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Biomedical subjects

H J Rumpelt

Publications and source records attributed to H J Rumpelt.

At least 19 recordsLinked to original sources
PubMed1996

Recurrent hematuria: a novel clinical presentation of hereditary complete complement C4 deficiency.

A 10-year-old boy suffered from recurrent attacks of fever, vomiting, and hematuria. During disease flares, circulating immune complexes were detected in the serum. Elevated levels of Bb, Ba, and C3a indicated complement activation through the alternative pathway. Complement C4 was undetectable. C4 phenotyping by agarose gel electrophoresis showed complete C4 deficiency. Restriction fragment length polymorphism (RFLP) studies showed a homozygous deletion of the C4B and 21-hydroxylase A genes. A mild mesangioproliferative glomerulonephritis with mesangial deposits of immunoglobulin (1g) G, IgM, IgA, Clq, C3, properdin, and terminal complement complex was probably caused by immune complex deposition and alternative complement pathway activation. Treatment with low-dose prednisolone substantially reduced the frequency of further episodes.

Biopsy↗
PubMed1995

[Cystic degeneration of the adventitia of the popliteal artery as a possible sequela of entrapment syndrome].

We report two cases of cystic adventitial degeneration of the popliteal artery in young sportsmen. The pathogenetic role of microtrauma is suggested by the localisation of the adventitial cysts, the clinical symptoms, as well as the immunohistochemical demonstration of foamy macrophages instead of endothelial cells in the wall of the cysts. In one case the cause of the intermittent claudication could be verified by duplex sonography and comparison of the arterial pressure of both legs with a Doppler method only after physical exercise. As expected, an angiodilatation has only a temporary effect. Therefore a surgical resection of the cyst or of the involved arterial segment had be performed.

Adult↗
PubMed1994

[Adenomatous neoplasia of the middle ear].

Adenomatous neoplasm occurring in the middle ear is rare. The clinical findings are non-specific and include otological symptoms as well as sometimes functional deficits of the lower cranial nerves. The final diagnosis will be made only after elaborate histological examinations because of the inconsistent radiological and clinical findings. DNA analysis yields some information on the prognosis of this tumour. We report on a case where a primary adenomatous neoplasm developed in the middle ear, and discuss our concept of therapy.

Adenoma↗
PubMed1994

Malignant rhabdoid tumor in the gastric wall of an aged orangutan (Pongo pygmaeus).

A 34-year-old female orangutan (Pongo pygmaeus) developed renal failure and became uremic. At necropsy, large gastric masses were present around the cardia and in the corpus. Abdominal metastases occurred in the liver, pancreas, and right ovary. Light microscopic examination of the tumor revealed polygonal cells with vesicular nuclei and prominent nucleoli. The growth pattern was predominantly solid. Focal areas contained excentric cytoplasmic intermediate filament inclusions, as identified by immunohistochemistry and electron microscopy. Immunohistochemical procedures demonstrated mainly the vimentin type of intermediate filaments. Except for occasional cytokeratin, other intermediate filament markers and neural, lymphocytic, and histiocytic markers stained negative. The morphologic and ultrastructural characteristics are typical for a malignant rhabdoid tumor, a term used in human pathology to describe a rare and extremely aggressive malignancy of uncertain histogenesis. Although usually located in the infant kidney, a few reports have documented the occurrence of similar lesions in extrarenal sites of adults. In human tumors, vimentin is often combined with the expression of cytokeratins. The sparsity of the cytokeratin filaments in this case might be due to species-specific variations and/or may reflect the hypothesis of a phenotypic concept encompassing a spectrum of histogenetic diversity.

Animals↗
PubMed1992

[Secondary achalasia in non-Hodgkin's lymphoma of low malignancy and leiomyomatosis of the cardia].

A 28-year-old man had been dysphagic for 9 months with a weight loss of 4 kg. A preliminary diagnosis of primary achalasia was made on the basis of typical radiological and manometric findings. Despite balloon dilatation of the cardia the symptoms did not improve and further diagnostic tests were performed. Ultrasound demonstrated a 4 cm tumour below the cardia. But its type and possible malignancy remained uncertain even at laparotomy. But as a malignant tumour was suspected a gastrectomy and omentectomy with removal of the local and regional lymph nodes were performed. After this the symptoms regressed and postoperative food intake was without problem. Histological examination of the surgical specimen revealed leiomyomatosis of the cardia and the gastric fundus, combined with a low-malignant B-cell lymphoma of the mucosa-associated lymphatic tissue. Oesophagus manometry 4 months postoperatively gave normal results. The patient has been free of symptoms and without evidence of recurrence for by now 18 months postoperatively.

