|
THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
|
Vol.119, No.6, June 2015
|
Original Article
Title
Growth Outcome at Term in Very and Extremely Low Birth Weight Infant by Early Aggressive Nutrition (EAN)
Author
Motohiro Taki1)2) Manabu Suzuki2) Yuuya Nakano2) Tokuo Miyazawa2) Motoichirou Sakurai2) Fumihiro Miura2) Katsumi Mizuno2) and Kazuo Itahashi2)
1)Department of Pediatrics, Tokyo Metropolitan Health and Medical Treatment Corporation Ebara Hospital
2)Department of Pediatrics, Showa University School of Medicine
Abstract
Objective: We sought to evaluate the association between early aggressive nutrition (EAN) and growth outcome at term in very low and extremely low birth weight neonates (VLBWI).
Subject and Method: A total of 319 VLBWI were admitted to the neonatal intensive care unit of Showa University Hospital between 2003 and 2013. We changed nutritional management in July 2007. Infants received EAN (provided ≥2.0 g/kg/day of amino acids (AA) within 24 hours [maximum ≥3.0 g/kg/day]) after July 2007. We classified infants around July, 2007.
Results: EAN was associated with significantly better growth at term (weight, height and head circumference). The incidence of extrauterine growth restriction (EUGR) was significantly lower (weight and height). The factors associated with SD score at term were EAN, SD score at birth and day of complete enteral feeding (100 ml/kg/day) by multiple regression analysis.
Conclusions: It was thought that the induction of EAN for the very low and extremely birth weight infant might contribute to better growth at term and prevention of EUGR. However, the evidence is still insufficient, and a large scale prospective study and long-term follow-up are necessary.
|
|
Original Article
Title
Risk Factors for Aortic Dilation and Heart Disease in Patients with Turner Syndrome Determined Using MRI
Author
Megumi Matsuoka1) Masaru Miura1) Takuya Tamame1) Shino Jinbo1) Hirotaka Ohki1) and Junko Igaki2)
1)Cardiovascular Department, Tokyo Metropolitan Children's Medical Center
2)Department of Endocrinology, Tokyo Metropolitan Children's Medical Center
Abstract
Little is known about sudden death caused by aortic dilation and dissection in Japanese patients with Turner syndrome (TS). We measured the aortic diameter in 36 patients with TS (age: 10-19 years; n=13: age 20-48 years; n=23) and investigated risk factors for aortic dilation and heart disease using MRI.
Heart disease found in 11 patients comprised bicuspid aortic valve (BAV; n=2), aortic regurgitation (n=2), aortic coarctation (COA; n=3), partial anomalous pulmonary venous connection (n=4) persistent left superior vena cava (n=3), atrial septal defect (n=3) and mitral stenosis (n=1). Heart disease was revealed for the first time in 5 of these patients. Aortic dilation (corrected aortic diameter=diameter of ascending aorta/body surface area>20 mm/m2) was discovered in 4 patients among whom, 3 aged>20 years had hypertension and other risk factors (BAV, n=1; COA, n=2), and one 10-year-old had BAV. These results suggest that MRI can help to determine heart disease in older individuals with TS who are at risk for aortic dilation. The aortic diameter should be regularly monitored using MRI, since aortic dilation might develop with age.
