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Biomedical subjects

J Hauss

Publications and source records attributed to J Hauss.

At least 19 recordsLinked to original sources
PubMed2007

Sirolimus improves early microcirculation, but impairs regeneration after pancreatic ischemia-reperfusion injury.

Ischemia and reperfusion injury remains a relevant problem in clinical pancreas transplantation. We investigated the effect of sirolimus (SRL) in a rodent model of 90-min warm pancreatic ischemia. Four groups were studied: (1) sham surgery and vehicle; (2) sham surgery and SRL; (3) warm ischemia and vehicle; (4) warm ischemia and SRL. SRL (1.5 mg/kg/day) and vehicle were administered intraperitoneally for 3 days prior to surgery until the animals were killed. Microcirculation was assessed immediately after reperfusion by means of intravital fluorescence microscopy. Histopathological injury, apoptosis, proliferation and biochemical parameters were analyzed at 2 h, 1 day and 5 days after surgery. Ninety minutes after ischemia, intravital microscopy revealed an improved functional capillary density (p < 0.05) and reduction of adherent leucocytes (p < 0.01) and platelets (p < 0.05) in the SRL-treated group compared to the vehicle-treated controls. In contrast, on day 5 after ischemia, the pancreatic tissue of SRL-treated animals showed a higher grade of histological injury (p < 0.05) and higher rate of apoptotic cells (p < 0.05) than the vehicle controls. In summary, our data indicate that administration of SRL improves microcirculation at a very early stage, but results in an impairment of the recovery phase after pancreatic ischemia-reperfusion injury.

Animals↗
PubMed2006

Histopathological outcome of 597 isolated soft tissue tumors suspected of soft tissue sarcoma: a single-center 12-year experience.

BACKGROUND: The aim of this present report was to analyze the patients referred to us with the presumptive diagnosis of soft tissue sarcoma (STS). METHODS: We reviewed all patients referred to us with suspected soft tissue sarcoma (STS) of the extremities or trunk over a 12-year period. RESULTS: We treated 597 patients with soft tissue tumors. Open biopsy revealed soft tissue sarcoma in 318 cases, benign mesenchymal tumor in 124 cases and isolated metastases (ISTM) from carcinomas in 98 patients; other pathologies were found in 57 patients. The primary carcinomas were lung cancer in 26 patients, breast cancer in 19 patients, renal carcinoma in 16 patients, carcinoma of the esophagus in 12 patients, colonic carcinoma in 5 patients, thyroid gland cancer in 6 patients, and in 14 patients carcinoma of unknown primary was diagnosed. CONCLUSIONS: In our collective with soft tissue tumor, 50% of the patients had the diagnosis of soft tissue sarcoma, 20% presented with a metastasis of carcinoma and 20% had a benign tumor. Referring to our results, in patients with the presumptive diagnosis of soft tissue sarcomas, soft tissue metastasis of a primary carcinoma was unexpectedly common, indicating that greater consideration should be given to this differential diagnosis.

Adolescent↗
PubMed2006

Successful staged kidney and liver transplantation for glycogen storage disease type Ib: A case report.

Glycogen storage disease type Ib is a rare metabolic disease caused by a defect of the G6P transporter. Patients suffer from hypoglycemic episodes; growth and developmental delay; osteoporosis; neutropenia; and tendency to infections, ovarian cysts, and liver adenomas. Terminal kidney disease is a rare complication. Liver transplantation has been performed to prevent malignant transformation of hepatic adenomas. We present the case of a female patient with glycogenosis type Ib who had severe hypoglycemic episodes and recurrent infections since early childhood. She became dialysis dependent at the age of 24 years. Kidney transplantation was performed at age 30, and liver transplantation 2 years later. The main indication for liver transplantation were the persistent, therapy-refractory hypoglycemic episodes. The transplanted kidney function is stable. The liver transplantation resulted in the disappearance of hypoglycemic episodes, with the patient leading a normal life and eating a normal diet. The neutropenia did not recover, but there were no more significant infectious episodes after liver transplantation. This is, to the best of our knowledge, the first communication of a dual kidney and liver transplant performed in a patient with glycogenosis type Ib. It confirmed the beneficial effect of liver transplantation on the quality of life of patients with severe hypoglycemia. The transplantation should be attempted earlier in the course of the disease to reduce complications and allow catch-up growth. Hepatocyte transplantation may be considered; however, long-term results seem to be rather poor in the few documented cases.

