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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY

Vol.117, No.12, December 2013


Review
1. Active Surveillance for Hospitalizations due to Rotavirus Gastroenteritis among Children (<5 years) in Tsu City, Mie Prefecture, Japan
 
Original Article
1. Investigation into Venovenous ECMO for Respiratory Failure in Children
2. Paired Pressurized/Non-pressurized Spiral CT in the Diagnosis of Tracheomalacia
3. Transport of Children with an Advanced Disease in a Commercial Aircraft Cabin
4. A Case of Childhood Idiopathic Refractory Uveitis Treated Successfully with Infliximab
5. A Case of the Non-progressive Hepatic form of Type IV Glycogen Storage Disease
6. A Rare Case of Primary Hyperparathyroidism Diagnosed with Ureteral Stones
7. A Case of Streptococcal Toxic Shock Syndrome and Pneumonia
8. A Case of Possible Infective Endocarditis Caused by Campylobacter showae
9. An Infant with Infection of Clostridium perfringens Type A
10. Infliximab Treatment of Refractory Kawasaki Disease
11. A Fatal Case of Acute Leukoencephalitis with Significant Brain Swelling
12. A Case of D-lactic Acidosis in a Congenital Central Hypoventilation Syndrome with Short Bowel Syndrome
13. A Case of Cystic Fibrosis in a 6-year-old Japanese Child


Review
Title
Active Surveillance for Hospitalizations due to Rotavirus Gastroenteritis among Children (<5 years) in Tsu City, Mie Prefecture, Japan
Author
Kazutoyo Asada1) Hajime Kamiya2) Shigeru Suga1) Mizuho Nagao1) Ryouji Ichimi1) Takao Fujisawa1) Kazunobu Oya3) Hisashi Tanida4) Takaaki Tanaka5) Hiroaki Ito6) Shigeki Tanaka7) Masaru Ido7) Toshiaki Ihara1) and Takashi Nakano5)
1)Department of Pediatrics, Mie National Hospital
2)Infectious Disease Surveillance Center, National Institute of Infectious Diseases
3)Department of Pediatrics, Mie University Hospital
4)RIKEN Center for Integrative Medical Sciences
5)Department of Pediatrics, Kawasaki Hospital
6)Field Epidemiology Training Program, Japan
7)Department of Pediatrics, Mie Central Medical Center
Abstract
A rotavirus vaccine was introduced in November 2011 in Japan. In order to evaluate the effectiveness of the vaccine, it was necessary to assess data on the burden of rotavirus disease and the circulating rotavirus strains before introducing the rotavirus vaccine. To obtain these data, we set up active surveillance for hospitalizations due to rotavirus gastroenteritis among children (<5 years) in Tsu city, Mie Prefecture, Japan. From November 1, 2007 through October 31, 2011, we enrolled children who were hospitalized with a diagnosis of rotavirus gastroenteritis. During this period, 205 children were hospitalized, and the incidence rate of hospitalization was 4.2 per 1,000 children (95% confidence interval, 3.7-4.8). Only 4.4% (9/205) of the hospitalized children were<6 months of age, 23.9% (49/205) were<1 year of age, and 61.5% (126/205) were<2 years of age. Of the 205 hospitalizations, 64% (131/205) cases underwent G and P typing using semi-nested PCR. The most dominant serotype (genotype) found during each season was G3P[8], which accounted for 62%-75% of cases. The second most dominant was G1P[8] (11%-28%). We assessed and compared clinical severity between each genotype by age but no significant differences were observed.




