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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY

Vol.124, No.3, March 2020


Original Article
1. Clinical Manifestations and Value of Scoring System for Clinical Diagnosis in Children Aged below 6 Years with Mycoplasma pneumoniae Pneumonia
2. Upper Esophageal Coin Removal Using Direct Laryngoscopy and Magill Forceps
Case Report
1. Four Cases of Kawasaki Disease Complicated by Seizures or Coma
2. Two Cases of Acute Flaccid Myelitis Primarily Presenting with Cranial Nerve Symptoms
3. Retroperitoneal Teratoma Discovered with the Detection of Proteinuria
4. Follow-Up of a Patient with Megalencephaly-Capillary Malformation Syndrome with the PIK3CA Mutation from the Fetal Period
5. A Case of Extradural Spinal Arachnoid Cyst with Recurrent Urinary Tract Infection


Original Article
Title
Clinical Manifestations and Value of Scoring System for Clinical Diagnosis in Children Aged below 6 Years with Mycoplasma pneumoniae Pneumonia
Author
Yuichi Akaba Keita Ito Hinako Yamamura Moyu Horii Eiki Nakamura Yoshiki Hirano and Koichi Murono
Department of Pediatrics, Nayoro City General Hospital
Abstract
Mycoplasma pneumoniae is an important pathogen of community-acquired pneumonia in children; however, the clinical manifestations in infants and toddlers have been scarcely reported. This study reviews the clinical manifestations in children aged <6 years infected with M. pneumonia based on the scoring items for clinical diagnosis.
We retrospectively analyzed 113 children with M. pneumoniae pneumonia diagnosed by loop-mediated isothermal amplification (LAMP). We categorized these patients into two groups, children aged <6 years and children aged ≥6 years. The prevalence of nonproductive cough (27% vs 61%) was lower and that of wheezing (17% vs 2%) was higher in younger children than in older children. Peripheral leukocyte count and C-reactive protein levels were not remarkably elevated, and interstitial infiltrate on chest radiograph was similarly observed in most of the younger children and older children (60% vs 69%). The score of the group of children aged <6 years was 4.6±1.2, which did not fulfill the diagnostic criteria.
The clinical manifestations in children aged <6 years with M. pneumoniae pneumonia were atypical compared to those in older children, especially in terms of the type of cough and lung sounds upon chest auscultation. Therefore, the current scoring system for clinical diagnosis cannot be applied in children aged <6 years. However, the peripheral leukocyte count, the C-reactive protein level, and the chest radiograph findings are likely to be typical even in children of this age group.




Original Article
Title
Upper Esophageal Coin Removal Using Direct Laryngoscopy and Magill Forceps
Author
Hiromasa Akada1) Yusuke Hagiwara1) and Nobuaki Inoue2)
1)Division of Pediatric Emergency Medicine, Department of Emergency and Critical Care Medicine, Tokyo Metropolitan Children's Medical Center
2)Department of Human Resources Development, Bureau of International Health Cooperation, National Center for Global Health and Medicine
Abstract
BACKGROUND
Coins are the most common cause of esophageal foreign body impaction in children and sometimes require removal with a Foley catheter or an endoscope. Several reports suggested the efficacy of direct laryngoscopy and Magill forceps to remove a coin from the upper esophagus in emergency departments (ED). To date, there have been no similar reports from Japan. Therefore, we summarized eight cases in which this technique was performed in our ED.
METHODS
Data were extracted from cases that performed upper esophageal coin removal using direct laryngoscopy and Magill forceps in the ED of Tokyo Metropolitan Children's Medical Center from October 2015 to April 2018. The data included gender, age, symptoms at presentation, coin types, procedure duration, success rates, complications, and dispositions.
RESULTS
All the procedures were done in the ED. The median time to complete the procedure was 31 min. In all the cases, the procedure was performed under tracheal intubation for airway protection. All the patients were discharged within 24 h without complications except for one case of trivial oral bleeding.
CONCLUSION
The success rate was high as previously published. The removal technique using direct laryngoscopy and Magill forceps could be a viable option for removing coins lodged in the upper esophagus.




