Neurodevelopmental outcomes of children with periventricular leukomalacia.

OBJECTIVES: To examine the neurodevelopmental outcomes of children with periventricular leukomalacia (PVL). MATERIALS AND METHODS: Twenty-five children diagnosed with grade 1, 2 or 3 PVL on the basis of magnetic resonance imaging (MRI) findings between January 2002 and December 2011 were enrolled and followed from 15 months to 10 years of age. RESULTS: Of the 25 children, one was a term and 24 were preterm-births. Nine (36%) had spastic diplegia and 12 (48%) had quadriplegia. Ten of the 25 (40%) were able to walk independently at 36 months utilizing short leg braces, whereas 13 children (52%) were unable to walk independently. MRI findings revealed grade 1 PVL in nine (36%), grade 2 in 12 (48%), and grade 3 in four (16%) of the 25 children. Eleven of the 16 children (69%) with grade 2 or 3 PVL had Papile III or IV intraventricular hemorrhage (IVH), and many of these children had severe neurologic motor abnormalities, severe psychomotor delay, and seizures. Five of the nine children (56%) with grade 1 PVL had normal psychomotor development. There were statistically significant differences in the motor impairment and walking ability between the children with grade 1 and those with grade 2 PVL (p = 0.008 and 0.005, respectively). CONCLUSION: Most children with grade 2 or 3 PVL had severe neurodevelopmental delays, but attention should also be paid to the 56% of children with grade 1 PVL who presented with normal psychomotor development. Further studies of larger populations, including long-term follow-up, are necessary to evaluate the outcomes of children with PVL.

Efficacy of single dose of oral mizoribine pulse therapy two times per week for frequently relapsing nephrotic syndrome.

We assessed the efficacy of a single dose of oral mizoribine (MZB) pulse therapy two times per week for children with frequently relapsing nephrotic syndrome (FRNS). Eleven children with FRNS in remission were treated with oral MZB pulse therapy (daily dose 6 mg/kg; maximum total dose 300 mg). We compared their clinical manifestations before and after oral MZB pulse therapy and examined the changes in serum MZB concentration in each patient on the days when MZB was administered. Eight patients had no subsequent relapses (responders), and prednisolone could be discontinued. Although 2 of the other 3 patients (nonresponders) had one relapse and the remaining patient had two relapses, both the dosages of prednisolone and frequency of relapse after oral MZB pulse therapy were significantly lower than before oral MZB pulse therapy. The peak blood concentration and AUC0-4 of MZB in the responders were higher than in the nonresponders. None of patients had severe adverse effects, such as uricacidemia, leukopenia, liver dysfunction or alopecia. Oral mizoribine pulse therapy consisting of a single dose two days a week may be effective and safe in some FRNS patients.

Efficacy of tonsillectomy pulse therapy versus multiple-drug therapy for IgA nephropathy.

We evaluated the efficacy of tonsillectomy plus pulse prednisolone, warfarin, and dipyridamole including methylprednisolone pulse (tonsillectomy plus pulse therapy), versus prednisolone, warfarin, and dipyridamole including mizoribine (PWDM) for the treatment diffuse IgA nephropathy (IgAN) in children. The patients were randomly assigned to be treated by tonsillectomy plus pulse therapy for 2 years (Group A, n=16) or PWDM for 2 years (Group B, n=16). The clinical features and pathological findings in both groups were analyzed prospectively. The mean urinary protein excretion after 6 months of treatment in both groups had decreased significantly compared with pre-therapy. The activity index (AI) in both groups was lower at the time of the second biopsy than at the time of the first biopsy. The chronicity index (CI) in Groups A and B did not differ between the first and second biopsy. At the latest follow-up examination none (0%) of the patients in either group had renal insufficiency. None of the patients in Group A, but six patients in Group B experienced an acute exacerbation of IgAN as a result of tonsillitis (P<0.05). In conclusion, although there was no untreated control group in this study, the results suggested that tonsillectomy plus pulse therapy is as effective as PWDM in ameliorating proteinuria and histological severity in IgAN patients and in preventing acute exacerbation of IgAN by tonsillitis.