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1.
Systemic amyloidosis can become clinically apparent as a multisystem disease due to renal symptoms, which are important for differential diagnostics of the nephrotic syndrome. Systemic amyloidosis can lead to irreversible renal damage. The aim of the treatment of amyloidosis is to quench the source of precursor amyloid stages by combating the underlying disease. The use of substances which inhibit the deposition of amyloid or accelerate disintegration is still in the test phase. For rare forms of hereditary amyloidosis which involve fibrillogen mutations of various protein coding genes and are decisive for hepatic synthesis of precursor proteins, a liver transplant is a therapeutic option.  相似文献   

2.
A pheochromocytoma is a rare differential diagnosis of a hypertensive crisis. This article describes the case of a patient who presented with recurrent episodes of nausea, vomiting, paleness of the skin and paroxysmal high blood pressure peaks and the most recent attack also included orthopnea with lung edema and chest pain. Pheochromocytomas arise from the chromaffin cells of the adrenal medulla and are the cause of 0.2% of arterial hypotension diseases: 85% are located in the adrenal medulla but they also occur in the thoracic and abdominal sympathetic trunk. Typical symptoms are paroxysmal or prolonged high blood pressure, headache, sweating, tachycardia and paleness of the skin. The initially diagnostic procedure includes a 24 h urine collection and testing, catecholamine testing in plasma and long term measurement of blood pressure. Localization and the search for metastases (10% of pheochromocytomas are malignant) are carried out by sonography, computed tomography or magnetic resonance tomography. Confirmation of extra-adrenal manifestations is performed by MIBG (123I-metaiodobenzylguanidine) scintigraphy. Associated syndromes, such as multiple endocrine neoplasia type 2 (MEN 2) and von Hippel-Lindau syndrome, must be excluded. The definitive treatment is surgery, preceded by application of an α-adrenoreceptor as mandatory preparation, followed by a β-adrenoreceptor blockade, if necessary.  相似文献   

3.
We report a case of a patient with thrombocytopenia. A sporadic MYH9-associated disease, May Hegglin anomaly, was identified by giant platelets, leucocyte inclusion bodies and the typical distribution of NMMHC-IIA in granulocytes in the absence of impaired renal function, cataract and hearing loss. MYH9-associated diseases are an underestimated differential diagnosis of idiopathic thrombocytopenia. The correct diagnosis is important to prevent unnecessary treatment of a patient with thrombocytopenia and to provide sufficient patient information and genetic counseling. Therefore, careful examination of the blood smear has to be the first diagnostic step in a case of unexplained thrombocytopenia.  相似文献   

4.

Background

Cholesterol embolization syndrome is an underestimated complication after catheter interventions of the main arterial vessels. Patients show manifestations, such as renal failure, cyanosis of the feet and other skin symptoms, diarrhoea and others, 8-10 weeks after the intervention.

Case report

Here we report the case of an 85-year-old patient with acute renal failure 8 weeks after percutaneous coronary angioplasty. Biopsy of the kidneys revealed a cholesterol embolism.

Conclusion

Acute renal failure can occur after cholesterol embolism which has a poor prognosis and due to the lack of causal therapy can only be treated symptomatically. Therefore, the indications for catheter intervention must be evaluated carefully.  相似文献   

5.
The case of a patient with renal vein thrombosis manifesting in nonspecific symptoms such as side pain, nausea and hematuria is presented. A definitive diagnosis was established using computed tomography. Concomitant nephrotic syndrome, a common accompanying disease of renal vein thrombosis, was caused by glomerulonephritis in our patient. This could be clarified, after 8 months of coagulation therapy for the thrombosis, by means of renal biopsy and was treated with ciclosporine. When treated appropriately (anticoagulation therapy) renal vein thrombosis with nephrotic syndrome does not carry an increased risk of mortality.  相似文献   

6.
7.
This case example of a 74-year-old patient with limited kidney function in stage 3 according to KDOQI (Kidney Disease Outcomes Quality Initiative) and proteinuria admitted for in-patient renal biopsy, describes the anamnesis, the clinical findings and the primary diagnostics. In the subsequent diagnostics the kidney biopsy supplied the first evidence of the presence of infiltration of the kidneys by a low malignancy non-Hodgkin B-cell lymphoma which was confirmed by iliac crest biopsy, computed tomography and hematological tests.  相似文献   

