Pulmonary embolism--a rare complication of Schimke immunoosseous dysplasia.
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Child, Preschool↗
Biomedical subjects
K Amann
Publications and source records attributed to K Amann.
Intrauterine growth retardation aggravates the course of acute mesangioproliferative glomerulonephritis in the rat.
Intrauterine growth retardation (IUGR) aggravates the course of acute mesangioproliferative glomerulonephritis (GN) in the rat. Observational studies in children suggest that IUGR may be associated with a severe course of kidney diseases such as IgA nephropathy. We tested the hypothesis that IUGR leads to aggravation of acute mesangioproliferative GN in former IUGR rats. IUGR was induced in Wistar rats by isocaloric protein restriction in pregnant dams. Litter size was reduced to six male neonates in low protein animals (LP) and normal protein animals (NP). At 8 weeks GN was induced by injection of an anti-Thy-1.1 antibody. Rats were killed on days 4 and 14 after induction of GN and kidneys were investigated for inflammation and sclerosis using real-time polymerase chain reaction and histological methods. On day 4 after induction of GN, LP animals showed more glomerulosclerosis and tubulointerstitial lesions. On day 14, inflammatory markers (expression of monocyte chemoattractant protein 1, osteopontin, tumor necrosis factor and interleukin-6), extracellular matrix accumulation and markers of sclerosis (plasminogen activator inhibitor-1 expression, transforming growth factor-beta1 expression, score for glomerulosclerosis, glomerular deposition of collagen I and collagen IV) were more severe in LP animals. Some degree of induction of inflammatory and profibrotic markers was also present in non-nephritic LP animals. However, these rats did not display marked glomerulosclerosis or interstitial fibrosis. We conclude that after IUGR inflammatory damage is aggravated and the reparation of the kidney is impaired during the course of acute mesangioproliferative GN, leading to more sclerotic lesions.
Animals↗
Plasmapheresis therapy in an elderly patient with rapidly progressive Henoch-Schönlein purpura with disseminated organ involvement.
Henoch-Schönlein purpura (HSP) frequently occurs in children below the age of 15 years and is rare with increasing age. Prognosis and therapy largely depend on the clinical presentation. The disease may be preceded by an upper respiratory tract infection, and drugs have also been implicated in the pathogenesis of the disease. Most children recover from the illness, whereas 40% of adults have persistent hematuria and 10% develop chronic renal failure. Recent studies strongly suggest that adults with HSP should be monitored for prolonged periods and treated aggressively. Here, we present a case of a patient with HSP who developed multiorgan failure requiring assistance in breathing and dialysis, and also sustained gastrointestinal bleeding despite aggressive immunosuppressive therapy. In analogy to published data in children with severe HSP, the patient was treated by plasma exchange in combination with low dose oral cyclophosphamide, while high dose steroids were reduced over time. The patient could be discharged 70 days after admission. One year after discharge, the patient is doing well without any signs of activity of HSP and completely unremarkable renal function. The maintenance daily dose of steroids is 7.5 mg.
Cyclophosphamide↗
Atherosclerosis and vascular calcification in chronic renal failure.
Cardiovascular complications are a major clinical problem in patients with chronic kidney disease and end-stage renal failure; cardiac death accounts for approximately 40-50% of all deaths in these patients. Death from cardiovascular causes is up to 20 times more common in uremic patients than in the general population with the risk being even higher than in patients with diabetes mellitus. A high rate of myocardial infarction and excessive cardiac mortality have repeatedly been documented in patients with kidney disease and renal failure. Not only is the prevalence of myocardial infarction high, but also the case fatality rate is significantly higher in uremic patients with and without diabetes, respectively, compared to nonuremic patients. This is of particular interest since the prevalence of coronary atheroma in uremic patients was shown to be approximately 30% by autopsy and coronary angiography studies. Thus, coronary factors, i.e. atherosclerosis, and non-coronary factors may play an important role in the genesis of cardiac complications in the renal patient. In addition, renal failure recently has also be identified as a predictor of mortality in different stages of peripheral vascular disease. In particular, marked differences in the pathogenesis, morphology and course of atherosclerosis and arteriosclerosis under the conditions of renal failure have been documented. Among others increased plaque formation and particularly higher proportion and intensity of vascular calcification have been found in clinical and autopsy studies. In addition to the so-called classical or traditional risk factors, an important role for nonclassical risk factors such as microinflammation, hyperphosphatemia and oxidative stress has been documented in patients with renal failure and is discussed in detail.
