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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
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Vol.123, No.10, October 2019
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Original Article
Title
Usefulness of Tricuspid Annular Plane Systolic Excursion in Extremely and Very Low-Birth-Weight Infants during an 8-week Period
Author
Yoshikazu Otsubo Kumi Omagari Hiroaki Shoji Mari Yokokawa and Muneichiro Sumi
Department of Pediatrics, Sasebo City General Hospital
Abstract
Background: The right ventricular (RV) function changes after birth due to the influence of several hemodynamic changes in the immature myocardium in extremely low-birth-weight (ELBW) and very low-birth-weight (VLBW) infants.
Aim: The aim of this study was to investigate the changes in RV function in ELBW and VLBW infants using M-mode and Doppler echocardiography during an 8-week period.
Subjects and method: A total of 30 ELBW and VLBW infants hospitalized in our neonatal intensive care unit were recruited in this study. They were examined prospectively on the first day and at 1, 2, 4, 6, and 8 weeks using M-mode, pulse Doppler imaging (PDI), and tissue Doppler imaging (TDI) from the tricuspid ring in the 4-chamber view. Tricuspid annular plane systolic excursion (TAPSE), systolic wall motion velocity (s'), early diastolic wall motion velocity (e'), and the Tei index assessed by PDI were examined over time.
Results: The TAPSE, s', and e' increased, whereas the Tei index decreased significantly over time. Furthermore, corrected TAPSE by total cardiac dimension revealed no significant difference between ELBW and VLBW infants from birth to 8 weeks.
Conclusion: This study demonstrated that the RV systolic and diastolic function in ELBW and VLBW infants improved from about 2 weeks after birth. TAPSE measurement is a simple method that should be included as a part of the routine assessment of RV function in ELBW and VLBW infants.
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Original Article
Title
Clinical Validity of the Family-Rated Kinder Infant Development Scale (KIDS) in Pediatric Disabled Patients
Author
Keiji Hashimoto1)4) Hidetoshi Mezawa1)2)3) Makoto Takekoh1) Satoshi Tamai1) Keiko Kato1)4) and Takeshi Kamikubo1)
1)Division of Rehabilitation Medicine and Developmental Evaluation Center, National Center for Child Health and Development
2)Japan Environmental and Children's Study Medical Support Center, National Center for Child Health and Development
3)Division of Allergy, National Center for Child Health and Development
4)Hashimoto Clinic Kyodo
Abstract
Objective: To investigate the clinical validity of the family-rated Kinder Infant Development Scale (KIDS) in screening for developmental delays.
Methods: The participants in this study were 404 children aged 0-5, who were referred to the Developmental Evaluation Center of the National Center for Child Health and Development due to a suspected developmental disorder or delay. All children were administered the Kyoto Scale of Psychological Development 2001 (KSPD) and KIDS in the same session.
Results: The KIDS Total DQ and Postural-Motor (P-M) domain DQ demonstrated a high correlation (r = 0.756, 0.774) with the KSPD Total and P-M DQ. The regression equation was KSPD Total DQ = 22.901+0.652 × KIDS Total DQ (R = 0.756, R2 = 0.571). The sensitivity was 62.6% and 52.3%, and the specificity was 94.0% and 95.3% when the cutoff values for the KIDS Total and Receptive language DQ were set at 70 to screen for developmental delays with a KSPD DQ of less than 70 in the Total and C-A domains, respectively.
Conclusion: This study demonstrates that the KIDS total DQ can predict the total DQ in the KSPD, but the sensitivity was low when the cutoff values were set at less than 70 for the KIDS Total and Receptive language DQ to screen for developmental delays with a KSPD DQ in the Total and L-S domains of less than 70.
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Original Article
Title
Pediatrician-involved Support Program for Socially High-risk Pregnant Women Reduced Severe Infant Maltreatment
Author
Takuya Masuda1) Mari Saito1) Yutaka Kikuchi1) Hiroki Yoshinari1)2) Masashi Sagara1) Hironori Shimozawa1)2) and Masaru Hoshina1)
1)Department of Pediatrics, Haga Red Cross Hospital
2)Department of Pediatrics, Jichi Medical University
Abstract
[Background] In 2016, we initiated the "pediatrician-involved support program" in association with the local government to support the parenting of socially high-risk women and their offspring. We demonstrate the implementation and the consequent problems of preventing infant maltreatment in this program.
[Method] We administered questionnaires to the local government and evaluated the medical records of pregnant women and their offspring who participated in this program.
