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Misdiagnosed as primary amyloidosis with nephrotic syndrome report of 2 cases

To provide adequate primary nephrotic syndrome, and anticoagulation symptomatic hormone therapy failed to alleviate the condition. Physical examination: BP: 125/75mmHg, nutrient medium, the body does not touch the superficial lymph nodes, heart and lung (a), liver and spleen no time,[link widoczny dla zalogowanych], limb muscle tone normal, moderate pitting edema of both lower extremities, neurological examination (I). Laboratory examinations: urine protein 3 (+), 24-hour urine protein 6.8g / L, serum albumin 22.9g / L, total cholesterol 6.39mmol / L, glycerol - Jla3 .10 mmol / L, renal B ultrasound showed slightly increased renal and abdominal ultrasound showed B, no obvious abnormalities, ECG showed roughly normal. Renal biopsy examination revealed: primary amyloidosis (diffuse mesangial proliferation, mesangial visible clumps of pink-like substance deposition,[link widoczny dla zalogowanych], Congo red staining was brick red, with 5% potassium permanganate oxidation continued to show positive) , angiotensin-converting enzyme inhibitor-line applications, promoting blood circulation and diuresis swelling and other symptomatic treatment, her condition improved and was discharged. Case 2: male patients, aged 47 to foam in urine increased with repeated edema hospitalized in June. Physical examination: blood pressure, 120/80mmHg, nutrition in general, does not touch the body of superficial lymph nodes, no abnormal heart and lung, liver and spleen no time, ascites (a), lower limb muscle tone normal, was moderate pitting edema, neurological examination (a). Laboratory examinations: urinary protein 4 +, 24-hour urinary protein 10.6g / L, serum albumin 23.8g / L, total cholesterol 6.70mmoL / L, triglyceride 2.66mmoL / L, renal and abdominal B ultrasound showed no obvious abnormalities, chest X-ray and electrocardiogram showed normal. Diagnosis of nephrotic syndrome,[link widoczny dla zalogowanych], and later confirmed by renal biopsy with primary amyloidosis (diffuse mild mesangial proliferation, mesangial visible clumps of pink-like substance deposition, Congo red staining was brick red, the 5% potassium permanganate oxidation continued to show positive). Line application of angiotensin Ⅱ receptor antagonist, promoting blood circulation and diuresis swelling and other symptomatic treatment, her condition improved and was discharged. Author: 237,006 Luan Department of Nephrology, Hospital of discussion 1. Misdiagnosis of clinical manifestations of amyloidosis, the lack of holding the opposite sex, the clinical misdiagnosis rate. Because of its high rate of misdiagnosis: The main is lack of awareness of amyloidosis, clinical manifestations of amyloidosis complicated, a few cases only a single organ infiltration. 2 patients in this group only the performance of the clinical syndrome, not yet found the performance of other organ system involvement,[link widoczny dla zalogowanych], and eventually was able to clear by renal biopsy; In addition, the First Hospital of Beijing Medical University, according to reports confirmed by biopsy of renal amyloidosis patients accounted for the performance of nephrotic syndrome was 57% L3], therefore, if not renal biopsy examination in patients with clinically easily be misdiagnosed. 2. Measures to avoid misdiagnosis due to the site of amyloid deposition, in varying degrees, individual differences are significant, where the following conditions should take into account the existence of this disease may be: ① without kidney disease based on a large number of proteinuria, nephrotic syndrome, standards compliance , for further identification of primary and secondary nephrotic syndrome, steroid therapy can not achieve the desired results, especially one should pay attention to other systems or to find evidence of organ damage; ② unexplained swollen tongue, limited mobility. ③ difficult to explain the intractable diarrhea; ④ unexplained gastrointestinal bleeding; ⑤ unexplained hepatomegaly, and mild liver dysfunction, cholestasis can occur (rare); ⑥ unexplained heart failure, arrhythmia echocardiography prompted septal thickening, pericardial effusion; ⑦ bilateral pleural effusion of unknown diagnosis; ⑧ unexplained numbness, reduced muscle strength, EMG revealed neurogenic damage; ⑨ unexplained fatigue, weight loss, lower extremity edema. Difficulties encountered in clinical diagnosis, try to use the Clinical diagnosis of amyloidosis is the key to first think of the disease, and biopsy of affected organs Congo red staining to confirm the diagnosis.
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