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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
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Vol.120, No.7, July 2016
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Review
Title
Perioperative Issues after Bilateral Pulmonary Artery Banding in Patients with Hypoplastic Left Heart Syndrome
Author
Kensaku Motono1) Nao Hamamoto1) Daisuke Hazeki1) Masaki Osaki1) and Yasuo Ono2)
1)Cardiac Intensive Care Unit, Shizuoka Children's Hospital
2)Department of Cardiology, Shizuoka Children's Hospital
Abstract
Background: While performance of bilateral pulmonary artery banding (BPAB) as the first-stage operation for hypoplastic left heart syndrome (HLHS) has been increasing, few studies have investigated its perioperative management. We sought to review the issues surrounding BPAB for HLHS and the postoperative course.
Methods: We retrospectively reviewed the medical records of patients who underwent BPAB as the initial intervention for HLHS at Shizuoka Children's Hospital from June 2007 to October 2012.
Results: Twenty-four patients met the inclusion criteria. Twenty-one (88%) patients survived to undergo the second-stage Norwood operation plus the Blalock-Taussig shunt/right ventricle to pulmonary artery conduit procedure. Three (13%) patients died early after BPAB due to sepsis. Although all patients received prostaglandin E1 infusion until the Norwood operation to maintain ductal patency, patent ductus arteriosus (PDA) became restrictive in 8 (33%) patients. Among them, 3 patients underwent an emergency Norwood operation. Readjustment of pulmonary artery banding (re-PAB) was required in 5 patients.
Conclusion: Sepsis, PDA closure, and re-PAB were identified as the main issues after BPAB. Three low birth weight infants died in the early perioperative period due to sepsis. Because hemodynamic changes occur very rapidly and could be lethal, detailed evaluation of PDA, performance of BPAB, and timely availability of surgical intervention are mandatory.
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Original Article
Title
Correlation between Genotype and Clinical Features in Patients with the Mild Form of Hypophosphatasia
Author
Tomotaka Kono1) Shuichi Suzuki1) Ayako Ozawa1) Katsuya Aizu1) Toshimi Michigami2) Keiichi Ozono3) and Hiroshi Mochizuki1)
1)Division of Endocrinology and Metabolism, Saitama Children's Medical Center
2)Department of Bone and Mineral Research, Osaka Medical Center and Research Institute for Maternal and Child Health
3)Department of Pediatrics, Osaka University Graduate School of Medicine
Abstract
Hypophosphatasia (HPP) is a heritable disorder of bone caused by deactivating mutations in the tissue nonspecific alkaline phosphatase (TNSALP) gene. To identify the correlation between genotype and clinical features in patients with the mild form of HPP, 3 patients were recruited.
They were diagnosed with HPP at the age of 7 months to 3 years 7 months due to short stature, dental manifestations, or bone deformity from the fetal period. None of them required treatment, and two cases showed spontaneous postnatal improvement of bone deformity. All cases had compound heterozygous mutations of TNSALP gene which retained some residual activities at least in one allele.
In conclusion, TNSALP gene analysis including evaluating the residual enzyme activity may be useful not only for genetic diagnosis, but also for the prediction of the clinical course and prognosis of HPP.
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Original Article
Title
The Clinical Significance of Disseminated Intravascular Coagulation (DIC) Diagnostic Criteria in Pediatric Multiple Organ Dysfunction Syndrome
Author
Hiroyuki Nagafuchi
The Department of Critical Care Medicine, Kanagawa Children's Medical Center
Abstract
Objective: To review the clinical significance of two diagnosis criteria of acute disseminated intravascular coagulation (DIC) developed by the Japanese Association for Acute Medicine (JAAM) and Japanese Ministry of Health and Welfare (JMHW) in pediatric patients with multiple organ failure.
Method: JAAM score, JMHW score, thrombin-antithrombin complex (TAT), plasmin-alpha2 plasmin inhibitor complex (PIC), plasminogen activator inhibitor-1 (PAI-1), and the number of failing organs were retrospectively reviewed in 40 patients.
Results: Twenty-two patients met JAAM criteria (JAAM+) and 10 patients met JMHW criteria (JMHW+). Ten patients were in the JAAM+JMHW+group, 12 were in the JAAM+JMHW- group, 0 were in the JAAM- JMHW+group, and 18 were in the JAAM- JMHW- group. TAT, PAI-1, and TAT/PIC, which indicate DIC with decreased fibrinolytic activity, showed a higher tendency in the JAAM- JMHW- group, JAAM+JMHW- group, and JAAM+JMHW+group, in descending order. Compared with the JAAM- JMHW- group, the JAAM+JMHW+group had significantly higher TAT (P=0.02) and PAI-1 (P=0.02) levels and a greater number of failing organs (P< 0.01).
Conclusion: The results indicate that JAAM+suggests the coexistence of DIC with decreased fibrinolytic activity, and multiple organ failure in patients could be severe at the advanced JMHW+stage.
