Comparison of pulse and oral steroid in childhood membranoproliferative glomerulonephritis.

BACKGROUND: There has been no controlled study comparing efficacy of pulse versus oral steroid therapy in childhood membranoproliferative glomerulonephritis (MPGN). This study aimed to compare these therapies and renal outcome over a long-term period for MPGN. METHODS: Outcome measures in 11 patients with MPGN treated with pulse methylprednisolone (MP) were compared with 8 patients with MPGN treated with oral prednisolone (P). RESULTS: Nineteen children with idiopathic MPGN (mean age 9.75 years, range 3.7-14 years) were followed for a mean period of 68.21 months (range 4-124 months). Both treatment groups were similar in demographic, clinical, laboratory and histopathological characteristics on presentation. In the pulse MP group, only 1 patient out of 11 progressed to end-stage renal failure (ESRF), compared with 4 patients out of 8 in the oral P group (p=0.041). For long-term renal survival, those patients with more than 8 years of follow-up were further evaluated. Twelve patients had completed 8 years of follow-up; in the pulse MP group, 1 of 7 patients, compared with 4 of 5 patients in the oral P group progressed to ESRF (p=0.039). Chronic damage in the presentation biopsy and lack of remission in patients with nephrotic syndrome (NS) were positively associated with adverse renal outcome (p=0.02, p=0.006, respectively). CONCLUSIONS: Pulse MP therapy may be superior to oral P therapy in children with MPGN in preserving renal function without any increase in steroid-related side effects. Chronic damage in the presentation biopsy and lack of remission of NS are adverse features.

An atypical case of POEMS syndrome with IgG kappa M protein and end stage renal failure.

POEMS syndrome is a rare plasma cell dyscrasia which is characterized by small amounts of monoclonal protein, and a multisystem complex manifested by various combinations of polyneuropathy, organomegaly, endocrinopathy and skin changes. Here, we presented an atypical case of POEMS syndrome with IgG kappa monoclonal protein, chronic demyelinating polyneuropathy, hepatosplenomegaly, hypothyroidism, gynecomastia and severe renal impairment. The finding of IgG kappa type of monoclonal protein in our patient was interesting because the majority of cases were reported to have lambda light chain. Also, the absence of typical skin and bone lesions were atypical. Though speculative, these atypical features may account for the unusual presentation of this case. Our patient rapidly progressed to end-stage renal failure and died of cachexia. Renal involvement in POEMS syndrome is rare but may show substantial clinical and pathological variations. Proteinuria, hematuria, renal dysfunction and renal failure requiring hemodialysis can be seen. The pathogenesis of renal dysfunction is unclear. As a conclusion, POEMS syndrome may present with diverse clinicopathologic manifestations. In this syndrome, renal involvement may lead to end stage renal failure and the course may be fatal due to severe polyneuropathy and wasting.

Prognostic value of Ki-67, CD31 and epidermal growth factor receptor expression in basal cell carcinoma.

Recurrence of basal cell carcinoma following treatment is common, and the majority of recurrences appear in the first 3 years. We examined the original tumors of 26 basal cell carcinoma cases, 14 of whom had a recurrence after an average of 3.7 years, and 12 of whom had no recurrence during an average of 4.4 years follow-up. Using immunohistochemistry, we tested for Ki-67, CD31 and epidermal growth factor receptor expressions in the tumor tissue. The percentages of expression for Ki-67, CD31 and epidermal growth factor receptor were significantly higher in the recurrent tumors than in the non-recurrent ones. Expression of Ki-67 and CD31 was 271.57 +/- 17.91 and 58.1 +/- 9.37 for the recurrent group and 187.08 +/- 21.48 and 23.9 +/- 5.45 for non-recurrent group respectively (p<0.0001; p<0.0001). Expression of epidermal growth factor receptor was positive in all basal cell carcinoma cells. The staining intensity was strong in 57% of recurrent and 8.3% of non-recurrent tumors (p=0.014). These results show that Ki-67, CD31 and epidermal growth factor receptor expression differ between basal cell carcinomas which later recur and those that do not recur.