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Biomedical subjects

H W Rotthauwe

Publications and source records attributed to H W Rotthauwe.

At least 37 records · Page 2Linked to original sources
PubMed1977

Selective vitamin B12 malabsorption (Imerslund-Gräsbeck syndrome). Studies on gastroenterological and nephrological problems.

In a girl 10 years of age with selective vitamin B12 malabsorption associated with proteinuria and residual symptoms of funicular myelosis an extensive study of the intestinal and nephrologic functions was done. Repeated Schilling tests pointed to a malabsorption pattern of vitamin B12. Gastric acid and intrinsic factor secretion as well as gastric morphology were normal. There were no antibodies against intrinsic factor and parietal cells in serum. Ileal mucosa showed on light- and electron-microscopy no pathologic changes. Pancreatic exocrine function as well as pH and calcium concentrations in the lumen of the gut were within the normal range. A general malabsorption syndrome could be excluded. A high selective glomerular proteinuria was found through different methods. Inulin clearance was slightly reduced, PAH clearance, however, markedly so. There was no further evidence for renal tubular dysfunction. Renal biopsy showed a minimal proliferative intercapillary glomerulonephritis (minimal changes). In electron-microscopic studies a fusion of a part of the foot processes of the podocytes was found. No familialhistory of the syndrome could be demonstrated in our patient.

Anemia, Macrocytic↗
PubMed1977

[Alpha1-fetoprotein: physiology, pathology and diagnosis especially in childhood (author's transl)].

Alpha1-fetoprotein (AFP) is an alpha1-glycoprotein which can be found in high concentration during fetal development in many mammals, birds, sharks and, also, man. The alpha-fetoproteins of various species have similar physico-chemical properties and often common antigenic determinants. Differences of microheterogeneity depend on a different content of sialin-acid. During human fetal development the serum AFP concentration falls with increasing gestational age. 4-5 weeks after birth AFP can be detected usually in low serum concentrations. Using more sensitive immunulogic techniques e.g. radioimmunoassay there was shown that AFP is present in sera of normal adults in concentrations of 10-20 ng/ml. AFP serum concentrations rise physiologically during pregnancy up to 500-550 ng/ml. During fetal development liver, yolk sac and gastrointestinal tract are the major sites of synthesis. In primary liver cell carcinoma, hepatoblastoma and in teratoblastoma containing yolk sac tissue AFP synthesis rises in tumor cells; the AFP serum concentration increases above 2 microgram/ml. In patients with benign liver diseases e.g. virus hepatitis, a transient rise of AFP serum concentrations was seen. Moreover, increased levels of AFP were found in hereditary diseases e.g. congenital tyrosinemia, ataxia-telangiectasia and in the amniotic fluid in congenital nephrosis of Finnish type. AFP assay in serum is clinically important for the control of course and treatment of primary liver cell carcinoma and teratoblastoma. AFP assay in amniotic fluid is a method for the prenatal detection of neural tube defects and the fetal distress syndrome, especially.

Adolescent↗
PubMed1976

[Serum-gastrin levels and gastric-acid secretion in infants (author's transl)].

Gastric acid secretion was measured in 20 infants aged 6-438 days. The values for the basal acid output and that after stimulation with 6 mug/kg pentagastrin subcutaneously were found to be related to age, body weight and body surface area. But these correlations were not comparable to those in adults. Standard values for different age groups in childhood must therefore be established. Furthermore, the results indicate parietal-cell immaturity during the first six months of life. Measurement of fasting serum-gastrin concentration by radioimmunoassay in 74 infants, aged 1-438 days, and 154 adults as controls revealed a high serum-gastrin level in infants, with an exponential decrease during the first year of life. Despite comparable pH values in gastric juice at one year of life, the gastrin concentrations were higher than those in adults (at a statistically significant level). On the other hand, normal serum-gastrin concentrations were found in ten pregnant women just before delivery. The results suggest a negative feed-back mechanism between gastric-acid secretion and fasting serum-gastrin levels, but such mechanism probably being limited by extragastric gastrin secretion.

Age Factors↗
PubMed1976

[HL-A histocompatibility antigens in children with coeliac disease (author's transl)].

HL-A antigens were determined in 41 unrelated coeliac children and in clinically healthy parents and siblings of 40 of these patients using a lymphocyte microcytotoxicity test. 58.5% of the coeliac patients had phenotype HL-A 8 compared with an HL-A 8 frequency of 16.6% in a control group of 320 unrelated individuals (P less than 0.0005). Excluding five patients not of pure German origin HL-A 8 frequency increases to 63.9%. The increase of HL-A 1 frequency in coeliac patients is attributed to linkage disequilibrium between HL-A 8 and HL-A 1. The haplotype HL-A 1.8 frequency was significantly increased in coeliac children (P less than 0.0001) with frequency elevation also in parents (P approximately 0.025) but not in siblings. Furthermore, an increase in frequency of HL-A 12 and a decreased frequency of HL-A 7 and HL-A 9 was found in coeliac patients. Five clinically healthy siblings had the same HL-A haplotypes as their affected sisters and brothers.

Adolescent↗
PubMed1976

Protein patterns of brush-border fragments in congenital lactose malabsorption and in specific hypolactasia of the adult.

Brush-border membrane proteins of the small-bowel mucosa were separated on polyacrylamide gels from intestinal biopsy specimens obtained from four children with congenital lactose malabsorption and from two adults with specific hypolactasia. In three patients with the congenital type of lactase deficiency the protein band corresponding to brush-border lactase was reduced in intensity, but was never completely absent. No difference in gel patterns was detected when this pattern in congenital deficiency was compared to that obtained from the two patients with adult-type selective hypolactasia. In one patient with congenital lactose malabsorption the protein band corresponding to lactase activity was not detectable. The findings suggest that the mechanisms leading to low lactase activity in the congenital and adult forms of lactose intolerance are similar.

