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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
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Vol.120, No.5, May 2016
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Review
Title
The Correlation between Anthropometric Measurement and Serum Protein Levels in Children with Severe Motor and Intellectual Disabilities
Author
Yukie Higashiyama1) Masaru Kubota1) Ayako Nagai1) Yoshihiro Wakazono2) Chiharu Kawaguchi3) and Kiyotaka Tomiwa3)
1)Department of Human Life and Environment, Nara Women's University
2)Department of Pediatrics, Kyoto Katsura Hospital
3)Department of Pediatrics, Todaiji Medical and Educational Center
Abstract
Children with severe motor and intellectual disabilities (SMID) are often evaluated as malnutrition because of their diminished growth. This study aimed at clarifying the correlation between anthropometric measurement including height (Ht), body weight (BW), mid-upper arm circumference, triceps skin-fold thickness and mid-upper arm muscle circumference, and biochemical markers including albumin (Alb), transthyretin (TTR), retinol binding protein (RBP) in children with SMID. The subjects consisted of 28 children (16 boys, 12 girls) aged 3 to 20 years old with SMID. Anthropometric measures and blood examination were performed twice, i.e. an initial measurement (baseline) and at 12 months afterwards. Children with SMID had lower baseline anthropometric measures (i.e. Ht, BW, BMI and the percentage of overweight), and Alb levels than those of controls. No z score of anthropometric measures showed significant correlation with the levels of serum proteins. BMI and BW z scores of subjects decreased significantly at 12 months later. However, the magnitude of changes of anthropometric measurements and serum protein levels during the one year period did not show any correlation.
This study may indicate that we should be careful when we try to evaluate the nutritional status of SMID children based on serum proteins.
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Original Article
Title
The Implementation of an Allergy Hotline in Schools and Childcare Facilities
Author
Kenichi Akashi1) Toshio Katsunuma1) Ryohei Suzuki1) Takashi Inoue1) Tae Yanagisawa1) Takanori Motoki1) Yuko Yamauchi1) Eiichiro Tamura1) Masako Watanabe1) and Hiroyuki Ida2)
1)Department of Pediatrics, Jikei University Daisan Hospital,
2)Department of Pediatrics, Jikei University School of Medicine
Abstract
Although safety measures exist for children with food allergies (FAs) in schools and childcare facilities, including the adoption of management sheets and guidelines, death from anaphylaxis (An) occurred in 2012 in an elementary school in Chofu, Tokyo. This case revealed the insufficiency of the existing measures, which prompted us to collaborate with Chofu and Komae townships to formulate and implement additional measures (workshops/continuous education, personal emergency instruction cards, and a Allergy Hotline [HL]). The HL service is the first national attempt to establish a medical collaboration system specializing in allergic symptoms among children in schools and childcare facilities. We compiled data from the first year of its implementation. The HL was used 35 times (average, 2.9 cases/month; 8.1±4.6 years). We found 12 cases of An. Of 26 FA-related cases, 7 (26.9%) were induced by known food sources. Although 18 cases (69.2%) were caused by unknown food sources, all of the patients were treated appropriately via the HL. For An, the average duration from symptom onset to discovery was 11 minutes, which tended to be longer in junior high school students, at 39 minutes. Among the cases induced by known food sources, 2 were An-attributable to oral immunotherapy (OIT). Risks associated with OIT are normally the responsibility of the administering medical institution or the student's family. Therefore, its administration method should be reconsidered. Our approach may serve as a useful reference for future efforts to establish collaboration between schools and childcare facilities and community pediatric medical centers.
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Original Article
Title
Evaluation of SBO Screening with Plain Abdominal X-ray Images for Cases with LUTS
Author
Hirokazu Ikeda1) Tsuneki Watanabe1) Masaki Fuyama1) Keiichi Isoyama1) and Takaki Hayashi2)
1)Department of Pediatrics, Showa University Fujigaoka Hospital
2)Department of Radiology, Showa University Fujigaoka Hospital
Abstract
[Objectives] Several studies have reported that lower urinary tract symptoms (LUTS), such as nocturnal enuresis (NE) and daytime incontinence (DI), have higher incidence rates in children with spina bifida occulta (SBO) than in the general population. Furthermore, they report that NE and DI cases complicated by SBO are resistant to treatment, but these conditions are still being debated. We examined SBO incidence rates in children with NE and DI and investigated the effect of SBO on the treatment response for NE and DI.
