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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY

Vol.126, No.7, July 2022


Original Article
1. Maternal Risk Factors for Postpartum Depression in a 2-Week Medical Examination in Our Hospital
2. Prophylactic Elective Surgery for Innominate Artery Compression of the Trachea in Patients with Severe Motor and Intellectual Disabilities
Case Report
1. The 11-Year Course of a Patient with Left Ventricular Non-Compaction Who Developed Cardiogenic Shock in Infancy
2. A Girl with Genetic CYP2C19 Polymorphism Showing Depressed Conscious State after Fosphenytoin Treatment
3. Anorexia Nervosa-Associated Constipation Complicated by Pneumatosis Intestinalis
4. Down's Syndrome with Atophic Thyroiditis
5. Severe Neonatal Pneumonia Secondary to Coronavirus Disease-2019
6. A Case of Inguinal Pyogenic Lymphadenitis in an Infant Rapidly Progressing to Fournier's Gangrene Requiring Emergency Debridement


Original Article
Title
Maternal Risk Factors for Postpartum Depression in a 2-Week Medical Examination in Our Hospital
Author
Yoshiko Iwamaru Akio Nakamura Yoshimi Nishizaki Mari Hagio Kumiko Inoue and Shoichi Oyama
Department of Pediatrics, Saiseikai Kawaguchi General Hospital
Abstract
We carried out a 2-week medical examination for newborns and mothers in our hospital to examine postpartum depression from the pediatrician's viewpoint discovered within that period. The newborns and their mothers who gave birth in our hospital between June 2016 and March 2020 underwent the Edinburgh Postnatal Depression Scale (EPDS) during a 2-week medical examination, and were assessed as having a depression tendency if their EPDS score was 9 or higher. We examined the relationship between depression tendency and each factor using the χ2 test, and found that parturition history and whether or not parenting support forms had been submitted or not was associated with depression tendency. It seemed that parturient women who had previously experienced child-raising, and those who had submitted parenting support forms and subject to more interventions by medical institutions and regional welfare organizations, before and after parturition, seemed to have lower depression tendency. This study indicated that many mothers newly experience depression tendency after parturition, therefore it was difficult to specify target subjects for the 2-week medical examination. A 2-week medical examination should be conducted for all births since there are many cases in which multiparous women have difficulties raising children with siblings and require different interventions than primiparous women.




Original Article
Title
Prophylactic Elective Surgery for Innominate Artery Compression of the Trachea in Patients with Severe Motor and Intellectual Disabilities
Author
Tomomi Hasegawa
Department of Pediatric Critical Care Medicine, Kobe Children's Hospital
Abstract
Recent advances in respiratory support technologies have had a significant impact on the natural history of patients with severe motor and intellectual disabilities (SMID). However, the development of thoracic deformities, and placement of a tracheostomy tube in these patients can result in an innominate artery compression of the trachea (IACT), leading to a fatal tracheoinnominate artery fistula (TIF). This study aimed to review our surgical management of IACT in patients with SMID, and to evaluate the efficacy of prophylactic elective surgery for IACT. This retrospective study examined 34 patients with SMID who underwent innominate artery transection for IACT. The clinical outcomes of prophylactic elective surgeries (Group P) for IACT were compared with the outcomes of emergent surgeries (Group E). The number of patients in whom cerebral circulation was assessed before surgery in Group P was significantly higher than those in Group E. The operative benefits in Group P included a smaller skin incision, more limited sternotomy, less blood loss, less blood transfusion, and shorter operative time and hospital stay. All of these are valuable for minimally invasive surgery. There were no cases of postoperative ischemic complications of the right-sided brain or arm due to innominate artery transection. These findings suggest that prophylactic elective surgery for IACT can not only prevent the fateful occurrence of TIF, but also provide sufficient preoperative assessment and minimally invasive surgery, leading to an improved postoperative recovery in patients with SMID. With appropriate risk management for prophylactic elective surgery, this would be a helpful surgical treatment for IACT in patients with SMID.




