An unusual case of anti-glomerular basement membrane disease presenting with nephrotic syndrome

Anti-glomerular basement membrane (anti-GBM) disease is a vasculitic disease characterized by acute kidney injury, oliguria, hematuria and proteinuria. Proteinuria is rarely in the nephrotic range. A case of anti-GBM disease with proteinuria of 22.5g/day is discussed. Immunofluorescence showed strong linear IgG deposits while electron microscopy showed widespread visceral epithelial cell foot cell process effacement. No electron dense immune complex-type deposits were identified. Pathology findings were not suggestive of simultaneous presentation of anti-GBM disease and other diseases associated with nephrotic range proteinuria. Anti-GBM disease should be considered in a comprehensive differential diagnosis of severe proteinuria.     

An unusual case of nephrotic syndrome and glucosuria

We report a case of a 57-year-old man with hypertension and smoking history who presented with decreased glomerular filtration rate, nephrotic-range proteinuria, and persistent glucosuria. He underwent a kidney biopsy that showed nodular glomerulosclerosis. We discuss the clinicopathologic entities of idiopathic nodular glomerulosclerosis and primary renal glucosuria.     

Coexistent light chain deposition disease,light chain cast nephropathy,and vascular light chain amyloidosis in a patient with IgD lambda multiple myeloma

International Urology and Nephrology -     

Fibronectin glomerulopathy with nephrotic syndrome in a 3-year-old male

Familial non-immune-mediated glomerulopathy has recently been recognized as a distinct clinical entity. The presentation includes proteinuria, often in the nephrotic range, microscopic hematuria, and hypertension. Renal function may remain intact long term, or may progress slowly to renal failure. A 3-year-old boy was referred with proteinuria (>8 g/day), microscopic hematuria, and hypertension (184/150 mmHg). Renal function was intact. Diagnostic evaluation uncovered no evidence of systemic disease. A renal biopsy specimen showed no immune deposits in the glomeruli, but fibronectin deposits were detected in the peripheral loop and mesangium by immunofluorescence. The basement membrane was intact. Twelve other family members subsequently were found to have some renal pathology. Renal function was preserved during 7 years of follow-up. The pathogenesis of fibronectin glomerulopathy is discussed. Received: 06 April 2001 / Revised: 13 December 2001 / Accepted: 13 December 2001     

An unusual cause of nephrotic syndrome and hypertension in a young woman.

Introduction The most common causes of adult nephrotic syndrome in our countryare focal glomerulosclerosis, membranous nephropathy and minimal-changedisease [1]. There are, however, less common causes to be considered[2–8]. We describe a 15-year-old Spanish woman with nephrotic syndromesecondary to pre-eclamptic nephropathy in association with atotal hydatidiform mole, in whom pregnancy was not initiallyconsidered. Case A 15-year-old Spanish woman was admitted to our hospital becauseof a 4-week history of asthenia, anorexia, and progressive oedemaof her face, hands, and lower extremities. Two weeks beforeadmission she had developed acute back pain without urinaryor digestive symptoms. After an unsuccessful trial of non-steroidalanti-inflammatory drugs, she was brought to our hospital. Atadmission she had vaginal bleeding, which was interpreted asnormal menstruation, starting a few     

An unusual case of female hypospadias associated with a pelvic mass

Female hypospadias is a rare malformation. We report on an unusual case that presented with a urethral dimple in the perineum in association with a pelvic soft tissue sarcoma compressing and displacing the urethra. The diagnosis was made by cystoscopy, and, as the patients remained asymptomatic, no treatment was eventually required for her urologic malformation. This is, to our knowledge, the first reported case of female hypospadias associated with a perineal urethral dimple. The possible pathogenesis of the malformation is discussed also in relation to the simultaneous presence of a pelvic mass.     

Anti-VEGF-related thrombotic microangiopathy in a child presenting with nephrotic syndrome

Background

Targeting the vascular endothelial growth factor (VEGF) signaling pathway has become an important approach to current cancer therapy. Anti-VEGF therapy-related renal adverse effects may present as hypertension, non-nephrotic proteinuria, and rarely as nephrotic syndrome (NS) and acute kidney injury.