[Method] Subjects were pediatric patients between 5 and 15 years of age who were examined at our outpatient department for DI or NE over nine years. LUTS was defined as three of more episodes of NE per week and one or more episodes of DI per week. Cases in which plain abdominal X-ray images showed vertebral arch nonfusion in the lumbosacral spine were defined as SBO positive. Subjects comprised a total of 428 cases, with 253 cases of NE and 175 cases of DI. A total of 121 (28.3%) cases were SBO positive, including 85 with NE (33.6% of the total NE cases) and 36 with DI (20.6% of the total DI cases). This was mostly consistent with incidence rates for the general population. No significant differences were observed between SBO-positive and SBO-negative cases for response rates at 3 months after treatment for NE and DI or for the proportion of cases that were cured within 2 years of treatment. Our investigation of short-term and long-term treatment effects for NE and DI and SBO complication rates indicates that it is not imperative to perform SBO screening with plain abdominal X-ray images for cases with LUTS such as NE and DI.
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Original Article
Title
The Prevalence of Functional Constipation and Its Relationship with Dietary Habits among Japanese Children Aged 3-9 Years
Author
Asami Fujitani1)7) Masumi Okuda2) Tsuyoshi Sogou3) Shinobu Ida4) Yukiko Nishimoto5) Takeshi Tomomasa6) and Kiyoshi Kawakubo7)
1)Department of Nutritional and Food Management, Saiseikai Yokohamashi Tobu Hospital
2)Department of Pediatrics, Sasayama Medical Center, Hyogo College of Medicine
3)Department of Pediatrics Hepatology and Gastroenterology, Saiseikai Yokohamashi Tobu Hospital
4)Department of Gastroenterology, Nutrition and Endocrinology, Osaka Medical Center and Research Institute for Maternal and Child Health
5)Department of Nutritional and Food Management, Osaka Medical Center and Research Institute for Maternal and Child Health
6)PAL Clinic
7)Kyoritsu Women's University
Abstract
[INTRODUCTION] There are almost no Japanese reports about the prevalence of childhood functional constipation and its relationship with lifestyle and dietary habits because defecation is different for each person, and it is difficult for children to recognize symptoms of constipation by themselves. [METHODS] A questionnaire concerning defecation, lifestyle, and dietary habits was administered to parents of 1,277 children attending nursery or elementary schools in the Sasayama district of Hyogo prefecture. Seven hundred twelve parents (55.8%) responded, and 634 children aged 3-9 years were included in the study. Ninety-four children (14.6%), including four undergoing constipation treatment, were diagnosed with functional constipation according to the Rome III criteria and 549 were diagnosed with nonfunctional constipation. [RESULTS] When compared between two groups (i.e., functional constipation vs. nonfunctional constipation), the frequency of a late or irregular wake-up time (P = 0.012), an irregular defecation time (P <0.001), consumption of a main dish at breakfast (P = 0.002), no rice at breakfast (P = 0.004), and a few or no side dishes at supper (P <0.001) were significantly higher among the functional constipation group. Even if age was adjusted, the functional constipation group consumed significantly less side dishes at supper than the nonfunctional constipation group (P <0.001). [CONCLUSION] The incidence of functional constipation among Japanese children was 14.5%, which is very high. However, there was a very low rate of treatment (0.6%). The epidemiological observation in this study suggests that the incidence of constipation is affected by lifestyle and dietary habits, i.e., the intake timing of rice and vegetable dishes.
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Original Article
Title
Chloral Sedatives in Combination with Intravenous Midazolam for Pediatric Sedation in MRI
Author
Takuto Takahashi1) Yoshihiko Morikawa2) Kazuhisa Akiba1) Saki Akatsuka1) Yumi Ikari1) Shun Kishibe1) Yoshikazu Kitami1) Junpei Kuroda1) Naoki Takemoto1) Naoko Miwa1) Tatsuo Kono3) and Yukihiro Hasegawa1)
1)Department of General Pediatrics, Tokyo Metropolitan Children's Medical Center
2)Clinical Research Support Center, Tokyo Metropolitan Children's Medical Center
3)Department of Diagnostic Radiology, Tokyo Metropolitan Children's Medical Center
Abstract
Background: Despite its widespread use in Japan, oral triclofos is considered both unsafe and ineffective, especially when used alone. We hypothesized that the standard dose of oral or rectal chloral sedatives combined with intravenous midazolam (the CM method) would improve efficacy and safety.
Objective: To investigate the efficacy and safety of the CM method.
Methods: We conducted a prospective observational study of patients who underwent MRI between 6 months and 6 years of age. Those with a higher risk of adverse effects under sedation were excluded. Chloral sedatives (Oral triclofos: 80 mg/kg or rectal chloral hydrate: 30-50 mg/kg) were used as the initial sedative, followed by intravenous midazolam (single dose 0.1-0.2 mg/kg, maximum dose 0.6 mg/kg) for inadequately sedated patients. The primary outcome was the rate at which the patient was successfully sedated to allow MRI image quality sufficient for diagnostic purposes.