Original Article
Title
Investigation into Venovenous ECMO for Respiratory Failure in Children
Author
Miho Nakamura1) Chiaki Toida1) Takashi Muguruma1) Kentaro Ide1) and Eisuke Isobe2)
1)Division of Acute and Critical Care, National Medical Center for Children and Mothers
2)Division of Medical Engineering, National Medical Center for Children and Mothers
Abstract
Background: The rate of carrying out Venovenous Extracorporeal Membrane Oxygenation (VV ECMO) for children presenting with serious respiratory failure presenting with is low in Japan.
Purpose: Investigation into the efficacy of VV ECMO
Objectives: 6 cases of patients that underwent VV ECMO from '02 to '12
Method: Perspective visual examination of the medical records
Result: median (minimum-maximal)
Months 1.5 (0-19), weight 4.1 (3.2-11.9) kg. The diagnoses of 6 cases in detail were econium aspiration syndrome 2/Acute respiratory distress syndrome 2 asthma 1/Respiratory syncytial virus bronchiolitis 1. Ventilator days before VV ECMO were 6 (4-13). VV ECMO days were 6 (4-13). There was 1 case of venoarterial inversion while carrying out VV ECMO. Leaving the ICU alive: No neurological aftereffects were observed regarding 5 cases.
Consideration: Regarding the management of VV ECMO, if management proves to be difficult due the presence of a circulatory disturbance, efficiency, recirculation, etc., than early venoarterial inversion should be considered rather than adjusting the location of the catheter tip.
Conclusion: VV ECMO may be effective in children with severe respiratory failure.




Original Article
Title
Paired Pressurized/Non-pressurized Spiral CT in the Diagnosis of Tracheomalacia
Author
Jun Miyahara Sho Takeda and Tadashi Matsubayashi
Department of Pediatrics, Seirei Hamamatsu General Hospital
Abstract
Objective:
To investigate airway collapsibility using quantitative evaluation in children using paired pressurized/non-pressurized spiral CT.
Methods:
In three patients with tracheomalacia (TM) and two patients without TM, chest CT was performed under two conditions: pressurized (PEEP 10 or 15 cmH2O) and non-pressurized (ZEEP). Changes in cross-sectional areas of the trachea were measured to calculate estimated airway closing pressures. In the TM patients, we also calculated estimated airway closing pressures from an airway pressure cross-sectional area study under bronchoscopy, as reported by Okazaki et al.
Results:
Estimated airway closing pressures calculated from paired pressurized/non-pressurized spiral CT were higher in the patients with TM than in the patients without TM (trachea, -14 to -55 cmH2O vs. -165 to -310 cmH2O). In addition, in the TM patients, estimated airway closing pressures calculated from paired pressurized/non-pressurized spiral CT were similar to values reported by Okazaki et al.
Conclusion:
Our findings suggest that paired pressurized/non-pressurized spiral CT is useful as a minimally invasive and convenient method for quantitative evaluation of airway collapse in children.




Original Article
Title
Transport of Children with an Advanced Disease in a Commercial Aircraft Cabin
Author
Kazunori Aoki1) Takashi Muguruma1) Satoko Uematsu2) and Tomoya Ito2)
1)Pediatric Intensive Care Unit, National Center for Child Health and Development
2)Department of Emergency Medicine and Transport Services, National Center for Child Health and Development
Abstract
[Background] The frequency of serious diseases in children is low, but institutions at which patients with serious diseases can be treated are limited. Therefore, some patients have to travel long distances by commercial aircraft for treatment. However, physiological changes during the flight, children's individuality, and the original disease status may cause problems during transport.
[Purpose] To monitor the actual condition of children with an advanced disease during travel in a commercial aircraft cabin.
[Method] We retrospectively examined children with advanced diseases who had travelled in a commercial aircraft cabin between 2002 and 2012, to our hospital.
[Result] The median age of the patients (8) was 9.5 months (1-70 months); weight, 5.7 kg (3.6-17.6 kg); and transport distance, 882 km (386-8,835 km). No respiratory tract-related adverse event was observed. Although medical equipment or batteries caused some problems during transport, there was no effect on the patients.
[Conclusion] Respiratory tract management is very important during air travel. To avoid complications, it is important to carry sufficient oxygen supply, batteries, and medical equipment when transporting children with an advanced disease in commercial aircraft cabins.




