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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
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Vol.118, No.11, November 2014
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Original Article
Title
Significance of Postmortem Imaging and Autopsy Findings in a Neonatal Intensive Care Unit
Author
Satoru Yoshida Tokio Sugiura Nikiko Sato Hiroko Ueda Tatenobu Goto Koichi Ito Rika Nagasaki Takenori Kato and Shinji Saitoh
Department of Pediatrics and Neonatology, Graduate School of Medical Sciences, Nagoya City University
Abstract
Rates of traditional medical autopsy are low in Japan. In particular, obtaining consent for autopsy of children from parents is difficult. Although postmortem imaging of adults has been thouroughly studied, this is not the case in children. Few studies have investigated the accuracy of postmortem imaging in diagnosing the causes of neonatal deaths. We aimed to identify the accuracy of postmortem computed tomography (CT), and compared postmortem CT findings to clinical diagnosis and autopsy findings in the neonatal intensive care unit (NICU). Consent for autopsy was obtained for 10 cases (40%) of 25 who died in our NICU from 2010 to 2012. and consent for postmortem imaging was obtained for 19 (76%). Both postmortem imaging and autopsy were able to be performed in 10 cases (40%). The concordance rate between cause of death from postmortem CT and that from clinical diagnosis was 74% (14/19), while the rate between cause of death from postmortem CT and that from autopsy was 70% (7/10). Moreover, postmortem CT uncovered unrecognized diagnoses, such as pericardial emphysema, that remained undetected from the clinical course or autopsy. We also compared the following characteristics between patients whose families provided consent for postmortem CT and those whose families did not: gender; gestational age; birth weight; days of life; delivery method; birth at our hospital or another hospital; and prenatal visits. No significant differences were observed for any of these characteristics. The same was seen for comparisons between patients whose families provided consent for autopsy and those whose families did not. We conclude that postmortem imaging is reliable and valid in NICU settings, and the combination of postmortem imaging and autopsy could improve the accuracy of determining causes of neonatal deaths.
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Original Article
Title
Clinical Courses of Children with Incidentally Found Intracranial Lesions
Author
Kohei Fukuoka and Takaaki Yanagisawa
Division of Pediatric Neuro-Oncology, Department of Neuro-Oncology/Neurosurgery, Saitama Medical University International Medical Center
Abstract
Introduction: The clinical courses of intracranial lesions of which related neurological symptoms were absent at diagnosis are not well documented. We report clinical characteristics of children with "incidentally" found intracranial lesions.
Patients: Clinical courses of 14 patients with intracranial lesions which were found by check-up for screening for other reasons are surveyed.
Results: There were 5 boys and 9 girls. The median age at diagnosis was 3.3 year (1-15.5 years, 5: less than 3 years). The diagnoses were ependymoma in 3 patients, optic pathway glioma in 2, brain stem glioma in 2, medulloblastoma in 1, oligodendroglioma in 1, diffuse astrocytoma in 1 and unknown in 4. The location of the tumors were cerebellum in 5 (including 4 without clinical or histological diagnosis), 4th ventricle in 3, optic pathway/hypothalamus in 2, brain stem in 2, thalamus in 1 and cerebral hemisphere in 1. The reasons for initial radiological examination were minor head injury in 6, mental retardation in 2, precocious puberty 1, facial palsy 1, headache 1 and screening in 1. All histologically or clinically diagnosed patients underwent multimodailty treatment (8) or appropriate surgery alone (3). The median follow up was 41.8 months (6 months-11 years). Three patients died and two patients with optic pathway glioma have been suffering from panhypopituitarism and decreased visual acuity.
Conclusion: In childhood brain tumor, incidental onset does not always lead to a good prognosis and functional outcome.