Results: Twenty-eight patients were enrolled in this study, of which 13 were male and 15 were female. The mean age was 26 months (standard deviation: 16). Of the 23 who underwent successful sedation, oral triclofos was the only sedative used in 7, while intravenous midazolam was added in 16. Nine of 23 (39%) started MRI scan in time, and the median delayed time was 9.5 minutes (range 1-30). No serious adverse event was observed.
Conclusion: Although the CM method was designed as a safety-orientated approach, it was inadequate in efficacy.
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Case Report
Title
Successful Living Donor Liver Transplantation for Fulminant Hepatic Failure in a Patient with Neonatal Hemochromatosis
Author
Yoshikazu Otsubo1) Rieko Sakamoto2) Taro Kanbe1) Muneichiro Sumi1) Tatsuya Kawasaki2) Yukihiro Inomata3) and Hiroshi Mitsubuchi4)
1)Department of Pediatrics, Sasebo City General Hospital
2)Department of Pediatrics, Kumamoto University Hospital
3)Department of Pediatric Surgery and Transplantation, Kumamoto University Hospital
4)Department of Neonatology, Kumamoto University Hospital
Abstract
Neonatal hemochromatosis (NH) is a clinical condition in which severe liver disease in the newborn is accompanied by extrahepatic siderosis. Liver transplantation is indicated particularly in patients with hepatic failure resistant to medical therapies. The pathology of this disease is entirely different from that of hereditary hemochromatosis due to abnormal iron metabolism; thus, the condition is proposed to be a disorder of gestational alloimmune liver disease (GALD) associated with maternal-fetal alloimmunity.
We report a 14-day-old patient with neonatal hemochromatosis. The patient was hospitalized with vomiting and jaundice. At the time of admission, hepatic failure accompanied by decreased consciousness level was noted and the diagnosis of neonatal hemochromatosis was determined by abdominal magnetic resonance imaging. The patient's condition was resistant to medical therapies such as immunoglobulin administration, exchange blood transfusion, and continuous hemodialysis. Living donor liver transplantation from his grandfather was successfully performed on the patient at 28 days of age, saving the patient's life.
Histological findings of the extracted liver showed very few preserved hepatocytes accompanied by significant atrophy. Furthermore, it showed bile duct proliferation and fibrosis around the central veins. Iron staining revealed extensive siderosis.
Practitioners should consider GALD-NH in case of neonatal acute liver failure. Identification of infants with GALD-NH is important as treatments including a liver transplantation are available and effective for subsequent pregnancies.
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Case Report
Title
Delayed Onset of Cerebellar Ataxia and Altered Mental Status due to Mumps Vaccination
Author
Marie Sasaki1) Yuri Sakaguchi1) Toshiki Takenouchi1) Naomi Shinohara1) Masayoshi Shinjoh1) Tetsuo Nakayama2) and Takao Takahashi1)
1)Department of Pediatrics, Keio University School of Medicine
2)Laboratory of Viral Infection, Kitasato Institute for Life Sciences
Abstract
A 15-month-old boy presented with fever and seizures 4 weeks after receiving mumps vaccination. He had multiple seizures with a lymphocytic pleocytosis in his cerebrospinal fluid. There were no parotid swellings, or elevated serum amylase. On the fifth day of admission, the patient defervesced and began to exhibit cerebellar ataxia and altered mental status. He was unable to sit or control his head, and was uninterested in the external stimuli. These neurological symptoms persisted for two days, and then gradually subsided. Mumps virus was isolated from the cerebrospinal fluid. Genotyping of the isolated mumps virus showed Hoshino strain, which had been administered to the patient 32 days prior to the clinical manifestation. Herein, we report an infant with mumps meningoencephalitis, who exhibited delayed onset and reversible cerebellar ataxia and altered mental status. The strain of the mumps virus isolated from the patient's cerebrospinal fluid, i.e., Hoshino strain, was identical to that of the preceding mumps vaccination. In most cases, adverse events of mumps vaccination become apparent 2 to 3 weeks after vaccination. However, in patients who presented after longer latency period, a thorough investigation should be undertaken to rule out possible vaccine adverse events.
