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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY

Vol.120, No.12, December 2016


Review
1. Relationship between Fever and Serum Sodium Level in Children
Original Article
1. 10 Cases of Idiopathic Mitral Valve Chordae Rupture in Infants
2. The Utility and Problems of a Portable Oxygen Concentrator in Patients with Pediatric Cardiovascular Diseases
3. Subcutaneous Immunoglobulin Replacement Therapy for 50 Patients with Primary and Secondary Hypogammaglobulinemia in a Single Institution
4. Characteristics of At-Risk Children with Invasive Pneumococcal Disease
Case Report
1. Formation of Mixed Calcium Oxalate and Ammonium Acid Urate Stones in a Boy during Refractory Nephrotic Syndrome Treatment
2. A Patient with Secondary Carnitine Deficiency Presenting with Ketotic Instead of Non-ketotic Hypoglycemia
3. A Large Bowel Obstruction Caused by Persimmon Phytobezoar Impaction
Brief Report
New Diagnosis Method of Tachiarrythmia in Newborns by Attaching the Esophageal Lead Electrode to the Heart Rate Monitor


Review
Title
Relationship between Fever and Serum Sodium Level in Children
Author
Akira Kusakari1) and Tatsuo Nishimura2)
1)Kusakari Pediatric Clinic
2)Nishimura Pediatric Clinic
Abstract
It is widely believed that febrile children are prone to suffer from dehydration due to the increase of insensible perspiration. In this case, it is considered to be a hypertonic dehydration, but febrile diseases such as pneumonia and bacterial meningitis hyponatremia often becomes a clinically important issue. In fact, as for hospitalized patients, hyponatremia was obviously found in a lot of febrile patients, and a positive correlation was observed between hyponatremia and white blood cell count, neutrophil count, and CRP value. Febrile patients also presented high concentration of plasma AVP and it is considered that SIADH occurred. It was confirmed that body temperature and serum sodium level had a negative correlation.
Recently, two mechanisms of excitation of the AVP-secreting neurons in febrile and inflammatory diseases were revealed. One is direct stimulation by inflammatorycytokines, such as IL6, the other is to cause increased secretion of AVP in response to elevation of body temperature through TRPV1 channel. Regardless of the type and severity of the disease, the latter mechanism means that a febrile patient is in the AVP hypersecretion condition and prone to suffer from hyponatremia, due to impairment of water excretion from the kidneys. When sufficient clean water and medical care cannot be supplied, this mechanism serves to prevent the development of dehydration and promote recovery from the febrile illness, such as infectious diseases, and to increase the chance of survival. Conversely, intake of excessive water in febrile patients or hypotonic infusion to them can cause exacerbation of hyponatremia and become an impediment to healing. When the medical staff examines febrile patients, it is necessary to pay careful attention to this point.




Original Article
Title
10 Cases of Idiopathic Mitral Valve Chordae Rupture in Infants
Author
Megumi Nitta1) Sadahiro Sai2) Takashi Tanaka1) Akinobu Konishi2) Akira Ozawa1) Yuji Murata3) and Toshihiro Ohura4)
1)Department of Cardiology, Miyagi Children's Hospital
2)Department of Cardiovascular Surgery, Miyagi Children's Hospital
3)Department of Emergency, Sendai City Hospital
4)Department of Pediatrics, Sendai City Hospital
Abstract
Idiopathic mitral valve chordae rupture in infants result in acute heart failure in previously healthy infants and usually necessitates emergency surgical repair. We recorded 10 cases during 10 year period from 2004 to 2013, and reported the clinical characteristics this disease. A retrospective analysis of these cases revealed the following: a mean onset age of 4.6 months; presence of concomitant infection in 9 cases; initial symptoms of poor sucking, tachypnea, and vomiting; grade III-IV mitral valve regurgitation (MR); a cardiothoracic ratio of > 60% in 3 cases; mean WBC of 16,736/μL; mean C-reactive protein level of 2.3 mg/dL; and brain-type natriuretic peptide level of > 500 pg/mL in 8 cases. The points for diagnosis were the absence of auxocardia because of the sudden onset of the disease and difficulty in hearing the systolic heart murmur because of the moist rale caused by the left-sided heart failure. Furthermore, we found the mean time from rupture onset to surgery to be 4.8 days in 5 anterior leaflet cases, 5 in 4 posterior cases,: 1 case each of annuloplasty in 2 cases: additional artificial chordae reconstruction (A-C) in 8 cases; and grade I-II MR after surgery in most cases. All cases survived and were not re-operated in the median term. We were concerned that the growth of the infants would cause the A-C to fail, but we concluded that this procedure was appropriate for this disease after comparison with normal control cases.




