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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
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Vol.125, No.10, October 2021
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Original Article
Title
The Age of the Last Hypoglycemic Observation in Patients with Congenital Transient Hyperinsulinemia
Author
Toshikazu Hayashiya1)2) Takayuki Azukizawa3) Hiromu Umehara1)4) Yuko Anzai1) Yasuyo Shimizu1) Masahito Yamamoto3) and Seiro Narumiya1)
1)Department of Pediatrics, Nagahama Red Cross Hospital
2)Department of Pediatrics, Shiga University of Medical Science Hospital
3)Department of Neonatology, Nagahama Red Cross Hospital
4)Department of Pediatrics, Saiseikai Shiga Hospital
Abstract
Congenital hyperinsulinemia (CHI) causes neonatal hypoglycemia, and diazoxide is the first choice of drug treatment. However, diazoxide is associated with some serious side effects, and the indication should be carefully evaluated. It is often difficult to determine when diazoxide should be initiated, as there is no unified view on the time of resolution of hypoglycemia. We retrospectively investigated cases with CHI with the aim of studying the indications for diazoxide and the timing of its initiation. Of the 1,482 patients admitted to our NICU/GCU during the study period, 40 had hyperinsulinemia. The median age of the last hypoglycemic observation was day 7.5. The age of the last hypoglycemic observation was correlated with birth weight, gestational age, and serum insulin levels, but not with the standard deviations of birth weight. In many cases, blood glucose levels can be controlled within 2−3 weeks after birth, and in patients with CHI whose blood glucose can be controlled by glucose administration, it may be possible to avoid unnecessary diazoxide administration by waiting until about 3 weeks after birth. In addition, CHI may be more common than previously reported, which suggests the importance of measuring blood glucose in high-risk cases and collecting critical samples.
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Original Article
Title
Clinical Features of Kawasaki Disease in Children with Congenital Heart Disease
Author
Takehiro Tanabe1) Sung-Hae Kim1) Machiko Kito1)2) Mizuhiko Ishigaki1) Keisuke Sato1) Jun Yoshimoto1) Norie Mitsushita1) Masaki Nii1) and Yasuhiko Tanaka1)
1)Department of Pediatric Cardiology, Shizuoka Children's Hospital
2)Department of Pediatric Cardiology, Aichi Children's Health and Medical Center
Abstract
The survival rate of patients with congenital heart disease (CHD) has improved, and the long-term survival of children with CHD has increased since the early 2000s due to improvements in the outcomes of catheter treatment and surgical treatment for CHD. Kawasaki disease (KD) may sometimes develop in children with CHD, but the clinical features of children with CHD and KD remain unclear. In KD that occurs with CHD, it is necessary to pay more attention to coronary arterial lesions (CAL). However, there are few reports on the actual incidence of CAL in CHD-KD; therefore, standard methods to evaluate CAL have not yet been established.
We selected all patients who had KD among patients with CHD (between April 1977 and December 2019) and reviewed the clinical features; 52 patients with CHD (12 with single ventricular physiology and 40 amenable to biventricular repair) developed KD. There were 5 cases (10%) of acute CAL and 2 cases (4%) of late coronary sequelae in CHD-KD cases, which presented similarly to acute CAL and late coronary sequelae in non-CHD-KD cases. In addition, transthoracic echocardiography was often insufficient to observe coronary arteries in CHD-KD cases. In such cases, and especially in more complex cases after open-heart surgery, it is necessary to perform diagnostic catheterization in order to detect CAL.
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Original Article
Title
Reconsideration of Follow-up Timing for SFU Grade 2 Congenital Hydronephrosis in Terms of Natural Prognosis
Author
Takahiro Suzuki1)2)3) Ryoko Harada1) Riku Hamada1) Naoaki Mikami1) Keiji Akamine1) Chikako Terano1) Masataka Honda1) and Hiroshi Hataya1)2)
1)Department of Nephrology, Tokyo Metropolitan Children's Medical Center
2)Department of General Pediatrics, Tokyo Metropolitan Children's Medical Center
3)Department of Radiology, Tokyo Metropolitan Children's Medical Center
Abstract
Background According to the Society for Fetal Urology classification, grade 2 congenital hydronephrosis generally has a good prognosis, but aspects of its prognosis, such as the time required for improvement and the follow-up method, have not been adequately examined.
Objective The present study aimed to investigate the prognosis of grade 2 hydronephrosis in patients younger than 1 year of age.
Method This study was a monocentric, retrospective, observational study. The subjects were infants under 1 year of age with grade 2 hydronephrosis diagnosed between March 1, 2010 and July 31, 2012. The hydronephrosis grade during the observation period and the timing of improvement and aggravation were investigated.