Case-Diagnosis/Treatment

In this report, we present a 15-year-old boy who had developed nephrotic syndrome and thrombotic microangiopathy 26 months after administration of anti-VEGF therapy. Treatment was discontinued and nephrotic syndrome remitted spontaneously within 3 months.

Conclusions

Nephrologists should be aware of the side effects of anti-VEGF therapy. Early diagnosis and prompt management with withdrawal of the agents will result in spontaneous remission.

     

A case of necrotizing glomerulonephritis presenting with nephrotic syndrome associated with pulmonary cryptococcosis

We describe a 68-year-old man with necrotizing glomerulonephritis who presented with nephrotic syndrome accompanied by pulmonary cryptococcosis. He developed rheumatoid arthritis in July 1999 and was treated with low-dose prednisolone. He was admitted to our hospital on November 22 following the appearance of bilateral leg edema in October 2000. Laboratory tests at presentation revealed nephrotic syndrome with renal impairment. Renal biopsy specimens revealed necrotizing glomerulonephritis with crescent, but immunofluorescence study showed lack of staining for immunoglobulins or complement components. Chest X-ray and CT showed abnormal shadows in the right upper lung field, and Cryptococcus neoformans was isolated in a transbronchial lung biopsy. After the diagnosis of pulmonary cryptococcosis was made, the patient was treated with 200mg/day fluconazole. The pulmonary abnormal shadows immediately improved and urinary protein excretion dramatically decreased. A second renal biopsy, performed about 2 months after the first biopsy, showed disappearance of crescent. Electron microscopic examination of the second renal biopsy showed partial effacement of foot processes without electron-dense deposits. Our findings suggest that necrotizing glomerulonephritis with nephrotic syndrome in this patient represented pauci-immune T-cell-mediated injury related to pulmonary cryptococcosis.     

Metachronous development of nonamyloidogenic [lambda] light chain deposition disease and IgG heavy chain amyloidosis in the same patient

We report a highly unusual case of monoclonal immunoglobulin deposition disease-associated nephrotic syndrome in which a patient developed both lambda light chain deposition disease and 6 years afterward IgG-heavy chain amyloidosis. The patient initially underwent autologous peripheral blood stem cell transplantation as treatment of the underlying plasma cell dyscrasia causing the light chain deposition disease-related nephrotic syndrome. After 6 years of clinical remission, recurrence of the nephrotic syndrome led to a renal biopsy demonstrating IgG-heavy chain amyloidosis. Interestingly, much of the characteristic nodular glomerular sclerosis seen in light chain deposition disease regressed between the time of the first biopsy and the second. Given the length of time between the development of the two diseases and the apparent success of stem cell transplantation in treating the first, we think that the patient produced two distinctly different abnormal plasma cell clones. To our knowledge, this is the first report of two different monoclonal immunoglobulin deposition diseases occurring in the same patient.     

An unusual case of complete Carney's triad in a 14-year-old boy

Carney's triad represents the association of gastric gastrointestinal stromal tumor, pulmonary chondroma, and extraadrenal paraganglioma. Only 79 cases of this rare condition have been described. Here, the authors describe the unusual case of a 14-year-old boy who presented with a complete Carney's triad. This is only the second reported case in the world literature of a patient manifesting a complete Carney's triad at presentation. The management of each tumor is discussed. J Pediatr Surg 37:1228-1231.     

Incarcerated transmesosigmoid hernia presenting in a 28-year-old female

We report the case of a transmesosigmoid hernia in a 28-year-old female who presented to the emergency room complaining of abdominal pain. History, clinical and radiographic examination revealed small bowel ileus, but without being able to diagnose the cause. We decided to perform laparoscopy, which showed herniation of a small intestine loop through a small defect of the mesosigmoid. Due to the inability to reduce the internal hernia laparoscopically, we converted to mini-laparotomy; we reduced the hernia and performed segmental small bowel resection and anastomosis. It is essential to suspect the presence of an internal hernia as soon as possible, because if the operation is delayed, it will often eventually lead to bowel necrosis.