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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY

Vol.116, No.3, March 2012


1. An Analysis of Free Writing Sections in a Cross-sectional Survey of Childhood Cancer Survivors
2. Two Cases of Asplenia Syndrome Resulting in with Fatal Pneumococcal Sepsis
3. A Case of Primary Bacterial Peritonitis in Mitochondrial Encephalomyopathy with Lactic Acidosis and Stroke-like Episodes (MELAS) with Irreversible Acute Renal Failure
4. Three Childhood Autoimmune Limbic Encephalopathy Cases
5. A Case of Pneumatosis Cystoides Intestinalis Presenting with Intussusception
6. A Case of Nasal Hemangioma Discovered during Clinical Work-up for Severe Anemia


Original Article
Title
An Analysis of Free Writing Sections in a Cross-sectional Survey of Childhood Cancer Survivors
Author
Yasushi Ishida1)2) Misato Honda2) Naoko Sakamoto3) Shuichi Ozono4) Kiyoko Kamibeppu5) Tsuyako Iwai6) Naoko Kakee7) Jun Okamura8) Keiko Asami9) Hiroko Inada4) Naoko Maeda10) and Keizo Horibe10)
1)Department of Pediatrics, St. Luke's International Hospital
2)Department of Pediatrics, Ehime University Graduate School of Medicine
3)Department of Epidemiology, National Research Institute for Child Health and Development
4)Department of Pediatrics, Kurume University School of Medicine
5)Department of Family Nursing, The University of Tokyo
6)Department of Oncology, Kagawa Children's Hospital
7)Department of Health Policy, National Research Institute for Child Health and Development
8)Institute for Clinical Research, National Kyusyu Cancer Center
9)Department of Pediatrics, Niigata Cancer Center Hospital
10)Center for Clinical Research and Department of Pediatrics, Nagoya Medical Center
Abstract
We performed a cross-sectional survey of childhood cancer survivors (CCSs) using self-rated questionnaires and qualitatively analyzed the free-writing sections to investigate sources of anxiety, including solutions, and regarding future medical care systems for long-term follow-up (LTFU). Compared to CCSs who did not offer opinions in the free writing sections, those offering opinions tended to be older and university graduates, and were more likely to receive radiotherapy and suffer from problems with daily activity or social adaptation.
CCSs worried more often than siblings, with 28% of CCSs reporting "no worry," vs. 60% in siblings, while 54% and 24% of CCSs reported vague worry about the future and late effects, respectively (p<0.001). CCSs had more concerns regarding discussing their disease with colleagues (p<0.01), including their health/physical strength (p<0.05). Female CCSs reported more worry than males about discussing disease with colleagues, as well as effects on pregnancy, delivery, or offspring (p<0.05). CCSs between 20-24 years old reported their anxieties in the free writing sections most often. CCSs listed self-consciousness, availability of consultation sites, being understood, as well as support from those around them and communication with each other as mitigating factors. Both CCSs and siblings reported a desire for a nation-wide system of comprehensive LTFU care, including psychosocial support and appropriate patient education, and supporting present JPLSG LTFU activities.




Original Article
Title
Two Cases of Asplenia Syndrome Resulting in with Fatal Pneumococcal Sepsis
Author
Nobuhiko Nagano Mamoru Ayusawa Yuriko Abe Maki Hasegawa Yousuke Taguchi Takahiro Nakamura Junji Fukuhara Rie Ichikawa Masaharu Matsumura Michio Miyashita Hiroshi Kanamaru Naokata Sumitomo Tomoo Okada and Hideo Mugishima
Department of Pediatrics and Child Health, Nihon University School of Medicine
Abstract
Asplenia is at high risk for sepsis caused by polysaccharide encapsulated bacteria. We encountered two cases of asplenia syndrome that resulted in fatal pneumococcal sepsis.
The first case was a 1-year-old boy who had received a Glenn shunt operation, and was not given preventive antibiotics. He visited our hospital because of fever for 8 hours, however, he was sent home with a prescription for antibiotics, since laboratory and physical examinations were not grave. He returned in another 6 hours with cardiopulmonary arrest and could not recover.
The second case was a 3-years-old girl who had received a Fontan operation. She had been inoculated pneumococcal polysaccharide vaccine at age 24 months and was given oral antibiotics until age 30 months. She presented with sudden fever and convulsions. When admitted to the hospital, DIC and renal failure with septic shock were diagnosed. She passed away 24 hours after admission despite treatment with ceftriaxone, PAPM/BP and corticosteroid. Streptococcus pneumonia was obtained from blood cultures of both patients. The serotype from the second case was '6B' which is covered by the conventional vaccine.
These experiences suggested that preventive use of oral antibiotics for asplenia syndrome is necessary even after vaccination. When infection occurrs, sufficient antibiotic treatment should be urgently initiated.




