Biomedical subjects
G Strohmeyer
Publications and source records attributed to G Strohmeyer.
Survival and causes of death in cirrhotic and in noncirrhotic patients with primary hemochromatosis.
We analyzed survival and causes of death among 163 patients with primary hemochromatosis diagnosed between 1959 and 1983. The mean follow-up period was 10.5 +/- 5.6 years (+/- S.D.). Cumulative survival was 92 per cent at 5 years, 76 per cent at 10 years, 59 per cent at 15 years, and 49 per cent at 20 years. Life expectancy was reduced in patients with cirrhosis of the liver as compared with those without cirrhosis (P less than or equal to 0.05), in patients with diabetes mellitus as compared with those without diabetes (P less than or equal to 0.002), and in patients who could not be depleted of iron during the first 18 months of venesection therapy as compared with those who could be depleted (P less than or equal to 0.001). Prognosis was not influenced by sex (P less than or equal to 0.5). Patients without cirrhosis had a life expectancy that was not different from that expected in an age- and sex-matched normal population. Analysis of the causes of death in 53 patients, as compared with the normal population, showed that liver cancer was 219 times more frequent among the patients (16 patients), cardiomyopathy was 306 times more frequent (3 patients), liver cirrhosis was 13 times more frequent (10 patients), and diabetes mellitus was 7 times more frequent (3 patients). Death rates for other causes, including extrahepatic carcinomas (seven patients), were not different from the rates expected. We conclude that patients with hemochromatosis diagnosed in the precirrhotic stage and treated by venesection have a normal life expectancy, whereas cirrhotic patients have a shortened life expectancy and a high risk of liver cancer even when complete iron depletion has been achieved.
[Genetics of Crohn disease: study of the HLA association in 169 patients].
Histocompatibility (HLA) antigen phenotypes have been studied in 169 patients with Crohn's disease. The following results could bei shown: HLA-Aw33, -B45 and -Cw3 showed a positive association and HLA-A26, -DR3 and -DRw8 a negative association with Crohn's disease compared to healthy controls. However, when the p-values were corrected by multiplying them by the number of determined antigens per gen-locus, the differences were not significant. Patients with a late onset of the disease (greater than 25 years) showed a statistical significant negative association with HLA-DR3. Numerous studies revealed no significant association between Crohn's disease and HLA-antigens except Smolen et al. (HLA-B12). The significant association of Crohn's disease and HLA-B12 reported by Smolen et al. could be caused by an increased frequency of HLA-B45 as we found in our patients.
[Indications for drug therapy of chronic active hepatitis and posthepatitis liver cirrhosis].
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Differing abnormalities in estrogen and androgen and insulin metabolism in idiopathic hemochromatosis versus alcoholic liver disease.
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Isolation and partial characterization of a fatty acid binding protein in rat liver plasma membranes.
When [14C]oleate-bovine serum albumin complexes were incubated in vitro with rat liver plasma membranes (LPM), specific, saturable binding of oleate to the membranes was observed. Maximal heat-sensitive (i.e., specific) binding was 3.2 nmol/mg of membrane protein. Oleate-agarose affinity chromatography of Triton X-100-solubilized LPM was used to isolate a single 40-kDa protein with high affinity for oleate. On gel filtration, the protein comigrated with various fatty acids but not with [14C]bilirubin, [35S]sulfobromophthalein, [14C]taurocholate, [14C]phosphatidylcholine, or [14C]cholesteryloleate. A rabbit antibody to this membrane fatty acid-binding protein gave a single precipitin line with the antigen but no reactivity with concentrated cytosolic proteins, LPM bilirubin/sulfobromophthalein-binding protein, or rat albumin or other rat plasma proteins. The antibody selectively inhibited heat-sensitive binding of [14C]oleate to LPM. Immunofluorescence studies localized the antigen in liver-cell plasma membranes as well as in other major sites of fatty acid transport. These data are compatible with the hypothesis that this protein may act as a receptor in a hepatocellular uptake mechanism for fatty acids.
Identification, isolation, and partial characterization of a fatty acid binding protein from rat jejunal microvillous membranes.
