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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
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Vol.129, No.6, June 2025
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Original Article
Title
Red Blood Cell Transfusion for Severe Gradual-onset Anemia in Children: A Retrospective Study in a Single Institute
Author
Mamoru Honda1) Yuki Arakawa1) Yoshitaka Mizushima1) Tomoya Irikura1) Takahiro Ishikawa1) Ryota Kaneko1) Hirohito Kubota1) Yuichi Mitani1) Makiko Mori1) Kohei Fukuoka1) Koichi Oshima1) Satoshi Nakano2) Takehiro Niitsu3) Fumika Ozawa4) Kazumi Satake4) and Katsuyoshi Koh1)4)
1)Department of Hematology/Oncology, Saitama Children's Medical Center
2)Department of Emergency Medicine, Saitama Children's Medical Center
3)Department of Critical Care Medicine, Saitama Children's Medical Center
4)Department of Transfusion, Saitama Children's Medical Center
Abstract
BACKGROUND: Pediatric patients diagnosed with hematologic and malignant diseases may present with severe anemia that develops gradually, resulting from chronic disruption of normal hematopoiesis. To prevent transfusion-associated circulatory overload (TACO), the standard practice in Japan is to slowly transfuse red blood cell concentrates (RBCs) based on the hemoglobin (Hb) level (g/dL) multiplied by the body weight (kg); however, no studies have verified the safety of this method.
METHODS: The study included pediatric patients with hematologic and malignant diseases, excluding nutritional anemia and hemolytic anemia, who underwent RBC infusion for severe gradually developing anemia with Hb levels <5 g/dL between January 2013 and December 2022 at Saitama Children's Medical Center. Their clinical course and whether they developed TACO were retrospectively reviewed based on their medical records.
RESULTS: The study population included 42 patients (20 boys), of which 26 had acute lymphocytic leukemia, 8 had acute myeloid leukemia, 4 had aplastic anemia, 3 had myelodysplastic syndrome, and 1 had chronic myeloid leukemia. The median age at diagnosis, Hb level, and BNP level were 5.2 years, 3.9 g/dL, and 31.0 pg/mL, respectively. The transfusion time, Hb elevation level before and after transfusion, rate of Hb elevation, and number of transfusions required to achieve Hb >5 g/dL were 6.9 h, 0.6 g/dL, 1.1 times, and 2 times (all median values), respectively. No patient experienced TACO.
CONCLUSION: Transfusion therapy with (Hb [g/dL]) mL/kg) RBCs administered slowly is safe for children with severe, gradually developing anemia.
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Case Report
Title
Isoflurane Inhalation Therapy for the Management of Extremely Refractory Status Epilepticus in Case of Acute Encephalitis with Refractory, Repetitive Partial Seizures
Author
Takahiro Ishida1) Norihisa Miyashita1) Kazunori Aoki1) Takuya Ueda2) Masahiro Nishiyama2) Azusa Maruyama2) and Hiroshi Kurosawa1)
1)Department of Pediatric Intensive Care, Hyogo Prefectural Kobe Children's Hospital
2)Department of Neurology, Hyogo Prefectural Kobe Children's Hospital
Abstract
Acute encephalitis with refractory, repetitive partial seizures (AERRPS) is a disease characterized by frequent, refractory focal seizures occurring after a fever, and is similar to febrile infection-related epilepsy syndrome (FIRES) and new-onset refractory status epilepticus (NORSE). The standard treatment for refractory status epilepticus (RSE) associated with AERRPS is the use of intravenous anesthetics such as barbiturates, aiming for burst suppression. However, many patients show treatment resistance. Furthermore, prolonged burst suppression carries the risk of cognitive decline. In particular, there is no established treatment for super-refractory status epilepticus (SRSE) in the acute phase of AERRPS that is resistant to conventional therapy. One potential treatment option is isoflurane inhalation therapy; however, data regarding its efficacy and safety remain limited. In this report, we describe a 14-year-old adolescent girl with acute-phase SRSE of AERRPS who was successfully treated with isoflurane inhalation therapy under mechanical ventilation. Seizure control was achieved without the development of intellectual or motor impairments and was able to return to regular school settings. Since many aspects of isoflurane inhalation therapy remain unclear, further case studies are necessary to evaluate its efficacy and safety.