Results Twenty-eight patients and 32 kidneys were included. Regarding the natural prognosis during the median observation period of 3.3 years, 25 patients improved, five were unchanged, and two showed exacerbation (6%). Initial exacerbation was observed in two kidneys at ages 5 and 8 months, respectively, and one required surgery during infancy.
Discussion The prognosis was considered favorable in many cases of grade 2 congenital hydronephrosis. Among them, 6% of cases showed exacerbation, with one case requiring surgery during infancy. These findings suggest that age 6 months and 1 year are appropriate follow-up times for patients with grade 2 congenital hydronephrosis.
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Original Article
Title
Clinical Characteristics of Coronavirus Disease 2019 at a Children's Hospital in 2020
Author
Kazuhiro Uda1) Hiroshi Sakakibara2) Kimihiro Taniguchi1) Hiroshi Higuchi3) Kazue Kinoshita4) Yuho Horikoshi1)5) Hanako Funakoshi1) Meiwa Shibata1) Satoko Suzuki2) and Hiroshi Hataya2)
1)Division of Infectious Diseases, Tokyo Metropolitan Children's Medical Center
2)Department of General Pediatrics, Tokyo Metropolitan Children's Medical Center
3)Division of Microbiology, Tokyo Metropolitan Children's Medical Center
4)Division of Molecular Laboratory, Tokyo Metropolitan Children's Medical Center
5)World Health Organization
Abstract
Although the characteristics of coronavirus disease 2019 (COVID-19) in adults have been reported, pediatric reports are scarce in Japan. This study aimed to describe the detailed clinical characteristics of COVID-19 in children. We reviewed medical records of COVID-19 patients younger than 18 year of age. The demographics, sick contacts, clinical symptoms, severity, co-detections, and viral load were obtained. In total, 46 patients were identified. The median age was 6 years (IQR 4-10 years), and 41 patients (89%) had a history of contact with a COVID-19 patient; of these, 35 patients (76%) had contact with an infected adult. The number of asymptomatic and symptomatic cases were 16 and 30, respectively. The severity of all cases was mild. Common symptoms were fever (73%), rhinorrhea (53%), and cough (30%). The median duration of fever was one day (IQR 1-2). Co-detection occurred in seven cases (17%). We found no significant difference in the viral load in nasal and stool samples between asymptomatic and symptomatic patients (p=0.43, p=0.16). Symptoms after discharge were observed in four patients, all of whom recovered within one month. All patients had mild symptoms, suggesting that pediatric COVID-19 might be difficult to differentiate from the common cold. In our cohort, most of the patients had a history of contact with an infected individual.
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Case Report
Title
Excessive Warfarin Anticoagulation Due to Gastroenteritis without the Use of Antibiotics
Author
Takaya Hanawa Miki Akiyama Katsutoshi Nakano Ryo Nakagawa Susumu Urata Hiroko Asakai Yoichiro Hirata Ryo Inuzuka and Akira Oka
Department of Pediatrics, the University of Tokyo
Abstract
Warfarin is a long-established vitamin K antagonist whose overload increases the risk of hemorrhage. Although various drug interactions have been reported, other conditions that have the potential to alter the effect of warfarin are not well-understood. This is particularly true in pediatric populations, given the small number of patients on anticoagulation therapy.
Here, we report two pediatric patients with excessive over-anticoagulation after gastroenteritis without antibiotic usage. Decreased absorption of dietary vitamin K might have led to this over-anticoagulation. Our experience sheds light on the possibility of over-anticoagulation due to warfarin in pediatric patients through a mechanism other than drug interactions.
When a child receiving an anticoagulant presents with vomiting and gastroenteritis is suspected, caution is required to prevent over-anticoagulation due to malabsorption of vitamin K. Frequent PT-INR monitoring and a temporary decrease (or suspension) of the warfarin dosage is recommended regardless of antibiotic usage in this setting.
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Case Report
Title
A Rare Case of Pediatric Thrombotic Thrombocytopenic Purpura Associated with Sjögren's Syndrome
Author
Mitsumasa Osuna1) Masanobu Takeuchi1) Seira Hattori1) Kenichi Nishimura1) Yumiko Takaishi2) Atsuhiro Iizuka1) Masahiro Yoshitomi1) Koji Sasaki1) Norio Shiba1) and Shuichi Ito1)
1)Department of Pediatrics, Yokohama City University
2)Department of Clinical Laboratory, Yokohama City University
Abstract
Thrombotic thrombocytopenic purpura (TTP) is a potentially fatal disease caused by the formation of microvascular thrombi throughout the whole body and it is classified as thrombotic microangiopathy. Most cases of secondary TTP occur due to autoimmune disease, especially systemic lupus erythematosus and scleroderma. On the other hand, TTP associated with Sjögren's syndrome (SS) is extremely rare. An eight-year-old boy with a medical history of recurrent parotitis presented with purpura and thrombopenia. A diagnosis of immune thrombocytopenic purpura (ITP) was made. However, his anemia gradually progressed, and he was transferred to our hospital for further examination and treatment. We initially diagnosed Evans syndrome based on thrombocytopenia and hemolytic anemia and administered prednisolone. After 5 days in the hospital, schizocytes were observed in a hemogram and TTP was suspected. We diagnosed acquired TTP based on a severe deficiency of ADAMTS13 (a disintegrin-like and metalloproteinase with thrombospondin type 1 motifs 13) activity and ADAMTS13 inhibitor positivity. In addition, SS was suspected based on Anti SS-A antibody-positivity. The final diagnosis was acquired TTP associated with SS.