Original Article
Title
A Case of Childhood Idiopathic Refractory Uveitis Treated Successfully with Infliximab
Author
Toshihiro Fujiki1)2) Keiko Yamada1) Michio Konishi1) Masaki Shimizu2) and Akihiro Yachie2)
1)Department of Pediatrics, Tonami City Hospital
2)Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University
Abstract
Initial treatment for childhood uveitis mainly comprises topical and systemic corticosteroid therapy. Infliximab, a monoclonal antibody against tumor necrosis factor α, has been gaining recognition as an important therapeutic modality for refractory uveitis. We report a case of a 14-year-old Japanese girl with childhood idiopathic uveitis refractory to topical and systemic corticosteroid therapy, who was treated successfully by infliximab. The patient presented with blurred vision in the left eye. She was diagnosed as idiopathic uveitis. Her condition did not improve with topical and systemic corticosteroids, and she failed to both left and right visual field defects. Infliximab and methotrexate were started, following which her right visual field markedly improved. Systemic corticosteroid therapy was successfully tapered. She is currently undergoing treatment with infliximab, and no recurrence or adverse effects have occurred. Although infliximab is considered effective for the treatment of childhood refractory uveitis, randomized controlled studies are required to further confirm its efficacy and safety.




Original Article
Title
A Case of the Non-progressive Hepatic form of Type IV Glycogen Storage Disease
Author
Kaori Kawano1)4) Masanori Adachi1) Yumi Asakura1) Koji Muroya1) Yoshiaki Shikama2) Kunihiko Akagi2) Yukichi Tanaka3) Tokiko Fukuda5) and Hideo Sugie5)
1)Department of Endocrinology and Metabolism, Kanagawa Children's Medical Center
2)Division of Infection, Immunology and Rheumatology, Kanagawa Children's Medical Center
3)Diagnostic Pathology, Kanagawa Children's Medical Center
4)Department of Pediatrics, School of Medicine, Tokyo Women's Medical University
5)Department of Pediatrics, School of Medicine, Jichi Medical University
Abstract
Glycogen storage disease (GSD) type IV is a heritable metabolic disorder caused by a defective glycogen branching enzyme (GBE) that frequently gives rise to cirrhosis. Our patient was 1 year old when he was found to have abnormal liver functions with hepatosplenomegaly. A histological examination revealed periodic acid Schiff (PAS)-positive amylopectin-like material in the hepatocytes that was partially resistant to diastase digestion. GSD-IV was suspected and confirmed by decreased GBE activity in the erythrocytes. Because the patient had no liver damage or hepatomegaly at 11 years of age, it was most likely the non-progressive hepatic form of GSD-IV. In conclusion, as GSD-IV has a wide spectrum of clinical features, physicians should note that PAS staining is essential for diagnosing GSD-IV.




Original Article
Title
A Rare Case of Primary Hyperparathyroidism Diagnosed with Ureteral Stones
Author
Masahiro Ishii1) Yukiyo Yamamoto1) Reiko Saito1) Motohide Goto1) Shunsuke Araki1) Rinko Kawagoe1) Yasusada Kawada1) Takahisa Sakuma2) and Koichi Kusuhara1)
1)Department of Pediatrics, School of Medicine, University of Occupational and Environmental Health
2)Sakuma Pediatric Clinic
Abstract
We describe a rare case of primary hyperparathyroidism (PHPT) due to a parathyroid adenoma in a 13-year-old boy, in whom diagnosis was established after complaints of abdominal pain resulting from ureteral stones. At 12 years of age, the patient had macroscopic hematuria, which disappeared without treatment. One year later, he presented with abdominal pain and macroscopic hematuria. An abdominal X-ray examination showed stones in the left ureteral tract. His laboratory data revealed hypercalcemia, hypercalciuria and an elevated serum level of parathyroid hormone, which indicated the presence of PHPT. Cervical ultrasonography showed a 4×2×2 mm hypoechoic region posterior to the right thyroid gland. A parathyroid adenoma was suspected based on these findings. The parathyroidectomy was performed without complications. Further pathological examination confirmed the diagnosis of parathyroid adenoma. He has not suffered from any subsequent relapse. Although PHPT is very rare during childhood, it can often be diagnosed in the presence of macroscopic hematuria and urolithasis in routine practice. Furthermore, urine screenings at school may help diagnose asymptomatic microscopic hematuria. We suggest that a careful differential diagnosis is necessary for any subjects with these clinical signs, even when they are present during childhood.