Case Report
Title
The 11-Year Course of a Patient with Left Ventricular Non-Compaction Who Developed Cardiogenic Shock in Infancy
Author
Yoshikazu Otsubo1) Tadaharu Matsuyama2) Yasuo Kido2) Mari Yokokawa1) and Katsuhiko Yamada3)
1)Department of Pediatrics, Sasebo City General Hospital
2)Department of Radiology, Sasebo City General Hospital
3)Department of Pediatrics, Sasebo Chuo Hospital
Abstract
Left ventricular non-compaction (LVNC) is a form of cardiomyopathy with the left ventricular myocardium morphologically characterized by a two-layered structure of a compacted and a non-compacted layer and excessive trabeculation presenting concomitant deep recesses. It is caused by the interruption of myocardial compaction seen in the early stages of embryonic development. While the clinical features range from no symptoms to severe heart failure, and the diagnosis period varies from newborns to adults, cases of severe heart failure under 1 year of age, LVNC-related gene mutations, and myocardial fibrosis can be associated with poor prognoses.
We, herein, report a case of an 11-year-old boy with infantile onset LVNC demonstrating a stable course through oral medication, such as β inhibitors, alone for 11 years after experiencing cardiogenic shock at 3 months of age. Although the left ventricular ejection fraction remains around 40%, the N-terminal pro b-type natriuretic peptide level normalized in 10 years after onset. T1 mapping of cardiac MRI revealed an extension of native T1 and an increase in extracellular volumetric fraction indicating myocardial fibrosis. Genetic testing confirmed heterozygous missense mutations in the tropomyosin 1 gene. Therefore, it is necessary to manage this patient who is expected to have a long-term poor prognosis.




Case Report
Title
A Girl with Genetic CYP2C19 Polymorphism Showing Depressed Conscious State after Fosphenytoin Treatment
Author
Fumika Kuribayashi1) Norimoto Kobayashi1) Kanae Hirabayashi1) Yoichiro Kawasaki1) Takefumi Ishida1) Yoshiro Amano1) and Hidemoto Fujiwara2)
1)Department of Pediatrics, Nagano Red Cross Hospital
2)Department of Neurosurgery, Nagano Red Cross Hospital
Abstract
Fosphenytoin (fos-PHT) is a prodrug of phenytoin (PHT) and is used as a second-line drug for status epilepticus. Reports of pediatric cases of depressed conscious state caused by fos-PHT are rare. A 3-year-old girl underwent craniotomy for left epidural hematoma on the left side of the head. Fos-PHT was administered at the recommended dosage to prevent epileptic seizures after brain surgery. Two hours after receiving the second maintenance dose, the patient complained of dizziness and became restless with frequent vomiting. The serum concentration of PHT was 51 μg/mL, which is within the toxic range, and her symptoms improved as the serum PHT concentration decreased. CYP2C9 and CYP2C19 are the enzymes responsible for metabolizing of PHT, and their variants have low clearance of PHT. This patient had a polymorphism in the CYP2C19 gene, making her a poor metabolizer of PHT. In addition, multiple administrations of the maintenance dose were considered to be the cause of increase in the serum concentration. Considering the high prevalence of CYP2C19 gene polymorphisms in the Japanese population, close monitoring of the serum PHT concentration and adverse neurological symptoms is essential when fos-PHT is administered multiple times.




Case Report
Title
Anorexia Nervosa-Associated Constipation Complicated by Pneumatosis Intestinalis
Author
Yutaro Kawakami Takumi Yamashita Tomokuni Yoshihashi Tae Omori and Masahiro Misawa
Department of Pediatrics, Tokyo Metropolitan Bokutoh Hospital
Abstract
Anorexia nervosa (AN) causes various physical symptoms secondary to long-term malnutrition. It is often accompanied by abdominal pain caused by constipation. We encountered a patient with pneumatosis intestinalis (PI) who presented with abdominal pain during treatment of AN-associated constipation.
The patient was an 11-year-old girl with AN accompanied by constipation. Treatment was initiated with tube feeding, laxatives, and an enema. Abdominal X-ray imaging undertaken for follow-up of constipation revealed PI. Only conservative treatment methods, such as fasting, intestinal rest, drug suspension, and low-flow oxygen therapy, were administered and found to be effective.
AN is frequently accompanied by constipation. Patients with constipation accompanied by abdominal pain may present with PI, which requires emergency surgical intervention. It is therefore necessary to consider regular imaging in suspected cases. Additionally, low-flow oxygen therapy may potentially improve the symptoms of PI. Therefore, this therapy should be considered for implementation, considering its efficacy.




