical signs or laboratory data of liver disease had lasted for more than 6 months. 24 patients showed the histological pattern of the aggressiv type of chronic hepatitis according to the definition of the European Association for the Study of the Liver (1968). In this group only 5 children had autoantibodies in the serum (so-called lupoid hepatitis). The HBAg positive courses played the most important part in the chronic persistent group as well as in the aggressive one. According to literature only the patients with the aggressive type have been treated with prednison, because chronic persistent hepatitis has a good prognosis without any treatment. In nearly all cases high transaminases and gammaglobulin levels decreased during the treatment with prednison, whereas the histological signs of inflammation seldom changed. Cirrhosis of the liver has developed in 2 HBAg positive patients of the aggressive group, who had not consequently received their daily dose of prednison.

Alanine Transaminase↗
PubMed1975

Portal hypertension in children following neonatal umbilical disorders.

10 children presented pre-hepatic portal vein obstruction during their first 6 years of life. 8 of them had massive esophageal varices, 1 died from acute esophageal hemorrhage. The perinatal history of these childre was studied: All of them had an abnormal birth history and all had been hospitalized during their neonatal period. In 5 of them an umbilical infection had been diagnosed, one had an injection of THAM and one other an exchange transfusion through an umbilical vein catheter. Pre-hepatic portal vein occlusion in children is presumed to be mainly an acquired disease resulting from neonatal umbilical disorders.

Child↗
PubMed1975

[Diagnostic and Therapeutical problems of extrahepatic portal vein stenosis].

In seven children the clinical course of extrahepatic portal vein stenosis was studied angiographically. In all children with splenomegaly it was deemed necessary to perform an X-ray barium examination of the oesophagus. Transsplenic portal venography was performed when oesophageal varices were detected to set up the best scheme for emergency treatment. In our patients with hypersplenism there were no specific bone marrow alterations. In contrast to the literature most of our cases had fibrosis of the liver.

Adolescent↗