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Original Article
Title
NT-proBNP as a Predictor of Nonresponsiveness to Intravenous Immunoglobulin in Patients with Kawasaki Disease
Author
Sachiko Matsui1) Ryo Unita1) Yoshihiko Morikawa2) Hirotaka Takahashi1) and Masaru Miura3)
1)Department of Pediatrics, Tokyo Metropolitan Otsuka Hospital
2)Department of Clinical Research, Tokyo Metropolitan Children's Medical Center
3)Department of Cardiology and Department of Clinical Research, Tokyo Metropolitan Children's Medical Center
Abstract
There are few reports on the relation between N-terminal pro-brain natriuretic peptide (NT-proBNP) levels and the response to intravenous immunoglobulin (IVIG) therapy in acute Kawasaki disease (KD). In this study, we measured serum NT-proBNP levels in a total of 47 KD patients (age 2-109 months, median 21 months, 27 boys and 20 girls) who received IVIG and evaluated the relations of response to IVIG, blood test results, and cardiac function. Among the subjects, 40 (85.1%) were responders to IVIG and 7 (14.9%) were non-responders. The NT-proBNP levels before IVIG were significantly higher in the non-responders (1,794 [1,082-4,066] pg/mL vs. 286 [179-543] pg/mL, median [25th to 75th percentile], p=0.001). The area under the receiver-operator characteristic curve was 0.871, and when we set the cut-off value of 530 pg/mL, the sensitivity and specificity to identify non-responders were 85.7 (95% confidence interval: 42.1-99.6%) and 77.5 (61.6-89.2)%, respectively. NT-proBNP levels did not significantly correlate with left ventricular ejection fraction, fractional shortening, white-cell counts, or C-reactive protein levels. NT-proBNP is considered a useful biomarker for predicting non-responders to IVIG among KD patients and may facilitate the formulation of treatment strategies according to disease severity.
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Original Article
Title
Evaluation of Inter-observer Variation in Interpretation of Chest Radiographs Using World Health Organization Criteria for Diagnosis of Pneumonia in Children
Author
Junko Tanaka1) Naruhiko Ishiwada2) Ken Motoori3) Haruka Hishiki1) and Yoichi Kohno1)
1)Department of Pediatrics, Graduate School of Medicine, Chiba University
2)Division of Control and Treatment of Infectious Diseases, Chiba University Hospital
3)Department of Radiology, Graduate School of Medicine, Chiba University
Abstract
Background: The World Health Organization (WHO) working group developed a method for standardizing the interpretation of chest radiographs in children in 2001. Our objective was to compare the level of inter-observer agreement in diagnosing pneumonia with or without the WHO guidelines.
Methods: Thirty-seven observers (30 pediatricians, 7 radiologists) interpreted 20 pediatric chest radiographs first using Japanese guidelines, and then in English, using the WHO guidelines.
Results: The median number of films diagnosed as pneumonia, and broncho-pneumonia among pediatricians and radiologists was 12, 2, 3 and 1, and 11, 0, 5 and 6 respectively. Pediatricians over-diagnosed broncho-pneumonia when compared with radiologists (p< 0.01). The kappa values of observers versus the team consisting of a pediatrician and a radiologist were 0.22 and 0.24, respectively. The median number of films diagnosed as end-point pneumonia using the WHO guidelines among pediatricians and radiologists was 7 and 6, respectively. No differences were observed between pediatricians and radiologists. The number of films diagnosed as end-point pneumonia decreased when compared with the number of films diagnosed as pneumonia in Japanese among among both pediatricians (p< 0.01) and radiologists (p=0.02). The kappa values of observers versus the team consisting of a pediatrician and a radiologist were 0.24 and 0.36, respectively.
Conclusions: We demonstrated poor inter-observer agreement in the interpretation of child chest radiographs. There were differences between diagnosis using Japanese and WHO guidelines. We emphasized the importance of standardizing the categorization of pneumonia in order to compare the incidence. However, the WHO guidelines for interpretation of chest radiographs have not always increased the level of agreement between readers when used without training.
