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Kamisawa T.,Tokyo Metropolitan Komagome Hospital | Ando H.,Aichi Prefectural Colony | Hamada Y.,Kansai Medical University | Fujii H.,Yamanashi University | And 4 more authors.
Journal of Hepato-Biliary-Pancreatic Sciences | Year: 2014

Pancreaticobiliary maljunction is a congenital malformation in which the pancreatic and bile ducts join anatomically outside the duodenal wall. The diagnostic criteria for pancreaticobiliary maljunction were proposed in 1987. The committee of The Japanese Study Group on Pancreaticobiliary Maljunction (JSGPM) for diagnostic criteria for pancreaticobiliary maljunction began to revise the diagnostic criteria from 2011 taking recently advanced diagnostic imaging techniques into consideration, and the final revised version was approved in the 36th Annual Meeting of JSPBM. For diagnosis of pancreaticobiliary maljunction, an abnormally long common channel and/or an abnormal union between the pancreatic and bile ducts must be evident on direct cholangiography, such as endoscopic retrograde cholangiopancreatography, percutaneous transpehatic cholangiography, or intraoperative cholangiography; magnetic resonance cholangiopancreatography; or three-dimensional drip infusion cholangiography computed tomography. However, in cases with a relatively short common channel, it is necessary to confirm that the effect of the papillary sphincter does not extend to the junction by direct cholangiography. Pancreaticobiliary maljunction can be diagnosed also by endoscopic ultrasonography or multiplanar reconstruction images provided by multi-detector row computed tomography. Elevated amylase levels in bile and extrahepatic bile duct dilatation strongly suggest the existence of pancreaticobiliary maljunction. © 2013 Japanese Society of Hepato-Biliary-Pancreatic Surgery.


Morimoto A.,Jichi Medical University | Oh Y.,Jichi Medical University | Shioda Y.,National Center for Child Health and Development | Kudo K.,Shizuoka Childrens Hospital | Imamura T.,Kyoto Prefectural University of Medicine
Pediatrics International | Year: 2014

The purpose of this review is to provide an updated overview of the pathogenesis and treatment of Langerhans cell histiocytosis (LCH). The pathogenesis of LCH remains obscure and the optimal treatment for LCH has not been established, although incremental progress has been made. Proinflammatory cytokines and chemokines are known to play a role in LCH, which suggests that LCH is an immune disorder. However, the oncogenic BRAF mutation is also detected in more than half of LCH patients, which suggests that LCH is a neoplastic disorder. Remaining major issues in the treatment of LCH are how to rescue patients who have risk-organ involvement but do not respond to first-line therapy, the optimal treatment for the orphan disease of multifocal adult LCH, and how to reduce and treat central nervous system-related consequences, such as central diabetes insipidus and neurodegeneration. More research is needed to better understand the pathogenesis of this disease and to resolve the treatment issues. © 2014 Japan Pediatric Society.


Suzuki T.,Osaka City General Hospital | Sumitomo N.,International University of Japan | Yoshimoto J.,Shizuoka Childrens Hospital | Miyazaki A.,National Cerebral and Cardiovascular Center | And 3 more authors.
Circulation Journal | Year: 2014

Background: In Japan, the use of implantable cardioverter defibrillators (ICDs), cardiac resynchronization therapy with a biventricular pacemaker (CRTP) and CRT with a defibrillator (CRTD) in children has not been studied statistically, and dual-chamber (DDD) pacemakers are still used for pediatric CRT because of current government regulations. Methods and Results: Data were obtained from 15 children's and 74 general hospitals through a questionnaire survey regarding the aforementioned therapies performed before 2012 in Japanese children (<16 years old). ICD, CRT with DDD, CRTP and CRTD were used in 64 (42%), 47 (31%), 34 (22%) and 7 (5%), respectively, of all cases reported (n=152). Among all CRTP and CRT-DDD cases (n=81), the use of DDD accounted for 41% in general hospitals vs. 89% in children's hospitals, and CRT-DDD and CRTP were effective in 67 cases (83%). Of 64 ICD cases, appropriate shocks were experienced in 28 cases (44%), and inappropriate shocks in 19 cases (29%). Additionally, data from the Japan Arrhythmia Device Industry Association obtained for overall device usage analysis revealed that CRTP was more commonly used in children than in adults. Conclusions: There is an increasing need for pediatric device therapy, especially CRTP. However, many children's hospitals were still using DDD pacemakers in 2012. Although the demand for device therapy in children may be small, it is indispensable in pediatric cardiology.