Adult↗
PubMed1992

Fibrosing alveolitis responsive to corticosteroids following Legionnaires' disease pneumonia.

Two male patients ages 54 and 58 years had persisting pneumonia with dry cough, dyspnea, weight loss, and fever up to 39 degrees C that did not respond to erythromycin treatment. There was extensive restrictive impairment of ventilation and loss of diffusing capacity for carbon monoxide. Histologic examination of the basal pulmonary infiltrates showed fibrosing alveolitis. Serologic titers indicated that the patients had suffered from Legionella pneumophila infection. We believe that Legionella had caused the fibrosing alveolitis since there was absence of any other causative agents or factors. Both patients responded to corticosteroid treatment with rapid clinical improvement but delayed radiologic regression.

Humans↗
PubMed1992

Alport-type glomerulopathy: evidence for diminished capillary loop size.

Hereditary nephropathy of the Alport type is morphologically characterized by a specific and diagnostic thinning and splitting lesion of the glomerular basement membranes, which can be recognized only by electron microscopy. The light microscopical aspect has not been considered to be characteristic until now. This paper describes a light microscopical constellation of glomerular alterations by which ATGP can be recognized with high probability. Three histological features are of importance: 1. ATGP glomeruli in patients older than 10 years of age mostly have smaller capillary loops than age-matched controls. However, during the first 10 years of life no difference in glomerular capillary loop size was noticed. 2. ATGP loops often stain less intensely with basement membrane stains. 3. Presence of fetal-like glomeruli. Using this triad of light microscopic parameters as a screening tool, ATGP-cases were found without knowledge of any clinical data among other glomerulopathies with a sensitivity of 72% and a specificity of 93%. The definitive diagnosis, however, depends on electron microscopy.

Adolescent↗
PubMed1991

[An association of arterial occlusive disease with cyclosporine therapy after kidney transplantation].

Renal transplantation followed by immunosuppression with cyclosporine (whole-blood levels 200-300 micrograms/l) and methylprednisolone (4 mg daily) was performed in a 54-year-old man with chronic glomerulonephritis. Three years later rapidly progressive arterial obstructive disease (peripheral type) developed. Parenteral treatment with prostaglandins, calcium antagonists and nitrates, as well as a lumbar sympathectomy, was unsuccessful so that, in rapid succession, several amputations on upper and lower limbs became necessary. The disease progression was arrested only when azathioprine replaced cyclosporine. Raynaud's phenomenon, present at the time, also disappeared and analgesics were no longer required. Histological examination revealed severe Mönckeberg arteriosclerosis and thromboembolic occlusion of the affected vessels. The cyclosporine treatment, especially the hypercoagulability induced by it, and the Mönckeberg arteriosclerosis were thought to be significant factors in the pathogenesis of the rapidly progressive arterial disease. It is concluded that, in the presence of progressive arterial obstructive disease occurring under cyclosporine treatment, the replacement by other immunosuppressive agents should be considered.

Amputation, Surgical↗
PubMed1991

Autosomal dominant polycystic kidney disease--in vitro culture of cyst-lining epithelial cells.

The major form of autosomal dominant polycystic kidney disease (ADPKD) in humans is linked to the PKD1 gene on chromosome 16p. The identity of the gene and the underlying pathogenetic mechanisms are not yet defined. Cyst-lining epithelial cells derived from a polycystic kidney were successfully grown in culture and designated MZ-PKD-1 cells. By linkage analysis, the related pedigree of the nephrectomized patient could be linked to the PKD1 gene on chromosome 16p. Thus, these cells exhibit the genotype of a mutated PKD1 gene and represent an in vitro culture model for ADPKD involving chromosome 16p. The antigenic phenotype was characterized immunohistologically by epithelial differentiation antigens and markers of individual nephron segments. An essentially identical antigenic pattern of proximal tubular cells was observed both in vitro and in fresh frozen tissue. Electron microscopy showed the formation of a microvillous-like coating. During growth phases in vitro successive changes in the cell shape were observed. MZ-PKD-1 cells exhibited a limited lifespan ending in replicative senescence. Northern blot analysis of kidney-growth-related genes, c-myc, TGF-alpha, TGF-beta 1, and EGF receptor revealed abundant expression of all of these genes in MZ-PKD-1 cells.

