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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
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Vol.118, No.10, October 2014
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Original Article
Title
Nonconvulsive Seizures in Children with Prolonged Febrile Seizures
Author
Azusa Maruyama Masahiro Nishiyama Kyoko Fujita and Hiroaki Nagase
Department of Neurology, Hyogo Prefectural Kobe Children's Hospital
Abstract
Objective: To clarify the prevalence of nonconvulsive seizures (NCSs) in children with prolonged febrile seizure (PFSs), the time when the first seizure was recorded on EEG, and the relationship between NCS and neurological outcome.
Method: We studied 30 children with PFS. The children underwent continuous EEG monitoring on admission to a tertiary pediatric care center at Kobe Children's Hospital from February 2007 through May 2010. Children with prior neurological abnormalities were excluded. Clinical profiles and prognosis were compared between the patients with NCSs and those without NCSs (non-NCSs).
Results: Of the 30 children, NCSs occurred in 11 children (37%). Neurological morbidity was higher in NCS patients (4/11, 36.4%) than in non-NCS patients (1/19, 5.3%; p=0.028).
Conclusion: The occurrence of NCSs in children with PFS is associated with neurological outcome.
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Original Article
Title
Susceptibility-weighted Imaging in Acute-stage Pediatric Convulsive Disorders
Author
Hiroki Iwasaki1) Yukihiko Fujita2) and Mitsuhiko Hara1)
1)Department of Pediatrics, Tokyo Metropolitan Hiroo General Hospital
2)Department of Pediatrics and Child Health, Nihon University School of Medicine
Abstract
OBJECTIVE: The purpose of this preliminary study was to describe the clinical use of acute-stage susceptibility-weighted imaging (SWI) in children with prolonged convulsive disorders.
PATIENTS: We enrolled 11 children (5 boys and 6 girls; age range, 0-7 years: average age, 3.7 years) with prolonged convulsive disorders who had undergone SWI within 2 h after their seizures had terminated (the acute-stage SWI group), and 15 control children (12 boys and 3 girls; age range, 0-15 years: average age, 7.8 years) with various conditions who had undergone SWI for reasons other than acute-stage convulsive disorders. Cerebral venous vasculatures between the groups were compared. The acute-stage SWI group was further divided into two groups: those who showed focal low signals of cerebral vein (focal group) and those who showed diffuse low signal of cerebral vein (generalized group). Inter-ictal electroencephalographic (EEG) findings and venous blood gases during seizure activity were compared between these two groups.
RESULTS: All patients in the acute-stage SWI group showed low signal of cerebral vein. Five patients (2 boys and 3 girls; age range, 0-7 years; average age, 4.7 years) were assigned to the focal group and six patients (3 boys and 3 girls; age range, 1-6 years; average age, 2.9 years) to the generalized group. Respiratory compromise during seizure activity was more severe in the focal group than in the generalized group. The areas of low signal of cerebral vein in the focal group were consistent with the areas of abnormalities in the EEG and had resolved completely in all patients. Eleven patients in the control group showed normal SWI findings and four patients in this group showed generalized low signal.
CONCLUSIONS: This is apparently the first study to demonstrate that acute-stage SWI may be a useful alternative method for differentiating focal seizures from generalized seizures in children with prolonged convulsive disorders.
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Original Article
Title
Clinical Presentations of 80 Pediatric Patients with CNS Tumors
Author
Yoshiko Nakano Kai Yamasaki Chika Tanaka Keiko Okada Hiroyuki Fujisaki Yuko Osugi and Junichi Hara
Department of Pediatric Hematology/Oncology, Children's Medical Center, Osaka City General Hospital
Abstract
Early diagnosis of pediatric brain tumor is important because delay in diagnosis can lead to irreversible neurological disorder. However, as previously reported from overseas, the average time from onset of initial symptoms to diagnosis was three months and sometimes it took more than a year. With the aim of exploring the condition in Japan, we retrospectively analyzed initial sings and symptoms of 80 children newly diagnosed with bran tumors from 2006 to 2012 in our institution.
The median interval to diagnosis was two months and 13% of patients were diagnosed more than one year later from onset of initial symptom onset. The major reasons for their hospital visits at diagnosis were headache accompanied with vomiting and impaired consciousness and seizures, followed by abnormal eye movement. Most patients were correctly diagnosed only after they showed critical neurological symptoms or several tumor-related symptoms. Some patients were initially misdiagnosed with common diseases including gastritis, benign strabismus, and psychosomatic disorders.
