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Biomedical subjects

B Kremer

Publications and source records attributed to B Kremer.

At least 19 recordsLinked to original sources
PubMed1992

New insights into the clinical features, pathogenesis and molecular genetics of Huntington disease.

Traditionally, a clinical diagnosis of Huntington disease (HD) presents no problems in patients with a positive family history, consistent with autosomal dominant inheritance, chorea or other extrapyramidal motor signs, and progressive mental decline. However, due to the slowly progressive nature of the disease and the slow evolution of signs and symptoms, it is often difficult to determine when at risk individuals are showing early signs. Moreover, the clinical recognition of both early and late-onset cases, and of choreic patients in whom a family history is lacking, presents special diagnostic challenges. In recent years, much progress has been made in the recognition of early clinical signs of the disease. Factors which have contributed to this understanding include the longitudinal study of large cohorts of at-risk individuals, particularly in Venezuela, the data from predictive testing programs, and the application of positron emission tomography (PET)-scanning to individuals without overt chorea. We are now able to identify persons at risk as being affected before they display overt and obvious involuntary movements.

Adolescent↗
PubMed1992

Primary sarcoma of the liver in the adult. Report of five surgically treated patients.

During the 5-year period between 1988 and 1989, five patients with primary sarcomas of the liver underwent surgery. Since the patients presented in an early stage of the tumor, all the sarcomas were resectable, in three cases with wide margins (R-0 resection). Five histological types were detected: malignant hemangiopericytoma, malignant fibrous histiocytoma, hemangiosarcoma, rhabdomyosarcoma, and embryonal sarcoma. Two patients with high-grade sarcomas received adjuvant chemotherapy. The follow-up was favorable in three patients with R-0 resections (two had adjuvant chemotherapy). They were still alive, with no evidence of disease 30, 46, and 63 months after the diagnosis. The two other patients had to be reoperated on for local recurrences. Both died of their tumor disease, 30 and 35 months after the initial diagnosis. Extensive chemotherapy in one of these cases failed to arrest tumor progression. Hence, liver resection with wide margins is a very important measure in such cases.

Adult↗
PubMed1992

[Injuries of the portal vein and vena cava in severe blunt abdominal trauma].

Between 1/87 and 7/90 54 severely traumatized patients underwent emergency laparotomy because of blunt abdominal trauma. In 55.5% of the cases one intraabdominal organ, in 20.4% two organs, in 16.6% three and in 7.4% four or more organs were injured. Additionally in 14.8% of these cases (n = 8) a major vascular injury (portal vein n = 5, vena cava n = 2, mesenteric root n = 1) was found intraoperatively. Injuries of the portal vein were always associated with complete rupture of the pancreas, requiring left pancreatic resection in 4 cases and a duodenum preserving resection of the pancreas head in one case. In 2 of these patients the portal vein had to be reconstructed with a prosthesis. Lethality was 9.3% for the whole group (n = 54) and 25% for the patients (n = 8) with additional vascular injuries.

Abdominal Injuries↗
PubMed1991

[Chemoembolization in primary hepatocellular carcinoma].

The only generally recognised form of treatment for primary liver cell carcinomas is partial liver resection; occasionally this may be curative. This form of treatment is only applicable in 5% to 30% of patients. All drug-based forms of treatment have proved of little value. The new interventional radiological method of chemo-embolisation has shown results in improving survival rates which are comparable with resection. By using a new coaxial catheter system, embolisation can be performed as a selective intrahepatic procedure; it has few side effects and the quality of life subsequently does not deteriorate.

Carcinoma, Hepatocellular↗
PubMed1991

[Experiences with monoclonal antibody orthoclone OKT3 following liver transplantation].

In 7 patients 10 cycles (5 to 21 days, dosage: 30 to 430 mg, median 138 mg) of OKT3 therapy were performed after liver transplantation. In all patients reactivation of an EBV-infection with hepatitis was observed. Two patients treated with high dosages (430 mg respectively 195 mg) developed lymphoproliferative lesions. In one patient with persistent EBV-infection (dosage: 360 mg) a B-cell-lymphoma was diagnosed. This patient died 26 months after transplantation.

