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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
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Vol.118, No.6, June 2014
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Review
Title
Clinical Surveillance of Newborn Hearing Impairment
Author
Yuko Tanaka Makiko Goto and Hiroshi Koga
Department of Pediatrics, National Hospital Organization Beppu Medical Center
Abstract
We surveyed the prevalence and causes of congenital hearing impairment in the eastern part of Oita Prefecture from 2009 to 2013. Auditory screening tests were performed in nearly all newborns at delivery centers in the region. During a 4-year period, 25 (0.3%) of 8,392 newborns had congenital deafness. Syndromic deafness was found in 10 (40%) of the 25 children with congenital deafness. Deafness genes were analyzed in 10 of the 15 patients with non-syndromic deafness, and GJB2 gene mutations were identified in 2 patients (20%). Our study confirmed that the incidences of congenital deafness and GJB2 gene mutations were generally consistent with the results of previous epidemiological studies, even within a specified region in Japan. The causes of syndromic deafness were diverse. It is essential for pediatricians involved in the diagnosis and treatment of congenital deafness to understand deafness genes as well as congenital abnormalities related to syndromic deafness.
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Original Article
Title
Phase 3 MR Vaccination in Kyoto City
Author
Masahiro Ito1) and Koichi Takeuchi2)
1)Kyoto City Health Center
2)Kyoto City Medical Association
Abstract
Regular vaccination with measles and rubella combined vaccine (MR vaccine) was performed in phase 3 (corresponding to first-year junior high school students) and 4 (corresponding to third-year senior high school students) children as a time-limited strategy from April 1, 2008 until March 31, 2013. Despite publicity activities and individual recommendations, the phase 3 vaccination rate was only 86% in 2008 in Kyoto City. To improve the phase 3 vaccination rate, a mass vaccination system was introduced in municipal junior high schools in 2009. The subjects were 41,381 first-year students belonging to municipal junior high schools from 2009 until 2012. Pre-consultation sheets were collected from 40,900 (98.8%). Of these, 32,657 (79.8%) wished to undergo vaccination. Vaccination was performed in 30,946 (94.8%). The number of those who actually underwent mass vaccination was 30,946, accounting for 64.2% of the total number of students targeted for phase 3 MR vaccination in Kyoto City, (48,168). Individual recommendations for MR vaccination were made to unvaccinated students at the end of each year. The adoption of the mass vaccination system has increased the phase 3 measles vaccination rate (the number of those undergoing mass or individual MR and single-antigen measles vaccination as a percentage of the total number of students targeted for measles and rubella vaccination) to 95% or more since 2009. The mass vaccination system may be an effective public-health strategy to prevent infectious diseases only if the health status is evaluated, as indicated for individual vaccination.
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Original Article
Title
Pediatric Histiocytic Necrotizing Lymphadenitis: a Diagnostic Challenge Suitable for Fine Needle Aspiration Biopsy
Author
Rieko Kondo Tsutomu Watanabe Mari Kubota Ayumi Tomimoto Takako Taniguchi Koichi Shichijo Akiyoshi Takahashi and Tadanori Nakatsu
Division of Pediatrics, Tokushima Red Cross Hospital
Abstract
In the present study, we examined 15 patients aged 16 years of age or younger, who were given diagnoses of histiocytic necrotizing lymphadenitis. We retrospectively assessed their clinical characteristics, laboratory findings, treatment, and prognosis over the past 5 years. A histological lymph nodes examination was performed in all 15 patients: including excisional biopsy in 1 and fine needle aspiration cytology (FNAC) in the other 14 patients. Typical pathological findings were detected on FNAC in most patients, which led to a diagnosis of histiocytic necrotizing lymphadenitis. The median duration of fever was 12 days (range 5-27 days), and the cervical nodes were affected in all patients. The mean white blood cell count was 2,180/μL(range 1,450-3,510/μL), the mean serum lactate dehydrogenase level was 426 IU/L(range 212-1,448 IU/L), and the mean serum ferritin level was 231 ng/mL(range 27-3,035 ng/mL). Moreover, the mean serum soluble interleukin-2 receptor level was 756 U/mL(range 230-1,246 U/mL). Seven patients underwent serum antinuclear antibody level testing, and only 1 patient exhibited an elevation of this level. Twelve patients underwent treatment with prednisolone, and the body temperature returned to normal within 24 hours in 9 patients. The other 3 patients spontaneously achieved defervescence within an average of 12 days. Only 1 patient experienced recurrence, and no complications, including systemic lupus erythematosus, were noted. In conclusion, we believe that FNAC of the lymph nodes is easy to perform and can be useful for making a correct diagnosis in patients with histiocytic necrotizing lymphadenitis.
