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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
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Vol.128, No.12, December 2024
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Review
Title
Asymptomatic Congenital Right Coronary Artery Fistula Diagnosed during Manifesting Kawasaki Disease-like Symptoms: A Case Report and Literature Review
Author
Haruka Ito1) Shintaro Kishimoto1) Kenji Hirae2) and Kenji Ihara1)
1)Department of Pediatrics, Oita University Faculty of Medicine
2)Department of Pediatrics, National Hospital Organization Beppu Medical Center
Abstract
Kawasaki disease (KD) is a well-recognized cause for coronary artery dilatation in children. In addition to KD, rare congenital anomalies like congenital coronary artery fistula (CAF) also cause the dilation of coronary arteries. We present a case of congenital CAF, which eluded detection during a routine medical examination for a cardiac murmur at 6 years of age but was subsequently identified at 7 years of age when he manifested KD-like symptoms. A comprehensive review of previously reported asymptomatic congenital CAF cases detected during childhood was conducted, emphasizing the physical examination and echocardiological findings crucial in diagnosing congenital CAF. We advocate all general pediatricians must be vigilant of potential congenital coronary artery diseases such as CAF, when encountering heart murmurs during routine health checkups or evaluating children with suspected KD. Since CAF can gradually enlarge, pediatric cardiologist's evaluation for long-term follow-up becomes imperative for all such patients.
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Original Article
Title
Collaborative Support of a General Hospital and Local Government for Pregnant Women with Special Needs during the COVID-19 Pandemic
Author
Mari Saito1) Yutaka Kikuchi1) and Masanori Kurosaki2)
1)Department of Pediatrics, Haga Red Cross Hospital
2)Department of Pediatrics, Jichi Medical University
Abstract
Beginning in 2020, the COVID-19 pandemic had wide-ranging repercussions on the social and economic systems of Japan. The pandemic affected the mental health, financial stability, and familial support received by pregnant women. This study examined these multifaceted challenges to improve support networks for pregnant women at increased risk of subjecting their children to abuse. This effort was facilitated by collaboration between our medical institution and the local government.
We divided the pregnant women into two groups for comparison: those who delivered in our hospital during the two years post-January 2020 ("pandemic-era") and those who delivered before the pandemic. The number of pregnant women with special needs increased significantly during the pandemic. Although perinatal background factors remained relatively consistent, fewer women returned to their hometowns for family support during the pandemic. Additionally, the pandemic-era cohort showed trends to increase the number of categories such as "refusal of social support", "low household income", "welfare assistance", "difficulty understanding Japanese" and "lack of comprehension" compared to their pre-pandemic counterparts. The number of newborns who were not followed up increased during the pandemic; however, the ratio of newborns with no suspicion of child abuse after 5 months of age remained consistent.
In conclusion, the COVID-19 pandemic increased the number of special-need pregnancies, however, collaborative support from our medical institution and the local government helped ensure high-quality maternal and newborn care throughout the COVID-19 pandemic.
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Case Report
Title
A Neonatal Case of Transient B-cell Depletion with Undetectable KREC Level on Newborn Screening
Author
Takashi Matsunaga1) Kuniaki Tanaka1)2) Yoshiki Kusama3) Atsushi Iwai1)2) Kenichiro Kobayashi1)2) Kazushi Izawa4) Takahiro Yasumi4) Toshiro Maihara1) Ikuya Usami1)2) and Toshio Heike1)
1)Department of Pediatrics, Hyogo Prefectural Amagasaki Medical Center
2)Department of Pediatric Hematology and Oncology, Hyogo Prefectural Amagasaki Medical Center
3)Department of Infection Control and Prevention, Graduate School of Medicine, Osaka University
4)Department of Pediatrics, Graduate School of Medicine, Kyoto University
Abstract
Recently, kappa-deleting recombination excision circle (KREC) PCR quantification is widely used as a newborn screening test for congenital B-cell aplasia. However, the influence of maternal factors on KREC levels remains to be fully elucidated.
A male infant whose mother took azathioprine for SLE was referred to our hospital due to undetectable KREC PCR quantitative test on newborn screening. Sequential flowcytometry analyses of peripheral blood revealed that the counts of B cells had gradually increased along with decline of the ratio of CD10 positive B cells. These data indicated transient B cell aplasia in fetal period due to maternal AZA administration. We analyzed the codon139 polymorphism of the NUDT15 gene encoding a metabolizing enzyme of AZA and found that he had heterozygous p.Arg139Cys polymorphism of paternal origin. We considered that this polymorphism reduced NUDT15 activity and resulted in the suppression of fetal B cell production by transplacental AZA.
This case suggests that suppression of B cells due to maternal AZA administration during pregnancy should be noted as a cause of low KREC levels at newborn screening test. Additionally, genetic polymorphisms of NUDT15 can be related to the effect of maternal AZA administration on immunosuppression during fetal and neonatal period.
