Vol.116, No.6, June 2012

1. Improvement of Adult Height in GH-treated Idiopathic Growth Hormone Deficiency
2. A Case of Kawasaki Disease Complicated by Guillain-Barré Syndrome
3. Thyroxine Replacement Therapy for Hypothyroidism Caused by ACTH Therapy for West Syndrome
4. Two Pediatric Cases of Deceased Donor Split Liver Transplantation
5. A Pediatric Case of Pulmonary Alveolar Microlithiasis with Exertional Dyspnea

Original Article
Improvement of Adult Height in GH-treated Idiopathic Growth Hormone Deficiency
Masamichi Ogawa1)2) Osamu Nose1)3) Toshihisa Okada1)4) Takashi Kamijo1)5) Eiichi Kinoshita1)6) Masakuni Tokuda1)7) Noboru Tsuru1)8) Satoshi Matsuo1)9) Takeki Hirano1)10) Nobuko Hashimoto1)11) Haruo Ogawa1)12) Yutaka Igarashi1)13) and Toshiaki Tanaka1)14)
1)Study Group of Out-patient Clinics on Short Stature
2)Ogawa Clinic
3)Nose Clinic
4)Kumamoto Growth Clinic
5)Nagoyaka Children's Clinic
6)Kinoshita Children's Clinic
7)Tokuda Children's Clinic
8)Tsuru Noboru Clinic
9)Matsuo Children's Clinic
10)Hirano Children's Clinic
11)Hashimoto Children's Clinic
12)Ogawa Clinic
13)Igarashi Children's Clinic
14)Tanaka Growth Clinic
Adult heights of growth hormone (GH)-treated children with idiopathic growth hormone deficiency (GHD) who had been treated at 13 clinics of pediatric endocrinology were analyzed. Eighty-nine idiopathic GHD (56 boys, 33 girls) were classified into severe GHD (11 cases), moderate GHD (41 cases) and mild GHD (37 cases) according to maximum peak GH level to GH stimulation tests. Mean age and mean height SD score at start of GH were 9.33 years and -2.76 SD, respectively.
Patients were treated with a mean initial dose of 0.192 mg/kg/week and for mean treatment period of 6.5 years. Five patients received concomitant anabolic steroid hormone, four patients LHRH analog and 3 patients both anabolic steroid hormone and LHRH analog. Mean adult heights of boys and girls were 163.9 cm (-1.16 SD) and 150.9 cm (-1.40 SD), respectively. Mean improvement of height SD scores were +1.52 SD in boys and +1.50 SD in girls. Adult heights in 86.9% of boys and 90.3% of girls were within target height range.
Improvement of adult height results in this study was attributed to early start of GH treatment, higher height SD score at start of GH treatment, and higher treatment dose.

Original Article
A Case of Kawasaki Disease Complicated by Guillain-Barré Syndrome
Yukiko Kuroda1) Masakazu Mimaki1) Hiroshi Terashima1) Atsushi Sato1) Kan Takahashi1) Hirotsugu Kano1) Akira Oka1) Masaru Mizuguchi2) and Takashi Igarashi1)
1)Department of Pediatrics, Graduate School of Medicine, University of Tokyo
2)Department of Medical Sciences, Graduate School of Medicine, University of Tokyo
A 2-year-old boy with Kawasaki disease (KD) showed quadriparesis on the 5th day of illness. He became unable to stand alone and showed bilateral lower limb-dominant muscle weakness with hypoactive tendon reflexes. Nerve conduction studies showed the absence of F waves in both the upper and lower extremities, while the conduction velocity and amplitude of the M waves were normal. Guillain-Barré Syndrome (GBS) was diagnosed. Immediately after treatment with intravenous immunoglobulin on the 8th day, he became afebrile and regained the ability to stand and walk independently. F waves were observed on the 15th day. His motor symptoms were completely alleviated within a month after the disease onset. This is a rare case of simultaneous occurrence of KD and GBS.