Case Report
Title
Four Cases of Kawasaki Disease Complicated by Seizures or Coma
Author
Reiko Kawai1) Koichi Miyata2) Hiroshi Hataya1) Takuya Tamame3) Satoshi Araki4) Masato Nishioka5) Yukio Yamashita6) and Masaru Miura2)7)
1)Department of General Pediatrics, Tokyo Metropolitan Children's Medical Center
2)Department of Cardiology, Tokyo Metropolitan Children's Medical Center
3)Department of Pediatrics, Saitama City Hospital
4)Department of Pediatrics, Nerima Hikarigaoka Hospital
5)Department of Pediatrics, Kawaguchi Municipal Medical Center
6)Department of Pediatrics, Yokohama Municipal Citizen's Hospital
7)Clinical Research Support Center, Tokyo Metropolitan Children's Medical Center
Abstract
The majority of patients who develop central nervous system complications of Kawasaki disease (KD) recover without permanent sequelae, but a few of them have a poor prognosis. We report four cases of KD with seizures or coma. All the patients were boys aged from 5 months to 3 years. Three patients developed seizures, while two showed light to deep coma. The Kobayashi score, which indicates the risk of resistance to initial intravenous immunoglobulin (IVIG) therapy, ranged from 2 to 11 points. All four patients were resistant to initial IVIG therapy and had multiple organ complications. Three of them died or showed severe sequelae, including giant coronary artery aneurysm and brain atrophy. Only one patient, who received initial treatment with intravenous methylprednisolone (IVMP) pulse therapy for 3 days plus IVIG, recovered without sequelae. In patients with KD suffering from seizures or coma, we recommend aggressive initial treatment at advanced medical institutions, since some of them have a severe clinical course.




Case Report
Title
Two Cases of Acute Flaccid Myelitis Primarily Presenting with Cranial Nerve Symptoms
Author
Masaki Shibanuma1) Yuji Fujii1) Takeki Asano1) Shintaro Funaki1) Chinami Matsumoto1) Hiroki Izumo1) Yumi B. Tamura1) Tomoki Sato1) Syuji Yoshino1) Takaki Furue1) Hiroyuki Shimozono1) Keita Matsubara1) Rika Okano1) and Nobutsune Ishikawa2)
1)Department of Pediatrics, Hiroshima City Funairi Citizens Hospital
2)Department of Pediatrics, Hiroshima University Hospital
Abstract
Recently, several cases of acute flaccid paralysis with myelitis, demonstrated on magnetic resonance imaging, have been reported concomitant with an enterovirus D-68 (EV-D68) epidemic. These cases have been defined as acute flaccid myelitis (AFM) and are currently receiving global attention. We encountered two cases of AFM involving patients presenting with conspicuous cranial nerve symptoms. Case 1 was a 6-year-old Japanese boy, and case 2 was a 4-month-old Japanese boy. Both patients presented with preceding fever and vomiting and then developed acute flaccid paralysis with cranial nerve symptoms. They were diagnosed with AFM based on high signal intensity in the cervical spinal cord on T2-weighted images and an elevated mononuclear cell count in the cerebrospinal fluid. In both cases, EV-D68 was detected in the pharynx.
Both patients presented with cranial nerve symptoms conspicuously. In particular, case 2 presented with right facial paralysis before experiencing other neurological symptoms. If a patient presents with brainstem symptoms during an EV-D68 epidemic, AFM should be considered as one of the differential diagnoses.




