|
THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
|
Vol.125, No.12, December 2021
|
Review
Title
The Latest Treatment Strategy Considering the Pathophysiology of Lupus Nephritis
Author
Yukihiko Kawasaki
Department of Pediatrics, Fukushima General Rehabilitation Center
Abstract
Systemic lupus erythematosus is a systemic chronic inflammatory disease of blood vessels and connective tissue. The pathogenic mechanism is that the addition of environmental factors to the genetic predisposition causes an abnormal immune response and the formation of an immune complex. Organ damage is caused by the deposition of these immune complexes in tissues. Lupus nephritis is the most common organ lesion. Compared with adults, childhood lupus nephritis has higher disease activity and is one of the factors that determine prognosis.
We discuss the genetic predisposition, environmental factors and immune response abnormalities and immune complex formation processes associated with the pathogenesis of lupus nephritis and review the mechanism of inflammatory progression of nephritis and therapeutic strategies for controlling these pathologies. Furthermore, the efficacy and problems of molecular-targeted drugs including belimumab are also mentioned. In the treatment of LN, it is important to select a treatment that balances the therapeutic effect and long-term side effects.
|
|
Review
Title
The Status of Vitamin K Deficiency Bleeding in Infancy after Revision of Guidelines
Author
Kazufumi Yaginuma Nobuo Momoi Miku Munakata Miki Yaegashi Mamiko Nakazawa Yasushi Saito Kazuhide Suyama and Mitsuaki Hosoya
Department of Pediatrics, Fukushima Medical University
Abstract
The incidence of vitamin K deficiency bleeding (VKDB) in infancy has been reduced since the administration of three doses of oral vitamin K2 supplements (3-dose regimen) became widespread in Japan. However, VKDB cases have been reported in recent years.
We report the treatment of two infants who developed VKDB after undergoing the 3-dose regimen and reviewed all VKDB cases reported in Japan since 2011. Sixty cases, including our two cases have been reported. Forty-eight patients had an underlying cholestasis illness, the most common of which (30 cases) was biliary atresia, and 9 cases were idiopathic. The three times method was employed in four of the idiopathic cases and 13 of the secondary cases. To date, there have been no reported Japanese cases where patients were administered 13 weekly oral doses of vitamin K2 supplement until the age of 3 months (weekly method). Fifty-nine patients developed intracranial hemorrhage. Of the 32 patients whose prognosis was reported, 8 died and 13 had neurological sequelae. These findings reveal that the three times method may be insufficient to prevent VKDB, patients who develop cranial hemorrhage have serious outcomes, and there have been no Japanese VKDB patients who have undergone the weekly method. The weekly method should be considered for all healthy term neonates and infants.
|
|
Original Article
Title
Clinical Experience of Using Cefmetazole for the Treatment of New-onset Pediatric Upper Urinary Tract Infection
Author
Shota Fujito1) Hideki Ban1)2) Mariko Nagayoshi1) Naoto Hotokebuchi1) Akio Furuse1) Katsuki Hirai1) and Masahiro Migita1)
1)Department of Pediatrics, Japanese Red Cross Kumamoto Hospital
2)Department of Pediatric Nephrology, Tokyo Women's Medical University
Abstract
Introduction
Among the causative agents of pediatric upper urinary tract infection (UUTI), an increase in the percentage of extended-spectrum β-lactamase (ESBL)-producing bacteria has been observed recently. In our hospital, cefmetazole (CMZ) is used as the first-line treatment for pediatric UUTI.
Methods
Among patients with new-onset pediatric UUTI admitted to our department between April 1, 2015 and March 31, 2019, data on those with singly arranged gram-negative bacilli urine Gram staining samples on admission and those who were administered with CMZ as the first-choice antibiotic based on medical records were retrospectively reviewed.
Results
We included 63 patients−17 (27%) with UUTI caused by ESBL-producing bacteria and 46 (73%) with UUTI caused by non-ESBL-producing bacteria. All causative agents showed susceptibility to CMZ. In four (6%) patients, the antibiotic was switched to meropenem before identification of antimicrobial susceptibility. The median treatment duration was 13 days. No patients experienced a relapse within 4 weeks after treatment completion. No adverse reactions associated with CMZ use were reported.
Conclusion
After considering the regional antibiogram based on the children's age and urine Gram staining results, CMZ was used as the first-choice antibiotic for treating new-onset pediatric UUTI. This protocol allowed us to plan and administer susceptible antibiotic therapy from treatment initiation.
|
|
Original Article
Title
Factors Influencing Parents' Agreement to Include Their Children in Clinical Trials for COVID-19 Vaccine Development
Author
Kayo Yoshimura1) Shinobu Tatsunami1) Ran D Goldman2) Yukitsugu Nakamura1) Ayano Shinagawa1) Maho Kobayashi1) Rei Fumimoto1) Takumi Moriuchi1) Ryo Niiya1) Tomohiro Katsuta1) and Naoki Shimizu1)
1)Department of Pediatrics, St. Marianna University School of Medicine Hospital
2)University of British Columbia, BC Children's Hospital
Abstract
In view of the coronavirus disease 2019 (COVID-19) pandemic, urgent vaccine development is required. Clinical trials in children are essential for evaluating the safety and efficacy of vaccines in children. This study examined factors influencing parents' agreement to allow their children to participate in COVID-19 vaccine clinical trials.