|
|
Original Article
Title
Efficacy and Safety of Levetiracetam in 85 Patients with Childhood Refractory Epilepsy
Author
Yuko Hirata1)2) Shin-ichiro Hamano1)3) Ryuki Matsuura2) Motoyuki Minamitani1) Manabu Tanaka3) Kenjiro Kikuchi1)2) and Hiroyuki Ida2)
1)Division of Neurology, Saitama Children's Medical Center
2)Department of Pediatrics, Jikei University School of Medicine
3)Department for Child Health and Human Development, Saitama Children's Medical Center
Abstract
To assess the efficacy and tolerability of levetiracetam (LEV) including its use in combination treatments in patients with childhood refractory epilepsy, we retrospectively reviewed the medical records of 85 patients aged 1.0-28.2 years (median 9.2 years) at the time they were started on LEV. All had experienced seizures more than once within a 3-month period and had used 1-14 antiepileptic drugs (median 4.0) before starting LEV. The response rate (patients with a reduction in seizure frequency ≥ 50%; RR) was 44.7%, and 18.8% of whom became seizure-free for more than 6 months. The RR of patients with partial seizures was 50.0% and that of patients with generalized seizures was 23.5% (p=0.049). Eleven patients (12.9%) had an increase in seizures. Adverse events occurred in 26 patients (30.6%) and LEV was eventually discontinued in 17 due to seizure exacerbation or agitation. The RR of LEV combined with carbamazepine or lamotrigine was higher than that of other combinations. Among all combinations, adverse events were more frequent for a combination of LEV with phenytoin or phenobarbital and less frequent with zonisamide or lamotrigine. Overall, LEV was effective as an add-on therapy in patients with childhood refractory epilepsy, particularly in those with partial seizures. To ensure the safe use of LEV, more attention should be paid to combination treatments.
|
|
Original Article
Title
Symptoms of Child Stress Related to the Great East Japan Earthquake
Author
Youichi Tomita1)2) Aya Takeyama1) Masaki Ito1) and Mitsuaki Hosoya2)
1)Department of Pediatrics, Soma Public General Hospital
2)Department of Pediatrics, Fukushima Medical University
Abstract
The Great East Japan Earthquake occurred on March 11, 2011. Soma City in Fukushima Prefecture, where the Soma Public General Hospital is situated, was significantly damaged by the tsunami. Many children were subsequently examined for symptoms that appeared to indicate stress disorders related to the disaster. To clarify the clinical features of these children, we retrospectively examined the children who had been referred to our hospital as outpatients. The subjects comprised children between the ages of 3 and 14 years (as of March 2011) who were examined over 1 year at our hospital following March 11, 2011. A total of 1,701 children were referred to us as outpatients; 28 children were diagnosed as having stress symptoms related to the disaster. The most common symptoms were headache, abdominal pain, vomiting, and sleep disorder. Symptoms improved in 13 of the 28 children through pediatric outpatient counseling, but the remaining 15 required psychiatric treatment. Ultimately, 6 children were diagnosed with posttraumatic stress disorder (PTSD) in the psychiatric department. Examination of the tsunami victims and their residences revealed that 4 of the 6 children had lost close friends or immediate family. After a disaster, stress symptoms related to the disaster may not be immediately evident in daily medical practice. Therefore, it is important to differentiate these cases through sufficient interviews and examination. Furthermore, because disasters are known to be related to PTSD, caution is required for children who may suffer from serious psychological damage.
|
|
Original Article
Title
Orthostatic Dysregulation: A Report of 132 Children and Discussion of Factors Influencing School Non-Attendance
Author
Miki Inutsuka and Katsuhiko Yamada
Department of Pediatrics, Sasebo Chuo Hospital
Abstract
We divided 132 children with orthostatic dysregulation (OD) into two groups: Group A comprising 73 children who had a tendency to skip school and Group B comprising 59 children who did not have this tendency. The following factors were compared between the two groups: OD symptoms, age, sex, changes in heart rate and systolic blood pressure during the orthostatic test, and the presence of comorbid psychosomatic and developmental disorders. Difficulty in waking up in the morning, abdominal pain, and malaise, which are all OD symptoms, were significantly more common in Group A, and therefore appeared to influence the tendency to skip school. A total of 24% of the children could not endure the orthostatic test for even 10 min because of dizziness or uneasiness. This suggested that school attendance of some children was affected because of severe symptoms of circulatory failure. No significant differences in the rate of comorbid postural orthostatic tachycardia syndrome were noted between the two groups. However, Group A had relatively higher heart rates after standing for 10 min during the orthostatic test. Dizziness was the most common symptom, observed in 92% of the children of postural orthostatic tachycardia and 100% of delayed orthostatic hypotension. No significant differences were observed in the presence of comorbid psychosomatic diseases including migraine (n=16), and irritable bowel syndrome (n=6). Developmental disorders were noted in 18 of the 132 children (14%), the most common being pervasive developmental disorder, which was present in 14 children. However, no significant difference was noted between the two groups regarding developmental disorders. These results suggested that irrespective of whether they have a tendency to skip school, many children with OD may have developmental disorders and therefore require attention.
