We treated a 54-year-old woman who was suffering from membranoproliferative glomerulonephritis associated with a complete type of hydatidiform mole. The renal manifestations were proteinuria and hematuria. A renal biopsy, performed before gynecologic management, disclosed focal and segmental subendothelial deposits with a proliferation of the mesangial cell and showed irregularly thickened capillary loops by light and electronmicroscoy. Genralized edema, proteinuria and hematuria were completely recovered by suction and curettage of the hydatidiform mole with prophylactic chemotherapy. The clinical manifestation of earlier presented 3 cases have been the nephrotic syndrome. The common feature of them was a complete remission of the nephropathy after the removal of the hydatidiform mole. The relationship between the hydatidiform mole and glomerulonephritis remains unresolved at present. But we concluded that the hydatidiform mole might be a cause of glomerulonephritis in this case.
Case Report ; Diagnosis, Differential ; Edema/etiology ; Female ; Glomerulonephritis, Membranoproliferative/pathology ; Glomerulonephritis, Membranoproliferative/etiology* ; Hematuria/etiology ; Human ; Hydatidiform Mole/therapy ; Hydatidiform Mole/diagnosis* ; Hydatidiform Mole/complications* ; Middle Age ; Pregnancy ; Proteinuria/etiology ; Uterine Neoplasms/therapy ; Uterine Neoplasms/diagnosis* ; Uterine Neoplasms/complications*

OBJECTIVE: To discuss the pathologic features, treatment and prognosis of the children with isolated proteinuria (IP). METHODS: Twenty-one children with IP were enrolled according to their renal biopsy and were followed up for 0.5 to 10 years. RESULTS: Renal biopsy was performed in all children. Among them 13 were mesangial proliferation glomerulonephritis (MsPGN) (including 3 minor, 6 moderate, and 4 severe ones), 2 minimal change nephritis (MCN), 3 IgA nephropathy (IgAN) (1 in Grade I and 2 in Grade II), 2 focal segmemtal glomerulosclerosis (FSGS) and 1 endocapillary proliferative glomerulonephritis (EnPGN). Interstitial changes could be found in MsPGN and FSGS mostly, presenting interstitial fibrosis, infiltration of inflammatory cells, atrophy of renal tubule, and the vacuolar degeneration of epithelia. All children accepted the medical treatment except the EnPGN case. Fifteen children recovered with no relapse; proteinuria persisted in 3 severe MsPGN and FSGS cases; 2 got the impaired renal function accompanied by persistent proteinuria; and 1 had hypertension. CONCLUSION The different degrees of renal damage can be found in all IP children who have persistent proteinuria. Most patients can get good outcome after aggressive therapies. However, the prognosis of those with severe MsPGN and FSGS was not so optimistic, and some reno-protective treatments should be given to postpone the deterioration of the renal function.
Adolescent ; Biopsy, Needle ; Child ; Child, Preschool ; Female ; Glomerulonephritis, Membranoproliferative ; pathology ; Humans ; Kidney ; pathology ; Male ; Prognosis ; Proteinuria ; etiology ; pathology

OBJECTIVEThe pathogenesis of mesangial proliferative glomerulonephritis (MsPGN) and mechanisms of glucocorticoid (GC) resistance have not been fully identified. Cytotoxic T-lymphocyte antigen-4 (CTLA-4) is an important inhibitor of T-lymphocyte activation. The objective of the study is to investigate the CTLA-4 expression and apoptosis in lymphocytes of children with MsPGN and the effects of dexamethasone (Dex) on the CTLA-4 expression and apoptosis.

METHODSBlood samples were collected from 36 children with MsPGN and 30 healthy children. CTLA-4 expression in in vitro cultured lymphocytes with or without Dex treatment was measured by flow cytometry following direct immune fluorescene. The rate of apoptosis in the lymphocytes was evaluated by annexin V-FITC and propidium iodide staining.

RESULTSThe CTLA-4 expression and apoptosis in lymphocytes from children with MsPGN were significantly lower than those in the healthy control children in the absence or presence of Dex treatment (p<0.05). There was a positive correlation between CTLA-4 expression and apoptosis in lymphocytes (p<0.05).

CONCLUSIONSAbnormal CTLA-4 expression may participate in the pathogenesis of MsPGN and be one of mechanisms of GC resistance.

Antigens, CD ; blood ; Apoptosis ; drug effects ; CTLA-4 Antigen ; Child ; Dexamethasone ; pharmacology ; Female ; Glomerulonephritis, Membranoproliferative ; drug therapy ; etiology ; Humans ; Lymphocytes ; drug effects ; immunology ; Male

The purpose of this study was to investigate the clinical, laboratory, and pathologic characteristics of dense deposit disease (DDD) in Korean children and to determine whether these characteristics differ between Korean and American children with DDD. In 2010, we sent a structured protocol about DDD to pediatric nephrologists throughout Korea. The data collected were compared with previously published data on 14 American children with DDD. Korean children had lower 24-hr urine protein excretion and higher serum albumin levels than American children. The light microscopic findings revealed that a higher percentage of Korean children had membranoproliferative glomerulonephritis patterns (Korean, 77.8%; American, 28.6%, P = 0.036), whereas a higher percentage of American children had crescents (Korean, 0%; American, 78.6%, P < 0.001). The findings from the electron microscopy revealed that Korean children were more likely to have segmental electron dense deposits in the lamina densa of the glomerular basement membrane (Korean, 100%; American, 28.6%, P = 0.002); mesangial deposit was more frequent in American children (Korean, 66.7%; American, 100%, P = 0.047). The histological findings revealed that Korean children with DDD were more likely to show membranoproliferative glomerulonephritis patterns than American children. The degree of proteinuria and hypoalbuminemia was milder in Korean children than American children.
Adolescent ; Asian Continental Ancestry Group ; Child ; Child, Preschool ; Creatinine/blood ; Edema/etiology ; Female ; Glomerulonephritis, Membranoproliferative/*pathology ; Hematuria/etiology ; Humans ; Infant ; Infant, Newborn ; Male ; Microscopy, Electron ; Proteinuria/etiology ; Republic of Korea ; Serum Albumin/analysis ; United States

No abstract available.
Adenocarcinoma/*secondary/surgery ; Chemotherapy, Adjuvant ; Colectomy ; Glomerulonephritis, Membranoproliferative/diagnosis/*etiology/therapy ; Hepatectomy ; Humans ; Liver Neoplasms/*secondary/surgery ; Male ; Middle Aged ; Paraneoplastic Syndromes/diagnosis/*etiology/therapy ; Renal Dialysis ; Renal Insufficiency/etiology ; Sigmoid Neoplasms/*pathology/surgery ; Treatment Outcome