Background: There is no clear consensus as to which patients with Henoch-Schönlein purpura nephritis (HSPN) at risk of a poor outcome should be treated and what therapeutic regimen should be used. Methods: Nine children with heavy proteinuric HSPN received prompt initiation of methylprednisolone pulse therapy (MPT) combined with tonsillectomy in a prospective study. Results: At presentation, the mean values for the patients’ urine protein excretion (early-morning urinary protein/creatinine ratio), serum IgA, activity index (AI), and chronicity index (CI) were 5.0 ± 5.6 g/g Cr, 135.6 ± 56.5 mg/dl, 4.0 ± 0.7, and 1.7 ± 1.3, respectively. At the second biopsy, conducted approximately 24 months after initiation of therapy, the patients’ serum albumin had significantly increased (4.4 ± 0.2, p < 0.01), and the serum IgA and AI had significantly decreased (88.1 ± 30.8 mg/dl, p < 0.05; 2.0 ± 1.2, p < 0.01, respectively), whereas the CI remained unchanged. Proteinuria disappeared within 24 months in all but 1 patient, and hematuria disappeared within 38 months in all patients. No patient showed renal impairment or experienced a recurrence and/or exacerbation of HSP/HSPN. Conclusions: Early treatment with MPT combined with tonsillectomy is effective in ameliorating the histopathological progression and improving the clinical course of children with heavy proteinuric HSPN.
Age ≥ 10 is an independent risk factor for post-PRB complication. After the procedure, the formation of a large hematoma predicted a complicated clinical course.
Dysosteosclerosis is a sclerosing bone dysplasia with skeletal changes resembling those
of osteopetrosis. The disorder is associated with dental anomalies and occasionally mental
retardation. Because of the rarity and phenotypic diversity of dysosteosclerosis, it
remains unsolved whether or not the disorder is heterogeneous. We report here on an
affected boy associated with brain calcification and epilepsy with developmental delay.
Prenatal ultrasound revealed ventriculomegaly, and brain CT in the neonatal period showed
periventricular calcifications. At 13 mo of age, he presented with generalized convulsion
with developmental delay. Metaphyseal sclerosis, metaphyseal undermodeling, and
oval-shaped vertebral bodies on skeletal survey warranted a diagnosis of
dysosteosclerosis. Retrospective review of radiographs as a neonate showed metaphyseal
radiolucency, but not metaphyseal sclerosis. Since then, neither the bone changes nor
neurological symptom has progressively worsened up to 4 yr of age. Thus, it is thought
that the clinical and radiological manifestations of the sclerotic disorder become obvious
during infancy. Brain calcification of prenatal onset may be an essential syndromic
constituent of the disorder.
Although renal hypouricemia is mostly asymptomatic, it is known to present a high risk of exercise-induced acute renal failure, especially in young males. However, there is little information regarding the clinical features of urolithiasis as a complication in childhood renal hypouricemia. Here we report a 4-year old female with idiopathic renal hypouricemia who presented with macroscopic hematuria due to obstructive calcium oxalate urolithiasis. She was treated successfully with percutaneous nephrolithotripsy and thereafter hematuria disappeared. Sequence analysis of the patient and her family's URAT1 gene confirmed a nonsense mutation in exon 4 (W258X). To the best of our knowledge, this is the youngest case of hereditary renal hypouricemia caused by URAT1 gene mutation, which was found by hematuria due to calcium oxalate urolithiasis.
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