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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY

Vol.122, No.12, December 2018


Original Article
1. Neurodevelopmental Outcomes in Children with Congenital Heart Disease Born with Very Low Birth Weight
2. Comparison of Clinical Characteristics between Primary Urinary Tract Infections Caused by Either Extended-spectrum β-lactamase (ESBL)-producing or non-ESBL-producing Escherichia Coli
3. Lesion Distribution in Pediatric Pneumonia Diagnosed by Ultrasound and a Comparison of Sensitivity with Chest Radiography
Case Report
1. Non-IgE Mediated Gastrointestinal Food Allergy in Neonates and Infants with Persistent Hypertransaminasemia
2. A Case of a 45,X/47,XYY Turner Syndrome with Normal Female Internal and External Genitalia


Original Article
Title
Neurodevelopmental Outcomes in Children with Congenital Heart Disease Born with Very Low Birth Weight
Author
Yasumi Nakashima1) Tomotaka Murakami1) Nao Inoue1) Sachie Kaneko1) Etsuko Hirose2) and Yoshiki Mori1)
1)Division of Pediatric Cardiology, Seirei Hamamatsu General Hospital
2)Division of Neonatology, Seirei Hamamatsu General Hospital
Abstract
Objectives: To elucidate neurodevelopmental outcomes in school-age children with congenital heart disease (CHD) born with very low birth weight (VLBWI).
Methods: We reviewed clinical records from VLBWI admitted to our neonatal intensive care unit from 2000 through 2010 and who had survived up to six years of age. We divided the children into CHD and non-CHD groups. Frequencies of neurological outcomes (cerebral palsy, mental retardation (MR) and developmental disorder), full scale IQ, and special educational needs were evaluated.
Results: We enrolled 503 patients (CHD: n=30, non-CHD: n=473) were enrolled in this study. Relative frequencies of cerebral palsy and developmental delay were equal (CHD vs. non-CHD, 20% vs. 10.4%, 16.7% vs. 14.6%, respectively). MR was significantly more common in the CHD group (CHD vs. non-CHD; 56.7% vs. 24.5%). The advancement rate to regular schools was lower in the CHD group (CHD vs. non-CHD; 40% vs. 75.5%). Comparison of CHD and non-CHD patients excluding chromosomal abnormalities showed that MR and special educational needs were more frequent in the CHD group. Comparison between surgery (n=11) and no-surgery patients in CHD (n=12) revealed that the frequencies of neurological outcomes and special educational needs were not significantly different between the two groups.
Conclusions: Many CHD with VLBWI patients have severe mental retardation that requires educational support.




Original Article
Title
Comparison of Clinical Characteristics between Primary Urinary Tract Infections Caused by Either Extended-spectrum β-lactamase (ESBL)-producing or non-ESBL-producing Escherichia Coli
Author
Katsuaki Kasahara Hisakazu Mazima and Yoshimitsu Gotoh
Department of Pediatric Nephrology, Japanese Red Cross Nagoya Daini Hospital
Abstract
Introduction: The increasing number of extended-spectrum β-lactamase (ESBL)-producing bacterial infections is problematic. Methods: We investigated annual trends in positivity for ESBL-producing Escherichia coli (E. coli). We also compared clinical characteristics between groups of ESBL-producing E. coli UUTIs (Group A) and non-ESBL-producing E. coli UUTIs (Group B). Results: Annual percentages of ESBL-producing E. coli from all E. coli identified in pediatric urine and stool cultures from 2011 to 2017 indicated an increasing trend. Annual percentages of ESBL-producing E. coli in primary UUTI patients identified from 2011 to 2016 also increased. Although no significant differences were observed between Groups A and B regarding gender, age, urinary tract morphological abnormalities, vesicoureteral reflux, 99Tc-DMSA renal scarring, or kidney injury, Group A had a longer fever course after antibiotic initiation than Group B (P = 0.03). Fourteen patients were resistant to the primary antibiotic; two of them continued the primary antibiotic. Twelve patients were switched to cefmetazole, which is sensitive to ESBL-producing E. coli, resulting in successful treatment without adverse effects. Conclusion: We observed an increasing trend of ESBL-producing E. coli UUTIs from 2011 to 2016. ESBL-producing E. coli infections had a more delayed response to antibiotic treatment than non-ESBL-producing E. coli infections, but we found no significant differences regarding renal scarring between the two types of UUTIs.