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Case Report
Title
Aorto-pulmonary Collateral Arteries in Epilepsy with KCNT1 Mutations Treated with Arterial Embolization Using NBCA
Author
Yoshikazu Otsubo1) Sachika Yoshioka1) Saori Arisato2) Ryoko Honda3) Tatsuharu Sato4) and Atsushi Ishii5)
1)Department of Pediatrics, Sasebo City General Hospital
2)Department of Radiology, Sasebo City General Hospital
3)Department of Pediatrics, National Hospital Organization Nagasaki Medical Center
4)Department of Pediatrics, Nagasaki University Hospital
5)Department of Neurology, The University of Arizona
Abstract
Epilepsy of infancy with migrating focal seizures (EIMFS) is a type of intractable epilepsy that may be complicated by vascular malformations such as aorto-pulmonary collateral arteries (APCA). We report a case of a 10-year-old girl who was diagnosed with EIMFS at 2 months, and a pathological KCNT1 mutation was confirmed at 3 months. Vagus nerve stimulator implantation was performed at 1 year of age due to refractory epileptic seizures, followed by laryngotracheal separation at 4 years old for dysphagia, and gastrostomy at 5 years old, respectively. Thereafter, she has needed only oxygen administration at night. At 10 years old, she showed fresh bleeding from the trachea for two consecutive days and was diagnosed with pulmonary hemorrhage due to racemose hemangioma of the bronchial artery based on contrast CT findings. An angiogram revealed flexion and tortuosity in the right inferior branch as well as the left superior and inferior branches of the bronchial artery, along with a shunt into the pulmonary artery. As reliable embolization would be difficult with metal coils and gelatin sponges, embolization using n-butyl-2-cyanoacrylate (NBCA) was performed instead. Post-embolization angiography indicated that the shunt blood flow had disappeared.
Despite various reports of pulmonary hemorrhage due to systemic to the pulmonary collateral arteries in EIMFS cases with a KCNT1 mutation, to the best of our knowledge, there are no reports of embolization using NBCA. Lung lobectomy and ligation of the bronchial artery are performed for those situations; however, embolization using NBCA is less invasive. As a result, it might be a useful option for multifocal and high-recurrence bleeding in EIMFS cases.
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Case Report
Title
Acute Inguinal Pyogenic Lymphadenitis in Infants: Case Series and Essentials of Differentiation from Inguinal Hernia Incarceration
Author
Junki Tsuda1) Tsunehisa Nagamori1) Masayuki Sato1) Emi Ishibazawa1) Yuki Meya2) Daisuke Ishii2) Hisayuki Miyagi2) and Satoru Takahashi1)
1)Department of Pediatrics, Asahikawa Medical University
2)Division of Pediatric Surgery, Department of Surgery, Asahikawa Medical University
Abstract
Pyogenic lymphadenitis of the inguinal region (PLI) requires differentiation from incarcerated inguinal hernia, a medical emergency that can cause intestinal obstruction and organ necrosis if untreated. PLI is indicated for conservative antimicrobial therapy with observation to assess the necessity of surgical intervention such as puncture and drainage. Here we present four cases of PLI and review the clinical features for differentiation. In three cases, incarcerated inguinal hernia was initially suspected, and they were referred to our hospital, which includes a pediatric surgical unit. Patients aged 1-2 months presented with inguinal bulging, redness, tenderness, and fever in one case. Ultrasonography consistently showed lymph node swelling or abscess formation in the subcutaneous groin area. This imaging simplicity aided differentiation from inguinal hernia, which involves intra-abdominal organs in the mass. Concurrent umbilical inflammation and eczema in the three cases suggested a potential route of invasion and afforded an additional distinguishing feature. Early diagnosis upon observing inguinal swelling, redness, or tenderness is benefited by umbilical region examination and ultrasonography.
