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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY

Vol.122, No.3, March 2018


Original Article
1. Significance of Early Diagnosis and Measurement of Total Kidney Volume in Children with Autosomal Dominant Polycystic Kidney Disease
Case Report
1. Swallow Syncope Associated with Paroxysmal Atrioventricular Block in a Young Girl
2. A Case of Guillain-Barré Syndrome with Headache as the Primary Symptom, Followed by Neck Stiffness
3. The Clinical Course of Enzyme-replacement Therapy in Patients with Severe Hypophosphatasia
4. Lactic Acidosis Following Procaterol Overdose during Bronchial Asthma Attack


Original Article
Title
Significance of Early Diagnosis and Measurement of Total Kidney Volume in Children with Autosomal Dominant Polycystic Kidney Disease
Author
Shinichiro Ohara1)3) Kenichiro Miura1) Yuko Akioka1)4) Masaki Yoshida2) Naoto Kaneko1) Tomoo Yabuuchi1) Yuri Nawashiro1) Norimasa Tada1)5) Hiroyuki Miyai1)6) Shoichiro Kanda1)7) Noriko Sugawara1)8) Kiyonobu Ishizuka1) Hiroko Chikamoto1) Yukihiko Kawasaki3) and Motoshi Hattori1)
1)Department of Pediatric Nephrology, Tokyo Women's Medical University
2)Department of Pediatrics, Tokyo Women's Medical University, Yachiyo Medical Center
3)Department of Pediatrics, Fukushima Medical University
4)Department of Pediatrics, Saitama Medical University
5)Department of Pediatrics, Tokyo Medical and Dental University
6)Department of Pediatrics, Okayama University
7)Department of Pediatrics, Tokyo University
8)Department of Pediatrics, Sendai Red Cross Hospital
Abstract
Background: Autosomal dominant polycystic kidney disease (ADPKD) in children is generally considered to be a benign condition, with morbidity manifesting in adulthood. Therefore, the diagnostic screening of children at risk is controversial. On the other hand, previous reports showed that an increase in total kidney volume (TKV) was associated with progression of chronic kidney disease in children with ADPKD.
Methods: A retrospective chart review was performed in 19 children with ADPKD aged less than 15 years old at diagnosis between 2001 and 2015 in a single center. TKV was measured using a CT/MRI volumetric measurement method.
Results: TKV was smaller than 750 ml/1.73 m2 in 13 patients (Group A) and larger than 750 ml/1.73 m2 in 6 patients (Group B). Eleven of the 13 children in Group A were asymptomatic, while all the children in Group B were diagnosed based on symptoms such as abdominal pain and/or macroscopic hematuria. Hypertension was more frequently noted in Group B (5 patients, 83.3%) than in Group A (2 patients, 15.4%) (p< 0.05). CKD stage ≥3 at last follow up was more frequently noted in Group B (3 patients, 50.0%) than in Group A (1 patient, 7.7%) (p< 0.05). The decrease rate of estimated glomerular filtration rate tended to be larger in Group B than in Group A.
Discussion: These results demonstrate that the measurement of TKV in ADPKD children might be helpful for the prediction of renal outcome. We suggest that at-risk children should receive blood pressure management in the early phase of the disease, and novel therapies including tolvaptan should be considered for minimizing cystic disease progression.




Case Report
Title
Swallow Syncope Associated with Paroxysmal Atrioventricular Block in a Young Girl
Author
Ayako Kuraoka1) Hiroya Ushinohama1) Michihiko Uchida2) Kenji Furuno2) Makoto Nakamura1) Kouichi Sagawa1) and Shiro Ishikawa1)
1)Division of Pediatric Cardiology, Fukuoka Children's Hospital
2)Division of General Medicine, Fukuoka Children's Hospital
Abstract
Swallow syncope is a rare cause of syncope that belongs to the neurally-mediated reflex syncopal syndromes. This syndrome is more common in middle-aged and older men, sometimes associated with esophageal and cardiac disease.
A 9-year-old girl presented with a history of syncopal episodes since the age of 7 years. Syncope occurred only while eating solid foods. She described the episodes as dizziness followed by visual disturbances and hearing loss. The physical examination, electrocardiography, echocardiography, electroencephalogram, head CT and MRI were within normal limits. The Holter-ECG revealed multiple episodes of paroxysmal atrioventricular block with up to a 5.1 second pause associated with swallowing. She underwent implantation of a single chamber permanent pacemaker and her symptoms resolved.
The diagnosis can be suspected on the basis of clinical presentation and confirmed with the demonstration of transient brady-arrhythmia during swallowing. Medical management includes avoiding trigger foods and implantation of a permanent cardiac pacemaker.




