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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
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Vol.125, No.9, September 2021
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Original Article
Title
Effect of Intravenous Immunoglobulin Preparation on Serum Sodium Concentration in Acute Kawasaki Disease
Author
Yusuke Tokuda1) Akira Ishiguro1) Hiroshi Masuda2) Takanori Suzuki3) Tetsumin So4) and Tohru Kobayashi5)
1)Center for Postgraduate Education and Training, National Center for Child Health and Development (NCCHD)
2)Division of General Pediatrics, Department of Interdisciplinary Medicine, National Center for Child Health and Development (NCCHD)
3)Division of Cardiology, Department of Medical Subspecialties, National Center for Child Health and Development (NCCHD)
4)Department of Critical Care, National Center for Child Health and Development (NCCHD)
5)Department of Data Science, Clinical Research Center, National Center for Child Health and Development (NCCHD)
Abstract
Kawasaki disease is a vasculitic syndrome with unknown cause in childhood. Although the sodium content differs greatly between immunoglobulin (IVIG) preparations, the effect of the difference in sodium content of IVIG preparations on pathological conditions has not been fully investigated. In this retrospective observational study, IVIG preparations were classified into a low sodium-containing preparation group and a high sodium-containing preparation group. The subjects were 337 patients who were admitted with a diagnosis of Kawasaki disease. Serum sodium concentration increased significantly in both groups before and after IVIG (P<0.001), but the increase in serum sodium concentration in the high sodium-containing preparation group was larger than that in the low sodium-containing preparation group (5.4±3.1 mEq/L vs 2.5±3.2 mEq/L, P<0.001). On the other hand, in the subgroup analysis classified by serum sodium concentration before IVIG administration, the amount of increase in serum sodium concentration before and after IVIG administration was the same. No serious adverse events due to hyponatremia were observed in either group. In acute Kawasaki disease, the difference in sodium content of IVIG preparations has a limited effect on the change in serum sodium concentration and its safety.
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Original Article
Title
Establishment of Mass Cytometry-based Analytical Methods for Investigating the Immune Microenvironment of Bone Marrow in Children
Author
Asami Koyama1) Takashi Mikami2) Itaru Kato2) James Badger Wing3) Tatsuya Kamitori2) Kuniaki Tanaka2) Satoshi Saida2) Katsutsugu Umeda2) Hidefumi Hiramatsu2) Souichi Adachi1) Keiko Iwaisako4) Shimon Sakaguchi5) and Junko Takita2)
1)Human Health Sciences, Graduate School of Medicine, Kyoto University
2)Department of Pediatrics, Graduate School of Medicine, Kyoto University
3)Laboratory of Human Immunology, WPI Immunology Frontier Research Center, Osaka University
4)Department of Medical Life Systems, Faculty of Life and Medical Sciences, Doshisha University
5)Laboratory of Experimental Immunology, WPI Immunology Frontier Research Center, Osaka University
Abstract
【Introduction】Recently, many studies have shown that the tumor immune microenvironment is closely related to the progression of hematological malignancies. However, few studies have focused on the importance of the bone marrow immune microenvironment in pediatric leukemia. To enable such studies, we developed advanced methods for immunomonitoring. 【Methods】Mass cytometry (Helios™, a CyTOFⓇ System) is a technology that can detect more than 40 types of cell surface and intracellular proteins at the single-cell level. We investigated the optimum conditions for mass cytometric analysis of pediatric bone marrow, and developed a method for exploring biomarkers related to disease and immune response.【Results and Discussions】After validating that mass cytometry had sufficient resolution, we identified various markers in bone marrow immune cells, and succeeded in classifying the cell populations using unsupervised clustering algorithms. Furthermore, cisplatin-based barcoding system enabled us to stain multiple samples simultaneously. This novel technique has potential applications for the immunomonitoring of leukemia treatments in children.
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Original Article
Title
Neonatal Invasive Pneumococcal Infection: A Case Report of Serotype 16F and a Review of the Literature
Author
Naohiro Nomura1)2) Yukihiro Noda1)2) Miki Minami2) Masato Hirabayashi2) and Kazunari Kaneko2)
1)Department of Pediatrics, Saiseikai Noe Hospital in Osaka
2)Department of Pediatrics, Kansai Medical University
Abstract
Streptococcus pneumoniae (S. pneumoniae) serotype 16F, a non-vaccine serotype, is a rare cause of invasive pneumococcal disease (IPD). Here, we report a case of neonatal IPD due to vertical infection with serotype 16F from chorioamnionitis.