Animals↗
[Diagnostic procedures and therapeutic options in xanthogranulomatous pyelonephritis -- experience gathered in Erlangen].
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Humans↗
Bone morphogenetic protein-7 expression and activity in the human adult normal kidney is predominantly localized to the distal nephron.
Bone morphogenetic protein-7 (BMP)-7 plays an important role during fetal kidney development. In the adult, BMP-7 is most strongly expressed in the kidney compared to other organs, but the exact expression pattern as well as the function of BMP-7 is unclear. The major aim of the present study was to define which parts of the human kidney do physiologically express BMP-7 and which cells appear to be targets of BMP activity by showing phosphorylated BMP-receptor-associated Smads 1, 5, or 8 and inhibitor of differentiation factor 1 (ID1) expression. BMP-7 expression was localized by immunohistology to the epithelia of the distal tubule as well as the collecting ducts (CDs). Phospho-Smads 1/5/8 and ID1 expression largely colocalized with BMP-7 and was also localized in the epithelia of the distal tubule and the CDs. This was confirmed by polymerase chain reaction-based mRNA expression analysis. In vitro, proximal tubular cells (PTCs) expressed BMP receptors and BMP-receptor-associated Smads and were reactive to BMP-7. Our data indicate that BMP-7 expression in the adult human kidney appears to be more restricted than in the fetal situation and predominantly found in the distal nephron. Also, evidence of in vivo BMP signalling (i.e. phospho-Smads and ID1 expression) was found there. These findings suggest that BMP-7 plays a physiological role mostly in this part of the kidney. Still, as reported previously, PTCs are responsive to BMP-7, but presumably not in an autocrine or paracrine mode in normal adult kidneys.
Bone Morphogenetic Protein 7↗
Low-grade T-cell lymphoma of the kidney and Waldenström's macroglobulinemia in a patient presenting with renal failure.
Renal failure is rarely the presenting manifestation of non-Hodgkin's lymphoma. We describe the unusual case of a patient who presented with uremia due to lymphomatous infiltration of the kidney by a low-grade T-cell lymphoma. The diagnosis of lymphoma was made by renal biopsy. Extrarenal nodular or extra-nodular involvement could not be detected. However, simultaneously, a lymphoplasmacytic lymphoma was found on bone marrow biopsy associated with IgM paraproteinemia. To our knowledge, this is the first report of a renal T-cell lymphoma associated with Waldenström's macroglobulinemia.
Aged↗
Management of spontaneous enophthalmos due to silent sinus syndrome: a case report.
Silent sinus syndrome (SSS) is a rare disease exhibiting unilateral enophthalmos and hypoglobus. A 26-year-old white female presented with right side enophthalmos and hypoglobus. There was no history of previous trauma or maxillary sinus diseases. A CT scan showed an opacified right maxillary antrum with decreased volume and downward bowing of the right orbital floor. From clinical and radiological findings the diagnosis SSS was made. Biopsies were collected from the maxillary sinus for the exclusion of malignancy. Two months later orbital floor reconstruction was carried out. Before antrostomy of the affected maxillary sinus, a relative enophthalmos of 4mm was determined. Five days after antrostomy the value reduced to 2.3mm. During the following 2 months the enophthalmos remained constant. At the end of the operation for orbital floor reconstruction it was 0.1mm. Five days after surgery the relative enophthalmos increased to 0.8mm. The value remained constant during the following 3 months. Initial antrostomy of the affected maxillary sinus may lead to a relevant, spontaneous reduction of enophthalmos. After a minimum period of 2 months a re-evaluation should be made, if a reconstruction of the orbital floor is still necessary for the correction of the globe position.