For better neurological prognosis of children with brain tumors, general pediatricians should consider brain tumors in the differential diagnosis.
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Original Article
Title
The Incidence of Infantile Umbilical Hernia in Japan and the Effect of Its Treatment Using a Plug and Transparent Tape
Author
Masahiro Hiraoka
Aiiku Pediatric Clinic
Abstract
The incidence of umbilical hernia was prospectively examined in Japanese infants and the effects of its treatment using a resin plug fixed by a transparent tape were retrospectively reviewed. Umbilical hernia was diagnosed during the past two years in 44 (11%) out of 400 infants aged less than four months who visited our outpatient clinic for their first vaccination. Among 62 babies diagnosed with the hernia for the past three years, 54 babies received the plug treatment. Forty nine of these infants were healed of the hernia, all their treatment were started before the age of four months. The cumulative cure rates in 54 infants who received the plug treatment before four months of age were 36% in 30 days, 75% in 60 days, and 88% in 90 days. Among 11 infants with a fascial defect diameter of 5 mm or less, the six with the plug treatment had earlier resolution of the hernia than the five without the treatment. Among the 50 children with a fascial defect diameter of 6 mm or more, all the four without the plug treatment before the age of four months had a persistent hernia or outie, while only three out of 46 children with the treatment did, and the three children had frequent interruption of the plug treatment mainly because of dermatitis. The plug treatment was interrupted more often in children with eczema in trunk than in those without the eczema. The plug treatment is, thus, effective for facilitation of resolution of the hernia and for prevention of a persistent outie.
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Case Report
Title
A Comatose, Respirator-dependent Case with Severe Brain Damage and Approach to End-of Life Care in Accordance with Advanced Care Planning
Author
Masahisa Funato Kiyoshi Baba Kiyoshi Takemoto Yoshitaka Iijima Atsuko Kashiwagi and Tamami Katayama
Department of Pediatrics, Osaka Developmental Rehabilitation Center
Abstract
We contributed to the end-of-life care provided by a palliative care team in accordance with advanced care planning in the case of a comatose, respirator-dependent patient with severe brain damage. The case was a 20 year old man with extremely severe motor and intellectual disabilities (SMID), who was born prematurely at 24 gestational weeks. He developed hypoxic, ischemic encephalopathy after the occurrence of severe asphyxia and brain hemorrhage. He required 24 hour artificial ventilation and tube feeding and was transferred to our center 5 years previously. The patient developed a hiccup associated with a sudden drop in SpO2 during feeding, when the volume was increased, Eventually during his last 2 years he began losing weight due to poor nutritional intake. Therefore in a family conference, the team together with his mother (his legal representative), discussed matters concerning the possibility of end of life care. The mother did not want invasive treatment such intravenous hyperalimentation and aggressive cardiac resuscitation upon cardiac arrest. In accordance with the policy of shared decision-making (SDM), the multidisciplinary team focused on advance care planning (ACP) on the basis of palliative care. The SDM and ACP were signed by the mother and were submitted to the ethical committee of the center. Within the committee, the SDM and ACP were approved as a formal program. According to the ACP, with the limitations of invasive treatment and withholding end-of-life treatment, an appropriate farewell was provided for the patient. The process of SDM and ACP with the family are very important aspects in supporting total care of patients with a poor prognosis while focussing on their best interests.