The boy was born by vaginal delivery at gestational age 36 weeks, 2 days. His Apgar scores were 5 and 7 at 1 and 5 minutes, respectively, and he was transferred to the neonatal intensive care unit because of persistent respiratory distress. He was diagnosed with early-onset bacteremia on the basis of clinical findings. Penicillin-susceptible S. pneumoniae (PSSP) was isolated from swabs of the patient's pharynx, nasal cavity, and skin as well as from his urine and stool. His mother developed a fever post-delivery, and PSSP was also isolated from a culture of her blood. Because of pathological findings in the placenta, she was diagnosed with chorioamnionitis due to S. pneumoniae. The pneumococci detected in the mother and baby were both serotype 16F. As serotype 16F is not covered by pneumococcal conjugate vaccines available in Japan, serotype replacement is associated with his IPD.
Among reported cases of neonatal IPD in the Japanese literature, early-onset type which developed within 72 hours after birth consists almost entirely of all cases (97%) and the mortality rate was 44.1%. Thus, S.pneumoniae should be suspected as the causative agent in cases of neonatal early-onset bacteremia because of possible serotype replacement in Japan. In addition, Penicillin-resistant S.pneumoniae (PRSP) accounted for about 35% cases, so we should consider commencing an empiric therapy.
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Case Report
Title
Creatine Kinase Levels in the Early Postnatal Period in Patients with Duchenne Muscular Dystrophy
Author
Yoshiyuki Katayama1) Tomoko Lee1) Sachi Tokunaga1) Naoko Taniguchi1) Hideki Shimomura1) Akio Shibata1) Junji Mine1)2) Yoshiki Ishii3) Masayo Fujisaka4) Katsuhiko Yoshii4) Masumi Okuda1)2) and Yasuhiro Takeshima1)
1)Department of Pediatrics, Hyogo College of Medicine
2)Department of Pediatrics, Sasayama Medical Center, Hyogo College of Medicine
3)Department of Pediatrics, The Veritas Hospital
4)Department of Pediatrics, Chibune General Hospital
Abstract
Serum creatine kinase (CK) levels are typically elevated in patients with Duchenne muscular dystrophy (DMD); reportedly, infants with DMD usually show serum CK levels > 10,000 IU/L. However, limited data are available regarding these levels in the early postnatal period. In this study, we investigated serum CK levels during the early postnatal period in three male infants (born at gestational age 38—40 weeks, birth weight 2,330—3,566 g), who were diagnosed with DMD based on hyperCKemia observed in the neonatal period. Serum CK levels (IU/L) showed the following changes: case 1: 19,171 (day 0), 3,118 (5), 7,340 (37), 6,655 (42); case 2: 53,077 (day 0), 7,999 (4), 11,771 (8), 4,909 (33), 14,847 (66); case 3: 9,649 (day 2), 2,230 (7), 1,520 (19), 2,315 (33), 7,897 (75). Serum CK levels showed significant changes in the early postnatal period and were relatively low for DMD depending on the timing. Early diagnosis based on neonatal screening using serum CK levels has been investigated as a new therapeutic strategy for DMD; however, this approach has not yet been conclusively established. Accumulation of further data regarding serum CK values in the early postnatal period is warranted to gain a deeper understanding of this project.
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Case Report
Title
Severe Anemia Associated with Cytomegalovirus Infection in a Child with Hereditary Spherocytosis
Author
Risa Makino Kazuyuki Komatsu Koji Kawaguchi Takayuki Takachi Taemi Ogura Yasuo Horikoshi and Kenichiro Watanabe
Department of Hematology and Oncology, Shizuoka Children's Hospital
Abstract
Patients with hereditary spherocytosis (HS) develop severe anemia in response to the inhibition of erythropoiesis. The most common cause of aplastic crisis is human parvovirus B19 (PVB19) infection, but there have been no reports of any association with cytomegalovirus (CMV) infection. We herein report a child with HS who presented with severe anemia after initial infection with CMV. A one-year-old girl, whose father and sister both had HS, was diagnosed with HS herself soon after birth. Thereafter, she had no apparent anemic episodes, and her hemoglobin level remained at around Hb 10 g/dL. After having a persistent fever, pale lips, and fatigue for one week, she was admitted to our hospital because of severe anemia with an Hb level of 5.6 g/dL and received red blood cell transfusion due to a profound decrease in activity. PVB19 IgM was negative. We suspected CMV infection because of the increase in atypical lymphocytes and liver enzyme levels. Antibody tests revealed positive findings for CMV IgM and IgG on admission and negative findings for CMV IgM with an increased CMV IgG antibody titer after three months, suggesting that she may indeed have had a CMV infection initially. Patients with HS may develop severe anemic episodes in association with CMV infection.