8.
9.
A patient presented with pathologically high serum creatinine level and unplanned weight loss. Chemical analysis showed hypercalcemia, tubular proteinuria, and raised concentrations of calcitriol and angiotensin converting enzyme; computed tomography showed thoracic and abdominal lymphadenopathy and increased reticulonodular signs. Once a kidney biopsy demonstrated granulomatous interstitial nephritis and excluded other granulomatous (mycobacterial) disorders, the diagnosis could be made of sarcoidosis with pulmonary, abdominal, and renal involvement. Correct diagnosis of this disorder is difficult, especially if there is no or clinically inapparent pulmonary involvement. Sarcoidosis responds very well to corticoids such as prednisolone, the long-term administration of which is required due to the probability of relapse.  相似文献   

10.
Zusammenfassung Epileptische Anf?lle geh?ren zu den h?ufigsten neurologischen Notf?llen im Kindes- und Jugendalter. Nach erstmaligem Auftreten eines Krampfanfalls ist eine rasche Kl?rung der zugrundeliegenden Ursache in Hinblick auf eine m?glichst frühe zielgerichtete Behandlung von gro?er Bedeutung. Als m?gliche Ursachen kommen Raumforderungen, Blutungen oder Traumen, infekti?se Erkrankungen des Zentralnervensystems, Stoffwechselerkrankungen, Elektrolytentgleisungen, Drogen- oder Alkoholabusus sowie Schlafentzug in Frage. In nur etwa 1% sind die Anf?lle Ausdruck einer Epilepsie.    Wir berichten den Fall einer bis dato immer gesund gewesenen 17-j?hrigen, die wegen eines erstmals aufgetretenen, generalisierten tonisch-klonischen Anfalls an unserer Abteilung aufgenommen wurde. In der Anamnese fanden sich seit zwei Tagen bestehende starke Kopfschmerzen, die erfolgreich analgetisch behandelt wurden. Im klinisch physikalischen Status war eine Adipositas auff?llig, im neurologischen Status, im Labor sowie in der craniellen Computertomographie zeigten sich keine pathologischen Befunde, sodass die Patientin prim?r unter der Verdachtsdiagnose einer idiopathischen Epilepsie mit Antiepileptika behandelt wurde. Unter Therapie kam es zu einer Gesichtsfeldeinschr?nkung und schlie?lich zu weiteren generalisierten Anf?llen. Eine erweiterte Diagnostik mit Lumbalpunktion, Bestimmung der Schilddrüsenhormone und einem Drogenscreening blieb ebenfalls ohne pathologischen Befund.    Schlie?lich lieferte eine gezielte Befragung und die Harnanalyse den entscheidenden Hinweis für die Ursache des Anfallgeschehens, welches nun doch als Symptom einer schweren Grunderkrankung zu werten war. Eingegangen: 20. Dezember 2000 Akzeptiert: 2. Januar 2001  相似文献   

11.
12.
We report on a male patient suffering from loss of weight, fatigue, fever, eosinophilia, and hyperthyreoidism. The echocardiogram revealed a left atrial mass originating from the posterior mitral leaflet. In combination with the constitutional symptoms a left atrial myxoma was diagnosed. The tumor was surgically removed. Postoperatively therapy with corticosteroids and thiamazole was stopped. During follow-up, eosinophilia and hyperthyreodism could no longer be detected.  相似文献   

13.
A 70-year-old man presented with subacute dyspnea, cough, weight loss, and mild fever. Blood analysis revealed an elevated C-reactive protein level. Chest x-ray and CT of the chest showed alveolar opacities with a migratory tendency during the clinical course. After extensive diagnostics, treatment with prednisolone under the presumed diagnosis of a cryptogenic organizing pneumonia was started, which lead to a rapid clinical response.  相似文献   

