Osamu Motoyama

Henoch-Schönlein purpura with hypocomplementemia in children

Abstract  Background : The clinical course and prognosis of Henoch‐Schönlein purpura (HSP) associated with hypocomplementemia are not clear.

Familial cases of Henoch-Schonlein purpura in eight families

Abstract Background : Familial cases of Henoch‐Schönlein purpura (HSP) have rarely been reported.

Final height in children with steroid-sensitive nephrotic syndrome

Background: Growth retardation following steroid treatment in children is a major problem. Reduction of steroid dose has been tried using immunosuppressive agents such as cyclosporine A or mizoribine in children with frequently relapsing nephrotic syndrome. Few reports concerning final height in steroid‐sensitive nephrotic syndrome (SSNS) are available.

Head circumference and development in young children after renal transplantation

Background:  Growth impairment, microcephaly and developmental delay in young children with chronic renal failure improve after successful renal transplantation. There have been few reports on head circumference (HC) and development after transplantation.

Steroid-sensitive nephrotic syndrome in two families

Few reports of familial cases of steroid-sensitive nephrotic syndrome (SSNS) are available. Of 123 children with SSNS, four cases in two families are presented. Two sisters and a father and his daughter developed SSNS. The incidence of SSNS siblings in Japan seems to be similar to the incidence reported in other countries. SSNS in two generations is rare. To our knowledge, cases of SSNS in a parent and a child in Japan have not been reported.

Clinical course of congenital nephrotic syndrome and Denys‐Drash syndrome in Japan

Abstract Background : The prognosis of Japanese patients with congenital nephrotic syndrome (CNS) and Denys‐Drash syndrome (DDS) is not clear.

Pathological evaluation of steroid withdrawal in pediatric renal transplant recipients

Abstract: Protocol biopsies were performed to detect and treat sub‐clinical rejection in eight living related pediatric renal transplant recipients who had been withdrawn from steroids. Low‐grade tubulitis (t1) and mononuclear cell interstitial inflammation (i1), typical of borderline rejection and low‐grade interstitial fibrosis (ci1), and tubular atrophy (ct1), characteristic of chronic rejection, appeared frequently in protocol biopsies more than 3 yr after steroid withdrawal. In contrast, early‐type glomerulitis (g), allograft glomerulopathy (cg), arteriolar hyaline thickening (ah), intimal arteritis (v), and fibrous intimal thickening (cv) were not observed in protocol biopsies after steroid withdrawal. Low‐dose pulse therapy (methylprednisolone (MP) 250 or 500 mg/d for 3 d) was administered to five patients for borderline and/or acute grade 1a rejection, as determined by protocol biopsies in the absence of clinical rejection. No oral steroids were administered. Renal function in all patients remained satisfactory, without proteinuria, in follow‐up periods ranging from 22 to 68 months after steroid withdrawal. Of the eight patients, three grew to almost normal height (>mean – 2SD) and four exhibited catch‐up growth. Thus, steroid withdrawal can be safe and improves growth in pediatric renal transplant recipients if undertaken with careful clinical follow‐up, including protocol biopsies.

A girl with Peters plus syndrome associated with myelomeningocele and chronic renal failure

A girl was born with sclerocornea of the right eye, corneal staphyloma of the left eye and lumbar myelomeningocele. The myelomeningocele was repaired soon after birth. The corneal staphyloma was perforated during infancy. She received keratoplasty and achieved light perception. Her right kidney revealed multicystic dysplasia and was non-functioning at birth. She had neurogenic bladder, and her renal function deteriorated gradually. Peters plus syndrome was diagnosed based on anterior ocular segment anomalies, short stature, developmental delay and characteristic face. Anterior ocular segment anomalies are rare findings, but seem to be occasionally associated with spina bifida and renal anomalies. Myelomeningocele and chronic renal failure in patients with Peters plus syndrome have not been reported to our knowledge.

Adult height of three renal transplant patients after growth hormone therapy.

Three girls with normal growth hormone secretion had received renal transplantation when aged 2 to 6 years. They had had severely retarded growth (SD for height score was −7.4 to −3.7) at the time of transplantation. After renal transplantation, steroid was withdrawn and they were treated with recombinant human growth hormone; they subsequently reached adult heights of 145 to 156 cm. The SD for adult height score was −2.6 to −0.3. The adult height in two patients was over their target height, calculated using the mean of the parents’ height. This report shows the efficacy of steroid withdrawal and recombinant human growth hormone therapy in achieving adult height in these three girls after renal transplantation.

Final height in a prospective trial of late steroid withdrawal after pediatric renal transplantation treated with cyclosporine and mizoribine

Motoyama O, Hasegawa A, Aikawa A, Shishido S, Honda M, Tsuzuki K, Kinukawa T, Hattori M, Ogawa O, Yanagihara T, Saito K, Takahashi K, Ohshima S. Final height in a prospective trial of late steroid withdrawal after pediatric renal transplantation treated with cyclosporine and mizoribine. 
Pediatr Transplantation 2012: 16: 78–82.