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Original Article
Title
A Questionnaire Survey on Vaccination of Children with Febrile Seizures, Epilepsy and Severe Psychomotor Handicaps
Author
Takuya Tanabe1)3) Tatsuo Nishimura1)4) Hirofumi Kurose1)5) Tsuyoshi Yamazaki1)6) Ichiro Maki1)7) Yuko Imakita1)8) Kazuyo Nishijima1)9) Tohru Matsushita1)10) Gen Unishi1)11) and Minoru Ogawa2)12)
1)Academic Division, Osaka Pediatric Association
2)Osaka Pediatric Association
3)Tanabe Children's Clinic
4)Nishimura Pediatric Clinic
5)Kurose Pediatric Clinic
6)Yamazaki Children's Clinic
7)Department of Pediatrics, Ikeda Municipal Hospital
8)Department of Pediatrics, Asakayama Hospital
9)Nishijima Family Clinic
10)Matsushita Kid's Clinic
11)Unishi Children's Clinic
12)Ogawa Clinic
Abstract
The aim of this study was to investigate the understanding of and compliance with vaccination guidelines for children with febrile seizures (Fs), epilepsy (Epi), and children with severe psychomotor handicaps.
Subjects and Methods; A questionnaire survey was sent to 655 primary care doctors belonging to the Osaka Pediatric Association in July of 2012, of whom 219 doctors (33.4%) responded.
Results; Of these, 193, 162, and 140 doctors responded that they understood the guidelines for children with Fs, Epi, and severe psychomotor handicaps, respectively. A 2-3 month observation period from the last seizure until vaccination was acceptable for Fs according to 143 physicians. On the other hand, 70 doctors responded that a shorter duration was acceptable. Six doctors answered that vaccination was contraindicated for children with febrile status epilepticus. According to 159 doctors, an observation period of 2-3 months after the last seizure was acceptable for well controlled epilepsy. On the other hand, 43 doctors responded that a shorter duration was adequate. Vaccination was judged by 10 physicians to be contraindicated when the epileptic seizure was intractable, and by 13 doctors when the epileptic seizure was provoked by fever. In cases with severe psychomotor handicaps, some doctors responded that the advice of a pediatric neurologist was necessary.
Conclusions; Guideline for Fs and well controlled Epi were widely and well-accepted by primary care doctors. It might be feasible to shorten observation periods from the last seizure until vaccination. On the other hand, vaccination for children with intractable epilepsy and severe psychomotor handicaps requires cooperation between primary care doctors and pediatric neurologists.
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Case Report
Title
A Case of Pyridoxine-Responsive Seizures Caused by Infantile Hypophasphatasia
Author
Yosuke Kosugi1) Hideomi Asanuma1) Yoko Hoshino1) Satoko Noguchi1) Shuku Ishikawa1) Yuichi Niida1) Shinobu Fukumura2) and Toshimi Michigami3)
1)Department of Neonatology, Hokkaido Medical Center for Child Health and Rehabilitation
2)Department of Neurology, Hokkaido Medical Center for Child Health and Rehabilitation
3)Department of Environmental Medicine, Osaka Medical Center and Research Institute for Maternal and Child Health
Abstract
We report a case of infantile hypophosphatasia with pyridoxine-responsive seizures, presenting in the neonatal period. A 3-day-old male neonate was transferred to our neonatal intensive care unit with seizures. He had no respiratory insufficiency on admission. Serum alkaline phosphatase levels were very low (37 U/l) with hypercalcemia, elevated urinary excretion of phosphoethanolamine, and radiological evidence of osteopenia. The seizures could not be controlled by midazolam and phenobarbital, and electroencephalography remained abnormal. Therapy was therefore switched to pyridoxine, which controlled the seizures and resolved the electroencephalography abnormalities. Based on the clinical findings and course, we diagnosed infantile hypophosphatasia. A genetic study of the patient's tissue-nonspecific alkaline phosphatase gene revealed compound heterozygous mutations derived from both the mother and father. He was discharged on day 37; however, he gradually developed respiratory compromise and required intubation and mechanical ventilation at age 3 months. Not all patients present with respiratory insufficiency at an early stage and careful follow-up is often required. This case serves as a reminder that refractory neonatal seizures may be pyridoxine-responsive. Currently, no definitive treatment is available for infantile hypophosphatasia, although recombinant bone-targeted alkaline phosphatase replacement therapy is currently under trial.
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Case Report
Title
A Case of Pediatric Wernicke's Encephalopathy due to Excessive Intake of Isotonic Drinks
Author
Tsutomu Shioda1) Seiji Watanabe2) Takanori Kyogoku1) Hiroyuki Kato1) Yoshinori Okumura2) and Hideo Aiba2)
1)Department of Emergency and General Pediatrics, Shizuoka Children's Hospital
2)Department of Pediatric Neurology, Shizuoka Children's Hospital
Abstract
Wernicke's encephalopathy is caused by thiamine (Vitamin B1) deficiency. Most adults who develop this condition are alcoholics, however, pediatric cases are increasing. We report the case of an infant who consumed excessive isotonic drinks and developed Wernicke's encephalopathy.
