|
THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
|
Vol.116, No.12, December 2012
|
Original Article
Title
Plasma Ketone Values Are Low in Hypoglycemic Neonates Up to 48 Hours after Birth
Author
Hiroshi Mizumoto Kazutoshi Ueda Hirofumi Shibata Masashi Taniguchi Masamitsu Mikami Ryoko Akashi Takayuki Hamabata Michio Matsuoka Masatoshi Nakata Hirotsugu Oda Hironori Ebijima Keiko Yamamoto Yoshie Nakamura Hitoshi Nishida Akira Kumakura Takakazu Yoshioka Yoko Yoshida Mitsutaka Shiota Atsuko Hata and Daisuke Hata
Department of Pediatrics, Kitano Hospital, the Tazuke Kofukai Medical Research Institute
Abstract
We examined serum immunoreactive insulin (IRI) and 3-hydroxybutyric acid (3-HB) levels in 25 hypoglycemic neonates up to 48 hr after birth. All cases showed a low 3-HB level (≤0.8 mmol/l) and 21 cases showed a high IRI level (≥2.0 μIU/l) at the time of hypoglycemia. Neonates, who later required an infusion rate of more than 6-8 mg/kg/min of glucose to maintain normoglycemia (high-GIR group), showed significantly lower 3-HB levels than those who did not (low-GIR group)(0.02±0.02 mmol/l vs. 0.18±0.26 mmol/l). After 12 hr of birth, 5 of 7 cases in the low-GIR group showed 3-HB levels of 0.36 to 0.80 mmol/l, while 2 cases in the high-GIR group had a 3-HB level of less than 0.10 mmol/l.
Caution is needed when current diagnostic criteria are applied to neonates with hyperinsulinemic hypoglycemia during the period up to 48 hr after birth. Extremely low 3-HB (<0.10 mmol/l) 12 hrs after birth could be an important indicator for predicting whether intensive treatment is necessary or not.
|
|
Original Article
Title
Estimation of Glomerular Filtration Rate without Urine Collection
Author
Koichi Kamei Akinori Miyazono Mai Sato Tomoaki Ishikawa Takuya Fujimaru Masao Ogura and Shuichi Ito
Department of Nephrology and Rheumatology, National Center for Child Health and Development
Abstract
Measuring inulin clearance by urine collection is widely accepted as the gold standard for estimating the glomerular filtration rate (GFR). However, this method has several limitations, including large transfusion volumes and the need to collect several blood samples. Residual urine also causes inaccurate estimations of GFR and infants require bladder catheterization. After constant infusion, inulin reaches an equilibrium in which the inulin urinary excretion rate is equal to the infusion rate. Therefore, inulin clearance can be calculated based on the plasma inulin concentration and the rate of infusion, meaning that urine collection is unnecessary. We published the results of a study in which we determined inulin clearance during constant infusion for 10 hours, and have since modified the protocol to six hours. We measured inulin clearance by urine collection (Cin) and by constant infusion (eCin) in 19 patients (age, 3-35 years). Four patients were excluded from the study because of inaccurate Cin data. Therefore, we compared values among 15 patients. The values for Cin and eCin were similar and linear correlation was good (R2=0.78). The mean±standard deviation of eCin/Cin was 1.072±0.181 and the ratio was between 0.8 and 1.2 in 9 (60%) patients. We believe that constant infusion is clinically useful because it is noninvasive, suitable for infants, and generates GFR values that are comparable with those obtained by urine collection.
|
|
Original Article
Title
A Case of Autistic Spectrum Disorder Who Developed Hypothyroidism due to an Iodine Deficiency from a Long-term Severe Unbalanced Diet
Author
Motohide Goto Yukiyo Yamamoto Masahiro Ishii Reiko Saito Shunsuke Araki Kazuyasu Kubo Rinko Kawagoe Yasusada Kawada and Kouichi Kusuhara
Department of Pediatrics, School of Medicine, University of Occupational and Environmental Health
Abstract
We report an 11-year-old boy with autistic spectrum disorder who developed hypothyroidism due to an iodine deficiency because of a severely unbalanced diet over an extended period. This patient was given a diagnosis of autistic spectrum disorder when he was 2 years old. Since the diagnosis, his diet has consisted of only polished rice and an electrolyte liquid. He developed hemolytic anemia due to a vitamin B12 deficiency at 8 years of age. Although nutritional education was provided to him, improvement of his unbalanced diet was difficult. At 11 years of age, he had marked general fatigue and a decreased growth rate. His endocrinological examination revealed primary hypothyroidism and a low concentration of total urinary iodine. Because of his history of a long-term severe unbalanced diet, he was diagnosed as having hypothyroidism due to iodine deficiency. His thyroid function was normalized after replacement with 200 μg/day of iodolecithin. His general fatigue disappeared, and his growth rate improved along with the normalization of his thyroid function. After 2 months, we changed the iodolecithin replacement to addition of a seaweed drink to his diet. His thyroid function has remained and he is asymptomatic. Patients with autistic spectrum disorder often have unbalanced diets. However, there has been no report about hypothyroidism due to iodine deficiency in Japan. Because of the generally sufficient intake of seafood products in Japan, it is possible to miss a diagnosis of iodine deficiency. Thyroid function should be checked in cases of autistic spectrum disorder with long-term severe unbalanced diets.