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Case Report
Title
Thyrotoxic Periodic Paralysis in a 13-year-old Girl with Graves' Disease: A Case Report
Author
Miki Kamimura1)2) Junko Kanno1) Ikumi Umeki1) Mitsuji Moriya3) Dai Suzuki1) Ikuma Fujiwara1)3) and Shigeo Kure1)
1)Department of Pediatrics, Tohoku University Hospital
2)Department of Pediatrics, Sendai Medical Center
3)Department of Pediatrics, Sendai City Hospital
Abstract
Typically prevalent in East Asian males, thyrotoxic periodic paralysis (TPP) is characterized by a sudden onset of muscle weakness owing to hypokalemia. The main cause of hypokalemia is believed to be the stimulation of the sodium-potassium adenosine triphosphatase (Na-K-ATPase) activity in skeletal muscle by thyroid hormone. This increases the transport of potassium from the extracellular to the intracellular space, causing hypokalemia. In addition, insulin, catecholamine, and androgen levels also intensify the Na-K-ATPase activity.
Here, we describe a rare case of a 13-year-old Japanese girl diagnosed with TPP. She presented with paralysis of the extremities after consuming a carbohydrate-rich meal the previous night. On admission, laboratory data revealed hypokalemia (2.1 mEq/L), and goiter and exophthalmos were noted. Thyroid function test showed thyrotoxicosis (free thyronine 11.2 pg/mL, free thyroxine 2.8 ng/dL, and thyroid stimulating hormone <0.01 μIU/mL), and thyroid stimulating hormone receptor antibody level was 292.4 IU/L. Thyroid ultrasonography revealed diffuse enlargement and hypervascularity. Therefore, she was diagnosed with TPP associated with thyrotoxicosis due to Graves' disease. She was treated with intravenous potassium supplementation and thiamazole and her paralysis resolved gradually until the following day. Oral glucose tolerance test revealed mild insulin resistance and oversecretion of insulin, and serum potassium decreased with insulin secretion. Hyperinsulinemia owing to carbohydrate-rich meal could be the precipitating factor of the onset of TPP in this patient. Although TPP is rare in children, TPP should be considered when patients have paralysis of the extremities, and serum potassium level and thyroid function should be checked.
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Case Report
Title
Appearance of Precocious Puberty Symptoms in a Boy before Discovery of a Pineal Germ Cell Tumor
Author
Takuya Yamamoto1) Mayumi Muramatsu1) Hiroyuki Ono1) Konosuke Ohtaka2) Kimiyoshi Sakaguchi1) Tsutomu Ogata1) and Yasuko Fujisawa1)
1)Hamamatsu University School of Medicine, Pediatrics
2)Iwata City Hospital, Pediatrics
Abstract
Precocious puberty (PP) in boys is often caused by diseases such as brain tumors. Here, we describe a case of PP due to a human chorionic gonadotropin (HCG) -secreting pineal germ cell tumor in a boy. Signs of PP preceded the detection of the intracranial HCG-secreting germ cell tumor. A 7-year-old boy was referred to Iwata City Hospital because of rapid height and pubic hair growth. His pubertal staging was classified as P3 G3 according to Tanner, and his testicular volume was 3 mL bilaterally. Serum testosterone and HCG-β levels were below the sensitivity of the assays. In the gonadotropin-releasing hormone test, the peak luteinizing hormone (LH) level was within the normal range, but the peak follicle-stimulating hormone (FSH) level was below normal range. Any abnormal findings were detected by head MRI. After 6 months with careful follow-up, repeated head MRI revealed a pineal region mass. Circulating FSH and LH levels were suppressed below the level of assay sensitivity. The HCG-β level was significantly elevated (1.0 ng/mL) and the testosterone level (13.9 ng/mL) was higher than that of age-matched controls. Intracranial tumor extraction was performed at Hamamatsu University School of Medicine, University Hospital. The removed tumor was pathologically diagnosed as a mixed germ cell tumor (immature teratoma+germinoma). This case shows that after diagnosis of male idiopathic precocious puberty, careful monitoring and re-examination are necessary.