|
|
Original Article
Title
Clinical Characteristics of 100 Patients with PFAPA
Author
Masahiro Furumoto1) Mayumi Okada1) Naoko Shiba1) Yuta Maruyama1) Tomonori Shigemura1) Norimoto Kobayashi1) Kenichi Koike1) Sho Houkibara2) Hitoshi Kanda3) Kimiko Honda4) Yoh Umeda4) and Kazunaga Agematsu5)
1)Department of Pediatrics, Shinshu University School of Medicine
2)Department of Pediatrics, Suwa Red Cross Hospital
3)Department of Pediatrics, Iiyama Red Cross Hospital
4)Showa University, Northern Yokohama Hospital, Children's Medical Center
5)Department of Infection and Host Defense, Graduate School of Medicine, Shinshu University
Abstract
The syndrome of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) is the most common cause of periodic fever in childhood. This study investigated the clinical characteristics of 100 children (56 boys and 44 girls) with PFAPA to characterize this syndrome in pediatric patients. There was no significant gender difference in our cohort. The average onset age was 2.9 years, and 56% of subjects had a family history of periodic fever or tonsillectomy. The average maximum temperature of fever attacks was 39.7°C, the average interval between episodes was 1.1 months, and the mean duration of episodes was 4.7 days. Some patients were noticeably fatigued during attacks. Major accompanying symptoms were pharyngitis/tonsillitis with white spots, cervical adenitis, aphthous stomatitis, sore throat, headache and vomiting. At the onset of fever, white blood cell counts were typically normal or increased without a left shift of neutrophils. CRP and serum amyloid A levels were elevated in all patients. Serum IgD was increased in 36.8% of subjects (average 26.4 mg/dl). Patients not exhibiting serious malaise during fever attacks were followed without any treatment. We observed that 53% of patients treated with preventive cimetidine therapy showed improvement, and symptoms including fever were improved dramatically by oral administration of prednisolone. Tonsillectomy was also effective for resolution of symptoms. Although mild cases of PFAPA tended to resolve spontaneously in several years, cases of symptoms persisting for more than eight years were also encountered. Our findings demonstrate that family history, frequency and duration of attacks, accompanying symptoms, serum inflammation data, serum IgD levels and responses to prednisolone treatment are all useful for the diagnosis and management of pediatric PFAPA.
|
|
Original Article
Title
Use of Low Urinary Tract Symptom Scores for Evaluating Dysfunctional Voiding in Children with Daytime Incontinence
Author
Hirokazu Ikeda Tsuneki Watanabe Masaki Fuyama and Keiichi Isoyama
Department of Pediatrics, Showa University Fujigaoka Hospital
Abstract
Background: Dysfunctional voiding (DV) is a serious lower urinary tract dysfunction that may result in lower urinary tract symptoms (LUTS) as well as vesicoureteral reflux and recurrent urinary tract infections. Recently, there have been reports of trials that have diagnosed DV using a LUTS score. However, the cut-off value for diagnosis is unknown. Here we investigated and reported on the cut-off value for the LUTS score used to arrive at a diagnosis of DV.
Subjects and methods: Subjects consisted of outpatients seen for diurnal incontinence during the past 2 years, who were 5 years or older but younger than 15 years and experienced incontinence more than 4 times a week, and who were able to be followed up after voiding and lifestyle guidance for 12 weeks or more. Two consecutive uroflowmetry measurements were taken, and subjects with a staccato pattern observed on the urine flow curve and a residual urine volume of 20 mL or greater were designated as the DV group. The other patients were designated as the non-DV group. LUTS scores in the DV and non-DV groups were compared, and a cut-off value was investigated using the receiver operating characteristics curve in the DV group.
Results: Of the 97 subjects studied, 20 (20.6%) were in the DV group and 77 (79.4%) were in the non-DV group. If a LUTS score cut-off value of 10 was used for the subjects in the DV group, the sensitivity was 85%, specificity was 66.2%, and the area under the curve was 0.816.
