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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
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Vol.117, No.5, May 2013
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Original Article
Title
Eating Disorder Counseling Worksheets: A Multidimensional and Eclectic Approach
Author
Yuko Fukuda
Department of Pediatrics, Inuyama Chuo Hospital
Abstract
Counseling and psychotherapy are essential to the treatment of eating disorders; however, few specialists are currently available in Japan, particularly in rural areas. Therefore, pediatricians in Japan often struggle with how to provide psychological treatment until the patient can be examined by a specialist. I therefore created counseling worksheets based on the etiologic factors of eating disorders, such as genetic, family, individual, sociocultural/media, pubertal and peer factors. These worksheets were used in the psycho-educational and nutritional counseling of 3 patients with eating disorders, and they proved effective. Counseling worksheets based on guided self-help cognitive-behavioral therapy enable pediatricians to implement a multidimensional and eclectic approach to the treatment of eating disorders, save time, understand eating disorder pathology, cooperate with nutritionists, and build rapport with patients. In addition, the use of counseling worksheets improves patients' communication skills, self-esteem, resilience, and family dynamics.
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Original Article
Title
Long-term Follow Up of Childhood-onset Cerebral Adrenoleukodystrophy Treated by Cord Blood Transplantation Using a Reduced-intensity Conditioning Regimen
Author
Takeo Kato1) Kenji Yoshida1) Tomonari Awaya1) Minoru Shibata1) Michihiro Yano3) Ikuko Takahashi3) Satoshi Saida1) Itaru Kato1) Hisanori Fujino4) Katsutsugu Umeda1) Hidefumi Hiramatsu1) Kenichiro Watanabe1) Souichi Adachi2) and Toshio Heike1)
1)Department of Pediatrics, Graduate School of Medicine, Kyoto University
2)Applied Laboratory Science, School of Human Health Science, Graduate School of Medicine, Kyoto University
3)Department of Pediatrics, Graduate School of Medicine, Akita University
4)Department of Pediatrics, Osaka Red Cross Hospital
Abstract
Adrenoleukodystrophy (ALD, MIM #300100), an X-linked disorder caused by the mutation of the ABCD1 gene, is a neurodegenerative disorder that affects the cerebral white matter and adrenal cortex. Among the several clinical phenotypic forms of ALD, childhood onset cerebral type (CCALD) is the most severe, resulting in rapid neurological deterioration and early death. At present, the only treatment to prevent the progression of cerebral ALD is hematopoietic stem cell transplantation (HSCT). However, several reports have demonstrated that the survival and neurological outcomes of advanced CCALD cases treated with HSCT are poor and have suggested that advanced cases are poor candidates for HSCT. Here, we report the long-term follow-up of 4 CCALD cases, including 2 advanced cases, treated by umbilical cord blood transplantation using a reduced-intensity conditioning regimen (RIC). Our treatment showed excellent results with no disease progression after HSCT. Based on our cases, we thus considered that one of the most important factors involved in the therapeutic approach contributing to a good outcome is the choice of the RIC as a preparative regimen to reduce toxicity and transplant-associated morbidity and mortality. The other factor is the correct selection of unrelated donor umbilical cord blood for early HSCT to avoid further progression of disease before HSCT.
Our study indicated that HSCT using RIC is more effective for CCALD, even in advanced cases.
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Original Article
Title
Various Vascular Lesions in a Girl with Bannayan-Riley-Ruvalcaba Syndrome
Author
Aiko Igarashi1) Ikue Hata1) Taihei Hayashi1) Masao Kawatani1) Yusei Oshima1) Mitsufumi Mayumi1) and Yoshiaki Kubo2)
1)Department of Pediatrics, Faculty of Medical Sciences, University of Fukui
2)Board of Trustees, University of Fukui
3)Department of Dermatology, Institute of Health Biosciences, The University of Tokushima Graduate School
Abstract
A girl with Bannayan-Riley-Ruvalcaba syndrome (BBRS) presented with various vascular lesions and had a heterozygous mutation of Phosphatase and tensin homologue deleted on chromosome 10(PTEN). The patient had nevus sebaceous glands and macrocephaly at birth, followed by epilepsy and developmental delay. Optic nerve glioma, intestinal polyposis, angiolipoma, and mesenteric lipoma successively developed from early childhood. At 17 years of age, the diagnostic work-up for feeding difficulty revealed portosystemic shunts, and aneurysms of the portal vein and hepatic artery. PTEN deletion is known to inhibit the phosphatidylinositol-3-kinase-dependent signaling pathway, which is involved in tumor angiogenesis and neovascularization. Thus, the PTEN mutation in this patient is implicated in her characteristic vascular lesions. In the management of BBRS patients with a PTEN mutation, systemic screening for vascular lesions and assessment of complications associated with vascular fragility should be conducted.