Original Article
Title
The Utility and Problems of a Portable Oxygen Concentrator in Patients with Pediatric Cardiovascular Diseases
Author
Seigo Okada1)2) Masahiro Kamada1) Naomi Nakagawa1) Yukiko Ishiguchi1) Yuji Moritoh1) Mayuko Shohi1) and Kengo Okamoto1)
1)Department of Pediatric Cardiology, Hiroshima City Hiroshima Citizens Hospital
2)Department of Pediatrics, Yamaguchi University Graduate School of Medicine
Abstract
Background: The use of portable oxygen concentrators (POC) has been widespread among adult patients, although there has not been enough evidence of the utility of POC in pediatric patients.
Methods: We performed a questionnaire of patients or their parents with pediatric cardiovascular diseases who were introduced to POC on a trial basis at Hiroshima City Hiroshima Citizens Hospital. A total of 8 patients were included in this study (3 males and 5 females; age, 4-44 years old; median age, 7 years old; 7 univentricular heart; 1 idiopathic pulmonary hypertension). Patients (or their parents) were provided with a questionnaire that included four main topics about POC; portability and operability, the levels of generated sound, the performance of the battery, and oxygen saturation under the use of continuous flow while sleeping.
Results: Four (50%) patients felt that POC was heavier than the oxygen tank previously used. Five (63%) patients answered that POC was more portable. Operability was tolerable for children. Five (63%) patients answered that POC was noisier compared with the stationary oxygen concentrator. The most common answer was that at least 2 backup batteries were required for going outside. The major reasons of POC had not been available in their own school were because of the limitations usage of batteries. There were no significant differences with respect to the oxygen saturation while sleeping before and after the introduction of POC.
Conclusions: POC had superiority in part compared with the stationary oxygen concentrator or oxygen tank although there is still room for improvement.




Original Article
Title
Subcutaneous Immunoglobulin Replacement Therapy for 50 Patients with Primary and Secondary Hypogammaglobulinemia in a Single Institution
Author
Miho Ashiarai1) Hirokazu Kanegane1) Kohsuke Imai2) Namiko Kimura3) Naho Chin3) Tsubasa Okano1) Shintaro Ono1) Mari Tanaka-Kubota1) Satoshi Miyamoto1) Chika Kobayashi1) Noriko Mitsuiki1) Yuki Aoki1) Eriko Tanaka1) Masatoshi Takagi2) and Tomohiro Morio1)
1)Department of Pediatrics and Developmental Biology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University
2)Department of Community Pediatrics, Perinatal and Maternal Medicine, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University
3)Nursing Department, Tokyo Medical and Dental University Medical Hospital
Abstract
Immunoglobulin replacement therapy is a life-saving treatment for patients with immunodeficiencies. Intravenous immunoglobulin remains a mainstay; however, subcutaneous immunoglobulin has been widespread mainly in Western countries, and it was reported to offer many advantages. SCIG has been available in Japan since January 2014. Fifty patients were treated with SCIG from April 2014 to December 2015 in our institution. The patients ranged in age from 0 to 55 years (mean age: 22 years), and this study included 45 patients with primary immunodeficiencies and 5 patients with secondary hypogammaglobulinemia. Thirty-six patients (72%) switched treatments from IVIG to SCIG. Local reactions were observed in 21 patients (42%), but severe adverse events were not observed. Six patients who had adverse events associated with IVIG could receive SCIG treatment. Three patients withdrew SCIG treatment. No severe infections were observed in 30 patients receiving SCIG more than half a year. SCIG offers a very low side-effect profile and almost physiological IgG level. It is able to provide home-based therapy under the control of physician. We discuss clinical practice, advantages and problems of SCIG in our institution.




