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Biomedical subjects

B Kremer

Publications and source records attributed to B Kremer.

At least 19 recordsLinked to original sources

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

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

[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

The hypothalamic lateral tuberal nucleus in Alzheimer's disease.

The hypothalamic lateral tuberal nucleus was investigated in 5 young patients, aged 45 to 64 years, with Alzheimer's disease (AD) and in 5 age-matched control subjects. Combining conventional histopathological and immunocytochemical staining with neuronal counts, a peculiar form of neuronal pathology was characterized. Although neurons and neurites in the lateral tuberal nucleus of AD specimens were heavily stained by Alz-50, silver and thioflavine-S stains disclosed few neurofibrillary tangles or neurites. The numbers of neurons in the lateral tuberal nucleus of patients with AD (67,450; SEM = 5,050) were no different from those of control subjects (58,900; SEM = 2,450). In the AD patients, few plaques were present and were almost exclusively of the amorphous variety. We conclude that neurons in the lateral tuberal nucleus show an early stage of AD-related cytoskeletal pathology (Alz-50 positivity), but without plaques or neuronal death.

Alzheimer Disease

Intraperitoneal hepatocyte transplantation: morphological results.

The value of isolated intraperitoneal hepatocyte transplantation as a temporary support in acute hepatic failure is controversial since the functional capacities and survival rate of auxiliary transplanted liver cells are uncertain. It was the aim of this study to investigate the survival rate of intraperitoneally (i.p.) transplanted hepatocytes and microcarrier attached hepatocytes. In 25 rats (group A) an i.p. hepatocyte transplantation and in 30 rats (group B) an i.p. microcarrier attached hepatocyte transplantation was performed and the animals were killed at 3 h, 12 h, 24 h, 3 and 7 days (group A) and 1, 2, 3, 7, 14 and 28 days (group B) after transplantation. Histological investigation showed that transplanted hepatocytes undergo complete cell necrosis within 3 days (groups A and B). Liver cell necrosis was followed by a peritoneal reaction resulting in granuloma formation in both groups. Since liver cell necrosis occurred soon after transplantation and could not be avoided by attachment of liver cells to microcarriers, it can be concluded that metabolic support from transplantation cannot be expected in acute hepatic failure.

Animals

[Managing a high risk pregnancy after liver transplantation from the obstetrician's point of view. A case report].

We report about a pregnancy and obstetrical management of a patient who had undergone a liver transplantation. Following a normal pregnancy, the newborn was born spontaneously in the 39th week of pregnancy. During the pregnancy the CSA blood levels were controlled frequently as well as the fetal development. An amniocentesis for chromosomal analysis, measurement of AFP blood levels, virus diagnostic and a phase III ultrasound diagnostic were performed. Immediately after delivery the baby was examined carefully by a pediatrician. Management of such a risk pregnancy, after organ transplantation, is possible if there is a close interdisciplinary cooperation between obstetricians, pediatricians, transplant surgeons and internal medicine specialist.

Adult

[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

[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

[Computer-assisted placement for after-loading procedures with iridium 192].

Extremely high precision is needed to place radiation sources in the after-loading technique, but localisation techniques so far described do not offer the required accuracy. We used the computer-assisted surgery (CAS) localising system for the placement of after-loading probes in patients with head and neck carcinoma. Surgical instruments can be positioned without visual control using the CAS system. Since 1989 after-loading tubes have been placed using the CAS method. We evaluated the accuracy to be better than 1 mm. Ten after-loading treatments with iridium 192 of head and neck carcinomas were carried out between August 1989 and February 1991 with exact placement of the after-loading tubes in the nasopharynx. It seems feasible to treat cerebral tumours as well as tumours of the pelvic region by computer-assisted after-loading brachytherapy.

Adult

[Pancreatic pseudocysts and their interdisciplinary therapy].

A retrospective analysis was performed of 87 patients with pancreatic pseudocysts admitted at the Dept. of Surgery University of Hamburg from 1985 to 1989. Pseudocysts as a result of chronic pancreatitis were found in 54%. 42 patients underwent 49 operations, 35 have been drained endoscopically. In 10 cases there was spontaneous remission. 25 patients were treated by Roux-en-Y cystojejunostomy. In 14 patients a resection of the pancreas was performed. The mean follow-up interval was 26 (operative treated) and 22 months (endoscopic treatment). 50% of all patients in both groups reported pain relief. The mortality in operated patients was 7% and 0% for endoscopic treatment. In ten cases a recurrent disease was observed.