Park S.,Shizuoka Childrens Hospital
Japanese Journal of Plastic Surgery | Year: 2011

The speech outcome was studied retrospectively in 36 isolated cleft palate patients who underwent intravelar veloplasty. The mean age at the most recent postoperative evaluation was 8 years and 6 months (range, 4∼14 years). Acceptable velopharyngeal function (normal or good) was noted in 33 patients (91.7%). Articulation disorders were observed in 3 patients (lateralized articulation 2 cases, palatalized articulation 1 case). These results were compared to the results of those after Furlow's procedure. There were no significant differences in postoperative speech assessments between the two palatoplasties.


Kimura M.,Shizuoka Childrens Hospital | Oh S.,Shizuoka Childrens Hospital | Narabayashi S.,Shizuoka Childrens Hospital | Taguchi T.,Shizuoka Childrens Hospital
International Archives of Allergy and Immunology | Year: 2012

Background: Bottle-fed infants sometimes develop intestinal cow's milk allergy (ICMA). Because cow's milk-specific IgE antibody (CM-IgE) levels are normal, the lymphocyte stimulation test (LST) has been proposed as an alternative diagnostic test for ICMA. The present study evaluated the diagnostic value of LST in a large number of patients with ICMA in Japan. Methods: Ninety-six infants who developed intestinal symptoms after ingestion of cow's milk formula and showed remission of symptoms after elimination of this food were enrolled as patients with probable ICMA. Seventy-two subjects with normal CM-IgE levels and a positive result in an oral food challenge test (OFCT) for cow's milk formula were diagnosed with ICMA. Another 10 infants with normal CM-IgE levels and a negative OFCT result were diagnosed with nonspecific intestinal symptoms (NIS). The status of cell-mediated immunity against cow's milk proteins was estimated by LST for κ-casein. Results: In the 72 patients with ICMA (38 boys and 34 girls), the median age at onset was 9 days. Sixty-two of 72 (86.1%) patients with ICMA tested positive in the LST for κ-casein. In contrast, only 2 of the 10 NIS infants tested positive. The incidence of a positive LST result was significantly higher in the ICMA group than in the NIS group (p < 0.0001). The area under the receiver-operating characteristic curve for this test was as high as 0.856. Conclusions: This study strongly suggests that the LST for κ-casein is a useful diagnostic test for ICMA. Copyright © 2011 S. Karger AG, Basel.


Tashiro Y.,Shizuoka Childrens Hospital
Japanese Journal of Neurosurgery | Year: 2015

A case of Chiari II malformation (Ch-II) may be very rare in a general hospital, but knowledge of the embryological mechanism, complications and prognosis is somewhat related to various kinds of congenital central nervous system anomalies including tethered cord syndrome and so on. The author herein introduces key points to determine a prognosis of Ch-II with the presentation of clinical courses and therapeutic results of thirty-six cases experienced in our hospital from January, 2001 to December, 2013. Additionally, the historical change of the treatment method, is also described with topics including prenatal diagnosis, control of combined hydrocephalus, mental development, intensive therapies and different diagnoses in cooperation with other departments, and fetal surgery for the intrauterine repair for the myelomeningocele. © 2015, Japanese Congress of Neurological Surgeons. All rights resrved.