Original Article
Title
Characteristics of At-Risk Children with Invasive Pneumococcal Disease
Author
Takanori Funaki1) Kimiko Ubukata2) Satoshi Iwata2) and Isao Miyairi1)
1)Division of Infectious Diseases, Department of Medical Subspecialties, National Center for Child Health and Development
2)Department of Infectious Diseases, Keio University
Abstract
[Background] Introduction of the universal pneumococcal conjugate vaccination (PCV) has resulted in a decrease in invasive pneumococcal diseases (IPD). The demographics and clinical characteristics of children with known risk factors for IPD in Japan have yet to be characterized.
[Methods] We investigated the demographics and clinical characteristics of patients diagnosed with IPD with known risk factors, compared to those without any risk factors. Patients who had pneumococcal bacteremia at< 18 years of age between June 2008 and August 2014 were identified from our institution's microbiology database. A retrospective chart review was performed on patients' demographics including age, gender, underlying diseases, focus of infection, treatment, and outcome. Patients were classified and compared according to the presence or absence of known risk factors based on recommendations for pneumococcal conjugate and polysaccharide vaccine administration by the Centers for Disease Control and Prevention.
[Results] Of the 112 cases with pneumococcal bacteremia (107 patients [57 male]) identified during the study period, 21 patients had known risk factors. At-risk patients consisted of immunodeficiency (62%), use of medical devices (33%), history of mechanical ventilation (81%), and developed IPD despite a history of pneumococcal vaccination (24%). The median age at the onset of the disease was significantly higher in the at-risk group at 54 months (range 8-123), compared with those without known risks at 18 months (range 3-157) (OR=1.1, [95%CI: 1.018-1.097], p=0.005) by multivariate analysis. Those at risk also had lower white blood cell count (median± SD: 21,930± 7,458 [no-risk patients] and 13,410± 6,456 [at-risk patients], p=0.002), and lower body weight at the onset of illness (OR=0.813, [95%CI: 0.668-0.961], p=0.022).
[Conclusion] Children with risk factors may develop IPD beyond the recommended age for current PCV vaccination and aggressive vaccination may be required.




Case Report
Title
Formation of Mixed Calcium Oxalate and Ammonium Acid Urate Stones in a Boy during Refractory Nephrotic Syndrome Treatment
Author
Akiko Kasuga Yasuyo Kashiwagi Koko Kato Norito Tsutsumi Nobuko Akamatsu Soken Go and Hisashi Kawashima
Department of Pediatrics, Tokyo Medical University Hospital
Abstract
Nephrotic syndrome has many reported risk factors for stone formation, including fluid restriction, prolonged immobility, and long-term use of medication. However, there are few reports of mixed calcium oxalate and ammonium acid urate stones. Our case is a 2-year-old boy with refractory nephrotic syndrome treated with prednisolone. Mixed calcium oxalate and ammonium acid urate stones were formed in his bladder after rotavirus infection. It is necessary to be aware of possible stone formation in rotavirus gastroenteritis, particularly when associated with nephrotic syndrome, and we should strive at early detection with the optimal use of urinalysis tests for the early prevention of stone formation.