We conducted a questionnaire survey among parents of pediatric patients who visited either the emergency department or the pediatric outpatient department of St. Marianna University School of Medicine Hospital and Kawasaki Municipal Tama Hospital between May 22, 2020 and July 31, 2020. Subsequently, we statistically examined factors that were associated with parents' agreement to allow their children to participate in vaccine clinical trials.
A total of 219 parents participated in the survey, among which 214 responded to whether their children could participate in a vaccine clinical trial. Only 20 (9.3%) parents agreed to allow their children to participate in the clinical trial, compared to 18.4% in studies conducted outside of Japan (p = 0.0012). Parents willing to participate themselves were more inclined to agree for their children's participation (p < 0.001). Additional evaluation is needed to understand the factors influencing Japanese parents' agreement to allow their children to participate in such trials.
|
|
Original Article
Title
Analysis of Childhood Bacterial Meningitis, Using an Online Surveillance System
Author
Nozomi Hamamoto1)2) Tadashi Matsubayashi1)2) and Toshihiro Tanaka1)3)
1)Shizuoka Pediatric Infectious Disease Surveillance Study Group
2)Department of Pediatrics, Seirei Hamamatsu General Hospital
3)Department of Pediatrics, Shizuoka Kosei Hospital
Abstract
A nationwide immunization program of Haemophilus influenzae type b (Hib) vaccine and pneumococcal conjugate vaccine (PCV) was introduced in April 2013. We registered cases of childhood bacterial meningitis at 30 medical institutions in Shizuoka prefecture, Japan, using an online surveillance system and analyzed the trends of the incidence before and after the introduction of vaccines. The patients affected prior to September 2013, when the system was launced, were enrolled retrospectively, and thereafter enrolled prospectively until December 2019. We analyzed patients affected between January 2006 and December 2019. During this period, 188 patients (102 boys and 86 girls) were enrolled. The number of H. influenzae and pneumococcal meningitis cases has decreased significantly, while the number of cases of menitngitis caused by Group B Streptococcus (GBS) and Escherichia coli, for which no effective vaccine is available, remained unchanged. These results indicate that the introduction of Hib vaccine and PCV contributes to the reduction of meningitis caused by H. influenzae and pneumonocci. The future issues are to sustain high rates of vaccination coverage and prevent meningitis caused by non-PCV serotype pneumococci, GBS and E. coli. The construction and effective use of the online surveillance system can enable rapid feedback of information to medical institutions, which will help to maintain motivation for vaccine promotion.
|
|
Original Article
Title
The Effect of Public Health Measures on Pediatric Outpatients and Incidence of Respiratory Infections during the COVID-19 Pandemic in a Japanese Hospital
Author
Hiroshi Fukumasa and Kazutaka Nishiyama
Department of Pediatrics, Kitakyushu City Yahata Hospital
Abstract
Introduction: In Japan, the state of emergency was declared nationwide against Coronavirus disease 2019 (COVID-19) in 2020. As various modes of voluntary restraint spread, avoidance of seeking medical care developed into a social problem.
Objective: The purpose of this study was to survey the current number of pediatric outpatients at the Kitakyushu City Yahata Hospital during the COVID-19 pandemic.
Methods: We undertook an observational study of the number of pediatric outpatients, who visited our hospital in 2020. We also surveyed the following: influenza virus (Flu), respiratory syncytial virus (RSV), and human metapneumovirus (hMPV) during the same period. We compared the number of pediatric outpatients and the spread of infectious diseases in 2018, 2019, and 2020.
Results: The proportion of outpatients was 72.1% in 2020 compared to 2018 and 2019. During the state of emergency, the proportion of outpatients decreased the most (44.7%). After the state of emergency was lifted, the proportion of outpatients was 69.7%, and a remarkable decrease was found particularly in emergency outpatients (56.6%). The Flu and hMPV seasons were shorter and RSV had remained low compared to the previous season.