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Case Report
Title
A 2-month-old Boy Who Developed Intestinal Cow's Milk Allergy While Receiving a Potent Anti-inflammatory Therapy for Refractory Kawasaki Disease
Author
Masaki Shimomura1) Takaaki Meguro1) Fumika Tokunaga1) Yasunori Ito1) Shiro Seto1) Mitsuaki Kimura1) Ikuya Ueta2) and Yasuo Ono3)
1)Department of Allergy and Clinical Immunology, Shizuoka Children's Hospital
2)Division of Pediatric Intensive Care Unit, Shizuoka Children's Hospital
3)Devision of Pediatric Cardiology, Shizuoka Children's Hospital
Abstract
A 2-month-old boy receiving treatment of refractory Kawasaki disease with multiple anti-inflammatory agents developed intestinal cow's milk allergy (ICMA). The patient showed symptoms of Kawasaki disease at the age of 1 month and was treated with an intravenous immunoglobulin (IVIG) at a dose of 2 g/kg. However, the disease did not respond to the IVIG, or to second line therapy with continuous administration of prednisolone (PSL; 2 mg/kg daily) in combination with additional IVIG therapy. Because coronary arterial lesions appeared as a complication thereafter, a biological agent, infliximab (IFX), was used as the third line therapy in addition to the continuous PSL therapy. Fever subsided and laboratory findings were improved after IFX administration. However, the boy developed bloody stool 17 days after IFX administration despite the continuation of the PSL therapy. Considering that the baby was bottle-fed with a cow's milk formula, we suspected that ICMA may have caused this condition. In fact, blood in the stool disappeared after the introduction of an amino-acid formula milk, and reappeared following an oral food challenge test with a cow's milk formula. Although the result of the cow' milk-specific IgE antibody test was negative, the allergen-specific lymphocyte stimulation test (ALST) indicated positive results for beta-casein, a major component of cow's milk proteins. Based on these results, we diagnosed with ICMA. The present case indicates the difficulty in the treatment of ICMA with potent anti-inflammatory agents, which emphasizes the importance of the elimination of causal food allergens for the treatment of ICMA.
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Case Report
Title
A Case of Frasier Syndrome with Prophylactic Gonadectomy before Renal Transplantation
Author
Tatsuo Asano Shoichiro Kanda Yuko Akioka Tohaku Jo Kei Nishiyama Takayuki Miyai Noriko Sugawara Kiyonobu Ishizuka Hiroko Chikamoto and Motoshi Hattori
The Department of Pediatric Nephrology, Tokyo Women's Medical University School of Medicine
Abstract
Renal transplantation (RTx) is a therapeutic option in pediatric patients with end-stage renal disease (ESRD). A thorough evaluation of the pediatric transplant recipient is essential, and it is important to confirm the precise cause of ESRD before RTx. Frasier syndrome (FS) phenotype 46,XY usually is characterized by female external genitalia, gonadal dysgenesis, high risk of gonadoblastoma, and steroid resistant nephrotic syndrome (SRNS) with the development of ESRD. It is caused by heterozygous de novo intronic splice site mutations of the Wilms' tumor suppressor gene (WT1). We report a case of a 16-year-old 46,XY phenotypic female with FS. She developed SRNS at 3 years, and presented with ESRD at 16 years. Peritoneal dialysis was initiated and preparations for RTx were performed. As a result of the assessment of causes of delayed puberty, she was diagnosed with FS presenting the IVS9+5 G> A of WT1. Pelvic MRI showed streak gonads and elective bilateral gonadectomy was performed before RTx to avoid the potential risk of malignancy following posttransplantation immunosuppression. The histological examination bilaterally showed foci of gonadoblastoma. This case report emphasizes that we should take into consideration the possibility of FS in ESRD patients with SRNS accompanied by delayed puberty, and a definitive diagnosis, including WT1 mutation analysis, is necessary before RTx.
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Case Report
Title
Analysis of Bone Turnover Markers during Oral Calcitriol Pulse Therapy in a Patient with Craniometaphyseal Dysplasia
Author
Yusuke Takeuchi1)2) Yuji Inaba1) Hiroki Matsuura1) Kenji Kurata3) Yuka Misawa1) Mitsuo Motobayashi1) Taemi Niimi1) Takahumi Nishimura1) Naoko Shiba1) and Kenichi Koike1)
1)Department of Pediatrics, Shinshu University School of Medicine
2)Department of Pediatrics, Omachi Municipal Hospital
3)Department of Pediatrics, Matsumoto Medical Center
Abstract
Craniometaphyseal dysplasia (CMD) is a rare genetic disorder characterized by progressive thickening of the craniofacial bones and aberrant development of the metaphysis of long bones. Cranial nerve disturbances and hydrocephalus may be caused by diffuse hyperostosis of the skull base. Here, we describe the clinical course of an 11-year-old boy with CMD. The patient had displayed enlargement of head circumference, visual impairment, and hearing loss since 4 months of age. At the age of 13 months, CMD was diagnosed due to a characteristic facial appearance, several radiological features, and an ank gene mutation (c.1122-1124 del CTC, p.S375del) reported as causative for CMD. The patient was treated with oral calcitriol pulse therapy combined with a calcium-restricted diet. To evaluate the effects of the combination therapy on his bone metabolism, we assayed 4 bone turnover markers during his clinical course (bone-type alkaline phosphatase and osteocalcin as bone formation markers, and urinary-type I collagen cross-linked N-telopeptide and deoxypyridinoline as bone absorption markers). Although the levels of these markers were markedly higher than reference values of children of the same age at the onset of therapy, they all decreased during the treatment course. His clinical symptoms of macrocephaly, visual disturbance, and hearing loss did not deteriorate, and no evidence of hydrocephalus was observed. Oral calcitriol pulse therapy combined with a calcium-restricted diet may be effective for CMD.