Case Report
Title
A Patient with Secondary Carnitine Deficiency Presenting with Ketotic Instead of Non-ketotic Hypoglycemia
Author
Takahiro Hayashi Yoshiki Okayama Shota Ono Takeshi Katayama Shuji Sugimoto Shunsaku Kaji and Yoshio Fujimoto
Department of Pediatrics, Tsuyama Chuo Hospital
Abstract
We report the case of a 2-year-old girl with secondary carnitine deficiency presenting as ketotic hypoglycemia. She was admitted to our hospital because of a prolonged seizure. Marked hypoglycemia (18 mg/dl) and elevated plasma ketone bodies (6,215 μmol/l) were revealed on laboratory tests. Her urine was also positive for ketone bodies. A prolonged seizure was caused by ketotic hypoglycemia. Despite the fact that she presented with ketotic hypoglycemia, a secondary carnitine deficiency was found with a free carnitine level of 5.5 μmol/l and an acylcarnitine level of 5.0 μmol/l. This was because of the intermittent oral administration of pivalate-containing antibiotics for acute otitis media for the previous 22 days. After admission, she received treatment with a daily oral administration of carnitine (100 mg/kg/day), divided into three separate doses, in addition to an intravenous glucose infusion. She was discharged from our hospital without any complications on the 4th day after admission. Hypocarnitinemia typically causes non-ketotic hypoglycemia. However, secondary carnitine deficiency should be considered in patients receiving orally administered pivalate-containing antibiotics, even if they demonstrate ketotic hypoglycemia.




Case Report
Title
A Large Bowel Obstruction Caused by Persimmon Phytobezoar Impaction
Author
Yukiko Mori Nanae Tanaka Soichi Tamamura Yasuhiro Watanabe and Yoshihiro Taniguchi
Department of Pediatrics, Fukui Red Cross Hospital
Abstract
We report a case of colonic obstruction caused by a persimmon phytobezoar in a 1-year-old girl who consumed a half persimmon every day for a week. The patient presented to the hospital because of a loss of appetite, vomiting, and malaise. We performed radiography, ultrasonography, and computed tomography assessment, and found that the area from the ileum to the transverse colon was filled with feces, which had a bubbly appearance. We performed colonoscopy and found fecal incarceration, with a large piece of solid feces completely obstructed the transverse colon. It was successfully removed via colonoscopy, and the dark brown fibroid substance was identified as a persimmon phytobezoar in the feces. The persimmon is a fruit that is commonly consumed in Japan; however, it was reported to cause intestinal obstruction frequently. Chewing ability in infants is underdeveloped; therefore, the intake of food that can cause a phytobezoar (e.g. persimmon, kelp, rice cake) needs to be monitored carefully.




Brief Report
Title
New Diagnosis Method of Tachiarrythmia in Newborns by Attaching the Esophageal Lead Electrode to the Heart Rate Monitor
Author
Noriko Ohbuchi1) Kenjirou Saigou1) Haruka Ohta1)3) Shin-ichi Terachi1) Yoshio Nose1) Ryou Kadoya1) Mamie Watanabe2) Kunitaka Joo2) and Shouichi Ohga3)
1)Division of Pediatrics, Yamaguchi Red Cross Hospital
2)Division of Pediatrics, JCHO Kyushu Hospital
3)Department of Pediatrics, Yamaguchi University
Abstract
Diagnosis of tachyarrhythmia in newborns and infants is difficult because the detection of the P wave is often difficult. An esophageal lead electrocardiogram determines the P wave clearly by recording the electrical potential from the back of the atrium. However, attaching this as a part of a 12-lead electrocardiogram makes it difficult to detect abnormal waves of non-sustained tachycardia. By attaching the esophageal lead electrode to the heart rate monitor, low-frequency arrhythmia can be detected automatically. As this method uses a bipolar lead in both the esophagus and the chest, the electrocardiogram clearly defines any aberrant P wave having a steady baseline, and subtle changes in atrial excitation propagation are easily captured. This technique can be easily used in any hospital, and is useful in the diagnosis of tachyarrhythmia.




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