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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
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Vol.120, No.8, August 2016
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Original Article
Title
Clinical Features and Outcomes in 17 Patients with Pediatric Idiopathic Pulmonary Arterial Hypertension
Author
Jun Muneuchi Eiko Terashi Ayako Kuraoka Satoshi Takenaka Yuichiro Sugitani Yusaku Nagatomo Mamie Watanabe and Kunitaka Joo
Department of Pediatrics, Japan Community Healthcare Organization, Kyushu Hospital
Abstract
Clinical outcomes remain unclear, especially among children with idiopathic pulmonary hypertension (IPAH), since the introduction of pulmonary vasodilators such as prostacyclin (PGI2), endothelin receptor antagonist (ERA), and phosphodiesterase-5 inhibitor (PDE-5i). We retrospectively reviewed 17 consecutive patients with IPAH referred to our institution. Median age at diagnosis was 8.4 (4.4-11.2) years. Of these patients, 29% were diagnosed in a school-based cardiovascular screening program. The median value of the mean pulmonary arterial pressure was 65 (45-79) mmHg. The initially administered pulmonary vasodilators included oral PGI2 in 4, ERA in 4 and PDE-5i in 3 patients. Ten patients (58%) were treated with two or more pulmonary vasodilators. During median follow-up duration of 55 (24-108) months, 8 patients died. Over-all survival rates at 1 year, 5 years and 10 years were 88%, 68%, and 59%, respectively. Cox-hazard analysis revealed the WHO functional class to be III/IV (HR=293: 95%CI 2.8-30,826) and the administration of two or more pulmonary vasodilators (HR=0.03: 95%CI 0.001-0.68) were significantly related to the survival rate. Compared characteristics and outcomes between 9 patients in the early period (before the introduction of ERA) and 8 patients in the late period, the patients in the late era tending to have a lower WHO functional class at diagnosis and be detected in the school-based cardiovascular program. There was no significant difference in the survival rate between the early and late periods. We consider that IPAH patients should be treated with combinations of pulmonary vasodilators as early as possible before symptoms are exacerbated.
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Original Article
Title
A Cohort Study of L-carnitine Supplementation for Patients with Severe Physical and Mental Disabilities Who Also Have Hypocarnitinemia
Author
Tomomi Ogata1) Kazuhiro Muramatsu1) Hiroko Tanaka2) Kouji Kaneko3) Takanori Kowase4) Noriko Sawaura1) Reiko Hatori1) Kanako Kurata1) Yoshiaki Ohtsu1) Akihiro Morikawa5) and Hirokazu Arakawa1)
1)Department of Pediatrics, Gunma University Graduate School of Medicine
2)Kibounoie Ryouiku Hospital
3)Hanna-Sawarabi Ryouikuen
4)Gunma Seisi Ryougoen
5)Kitakanto Allergy Institute
Abstract
[Introduction] Long-term tube feeding is often necessary for patients with severe physical and mental disabilities because of various factors such as the severity of underlying diseases, impaired swallowing function, and respiratory failure. They have a high risk of hypocarnitinemia because of low carnitine intake, changes in their physique, and long-term use of antiepileptic drugs and antibacterial agents. [Methods] We evaluated the effects of L-carnitine administration (30 mg/kg) in 19 patients with severe physical and mental disabilities who had hypocarnitinemia (<35 μmol/l) and were fed carnitine supplemental formulas and valproic acid. For 6 months, their physical state, blood samples, and serum carnitine levels were monitored. Ethical approval for this study was obtained. [Results] After 1 month of L-carnitine supplementation, the serum levels of carnitine, free-carnitine, and acylcarnitine had increased. The ammonia level had significantly decreased in patients with hyperammonemia. No adverse events or other clinical symptoms were observed. [Conclusion] Periodical measurements of carnitine levels and supervision for hypocarnitinemia are important. L-carnitine is a relatively safe medication that is effective in treating hyperammonemia.
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Original Article
Title
FDG-PET Evaluation of Infection before Hematopoietic Stem Cell Transplantation in Patients with Primary Immunodeficiency Diseases
Author
Midori Yukizawa Masakatsu Yanagimachi Koji Sasaki Reo Tanoshima Hiromi Kato Tomoko Yokosuka Ryosuke Kajiwara Fumiko Tanaka Hiroaki Goto and Shumpei Yokota
Department of Pediatrics, Yokohama City University Hospital
Abstract
Hematopoietic stem cell transplantation (HSCT) as a curative treatment for primary immunodeficiency diseases (PID) is becoming established, but the persistence of intractable infections at the time of HSCT is a risk factor for graft failure and infectious complications.
In recent years, the utility of positron emission tomography (PET) examination in the diagnosis of inflammatory diseases has been reported.
We performed FDG-PET and PET/CT for evaluation of infectious diseases in patients who were scheduled to undergo HSCT at our institution.