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Case Report
Title
Recurrent Group B Streptococcus Infection with Knee Arthritis in an Infant
Author
Yusuke Akatsuka1) Tamaki Ikuse1) Saki Sekiguchi1) Naoki Murota1) Tatsuki Nishiyama1) Ibuki Tsukada1) Itsuhiro Oka2) Masamichi Sato1) Noboru Yoshida2) Akinori Yaguchi1) Atsushi Tsujiwaki2) Mitsutaka Komatsu1) Yuko Sakamoto3) Yoshiyuki Otomo2) Shinichi Niijima2) and Toshiaki Shimizu1)
1)Department of Pediatrics, Juntendo University Faculty of Medicine
2)Department of Pediatrics, Juntendo University Nerima Hospital
3)Department of Orthopedics, Juntendo University Nerima Hospital
Abstract
A 46-day-old female infant developed group B Streptococcus (GBS) meningitis and bacteremia at the age of 11 days and was treated with antimicrobial agents for 3 weeks until day of life 32. At the age of 45 days, the patient developed poor extension of the right knee without pyrexia and visited our emergency department the next day. Based on the clinical and laboratory findings, the patient was diagnosed with pyogenic right knee arthritis and bacteremia due to GBS type III, which was the causative organism with same serotype for the patient's initial infection. Adequate antibacterial agents were administered at the time of the initial infection, and the blood culture after treatment became negative. The patient had no clinical signs or laboratory findings suggesting immunodeficiency. Furthermore, the patient was not suspected to be a compromised host after treatment for the recurrent infection. The culture of breast milk, which was reported as a route of repeated infections, was negative.
In this patient, bacteremia was detected at the time of recurrent infection; therefore, it is believed that pyogenic arthritis resulted from bacteremia caused by horizontal infection. Recurrent infections have been reported to occur in approximately 1% of patients after treatment for invasive GBS infections; however, no case of recurrent infection presenting with pyogenic arthritis has been reported. Pyogenic arthritis in neonates can be difficult to diagnose as the clinical findings are atypical. After treatment of an invasive GBS infection, it is important that a patient be carefully monitored for a recurrent infection and careful examinations should be performed to detect the various clinical presentations of recurrent bacteremia.
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Case Report
Title
Hematidrosis with Bleeding from the Skin and Tongue
Author
Nao Kimura1) Atsushi Ono1) Kazuhide Suyama1) Masaki Ito2) and Mitsuaki Hosoya1)
1)Department of Pediatrics, Fukushima Medical University School of Medicine
2)Department of Pediatrics, Public Soma General Hospital
Abstract
Hematidrosis, an extremely rare disease characterized by blood and sweat exudation from non-traumatized skin, is thought to occur when erythrocytes leak from capillary blood vessels that feed the sweat glands and reach the skin surface via sweat ducts. We encountered a 6-year-old girl with bleeding from area-specific sweat glands, such as those on the forehead and limbs, and the tongue, where there are no sweat glands. There were no skin wounds. Blood tests showed no coagulation disorder, and skin biopsy findings were normal. She was diagnosed with hematidrosis and beta-blockers were administered to control bleeding from the skin. Herein, we summarize this and previous cases and elaborate the pathology and mechanisms of hematidrosis.
Sympathetic overactivity might have increased the vascular permeability of the capillary blood vessels feeding the sweat glands, causing erythrocytes to leak out of the vessels and sweat ducts to the skin surface. Also, similarities between the structures of the capillary blood vessels that feed the sweat glands and those that feed the Ebner glands, suggest that the bleeding from the tongue might have occurred by the same mechanism as that of the skin. A skin biopsy performed immediately after the bleeding revealed no organic abnormalities in the blood vessels or sweat glands, and beta-blockers were effective for psychological stress- and fatigue-induced hematidrosis, indicating that hematidrosis might occur when sympathetic overactivity leads to functional impairment of the blood vessels.
Large-scale studies on hematidrosis are lacking and the disease pathology can be elucidated only after accumulation of cases.
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Brief Report
Title
Telemedicine for Pediatric Patients with Coronavirus Disease 2019
Author
Toshihiro Tanaka1) and Masayuki Kaji2)
1)Department of Pediatrics, Shizuoka Kosei Hospital
2)Shizuoka City Public Health Center
Abstract
Most pediatric cases of coronavirus disease 2019 (COVID-19), a designated infectious disease under the Infectious Diseases Control Law, Japan, show mild symptoms or are asymptomatic. In Shizuoka city, pediatric patients with mild COVID-19 symptoms, in principle, stay at home or in care facilities, and physicians attend to them via telemedicine: 'The Shizuoka City System'. By the end of December 2020, the system had been applied to 21 pediatric patients together with 33 family members (patients: 16 adults, close contacts: 4 children / 13 adults) in 18 households. None of the pediatric patients became worse or was converted to hospital care during the observation period. The Shizuoka City System is considered useful in providing the best recuperation environment for pediatric patients with COVID-19 and their families, and to protect regional medical resources.
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