Susam Park M.Z.,Shizuoka Childrens Hospital
Japanese Journal of Plastic Surgery | Year: 2013

In the third week of gestation the embryo consists of three principal layers : an outer protective layer termed ectoderm, an inner nutritive layer termed endoderm, and the mesoderm. The primitive gut is not yet separate from the yolk sac. The amniotic cavity is located dorsally. The umbilical vessels develop in the extraembryonic mesoderm and connect the embryo to the developing placenta. During the fourth week of gestation, the abdominal wall begins to develop from the lateral plate of the embryonic mesoderm and forms from differential growth of the embryo causing infolding in the craniocaudal and mediolateral directions. Infolding and flexion of the embryo draw the amnion around the body. As the embryo develops, the amnion is drawn to surround the embryo and cover the developing umbilical cord. The vitelline duct and the alimentary duct are lost between the fifth and seventh weeks. Finally the umbilical cord contains the umbilical arteries, the umbilical vein, the rudimentary allantois, the remnant of the vitelline duct, and a gelatinous substance called Wharton's Jelly. The fibromuscular umbilical ring is closed by the time of birth. After separation of the cord, the umbilicus may have many appearances. A normal umbilicus is characterized by depression. Persistence of the fascial opening and umbilical hernia sometimes occurs and most often corrects spontaneously without specific treatment. Congenital abdominal wall defects that relate to the umbilicus are omphalocele and gastroschisis, and other diseases are also described.


BACKGROUND: Among the casein components, αs-casein (αs-CAS) is considered the major allergen in Japan, and there are very few reports on the allergenicity of β-casein (β-CAS). In this study, we compared the allergenicity of β-CAS with that of αs-CAS in Japanese children with cow milk allergy (CMA).METHOD: The allergenicity of αs-CAS and β-CAS in 29 CMA patients and 11 control subjects was assessed using the basophil activation test (BAT). The accuracy of the BAT to distinguish the patients with CMA from the control subjects was estimated using a receiver operating characteristic (ROC) analysis and was expressed as the area under the curve (AUC).RESULTS: BAT results for CM were positive in 93.1% of the CMA patients. The results of the β-CAS-BAT and αs-CAS-BAT were found to be positive in 86.2% and 69.0% of the CMA patients, respectively, however, the difference was not significant. The AUC for the β-CAS-BAT was 0.893, which was not significantly different from that for the αs-CAS-BAT (0.859).CONCLUSIONS: These results suggest that the allergenicities of β-CAS and αs-CAS are similar in Japanese patients with CMA.


Kinoshita K.,Shizuoka Childrens Hospital
Kyobu geka. The Japanese journal of thoracic surgery | Year: 2012

Breast deformities are seen as one of the late postoperative complications in thoracotomy, but there are very few reports in the literature. We investigated causes and treatments in 5 patients who have consulted to our department after operations for congenital heart disease between April 1989 and March 2011. The injured breast bud in the cardiac operation resulted in hypoplastic deformities, and deformities became apparent during puberty with breast growth. These patients required release of the scar contracture to lead to normal breast development, and also have to been treated chest deformity. After stopping at breast growth in adolescence, some augmentations were necessary if bilateral asymmetry had been remained. Breast deformities are very important issue in the viewpoints of patients' quality of life (QOL), though not relating directly to vital prognosis. We'd like to introduce these complications and some choices about breast reconstruction to thoracic surgeons.


Tashiro Y.,Shizuoka Childrens Hospital
Japanese Journal of Neurosurgery | Year: 2016

Occult spinal dysraphism(OSD), recognized as a twin word for cystic spinal dysraphism, is uncommon to general neurosurgeons. It is a big problem that OSD is apt to be overlooked, because the most cases might have only tiny signs of cutaneous stigmata in the lumbosacral region with insidious worse of the neurological symptoms. Moreover, the entity of OSD is complicated that it is strictly classified into many divisions-spinal lipoma, congenital dermal sinus, thickened filum terminale, terminal myelocystocele, diastematomyelia, and so on. Therefore, the proper time and method for the treatment differs in each malformation. To study the mechanisms and differences among those embryonic malformations is a valuable clue in interpreting the structural malformation on the graphical images, understanding the neurological symptoms with combined anomalies in other organs, and also learning the important keys of surgical treatment. In this paper, I have demonstrated the representative cases of OSD operated in our hospital in the classified order, and argued on what kind of cutaneous stigmata should be suspected of OSD and how we should deal with the lesion along with some discussion and review of literature. © 2016, Japanese Congress of Neurological Surgeons. All rights reserved.

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