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Original Article
Title
A Case of Pleurisy with Recurrent Kawasaki Disease
Author
Shinya Murata1) Kenichi Okumura1) Kosuke Syabana1) Jun Shinohara1) Hideki Matsumura1) Mitsuru Kashiwagi1) Maki Koh1) Keisuke Okasora1) and Hiroshi Tamai2)
1)Department of Pediatrics, Hirakata City Hospital
2)Department of Pediatrics, Osaka Medical College
Abstract
We report a 7-year-old girl with pleurisy associated with the acute phase of recurrent Kawasaki disease (KD). She was referred to our hospital after lymphadenitis was diagnosed on the basis of the chief complaints of swollen cervical lymph nodes and high fever. KD was diagnosed on day 4, and high-dose immune globulin, steroid pulse therapy, ulinastatin, and aspirin were administered. On day 9, at the peak of the KD-induced inflammation, she suddenly developed a severe cough. Chest radiography and computed tomography (CT) on day 9 demonstrated pleural fluid and pleural thickening in her left lung, suggesting the development of pleurisy as a complication of KD. At the same period, echocardiography showed transient dilation of the main trunk of her left coronary artery and pericardial effusion. The cough improved with KD improvement. She was discharged from our hospital on day 23, after chest CT confirmation of pleurisy improvement. This is apparently the first report of pleurisy as a complication of KD, which was observed as pleural inflammation on chest CT. Respiratory complications, commonly due to mycoplasma infection, constitute 20% of the many reported non-cardiac complications of KD, the symptoms of which often present before the onset of KD. In our case, the anti-mycoplasma antibody titer decreased from 1: 640 on day 1 to 1: 160 by day 7, eliminating mycoplasma infection as a possible cause of her respiratory symptoms and pleurisy. Considering the clinical course and response to treatment, we considered her pleurisy as a clinical complication of KD-induced vasculitis. The mechanism and sequelae of the KD-associated pleurisy is unknown. Our case required a careful follow-up, and a prospective study of pleurisy-complicating KD is necessary to further elucidate its mechanism.
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Original Article
Title
Three Cases of Arthritis Preceded by Streptococcal Infection
Author
Satoshi Sato Masako Chiyotanda Norito Tsutsumi Tae Hijikata Shinichiro Morichi Yu Ishida Shingo Oana Gaku Yamanaka Hisashi Kawashima and Akinori Hoshika
Department of Pediatrics, Tokyo Medical University
Abstract
The incidence of rheumatic fever in developed countries including Japan is decreasing. However, post-streptococcal reactive arthritis (PSRA) is currently prevalent. It is not clear whether PSRA is a separate disease entity or a condition within the spectrum of rheumatic fever. In this report, we present 3 cases of rheumatic fever and PSRA within a 3-month period in western Tokyo. We investigated similarities and differences in symptoms and signs, and disease courses between rheumatic fever and PSRA. The results suggest that pediatricians should be fully aware of rheumatic fever including PSRA, and that identification and treatment of group A streptococcal pharyngotonsillitis should be a continuing public health concern.