Original Article
Title
A Case of Primary Bacterial Peritonitis in Mitochondrial Encephalomyopathy with Lactic Acidosis and Stroke-like Episodes (MELAS) with Irreversible Acute Renal Failure
Author
Hiroaki Tamura Atsuko Noguchi Ikuko Takahashi Satoko Tsuchida and Tsutomu Takahashi
Department of Pediatrics, Akita University School of Medicine
Abstract
Primary bacterial peritonitis is defined as the infection of ascitic fluid without a definitive intra-abdominal source that can be surgically treated and it includes spontaneous bacterial peritonitis (SBP), a common complication that develops in patients with cirrhosis and bacterial peritonitis with idiopathic nephrotic syndrome of childhood. In cases of bacterial peritonitis complicated with cirrhosis or nephrotic syndrome, acute renal failure due to renal tubular impairment occurs frequently. Here we describe a case of a non-cirrhotic and non-nephrotic patient with primary bacterial peritonitis and mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS). The rapid evolution from normal renal function to end-stage renal failure was probably related to primary bacterial peritonitis. The pathological analysis of the renal biopsy specimen revealed severe renal tubular injury, which was attributed to acute hemodynamic change as observed in renal tubular impairment with SBP. The findings of interstitial fibrosis and ultrastructural analysis, that is marked proliferation of abnormally shaped mitochondria in proximal renal tubular cells which imply an association with chronic mitochondrial dysfunction with MELAS. To diagnose primary bacterial peritonitis, we should perform abdominal paracentesis in patients, to show an onset of bacterial peritonitis along with persistent hypoproteinemia, low ascitic fluid protein concentration, and an immunosuppressive state. Patients diagnosed with primary bacterial peritonitis should be carefully observed for deterioration of renal function along with acute renal impairment. In our case, the proliferation of abnormally shaped mitochondria indicated chronic renal impairment due to mitochondrial dysfunction with MELAS prior to acute renal failure due to SBP. This fact indicated that we should pay attention to the decreased of renal reserve and recovery potential in mitochondriopathy.




Original Article
Title
Three Childhood Autoimmune Limbic Encephalopathy Cases
Author
Takahiro Nishihara1) Katsuki Hirai1) Mio Saitoh1) Hirofumi Kurata1) Chizuru Ikeda2) Yuko Hirao1) Shin Namikawa1) Tomoko Kashiki1) Takeshi Chirioka1) Masaho Mochinaga1) Shigetake Nishihara1) Masahiro Migita1) and Akio Furuse1)
1)Department of Pediatrics, Japanese Red Cross Kumamoto Hospital
2)Department of Pediatrics, Kumamoto Saisyunso Hospital
Abstract
We report three cases with childhood limbic encephalopathy. All were positive for autoantibodies including anti-voltage-gated potassium channel antibodies, anti-glutamate receptor ε2 antibodies, and/or anti-N-methyl-D-aspartate receptor antibodies in serum or cerebrospinal fluid (CSF). None were accompanied by teratoma or any other tumor. They had prodromal symptoms, prolonged impairment of consciousness, and frequent involuntary movements consisting of dyskinesia, athetosis and oro-lingual-facial dyskinesia. Lymphocytic pleocytosis was observed in CSF. There were no abnormal findings on brain magnetic resonance imaging. The SPECT findings of two patients showed high uptake regions. Both were treated with methylprednisolone pulse and/or intravenous immunoglobulin therapy and plasma exchange. Their clinical abnormalities resolved within 3 months in all 3 cases. There was no recurrence during follow-up. We conclude that if limbic encephalitis is suspected, autoantibodies in serum and CSF should be investigated and immunotherapy consisting of steroid pulse, immunoglobulin therapy and plasma exchange should be initiated early.




Original Article
Title
A Case of Pneumatosis Cystoides Intestinalis Presenting with Intussusception
Author
Tomokazu Kimizu Kumiyo Matsuo Nobuhiro Kawakami Makiko Kikkawa Yasuyuki Tokunaga and Taro Matsuoka
Department of Pediatrics, Toyonaka Municipal Hospital
Abstract
Pneumatosis cystoides intestinalis (PCI) is a relatively rare disease featuring multilocular cysts in the submucosa and subserosa of the gastrointestinal tract. We report a case of PCI associated with intussusception. A 14-year-old boy complaining of abdominal pain was brought to our hospital by ambulance. Abdominal contrast-enhanced CT scan showed intussusception and multilocular cysts in the intestinal wall at the site of intussusception. Intussusception was treated with hydrostatic reduction. Colonoscopy performed after reduction showed multiple smooth-surfaced protruding lesions like submucosal tumors at the ileocecal junction. From these findings, we diagnosed PCI associated with intussusception. Because there was a possibility of the recurrence of intussusception if PCI were left untreated, we attempted to treat PCI with high-flow oxygen therapy (HFOT). An apparent improvement of PCI was recognized on an abdominal X-ray after 2 weeks of therapy. There are several reports of PCI cases associated with intussusceptions, and most of them were treated with open surgery. In this case, because intussusception was treated nonsurgically, it was preferred also to treat PCI noninvasively. HFOT is a simple treatment and its efficacy has been demonstrated for the treatment of PCI. Therefore, HFOT is considered to be a useful initial treatment for PCI in a general hospital.




Original Article
Title
A Case of Nasal Hemangioma Discovered during Clinical Work-up for Severe Anemia
Author
Chikako Inoue Syuji Hashimoto Tomohiro Katsuta Tetsuo Miyake Yachiyo Kurihara Keiji Doi and Masashi Taki
Department of Pediatrics, St. Marianna University School of Medicine Yokohama City Seibu Hospital
Abstract
We report the case of 14-year-old girl who was diagnosed as having nasal hemangioma during a clinical work-up for severe anemia.
She was diagnosed as having iron deficiency anemia by a private practitioner, and administered iron therapy without considering possible blood loss as the cause of the anemia. However, the anemia did not improve with the therapy, and the patient was referred to our hospital by the practitioner. Although she did not admit to a history of epistaxis during our medical history-taking, we detected the bleeding nasal hemangioma at the bottom of the right inferior nasal concha during examination. Her anemia improved promptly after enucleation of the hemangioma, indicating that it was most likely caused by chronic blood loss from the lesion. Because anemia associated with hemorrhage from the digestive tract is also not rare, it is necessary to exclude any possible source of blood loss before starting a patient on therapy with iron preparations. Furthermore, the cause of anemia should be investigated in detail again when iron deficiency anemia dose not improved with iron therapy.




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