Adult↗
[Xanthogranulomatous pyelonephritis: presentation of an unusual case].
INTRODUCTION: Xanthogranulomatous pyelonephritis is a morphologically and clinically unique manifestation of chronic pyelonephritis with the formation of pus or granulomas. The most frequent predisposing factors for the development of xanthogranulomatous pyelonephritis are urinary obstruction (e. g., stones, tumors, congenital anomalies and functional impairment) and infection of the collecting system. CASE REPORT: We describe a 2-year-old female patient with unclear abdominal complaints, diarrhea, malaise, loss of appetite, weight loss, pale skin color, and recurrent and undulating fever in the presence of known left nephrolithiasis. Based on the clinical examination and imaging, above all, CT, the presumptive diagnosis of xanthogranulomatous pyelonephritis of the left kidney was made. A left lumbar nephrectomy was performed and histology confirmed the diagnosis. CONCLUSION: Xanthogranulomatous pyelonephritis is a relatively rare entity that is associated with obstruction (e. g., stones) and infection of the urinary tract. Its rarity and resultant unfamiliarity often delay diagnosis and therapy, which in turn affect the prognosis. Furthermore, this entity can be mistaken for renal tumors (renal cell carcinoma and Wilms tumor), but nowadays this should be mostly eliminated with the advances in the imaging methods.
Child, Preschool↗
[Retroperitoneal ganglioneuroma].
The ganglioneuroma is a benign neuroblastal tumor. All neuroblastomas and ganglioneuromas derive from immature cells of the sympathetic nerve system. The ganglioneuroma is a very rare disease and effects newborns and infants more often than adolescents and adults. The benign tumors are relatively difficult to diagnose since they usually are asymptomatic. A 5-year old girl with persistent bladder wall thickening and prominent course of the prevesical ureter presents for diagnosis and therapy. Sonography demonstrated a space-occupying lesion of the left kidney. Subsequent MRI raises the suspicion of a retroperitoneal neuroblastoma. The tumor could be removed in two surgical sessions. The ganglioneuroma is a benign tumor with symptoms depending on location and with a relatively good prognosis. Its low incidence and resultant limited experience often leads to delayed diagnosis, potentially determining therapy and diagnosis.
Child, Preschool↗
Mechanisms of everolimus-induced glomerulosclerosis after glomerular injury in the rat.
Despite the lack of nephrotoxicity, adverse effects of the new antiproliferative immunosuppressant everolimus have been reported. By varying time point and dose of everolimus treatment as well as the degree of glomerular injury, the specific conditions and potential mechanisms leading to adverse actions in the anti-Thy1 model have been determined. Only the combination of early and high-dose everolimus treatment (1-3 mg/kg bw) with a severe glomerular lesion ('full-dose' anti-Thy1 model) caused adverse effects with a high mortality rate, progressive apoptosis, crescent formation and glomerulosclerosis. In contrast, either later start or low-dose (0.3 mg/kg bw) therapy or treatment of a less severe lesion ('reduced dose' anti-Thy1 model) appeared to be relatively safe for the glomerular architecture. The adverse effects of everolimus were linked to its marked inhibition of endothelial cell, but not necessarily mesangial cell proliferation. In addition, everolimus markedly inhibited the angiogenic cytokine vascular endothelial growth factor in nephritic glomeruli in vivo. These experimental results suggest special caution regarding the use of everolimus in all situations of severe glomerular cell injury requiring extensive capillary repair, where at least adaption to a low dose needs to be considered.
Aneurysm↗
ACE-inhibition is superior to endothelin A receptor blockade in preventing abnormal capillary supply and fibrosis of the heart in experimental diabetes.