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Original Article
Title
A Case of Neonatal Pasteurella Multocida Meningitis Resulting from a Cat Bite
Author
Satoshi Tsuge Hideki Tsuiki Motoko Nakano Yutaka Negishi Aiko Mukai Hiroaki Taniguchi Kiyo Hamaguchi Takeshi Arakawa and Masao Nakano
Department of Pediatrics, Gifu Prefectural Tajimi Hospital
Abstract
We report a case of naonatal Pasteurella multocida meningitis resulting from a cat bite in a 21-day-old male neonatal patient. The patient presented with fever and poor health condition upon examination in our hospital. Lumbar puncture indicated meningitis. We initiated treatment with cefotaxime (CTX; 200 mg/kg/d) and meropenem (120 mg/kg/d). After initiating the treatment, we identified P. multocida as the cause of his meningitis. We reviewed the case history and learned from his father that the patient had been bitten by a cat. The patient gradually improved clinically; however, the levels of some serum inflammatory markers (e.g., WBC, C-reactive protein) again unexpectedly increased 9 days after admission. Computed tomography scan and magnetic resonance imaging (MRI) confirmed our suspicion of subdural abscess. We subsequently increased the CTX dosage to 300 mg/kg/d and placed the patient under close observation. We also considered surgical intervention. The adjusted treatment effectively reduced the abscess gradually, as shown on MRI, and decreased the high levels of serum inflammatory markers. We were able to halt CTX treatment 44 days after admission, and the patient was discharged 61 days after admission.
P. multocida is naturally found in the mouths and on the claws of cats, dogs, rabbits, cattle, birds, and pigs. Meningitis due to P. multocida is rare. In Japan, only 1 pediatric case has been reported. However, the increasing number of Japanese who own home pets in recent years has resulted in closer contact of pets with humans. As a result, P. multocida in samples from patients with infectious diseases has become more commonly detected in municipal hospitals. P. multocida infection should therefore be considered in the differential diagnosis when managing neonatal patients suspected of having an infectious disease. Pediatricians must also educate parents in ways of creating a safe living environment for their children to avoid such preventable infections.
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Original Article
Title
Syncope Resulting from Ostial Stenosis of the Left Coronary Artery during Intensive Exercise
Author
Shin Takahashi1) Yoko Sato1) Satoshi Nakano2) Wataru Soda1) Kotaro Oyama1) and Shoichi Chida2)
1)Departments of Pediatric Cardiology, Iwate Medical University
2)Departments of Pediatrics, Iwate Medical University
Abstract
We describe a 13-year-old girl (Case 1) and a 6-year-old boy (Case 2) who developed syncope resulting from ostial stenosis of the left coronary artery during intensive exercise. Continuous electrocardiography revealed ST segment depression on exercise in Case 1 and at rest in Case 2. Echocardiography revealed flow acceleration signals in Case 1 and absence of flow in Case 2 at the left coronary ostium. Ischemic changes in the myocardium and stenotic findings of the left coronary artery were confirmed by myocardial scintigraphy and computed tomography, respectively. Both patients underwent coronary artery bypass surgery using the internal thoracic artery, which relieved their syncope. Neither of the children had a history of systemic inflammatory diseases or other congenital heart diseases. Therefore, their syncope attacks were diagnosed as 'cardiac', resulting from congenital ostial stenosis of the left coronary artery. An initial investigation of the coronary artery ostium flow by echocardiography is required for patients who develop syncope during exercise, as cardiac syncope carries a high risk of sudden death.
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Original Article
Title
Successful Treatment of Multifocal Lymphangioendotheliomatosis with Thrombocytopenia Using Vincristine in an Infant with Bleeding Tendency Since Birth
Author
Hideto Teranishi1) Kozo Kawasaki1) Eisuke Kondo1) Aki Saito1) Hiroki Fujimoto1) Mika Inoue1) Tokio Wakabayashi1) Hiroto Akaike1) Tetsuya Yamaguchi1) Satoko Ogita1) Kihei Terada1) Takashi Nakano1) Kazunobu Ouchi1) and Yutaka Kawamoto2)
1)Department of Pediatrics, Kawasaki Medical School
2)Division of Neonatology, Kawasaki Medical School
Abstract
We report the apparently first infant case of multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT) in Japan. The patient was a 22-month-old female infant who had frequent gastrointestinal bleeding and purple discoloration in her abdomen, back and lower limbs since birth. She underwent skin biopsy of a neck lesion and histopathology revealed proliferation of dilated small vessels, confirming the diagnosis of MLT. Although oral prednisolone was not effective, weekly intravenous vincristine given at 0.05 mg/kg improved the degree of thrombocytopenia and gastrointestinal bleeding. During the treatment, she developed pneumonia necessitating respiratory management. As the patient showed positive cytomegalovirus (CMV) antigenemia test results and was also pasitive for CMV-DNA by PCR of a pharyngeal swab, the cause of the pneumonia was likely CMV. It is therefore critical to closely monitor any infectious signs and symptoms when infants are treated with steroids or antineoplastic agents for any underlying disease.
