Clinical Relevance of C4d Deposition in Pediatric Immunoglobulin A Nephropathy.

INTRODUCTION: Few studies have reported the association between the complement cascade and pediatric immunoglobulin A nephropathy (IgAN). This study aimed to investigate the association between C4d staining positivity and the clinical/histopathological characteristics of pediatric patients with IgAN. METHODS: Children diagnosed with IgAN through renal biopsy were retrospectively reviewed. Renal biopsy specimens were stained using C4d immunohistochemistry. RESULTS: Among the 56 patients, 31 (55.4%) showed positive mesangial or peripheral capillary C4d staining in glomeruli. Urine protein-to-creatinine ratio was significantly higher in C4d-positive patients (p = 0.001). The severity of mesangial proliferation according to the Haas and Oxford classification was positively associated with positive C4d staining (p < 0.001). CONCLUSION: Positive C4d staining was found to be significantly associated with the clinical/histopathological severity of IgAN.

Clinical manifestations of IgA nephropathy combined with thin glomerular basement membrane nephropathy in children.

BACKGROUND: Immunoglobulin A nephropathy (IgAN) and thin glomerular basement membrane nephropathy (TBMN) are the most common causes of persistent hematuria during childhood. The objective of this study is to determine the difference in clinical features and laboratory findings between pediatric patients with IgA deposited TBMN and IgAN alone. METHODS: Between January 2000 and March 2009, 95 children were diagnosed with IgAN by renal biopsy. Clinical features and laboratory findings of patients with isolated IgAN and with IgAN plus TBMN were compared; the children diagnosed with IgAN were compared to 127 children who had been diagnosed with TBMN alone during the same period. RESULTS: There were 71 (74.7%) of a total 95 patients that were diagnosed with isolated IgAN (Group1); in 24 (25.3%) of the 95 patients IgAN was combined with TBMN (Group 2). There was marked difference in the gender distribution between Group 2 and isolated TBMN patients. The degree of proteinuria and pathologic severity was higher in Group 1 compared with Group 2. Gross hematuria was present in both groups. There were no distinguishing features in the other laboratory parameters. CONCLUSION: Patients with both IgAN and TBMN seem to have similar clinical features to patients with isolated IgAN; however, the latter tend to have better pathologic and laboratory findings, compared to the patients with IgAN alone.

A Case of Primary Pulmonary Hypertension Associated with Pregnancy

Primary pulmonary hypertension is a rar, incurable, and progressive clinical entity. When associated with pregnancy, the prognosis of primary pulmonary hypertension is worsened with maternal mortality rates of at least 50%. The patient was a 29-year-old mother in her 2nd pregnancy, with previous uncomplicated gestation, 5 years ago. She had been well until the 28th week of present gestation when she was admitted because of increasing dyspnea on exertion. She was a housewife with no remarkable family and past histories. We have experienced a patient with primary pulmonary hypertension in pregnancy. She was treated with an oral calcium-channel blocker and low-molecular-weight heparin and was delivered vaginally with good maternal and fetal outcome. So we report a case of primary pulmonary hypertension associated with pregnancy and review literature.