Original Article
Thyroxine Replacement Therapy for Hypothyroidism Caused by ACTH Therapy for West Syndrome
Katsuyuki Matsui1) Youji Uehara1) Atsushi Tsukamura2) Fukiko Ryujin1) Kumiko Matsumake1) Seiichirou Yoshioka1) Yoshihiro Maruo1) Tomoyuki Takano1) and Yoshihiro Takeuchi1)
1)Department of Pediatrics, Shiga University of Medical Science
2)Department of Pediatrics, Omihachiman Community Medical Center
West syndrome is an epileptic encephalopathy characterized by infantile spasms, hypsarrhythmia, and intellectual disability. Vigabatrin is one of the main agents to treat this syndrome, but is unapproved in Japan. Thus, ACTH therapy is recognized as the most effective available therapy for West syndrome. ACTH therapy is associated with possible complications, e.g., brain shrinkage, hypertension, adrenal failure, and subdural hematoma. However, hypothyroidism has not been sufficiently recognized as a complication of ACTH therapy. Here, we report the case of a 5-month-old girl with West syndrome, who required treatment with thyroxine replacement therapy for hypothyroidism caused by ACTH therapy. Initial treatment involved the oral administration of pyridoxine, and, subsequently, valproic acid and zonisamide. However a series of spasms and hypsarrhythmia continued. Thus, she was treated with ACTH therapy. Her thyroid function was in the normal range before ACTH therapy. At 7 days of ACTH therapy (0.0125 mg/kg/day), hypothyroidism became apparent: TSH 0.29 μIU/ml; free T3 0.7 pg/ml; free T4 0.62 ng/dl. A TRH stimulation test revealed low-reactive TSH, suggesting hypothyroidism due to central dysfunction. Thyroxine replacement therapy was performed for 10 weeks. After the withdrawal of replacement therapy, her thyroid function recovered. Several cases of hypothyroidism caused by ACTH therapy were reported in the 1980's and 1990's, and it was revealed that 50-70% of patients treated with ACTH developed hypothyroidism. In spite of the deleterious effect of hypothyroidism on brain development, this complication has not been reported recently, and had never been described in therapy guidelines. The developmental prognosis on receiving thyroxine replacement therapy after ACTH therapy may be unclear, because West syndrome also causes mental retardation. We should employ this replacement therapy for patients with hypothyroidism, because mental retardation caused by hypothyroidism is preventable. Therefore, we should routinely examine the thyroid function of patients receiving ACTH therapy. Additionally, a multicenter study of ACTH therapy considering thyroxine replacement therapy for secondary hypothyroidism is needed.

Original Article
Two Pediatric Cases of Deceased Donor Split Liver Transplantation
Toshihiko Kakiuchi Mureo Kasahara Seisuke Sakamoto Hiroyuki Kanazawa Chiaki Karaki Takanobu Shigeta Akinari Fukuda Satoshi Nakagawa Atsuko Nakazawa and Akira Matsui
National Center for Child Health and Development
The revised Japanese organ transplant law was enacted in July, 2010, and organ donation in cases of brain death became possible if there was family consent. An increase in organ donation from pediatric brain death was therefore expected; however, only one case has been reported, as of March 2011. Split liver transplantation from brain death donors is an operative procedure in which the liver of an adult donor is split, and is transplanted to two recipients. This is also a measure for expanding the donor pool. We herein report on two cases in which split liver transplantation from brain death donors was implemented at this center after the revision of the organ transplant law.
Case 1 was a 9-year-old girl with fulminant hepatic failure. Both parents were unsuitable as living donors for liver transplantation, for medical reasons. A brain death donor appeared three days after registering for liver transplantation from a brain death donor, and split liver transplantation was performed. Case 2 was a 11-month-old girl with biliary atresia. Registration for liver transplantation from a brain death donor was performed at the family's consent, simultaneously with the arrangements for a live-donor liver transplant. A brain death donor appeared 45 days after registration, and split liver transplantation was performed. Since the enactment of the revised organ transplant law, organ donation from brain death has been on the rise. In order to reduce the burden on living donors / families also, it is believed that liver transplantation from brain death donors for pediatric cases should be actively considered in the future. Split liver transplantation has the potential of increasing pediatric liver transplantation from brain death donors, and we believe that it should be actively adopted.

Original Article
A Pediatric Case of Pulmonary Alveolar Microlithiasis with Exertional Dyspnea
Rumi Taniguchi Yohei Hosoi Anzu Noda Reimi Sayama Yoko Okamoto Yuki Koshino Hajime Nishimoto and Masaru Takamizawa
Departments of Pediatrics, Saitama Citizen Medical Center
The patient was a 13-year-old boy with no family medical history of note. He was given diagnosis of allergic rhinitis at age 10 years. He was referred to our hospital to assess premature ventricular contractions detected during his school medical checkup. Both the echocardiogram and the electrocardiogram appeared normal, but the chest X-ray film showed a diffuse micronodular shadow. His serum examinations, including KL-6, were normal. Trichosporon asahii antibody was negative, and respiratory infections such as tuberculosis were ruled out. Computed tomography scan demonstrated a micronodular pattern predominantly at the site of the subpleural parenchyma. The patient was asymptomatic at presentation, but several days later, he complained of mild exertional dyspnea. He was admitted to our hospital for 2 weeks, but his dyspnea continued. After 2 months, thoracoscopic lung biopsy was performed, and pulmonary alveolar microlithiasis was diagnosed. This disease is a rare inherited respiratory disorder, and there have been only a few cases with symptoms from childhood. The patient will need treatment, including continuous oxygen therapy and lung transplantation in the near future.

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