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Original Article
Title
Clinical Courses of Paroxysmal Cold Hemoglobinuria in Four Children
Author
Yuki Arakawa Katsuyoshi Koh Ryosuke Matsuno Ayumu Arakawa Kanako Sekinaka Motohiro Kato and Ryoji Hanada
Department of Hematology/Oncology, Saitama Children's Medical Center
Abstract
Paroxysmal cold hemoglobinuria (PCH) is an autoimmune hemolytic disease caused by the biphasic Donath-Landsteiner (D-L) antibody. Detection of this antibody by the D-L test is essential for the definitive diagnosis of PCH. Erythrophagocytosis by monocytes is a characteristic of PCH. Idiopathic PCH is rare in children, and little is known regarding its clinical course and optimal treatment. We retrospectively reviewed the clinical courses of PCH in 4 children. All had a history of a previous infection and characteristic sudden onset of hemoglobinuria. They required hospitalization because of anemia. Laboratory findings demonstrated normocytic anemia and low haptoglobin levels, suggesting severe hemolytic anemia. The results of blood cross-reactivity tests included a positive direct Coombs test, a negative indirect Coombs test, and positivity for the C3d antibody. A direct Coombs test using a polyspecific antiglobulin reagent and an anti-C3d antibody was positive, and an indirect Coombs test was negative. Examination of a peripheral blood smear showed erythrophagocytosis by monocytes, and the D-L test was positive for all children. Each child was treated with a corticosteroid for 10-31 days, and none showed PCH recurrence after termination of steroid therapy. PCH associated with syphilis, which was the most common form, was not severe, and any such patients only required keeping them warm and adequate bed rest. In children with idiopathic PCH, the condition poses a risk of severe hemolytic anemia, necessitating hospitalization. Although most of the cases of PCH induced by Treponema pallidum infection could be managed by keeping the patient warm and providing bed rest, idiopathic PCH in children often caused severe anemia, and this condition was effectively treated with steroids. It is important to administer erythrocyte transfusion in PCH cases with severe anemia.
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Original Article
Title
A Case of Anorexia Nervosa Causing Takotsubo Cardiomyopathy
Author
Yuji Kamimura Haruyuki Yamazaki Shuji Kuga and Takashi Muguruma
Intensive Care Unit Division, National Center for Child Health and Development
Abstract
We encountered a case of anorexia nervosa causing fatal cardiac hypofunction and arrhythmia due to takotsubo cardiomyopathy, requiring strict intensive care management. The case pertains to a 15-year-old girl who presented with respiratory failure, hypotensive shock, and hypoglycemia. With the observations of reduced wall motion mainly in the apex on echocardiography and ST change on an electrocardiogram, the condition of the patient was diagnosed as acute heart failure accompanying takotsubo cardiomyopathy. She required treatment with a circulation agonist for hypotensive shock, and on day 2 of illness, the epinephrine dosage was increased to a maximum of 0.55 μg/kg/min to maintain her blood pressure. Although the patient recovered from a state of hypotensive shock, she presented with pulseless ventricular tachycardia on day 4 of illness, necessitating cardiopulmonary resuscitation for 2 minutes and amiodarone administration. Cardiac hypofunction remained but her condition gradually improved, thereafter achieving an ejection fraction of 70% by day 20 of illness. The patient was discharged from the hospital on day 104 of illness. The results suggest that in the acute phase of anorexia nervosa, sufficient attention must be given to cardiac complications, and a condition that allows immediate treatment must be pre-established.
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