Conclusion: We elucidated a cut-off value for the LUTS score to be used when diagnosing DV and expect it to become a useful tool during diagnostic screening for DV.
|
|
Original Article
Title
Ultrasound Imaging Measurement of Skin Thickness in Children to Guide Proper Intradermal Vaccine Injection
Author
Hiroko Sumita1) Norio Hanzawa2) Tsuyoshi Matsuyama3) and Masaaki Mori4)
1)Sumita Kodomo Clinic
2)Shimonagaya Kodomo Clinic
3)Matsuyama Clinic
4)Department of Pediatrics, Yokohama City University Medical Center
Abstract
Intradermal administration is known as an effective route for vaccination. The intradermal influenza vaccine newly approved in the EU in 2009 has reduced amount of antigen and is highly immunogenic. The vaccine is provided with a dedicated intradermal injection device to simplify the intradermal administration procedure. The device is designed to achieve intradermal injection by inserting a 1.5 mm-long needle perpendicularly into the skin of the deltoid region. However, the practicality of its application in children is unknown since the current needle design was developed based on the thickness of adult skin. For influenza vaccination in Japan, the dose and schedule in children over the age of 13 is the same as for adults. We studied the skin thickness of children aged 13-15-years old (n=82) using ultrasound imaging. The mean skin thickness in the deltoid region was 1.81 mm, and the minimum recorded was 1.25 mm. There were no significant differences between males and females. In the suprascapular region, the mean skin thickness of males was 2.60 mm, and was significantly thicker than that of females (2.28 mm). The minimum was 1.51 mm. In the deltoid region, 16% (n=13) of the subjects had skin thickness under 1.5 mm. Children aged 13-15 years old have thinner skin than adults. For application of the intradermal injection device in children aged over 13 years old, we suggest shortening the needle or changing the injection site to an area with thicker skin such as the suprascapular area.
|
|
Case Report
Title
A Suspected Case of Spinal Cord Infarction Following Minor Trauma
Author
Syunsuke Nagara1) Yuji Nakamura1) Kaori Aiba1) Mari Sugimoto1) Norihisa Koyama1) and Kenji Yokoti2)
1)Department of Pediatrics, Toyohashi Municipal Hospital
2)Department of Pediatric Neurology, Seirei-Mikatahara General Hospital
Abstract
We report a case of suspected spinal cord infarction in a 2-year-old girl who developed flaccid paralysis of all limbs following minor head trauma. Prominent spinal swelling and a transverse lesion extending from the lower medulla oblongata to the Th2 level were revealed on spinal magnetic resonance imaging. The vascular supply through both the anterior and posterior spinal arteries was affected. The cause was considered to involve either fibrous cartilage embolism or vertebral artery occlusion, resulting in marked reduction of blood flow secondary to spasm. Cases of spinal cord infarction in children with minor head trauma are rare. We report the details of various medical examinations and the clinical manifestations.
|
|
Case Report
Title
Streptococcus pneumoniae Meningitis with Polyagglutination Caused by T Activation: A Case Report
Author
Ryosuke Matsuno Rei Ogawa Misa Nittono Yutaka Tanaka Tuneki Watanabe Takahiro Nishioka Hirokazu Ikeda and Keiichi Isoyama
Department of Pediatrics, Showa University Fujigaoka Hospital
Abstract
Polyagglutination (PA) is the unexpected agglutination of red blood cells by nearly all sera samples from adults but not by those from cord blood and newborns. PA occurs because of altered sugar chain structures on the cell surface resulting from antigen exposure associated with conditions such as bacterial infection, hematopoietic disease, malignancy, and inherited disease. We report a case of Streptococcus pneumoniae meningitis concomitant with PA caused by T activation. The patient was a 9-month-old girl who was admitted to our hospital because of continuous convulsions. S. pneumoniae meningitis was diagnosed. While treating the anemia, we observed non-specific agglutination during a minor crossmatch. PA caused by T activation was suspected because the patient's blood cells agglutinated in response to Arachis hypogea and Glycine soja during the lectin test. However, given the emergency of the situation, we initiated red cell concentrate (RCC) transfusion with a weak positive reaction in the minor crossmatch. Acute hemolytic anemia occurred 4 h after transfusion. PA detected via the adult/cord blood reaction and T antigen was considered responsible for this event. We believe that washed RCC should be transfused into PA patients; however, there are only few reports of hemolysis associated with RCC transfusions, which may involve smaller plasma volumes. These results indicate that minor crossmatch is important during blood transfusion. In case of a severe pediatric infection such as meningitis, we should consider the possibility of PA and be more careful during transfusion.