Case Report
Title
Retroperitoneal Teratoma Discovered with the Detection of Proteinuria
Author
Yukiko Tano1) Seishiro Nodomi1) Shuichi Katayama2) Kenji Waki1) and Tsuyoshi Imai1)
1)Department of Pediatrics, Kurashiki Central Hospital
2)Department of Pediatric Surgery, Kurashiki Central Hospital
Abstract
Most of the patients with retroperitoneal teratoma present with asymptomatic abdominal mass palpated or symptoms including abdominal pain. Herein, we report a 3-year-old girl who was given a diagnosis of retroperitoneal teratoma after the detection of proteinuria following streptococcal infection, and the findings of a retrospective review of pediatric patients under 15 years of age with retroperitoneal or ovarian teratomas whose urine was examined in our hospital.
Three months before the diagnosis, proteinuria was detected, but further investigation was not planned. At the time of diagnosis, an abdominal examination revealed a palpable mass, and ultrasonography showed a mass located beside the left kidney. The tumor was completely resected without any surgical complications despite compression to the left kidney and renal vein, and the histological diagnosis was immature teratoma. There were no further findings of proteinuria following the surgery. Our retrospective review showed five out of six patients who presented with proteinuria at the time of diagnosis, which suggests a possible correlation between the presence of a tumor and proteinuria.
The prognosis of patients with retroperitoneal teratomas is relatively good. However, we must pay attention to tumor rupture, infections, and perioperative complications. Therefore, early detection of retroperitoneal or ovarian teratomas is desirable. Proteinuria is a common laboratory abnormality in daily practice; therefore, pediatricians should examine the abdomen for possible retroperitoneal or ovarian teratomas and undertake ultrasonography if necessary.




Case Report
Title
Follow-Up of a Patient with Megalencephaly-Capillary Malformation Syndrome with the PIK3CA Mutation from the Fetal Period
Author
Mari Yokokawa Kohei Haraguchi Yoshikazu Otsubo and Muneichiro Sumi
Department of Pediatrics, Sasebo City General Hospital
Abstract
We report a case of megalencephaly-capillary malformation syndrome (MCAP) diagnosed by an enlarged head circumference in the fetal period, along with a PIK3CA gene mutation (p.His1047Thr) identified by genetic analysis. MCAP is a genetic syndrome, which is characterized by progressive megalencephaly and capillary malformations. The mammalian target of rapamycin (mTOR) pathway is an important signal pathway, regulating cell growth. Abnormal activation of the mTOR pathway accelerates cell proliferation, causing megalencephaly and hydrocephalus. Mutations in the PIK3CA, PIK3R2, and AKT3 genes, encoding regulators of the mTOR signaling pathway, have been identified in MCAP patients. Our case had vascular anomalies (right aortic arch and azygos communication and an absent inferior vena cava), hypothyroidism, and laryngomalacia. Brain MRI performed in the neonatal period showed no ventriculomegaly; however, a head ultrasound revealed progressive ventriculomegaly at 5 months of age, and a ventriculo-peritoneal shunt was performed to treat hydrocephalus at 6 months of age. MCAP is a progressive disease, but there is no effective mTOR inhibitor for MCAP. Currently, there are only symptomatic treatments for complications. MCAP patients are at risk of developing cerebellar tonsillar herniation because of progressive overgrowth of the cerebellum and life-threatening episodes due to congenital cardiac malformations and arrhythmia. An early diagnosis of MCAP based on genetic analysis is important for performing effective early intervention.




Case Report
Title
A Case of Extradural Spinal Arachnoid Cyst with Recurrent Urinary Tract Infection
Author
Mako Sawada1) Kentaro Miyai1) Seiya Tachikawa1)2) Youko Saitou1) Chika Hiraishi1) Yaeko Motoyoshi1) Satoshi Ihara3) and Koji Kiyohara1)
1)Department of Pediatrics, Tokyo-Kita Medical Center
2)Department of General Medicine, Tokyo-Kita Medical Center
3)Department of Neurosurgery, Tokyo Metropolitan Children's Medical Center
Abstract
Bladder and bowel dysfunction (BBD) is a major risk factor of recurrent urinary tract infection (UTI) in children. Currently, constipation is regarded as one of the major symptoms of BBD as well as an important factor of urination disorders. Therefore, constipation should be evaluated in children with recurrent UTI. In addition, it is important to be aware of the possibility that both constipation and urination disorders may be caused by spinal cord diseases. We report a case of a 2-year-old girl who presented with recurrent UTI and constipation since infancy. She showed no remarkable abnormalities except for high fever. By contrast computed tomography scan imaging, acute focal bacterial nephritis in the right kidney and extradural spinal arachnoid cyst compressing the spinal cord were detected. We considered the extradural arachnoid cyst as the cause of BBD leading to recurrent UTI. After surgical intervention, the recurrence of UTI was stopped, and the constipation was improved. Although constipation is commonly seen in children, it is necessary to take the possibility of spinal cord diseases into account as the cause of BBD.




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