Adult↗
PubMed2006

[Need and extension of lymph node dissection in gallbladder carcinoma].

Gallbladder carcinomas have the propensity to metastasize early, commonly into locoregional lymph nodes. For gallbladder carcinomas with infiltration of at least the muscularis (pT1b) or subserosa (pT2), surgical therapy with a curative intent always includes a locoregional lymphadenectomy besides cholecystectomy and an anatomical or atypical liver resection. In incidentally discovered gallbladder carcinomas, such a locoregional lymphadenectomy should be performed depending on the age of the patient and on tumor extension. However, this only is helpful with respect to prognosis, if the metastases are limited to the hepatoduodenal ligament or to the posterosuperior pancreaticoduodenal nodes. For gallbladder carcinomas with metastases into more distal lymph nodes, especially in paraaortal nodes, lymphadenectomy only rarely impacts long-term survival. An extended lymphadenectomy that includes resection of the bile duct and/or pancreatic head is associated with an increased rate of morbidity and mortality, therefore not being of proven advantage in terms of prognosis.

Cholecystectomy↗
PubMed2005

Alpha1-and beta2-adrenoceptors in the human liver with mass-forming intrahepatic cholangiocarcinoma: density and coupling to adenylate cyclase and phospholipase C.

Besides the regulation of hepatic metabolic pathways in which adrenoceptors are mainly involved, their effect on the second messenger cAMP is thought to be related to the growth and differentiation of neoplastic cells. However, few studies have been done on the status of these structures in the human liver affected by cholangiocarcinoma (CC). Thus, in this study, changes in densities of alpha1- and beta2-adrenoceptors (alpha1-and beta2-ARs) were investigated in membranes of human liver with cholangiocarcinoma, and for comparison, in membranes of non-adjacent non-tumour liver using the potent antagonists [3H]-prazosin and [1I]-iodocyanopindolol (ICYP) respectively. In addition, the activity of membrane-bound phospholipase C (PLC) and adenylate cyclase (AC) was also studied. In CC liver, the density of alpha1-and beta2-ARs was significantly reduced, compared with non-tumour liver tissues (alpha1-ARs: 23.38+/-4.69 vs 80.35+/-10.52, P=0.0002 beta2-ARs: 14.27+/-2.93 vs 33.22+/-4.32 fmol/mg protein, P=0.03), whereas the ligand affinities (KD) remained unchanged. The beta2-selective antagonist ICI 118,551 was about 100 times more potent in inhibiting ICYP binding than the beta1-selective antagonist CGP 20712A; thus, more than 98% of the beta-ARs were of the beta2-subtypes. The AC activity upon stimulants acting on beta-AR (isoprenaline), G-protein (GTP, NaF) and AC (forskolin) was decreased in CC liver. Similarly, noradrenaline-stimulated PLC activity was significantly reduced in tumour tissues. In conclusion, in CC liver the alpha1- and beta2-ARs density was down-regulated and the neoplastic invasion blunted AC and PLC activity. These quantitative changes may help to elucidate not fully understood pathogenetic mechanisms of disturbed hepatic metabolic processes, such as hypoglycemia during cancer in human liver.

Adenylyl Cyclases↗
PubMed2005

[Surgical management, prognostic factors, and outcome in hepatic trauma].

