Nephrotic syndrome associated with Kimura's disease

Kimura's disease consists of lesions that appear as single or multiple red-brown papules or as subcutaneous nodules with a predilection for the head and neck region. Although it principally affects the skin and soft tissues, there is a high prevalence of associated renal disease. We report a case of nephrotic syndrome associated with Kimura's disease. Our patient is distinctive in that his disease first manifested while residing in the Western hemisphere; the renal disease was characterized as mesangial proliferative glomerulonephritis with renal impairment, and his nephrotic syndrome remitted with standard doses of prednisone.     

Steroid-resistant nephrotic syndrome associated with Kimura's disease

Kimura's disease is a chronic inflammatory disease characterized by tumor-like lesions in the soft tissue and lymph nodes of head and neck area or parotid gland. It has a high frequency of an association with nephrotic syndrome. Reported cases of nephrotic syndrome and Kimura's disease were mostly from adult patients with only 5 children mentioned. This study reports the case of a 15-year-old-boy who manifested with steroid-resistant nephrotic syndrome for 4 years. Pathological examination of the kidney revealed mild mesangial proliferation. Subsequently, he developed a soft-tissue mass in the parotid gland area. Histopathological investigation of the mass revealed eosinophilic infiltration together with plasma cells and mast cells which is a main characteristic of Kimura's disease. The patient, however, continued to have nephrotic-range proteinuria after removing the subcutaneous mass.     

Minimal change glomerulonephritis associated with hydatid disease

A 63-year-old man presented to our department with dyspnea and peripheral edema. A cystic mass in the right upper abdomen, consistent with echinococcal disease was discovered. Proteinuria was also present, and a nephrotic syndrome was diagnosed. The kidney biopsy revealed minimal change glomerulonephritis. Treatment with the antiechinococcal drug albendazole induced complete remission of the nephrotic syndrome, suggesting an etiopathogenic role for a hydatid antigen in the development of an immune-mediated glomerulonephritis.     

Kimura's disease associated with minimal change nephrotic syndrome: A case report

Kimura's disease is a rare disorder that involves regional lymph nodes and the major salivary glands, which become infiltrated by eosinophils and lymphocytes. Renal lesions associated with Kimura's disease are rare. We describe the case of a 60-year-old Japanese woman who first noted a nodular mass in a salivary gland. As the nodule grew, nephrotic syndrome and heart failure developed. A biopsy of the nodule revealed Kimura's disease, and surgical excision was performed. After the operation, the heart failure and nephrotic syndrome, which were diagnosed as minimal change disease on renal biopsy, improved rapidly without steroid therapy. Four months later, the nephrotic syndrome recurred without recurrence of Kimura's disease. The patient showed marked improvement during prednisolone therapy (40 mg/d) and was in complete remission 4 weeks after the initiation of steroid therapy. This case shows that surgical excision and prednisolone therapy are useful for nephrotic syndrome associated with Kimura's disease.     

Celiac disease associated with immune complex glomerulonephritis.

A patient with immune complex glomerulonephritis and celiac disease without dermatitis herpetiformis or other underlying disease associated with glomerulonephritis is presented. Antibodies to wheat proteins were found in serum and withdrawal of gluten from the diet resulted in disappearance of immune complexes from serum and resolution of both renal and intestinal disease, suggesting a dietary source of antigen. Despite extensive immunopathologic studies of the renal biopsy, neither dietary nor endogenous brush border antigens were demonstrated in glomeruli.     

Minimal-change nephrotic syndrome associated with subcutaneous eosinophilic lymphoid granuloma (Kimura's disease)

A 29-year-old Japanese male with a 19-year history of subcutaneous eosinophilic lymphoid granuloma (Kimura's disease) was referred to the Nephrology Service of the Nihon University Hospital for evaluation of edema and massive proteinuria. The renal biopsy disclosed minimal glomerular lesions. In this paper a case of nephrotic syndrome associated with eosinophilic lymphoid granuloma is reported.     