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Original Article
Title
Review of Infant Deaths in Nursery Schools
Author
Toshimasa Obonai1)3) Hiroki Goshima1) Ikuo Endou2) Eiichi Hoashi2) and Hiroshi Nishida3)
1)Department of Pediatrics, Tamahokubu Medical Center
2)Japan Society for Well-being of Nursery-Schoolers
3)Department of Neonatal Medicine, Maternal and Perinatal Center, Tokyo Womens Medical University
Abstract
The demand for nursery schools (NS) is rapidly increasing because of the increasing number of working mothers with infants. Consequently, the age of infants accepted by NS has decreased. Meanwhile, infants are vulnerable to apnea, resulting in sudden unexpected death (SUD). Although SUD is a rare occurrence in NS, details of risk factors or causes of SUD remain unknown. To aid in the prevention of SUD of infants, we analyzed accident reports collected by the Ministry of Health, Labour and Welfare of Japan.
The number of SUD victims in NS exhibited an upward trend after 2008. Although deaths in NS were recognized in infants aged one month after birth to 6 years old, SUD was limited to children aged < 3 years and 66% of cases involved those aged < 1 year.
The cause of SUD was unidentified in 56% of these. SUD occurred mainly in autumn and winter, and 38% of cases were suspected of infection-related illnesses. Moreover, 56% of SUD victims were discovered in the prone position and, especially, 76.5% of victims aged ≥1 year were also found in the prone position. These data suggest that unaccustomed sleep areas, infection and a prone sleeping position may be main risk factors of SUD in NS.
It was impossible to determine risk factors or causes of all SUD cases because the information in accident reports was insufficient and/or unclear. Nonetheless, it is necessary to perform a review of infant deaths to prevent SUD.
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Case Report
Title
Heparin-induced Osteoporosis in a Case of Protein-losing Enteropathy after a Double-Switch Operation
Author
Mutsuki Shioda1)4) Megumi Matsuoka2) Eri Suzuki3) Takashi Naganuma2) Takuya Tamame2) Shino Jinbo2) Seiichiro Yokoyama2) Hirotaka Ooki2) Kazuhiko Shibuya2) and Masaru Miura2)
1)Department of General Pediatrics, Tokyo Metropolitan Children's Medical Center
2)Division of Cardiology, Tokyo Metropolitan Children's Medical Center
3)Division of Endocrinology and Metabolism, Tokyo Metropolitan Children's Medical Center
4)Department of Pediatrics, Tokyo Women's Medical University
Abstract
Heparin therapy reportedly ameliorates protein-losing enteropathy (PLE), a serious complication of operations for congenital heart diseases such as the Fontan procedure. There are few reports, however, on the adverse reactions of heparin therapy. We report a 14 year-old boy with heparin-induced osteoporosis. Heparin therapy had been administered for intractable PLE, in this case, after a double switch operation.
He had been born with dextrocardia, atrioventricular discordance, double outlet right ventricle, transposition of the great arteries, and pulmonary artery atresia. A double-switch operation had been performed at four years of age and PLE developed 2 years later. He underwent five percutaneous angioplasties for stenosis of the superior vena cava and a stent was implanted for stenosis of the conduit between the right ventricle and pulmonary artery. The pressure gradient did not disappear, and the PLE failed to improve. After starting regular heparin therapy at nine years of age, adequate serum albumin levels were maintained. He complained of low back pain at 12 years of age, and was given a diagnosis of heparin-induced osteoporosis based on fractures of multiple lumbar vertebrae and remarkably low bone density (Z score -5.58 SD). Administration of zoledronic acid hydrate increased bone density (-2.44 SD) and alleviated the low back pain.
Since high-dose long-term heparin therapy can cause osteoporosis, this patient was at high risk for osteoporosis because of high-dose heparin treatment for three years. Physicians must pay attention to osteoporosis in cases receiving long-term heparin therapy for PLE.
