Nephrotic syndrome can be caused by various diseases, from primary kidney diseases to systemic diseases. A kidney biopsy is useful for confirming the causes of nephrotic syndrome and in its management. We herein describe a case of nephrotic syndrome with thrombocytopenia, lymphadenopathy, systemic inflammation, splenomegaly, kidney enlargement, and progressive renal insufficiency. A kidney biopsy showed endothelial swelling with mild interstitial fibrosis and tubular atrophy. This case met the diagnostic criteria for TAFRO syndrome. Little is known about TAFRO syndrome, especially in relation to the associated kidney pathophysiology. The accumulation of a greater number of cases in which the kidney biopsy findings are investigated is needed to clarify the pathogenesis of kidney involvement in this condition.
A 63-year-old man was diagnosed with periodontitis and underwent tooth extraction. Several days later, he suffered a high fever, ischuria, a change in personality, and disorientation. A urologist examined him and found severe hyponatremia (117 mEq/L), and he was then transferred to our hospital. On admission, physical findings revealed dysfunction of the bladder and bowel, altered mental status, and hypovolemia. Blood chemistry showed serum sodium of 120 mEq/L, a serum urate of 1.4 mg/dL, urinary Na of 61 mEq/L, and fractional urate excretion of 16 %. Examination of the cerebrospinal fluid (CSF) showed monocytosis. Magnetic resonance imaging (MRI) of the brain and spinal cord showed multiple lesions characterized by hyperintensity on T2-weighted sequences, suggesting demyelinating disease. His sodium concentration normalized 3 days after volume replacement therapy, and his altered mental status along with the dysfunction of the bladder and bowel were promptly improved after the initiation of high-dose glucocorticoids. Additionally, the abnormal lesions on MRI markedly
A 71-year-old woman with polymyositis presenting with left thigh pain and an intermittent fever was admitted to Osaka Rosai Hospital. We initially diagnosed that her pain and fever were caused by a soft tissue infection because her polymyositis was controlled. She did not respond to various antibiotic therapies. Chest computed tomography demonstrated miliary tuberculosis (TB). Ziehl-Neelsen staining of liver biopsy specimens revealed epithelioid cell granuloma and acid-fast bacilli. Therefore, we finally diagnosed the lesion as TB fasciitis that improved with anti-TB drug therapy. The atypical presentation of TB fasciitis demonstrates the clinical importance of eliminating TB infections in immunocompromised hosts.
A 47-year-old man presented with severe hypokalemic paralysis and respiratory failure. A large amount of potassium was administered along with providing intensive care, and his condition improved. Hypokalemia was attributed to increased urinary potassium excretion. A kidney biopsy was performed to make a definitive histological diagnosis. It revealed acute tubulointerstitial nephritis (TIN). After the diagnosis, prednisolone was administered, and the TIN gradually improved. From the clinical course and laboratory findings, the TIN was presumed to be an autoimmune disorder. Further specific autoantibody tests were positive for anti-mitochondrial antibody (AMA), which has been gaining increasing attention in regard to TIN. In addition, all previous cases of TIN associated with AMA have affected females. The detailed pathogenetic mechanisms are as yet unclear and require further investigation.
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