The thrombocyte count tended to increase at the time of diagnosis. We therefore did not perform plasma exchange and continued prednisolone therapy. Prednisolone was gradually tapered in the outpatient clinic. He remains in full remission at 12 months after cessation of treatment. This is the first pediatric case to be reported in the world of TTP associated with SS.
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Case Report
Title
Hyper-IgE Syndrome with Leukemoid Reaction
Author
Kanako Yabumoto1) Shinya Osone1) Toshihiko Imamura1) Keisuke Okamoto2) Kazushi Izawa3) Hirokazu Kanegane2) Shigeru Makino4) Shinsaku Imashuku4) and Hajime Hosoi1)
1)Department of Pediatrics, Graduate School of Medical Science, Kyoto Prefectural University of Medicine
2)Department of Child Health and Development, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University
3)Department of Pediatrics, Graduate School of Medicine, Kyoto University
4)Division of Pediatrics, Uji-Tokushukai Medical Center
Abstract
Hyper IgE syndrome (HIES) is a rare primary immunodeficiency characterized by recurrent skin and lung infections, refractory eczema from neonates, and high serum IgE levels. Leukemoid reaction is a reactive hyperleukocytosis mimicking leukemia, caused by an underlying disease. HIES with leukemoid reaction has rarely been reported so far. A one-month-old boy was referred to our hospital due to generalized eczema, poor feeding and hyperleukocytosis despite receiving antibiotic therapy. He had been afebrile before referral. Upon admission, he was affected by pneumonia and blood examination showed marked hyperleukocytosis with neutrophilia and eosinophilia, and elevated serum IgE levels. Bone marrow examination revealed no leukemic blast. His pneumonia and leukocytosis improved with change in antibiotics based on the results of the antimicrobial susceptibility test. Because his mother was clinically suspected of HIES, we assumed the patient had autosomal dominant HIES. Genetic test confirmed the identical STAT3 mutation between him and his mother. Neutrophilia by prolonged bacterial infection and eosinophilia due to Th2-dominant deviation by loss of STAT3 function seemed to result in his leukemoid reaction. Although it is important to distinguish leukemoid reactions from hematologic malignancies, primary immunodeficiency should be considered when severe eosinophilia is present.
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Case Report
Title
Recurrent Hypoglycemia in an Infant with Difficult-to-control Glucose Storage Disease Type IIIa
Author
Aiko Honda1)2) Hiro Nakao1) Hiroyuki Iijima1) Akira Ishiguro2) and Mitsuru Kubota1)
1)Department of General Pediatrics, National Center for Child Health and Development
2)Center for Postgraduate Education and Training, National Center for Child Health and Development
Abstract
Glucose storage disease (GSD) type IIIa is a congenital metabolic disorder affecting glycogen debranching enzyme activity that causes accumulation of limit dextrin in various organs. The patients show hepatomegaly, hypoglycemia, growth retardation, and muscle weakness at presentation. Among these symptoms, hypoglycemia is generally thought to be mild compared to GSD type I, as gluconeogenesis is intact in GSD type IIIa patients. We report a case of a 10-month-old girl with GSD type IIIa who presented with severe hypoglycemia despite frequent high-carbohydrate meals. The patient was admitted because of hepatomegaly, dyspnea during milk feeding, and muscle weakness. The examination revealed elevation of liver deviation enzymes and creatine kinase. Based on glucose tolerance test results, glucagon loading during fasting, and measurement of leukocyte enzyme activity, her condition was diagnosed as GSD type IIIa. Although we immediately started dietary therapy, including uncooked cornstarch and GSD formula, she had repeated critical hypoglycemia. We had difficulty adjusting the quantity of diet to maintain normoglycemia. The probable reasons for unstable control of blood glucose levels are immature gluconeogenesis due to young age, insufficient protein intake, and deficient pancreatic amylase for digesting uncooked cornstarch. In conclusion, infantile GSD type IIIa patients might present with severe hypoglycemia. Therefore, physicians should consider the necessity of strict nutrition management in order to stabilize the blood glucose level.