Female↗
PubMed1990

Immunochemical characterization and quantitative distribution of pancreatic stone protein in sera and pancreatic secretions in pancreatic disorders.

A fluorometric immunoassay has been established to quantitate pancreatic stone protein providing a sensitivity for concentrations from 0.015 to 0.5 micrograms/mL. When concentrations of pancreatic stone protein were determined from pancreatic secretions obtained either from patients suffering from chronic pancreatitis (n = 31) [including the calcifying forms (n = 10)], pancreatic cancer (n = 22), or nonpancreatic diseases (n = 17), no significant differences were found. In contrast, increased concentrations were found in serum samples from patients with chronic (39/66) and acute pancreatitis (16/20) compared with control patients. The differences between these diagnostic groups and controls were highly significant (P less than 0.0001) and independent of pancreatic enzyme activity. Immunochemical analyses of serum pancreatic stone protein showed an isoelectric point (pH 9) similar to that reported for the pancreatic thread protein. With respect to recent communications, these data do not support the etiopathogenic role postulated for pancreatic stone protein in chronic pancreatitis and chronic calcifying pancreatitis by other investigators.

Adult↗
PubMed1990

[Initial results of combined surgical-medical therapy for metastatic cancer of the stomach].

Fifty patients with advanced gastric cancer underwent chemotherapy in accordance with the FAMTX protocol. Complete tumor remission was achieved in 6 patients and partial remission in 11 patients. In this group 9 patients (tumor stage: T3-4, N2-3, M chi-1) underwent a second-look operation after chemotherapy either to confirm the complete remission or to resect the remaining tumor mass. It is concluded that even in advanced stages of gastric carcinomas a more aggressive approach involving a combination of surgical and chemotherapeutic treatment improves the survival time of these patients.

Adult↗
PubMed1990

[Use of blood components in 30 liver transplantations at the Hamburg Eppendorf University Clinic 1984 to 1989].

Among all major organ transplantations, orthotopic liver transplantation (LTX) requires the most extensive blood component support. From 1984 till 1989 we performed 30 LTX procedures on 27 patients. The number of blood components used totalled 1,000 units of packed red cells (RBC), 966 units of fresh-frozen plasma, 246 units of whole blood, 170 units of random-donor platelet-rich plasma and 71 units of platelet concentrates from cell separator. Three retransplantations were performed within 2 days after the first LTX. The lowest use of blood components was registered in patients with hepatoma, the highest in alcoholtoxic cirrhosis. Ten of the 27 patients are still alive.

Blood Transfusion↗
PubMed1979

Toxin-specific ultrastructural alterations of the mouse liver after burn injuries and the possibility of a specific antitoxic therapy.

Electron-microscopic studies in mice revealed similar and comparable mitochondrial alterations of the liver cells 5-7 days after either a sublethal controlled burn injury or an i.p. injection of an equivalent dose of a burn toxin. Electron microscopy 5 days after i.p. application of different amounts of the burn toxin in rats showed that the extent of the liver alterations correlates directly to the applied dose (occurrence of: cristolysis--intramitochondrial vacuolization--total vacuolar changes of mitochondria(. Controls with the non-toxic/"native" compound isolated from normal skin or excision of a skin piece identical in size to the sublethal burn showed no ultrastructural changes in the liver of mice or rats. In a 2nd series of experiments the therapeutic effect of an antitoxic IgG raised in sheep was tested. The first 3 days after a standard burn or an i.p. injection of 15 mg burn toxin mice obtained 10 mg of the antitoxic IgG (2x/day). Controls injected with the "native" compound or excised as described were treated in the same way. The results showed a specific complete immunological protection from mitochondrial alterations by either the toxin or the burn injury. These results suggest the possibility of an antitoxic IgG-therapy in severe burns.

Animals↗
PubMed1979

Effect of cutaneous human or mouse burn toxin on the metabolic function of isolated liver cells.