|
|
Original Article
Title
Four Cases of Mild Encephalitis/Encephalopathy with a Reversible Splenial Lesion (MERS) Accompanying Acute Focal Bacterial Nephritis (AFBN)
Author
Yuh Fujiwara Fumiko Tanaka Takuya Wakamiya Taiga Kobori Kana Hashiguchi Mutsumi Sato Chie Arai Munenori Murata Takeshi Suzuki Saeko Yuzurihara Kazuko Yamaguchi Chiho Saito Hiroyuki Takahashi and Sumio Kai
Department of Pediatrics, Saiseikai Yokohamashi Nanbu Hospital
Abstract
Although there are many reports of virus-associated mild encephalitis/encephalopathy with a reversible splenial lesion (MERS), bacterial infections as the cause of MERS seems to be rare. Here we report 4 pediatric cases of MERS accompanying acute focal bacterial nephritis (AFBN).
The median age was 7 years (range 5 to 11 year-old), and two children were boys. They exhibited fever and abdominal pain as well as neurological symptoms including headache, vomiting, disturbance of consciousness, abnormal behaviors or meningeal irritation signs. Increased WBC count, elevated CRP, and hyponatremia were revealed by blood tests and contrast-enhanced abdominal CT showed round- or wedge-shaped contrast defects in kidney, indicating AFBN. As DWI images of brain MRI also disclosed high intensity area in splenium, all 4 cases were diagnosed as having MERS caused by AFBN. AFBN were successfully treated with antibiotics and MERS were immediately resolved without any treatment in all patients.
Since AFBN is sometimes difficult to diagnose, there have been no reports of AFBN with MERS so far.
When we see a patient who has signs and symptoms of encephalitis or encephalopathy accompanied by fever of unknown origin, it is important to keep AFBN in mind in making a differential diagnosis.
|
|
Original Article
Title
A Terminal Case of Neonatal Intracranial Immature Teratoma Who was Cared for in a General Pediatric Ward
Author
Yoshihiko Shitara1) Kohmei Ida3) Youhei Hosoi1) Jyun Ookubo1) Naoki Itou1) Keiji Goishi1) Jyunko Takita2) Akira Kikuchi4) and Takashi Igarashi5)
1)Department of Pediatrics, The University of Tokyo
2)Department of Cell Therapy and Transplantation Medicine, The University of Tokyo
3)Department of Pediatrics, Teikyo University School of Medicine University Hospital, Mizonokuchi
4)Department of Pediatrics, Teikyo University Hospital
5)National Center for Child Health and Development
Abstract
We report a rare case of neonatal intracranial immature teratoma who was cared for in the general pediatric ward for terminal care. The patient was born by caesarean section because of detection of ventricular dilatation by fetal ultrasound at 39 weeks of gestational age. She was operated on at the 9th day of age, and immature teratoma was diagnosed. Although chemotherapy was performed with carboplatin and etoposide from the 15th day of age, the tumor grew and infiltration into the brain stem was suspected. We explained to her parents that she had a poor prognosis. She was then moved to the general pediatric ward from the NICU to receive terminal care on the 34th day of age, where we performed palliative care such as treating pain and walking in the hospital. The parents and patient started living in the same room, and the parents attachment to their child increased as their attitude and sentiments changed. The newborn baby's terminal care was very difficult. However, it was thought that we could offer the medical treatment that the family hoped for by moving the infant to the general pediatric ward. Nevertheless, consideration should be given to preparing a room in the NICU in which parents can spend time with their children and terminal care can be provided.
|
|
Original Article
Title
A Japanese Family with Familial Juvenile Hyperuricemic Nephropathy and Novel Uromodulin Mutation G210D
Author
Sayu Omori1) Katsuhiro Maeyama1) and Seiji Sato2)
1)Department of Neonatology, Saitama Municipal Hospital
2)Department of Pediatrics, Saitama Municipal Hospital
Abstract
Familial juvenile hyperuricemic nephropathy (FJHN) is an autosomal dominant renal disease characterized by juvenile onset of hyperuricemia, gout, and progressive renal failure. Uromodulin(UMOD), which is the gene responsible for this disease, was recently identified. In the current study, a three-generation Japanese family diagnosed with FJHN was analyzed for the UMOD mutation. The index patient, a 14-year-old girl, was admitted to our hospital with swelling and tenderness in the bilateral first metatarsophalangeal joints of the toes. Her laboratory data showed hyperuricemia and low urate excretion. Her mother and maternal grandmother had a history of gouty arthritis and renal failure.
