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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
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Vol.116, No.8, August 2012
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Original Article
Title
A Clinical Study of 117 Hospitalized Children with Influenza A/H1N1 2009 Infection
Author
Tadashi Ohshiro Ryo Imakiire Toaki Kohagura Tomoko Uehara Yuji Tobaru Youhei Arakaki Motoko Kamiya Suzuka Toguchi Tetsu Iha and Asao Yara
Department of Pediatrics, Naha City Hospital
Abstract
We conducted a clinical study of 117 hospitalized patients (age range, 0-15 years) who had been diagnosed with type A influenza (Influenza A/H1N1 2009 infection [novel influenza]) between August 2009 and January 2010 at Naha City Hospital. We compared the conditions of these patients with those of 41 patients with seasonal type A influenza who had been hospitalized between January 2008 and July 2009. There were no significant differences between the two groups with respect to the following parameters: rate of hospitalization, period from the onset of fever until hospitalization, length of hospital stay, C-reactive protein values on admission, the proportion of patients with asthma or a history of asthma from among the hospitalized patients with respiratory complications, the proportion of patients who had received long-term steroid inhalation therapy from among the hospitalized patients with asthma or a history of asthma, complication rates for neurological diseases (convulsions, abnormal behaviors, and encephalitis/encephalopathy), and digestive symptoms. The proportion of patients less than 3 months of age from among the hospitalized patients did not differ significantly between the two groups; the conditions of these patients were mild, with no particularly serious complications. However, the rate of patients with wheezing and that of the patients who had continuous isoproterenol inhalation therapy among the patients with respiratory complications as well as the age and the rate of respiratory complications were significantly higher in the novel influenza infection group than in the seasonal infection group.
The clinical characteristics of the patients with novel influenza differed from those of the patients with seasonal influenza. In the novel influenza group, the frequency of admissions for respiratory complications with wheezing was high. In the future, epidemics of novel and seasonal influenza may occur simultaneously. Therefore, rapid management and prevention of influenza is required, and particular attention must be paid to patients with wheezing.
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Original Article
Title
Risk Factors for Various Categories of Child Abuse in Kanagawa Children's Medical Center
Author
Koji Tanoue Kiyoshi Matsui and Atsuko Yamamoto
Department of General Medicine, Kanagawa Children's Medical Center
Abstract
There were 174 cases of child abuse during the 10 year period from 2000 to 2009 in Kanagawa Children's Medical Center. We examined age, sex, categories of maltreatment and risk factors based on medical records and those of case conferences. Risk factors were those specific to the child, parents and family, lack of support and a nuclear family. We also compared risk factors with other categories of abuse. Prevention is the most effective strategy against child abuse. It is effective, to some degree, to identify a child at high risk of abuse and then provide supportive intervention. We examined differences in risk factors for each category of maltreatment and considered how to implement preventive strategies. Although several organizations in the community and within the hospital need to communicate and work with each other, our present focus is hospital-based prevention of child abuse.
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Original Article
Title
A Four-Month Girl Case of Food Allergy Developed Anaphylactic Shock due to an Allergen in Breast Milk
Author
Fumihiro Ochi1)3) Kazuyo Kuzume2)3) Munemitsu Koizumi1) Yoshiaki Yano1) and Eiichi Ishii3)
1)Department of Pediatrics, Sumitomobessi Hospital
2)Department of Pediatrics, Ehime Prefectural Niihama Hospital
3)Department of Pediatrics, Ehime University Graduate School of Medicine
Abstract
We reported a case of a 4-month-old female infant with food allergy who developed anaphylactic shock due to an antigen in breast milk. Her serum total IgE level was 27 IU/ml. Egg-, milk-, and casein-specific IgE antibody titers were 1.19, 7.17, 9.96 UA/ml, respectively, and peanut-, sardine-, chicken-, and pork-specific IgE antibody titers were less than 0.34 UA/ml. In the skin prick test (SPT: prick-prick test), she developed immediate type reactions against egg, milk, and peanuts. SPT was useful for the diagnosis of food allergy in the infant. We need to be careful with severe hypersensitive reactions by food allergens in breast milk even in exclusively breast-fed infants.
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Original Article
Title
Beriberi Presenting Reversible Severe Pulmonary Hypertension by Excessive Intake of Isotonic Drink
Author
Hisako Kataoka Yoshio Arakaki Kenji Waki Tomohiro Hayashi Kayo Ogino Yoshiyuki Hanaoka Yosuke Hayama and Daisuke Yoshinaga
Department of Pediatrics, Kurashiki Central Hospital
Abstract
Beriberi is a cardiovascular and neurological related disease occurring due to the disturbance of carbohydrate metabolism resulting from a lack of vitamin B1. A clinical state in which there is a rapid hemodynamic deterioration is known as Shoshin Beriberi.