Conclusion: During 2020, the number of pediatric outpatients decreased and continued to decline in our hospital. These findings might reflect not only avoidance of hospital visits due to fear of COVID-19 but also the effectiveness of school closures and infection preventive measures to reduce the transmission of infectious diseases.
|
|
Case Report
Title
A Case of Infantile Pyknocytosis Leading to Bilirubin Encephalopathy with UGT1A1 Polymorphism
Author
Yuka Ueda1) Kunihiro Matsunami1) Shuji Kuwabara1) Hiromi Ogura2) Hitoshi Kanno2) Takahide Yanagi3) and Atsushi Imamura1)
1)Department of Pediatrics, Gifu Prefectural General Medical Center
2)Department of Transfusion Medicine and Cell Processing, Tokyo Women's Medical University
3)Department of Pediatrics, Shiga University of Medical Science
Abstract
Infantile pyknocytosis (IP) is a rare disease that causes hemolytic anemia and jaundice mainly in the neonatal period. Although it was reported to be self-limited, we report a case of IP with severe jaundice requiring exchange blood transfusion, leading to bilirubin encephalopathy. A 15-day-old girl was admitted to our hospital because of severe jaundice and consciousness disturbance. Unconjugated hyperbilirubinemia was noted on admission and hemolytic anemia was found later. After exchange transfusion, the hyperbilirubinemia gradually improved; however, MRI performed on day 25 demonstrated characteristic findings of bilirubin encephalopathy. On investigation of the cause of hemolytic anemia, osmotic resistance increased and many pyknocytes were observed in peripheral blood smears. As both morphological abnormalities and metabolic disorders were excluded based on her red blood cells, she was diagnosed with IP. Genetic analysis of the UDP-glucuronosyltransferase 1A1 gene (UGT1A1), which regulates glucuronidation, revealed a heterozygous mutation of UGT1A1*6 (c.211G>A, p.G71R). UGT1A1*6 may have been associated with the severe jaundice.
|
|
Case Report
Title
Inherited Corticosteroid-Binding Globulin Deficiency Complicated with Orthostatic Dysregulation
Author
Yumiko Ikemoto1) Eriko Kitera1) and Kazunori Kageyama2)
1)Department of Pediatrics, Himeji St. Mary's Hospital
2)Department of Endocrinology and Metabolism, Hirosaki University Graduate School of Medicine
Abstract
We evaluated a 13-year-old girl with low serum cortisol levels in association with a severe symptom of orthostatic dysregulation. Laboratory data showed normal levels of plasma ACTH and 24-h urinary free cortisol excretion. Levels of other anterior pituitary hormones were within normal range and thyroid function results were also normal. Cortisol diurnal variation was not disturbed. Rapid administration of intravenous ACTH showed a normal response to cortisol. Both plasma ACTH and serum cortisol responses to CRH were normal. Eighty to 90% of circulating cortisol is bound to Corticosteroid-binding globulin (CBG), 10-15% is bound to albumin and approximately 5% of plasma cortisol is free and biologically active.
The patient's serum CBG level was extremely low. Genetic mutations encoding for CBG have been characterized with defects in production or cortisol-binding activity of CBG. Low levels of serum cortisol and CBG levels were also detected in some members of her family. Therefore, inherited CBG deficiency was suggested in our patient. In some individuals with CBG deficiency, hypotension or chronic fatigue disorders would be present, even with normal circulating free cortisol levels. Her symptoms such as orthostatic dysregulation might be modified by low CBG levels.
|
|
Case Report
Title
A Case of Orbital Cellulitis with Septic Cavernous Sinus Phlebitis and Pulmonary Embolism
Author
Yukari Kinjo1) Yoshiaki Cho2) Tomoki Kawahara1) Teppei Okawa1) Tomotada Takayama1) Naoya Tonegawa1) and Takashi Matsuoka1)
1)Okinawa Prefectural Nanbu Medical Center & Children's Medical Center, Department of General Pediatrics
2)Okinawa Prefectural Nanbu Medical Center & Children's Medical Center, Department of Pediatric Infectious Diseases
Abstract
A 6-year-old boy who presented with fever and swelling of the left eyelid with a history of bronchial asthma, was admitted to our hospital with a diagnosis of left orbital cellulitis, MRSA bacteremia, asymptomatic septic pulmonary embolism, and left cavernous sinus thrombophlebitis. The patient had repeated remissions and exacerbations, was hospitalized for about 30 days and treated with intravenous antimicrobial agents and anticoagulation, followed by continued oral antimicrobial therapy on an outpatient basis.
Orbital cellulitis is often caused by sinusitis, commonly caused by Streptococcus pneumoniae, and Haemophilus influenzae. Staphylococcus aureus has become increasingly common to cause this condition in recent years. Staphylococcus aureus accounted for 28% of the causative organisms, and methicillin-resistant Staphylococcus aureus (MRSA) was reported to account for 6.5% of cases worldwide.
Orbital cellulitis can be triggered by oral infections, or trauma and is a prognostic factor in visual acuity. Therefore, it is important to treat the patient with appropriate antimicrobial agents at an early stage.
|
|
|
Back number
|
|