The mutation was detected by direct sequencing of the UMOD gene of the patients in this family. UMOD mutation was found in exon 4, which corresponded to G210D. The index patient was homozygous for the G210D mutation, whereas her mother and grandmother were heterozygous for this mutation. However, it was not concluded whether she is homozygous or not, because UMOD mutation analysis of her father has not been done.
This missense mutation has not been previously reported. We have assumed that this missense mutation is the likely cause for this disease because 1) G210 in the UMOD protein is highly conserved among different species and 2) the amino acid substitution dramatically alters charge characteristics.
|
|
Original Article
Title
A Case of a Child Who Developed Heparin-induced Thrombocytopenia in the Induction Period of Hemodialysis
Author
Natsumi Yamamura Yoko Santo and Kenichi Satomura
Department of Pediatric Nephrology and Metabolism, Osaka Medical Center and Research Institute for Maternal and Child Health
Abstract
It is sometimes hard to diagnose HIT (heparin-induced thrombocytopenia) on hemodialysis with less than severe symptoms because the platelet count is restored on non-dialysis days and the dialyzer catches the HIT antibody. Here we report a case of a pediatric patient who developed HIT at the induction phase of hemodialysis.
A 14-year-old boy on peritoneal dialysis due to chronic kidney disease underwent laparotomy for chronic pancreatitis. Preoperatively arteriovenous shunt was made and he was hemodialyzed with heparin once. The second and third dialyses were performed postoperatively with nafamostat mesilate (NM) and those from the fourth time onward were done with heparin. During the fifth dialysis, which was performed 16 days after the first hemodialysis, we noticed rise in the pressure of the extracorporeal circuit and clot formation to dialyzer. After we switched heparin to NM, the pressure decreased and we could continue hemodialysis. At the end of the dialysis, his platelet count decreased by 42% of the previous level. On the ninth dialysis with heparin, we had to switch heparin to NM again because of high circuit pressure. Therefore we suspected HIT and demonstrated that the HIT antibody was positive. We made a diagnosis of HIT type II. He could continue hemodialysis with NM and his platelet count did not decrease without heparin.
When we start hemodialysis, we should keep HIT in mind and monitor the extracorporeal circuit pressure and platelet count before and after dialysis.
|
|
Original Article
Title
Myopathy in an Adolescent Boy with Thiamine Deficiency due to Extremely Unbalanced Nutrition
Author
Yu Kawasaki Kousaku Matsubara Yoshiko Uchida Aya Iwata Kazuo Yura Hiroyuki Nigami and Takashi Fukaya
Department of Pediatrics, Nishi-Kobe Medical Center
Abstract
Thiamine deficiency in Japanese children is very rare. Most patients are affected during early infancy due to an excessive intake of isotonic drinks or restricted diet therapy for atopic dermatitis. We report an adolescent male with a thiamine (vitamin B1) deficiency complicated by myopathy. A 15-year-old boy was hospitalized because of a 3-week history of progressive muscle weakness, and edema. A physical examination on admission revealed prominent edema in the legs and muscle weakness and pain in the extremities, especially in the bilateral quadriceps femoris muscles. Deep tendon reflexes of the upper and lower extremities were absent. The patient could not walk without support or stand up from a squatting position. T2-weighted magnetic resonance imaging (MRI) in the legs demonstrated high signal intensities in the bilateral biceps femoris, quadriceps femoris, and gastrocnemius muscles. Motor nerve conduction velocities and sensory nerve conduction velocities in his median, ulnar, peroneal, and tibial nerves were within normal limits. Echocardiograms revealed right ventricular hypertension, pericardial effusion, and an increased ejection fraction. Peripheral blood analysis showed decreased blood thiamine levels (2.6 μg/dl) as well as decreased blood vitamin B2, vitamin B12, and folic acid levels. Therefore, we diagnosed multivitamin deficiency, and mainly thiamine deficiency, and he started to receive oral thiamine treatment (75 mg/kg/day) the day after admission. Thiamine supplements led to prompt drastic diuresis and improvements in cardiac failure symptoms, with a body weight loss of 8 kg within 4 days of treatment initiation. Muscle weakness also gradually improved and he could walk without support 9 days after beginning treatment. A detailed history identified that he had eaten mainly polished rice, approximately 2,000 kcal/day, and very few vegetables every day for the past 3-4 years. After educating him and his parents about nutrition, his body weight and height caught up to normal ranges. This case was important because the onset of the thiamine deficiency was in adolescent, who presented with myopathic syndrome, but no sensory disturbance, and was caused by extremely unbalanced nutrition.
|
|
|
Back number
|
|