14.
15.
A 43-year old patient came to our clinic with chronic diarrhea. Determination of the faecal alpha 1-antitrypsin-clearance led to the diagnosis of exsudative enteropathy. Blood counts showed pronounced lymphocytopenia. Histopathological findings from intestinal and colorectal biopsies showed diffuse lymphangiectasis. Following exclusion of secondary types, our diagnosis was primary intestinal lymphangiectasis. Additional distinctive morphological and anamnestic features strongly suggested presence of Noonan's syndrome. Characteristic manifestations of Noonan's syndrome include changes in the lymphatic vessels in accordance with primary lymphangiectasis. Frequently, these changes are localized in the lungs. To date, only rare cases of intestinal lymphangiectasia in Noonan's syndrome have been reported. Treatment consisted of a protein-rich diet, with reduced fat content enriched by middle-chain fatty acids, as well as twice-daily injections of 200 micrograms octreotide. With this therapy, the symptoms improved.  相似文献   

16.
A 79-year-old patient presented with weight loss, subfebrile body temperature and unclear jaw pain. After ruling out malignant and infectious causes, positron emission tomography-computed tomography (PET-CT) revealed markedly elevated glucose utilization of the large thoracic and upper limb arteries, suggesting systemic vasculitis. Color-coded duplex sonography confirmed thickening of the wall of the external carotid artery consistent with vasculitis. The patient was diagnosed with giant cell arteritis involving the large thoracic arteries and the upper limb arteries but without involvement of the superficial temporal artery. Based on the involvement of the external carotid artery, the jaw pain could be classified as jaw claudication. Clinical and laboratory remission was achieved with systemic glucocorticoids which could subsequently be tapered. The patient is well and asymptomatic 12 months after diagnosis and 2 months without steroids.  相似文献   

17.
Cerebral hyperperfusion following carotid artery stenting represents a rare neurological complication. It is characterized by ipsilateral headache, focal neurological symptoms, seizures and intracerebral oedema or haemorrhaging. Restriction of the intracerebral perfusion caused by high grade ipsilateral and/or contralateral carotid stenosis, previous contralateral carotid revascularization and preinterventional, peri-interventional and postinterventional hypertension are regarded to be risk factors. Cranial computed tomography (CT) and diffusion-weighted magnetic resonance imaging (MRI) might show normal findings for hyperperfusion syndrome. The most important aspects of prophylaxis and treatment are controlling the hypertension in a specialized intensive care unit.  相似文献   

18.
Zusammenfassung Ein 55j?hriger Patient wird wegen postprandialer Oberbauchbeschwerden internistisch untersucht, urs?chlich findet sich eine chronische B-Gastritis mit einem Ulcus duodeni, ferner besteht ein Gallensteinleiden. Als Nebenbefund wird sonografisch eine solide Raumforderung der Milz erfa?t, deren Genese erst histologisch am Splenektomiepr?parat gekl?rt werden konnte: Splenome sind au?ergew?hnlich seltene Hamartome der Milz, die intra vitam in der Regel als Zufallsbefund erfa?t werden. Es existiert daher auch keine wegweisende klinische Symptomatik; laborchemische Befunde sind unspezifisch. Zur weiteren diagnostischen Zuordnung ist die Kernspintomografie hilfreich; beweisend ist jedoch erst die histologische Untersuchung. Das Splenom ist neben H?mangiom, prim?ren Lymphom und Teratom eine seltene Differentialdiagnose der soliden Raumforderung der Milz.  相似文献   

19.
Treatment with tumor necrosis factor alpha (TNF-α) inhibitors is a well-established therapeutic strategy for various autoimmune diseases. However, little is known about renal complications and possible causality of renal injury due to this treatment. The following case of a patient with psoriasis demonstrates the difficulties in classifying renal complications of anti-TNF-α therapy versus kidney involvement caused by the underlying disease.  相似文献   

20.
A 41-year-old physically active man with no significant past medical history presented with sudden thoracic pain. The patient was referred to the next tertiary care hospital. A CT scan showed an ectasia of the ascending aorta with irregularities of the aortic wall without dissection. Despite initial refusal, the patient was referred to a university hospital with experience in aortic surgery. A triphase ECG-synchronized cardiothoracic flash protocol performed on a 256 line CT scanner confirmed an aortic intramural hematoma and a covered aortic perforation. Shortly afterwards the patient collapsed and had to be resuscitated.  相似文献   

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