AIMS/HYPOTHESIS: There is little information whether cardiac capillary supply is deranged in diabetes. Hyperglycaemia is a potent stimulus for endothelin-1 (ET-1) production. We therefore hypothesised that increased ET-1 production in Streptozotocin-induced Type 1 diabetes causes abnormalities of cardiac capillaries and the aorta. To this end we compared the effects of an ET receptor A blocker (ETA-RB) with that of an ACE-inhibitor (ACE-i) or their combination in rats with Streptozotocin (STZ) diabetes. METHODS: Sprague Dawley rats were injected with 65 mg STZ i.v. and subsequently developed diabetes. Rats were left untreated or received daily either the ACE-i Trandolapril, the ETA-RB Darusentan or a combination of both. After 6 months the experiment was terminated and the heart and the aorta were investigated using quantitative morphological techniques. RESULTS: ACE-i but not ETA-RB lowered blood pressure in STZ Type 1 diabetic rats. Capillary length density was lower in untreated STZ diabetic rats (2932+/-128 mm/mm3) compared to non-diabetic control rats (3410+/-252 mm/mm3). Treatment with ACE-i (3568+/-431 mm/mm3), but not with ETA-RB (2893+/-192 mm/mm3), prevented the decrease in capillary supply. Volume density of the myocardial interstitium was higher in untreated STZ diabetic rats (0.86+/-0.04%) compared to non-diabetic control rats (0.36+/-0.06%). In all three intervention groups the values were lower (ACE-i: 0.53+/-0.05%, ETA-RB: 0.7+/-0.08% and combination: 0.69+/-0.1). CONCLUSION/INTERPRETATION: Our study identifies a capillary defect of the heart in STZ diabetes, i.e. decreased capillary supply. This abnormality was reversed by ACE-i, but not by ETA-R blockade. A similar trend, although not complete normalisation, was seen in cardiac fibrosis.
Albuminuria↗
In vitro cultured autologous pre-confluent oral keratinocytes for experimental prefabrication of oral mucosa.
The reconstruction of large defects after head and neck cancer resection often requires composite tissue transfer to replace a combination of bone, muscle and mucosa. Thus, tissue engineering techniques may be useful for oral mucosal reconstructive surgery to prefabricate mucosal tissue on the muscle flap in vivo, instead of using conventional skin-bearing composite flaps. The aim of this study was to investigate whether autogenous pre-confluent oral keratinocytes (PCOK) cultured in vitro can create mucosal coverage on muscle in vivo, in a single grafting procedure. In 30 Wistar rats, with a small piece of oral mucosa (2 mm x 5 mm), oral keratinocytes were isolated and then seeded on a hydrophilic PTFE membrane (n = 50) in serum-free culture condition. After 48 h, the membrane, together with the PCOK, was transplanted onto the gracilis muscle to fabricate a mucosal flap in vivo. The wound bed was closed primarily until the time of examination. Biopsies were carried out 1, 2, 3, and 4 weeks, respectively, after transplantation and were evaluated immunohistochemically (AE1/AE3 anti-pancytokeratin, cytokeratin 5/6, collagen IV, laminin, lectin-specific labeling of N-acetylglucosamine oligomeres of endothelial cells) with relation to the following criteria: (1) graft acceptance; (2) inflammatory signs; (3) structural changes and keratinocyte lining; (4) expression of basement membrane components; and (5) vascularization. Ninety-one percent of the grafts showed uniform epithelial layers. The mean number of reconstructed epithelial cell layers was 1.7, 2.0, 1.85 and 2.7 at 1, 2, 3 and 4 weeks, respectively after transplantation (P = 0.342). Collagen IV, laminin and lectin-specific capillaries developed between the neoepithelium and the underlying muscular layer. Only two specimens showed signs of infection 2 weeks after transplantation. In conclusion, this experiment demonstrated that PCOK grafts on muscle in vivo can achieve uniform multi-layered oral epithelial coverage in a short period of time. This technique may be a useful alternative tool for oropharyngeal reconstructive surgery and is also worth considering for further clinical studies.
Acetylglucosamine↗
Comparison of renal morphology in the Streptozotocin and the SHR/N-cp models of diabetes.