Antibodies, Monoclonal↗
PubMed1991

[Obstructive jaundice: its histological diagnosis by percutaneous endoluminal bile duct biopsy].

32 forceps biopsies were performed in 30 patients with obstructive jaundice during percutaneous transhepatic biliary drainage procedures. In one patient an adequate specimen could not be obtained. In 25 of the remaining 31 cases diagnosis was confirmed histologically (malignant tumours: n = 22, benign stricture: n = 3). In 6 patients false-negative results were obtained. Transluminal biopsy is an easily performed adjunct to percutaneous transhepatic diagnostic interventions with minimal additional discomfort for the patient. In many cases percutaneous needle biopsy can be avoided. Forceps biopsy enables nonoperative histological diagnosis of small carcinomas of the bile ducts.

Adult↗
PubMed1991

Bellini duct carcinoma: further evidence for this rare variant of renal cell carcinoma.

Bellini duct carcinomas have recently been identified as a new entity in the spectrum of renal cell carcinomas and 10 cases have now been reported. The present paper adds detailed clinical and morphological data on six new cases. In addition, immunohistological and electronmicroscopical results support the origin of these tumours from the renal collecting ducts, especially the papillary ducts (Bellini ducts). A set of immunohistological reactions, including reactions to cytokeratins 13 and 19, vimentin and UEA-1 was found to facilitate the differential diagnosis of Bellini duct carcinomas from other renal cell carcinomas and infiltrating urothelial carcinomas of renal pelvis.

Adult↗
PubMed1990

[Classification of renal cell carcinoma/tumors and their relationship to the nephron-collecting tubules system].

After a controversial phase of nomenclature (including--among others--the terms "hypernephroma" and "hypernephroid carcinoma") a cytomorphologically defined subtyping of renal cell tumours (adenomas, carcinomas, oncocytomas) is offered, based on new electron microscopical and histochemical observations. These data are in part supported by cytogenetical findings reported in the literature. Phenotypical/histogenetical relations to different parts or cell types, respectively, of the nephron-collecting duct system could be demonstrated. Chromophobe cell carcinoma and oncocytoma exhibit features of the intercalated cells.

Carcinoma, Renal Cell↗
PubMed1990

[Persisting alveolitis after Legionella pneumonia].

Pneumonia was diagnosed radiologically in three patients (43, 54 and 58 years old, respectively), presenting with temperatures between 39 degrees and 40 degrees C, cough and weight loss. These signs persisted for 6, 7 and 13 weeks, respectively, but the pathogens could not be cultivated. Lung function analysis showed partial respiratory insufficiency with extensive restrictive impairment of ventilation. Samples of lung tissue were obtained in all three cases and histology revealed fibrosing alveolitis. In two patients serology yielded antibody titres of 1:512 and in one patient of 1:128, against Legionella pneumophila. Treatment with 1 g erythromycin three times daily was unsuccessful. Therefore, the patients were given prednisone at an initial dosage of 50-100 mg which was subsequently reduced. Lung function normalised during this treatment course, radiological findings and antibody titres receded. Hence, treatment with corticosteroids should be attempted if there is an urgent suspicion of fibrosing alveolitis caused by Legionella pneumophila, after having excluded a florid infectious pneumonia and after failure of erythromycin treatment.

Adult↗
PubMed1990

Surgical correction of a severely obstructed pulmonary artery bifurcation in Takayasu's arteritis.

A young male patient (NYHA III) presented with a severe pulmonary artery bifurcation stenosis of unknown origin. He underwent successful radical resection and endarterectomy of abundant endoluminal and perivascular inflammatory tissue. Surgery was performed with extracorporeal circulation (ECC) without transection of the aorta and the pulmonary artery bifurcation was enlarged with a patch. Right ventricular to peripheral pulmonary artery pressure gradient fell from 80 mmHg preoperatively to 25 mmHg 18 months postoperatively. The patient is fully rehabilitated and working (NYHA I). Histological examination confirmed Takayasu's arteritis as the underlying disease and medium-term follow-up angiography showed an almost normal pulmonary artery bifurcation. We conclude that the radical surgical approach is a valuable palliation in this disease.

Adult↗