|
|
Case Report
Title
A Case of Ceftriaxone Allergy Diagnosed by Graded Challenge
Author
Shigeyuki Narabayashi1) Ikuo Okafuji1) Kanako Maizuru1)2) Yuya Tanaka1) and Satoru Tsuruta1)
1)Department of Pediatrics, Kobe City Medical Center General Hospital
2)Department of Pediatrics, Shiga Medical Center for Children
Abstract
We describe a case of a 3-year-old boy who suffered anaphylaxis after being administered ceftriaxone (CTRX) intravenously. A drug provocation test (DPT), also referred to as a "graded challenge", is the gold standard for establishing or excluding the diagnosis of hypersensitivity to a certain substance, but carries a certain amount of risk. As a result, the diagnosis of drug allergy is usually reliant upon the results of skin tests. We conducted a DPT, and it confirmed that the patient had a positive reaction to CTRX. To provide safe alternatives, we undertook skin tests and the basophil activation test (which is now accepted as an additional and reliable diagnostic tool for evaluating drug allergy) for penicillin, carbapenem, a first-generation cephalosporin, and a third-generation cephalosporin. Both tests were negative, so we conducted a DPT. The patient reacted negatively to all the test drugs except CTRX. CTRX is one of the most widely prescribed antibiotics because of its long half-life and broad spectrum of activity on Gram-positive and Gram-negative bacteria. It is also the drug of choice for the treatment of bacterial meningitis. We were able to make an accurate diagnosis of CTRX allergy and provide safe alternatives not only to prevent serious or even life-threatening reactions, but also to avoid unnecessary drug restrictions. Physicians should recognize that allergic reactions to antibiotics are the most common cause of adverse drug reactions, and thus try to ensure appropriate use of antibiotics.
|
|
Case Report
Title
Deficiency in Carnitine Palmitoyltransferase-2 after Tandem Mass Screening: A Case Study
Author
Yu Kuwabara1) Michiko Okamaoto1) Toshihiro Jyogamoto1) Takahiro Motoki1) Takeshi Nakano1) Masatoshi Hayashi1) Kenji Yamada2) Hironori Kobayashi2) and Seiji Yamaguchi2)
1)Department of Pediatrics, Uwajima City Hospital
2)Department of Pediatrics, Shimane University School of Medicine
Abstract
We describe an unusual case of a pediatric patient whose level of 3-hydroxy-hexadecanoyl carnitine was above the upper cutoff value according to an initial tandem mass screening test (TMST). Follow-up was not possible, because the patient died suddenly of influenza at the age of 9 months although the second TMST was normal. Laboratory findings suggested that he may have had a congenital metabolic disorder. Post-mortem, the free carnitine value was below the cutoff value in the acute phase. Serum analyses showed an increase in the level of long-chain acylcarnitine. We diagnosed carnitine palmitoyltransferase (CPT2) deficiency, which was confirmed by genetic analyses. TMST can detect CPT2 deficiency, but this is considered to be a second-line investigation because diagnostic accuracy is not high, thus, CPT2 deficiency can be misdiagnosed or overlooked. However, the diagnostic robustness of TMST for CPT2 deficiency has been revised. By retrospectively examining blood spots of our patient 5 days post-mortem, we were able to detect abnormalities in the new markers of this deficiency. We hope that mass screening for CPT2 deficiency can become a first-line investigation in all examination centers in Japan in the near future.
|
|
|
Back number
|
|