The Streptozotocin (STZ) model of diabetes is commonly used for studies of diabetic nephropathy although the histological lesions of the kidney are mild and do not resemble those seen in diabetic patients. The SHR/N-cp rat model of type II diabetes spontaneously develops pronounced abnormalities in renal histology. In the present study, we compared renal morphology in the STZ rat and the diabetic SHR/N-cp rat. Sprague-Dawley rats received STZ, developed diabetes after 2 days and were treated with insulin. In the SHR/N-cp rat, obesity is inherited as an autosomal recessive trait. The progeny are either lean (used as controls) or obese and diabetic. After 6 months of observation, STZ and SHR/N-cp rats were killed. The renal damage was evaluated by assessing damage indices and by using stereological techniques. In addition, immunohistochemistry and electron microscopy were performed. The glomerular and tubulointerstitial changes were much more pronounced in the diabetic SHR/N-cp compared to the STZ model. In parallel glomerular PCNA+cells were significantly more frequent and expression of TGF-beta and PDGF by immunohistochemistry in glomeruli and in the tubulointerstitial space was more pronounced in SHR/N-cp compared to STZ rats. The glomeruli of SHR/N-cp contained less and larger podocytes as well as smaller mesangial cells embedded in more mesangial matrix compared to STZ. Similarly, less, but larger endothelial cells were found in SHR/N-cp than in STZ rats. The mean glomerular volume was similarly increased in the two models. Albumin excretion was only modestly increased in STZ diabetes, but pronounced in the SHR/N-cp rat. Although the STZ model of diabetes exhibits numerous biochemical sequelae of hyperglycemia, the morphological lesions are unimpressive. In contrast, the diabetic SHR/N-cp exhibits marked structural lesions, particularly podocyte damage and mesangial expansion that promise to make it a more suitable model for investigation of diabetic glomerulosclerosis.
Albuminuria↗
Brief adrenomedullin inhalation leads to sustained reduction of pulmonary artery pressure.
The effect of aerosolised adrenomedullin (ADM), a potent vasodilator peptide, on pulmonary artery pressure was studied for 24 h in a surfactant-depleted piglet model. Animals received either aerosolised ADM (50 ng.kg(-1).min(-1), ADM, n=6), or aerosolised normal saline solution (control, n=6). Aerosol therapy was performed for a 2 h treatment period followed by a 22 h observation period. Ventilator settings were adapted to keep arterial oxygen tension and carbon dioxide arterial tension between 13.3-14.6 kPa and 4.9-5.7 kPa, respectively. Aerosolised ADM reduced mean pulmonary artery pressure (MPAP) compared with the control group (end-point median 24 h after therapy start: DeltaMPAP -14.0 versus -8.0 mmHg; 23.5 h after therapy start). After therapy start, mean systemic arterial pressure (MAP) was not significantly different between the groups (end-point median: MAP ADM 70 (61/74) versus control 72 (54/81) mmHg). Endothelin-1, a potent pulmonary vasoconstrictor, is regulated by ADM via cAMP. Twenty two hours after inhalation of aerosolised ADM, endothelin-1 mRNA in lung tissue and endothelin-1 protein expression in pulmonary arteries was reduced compared with controls (median semi-quantitative immunhistochemical score: ADM 0.21, control 0.76). Aerosolised adrenomedullin significantly reduced mean pulmonary artery pressure independently of arterial oxygen tension.
Administration, Inhalation↗
The concept of a clinical round as a virtual, interactive web-based, e-learning model for interdisciplinary teaching.
The demonstration of patient case reports in the course of a clinical round is an essential part of teaching medicine and dentistry. However, suitable live patients with particular problems are not always available at a time when teaching is taking place. This project therefore had the objective of establishing a web-based, virtual e-learning concept for demonstrating case reports independent of time and place, with the possibility of an interactive examination, diagnosis, and interdisciplinary therapy decision making for medical and dental students. Anonymized case reports of diseases in the oral and maxillofacial region and the interdisciplinary treatment were digitized and prepared in a web-based format. The technical aspect was based on connecting flash modules with videos and animation, and monitoring through HTML and Javascript. Due to the modular concept and the programming used, the learning environment was independent of platform and open. Independent formats (.swf, .avi, .mpeg, etc.) were integrated into the individual modules. According to a hierarchic decision system, the user was guided interactively to the diagnosis through a differential diagnostic exclusion process. Sound was digitized and integrated in mp3 compressed form in the 3D models for lip-synchronous speech output. The speech output was connected with a virtual 3D tutor that acted in an advisory capacity in reaching a diagnosis and determining therapy. Further sources of information and literature with abstracts or pdf files of the subject-related publications were inserted to ensure that the teaching was objective. To conclude the virtual clinical round, a check on learning success was conducted in the form of a multimedia multiple choice test.