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Case Report
Title
Two Cases of Pneumatosis Cystoides Intestinalis in Children with Severe Motor and Intellectual Disabilities
Author
Toshiki Yanagisawa1) Miki Murayama1) Akihiko Kodama1) Chieko Takizawa1) Susumu Furukawa1) Kotaro Watanabe1) Chinatsu Kaneko1) Toshihiro Nomura1) Atsushi Harada2) Akito Sutani1) Yoshichika Maeda1) Tomonori Suzuki1) Tatsuya Yokoyama1) and Masato Nishioka1)
1)Department of Pediatrics, Kawaguchi Municipal Medical Center
2)Department of Pediatric Surgery, Kawaguchi Municipal Medical Center
Abstract
We report two cases of pneumatosis cystoides intestinalis (PCI) in children with severe motor and intellectual disabilities. A 14-year-old girl with a history of Shaken baby syndrome at age of 3 months, presented abdominal distension. Radiographic examinations revealed intramural pneumatosis extending from the cecum to the sigmoid colon. Meeting the diagnosis criteria of PCI, she underwent oxygen inhalation therapy, and the lesion disappeared approximately one month after diagnosis. A 9-year-old girl with cerebral palsy presented bloody stool, increase in gastric residual volume, and intermittent abdominal pain. Ultrasonography demonstrated the target sign and intussusception was diagnosed. Non-operative reduction of the intussusception was attempted with gastrografin enema but failed and operative reduction was selected. Manual reduction was successfully performed with Huntchinsons maneuver. After the reduction of the intussusception, submucosal masses composed of air-filled multiple cysts were observed from the ascending colon to the rectum. Meeting the diagnosis criteria of PCI, she underwent oxygen inhalation therapy, and the lesion disappeared.
We studied 10 cases of PCI in children with severe motor and intellectual disabilities. In addition to our cases, we searched the literature using PubMed and the Journal of Health Care and Society. We analyzed data on patient demographics, comorbidities, symptoms, concomitant events, and management. Seventy percent (7/10 cases) of cases required surgery.
It is difficult for children with severe motor and intellectual disabilities to describe their symptoms before severe concomitant events occur. Therefore, in addition to treating the underlying cause of PCI, we may start treatment such as oxygen inhalation, even if patients are asymptomatic.
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Case Report
Title
Hemodialysis Necessitated by Renal Failure Complicated by Long-term Parenteral Nutrition Due to Allied Disorders of Hirschsprung's Disease: A Case Report
Author
Kazuki Narita1) Eri Konishi1) Kanako Urakami1) Mikiko Koizumi1) Takashi Morita1) Masato Nishihara1) Yutaka Hino2) Kodo Tomida3) and Toshiko Yoshida3)
1)Department of Pediatrics, Yodogawa Christian Hospital
2)Vascular Access Hino Clinic
3)Department of Nephrology, Yodogawa Christian Hospital
Abstract
Renal failure is a complication of long-term parenteral nutrition in patients with intestinal failure, but is less commonly identified in childhood. In addition, depending on the underlying disease, its complications may cause difficulties in the selection and introduction of renal replacement therapy (RRT). Herein, we present a case of a patient with allied disorders of Hirschsprung's disease, in which intestinal failure and long-term parenteral nutrition from the neonatal period led to renal failure in adulthood, which was safely treated with hemodialysis.
A 36-year-old man with allied disorders of chronic idiopathic intestinal pseudo-obstruction with enterostomy and long-term parenteral nutrition had a history of repeated catheter-related bloodstream infections and dehydration. The renal function gradually declined, and end-stage kidney disease was diagnosed, requiring RRT. However, there were two concerns. The first was the treatment option. Neither peritoneal dialysis nor renal transplantation were considered appropriate because of the increased risk of peritoneal adhesions and infections, respectively. Therefore, hemodialysis was considered the only possible treatment option. The second concern was which site to create vascular access. Contrast-enhanced computed tomography revealed stenosis of the patient's subclavian veins, internal jugular veins, and inferior vena cava, with subsequent development of collateral vasculature, which led to the superior vena cava. We decided to create an arteriovenous graft shunting from the right femoral artery to the femoral vein. Even when patients are still young and have high enough kidney function, we should make efforts to preserve their kidney function in the long term.
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Brief Report
Title
Characteristic Changes of the Number of Patients with Kawasaki Disease before and after the COVID-19 Pandemic
Author
Ayumi Shikino Emi Sugita Takahiro Kodama Masaki Kanazawa and Masaru Terai
Department of Pediatrics, Chiba Kaihin Municipal Hospital
Abstract
We retrospectively studied the number of patients with Kawasaki disease, respiratory tract infections, and urinary tract infections at a single institution between 2017 and 2023. Hospitalizations for respiratory tract infections decreased in 2020 due to infection control measures but increased after coronavirus disease 2019 (COVID-19) was downgraded to a common infectious disease in May 2023. Hospitalizations for urinary tract infections remained unchanged. Some peaks in the number of Kawasaki disease cases were preceded by peaks in respiratory tract infections, supporting reports that respiratory infections may be involved in the onset of Kawasaki disease. The age distribution of Kawasaki disease in 2023 is similar to that before the COVID-19 pandemic, suggesting a return to pre-pandemic patterns despite the increase in cases following the relaxation of infection control measures.
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