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Biomedical subjects

G Mitterstieler

Publications and source records attributed to G Mitterstieler.

17 recordsLinked to original sources
PubMed1985

[Recurrent hemolytic-uremic syndrome with positive immunofluorescence].

The hemolytic uremic syndrome is a disease of infancy, its major clinical manifestations include reversible thrombocytopenia, hemolytic anemia, and renal failure. Although a great number of patients with HUS have been published, relapses as well as positive immunofluorescence studies are rare findings. In our patient the disease began at age of 7 years and recovered completely. At 10 1/2 years a relapse occurred and despite therapy the patient died two months later. Renal biopsy showed severe arterial and glomerular changes with remarkable similarity to the histological findings in thrombotic thrombocytopenic purpura, which could be explained as secondary hypertensive damage, and dense granular deposition of fibrinogen, IgG, IgA, C3, and Clq along the capillary loops of the glomerulus and throughout the wall of the renal arteries. The clinical data, histological findings, and the particularities of our patient with this special course of HUS are discussed.

Child↗
PubMed1983

Urothromboplastin evidence for similarity between urothromboplastin and human brain thromboplastin.

Urothromboplastin (UP) is a lipoprotein similar to Human Brain Thromboplastin (HBrTPL), the specific activity being bound to the presence of the whole complex. UP is found in normal human urine to be macroaggregates, therefore the substance is eluted from the Sepharose 2 B column immediately after the void volume and is found in the bottom of the tube after ultracentrifugation. In polyacrylamidgelelectrophoresis UP does not enter the gel and the UP activity can be eluted from the top of the gel. Natrium-desoxycholate reduces the activity of UP greatly, however after removal of this substance the original activity could be regained. There are similarities between UP and HBrTPL: In the same way as HBrTPL UP acts in the extrinsic pathway of the coagulation system, some evidence for binding of factor VII to UP in the presence of calcium could be found. Anti-apoprotein III antiserum (anti-HBrTPL antiserum) neutralizes UP, depending on the concentration of the antiserum. The same antiserum precipitates with UP using the immunodiffusion method.

Apoproteins↗
PubMed1981

[Thrombopoiesis and blood coagulation in pediatric patients with septicemia (author's transl)].

45 infants and children with thrombocytopenia and septicemia were studied. Many parameters of blood coagulation, the platelet diameters and the megakaryocytes of the bone marrow (Feulgen stained cytophotometry and maturity of the megakaryocytes) were examined. 15 patients had a consumption coagulopathy and 30 were classified as having an isolated septic thrombocytopenia. In both groups the number of the megakaryocytes of the bone marrow smears were normal. 81% of the megakaryocytes were mature. The patient group with isolated septic thrombocytopenia had significantly greater ploidy values of the megakaryocytes than a control group. In both groups the diameters of the platelets were also significantly greater than in an age matched control group of children with a normal platelet count. These results allow the conclusion that the thrombocytopenia in pediatric patients with septicemia is not caused by a diminished production of platelets.

Blood Coagulation↗
PubMed1980

[Glomerulonephritis following snake bite].

A snake bite may quite frequently lead to haemorrhagic symptoms ranging from hypofibrinogenaemia to disseminated intravascular coagulation. In rare cases acute renal insufficiency associated with tubular necrosis may occur due to a direct toxic lesion, shock symptoms or DIC. However, it is extremely rare that glomerulonephritis results from a snake bite. A thirteen-year-old girl develops gross haematuria, albuminuria and cylindruria without any shock symptoms two days after having been bitten by a European viper. Coagulation state and complement show slight changes, and the histological examination reveals proliferative nephritis with deposition of immune complexes. The clinical picture corresponds to recurrent haematuria; renal function is normal after follow-ups over a one-year-period.

Adolescent↗
PubMed1979

Familial occurrence of syngnathia congenita syndrome.

A case of familial occurrence of syngnathia congenita in mother and child is described; this syndrome has been seen only sporadically until now. Due to the distinct appearance of the intraoral chords--they were situated on both sides between the ridges of the jaw and also between the left half of the bifid uvula and the retrolingual region--a relationship to the autosomal-dominantly transmitted Cleft Palate Lateral Synechiae Syndrome (CPLS-Syndrome) is suspected. Complete penetrance with different expressivity may explain the different clinical manifestations of the cases seen up to now and the absence of bifid uvula in the mother of our patient. The probable pathogenesis during embryological development is discussed.

Adult↗
PubMed1979

[Asplenia and DIC (author's transl)].