Computer-Assisted Instruction↗
ACE-inhibitors but not endothelin receptor blockers prevent podocyte loss in early diabetic nephropathy.
AIMS/HYPOTHESIS: It was the aim of our study to investigate the influence of a selective ET-A receptor antagonist LU 135252 alone and in combination with the ACE-inhibitor, trandolapril on podocyte number and morphology in streptozotocin diabetic rats. METHODS: Male Sprague-Dawley rats were injected with 65 mg streptozotocin i.v. and subsequently developed diabetes. Animals were left untreated or received daily either trandolapril (0.3 mg/kg body weight), LU 135252 (50 mg/kg body weight) or a combination of both. After 6 months the experiment was terminated. Glomerular geometry and cellularity were assessed by stereological techniques. Protein expression of TGF-beta, ET-1, PDGF-AB, fibronectin, desmin and alpha-smooth muscle cell actin was investigated by immunohistochemistry. RESULTS: The mean number of podocytes per glomerulus was lower (86+/-17 vs. 138+/-25; p<0.05) and mean podocyte volume was higher in untreated diabetic animals than in non-diabetic controls. Only ACE-i alone and in combination, but not ET(A)-RB alone prevented loss of podocytes and podocyte hypertrophy. In diabetic rats, increased numbers of PCNA positive and p27(kip1) positive cells (mainly podocytes) were reduced by all treatments, but only ACE-i decreased numbers of desmin positive podocytes and tubulointerstitial expression of TGF-beta. Albuminuria was increased in untreated diabetes and was prevented only by ACE-i and combination treatment. CONCLUSION/INTERPRETATION: Podocyte hypertrophy and degeneration is an early event in diabetic nephropathy leading to a loss of podocytes. Treatment with an ACE-i, but not with an ET(A)-RB, prevented the development of albuminuria as well as damage and loss of podocytes. The well known anti-proteinuric effect of ACE-i is presumably due at least in part to conservation of podocyte structure. Increased plasma endothelin-1 (ET-1) concentrations and urine excretion of ET-1 have been documented in patients with diabetes and proteinuria [1]. It has been shown that experimental diabetes mellitus increases renal ET-1 gene transcription [2]. To assess the relevance of the ET-system in the pathogenesis of renal structural changes in the model of the STZ-induced diabetic rat we compared the effect of an ET(A)-receptor specific antagonist with the well known beneficial effect of an ACE-i, especially on podocyte cell number and morphology.
Angiotensin-Converting Enzyme Inhibitors↗
[Renal lesions of paraproteinemias and fibrillary glomerulopathies].
The term "paraproteinemia" or dysproteinemia" refers to a group of diseases caused by specific proteins that very often leads to kidney disease. In these cases a kidney biopsy is often the first diagnostic procedure leading to the diagnosis of a systemic disease. Due to the very variable presentation of the kidney disease in paraproteinemias a diagnosis is often very difficult without specific clinical data. Therefore, we recommend systematic investigation of the kidney biopsy including routine immunohistochemical stains for kappa- and lambda-light chains and electron microscopy in elderly patients with proteinuric kidney disease of unknown origin. In the following we will briefly discuss renal manifestations and clinical symptoms in multiple myeloma with light- or heavy chain deposition (LCDD: light chain deposition disease, HCDD: heavy chain deposition disease), macroglobulinemia Waldenström (Morbus Waldenström), primary amyloidosis or so called monoclonal gammopathy of uncertain dignity (benign monoclonal gammopathy) and fibrillary glomerulopathies.
Amyloid↗