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Case Report
Title
Three Cases of Congenital Hyperinsulinism Complicated with Gallstones or Biliary Sludge during Octreotide Treatment
Author
Yuka Nagashima Masahiro Goto Tatsuya Miyoshi Takashi Daitsu Kentaro Miyai Junko Igaki and Yukihiro Hasegawa
Division of Endocrinology and Metabolism, Tokyo Metropolitan Children's Medical Center
Abstract
Yorifuji et al. reported that long-term, continuous, subcutaneous octreotide (OCT) infusion was effective for patients with diazoxide-unresponsive congenital hyperinsulinism (CHI) (aged 0 to 11 months) without serious adverse events. However, all three CHI infants we treated with OCT developed either gallstones or biliary sludge. In these three patients, continuous subcutaneous OCT infusion (15.0 to 26.6 mcg/kg/day), in addition to diazoxide, normalized blood glucose levels. All of them developed either asymptomatic gallstones or biliary sludge within 4 months after the initiation of OCT treatment. The biliary sludge disappeared within 4 months in 2 of them, but it persisted and developed into gallstones in the remaining patient. OCT is considered to inhibit postprandial cholecystokinin secretion and consequent gall bladder contraction. It also delays small-bowel transit, leading to changes in the bile composition. These pharmacological effects are predisposing factors for the formation of gallstones or biliary sludge, a precursor of gallstones. UDCA treatment for biliary sludge and regular check-ups are indispensable to avoid gallstone attack. It is not clear why the incidence of gallstones or biliary sludge was higher in our institution. One explanation may be that biliary sludge was not taken seriously, even when detected by abdominal ultrasonography, in other institutions.
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Case Report
Title
Two Premature Neonates with Severe Clinical Manifestations of Congenital Syphilis
Author
Mai Kubota Moe Akahira-Azuma Shinichi Hosokawa Masao Kaneshige Noriko Yasuda Noriko Sato and Takeji Matsushita
National Center for Global Health and Medicine
Abstract
We report two premature neonates born with congenital syphilis (CS) with severe clinical manifestations; disseminate idiopathic coagulopathy (DIC) in Patient 1 (gestational age 31 weeks; birth weight 1,423 g) and refractory syphilis meningitis in Patient 2 (gestational age 34 weeks and 6 days; birth weight 2,299 g). Both mothers were unmarried, did not undergo syphilis screening tests, and did not receive antenatal care or appropriate treatment. Both neonates were born via emergency cesarean section, with both having birth asphyxia and transient tachypnea of the newborn. Physical examination revealed massive hepatosplenomegaly. Laboratory findings showed increased rapid plasma regain (RPR) and positive Treponema pallidum hemagglutination assay in maternal and neonatal blood. The diagnosis of CS was based on positive IgM-fluorescent treponemal antibody absorption in neonates and on the detection of massive amounts of T. pallidum in placenta. Initial treatment consisted of the antibiotics ampicillin and cefotaxime due to early bacterial sepsis/meningitis coexistent with CS. Patient 1 received fresh frozen plasma and antithrombin III to treat DIC. Patient 2 experienced a relapse of CS during the initial antibiotic treatment, prompting treatment with parenteral penicillin G. Treatment was effective in both neonates, as shown by reductions in RPR. Monitoring of growth and neurological development through age 4 showed no evidence of apparent delay or complications.
In the absence of adequate antenatal care and maternal infection tests, it is very difficult to diagnose CS by clinical manifestations at birth. Ampicillin was not sufficient to treat CS, necessitating treatment with penicillin G, one of the most potent antibiotics against T. pallidum.
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Brief Report
Title
Effects of Booster Vaccination for Children after a Vaccination Program for Prevention of Hepatitis B Vertical Transmission
Author
Tomoyuki Tsunoda1) Ayano Inui1) Tsuyoshi Sogo1) Haruki Komatsu2) and Tomoo Fujisawa1)
1)Department of Pediatric Hepatology and Gastroenterology, Saiseikai Yokohamashi Tobu Hospital
2)Department of Pediatrics, Toho University Medical Center Sakura Hospital
Abstract
In 1986, the Japanese government initiated a nationwide hepatitis B immunization program for infants born to HBV-carrier mothers. We evaluated the long-term protective efficacy of this program. Four children were undergone booster vaccine and we followed the levels of their serum anti-HBs antibodies (HBsAb). Levels of their HbsAb were significantly increased after booster vaccination. This excellent anamnestic response means that although serum HBs antibodies decreased to below protective levels with time, immune memory had been maintained. In conclusion, the long-term effectiveness of the national vaccination program for mother-to iinfant HBV infection seems to be satisfactory and HBsAb titers can be maintained with booster vaccination.
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