Original Article
Title
Lesion Distribution in Pediatric Pneumonia Diagnosed by Ultrasound and a Comparison of Sensitivity with Chest Radiography
Author
Koji Fuchimoto and Yuri Ishida
Department of Pediatrics, Otsu Red Cross Shiga Hospital
Abstract
Purpose: This study aimed to elucidate the distribution of lesions in pediatric pneumonia diagnosed by ultrasound and to clarify the impact of lesion location and patient age on chest radiography and ultrasound sensitivity. Methods: The subjects were all patients hospitalized in our pediatrics department between November 2012 and October 2017 following a diagnosis of pneumonia after lesions were observed on chest radiography or ultrasound. The main set comprised those patients confirmed to have lesions by ultrasound. The lesion locations were divided into 8 segments (right/left, anterior/posterior, upper/lower) and compared by patient age. A subset was also created of patients who underwent both ultrasound and chest radiography aggregated by age for each of the 4 lung field segments (right/left, upper/lower) to find and compare the respective sensitivities. Results: The total number of pneumonia patients was 406. The main set comprised 200 and the subset comprised 256. Lesions were concentrated in the right and lower segments on ultrasound. Children < 1 year old had significantly more lesions in the upper and posterior segments compared with children aged ≥ 1 year. Ultrasound sensitivity exceeded that of chest radiography in three segments, except the lower right segment, in children < 1 year old and in the lower left segment in children aged ≥ 1 year. Conclusions: In diagnoses of pediatric pneumonia by ultrasound, lesions are common on the upper dorsal side in children < 1 year old and in the lower segment in children aged ≥ 1 year. In terms of sensitivity, ultrasound is the most useful diagnostic tool for pneumonia in children < 1 year old and is very useful for diagnosing pneumonia in the lower left segment of the lung in children aged ≥ 1 year.




Case Report
Title
Non-IgE Mediated Gastrointestinal Food Allergy in Neonates and Infants with Persistent Hypertransaminasemia
Author
Hiroaki Nakamura1) Miku Hattori1) Tsuyoshi Sogo2) Ayano Inui2) Tomoo Fujisawa2) and Norikazu Shimizu1)
1)Department of Pediatrics, Toho University Ohashi Medical Center
2)Department of Pediatric Hepatology and Gastroenterology, Saiseikai Yokohamashi Tobu Hospital
Abstract
Non-IgE mediated gastrointestinal food allergies have witnessed an upsurge recently. Although clinicians attempt to diagnose and treat affected patients, these allergies are challenging to diagnose in patients classified with atypical types. Here, we report the case of a 51-day-old boy with failure to thrive and hypertransaminasemia. He developed non-IgE mediated gastrointestinal food allergies, as a septicemia-like phenotype, after consuming a cow's milk formula. After discontinuing the cow's milk formula, an amino acid formula was started, to which he developed no allergic reaction and soon gained body weight. However, hypertransaminasemia persisted. Then, he experienced three episodes of allergic reactions after consuming cow's milk products; transaminase levels were markedly elevated during each episode. After approximately 2 years, he acquired tolerance to cow's milk, and transaminase levels were completely normalized. It remains unclear whether normalized transaminases levels were associated with the acquired tolerance to cow's milk. However, to the best of our knowledge, no case of non-IgE mediated gastrointestinal food allergy in neonates and infants with persistent hypertransaminasemia has been reported.




Case Report
Title
A Case of a 45,X/47,XYY Turner Syndrome with Normal Female Internal and External Genitalia
Author
Kaoru Maruyama Hiromi Nyuzuki Sunao Sasaki Yohei Ogawa Keisuke Nagasaki and Akihiko Saitoh
Department of Pediatrics, Niigata University Medical & Dental Hospital
Abstract
Turner syndrome (TS) represents a common sex chromosome aberration. Most patients with TS have a 45,X karyotype, while the others have either mosaic karyotypes or structural X or Y chromosomal abnormalities. TS patients rarely present with a 45,X/47,XYY karyotype. Many of these patients present abnormal internal and external genitalia.
Herein, we report the case of a 5-year-old girl with short stature, a height of 100.3 cm (−2.2 SD), hypoplastic fingernails, and normal external genitalia. Her past medical history was significant only for multiple episodes of otitis media. She was diagnosed with TS based on the presence of ovarian dysfunction with a 45,X/47,XYY karyotype ascertained by G-banding of the peripheral blood lymphocytes. Her uterine size was consistent with her age and her internal genitalia were normal in appearance on an abdominal MRI. She underwent bilateral gonadectomy due to concern for gonadal malignancy, based on the presence of the Y chromosome. She had streak gonads but no evidence of malignancy. The predominant karyotype of her buccal mucosa and resected gonads was 45,X. Patients with TS 45,X/47,XYY mosaicism usually have abnormally appearing internal and external genitalia, regardless of their sex. This patient with TS had a 45,X/47,XYY karyotype, but had normal female internal and external genitalia. We surmised that the predominance of the 45, X cell lineage in her gonads led to her normally appearing female genitalia.




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