We retrospectively examined the usefulness of FDG-PET and PET/CT in the evaluation of infection prior to HSCT in 11 patients (age, range, 6 months old-31 yr) with PID, including 9 patients with chronic granulomatous disease and two patients with hyper IgE syndrome during 2002-2014.
By using PET, we could find infectious lesions in 3 patients that could not have been found by other imaging tests. According to the evaluation of the activity of existing infection, we were able to change the antibiotics or could postpone HSCT until the infectious disease was controlled. We were also able to measure the current therapeutic effect of antibiotics or antifungal therapy, and the activity of the infectious lesion by PET. PET examination resulted in valuable information for the treatment strategy in 10 out of the 11 HSCT patients. Thereafter, we were able to perform HSCT more safely for PID patients with infectious diseases.
FDG-PET and PET/CT are useful in the evaluation of infectious diseases prior to HSCT.
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Case Report
Title
Two Cases with Significant Pericardial Effusion during Convalescence from Kawasaki Disease
Author
Koji Tagawa1) Junpei Soumura1) Hironori Sagawa1) Tomoaki Kunitsu2) Aya Sato2) Mayumi Nakai2) Fukiko Ryujin2) Shinsuke Hoshino1) Ouki Furukawa1) Eisuke Itou2) Nobuhiko Okamoto3) and Yoshihiro Takeuchi1)
1)Department of Pediatrics, Shiga University of Medical Science
2)Department of Pediatrics, Saiseikai Shigaken Hospital
3)Department of Pediatrics, Omihachiman Community Medical Center
Abstract
We encountered two rare cases of significant pericardial effusion during convalescence from Kawasaki disease. The first case involved an 8-month-old girl. We diagnosed Kawasaki disease and gave immunoglobulin (IVIG) and aspirin (ASA). Her pyrexia soon improved and she was discharged after 15 days without complications. Her CRP level was 1.43 mg/dL upon discharge. On the 30th day following disease diagnosis, she was found to have slight tachycardia and pericardial fluid retention. She was prescribed prednisolone (PSL) and ASA. Her pericardial effusion quickly disappeared. The second case involved a 13-month-old boy. He was similarly given a diagnosis of Kawasaki disease and we administered IVIG and flurbiprofen treatment. His pyrexia improved and he was discharged after 11 days without complications. His CRP level was 0.75 mg/dL upon discharge. On the 27th day following disease diagnosis, he had slight tachycardia, loss of appetite and pericardial fluid retention. He was given PSL and ASA, and soon recovered. Mild symptoms, such as tachycardia and loss of appetite, frequently accompany convalescent cases of Kawasaki disease with pericardial effusion. However, there are some cases with no noticeable symptoms of pericardial effusion during regular medical examinations. Pericardial effusion may develop in infant Kawasaki disease patients, whose CRP levels remain positive after the acute period. In such cases, symptoms of cardiac failure should be carefully examined, and echocardiography and electrocardiography should be performed to exclude the possibility of pericardial effusion within at least 2 months after Kawasaki disease onset.
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Case Report
Title
Infections of the Central Nervous System due to Lumbosacral Congenital Dermal Sinuses Tracts: Report of Three Cases
Author
Masakazu Otsuka Yoshiaki Watanabe Kiyoko Watanabe Tatsuharu Sato Sumito Dateki and Hiroyuki Moriuchi
Department of Pediatrics, Nagasaki University Hospital
Abstract
Lumbosacral congenital dermal sinuses tracts (DSTs) range from non-pathological dimples, found occasionally among healthy infants, to tracts with significant diseases including central nervous system (CNS) infections, such as bacterial meningitis, spinal cord abscess and brain abscess. Therefore, it is necessary to determine if those lumbosacral DSTs penetrate the dura mater and are associated with significant intradural disease.
We here report 3 cases of lumbosacral DSTs. The diagnosis was made when they developed CNS infections such as bacterial meningitis and spinal cord abscess, which led to sequelae with bladder and rectal disturbance in 2 of them, despite antibiotic therapy and surgical resection of DSTs. Delayed diagnosis followed by delayed surgical interventions was considered to be responsible for the sequelae, since prompt surgical treatment appears to be necessary to prevent neurological deficits.
Previous studies indicated the importance of careful examination of the DSTs, including the dimple-to-anus distance, the diameter and the number of the dimple(s) and the presence of cutaneous stigmata, and imaging studies for suspected cases. All three cases in this study fulfilled the aforementioned conditions. It is important for everyone who is involved in regular health check-ups for infants to develop awareness of this clinical condition. Additionally, in the era of Hib and pneumococcal conjugate vaccines, we should always consider the underlying pathological conditions including DSTs, whenever we encounter cases of the CNS infections such as bacterial meningitis, spinal cord abscess and brain abscess.
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