[Results] A total of 63 cases of pregnant women and their offspring were investigated. Women participated in this program primarily due to mental disorders, being unmarried, and/or having no support. Two infants died, and 7 were placed in temporary custody during the program. Between the medical institution and the local government, new mothers were evaluated differently due to the differences in parameters, timing, and frequency of assessment. Assessment of economic status was particularly difficult, and the issue of necessity of sharing information within the organization was raised. After the initiation of this program, the number of collaborations doubled, joint meetings increased by 10-fold, the number of temporary custodies increased from 5 to 7, and the number of cardiopulmonary arrest cases with unknown cause decreased from 3 to 1.
[Conclusion] The "pediatrician-involved support program" appears to be effective in preventing infant maltreatment in high-risk mothers, and it also helps in integrating different evaluations between the medical institution and the local government.
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Case Report
Title
Two Cases of Myotonic Dystrophy with Atrial Flutter in Young Patients
Author
Kosuke Tsuchida1) Shinobu Fukumura1) Akiyo Yamamoto1) Shinsuke Kato1) Kentaro Kawamura1) Masato Yokozawa2) and Toshio Ohara3)
1)Department of Pediatrics, School of Medicine, Sapporo Medical University
2)Hokkaido Medical Center for Child Health and Rehabilitation
3)Department of Pediatrics, Tomakomai City Hospital
Abstract
Myotonic dystrophy type 1 (DM1) is an inherited disease characterized by myotonia, progressive weakness and atrophy of the skeletal muscles, and systemic manifestations. Cardiac involvement is a common complication of DM1 and is associated with a high risk of sudden death. However, it is rare for atrial flutter to present as the first clinical manifestation of DM1 in young patients. We report cases of two patients who were diagnosed with DM1 after presenting with atrial flutter at school age. The first patient presented with atrial flutter at the age of 14 years. She underwent catheter ablation and was observed to be recurrence-free during subsequent follow-up visits. However, her first child was diagnosed with DM1, and as a result, she was diagnosed with DM1 at the age of 24 years. The second patient had mild muscle weakness and mental retardation since childhood. He presented with atrial flutter at the age of 15 years and underwent catheter ablation. Subsequently, there was no recurrence, but he presented with atrial fibrillation. At the age of 19 years, he was diagnosed with DM1 because of myotonia. Atrial flutter in young patients can be the first clinical manifestation of DM1. Therefore, it is important to consider the diagnosis of DM1 in patients with atrial flutter because the latter is associated with a risk of sudden death and affects treatment selection for DM1.
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Case Report
Title
A Case of Pulmonary Mucosa-associated Lymphoid Tissue (MALT) Lymphoma with Pediatric Primary Sjögren Syndrome
Author
Genichi Uetsuki1) Sae Nishisho1) Hitoshi Okada1) Noriko Fuke1) Takashi Iwase1) Aya Tanaka2) Ryuichi Shimono2) and Takashi Kusaka1)
1)Department of Pediatrics, Faculty of Medicine, Kagawa University
2)Department of Pediatric Surgery, Faculty of Medicine, Kagawa University
Abstract
Mucosa-associated lymphoid tissue (MALT) lymphoma is an indolent B-cell lymphoma that is a common complication of Sjögren syndrome (SS) in adults but is rare in children. Here, we report a case of pulmonary MALT lymphoma as a complication of primary SS (pSS) in a 13-year-old girl. The patient was asymptomatic except for elevated KL-6 levels. Chest computed tomography detected multiple fine nodular opacities predominantly in the lower lung fields bilaterally and sporadic patchy opacities, the locations of which matched with sites of 18F-FDG accumulation on 18F-FDG-PET. While asymptomatic, she was put on watchful waiting but underwent video-assisted thoracoscopic surgery approximately 6 months later following worsening of her cough and impaired carbon monoxide diffusion capacity confirmed by pulmonaryfunction testing. Primary pulmonary MALT lymphoma was diagnosed on the basis of histopathology and the IgH Gene Clonality Assay. Rituximab monotherapy reduced the lesion size. Cases of MALT accompanying pediatric pSS have rarely been reported; therefore, the prognosis is uncertain. Long-term watchful waiting is necessary to monitor for relapse and the onset of aggressive lymphoma.