Case Report
Title
Down's Syndrome with Atophic Thyroiditis
Author
Risa Yanai Kaori Maruwaka and Yoshimitsu Goto
Japanese Red Cross Aichi Medical Center Nagoya Daini Hospital
Abstract
Atrophic thyroiditis is an autoimmune disease associated with hypothyroidism without thyroid enlargement. In autoimmune thyroiditis, the thyroid gland is not enlarged and is underactive. The disease is often detected long after onset and is commonly associated with multiple organ failure and myxoedema.
We report a case of a 6-year-old girl with Down syndrome as an underlying disease. Her growth rate had been arrested for one year until she was admitted at the age of six. She showed generalized edema and decreased liver and kidney function and was diagnosed with hypothyroidism based on clinical symptoms and laboratory findings. Treatment with levothyroxine sodium was started, which resulted in improvement of thyroid function and other complications.
Hypothyroidism in patients with Down syndrome is difficult to detect because they have similar symptoms such as constipation, obesity, and decreased appetite. To detect atrophic thyroiditis, it is recommended to focus on growth rate, as in children without Down syndrome. Hypothyroidism may occur in school-age children or later. Therefore, periodic screening for thyroid function in children with Down syndrome is important, even in cases that have progressed without problems in early childhood.




Case Report
Title
Severe Neonatal Pneumonia Secondary to Coronavirus Disease-2019
Author
Yuki Ami1) Keisuke Yoshino3) Koji Kanno1) Yoshiaki Cho2) and Naoki Fujiwara1)
1)Pediatric Intensive Care Unit, Okinawa Prefectural Nanbu Medical Center & Children's Medical Center
2)Pediatric Infectious Disease, Okinawa Prefectural Nanbu Medical Center & Children's Medical Center
3)Pediatrics, Okinawa Prefectural Miyako Hospital
Abstract
We report a case of severe pneumonia caused by coronavirus disease 2019 (COVID-19) in a previously healthy 16-day-old girl living on a remote island in Okinawa prefecture.
At the beginning of her illness, her condition was stable, with only fever and brief episodes of hypoxia during feeding and sleep, and she did not require supplemental oxygen administration. The fever improved in a few days, and the hypoxia improved in a week. After she was discharged, her condition rapidly deteriorated. She was readmitted and treated with remdesivir and dexamethasone and required mechanical ventilation. As soon as mechanical ventilation was established, she was transferred to a higher-level facility because it was challenging to manage a severe case of neonatal COVID-19 pneumonia on a remote island. During the airlift, there were difficulties with medical examination, monitoring, troubleshooting, and maintaining infection control, but the patient was safely transported to the referral facility. She was discharged from the referral facility 42 days after the onset of illness, without any sequelae or side effects of the antiviral drugs.
With cases of neonatal COVID-19, careful follow-up is necessary because the clinical condition may worsen rapidly, even after initial improvement. If the clinical condition worsens, with signs such of respiratory effort or development of hypoxia, the patient should be transferred to a higher-level medical facility as soon as possible. Particularly in environments such as remote islands, it is necessary to make rapid decisions regarding patient transport.




Case Report
Title
A Case of Inguinal Pyogenic Lymphadenitis in an Infant Rapidly Progressing to Fournier's Gangrene Requiring Emergency Debridement
Author
Riho Aoyama1) Sayaka Mii1) Kensuke Mizutani1) Yuichi Nishikado1) Nobuhiro Akita1) Daichi Fukumi1) Naruhiko Murase2) and Masahiko Fujino3)
1)Department of Pediatrics, Japanese Red Cross Aichi Medical Center Nagoya Daiichi Hospital
2)Department of Pediatric Surgery, Japanese Red Cross Aichi Medical Center Nagoya Daiichi Hospital
3)Department of Pathology, Japanese Red Cross Aichi Medical Center Nagoya Daiichi Hospital
Abstract
Fournier's gangrene is common in men in their 50s with underlying diseases, but rare in children1). We successfully treated an infant with no underlying disease by emergency debridement since his condition rapidly progressed from pyogenic lymphadenitis of the inguinal region to Fournier's gangrene.
Case: A 2-month-old male infant. The mother noticed swelling in the left inguinal region. The previous doctor suspected inguinal hernia, but manual repair failed. We diagnosed inguinal pyogenic lymphadenitis based on contrast-enhanced CT and administered intravenous antibiotics with drainage outside the left inguinal region. The fever did not resolve, and local findings rapidly deteriorated. Emergency debridement was performed 24 hours after onset. Intraoperative findings showed extensive necrosis of the subcutaneous tissue, but not the fascia. MSSA was detected in the external drainage wound and intraoperative necrotic tissue culture. Postoperative fever quickly resolved, and the wound improved markedly with wound lavage and intravenous antibiotics. No immunological abnormalities were detected.
Fournier's gangrene in children, who often have underlying diseases (e.g., trauma, circumcision, or immunodeficiency) is rare. The mortality rate in children < 3 months is 9-30%. Even in cases with no underlying disease, Fournier's gangrene should be kept in mind, especially in younger children.




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