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Case Report
Title
A Case of Pneumococcal Meningitis and Subdural Abscess after a Three-dose Series of 7-valent Pneumococcal Conjugate Vaccine
Author
Yutaka Tanaka1) Toshio Ishii1) Takahiro Nishioka1) Misa Nittono2) Ichiro Hosaki2) Hirokazu Ikeda1) and Keiichi Isoyama1)
1)The Department of Pediatrics, Showa University Fujigaoka Hospital
2)The Department of Pediatrics, Yokohama Asahi Central General Hospital
Abstract
Here we report pneumococcal meningitis in a male infant who received a 3-dose series of the 7-valent pneumococcal conjugate vaccine (PCV7). At 8 months of age, he was referred to our hospital with chief complaints of vomiting and fever. After observation of the bulging anterior fontanelle and nuchal rigidity, examination of the cerebrospinal fluid led to a diagnosis of pneumococcal meningitis. The child had previously received a 3-dose series of the 7-valent pneumococcal conjugate vaccine at 2, 3, and 6 months of age. Although treatments with ceftriaxone and panipenem appeared to be clinically effective, C-reactive protein levels and white blood cell counts were again elevated 6 days after the onset of meningitis. Magnetic resonance imaging of the head revealed subdural hygroma, and subsequent intravenous vancomycin infusions (45 mg/kg/day) reduced the fever after 4 days and improved the subdural hygroma. Because blood cultures were positive for pneumococcus serotype 33F, We hypothesized that the development of pneumococcal meningitis was caused by the fact that serotype-specific anti-33F antibodies are not included in PCV7. Although this case represents a rare condition of PCV7 resistant pneumococcal infection, several foreign reports describe the development of invasive pneumococcal disease after widespread PCV7 administration. Thus, the risk of meningitis caused by other pneumococcal serotypes is present even in patients vaccinated with PCV7.
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Case Report
Title
A Patient with Intramural Duodenal Hematoma Who Developed Ileus and Acute Pancreatitis after Abdominal Bruising
Author
Masahiro Nozawa Ryuji Sasaki and Satoshi Tsuji
National Center for Child Health and Development Emergency Service and Transport Medicine
Abstract
Traumatic intramural duodenal hematoma can be caused by abdominal bruising through a minor mechanism of injury in many cases. A 9-year-old boy bruised his right abdomen when he fell on a stone. There was no nausea or abdominal pain immediately after the event, and he returned home on foot. However, he gradually experienced abdominal pain and vomiting. Seven hours later, he was referred to our hospital. He complained of right hypochondrial tenderness, and enhanced abdominal computed tomography (CT) revealed intramural hematoma in the descending portion of the duodenum. Therefore, we observed him by inserting a stomach tube and stopping oral intake of food. On the fifth day, he developed pancreatitis, CT confirmed the increase in the hematoma, and complete obstruction of the duodenum was detected in an upper gastrointestinal series. We treated the pancreatitis and provided nutrition by a central venous catheter. On day 12, his ileus improved following the passage of bloody stool, and on day 37, he was discharged.
This disease is difficult to diagnose at the initial visit, because even after a minor injury, its symptoms worsen over time. However, duodenal hematoma may progress to ileus and acute pancreatitis, which require intensive management. Therefore, recognition of this disease is important if there is pain localized to the upper abdomen and an abdominal bruise history within a few days of a minor injury in a patient complaining of vomiting and abdominal pain.
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