End-stage renal disease from glomerulonephritis associated with anti-phospholipid syndrome

Primary anti-phospholipid syndrome (APS) is perceived to be an uncommon disorder, infrequently recognized as a cause of renal disease in childhood. While renal involvement in APS classically manifests as thrombotic events, other renal diseases associated with APS have been reported in adults, including membranous nephropathy and minimal change disease. We report our experience of caring for a child who presented with acute anuric renal failure due to anti-neutrophilic cytoplasmic antibody-negative rapidly progressive glomerulonephritis (RPGN), with concomitant thrombotic microangiopathy (TMA). Recognition of the APS as a cause of the patients TMA facilitated institution of anticoagulation. Our patients renal failure did not improve and the patient remained dependent on dialysis until he was successfully transplanted. The purpose of our report is to make health-care professionals aware of the previously unreported association of pauci-immune RPGN and APS in children; early recognition of APS will allow initiation of anticoagulation to prevent recurrent thromboses and enable successful transplantation.     

Lymphadenopathy of Kimura's disease

Kimura's disease is an important category of reactive lymphadenopathy in the Oriental population. The enlarged nodes are mostly located in the head and neck region. Salient pathological changes include florid germinal centers, Warthin-Finkeldey type polykaryocytes, vascularization of germinal centers, increased postcapillary venules in the paracortex, eosinophilic infiltration, and sclerosis. The pathology of Kimura's disease is quite different from that of angiolymphoid hyperplasia with eosinophilia (epithelioid hemangioma). Immunoperoxidase studies show IgE reticular networks in germinal centers. Nondegranulated surface IgE-positive mast cells are present in the paracortex. The authors propose that Kimura's disease represents an aberrant immune reaction to an as yet unknown stimulus. Although the individual histological features are nonspecific, the constellation of features is highly characteristic of Kimura's disease. Since lymphadenopathy can herald involvement of other tissues and the prognosis is excellent, accurate diagnosis of this disease in lymph node biopsies may spare the patients unnecessary radical surgery.     

Membranoproliferative glomerulonephritis associated with sickle cell disease in two siblings

The cases of two brothers with sickle-cell anemia complicated by the nephrotic syndrome and membranoproliferative glomerulonephritis are presented. The literature related to this infrequent association is reviewed and possible explanations for the occurrence of the latter in two brothers are discussed.     

Kimura's disease and membranous nephropathy

An interesting association of Kimura's disease and membranous nephropathy is reported in a 71-year-old Chinese patient, 40 years after emigrating to the UK from Hong Kong. Significant blood eosinophilia and a very high serum IgE level were detected, the latter with a moderate level of specificity to Candida albicans. Light microscopy of renal biopsy was unremarkable despite a proteinuria of nephrotic proportions; diffuse subepithelial dense deposits compatible with membranous nephropathy were identified on electron microscopy. The atopic nature of Kimura's disease is confirmed and C. albicans is suggested as a possible causative agent.     

Nephroblastoma associated with mesangioproliferative glomerulonephritis

The case of a girl showing left-sided Wilms tumor together with a nephrotic syndrome is presented. Recovery after tumor nephrectomy was uneventful. After cessation of cytostatic chemotherapy chronic renal failure developed. Open wedge biopsy of the right kidney revealed endstage glomerular disease. Renal histology in patients with this rare combination and in those presenting with male pseudohermaphroditism and nephron disorders shows a specific pattern. It is suggested that there is a genuine type of nephropathy linked either to Wilms' tumor and/or to male pseudohermaphroditism.     

Cryptococcosis associated with crescentic glomerulonephritis

Crescentic glomerulonephritis (CGN) is an uncommon form of renal injury in childhood. Whereas many infectious processes are known to be linked to CGN, fungal infections typically are not. This report describes an 11-year-old girl who presented with CGN, cutaneous anergy, and cryptococcal mediastinitis. Whereas cryptococcal disease in children is usually associated with immunodeficiency (inherited or acquired), extensive immunologic evaluation of the patient was notable only for relative CD4 lymphopenia with normal CD4/CD8 ratios. Testing for human immunodeficiency virus was negative. Clinical and diagnostic studies are presented, along with a review of the literature regarding glomerular disease and cryptococcal infections.