Adult↗
PubMed1976

[Antireticulin antibodies and precipitating antibodies to food proteins in the sera of children with coeliac disease (author's transl)].

Sera from 41 children suffering with histologically proven coeliac disease and from 40 healthy control children were investigated for the presence of antireticulin antibodies and precipitating antibodies to a watery extract of wheat flour and to cow's milk. Antireticulin antibodies were demonstrated by means of indirect immunofluorescence using sections of fresh rat kidney as substrat. For the detection of precipitating antibodies a combination of electrophoresis and immunodiffusion was used. Serum antireticulin antibodies were found in 11/13 children (85%) with active coeliac disease, in 7/17 children (41%) with clinically and biochemically silent coeliac relapse and in 0/16 children with treated coeliac disease. Serum precipitating antibodies to wheat flour and cow's milk were found respectively in 3/13 children (23%) and 2/13 children (15%) with active coeliac disease and in 1/16 children (6%) with treated coeliac disease. Precipitating antibodies could not be detected in the sera of 17 patients with silent relapse of coeliac disease. In the sera of 40 controls neither antireticulin nor precipitating antibodies were detectable. The presence of antireticulin antibodies in serum did not correspond to the presence of serum precipitins to wheat flour and cow's milk. The significance of serum antireticulin antibodies for screening investigations and for follow-up studies is discussed.

Adolescent↗
PubMed1976

[Hypertension and bilateral stenosis of the renal artery associated with congenital hypoplasia of the intrahepatic bile ducts (author's transl)].

Report of a 10-year-old boy with congenital hypoplasia of the intrahepatic bile ducts, the socalled MacMahon-Thannhauser-Syndrome. The patient had been suffering from a varying degree of jaundice since his 2nd day of life and from pruritus since his 21st month of life. Furthermore, he had hepatomegaly, a systolic cardiac murmur, hypogenitalism, retarded growth, and finally hypertension. Transitory xanthomas existed between 1 3/4 and 2 3/4 years of age. Signs of persistent intrahepatic cholestasis was manifested by increased levels of bilirubin and bile acids in serum as well as raised activities of leucine aminopeptidase, gamma-glutamyl transpeptidase and alkaline phosphatase. Pathological values of serum glutamic dehydrogenase pointed to a persistent destruction of liver cells. Without treatment, the activities of vitamin K dependent clotting factors were decreased. Cholesterol, phosphatides and triglycerides in serum were increased and lipoprotein-X was detectable. Aortography revealed stenosis of both renal arteries. An exploratory laparotomy and 5 liver biopsies led to the diagnosis of hypoplasia of the intrahepatic bile ducts. Therapeutic trials with steroids and the anion exchange resin "cholestyramine" were ineffective. Phenobarbital relieved the pruritus. Parenteral administration of fat soluble vitamins restored the activity of vitamin K dependent clotting factors to normal. The high blood pressure fell significantly due to treatment with adelphan. The etiology of hypoplasia of the intrahepatic bile ducts is unknown. It may be a malformation or an obliteration secondary to inflammation. In our patient, narrowing of the renal arteries, increase of plasma-renin activity and hypertension were probably secondary to hyperlipidemia. It has been suggested that hyperlipemia secondary to cholestasis may be due to a disturbance of lipoprotein metabolism. A review of reports on 118 patients suffering from intrahepatic bile ducts hypoplasia is included.

Bile Ducts, Intrahepatic↗
PubMed1975

[Alpha-antitrypsin deficiency in infancy].

Clinical, histological (including electron-microscopic), immunohistochemical and genetic studies were performed on two infants with alpha1-antitrypsin deficiency. The clinical picture was one of neonatal biliary stasis. Liver biopsies revealed multiple cytoplasmic acidophilic bodies within many cells of the liver parenchyma which were strongly periodic acid-Schiff-positive, diastase-resistant and stained selectively with fluorescein-labelled rabbit antihuman alpha1-antitrypsin. Ultrastructurally, the bodies were situated within enlarged cisterns of the endoplasmatic reticulum. Both infants were of the protease inhibitor (Pi) phenotype ZZ, having inherited on PiZ gene from each parent. Results of Pi typing of both families were consistent with an autosomal co-dominant inheritance. Both infants are clinically well except for slight hepatomegaly at one year of age. But transaminase and gamma-glutamyl transpeptidase activities have remained elevated.

Adult↗
PubMed1975

[Serum concentrations of vitamin A, carotene, retinol-binding protein and prealbumin in patients with cystic fibrosis (author's transl)].

Vitamin A, carotene, retinol-binding protein (RBP), and prealbumin (PA) have been measured in 42 children and adolescents with cystic fibrosis (CF) and in 92 normal controls. All patients with CF were on vitamin A palmitate in twice the dose for normals. For statistical analysis U- test of Wilcoxon, Mann and Whitney, parametric correlation coefficient and Spearman's rank correlation coefficient were used. Compared with those in normal controls mean serum concentrations of vitamin A, carotene and RBP were depressed in patients with CF (P smaller than 0.001), whereas PA levels did not differ significantly from those of normal individuals. In normal controls there was only in serum concentration of PA an elevation with age (r=0.455, P smaller than 0.001). In patients with CF, serum concentration of vitamin A decreased in correlation with age (r=--0.423, P smaller than 0.01). PA and RBP as well as RBP and vitamin A were positively related in both groups (P smaller than 0.001). In normal individuals there was a highly significant correlation between serum concentrations of vitamin A and carotene (rs=0.606, P smaller than 0.001), whereas in patients with CF this relationship was less significant (rs=0.311, P smaller than 0.02).

Adolescent↗