Adult

[Intraperitoneal hepatocyte transplantation: morphologic findings and clinical relevance].

In order to evaluate if intraperitoneal hepatocyte transplantation could include a therapeutic approach in acute hepatic failure, isolated hepatocytes as well as microcarrier attached hepatocytes were transplanted intraperitoneally and the survival rates of transplanted cells were evaluated morphologically. The histological investigations showed, that the transplanted hepatocytes undergo complete cell necrosis within 3 days due to a lack of substrate, which also cannot be avoided by an attachment to microcarriers. Liver cell necrosis is followed by a peritoneal reaction resulting in granuloma formation. Because liver cell necrosis occurred early after i.p. hepatocyte transplantation a functional support in acute hepatic failure cannot be expected.

Animals

Acute gastrointestinal bleeding as a complication of pancreatic pseudocysts.

We report on 6 patients with chronic pancreatitis and pancreatic pseudocysts, operated on for acute bleeding into the cyst. The splenic artery and vein, the renal artery, the aorta and the splenic parenchyma were identified as bleeding sites. In 4 patients the acute hemorrhage occurred during hospitalization. In all patients the operative procedure included hemostasis and/or drainage/resection of the cyst. In 3 cases an additional splenectomy was performed. None of the patients died postoperatively. In 5 patients the pancreatitis was induced by alcohol abuse. In all patients the pseudocysts were diagnosed prior to the acute hemorrhage. CT-scan and angiography were able to localize the origin of the bleeding in 2 cases.

Acute Disease

[Securing colon anastomoses by surrounding it with a polyglycolic acid filament net. An animal experiment study].

Resorbable polyglycolic-acid (PGA)-nets are used successfully for the treatment of traumatic ruptures of parenchymous organs. This experimental study was carried out to investigate whether PGA-nets can also re-enforce jeopardized colon anastomoses. In 75 Wistar rats the transverse colon was transsected and reanastomosed. Animals were divided into 2 groups: in one group the anastomosis was applied sufficiently, in the other group the anastomosis was applied potentially insufficient. Out of each group a PGA-net was placed around the anastomosis in one half of the animals. The results demonstrated that delayed healing of the anastomoses as well as spontaneous suture line disruptions followed by peritonitis only occurred in those animals in which a PGA-net had been used. The evaluation of the bursting strength could confirm this finding of delayed consolidation. The bursting pressure therefore was significantly decreased in all animals in which a PGA-net had been applied, compared to the control group operated without PGA-net application. We therefore conclude that PGA-net application in colon anastomoses inhibits the consolidation of the suture line, probably because of a lack of contact to the peritoneal and omental tissue.

Anastomosis, Surgical

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

[Pregnancy following organ transplantation].

In 17 of 74 patients, in the 20-40 years of age group, who had undergone an organ transplantation (kidney or liver), the course and outcome of pregnancy were evaluated. In three cases, the pregnancies ended in premature miscarriage and in five cases they were terminated for medical reasons. Nine infants were born alive between the 32nd to the 40th week of gestation, six of them spontaneously, three of them by abdominal Caesarean section. One of these infants born in the 32nd week of gestation with a birth weight of 800 grams died on the second day after birth. One infant born in the 33rd week of gestation showed incidence of a persistent ductus arteriosis Botalli. Four of the nine newborns suffered from intrauterine dystrophy. The birth weight of four further infants corresponded to the 10th to 25th percentile. Neither the incidence of a maternal varicella zoster infection in the early stages of pregnancy nor the reactivation of a herpes simplex (HSV) and cytomegalia virus infection during the pregnancy resulted in any perceptible damage to the infant or transplant. During pregnancy, three of the mothers were treated with immunosuppressants, either with a combination of azathioprine and prednisone (conventional) or cyclosporine (CSA) and prednisone, or with a combination of all three drugs (triple therapy). As opposed to the newborn of those mothers, who had been treated conventionally, the newborn of those treated with CSA showed post partum a tendency towards hypocalcaemia. Two of the mothers gave birth to their infants outside the Federal Republic of Germany.(ABSTRACT TRUNCATED AT 250 WORDS)

Abnormalities, Drug-Induced