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Case Report
Title
A Case of Bilateral Vaginal Atresia and Pyocolpos Accompanied by Unilateral Renal Agenesis
Author
Naomi Kawasaki Motoi Kawasaki Toshiyuki Shinozaki Saori Tanabe and Toshiyuki Kimura
Department of Pediatrics, Nihonkai General Hospital
Abstract
This case study describes a 14-year-old girl given a diagnosis of right renal agenesis at age 2 months. Menarche had occurred at 10 years of age, and dysmenorrhea was not reported. The patient presented with mild fever, malaise, and lower abdominal pain on walking and urination, as well as a change in urine color during the previous month. Antibiotics were administered initially for a suspected urinary infection because of urine turbidity; however, the fever did not resolve. Computed tomography (CT) scans and magnetic resonance imaging (MRI) revealed uterus didelphys and bilateral vaginal atresia, which was highly purulent, requiring drainage by transvaginal fenestration. Ultimately, the patient was diagnosed with obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome, a congenital uterine malformation induced by aberrant development of the Mü llerian duct. Because of the low incidence and complicated pathology of the OHVIRA syndrome, its early diagnosis is challenging. If uncontrolled, chronic intrapelvic inflammation may cause a decline in fertility, and an invasive surgical procedure may be necessary. The hallmarks of OHVIRA syndrome are uterus didelphys, hemivaginal obstruction, and ipsilateral renal agenesis. On the contrary, pyocolpos associated with bilateral vagina atresia is rarely reported. Although it is probable that the left vaginal walls had several perforations in this case, the patient did not have menstrual molimina syndrome. Therefore, an accurate diagnosis had not been made previously. If renal agenesis is suspected, examination for genital system abnormalities should be considered. This study suggests that minimally invasive surgery may be performed before menarche or development of pyocolpos.
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Case Report
Title
Lethal Pneumococcal Meningitis in an Infant Previously Vaccinated with 3 Doses of 13-valent Pneumococcal Conjugate Vaccine
Author
Aisa Moriyama1) Eri Konishi1) Mitsuru Endo1) Keisuke Wada1) Tsuyoshi Higuchi1) Daisuke Koike1) Yuri Uchida1) Hitoshi Sejima1) and Yasuhiro Tsuji2)
1)The Department of Pediatrics, Matsue Red Cross Hospital
2)The Department of Pediatrics, Matsue City Hospital
Abstract
The 7-valent pneumococcal conjugate vaccine (PCV) that was introduced in 2010 led to a dramatic decline in the number of children suffering from invasive pneumococcal disease in Japan. Pneumococcal meningitis has not been reported following the implementation in 2013 of the 13-valent PCV (PCV13), which contains an additional 6 serotype-specific antigens. Herein, we report the case of an infant with lethal pneumococcal meningitis who had received 3-doses of PCV13. A 5-month-old boy was brought to the local hospital with complaints of vomiting and hyperpyrexia. The next day, he was transferred to our hospital for critical care for status epilepticus and deteriorating respiratory failure. His anterior fontanelle was distended. Cerebrospinal examination confirmed that the boy was infected with pneumococcal meningitis. Meropenem, Ceftriaxone and dexamethasone were immediately initiated; however, he died after 4 hours. The bacterial examination demonstrated the presence of pneumococcus serotype 15, a serotype that is not included in PCV13, in his blood and cerebrospinal fluid. Our case represents a very rare condition that had escaped the PCV13 vaccination. Physicians need to be aware of the risk of pneumococcal meningitis caused by serotypes that are not covered by PCV13.
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Case Report
Title
Association between Localization of an Infantile Hemangioma and Regional Congenital Anomaly of PELVIS Syndrome
Author
Naoto Sakumura Yasuhiro Ikawa Masaki Shimizu and Akihiro Yachie
Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University
Abstract
We report a case of perineal hemangioma, external genital malformations, lipomyelomeningocele, vesicorenal abnormalities, imperforate anus and skin tag (PELVIS) syndrome in which the perineal hemangioma was misdiagnosed as diaper rash at birth. Examination demonstrated anterior displacement of the anus and a bifid scrotum. Magnetic resonance imaging of the pelvis showed no gross anomalies in other visceral structures. Purplish discoloration in the perineal area gradually increased in size and became a perineal hemangioma, leading to the diagnosis of PELVIS syndrome, which is rarely associated with perineal infantile hemangioma and underlying abnormalities. Thorough physical examination of the abdomen, genitals and lower extremities is warranted to clarify whether other anomalies are present in the same anatomical region. We report a case of PELVIS syndrome and discuss the mechanisms underlying the anatomical coincidence of hemangioma and related congenital abnormalities.
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Brief Report
Title
Add-on Omalizumab in Patients with Severe Asthma
Author
Hiroaki Nakamura1)2) Masako Watanabe2) Kenichi Akashi2) and Toshio Katsunuma2)
1)Department of Pediatrics, Toho University Ohashi Medical Center
2)Department of Pediatrics, Jikei University Daisan Hospital
Abstract
Over the past few years, asthma has been mitigated as a result of advances in asthma medication and the widespread adoption of treatment guidelines. However, some patients have persistent asthma that is poorly controlled with existing treatments, such as inhaled corticosteroids. This issue has yet to be resolved. Patients with severe persistent asthma represent about 5% of all patients with asthma but account for 50% of the health care costs for asthma. We report 3 patients with severe asthma who were given omalizumab. Two of the 3 patients responded to omalizumab, and all 3 patients tolerated omalizumab well.
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