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Case Report
Title
Usefulness of Multiplex PCR for Respiratory Pathogens in a Patient with Severe Combined Immunodeficiency
Author
Aoi Morishita1) Takeshi Isoda1) Motoi Yamashita2) Chihiro Takase1) Haruki Yamano1) Takahiro Tomoda1) Tsubasa Okano1) Takahiro Kamiya3) Masakatsu Yanagimachi1) Akifumi Endo3) Atsuna Tokumoto4) Tomohiro Watanabe4) Seiichi Watanabe4) Masatoshi Takagi1) Hirokazu Kanegane2) Kohsuke Imai5) and Tomohiro Morio1)
1)Department of Pediatrics and Developmental Biology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University
2)Department of Child Health and Development, Tokyo Medical and Dental University
3)Clinical Research Center, Tokyo Medical and Dental University Hospital of Medicine
4)Department of Pediatrics, Tsuchiura General Hospital
5)Department of Community Pediatrics, Perinatal and Maternal Medicine, Tokyo Medical and Dental University
Abstract
Severe combined immunodeficiency (SCID) is the most severe form of combined immunodeficiency and can be fatal during infancy without radical treatment. The most common type of SCID is X-linked SCID (X-SCID). We encountered a case of X-SCID in which influenza A virus was continuously detected by multiplex PCR after cord blood transplantation. In transplant patients and immunocompromised persons, sensitive PCR assay seemed useful. Although our case was complicated by influenza and other infections, the patient made favorable progress because the multiplex PCR assay was used to manage the infection more precisely, and hematopoietic cell transplantation could be performed while maintaining a good general condition. In SCID, early diagnosis and transplantation before infection are important, and the introduction of newborn screening is desirable. In the future, we will continue to investigate the screening of infections, therapeutic monitoring, and treatment outcomes using multiplex PCR in immunodeficiency diseases including SCID.
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Case Report
Title
Acute Infectious Purpura Fulminans Associated with Invasive Pneumococcal Disease
Author
Motoharu Ochi Hiroki Tsuchiya Takushi Inoue Junya Shimizu and Toshihide Kubo
Department of Pediatrics, National Hospital Organization Okayama Medical Center
Abstract
Purpura fulminans is a life-threatening disease that leads to a series of complications such as rapid progressive intravascular thrombosis, circulatory collapse, and cutaneous infarction. Purpura fulminans caused by severe infection is referred to as acute infectious purpura fulminans (AIPF). A previously healthy 6-month-old girl was admitted to our hospital due to fever, cold extremities, and cyanosis. The patient had difficulty breathing, and purpura was observed on her face and extremities. A blood test showed elevated inflammatory proteins and signs of disseminated intravascular coagulation (DIC). Empirical antibiotics, thrombomodulin alfa, antithrombin III concentrate, and fresh frozen plasma were quickly administered. Streptococcus pneumoniae was isolated from blood culture. Due to the complications associated with DIC, purpura on the extremities, and bacteremia, the patient was diagnosed with AIPF. Moreover, black necrosis on the right upper arm was observed, leaving a tiny scar. Despite these complications, the patient fortunately recovered without any other major sequelae. Early administration of antibiotics may have played an important role in mitigating the situation upon presentation of the patient at our department.
Serotyping of the Streptococcus pneumoniae isolate revealed a result of 33F, which is not covered by the 13-valent pneumococcal vaccine, further suggesting that 33F can lead to AIPF.
Although the 13-valent pneumococcal vaccine has been widely inoculated, serotype replacement was implemented. Therefore, AIPF associated with invasive pneumococcal disease can occur in otherwise healthy children. When suspected, early administration of antibiotics is important.
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Brief Report
Title
Impact of Coronavirus Disease 2019 on Pediatric Primary Emergency Medical Centers
Author
Hiroki Yabushita1) Shogo Otake1) Makoto Kimura2) Naohiro Kamiyoshi3) and Masashi Kasai1)
1)Division of Infectious Disease, Hyogo Prefectural Kobe Children's Hospital
2)Department of Pharmacy, Kobe Children's Primary Emergency Medical Center
3)Department of Pediatrics, Red Cross Society Himeji Hospital
Abstract
This study assessed the impact of coronavirus disease 2019 (COVID-19) on three pediatric emergency medical centers in Hyogo. We investigated children visiting these facilities between March and May 2019, and between March and May 2020. The number of patients decreased by 73.6%. The rate of contagious diseases decreased whereas the rate of non-contagious diseases and foreign bodies increased. This suggested an impact of social distancing and school closures. The proportion of patient visits by ambulance increased significantly from 0.9% to 1.6%, and patients referred to satellite hospitals also increased significantly from 3.0% to 4.3%, suggesting the impact of delayed access due to fear of COVID-19.
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