Original Article
Title
A Case of Streptococcal Toxic Shock Syndrome and Pneumonia
Author
Keiko Okumura1)2) Kenichiro Ito1) Tomohiko Tsuru1) Hajime Ikehara1) Hideyuki Okui1) Takashi Ichiyama3) Ryugo Hiramoto1) and Isao Komori1)
1)Department of Pediatrics, Matsudo City Hospital Children's Medical Center
2)Department of Pediatrics, Nadogaya Abiko Hospital
3)Department of Pediatrics, Yamaguchi University Graduate School of Medicine
Abstract
We report a case of a 14-year-old girl with streptococcal toxic shock syndrome (STSS) accompanied by pneumonia and hemophagocytic syndrome. The patient had some minor anomalies and had suffered from psychomotor delay and epilepsy. No abnormal data were detected regarding liver function or renal function. The patient was transferred to our hospital due to cardiopulmonary arrest two hours after the onset of fever. Although return of spontaneous circulation was once achieved, she died of multi-organ failure four hours after her arrival. Streptococcus pyogenes was found on culture in the sputum of the trachea, but not in the blood. STSS was diagnosed based on the proposal by the Working Group on Severe Streptococcal Infections. A pathological autopsy revealed micro-abscesses in the liver and many phagocytes in the bone marrow in addition to pneumonia. As to cytokine analysis in serum, IL-6, IL-10 and IL-2 were elevated, but TNF-α was within the normal range. STSS rarely occurs in the pediatric field, and there have been few reports of STSS accompanied by pneumonia and hemophagocytic syndrome. The mechanism of STSS remains unclear. It is important to examine both the cytokine profile and pathological changes including the bone marrow in cases of STSS.




Original Article
Title
A Case of Possible Infective Endocarditis Caused by Campylobacter showae
Author
Akina Ohmura1) Masahiko Matsumura2) Hiroki Tanaka2) Takeshi Shiba2) Shinichiro Yoshimura2) Naoki Miki2) Chutaro Yamanaka2) Akio Sunakawa3) and Mitsuhiko Nambu2)
1)Department of General Internal Medicine, Tenri Hospital
2)Department of Pediatrics, Tenri Hospital
3)Department of Pediatrics, Yamatotakada Municipal Hospital
Abstract
A 17-year old girl with Down syndrome was admitted to our hospital due to fever of unknown origin. She had a history of surgery for endocardial cushion defect on 7 months old. She had been followed for trivial mitral valve regurgitation. She had not taken undercooked meat nor undergone dental procedures for several days before her admission. The serum level of C-reactive protein was markedly elevated on admission. Although two sets of blood culture on the first day of admission were negative, an anaerobic blood culture bottle on the second day harvested Campylobacter showae. No vegetation was detected on several echocardiograms. Finally, she fulfilled the criteria for "possible infective endocarditis (IE)" of the modified Duke criteria. Her fever abated after 4 weeks of antibiotic treatment. Detection of Campylobacter species in blood culture has rarely been reported, which is probably because Campylobacter is difficult to incubate. Therefore, Campylobacter should be considered as a causative agent of IE, even when blood culture is negative.