A 10-month-old girl who had consumed 1 L of isotonic drinks per day for 4 months presented with recurrent vomiting. On examination, weight loss and physical regression were detected. She was admitted and received intravenous fluids containing glucose, but not vitamins. After a few days, she became unconscious. Blood tests revealed a low level of thiamine and an MRI revealed bilateral lesions in the basal ganglia and medial thalami. She was diagnosed with Wernicke's encephalopathy and was treated with high-dose parenteral thiamine. The treatment was effective and she recovered without permanent damage.
It is difficult to diagnose thiamine deficiency early because pediatric cases often exhibit non-specific symptoms, like vomiting and diarrhea. We need to determine if there is a history of nutritional deficiency or excessive intake of isotonic drinks. To prevent similar cases occurring, we have to increase awareness that excessive intake of isotonic drinks can be harmful to children.
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Case Report
Title
A Term Neonate, Born to a Mother with Gestational Thrombocytopenia, Who Exhibited Positive Results for Human Leukocyte Antigen Antibody with Neonatal Alloimmune Thrombocytopenia
Author
Toru Watanabe Tomoka Matsuda Ryoichi Kitagata Iwao Tajima Hiroyuki Ono Keiko Hirano Masami Shirai Akira Endo and Teruaki Hongo
Department of Pediatrics, Iwata City Hospital
Abstract
We report the case of a male infant-weighing 3,230 g and born at 38 weeks and 1 day of gestation-who presented with thrombocytopenia from day 1 after birth. His mother had thrombocytopenia at delivery. Blood examination of the infant on day 3 after birth indicated a platelet count of only 2.5×104/μL. However, he did not require platelet transfusion therapy and the thrombocytopenia spontaneously resolved without any complications within 11 days. Anti-human leukocyte antibodies, which reacted with platelets from the infant and those from his father, were detected in the infant's serum and his mother's serum. Thus, we diagnosed neonatal alloimmune thrombocytopenia. We believe that the platelet count should be carefully monitored in infants born to mothers who exhibit thrombocytopenia during pregnancy.
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Case Report
Title
A Case of Pathydermatodactyly
Author
Masaki Shimizu1) Noboru Igarashi2) and Akihiro Yachie1)
1)Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University
2)Department of Pediatrics, Toyama Prefectural Central Hospital
Abstract
Pachydermatodactyly is a rare benign disorder characterized by painless fusiform swelling of the soft tissues around the proximal interphalangeal (PIP) joints of the hands. The benign interpahalangeal soft tissue swelling characteristic of pachydermatodactyly may sometimes be misdiagnosed as polyarticular juvenile idiopathic arthritis (JIA). The patient, a 14-year-old boy, presented with a 6-month history of bilateral periarticular swelling around PIP joints. Physical examination revealed obvious diffuse soft tissue enlargement of the PIP joints of the 2nd, 3rd and 4th fingers of both hands, which was more noticeable on the lateral aspects of the fingers. Swelling involving PIP joints was not tender or warm, and there was full passive range of motion without pain. Laboratory data, including complete blood cell count, C-reactive protein level, erythrocyte sedimentation rate were normal. Plain X-ray and magnetic resonance imaging of the hands revealed soft tissue thickening, but synovitis and osseous abnormalities were not seen. Skin biopsy showed hyperkeratosis, with increased collagenous stroma in the dermis, and there was no associated evidence of inflammatory infiltrate or neoplasm. The combination of noninflammatory enlargement of the soft tissue of the PIP joints and results of skin biopsy confirmed the diagnosis of pachydermodactyly. Pachydermodactyly should be considered in cases with painless swelling around the PIP joints of the hands.
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Case Report
Title
Difficulty in Diagnosing a Laryngotracheoesophageal Cleft Leading to Respiratory Disorder in a Patient with Vascular Ring Involvement
Author
Mizuhiko Ishigaki1)2) Yoshihiko Kodama2) Keiji Tsuchiya1) and Tadasi Kawakami2)
1)Department of Pediatrics, Japanese Red Cross Medical Center
2)Department of Neonatology, Japanese Red Cross Medical Center
Abstract
We report a case of prolonged respiratory disorder caused by a laryngotracheoesophageal cleft that was difficult to diagnose in a patient who underwent vascular ring surgery. It took approximately 6 months to diagnose the cleft because of the absence of recognition of this disease and the presence of a vascular ring and tracheobronchial malacia.
Laryngotracheoesophageal cleft is a rare congenital anomaly caused by the lack of separation between the laryngotracheal axis and esophagus, often presenting with respiratory disorders. To understand this disease, we reviewed its clinical features by summarizing various pathological studies and Japanese case reports. The disease outcome can be fatal depending on cleft depth; therefore, bronchoscopy for early diagnosis is necessary in neonates exhibiting abnormal aspiration. This patient had prolonged respiratory disorder after vascular ring surgery; however, because of the absence of recognizable disease, the diagnosis of laryngotracheoesophageal cleft was delayed. Therefore, it is important to accurately evaluate the pathology in patients with various involvement.
This case indicates the importance of early diagnosis using bronchoscopy and consideration of a laryngotracheoesophageal cleft as a differential diagnosis in neonates with respiratory disorders accompanied by abnormal aspiration.
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