The mechanisms by which FFA are absorbed by the gut are unclear. To examine these processes, binding of [14C]oleate to isolated rat jejunal microvillous membranes (MVM) was studied in vitro. When [14C]oleate alone or compounded with bovine serum albumin at various molar ratios was incubated with MVM aliquots, binding was time- and temperature-dependent, inhibitable by addition of excess cold oleate, and decreased by heat denaturation or trypsin digestion of the membranes. When [14C]oleate binding to heat denatured MVM, which increased continuously as a function of the free oleate concentration and was taken as a measure of nonspecific binding, was subtracted from total binding to native MVM, a curve suggestive of saturable specific binding was observed. In contrast to fatty acids, there was no specific binding of [14C]taurocholate or [35S]sulfobromophthalein to jejunal MVM. After MVM solubilization with 1% Triton X-100, affinity chromatography over oleate-agarose and elution with 7 M urea yielded a single 40,000-mol-wt protein. This Sudan Black/periodic acid-Schiff-stain-negative protein co-chromatographed on Sephadex G-100 with [14C]oleate, [14C]palmitate, [14C]arachidonate, and [14C]linoleate, but not with the [14C]oleate ester of cholesterol, [14C]phosphatidylcholine, [14C]taurocholate, or [35S]sulfobromophthalein. A rabbit antibody to the previously reported hepatic membrane fatty acid binding protein (FABP) gave a single line of immunologic identity between the FABPs of rat jejunum and rat liver membrane. It inhibited the binding of [14C]oleate to native MVM but not heat denatured MVM, and, in immunohistochemical studies, demonstrated the presence of the FABP in the apical and lateral portions of the brush border cells of the jejunum, but not on the luminal surface of esophagus or colon. These data are compatible with the hypothesis that a specific FABP plays a role in fatty acid absorption from the gut.
Conversion of androgens to estrogens in idiopathic hemochromatosis: comparison with alcoholic liver cirrhosis.
Hypogonadism is common in patients with some liver diseases, such as idiopathic hemochromatosis (IHC) and alcoholic cirrhosis (AC). However, gynecomastia, a typical feature in AC, does not occur in IHC. To determine the hormonal basis for this difference, the following parameters were determined in patients with IHC and AC as well as in normal men: plasma concentrations of androgens and estrogens, metabolic clearance and production rates of androstenedione and testosterone, and the contribution of peripheral conversion of androstenedione and testosterone to the circulating estrogens. Severe impotence in both patients with IHC and those with AC was associated with more than 50% reduction in plasma testosterone. The reduction was due to 63% and 70% decreases in testosterone production in IHC and AC, respectively. The MCRs were less affected in IHC and AC (19% and 37% reductions, respectively). In IHC, the fall in testosterone concentrations was accompanied by decreased production and plasma concentrations of androstenedione, a precursor for estrogen synthesis. In contrast, production and plasma concentrations of androstenedione were significantly increased in AC. Patients with IHC had estradiol und estrone levels similar to those in normal men (mean +/- SD, 16.2 +/- 4.6 vs. 20.3 +/- 3.7 pg/ml; P = NS), whereas in AC, estradiol and estrone were significantly elevated (38.0 +/- 5.3 and 68.5 +/- 17.2 pg/ml, respectively). In IHC, sex hormone-binding globulin levels were in the same range as in the normal men, whereas sex hormone-binding globulin was increased in AC. In IHC, the instantaneous contribution of plasma androstenedione to estrone and estradiol was normal, whereas that of plasma testosterone to plasma estrogens was decreased by about 50%. In contrast, in AC, the instantaneous contribution of plasma androstenedione to estrogens was greatly enhanced, and that of testosterone was in the normal range. Since the MCRs of androgens and the conversion ratios of androgens to estrogens indicate normal peripheral metabolism of sex hormones in IHC, decreased androgen formation implies decreased testicular synthesis. This was confirmed by a significantly decreased LH level in IHC (5.5 +/- 1.9 vs. 10.5 +/- 3.1 mU/ml in normal men), indicating pituitary failure. In AC, however, increased LH (20.0 +/- 2.7 mU/ml) may be indicative of primary testicular failure. These results confirm clinical features of hypogonadism and normal estrogenic activity in patients with IHC.(ABSTRACT TRUNCATED AT 250 WORDS)
[HLA haplotype study in familial Crohn disease].
Among others, genetical factors have been discussed in the aetio-pathogenesis of inflammatory bowel diseases. This is supported by many studies reporting familial accumulation of Crohn's disease and ulcerative colitis. The use of HLA typing to demonstrate a genetical disposition has so far proved disappointing. Accordingly, if there is any association with definite HLA antigens, it is likely to be weak. We therefore investigated the HLA haplotypes in 13 families with multiple occurrences of Crohn's disease. Comparing observed and expected occurrences of HLA-haplotypes, we found a pronounced tendency towards common haplotypes in the affected siblings, uncles and nieces, and cousins. Although the data suggest a possible genetical disposition, additional factors such as microorganisms or possibly nutritional habits must be considered as causes for Crohn's disease.