Original Article
Title
An Infant with Infection of Clostridium perfringens Type A
Author
Goro Sasaki1) Naomi Shinohara1) Masayoshi Shinjo2) and Hiroyuki Eguchi1)
1)Department of Pediatrics, Tokyo Dental College Ichikawa General Hospital
2)Department of Pediatrics, School of Medicine, Keio University
Abstract
Clostridium perfringens is a pathogenic anaerobe generally associated with gastrointestinal infection. We here report a case of infantile hematochezia associated with infection of Clostridium perfringens type A. A 4-month-old girl was referred us for evaluation of persistent hematochezia. She did not appear to show any other manifestations such as fever, vomiting, anemia, weight loss, dehydration, or irritability, and continued to receive either breast milk or cow's milk formula without allergic symptoms. She had no past history of anal fissure, another gastrointestinal infection, or administration of antibiotics, and had been living uneventfully with healthy parents. Her laboratory findings exhibited increased numbers of total leukocytes and eosinocytes in peripheral blood, while serum C-reactive protein was nearly undetectable. Microbial analyses for fecal specimens isolated Clostridium perfringens by anaerobic culture, but were negative for other disease-causing bacteria, Entamoeba histolytica, and parasite's eggs. Further bacterial analyses determined the toxinotype and genotype as α toxin-positive and entrotoxin-negative Clostridium perfringens type A carrying the cpb2 gene that encodes the β2 toxin. Administration of metronidazole dramatically improved hematochezia of the patient, and was also effective for normalizing numbers of peripheral leukocytes and eosinocytes. After the treatment, Clostridium perfringens was not detected by repeated fecal culture, collectively suggesting the potential association between persistent infantile hematochezia and intestinal infection of Clostridium perfringens type A.




Original Article
Title
Infliximab Treatment of Refractory Kawasaki Disease
Author
Shizuka Machida Taku Ishii Dai Kuranobu Akira Takei Akiko Takahashi Mitsunori Nishiyama Shozaburo Doi and Shuki Mizutani
Department of Pediatrics and Developmental Biology, Graduate School of Medicine, Tokyo Medical and Dental University
Abstract
Patients with immunoglobulin-resistant Kawasaki disease are at high risk of developing the complication of coronary artery lesions (CALs). However, a standard treatment for refractory Kawasaki disease has not yet been established. Infliximab (IFX) is expected to be effective for curing refractory Kawasaki disease and decreasing the rate of CALs. However, its rate of adverse effects is not yet known.
[Case] A 7-month-old boy was diagnosed with Kawasaki disease on day 4 of illness. He had developed fever, conjunctival congestion, redness of the lips, swelling of the cervical lymph nodes, indurative edema of the palms and soles, and polymorphous erythema. He was treated with aspirin (50 mg/kg/day), 3 doses of intravenous immunoglobulin (2 g/kg), prednisolone (2 mg/kg/day) and methylprednisolone pulse therapy (20 mg/kg/day) for 3 d. However, his fever and other symptoms did not improve, and his C-reactive protein (CRP) level was 17.6 mg/dl. Therefore, he was given a single dose of IFX (5 mg/kg) on day 9. His symptoms and CRP level remarkably improved on the next day. We used a continuous cyclosporine infusion (1.3 mg/kg/day) to prevent complications of CALs from day 10. The patient did not develop CALs or IFX-related adverse effects.
[Conclusion] In this case, refractory Kawasaki disease was successfully treated with IFX, and no IFX-related adverse effects. However, the rates of adverse effects and late-onset complications are not clear for refractory Kawasaki disease. Consequently, there is an urgent need to develop criteria for the use of IFX for Kawasaki disease.




Original Article
Title
A Fatal Case of Acute Leukoencephalitis with Significant Brain Swelling
Author
Masaru Takayanagi1) Kohei Kusumoto1) Chisumi Sogi1) Wakaba Endo2) Rikio Suzuki1) Taro Kitamura1) Toshiyuki Nishio1) Toshihiro Ohura1) and Masatoshi Ohtake1)
1)Division of Pediatrics, Sendai City Hospital
2)Division of Pediatrics, Japanese Red Cross Ishinomaki Hospital
Abstract
The patient, a 2-year, 5-month-old girl, was hospitalized because of a febrile convulsion. Intractable convulsions followed by prolonged disturbance of consciousness were observed on the third hospital day, on which brain magnetic resonance imaging (MRI) revealed a high signal intensity of disseminated T2WI/FLAIR mainly in the left cerebellar hemisphere, pons, and left external capsule. Based on a diagnosis of acute disseminated encephalomyelitis, she was started on steroid-pulse therapy and anticonvulsants. However, uncontrollable brain swelling with expansion of the disseminated white-matter lesions progressed rapidly despite external decompression, and the intracranial pressure constantly exceeded the mean arterial pressure. Her brainstem function essentially stopped on the seventh day and she had a fatal cardiac arrest on the twenty-seventh day. This patient was considered to have intermediate type acute leukoencephalitis, with a severity between that of acute disseminated encephalomyelitis and acute hemorrhagic leukoencephalitis, based on the clinical features and imaging findings. Fatal cases of acute disseminated encephalomyelitis are rare. However, if a patient worsens rapidly, the possibility of acute hemorrhagic leukoencephalitis should be considered, and combined intensive therapy for managing the intracranial pressure and various immunosuppressive therapies should be started immediately.