Studies on isolated perfused rat livers 5 days after either a sublethal burn or an i.p. injection of human/mouse burn toxin showed a significant inhibition of the glucose/urea synthesis and the ATP production concomitantly with ultrastructural mitochondrial damages. A direct specific effect of these burn toxins on enzymatically isolated liver parenchyma cells was found either after direct incubation of the isolated cells with the compound or 5 days after injection of the toxin to the animals followed by the isolation of the cells. Control experiments were performed with the "native" non-toxic precursor from normal skin. Liver cells of rats pretreated with the toxin showed an 100% increase of the amino-acid release while this increase was 70% after direct toxin incubation. Glycogen synthesis from lactate, alanin and fructose was significantly decreased in both toxin groups while the glucose synthesis was not altered. The degree of the inhibition of the glycogen synthesis was directly correlated to the number of ATP-dependent metabolic steps. A disturbance of the oxygen transfer system by structural damages of the mitochondria seems to be the basic mechanism for these specific metabolic alterations due to ultrastructural mitochondrial damages.

Adenosine Triphosphate↗
PubMed1979

[Xanthoma disseminatum (Montgomery's syndrome) (author's transl)].

Montgomery's syndrome is distinguished by skin and mucous membrane xanthomatosis, predominant on folds, associated with diabetes insipidus and normal lipidic balance. From one typical case history occurring in a young gravida, the authors stresse upon: --Review of literature's cases. --Peculiar therapeutic problems during pregnancy. --Nosological discussion: the position of Montgomery's syndrome compared with histocytosis X and other nonnormolipidic xanthomatosis. This problem is all the more important as in this observation xanthomatosis was first with a normal lipidic measurement, lipidic disorders appearing only secondarily.

Adult↗
PubMed1978

[Pathophysiology of burns].

Ultrastructural and metabolic alterations of the liver were studied in mice and rats after standardized "in vivo" burn injuries or i.p. injections of a specific cutaneous burn toxin. The main findings of the ultrastructural investigations were delayed and specific intramitochondrial vacuolizations appearing not before the 5th-7th day after burning or toxin-injections. -By applying the Schimassek model of the isolated and perfused liver for investigating liver metabolism we found a drastically reduced ATP/ADP-quotient on the 5th day after burns or toxin-injection as compared to the normal controls. The total content of adenonucleotides, however, remained unchanged. The urea and glucose release into the perfusate was also significantly decreased in both groups of animals and the glucose synthesis was not adequately stimulated by an additional lactate load. The results show a direct effect on the burn toxin on the hepatic metabolism as well as the hepatic cell structure suggesting a marked alteration of the oxydative phosphorylation.

Adenosine Diphosphate↗
PubMed1977

[The liver sinusoids in rats following fractionated local telecobalt-irradiation. A transmission and scanning electron microscopic study (author's transl)].

Changes in liver sinusoids following fractionated irradiation were studied by use of TEM and SEM. Rats received telecobalt-irradiation of their liver regions in daily doses of 500 r for 9 days, a total dose of 4500 r. One day after the end of the irradiation series the fine structure of hepatocytes was almost intact. In SEM the liver sinusoids contained numerous corpuscles, which in TEM were recognized as cellular blebs separated from endothelial cells. 8 days after irradiation small foci of necrotic hepatocytes could be visualized. Instead of complexes of small fenestrations (sieve plates) the endothelial cells contained striking round or oval holes in their tenuous processes. After 120 days small groups of hepatocytes with alterated fine structure of their organelles and some necrotic parenchyma cells were still present. Away from these focal necroses the endothelial cells now resembled sinusoidal endothelium in control animals. Fat-storing cells appeared to have multiplied and were enlarged. There was distinct fibrosis only in perisinusoidal spaces. The formation and discharge of electron lucent blebs is discussed as a mechanism compensating for an enhanced uptake of fluid into irradiation injured endothelial cells. In order to form holes in the tenuos processes of endothelial cells a disordered synthetic pattern and the shearing forces of the circulating blood are thought to be necessary. Repair of these intracellular holes may be possible. In rats irradiation induced lesions of the small intrahepatic vessels can be excluded as a cause of the late changes in liver parenchyma cells.

Animals↗