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Case Report
Title
Emergency Airway Management after Tracheotomy Closure in a Child with Severe Motor and Intellectual Disabilities
Author
Hazuki Hanai1) Atsuko Arisaka1) Remi Kuwabara1) Akira Takei1) Tomokuni Yoshihashi1) Yu Suzuki2) Hisaya Hasegawa3) and Tae Omori1)
1)Department of Pediatrics, Tokyo Metropolitan Bokutoh Hospital
2)Department of Pediatrics, Tokyo Women's Medical University, Adachi Medical Center
3)Department of Neonatology, Tokyo Women's Medical University, Adachi Medical Center
Abstract
In cases of pediatric tracheotomy, decannulation is feasible in approximately 10% to 30% of patients, with tracheal cutaneous fistulas occurring in 6.2% to 52.2% of these instances. This report describes an emergency airway management case following a tracheal cutaneous fistula closure operation.
A 4-year-old boy underwent tracheotomy for recurrent apnea episodes following RSV-related acute encephalopathy and was deemed unsuitable for decannulation. Following the procedure, his apneic episodes ceased, and the cannula was removed 5 months later. However, the tracheotomy site failed to close naturally, necessitating a closure operation three months following decannulation. Although the procedure initially proceeded without complications, the patient developed inspiratory stridor, retracted breathing, and a decline in oxygenation the following day, leading to the decision to intubate for airway management.
This case underscores the necessity of vigilant preoperative evaluation and patient interviews to assess the risk of requiring emergency airway management post-tracheotomy closure. The presence of factors such as increased trachea wall mobility, elevated inspiratory and expiratory pressures, and periwound edema contribute to respiratory stenosis and acquired tracheomalacia, complications inherent to the postoperative trajectory of tracheotomy. Adequate preparation and comprehensive patient and caregiver education are critical to anticipate and mitigate potential complications.
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Case Report
Title
Successful Outcome of Coil Embolization of a Gastroduodenal Artery Pseudoaneurysm in a Boy with Chondrodysplasia Punctata
Author
Maki Asahina1) Yukichi Tanahashi2) Yohei Masunaga1) Kenichi Kinjyo1) Tomohiko Hasegawa3) Kaisyu Oda1) and Yasuko Fujisawa1)
1)Department of Pediatrics, Hamamatsu University School of Medicine
2)Department of Radiology, Hamamatsu University School of Medicine
3)Department of Orthopedic Surgery, Hamamatsu University School of Medicine
Abstract
Gastroduodenal artery aneurysms are rare in children. Chronic pancreatitis, pancreatic pseudocysts, and duodenal ulcers are underlying conditions for their development. Herein, we report a case of a pseudoaneurysm caused by a duodenal ulcer after posterior intervertebral fusion of an axial vertebral subluxation, which was successfully saved by coil embolization.
A 1-year-old boy with chondrodysplasia punctata underwent posterior vertebral body fusion to relieve cervical spinal cord compression due to the subluxation of the annular axis vertebrae. On postoperative day 4, fresh gastric blood and black stools were observed, but the obvious source of bleeding could not be identified on abdominal contrast-enhanced computed tomography (CT). Anemia was noted due to persistent gastrointestinal bleeding. On postoperative day 14, the anemia (Hb 6.3 mg/dL) worsened, now with disseminated intravascular coagulation syndrome (DIC). A repeated abdominal contrast-enhanced CT identified a gastroduodenal artery aneurysm, which was diagnosed as pseudoaneurysm in the duodenal ulcer area. Emergency angiography and coil arterial embolization were performed. The gastrointestinal hemorrhage and DIC then rapidly improved.
In cases of severe gastrointestinal hemorrhage, the possibility of a pseudoaneurysm should be considered, and arterial embolization may be a treatment option.
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Case Report
Title
Diagnosis of Paraovarian Cyst in an adolescent Using Abdominal Ultrasound Following Acute Abdomen Presentation
Author
Hitomi Mizumoto1) Naoki Yogo1) Yugo Takaki1) Takuya Sugimoto2) Kazuhiko Yoshimoto3) and Katsuki Hirai1)
1)Department of Pediatrics, Japanese Red Cross Kumamoto Hospital
2)Department of International Medical Relief, Japanese Red Cross Kumamoto Hospital
3)Department of Pediatric Surgery, Japanese Red Cross Kumamoto Hospital
Abstract
Paraovarian cysts are rarely reported in pediatric cases. However, the stretching and torsion of fallopian tubes associated with these cysts can affect fertility, requiring early diagnosis and surgical intervention. It is difficult to make a preoperative diagnosis.
The patient, a healthy 13-year-old adolescent girl, presented with a sudden onset of severe abdominal pain. Her discomfort persisted, and ultrasonography revealed a cystic lesion in the pelvis region. She was suspected to have ovarian cyst stem torsion. Furthermore, ultrasonography revealed cystic lesions bordering the bilateral ovaries and the left fallopian tube, along with increased blood flow velocity in the vicinity of the fallopian tube apparatus. Paraovarian cyst and tubal torsion were suspected, and an emergency laparoscopic surgery was performed. During the surgery, a cyst in the left parafollicular area and torsion of the left fallopian tube were observed. Upon releasing the torsion, the fallopian tubes appeared stretched and congested, without apparent signs of ischemia or necrosis. The enucleated cyst showed no malignant characteristics.
Considering paraovarian cysts as a potential cause of acute abdomen condition is crucial due to their propensity to cause tubal torsion, thereby affecting fertility.
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