Original Article
Title
A Case of D-lactic Acidosis in a Congenital Central Hypoventilation Syndrome with Short Bowel Syndrome
Author
Junko Kanno Tetsuji Morimoto Mitsuji Moriya Yuko Sato Fumiaki Kamada Akiko Hakoda Naonori Kumagai Ikuma Fujiwara and Shigeo Kure
Department of Pediatrics, Tohoku University School of Medicine
Abstract
D-lactic acidosis is a rare syndrome occurring in patients with short bowel syndrome. It is characterized by severe manifestation of metabolic acidosis with uncommon neurological symptoms due to excess accumulation of D-lactic acid, produced by an absorption disorder of carbohydrate and excessive proliferation of intestinal bacteria.
A 4-year-old girl with congenital central hypoventilation had undergone an intestinal resection for necrotizing enterocolitis at the age of 2-years. She had received total parenteral nutrition (TPN) and enteral alimentation. After discontinuing TPN because of increasing ingestion ability, she presented with a metabolic acidosis and consciousness disturbance. At the onset of the acidosis, her lactic acid concentration was found to be normal, however, her urinary lactic acid concentration was very high. D-lactate and L-lactate are optical isomers. Because D-lactate is undetectable, routine lactate assays used measured only L-lactate. As her urinary lactate concentration was very high at the time of the attack, we measured serum D-lactate concentration using an absorbance method with her preserved serum obtained during the attack. Her serum D-lactate concentration was found to be very high; thus, we diagnosed D-lactic acidosis. Symptoms improved following intravenous infusion of bicarbonate, but a recurrent attack occurred due to excess carbohydrate ingestion and enteral alimentation. Her attacks completely resolved after receiving a low by low carbohydrate diet and discontinuation of enteral alimentation.
In cases of a patient with short bowel syndrome developing metabolic acidosis with neurological presentation, D-lactic acidosis should be considered. For a definitive diagnosis, it is useful to measure the urinary lactic acid and serum D-lactate concentrations.




Original Article
Title
A Case of Cystic Fibrosis in a 6-year-old Japanese Child
Author
Daiei Kojima Daisuke Ieda Yoshiyuki Kuroyanagi Tomonori Goto Katsuaki Kasahara Yoshimitsu Goto Mutsuo Ishii Yasusi Kanda and Mitsuji Iwasa
Department of Pediatrics, Nagoya Daini Red Cross Hospital
Abstract
We report a Japanese girl who was found to have cystic fibrosis at age 6. She underwent emergency laparotomy for meconium peritonitis at birth and had repeated hospitalization due to dehydration during infancy. At the age of 3, she suffered from intractable cough at night which gradually became worse. At the age of 6, she was referred to our hospital. Plain chest radiography revealed diffuse nodular infiltrates and chest CT demonstrated multiple areas of bronchiectasis and mucous plugging. Blood test showed increased amylase levels. The chloride concentration of insencsible sweat collected from her thumb was 114.3 mEq/L. (normal value<40 mEq/L) Hence, cystic fibrosis was diagnosed. After a comprehensive search for mutations in the cystic fibrosis transmembrane regulator (CFTR) gene, the patient was found to carry L441P mutation in a maternal allele. Because the incidence of cystic fibrosis may increase in our country in the future, the possibility of cystic fibrosis should be suspected in patients with unexplained intractable cough, and sweat chloride tests are recommended for these patients.




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