This case involved a girl of 17-months who showed failure to thrive since 8 months of age. She presented with tachypnea, oliguria and edema and was consequently admitted to this hospital. Blood tests showed a high BNP level (1,900 pg/ml) and metabolic acidosis due to an increasing level of lactate (50.2 mg/dl). Her echocardiography showed a flat interventricular septum and tricuspid regurgitation (3.68 m/sec) that revealed severe pulmonary hypertension. Left ventricular systolic performance was not poor. After admission, it was revealed that she had been consuming around 4 liters/day of isotonic drink since 8 months of age, with a subsequent decrease in her intake of milk and food. Shoshin Beriberi due to the lack of vitamin B1 was suspected because of the patient's history and physiological findings. The intake of isotonic drink was stopped and the patient was started on vitamin B1, and her symptoms rapidly improved. On day 5, recovery from pulmonary hypertension was evident on echocardiography. The BNP level decreased to the normal range within two weeks. The serum level of vitamin B1 before administration was low (12 ng/ml).
Characteristics of Shoshin Beriberi are metabolic acidosis, a drop in systemic vascular resistance and high-output heart failure. In some cases it was reported that pulmonary hypertension is evident on echocardiography; however the etiology of pulmonary hypertension due to the lack of vitamin B1 is unclear.
This case suggests the importance of considering this disease as a possible diagnosis, in a patient with high output heart failure and pulmonary hypertension. Recently, multiple cases involving beriberi as a result of excessive intake of isotonic drinks have been reported, suggesting that it may be necessary to enlighten parents about the optimal intake of isotonic drinks for their children.
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Original Article
Title
A Case Report of a Boy Who Came to the Hospital Because of Short Stature and Was Diagnosed with Danon Disease
Author
Mie Awaya1) Shiro Baba1) Shinji Kaichi1)3) Tomonari Awaya1) Minoru Shibata1)3) Takeo Kato1) Noritaka Yokoo1) Takahiro Mima1) Chieko Seino2) Ichizo Nishino2) Ikuya Nonaka2) Toru Yorifuji1) Hiraku Doi1) Tatsutoshi Nakahata1) and Toshio Heike1)
1)Department of Pediatrics, Kyoto University Hospital
2)Department of Ultrastructual Research, National Institute of Neuroscience, National Center of Neurology and Phychiatry
3)Department of Pediatrics, Ako City Hospital
Abstract
Danon disease is a rare X-linked dominant lysosomal disorder caused by a mutation of the lysosome-associated membrane protein-2 (LAMP2) gene. This disease is characterized by a clinical triad of cardiomyopathy, myopathy, and mental retardation. In these phenotypes, cardiomyopcthy is the most common for all the patients, whereas the other two phenotypes vary. Here, we report a 13-year-old boy who came to the hospital to consult about his short stature and hypertrophic cardiomyopathy. His mother and sister also had a history of cardiomyopathy. Even after various examinations, we could not achieve a definitive diagnosis. Therefore, we performed cardiac and skeletal muscle biopsies. Skeletal muscle biopsy revealed characteristic features of autophagic vacuolar myopathy, which is typically observed in patients with Danon disease. In addition, LAMP-2 protein was not detected by Western blotting, and sequence analysis of the LAMP2 gene revealed a nonsense mutation in exon 7 (c.877C>T, R293X). Finally, we diagnosed this patient with Danon disease. Recent studies show that the estimated prevalence of LAMP-2 deficiency is 2.7-4.0% among unrelated patients with hypertrophic cardiomyopathy the cause of which is unknown. The possibility of Danon disease should be considered in young patients with idiopathic cardiomyopathy. Moreover, it is important to perform muscle biopsies to diagnose with Danon disease.
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Original Article
Title
The Efficacy of Donepezil Treatment on the Activity of Daily Life and Growth Rate in a 5-Year-Old Boy with Down Syndrome
Author
Yoshikazu Otsubo1) Yoko Ushiroda1) Tatsuro Kondoh2) and Hiroyuki Moriuchi3)
1)Department of Pediatrics, Sasebo Municipal General Hospital
2)Division of Developmental Disabilities, Misakaenosono Mutsumi, The Institute for Persons with Severe Intellectual/Motor Disabilities
3)Department of Pediatrics, Nagasaki University Hospital
Abstract
Down syndrome (DS) patients share certain neuropathological features with Alzheimer disease (AD) patients. Aside from AD-like dementia, DS patients occasionally develop neurobehavioral disorders of unknown causes that lead to rapidly progressive deterioration of their activities of daily lives in adolescence or early adulthood. Some DS children aged 10 or younger develop voiding dysfunction, possibly associated with cholinergic abnormalities. The anti-AD drug donepezil, a cholin esterase inhibitor, has been shown to effectively improve the activities of daily life in DS patients; however, its efficacy and safety in children with DS remain unknown. We herein report the successful treatment with donepezil of a 5-year-old boy with DS who had been suffering from intractable anorexia and an autistic state. To the best of our knowledge, he is the youngest patient treated with donepezil, and his clinical state and prompt response to donepezil treatment suggest that even young children with DS have a risk of progressive deterioration of behavioral and mental state that apparently results from transient or reversible cholinergic abnormalities.
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