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Case Report
Title
Acute Rheumatic Fever Diagnosed on the Basis of Sydenham's Chorea
Author
Miho Tachioka Ayako Shiihashi Takahiro Momoi Akira Kojima Akiko Koyama Noritaka Furuya Rumi Taniguchi Anzu Noda Hajime Nishimoto and Masaru Takamizawa
Department of Pediatrics, Saitama Citizen Medical Center
Abstract
Acute rheumatic fever (ARF) is a nonsuppurative inflammatory disease that may occur 2-3 weeks after an episode of pharyngitis caused by group A beta-hemolytic streptococci. The incidence of ARF has dramatically decreased in Japan and Western countries, and ARF is rarely observed even in pediatric practice. We describe the case of a 10-year-old boy who was diagnosed with ARF manifesting as Sydenham's chorea (SC) without carditis.
The patient had a group A beta-hemolytic streptococcal infection and continued to be febrile for 8 days despite oral antibiotic treatment; therefore, he was referred to our hospital. He presented with persistently low energy, purposeless frowning, jerky speech, pronation when raising his arms, difficulty maintaining tongue extrusion, restless movements, and variable grip force. On the basis of these findings, he was diagnosed with ARF manifesting as SC. He was administered only antibiotics because of the absence of carditis. Polyarthritis developed on the 6th day of hospitalization; however, it resolved with a short course of orally administered aspirin and steroids. ARF rarely presents with chorea as its primary manifestation. SC can be difficult to diagnose, particularly when symptoms are mild and triggers are unknown. Thus, adequate knowledge of this condition is important for both early diagnosis and appropriate treatment.
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Case Report
Title
A Case of Osteopathia Striata with Cranial Sclerosis Diagnosed during the Fetal Period
Author
Hideyuki Morita1)2) Koji Tatebayashi1) Yasushi Uchida2) and Hideo Kaneko1)
1)Department of Pediatrics, Nagara Medical Center
2)Department of Pediatrics, Chuno Kosei Hospital
Abstract
Osteopathia striata with cranial sclerosis (OS-CS) is an X-linked dominant inherited bone dysplasia characterized by longitudinal striations of the long bones and cranial sclerosis. Common clinical findings of OS-CS include macrocephaly, frontal bossing, ocular hypertelorism, a broad nasal bridge, hearing loss, and abnormalities of the palate. Rarely, fibular hemimelia and cardiac or kidney malformations have been reported. Pathogenic mutations of AMER1 have recently been identified. The hemizygous male phenotype in OSCS is more severe than that seen in female heterozygotes. We present the case of a 3-year-old boy initially evaluated for macrocephaly and fibular hemimelia. The diagnosis was confirmed by 3DCT and ultrasound examination during the fetal period. We analyzed AMER1 mutations in this family. Mutational analysis of the single coding exon of AMER1 resulted in the identification of one known mutation (c.867_868del). He had severe upper airway narrowing, constipation, and gaseous abdominal distension. We also provide a summary of the current knowledge on the clinical, radiological, and genetic features of OSCS.
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Case Report
Title
Recurrent Pneumococcal Bacteremia in a Patient with Juvenile Idiopathic Arthritis Receiving Infliximab
Author
Kensho Muramoto1) Shunsuke Kanno2) Etsuro Nanishi3) Masataka Ishimura3) and Shouichi Ohga3)
1)Department of Pediatrics, Yamaguchi Red Cross Hospital
2)Center for the Study of Global Infection, Kyushu University Hospital
3)Department of Pediatrics, Graduate School of Medical Science, Kyushu University
Abstract
We report a case of recurrent pneumococcal bacteremia in a 3-year-old girl with juvenile idiopathic arthritis who was receiving infliximab. She had received four immunizations with 13-valent pneumococcal conjugate vaccines (PCV13). However, Streptococcus pneumoniae serotype 24F, a non-PCV13 vaccine type, was isolated from her blood sample. Multilocus sequence typing analysis revealed that the sequence type of the pneumococcus was 5496, and the strain was susceptible to penicillin. The patient's condition improved after the administration of penicillin for 12 days and she recovered. One month after the antibiotic treatment, she again suffered from pneumococcal bacteremia. The same serotype and sequence type of S. pneumoniae was isolated from her blood samples. Because no apparent abnormality was detected in the immunological screening test results, immunosuppressive therapy comprising the tumor necrosis factor-α antagonist infliximab was suspected to be one of the causative factors for the recurrent pneumococcal bacteremia. Thus, she received 23-valent pneumococcal vaccine to prevent repetitive pneumococcal infections with the other serotypes. In recent years, the incidence of invasive pneumococcal disease caused by non-vaccine serotypes has been increasing after the widespread use of pneumococcal vaccines. Thus, development and use of practical applications that are able to confer broad protection against pneumococcal infection, such as protein-based vaccines, are expected.
