[Congenital post-papillary, membraneous duodenal stenosis as a rare cause of jaundice in adulthood].
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Biomedical subjects
Publications and source records attributed to B Kremer.
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The results of surgical treatment in 8 patients with polycystic liver disease (group A) and 6 patients with symptomatic simple cysts (group B) are presented. In group A, all patients underwent hepatic resection, in some cases combined with deroofing or fenestration procedures. Complications occurred postoperatively in 3 patients (37.5%). Two of these three patients had no perceived benefit from the surgical treatment. Two other patients had recurrent symptoms 12 and 20 months after operation, and one patient underwent a second resection after a 5-year-interval; she is now symptom-free after 19 months of follow-up. In group B, no complications occurred after surgical treatment consisting of either deroofing or pericystectomy. These patients were symptom-free after 5-32 months of follow-up. The therapeutic management of non-parasitic cystic liver disease is still under discussion. We advocate wide deroofing or pericystectomy for symptomatic simple cysts. Hepatic resection in highly symptomatic polycystic disease is surgically demanding and should be performed by an experienced liver surgeon. Relief of symptoms can be achieved, but the rate of complications is relatively high.
Between 1985 and 1991 268 liver resections were performed at the Department of Surgery of the University of Hamburg. 89 patients underwent liver resections because of colo-rectal secondaries. 82 patients could be followed for more than 1 year postoperatively. Their courses were analysed regarding the prognostic factors: age, sex, localisation of the primary tumor, size, number and localisation of metastases and radicality (R0-R1-R2-stage of resection). The results showed, that a significant prognostic influence regarding the long-term-survival rate could only be observed in patients, in which a R0-resection with a tumor-free margin of more than 1 cm was performed. In these patients the cumulative 5-year survival rate was 37% with a median survival time of 39 months. In patients with a tumor-free margin < 1 cm (R0b) the long-term prognosis did not differ significantly from cases with R1 or R2 resections (under 10%).
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.
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.
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.
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.
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.
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.
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.
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.
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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.
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.
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.
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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.
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