Hiroaki Kanai

Early Treatment with Methylprednisolone Pulse Therapy Combined with Tonsillectomy for Heavy Proteinuric Henoch-Schönlein Purpura Nephritis in Children

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.

Pediatric case of crescentic post-streptococcal glomerulonephritis with myeloperoxidase anti-neutrophil cytoplasmic antibody.

Post-streptococcal glomerulonephritis (PSGN) generally has a good renal prognosis, and immunosuppressive therapies are not needed. However, a few patients present with severe acute kidney injury and extensive crescent formations. The etiology of such patients is not well known, and involvement of anti-neutrophil cytoplasmic antibodies is rarely reported. A 9-year-old girl with rapidly progressive nephritic syndrome was diagnosed with PSGN. A biopsy showed diffuse crescentic glomerulonephritis with immunoglobulin G and C3 deposits; moreover, humps were observed on electron microscopy. After she was administered methylprednisolone pulse therapy and intravenous cyclophosphamide, followed by prednisolone and azathioprine therapy, her urinary abnormalities improved and renal function normalized. However, the myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA) titers gradually increased. We speculated that PSGN may be augmented by increased MPO-ANCA levels. Therefore, the patient is currently being treated with losartan, enalapril, azathioprine, and prednisolone. Although the MPO-ANCA titer remains high, urinary findings show mild proteinuria and her renal function has been norma for 18 months since onset. A progressive clinical course and severe histological findings may indicate the involvement of ANCA in deterioration of condition in patients with PSGN. Furthermore, in such cases immunosuppressive therapies should be considered even in pediatric PSGN.

Role of ultrasound in revealing complications following percutaneous renal biopsy in children.

BACKGROUND This retrospective case series aimed to investigate the role of ultrasound immediately post-percutaneous renal biopsy (PRB) for detecting post-biopsy complications in pediatric patients. METHODS Data from 380 (male/female = 209/171) consecutive biopsies of native kidney tissue of 344 children from January 2001 to October 2009 were analyzed to investigate the role of an ultrasound immediately post-PRB and the predictive value of demographic, clinical, and baseline chemistry factors in predicting the risk of post-PRB complications. RESULTS Post-PRB ultrasound identified hematoma formation in 33 (8.7%) patients. Of the 19 (5.0%) patients whose hematomas were large (≥ 1 cm), post-biopsy courses of 16 patients were clinically complicated. On the other hand, of the 14 patients whose hematomas were small (< 1 cm), all patients but one showed an uncomplicated clinical course. Of the 17 complications, 79.1% were detected within the first 24 hours and 21.9% (cases of resorption fever) between 24 and 144 hours post-PRB. Age ≥ 10 is an independent risk factor for post-PRB complication. CONCLUSIONS 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.

Urinary fibrin/fibrinogen degradation products measured using an anti-fibrinogen antibody predict orthostatic proteinuria

The aim of this study was to assess the diagnostic value of urinary fibrin/fibrinogen degradation products (uFDP) measured using an anti‐fibrinogen antibody in patients with orthostatic proteinuria (OP), and their use in differentiating between OP and glomerulonephritis (GN).

A Case Report of Dysosteosclerosis Observed from the Prenatal Period

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.

Fiberoptic laryngoscopy used to determine the origin of mediastinal emphysema: A case report

11カ月女児。ボールペンをくわえたまま炬燵の上から転落し口腔内に出血を認め, 当院に紹介された。入院時, 出血はみられず呼吸障害は認めなかった。頭蓋内損傷精査のため撮影した頭部CTで, 頚部に皮下気腫を認め, 胸部レントゲン写真で縦隔気腫と皮下気腫を認めた。舌圧子による視診では, 受傷部位を同定できなかった。緊急の外科治療を要する食道, 気管損傷による縦隔気腫の可能性も否定できず, 喉頭ファイバースコープ検査を実施したところ咽頭後壁に凝血塊を認め受傷部位を同定しえた。また気管支ファイバースコープで気管分岐部まで観察したが気管に損傷はなかった。抗生剤投与のみで経過観察したが, 皮下気腫, 縦隔気腫ともに速やかに改善し, 第9病日に退院した。縦隔気腫では原因を同定することが治療方針決定の上で重要であり, その際気道ファイバースコープは有用な検査であると思われた。