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Case Report
Title
A Case of Vanish Bile Duct Syndrome Caused by Drug-Induced Hepatitis with Mycoplasma Pneumonia
Author
Reijiro Azuma1) Kenji Sugiyama1) Eri Ushida1) Yutaka Otobe1) Naoto Sakurai1) Yusuke Omori1) Masahiro Ogawa1) Hisashi Nishimori1) Hodaka Ota1) and Ayano Inui2)
1)Department of Pediatrics, Mie Prefecture General Medical Center
2)Department of Pediatric Hepatology and Gastroenterology, Saiseikai Yokohamashi Tobu Hospital
Abstract
We report a 4-year-old boy with Vanish bile duct syndrome (VBDS) after Stevens-Johnson syndrome (SJS), which might be caused by mycoplasma pneumonia. Since intravenous immunoglobulin therapy for SJS was not effective at the previous hospital, he was admitted to our hospital on the ninth day of mycoplasma pneumonia. Although the skin lesions of SJS improved with steroid pulse therapy, obstructive jaundice was progressive. Liver biopsy led to diagnosis of VBDS. Drug-induced hepatitis by L-carbocisteine was also suspected from the scoring of drug-induced hepatitis and a drug lymphocyte stimulation test (DLST). Plasma exchange therapy suppressed progression of obstructive jaundice, and he was discharged with a prescription for oral ursodeoxy cholic acid (UDCA). We considered the possibility that VBDS might be caused by drug-induced hepatitis induced by mycoplasma infection influencing drug metabolism.
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Case Report
Title
A Case of Systemic Lupus Erythematosus Differentiated from Primary Immune Thrombocytopenic Purpura
Author
Setsuko Takeda1) Nami Okamoto1) Kosuke Shabana1) Yuko Sugita1) Keisuke Shindo1) Takuji Murata1)2) Hiroshi Tamai1) and Tsuyoshi Ito3)
1)Department of Pediatrics, Osaka Medical College
2)Murata Kids' Clinic
3)Department of Pediatrics, Toyohashi Municipal Hospital
Abstract
Patients with systemic lupus erythematosus (SLE) are known to develop various symptoms. We report a case of SLE that presented with hemorrhagic symptoms, which required differentiation from immune thrombocytopenic purpura (ITP).
The case was a 13-year-old girl. Severe nasal bleeding and abnormal genital bleeding appeared, and she was referred to a hospital for treatment of pancytopenia. Intravenous immunoglobulin therapy (IVIG) was performed for presumed ITP, but her platelet count did not improve. Serum complement levels were decreased, and she tested positive for anti-nuclear antibody, anti-double stranded DNA antibody, and anti-phospholipid antibody. She was referred to our hospital for suspected SLE. She met the classification criteria of SLE and was diagnosed with secondary ITP associated with SLE (SLE-sITP). A renal biopsy revealed lupus nephritis (Class II), but no other serious organ damage nor complication of hemolytic anemia was observed. There was no evidence of thrombosis, and the diagnostic criteria for anti-phospholipid antibody syndrome were not met. Remission was achieved with prednisolone and mycophenolate mofetil, and her symptoms and laboratory findings improved rapidly.
In this case, SLE-sITP was suspected because the decreased number of leukocytes and lymphocytes, and coagulation tests were abnormal. Since complement tests and various autoantibody tests were performed before the start of treatment, accurate and rapid diagnosis and early treatment were possible.
In the case of ITP with pancytopenia, the differentiation of sITP, including SLE-sITP, is important in cases of suspected ITP.
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Brief Report
Title
Positive High-sensitivity, Quantitative Hepatitis B Surface Antigen Test Results Following Recent Hepatitis B Virus Vaccination
Author
Shoko Yoshii1) Eiki Ogawa2) Kensuke Shoji2) Akira Ishiguro1) and Isao Miyairi2)
1)Department of Postgraduate Education and Training, National Center for Child Health and Development
2)Division of Infectious Diseases, National Center for Child Health and Development
Abstract
The hepatitis B virus (HBV) vaccine contains HBsAg as its active component. Recently, the qualitative commercial HBsAg test was replace with a highly sensitive, quantitative test (HBsAg [HQ]). The rate of HBsAg positivity before and after the introduction of HBsAg (HQ) testing in infants from January 2017 to June 2018 was compared retrospectively. None of the 945 cases who were tested by the qualitative method returned positive results. Three out of 198 cases tested positive according to HBsAg (HQ) tests performed within 4 days of HBV vaccination. All tested negative upon later follow-up; however, one case became positive for HBc antibody. HBsAg (HQ) tests may return positive results soon after HBV vaccination, and consideration should be given to the timing of testing administration.
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