[Tube nutrition by continuous feeding or bolus administration?].
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[Gastric or enteral administration?].
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[Enteral nutrition with formula diets--summary of the discussion].
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[Therapy of hemosiderosis with deferoxamine].
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[Diagnosis, therapy and prognosis of alcohol-induced liver diseases].
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Hyperinsulinaemia in non-cirrhotic haemochromatosis: impaired hepatic insulin degradation?
This study investigated early alterations of glucose metabolism in idiopathic haemochromatosis. Circulating concentrations of glucose, insulin, C-peptide, glucagon, and gastric inhibitory polypeptide (GIP) were measured after a 100-g oral glucose load in 10 men with idiopathic haemochromatosis in the non-cirrhotic stage of the disease. All had normal glucose tolerance and normal body weight. Ten matched healthy subjects were studied as controls. Insulin concentrations increased to significantly higher levels in patients with idiopathic haemochromatosis than in the control subjects from 30 to 180 min after the glucose load (p less than or equal to 0.01), while fasting insulin concentrations were not significantly different (p greater than 0.05). Concentrations of glucose, glucagon, C-peptide, and GIP were not significantly different at any time (p greater than 0.05). Thus, patients with idiopathic haemochromatosis show hyperinsulinaemia and hence insulin resistance without impaired glucose tolerance in the non-cirrhotic stage. Since pancreatic insulin secretion (C-peptide), glucagon secretion, and the entero-insulinar axis (GIP) are not impaired in these non-cirrhotic patients with idiopathic haemochromatosis, iron accumulation in the hepatocytes may be responsible for the impaired insulin effect and may cause impaired hepatic insulin extraction.
[Acute cholecystitis--early cholecystectomy?].
In acute cholecystitis different therapeutic approaches according to the individual situation are required: urgent (emergency) cholecystectomy is necessary in patients with acute life-threatening complications such as empyema and suspected or confirmed perforation. In uncomplicated cases early operation is recommended within 24 to 72 h. The reliability of diagnosis, technical complications and lethality are not different in patients undergoing early (less than 72 h) as opposed to delayed (less than 2 months) cholecystectomy. Early cholecystectomy prevents the complications of delayed operation and decreases costs for hospital care and is therefore the treatment of choice for most patients with acute cholecystitis.
HLA-A3 and -B7 in porphyria cutanea tarda.
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The defect of intestinal calcium transport in hyperthyroidism and its response to therapy.
Skeletal demineralization occurs in thyrotoxicosis. Fecal calcium excretion may be enhanced, and calcium balance tends to be negative. We investigated intestinal calcium transport in 12 hyperthyroid patients. Absorption was measured by segmental perfusion in the proximal jejunum at calcium concentrations commensurate with those in the fasting and postprandial states. At low luminal concentrations, under conditions where calcium is transported predominantly by active processes, the calcium absorption rate was reduced though not abolished in hyperthyroid patients (16 +/- 4 (SE) mumol/h . 30 cm segment) as compared to normal subjects (71 +/- 8 mumol/h; P less than 0.001). When perfusate calcium was raised to 5 mmol/liter there was little increment of the net absorption rate in the hyperthyroid group (45 +/- 11 mumol/h), whereas that in the normal subjects rose to 183 +/- 17 mumol/h. Likewise, the unidirectional calcium flux out of the lumen was low in hyperthyroidism (43 +/- 7 mumol/h), suggesting that low net absorption rates were not due to transmucosal calcium loss. D-Xylose permeation was similar in all study groups. Treatment of the thyroid disease led to a marked increase in calcium absorption rates from 33 +/- 10 to 124 +/- 20 mumol/h (at 2 mmol/liter P less than 0.001; n = 5) into the range of values in normal subjects (124 +/- 9 mumol/h). Circulating levels of 1,25-dihydroxyvitamin D were low in hyperthyroid patients (38 +/- 11 pg/ml) and increased during treatment to 63 +/- 11 pg/ml (P less than 0.025; n = 9), whereas 25-hydroxyvitamin D was normal and remained unchanged. We conclude that intestinal calcium transport, particularly its active component, is reversibly decreased in hyperthyroidism. The association with low plasma levels of the active vitamin D metabolite suggests that the decrease in calcium absorption may be related to calcium-regulating mechanisms as a consequence of the net calcium efflux from bone in this disease.