Hepatic trauma is a rare surgical emergency with significant morbidity and mortality. Extensive experience in liver surgery is a prerequisite for the management of these injuries. The medical records of 68 consecutive patients with hepatic trauma were retrospectively reviewed for the severity of liver injury, management, morbidity, mortality, and risk factors. Of the patients, 14 were treated conservatively and 52 surgically (24 suture/fibrin glue, 16 perihepatic packing, 11 resections, 1 liver transplantation). Two patients died just before emergency surgery could be performed. Overall mortality was 21% (14/68), and 13, 14, 6, 27, and 50% for types I, II, III, IV, and V injuries, respectively. Only nine deaths (all type IV and V) were liver related, while four were caused by extrahepatic injuries and one by concomitant liver cirrhosis. With respect to treatment, conservative management, suture, and resection had a low mortality of 0, 4, and 9%, respectively. In contrast, mortality was 47% in patients in whom only packing was performed (in severe injuries). Stepwise multivariate regression analysis proved prothrombin values <40%, ISS scores >30, and transfusion requirements of more than 10 red packed cells to be significant risk factors for post-traumatic death. Type I-III hepatic injuries can safely be treated by conservative or simple surgical means. However, complex hepatic injuries (types IV and V) carry a significant mortality and may require hepatic surgery, including liver resection or even transplantation. Therefore, patients with severe hepatic injuries should be treated in a specialized institution.

Adolescent↗
PubMed2005

Changes in quality of life after renal transplantation.

Quality of life is becoming an increasingly important concept in the evaluation of different therapeutic interventions. In this cross-sectional study, quality of life analyzed in 76 renal transplant patients was compared with 65 patients with end-stage renal disease (ESRD) awaiting transplantation. Both groups were asked to estimate their subjective quality of life by responding to a multidimensional questionnaire that sought information in the following areas: (1) sociodemographic data, (2) a personality test (Giessen-Test), (3) information about employment, (4) assessment of anxiety and depression (GHQ-28), (5) the SF-36 as an important measurement for quality of life, and (6) the ESRD symptom checklist-transplantation module. The sociodemographic profiles of both groups were similar. The SF-36 revealed significantly higher values for transplant recipients in terms of physical functioning (P < or = .001), general health perceptions (P < or = .01), social functioning (P < or = .01), and physical summary value (P < or = .001). The other tests (Giessen-Test and GHQ-28) and employment data showed no significant differences between the two samples. Collectively, the data demonstrate a considerable improvement in quality of life in renal transplant patients. Despite good physical recovery and higher quality of life after transplantation, the rate of vocational rehabilitation remained low, partially explained by the currently high levels of unemployment.

Affect↗
PubMed2005

Computerized CT-based 3D visualization technique in living related liver transplantation.

INTRODUCTION: For living donor liver transplantation (LDLT) accurate diagnostic workup is essential. Multiple imaging approaches are currently used. Problems arise in the assessment of vascular and bile duct anatomy, liver graft volume, and vascular territories involved. A 3D visualization system that improves anatomic assessment, allows interactive surgery planning, and acts as an intraoperative guide with enhanced precision is required. Refinements in computed tomography (CT) technology with the introduction of multidetector-row CT scanners and implementation of mathematical methods on computerized digital data has enabled CT-based 3D visualizations. MATERIALS AND METHODS: Sixteen LDLT candidates and three LDLT recipients were assessed by multislice CT examination. Image processing of the digital raw data for 3D visualization included segmentation and calculation of center lines. A hierarchical mathematical model representing the vascular and biliary tree was created. This allowed calculation of individual vascular territories. RESULTS: 3D CT-based visualization in LDLT facilitates diagnostic workup with high accuracy for analyses of vascular and bile duct variants, volumetry, and assessment of the optimal surgical splitting line of the living donor liver. Resultant areas of either arterial devascularization or venous congestion can be displayed and quantified preoperatively. The diagnostic method is of major impact on patient selection and directly influences intraoperative surgical guidance. The currently practiced "multiple imaging approach" approach, especially with regard to invasive diagnostics, can be avoided in the future.

Family↗
PubMed2005

[Current diagnostics and therapy for carcinomas of the biliary tree and gallbladder].