3 cases of severe septic shock are described; a 5 month old girl with congenital hyposplenia, a 2 3/12 year old boy splenectomized because of microspherozytosis and a 11 6/12 year old boy splenectomized because of Hodgkin's disease. In 2 cases pneumococci were found in the blood cultures. In all 3 cases the coagulation analysis showed a consumption coagulopathy. Intravenous streptokinase treatment was applied in addition to general treatment for shock and antibiotic therapy. 2 patients survived and made a complete recovery, whereas the 2 year old boy died. The histological findings showed a severe DIC. In the Department of Surgery, Innsbruck, 44 children have been splenectomized during the last 6 years, 38 of whom we were able to follow up on for an average of 3.3 years. After an average of 1.2 years following splenectomy, 4 patients (including the 3 cases mentioned above) contracted acute septicaemia; a further patient also incurring a probable sepsis with DIC. 3 of these 5 children died, representing a morbidity rate of 13% and a mortality rate of 8%. The mortality rate is thus as high as that caused by the primary disease, indicating the urgency of prophylaxis for infections of this kind. 3 prophylactic forms of treatment are suggested: protection with penicillin, active immunization with polyvalent pneumococcal antigen and spleen preservation whenever possible.

Bacterial Infections↗
PubMed1978

[Vitamin K deficiency bleeding as a leading symptom in celiac disease (author's transl)].

Haemorrhagic diathesis was a leading symptom in diagnosing celiac disease in 4 patients. In all 4 patients, a duodenal biopsy showed total villous atrophy. Although 3 of the children were typically dystrophic, the weight of the 4th child, an 8 month old boy, was within the normal range. In this patient, who suffered from neither diarrhea nor vomiting, heavy cutaneous and mucous membrane bleeding were the only symptoms of the disease. In all 4 cases the haemorrhagic diathesis could be explained by a low prothrombin complex, whereas the rest of the coagulation tests were normal. After the administration of Vitamin K1 there was an immediate rise in the prothrombin complex and bleeding was quickly stopped. Noteworthy is that due to infections, 3 of the 4 patients, received antibiotics just before the onset of the bleeding. In celiac disease, the conversion from a K-hypovitaminosis into a K-avitaminosis by the administration of antibiotics is discussed.

Anti-Bacterial Agents↗
PubMed1978

Congenital factor V deficiency. A family study.

A 14-month-old girl suffering from a heavy bleeding tendency, caused by a severe isolated congenital factor V deficiency is described. In this study 56 family members were examined. 10 of them had a factor V level ranging 26-60% of the normal--these were classified as heterozygotes. The case histories of the heterozygotes did not reveal a bleeding tendency. The inheritance of this factor V deficiency is autosomal recessive, with varying expressivity in the heterozygotes.

Factor V Deficiency↗
PubMed1978

[Anaphylactoid purpura in infancy and childhood (author's transl)].

The clinical picture and the laboratory data of 40 patients with anaphylactoid purpura as well as its etiology and pathogenesis are discussed. The clinical expression and the rate of complications does not differ from that described in the literature. 45% of the patients showed renal involvement, three patients had severe glomerulonephritis. Up to now there is a lack of typical laboratory tests to confirm the diagnosis of anaphylactoid purpura. This study suggests neutrophilia and initial creased fibrin/fibrinogen split products in serum and decreased C3 levels are poor prognostic criteria correlating with appearence and severity of a complicating glomerulonephritis.

Adolescent↗
PubMed1976

[Prophylactic replacement therapy in hemophilia. A case report (author's transl)].

In a six year old boy with severe hemophilia prophylactic substitution of factor VIII was started in 1973 in order to prevent early invalidity due to series of bleeding in the right anklejoint. A substitution of factor VIII over 8 months with 21 resp. 30 U/kg body-weight did not lead to a significant improvement. But since the factor VIII in a dosage of 18 U/kg body-weight is given three times a week no bleeding occurred during the treatment time of 14 months and also the ability to walk improved to an excellent degree.--So far no signs of hepatitis or an factor-VIII-antibody could be detected.--Some results from the prophylactic treatment of severe hemophilia and Christmas disease are cited from the literature.

Child↗
PubMed1975

[Consumption coagulopathy and isolated platelet deficiency in childhood septicaemia].

In a retrospective study 40 children were selected out of 53 cases of septicaemia with thrombocytopenia. They were divided into two coincidentally equally large groups of patients with consumption coagulopathy on the one side and patients with isolated thrombocytopenia without consumption coagulopathy on the other side. Both groups were of comparable age and sex distribution. Two-thirds of the children were under three months. For the differential diagnosis of both groups the activated partial thromboplastin time, the thrombotest, the factor V plasma concentration, the serum concentration of fibrin (fibrinogen) degradation products as well as control coagulation studies can be considered to have the greatest diagnostic value. The results of the study permit the following conclusions: 1. Platelet deficiency in sepsis does not prove the presence of consumption coagulopathy. 2. Consumption coagulopathy and isolated thrombocytopenia differ statistically significantly according to the bacteria cultured from the blood, the circulatory state and the pH of the blood. 3. The finding of thrombocytopenia in a patient with shock, acidosis and gramnegative septicaemia justify the suspicion of consumption coagulopathy.

Acidosis↗