Case Report
Title
A Case of Guillain-Barré Syndrome with Headache as the Primary Symptom, Followed by Neck Stiffness
Author
Nana Mizuno1) Satoru Kobayashi1) Tomoya Yoshida2) Naomi Kamioka1) Naoki Hamajima1) Kanji Muramatsu1) and Satoshi Suzuki1)
1)Department of Pediatrics, Nagoya City West Medical Center
2)Department of Pediatrics, Nagoya City East Medical Center
Abstract
Here, we report a 9-year-old girl with Guillain-Barré syndrome (GBS), who presented with prolonged severe headache as a primary symptom. She had suffered from acute sinusitis about 2 weeks before admission. On admission, she complained of systemic pain in addition to headache. Despite exaggerated deep tendon reflexes (DTRs) in the lower extremities, the results of the cerebrospinal fluid examination, nerve conduction studies, and magnetic resonance imaging, which showed marked enhancement of the nerve roots in the cauda equina after gadolinium injection, led to a diagnosis of GBS. Her symptoms improved after intravenous immunoglobulin therapy. DTRs were preserved throughout the clinical course. Although areflexia is among the clinical criteria for a diagnosis of GBS, DTRs may be exaggerated or preserved throughout the clinical course. Headache is atypical symptom of GBS, even if it is not an initial symptom. To avoid delays in the diagnosis and treatment of pediatric GBS, it is important to recognize atypical cases with unexplained headache, exaggerated or preserved DTRs, and neck stiffness suggesting central nervous system infection.




Case Report
Title
The Clinical Course of Enzyme-replacement Therapy in Patients with Severe Hypophosphatasia
Author
Hideomi Asanuma1) Yosuke Kosugi1) Akira Ishii2) and Hotaka Kamasaki2)
1)Department of Neonatology, Hokkaido Medical Center for Child Health and Rehabilitation
2)Department of Pediatrics, Sapporo Medical University
Abstract
We report the effects of 3 years of enzyme replacement therapy performed in a boy since the age of three months for severe hypophosphatasia. Long bones with significant rickets-like changes, a thorax with bell-shaped changes, and thinned ribs were seen during pre-treatment observation but showed evident changes two months after the treatment began. Rickets-like symptoms mostly disappeared at 12 months and the bell-shaped thorax improved at 7 months. The main cause of his respiratory distress was due to tracheomalacia; however, it improved after 5 months of treatment and he could be weaned off mechanical ventilator. Regarding motor development, head control was not seen at 10 months, although he was able to sit independently at 18 months and walk independently at 25 months. At 36 months, he showed age-appropriate development in lingual ability and socialization, while cognitive ability was delayed for about a year. He had two deciduous teeth exfoliating before the age of 2. That was considered to be due to cement and enamel hypoplasia. It is important for future follow-up to determine if he can possibly regain physical and cognitive abilities. Dental follow up is important to determine if his permanent teeth are affected by decreased alkaline phosphatase activity. Adverse events were injection site reaction and temporal nephrocalcinosis; however, they did not require therapeutic discontinuation.




Case Report
Title
Lactic Acidosis Following Procaterol Overdose during Bronchial Asthma Attack
Author
Yuji Fujita Daisuke Sawada Miyuki Hayashi Nobuyuki Takada Kaori Kinoshita Takayasu Arima and Shinichi Suwabe
Department of Pediatrics, Kimitsu Chuo Hospital
Abstract
Procaterol is an essential and commonly used bronchodilator for the treatment of bronchial asthma attacks due to its immediate effectiveness. Common adverse effects of procaterol include tachycardia, tremors, hypokalemia, and hyperglycemia. Procaterol has various dosage forms, enabling patients with bronchial asthma attacks to decide whether to inhale it or take it orally. However, caution must be exercised when using this drug to avoid its overuse and overdose.
In this paper, we report the case of an 8-year-old boy with palpitation, tachycardia, tremors, hypokalemia, and lactic acidosis following a procaterol overdose. He ingested 4 tablets of procaterol (25 μg/tablet) because of a bronchial asthma attack. He presented to the emergency department with palpitation, tachycardia, and tremors, and he was admitted to the pediatric ward. Blood examination revealed the K level as 2.9 mEq/L, and lactate level as 4.7 mmol/L at two hours after procaterol ingestion. Palpitation, tachycardia, tremors, and hypokalemia improved without medication after six hours, but lactate levels elevated to 6.4 mmol/L. Lactic acidosis improved after 24 hours and he was then discharged.
Few studies regarding lactic acidosis following procaterol overdose have been reported in Japan. When prescribing β-agonists to relieve bronchial asthma attacks, it is important to properly instruct patients regarding the medication.




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