Carcinoma of the biliary tree are rare tumours of the gastrointestinal tract with a rising incidence during the last years. Biliary neoplasms are classified into intra- and extrahepatic cholangiocarcinoma (Klatskin tumour, middle and distal extrahepatic tumours), gallbladder cancer, and ampullary carcinoma. Transformation of normal into malignant bile duct tissue requires a chain of consecutive gene mutations, similar to the adenoma-dysplasia-carcinoma-sequence in colon cancer. Abdominal ultrasound, combined non-invasive magnetic resonance cholangiography/tomography (MRC/MRT), and facultatively endoscopic retrograde cholangiography (ERC) for unclear diagnosis, represent the gold standard for primary diagnosis. For ampullary carcinoma, endosonography and endoscopic biopsy are the diagnostic tools of choice. Cure is attainable only by formal curative radical surgical resection. Increasing surgical radicality within the last years enabled clearly improved 5-year survival rates. In contrast, there has been no clinical benefit for adjuvant and neoadjuvant therapies. For palliation, bile duct stenting and photodynamic therapy are established methods. Radio- and chemotherapy should be reserved for clinical studies. New therapeutic approaches include brachytherapy, the use of modern chemotherapeutics, COX-2- and tyrosine kinase-receptor-inhibitors.

Algorithms↗
PubMed2003

Liver transplantation for hepatic and neurological Wilson's disease.

Wilson's disease (WD) is an autosomal-recessive inherited disorder of copper metabolism characterized by excessive deposition of copper throughout the body. If medical treatment fails in cases of fulminant hepatic failure and progressive hepatic dysfunction due to advanced cirrhosis, liver transplantation (OLTx) has been demonstrated to be a valuable treatment option. Between December 1993 and December 2002, 225 OLTxs in 198 patients were performed in our institution. In this consecutive series six patients (three females and three males) were liver grafted for WD. The follow-up ranged from 3 to 7 years. All patients are alive with well-functioning grafts at present. The ceruloplasmin levels increased after transplantation and remained normal. The Kayser-Fleischer ring disappeared in all patients, and urinary copper excretion normalized. The neurological manifestations in the two patients with severe neurological symptoms showed after 2 to 5 years a downward tendency; in one the ataxic movements disappeared completely. The psychiatric disorder in one patient disappeared as well the mild neurological symptoms in the patient with CHILD A cirrhosis. These two patients are fully recovered and returned to work. OLTx should be considered as a treatment option in patients with severe progressive neurological deficits even in cases with stable liver function since liver grafting definitely cures the underlying biochemical defect. In such cases an early decision for liver transplantation is justified because neurological deficits may become irreversible.

Copper↗
PubMed2003

Mutations of the BRAF gene in cholangiocarcinoma but not in hepatocellular carcinoma.

BACKGROUND: The Raf/MEK/ERK (mitogen activated protein kinase-MAPK) signal transduction cascade is an important mediator of a number of cellular fates, including growth, proliferation, and survival. The BRAF gene, one of the human isoforms of RAF, is activated by oncogenic Ras, leading to cooperative effects in cells responding to growth factor signals. AIMS: The aim of this study was to elucidate a possible function of BRAF in liver tumours. METHODS: Mutations of BRAF and KRAS were evaluated in 25 hepatocellular carcinomas (HCC) and in 69 cholangiocarcinomas (CC) by direct DNA sequencing analyses after microdissection. The presence of active intermediates of the MAPK pathway was assessed immunohistochemically. The results obtained were correlated with histopathological variables and patient survival. RESULTS: Activating BRAF missense mutations were identified in 15/69 CC (22%) and in one case of tumour surrounding liver. KRAS mutations were found in 31 of 69 (45%) CC examined and in two cases of tumour surrounding non-neoplastic liver tissue. In HCC, neither BRAF nor KRAS mutations were detected. All 31 CC with KRAS mutations had an intact BRAF gene. We failed to observe a correlation between BRAF or KRAS mutations and histopathological factors or prognosis of patients. CONCLUSIONS: Our data indicate that BRAF gene mutations are a relatively common event in CC but not in HCC. Disruption of the Raf/MEK/ERK (MAPK) kinase pathway, either by RAS or BRAF mutation, was detected in approximately 62% of all CC and is therefore one of the most frequent defects in cholangiocellular carcinogenesis.

Base Sequence↗