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Case Report
Title
A Case of Hepatoblastoma Diagnosed based on the Presence of Spontaneous Tumor Lysis Syndrome in a Patient with Trisomy 18
Author
Takashi Maeda1) Seizi Hayashi1) Maiko Matsusawa1) Toru Kato1) Noriko Nagai1) and Masahiro Hayakawa2)
1)Department of Pediatrics, Okazaki City Hospital
2)Maternity and Perinatal Care Center, Nagoya University Hospital
Abstract
We report a case of trisomy 18 with hepatoblastoma that was diagnosed based on the presence of spontaneous tumor lysis syndrome. A female infant was born at 29 weeks of gestation, with a birth weight of 618 g. Peripheral blood chromosomal analysis revealed 47,XX, +18, and an echocardiogram showed a large ventricular septal defect. Mechanical ventilation was required and the congenital heart defect was conservatively managed from birth. At 220 days of age, she became feverish and sallow. Hyperkalemia and ventricular arrhythmia occurred suddenly and calcium gluconate and glucose-insulin treatment were needed. At 222 days of age, subsequent examination revealed significant elevation of deviation enzymes, phosphorus, and uric acid. A large abdominal mass arising from the liver was detected by ultrasonography and computerized tomography scan, and her serum alpha fetoprotein was markedly elevated at 114,345 ng/mL. Therefore, the mass was diagnosed as hepatoblastoma, which caused spontaneous tumor lysis syndrome. As the number of infants with trisomy 18 surviving for longer periods of time is increasing in Japan, the occurrence of malignant tumors and the possibility of spontaneous tumor lysis syndrome should be kept in mind during follow-up.
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Case Report
Title
Two Neonates with Human Parechovirus Infection Accompanied with White Matter Injury on Brain MRI
Author
Hidetoshi Hagiwara1) Hiroshi Matsumoto1) Itsumi Hashimoto1) Motoshi Fujita1) Tomoaki Nomura1) Yoshiteru Tamura1) Akira Motegi1) Hajime Wakamatsu1) Hiroyuki Kawaguchi1) Yuta Aizawa2) Akihiko Saitoh2) and Shigeaki Nonoyama1)
1)Department of Pediatrics, National Defense Medical College
2)Department of Pediatrics, Niigata University
Abstract
Human parechovirus (HPeV) is known as a cause of sepsis-like syndrome with or without central nervous system involvement in neonates and young infants. We encountered two neonates with HPeV infection accompanied with white matter injury on brain MRI in the summer, 2014. Both patients had symptoms of fever, apnea, convulsion, poor feeding, and skin rash, which were compatible with neonatal sepsis. The laboratory study showed normal WBC counts and mild elevation of CRP, and no pleocytosis in the CSF. However, the elevation of serum AST, LDH, ferritin and sIL-2R were evident, and coagulopathy was also seen. These data suggested the presence of hypercytokinemia and hemophagocytic lymphohistiocytosis-like pathophysiology in both patients. Ventilatory support was needed; one was intubated and mechanically ventilated, and the other received nasal continuous positive airway pressure. Steroid therapy was not administered, and both patients are free from neurological sequelae. The diagnosis was made by the presence of HPeV genome in the patients' serum or cerebrospinal fluid examined by reverse transcription (RT)-PCR.
HPeV is a neurotropic virus and its infection can cause serious neurological complications and sequelae in neonates and young infants. Patients with severe HPeV infection who need admission in ICU with or without ventilatory support should be evaluated for white matter injury by brain MRI, and long-term monitoring for developmental delay will be considered in such patients. The hypercytokinemia in HPeV infection may be associated with the severity of the disease, which needs evaluation in many cases of HPeV infection.
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Case Report
Title
An Infant with Kawasaki Disease Followed by Femoral Osteomyelitis
Author
Mai Fukui Toshinori Nakamura Yoshifusa Abe Ayako Yagawa Takeshi Yamasaki Takeshi Mikawa Junya Iwasaki and Kazuo Itabashi
Department of Pediatrics, Showa University School of Medicine
Abstract
We report a 2-month-old boy with Kawasaki disease (KD) followed by femoral osteomyelitis. The patient required immunoglobulin (11 g/kg totally), prednisolone, and cyclosporin A (CyA) for improvement because he was refractory to the initial high-dose intravenous immunoglobulin. Thirty three days after the onset, the treatment was completed, but, on the next day, spontaneous motor activity decreased in the right lower limb, and heat and swelling occurred in the femur and knee. The patient looked unwell and irritable. Laboratory analysis showed elevated inflammatory markers suggesting increase in inflammatory responses. Imaging studies suggested osteomyelitis in the distal femur. Results of the blood culture were negative. Knee joint fluid, collected by aspiration, was serous and sterile. A total of six weeks of antibiotic administration and drainage of the fluid improved inflammation in the affected site, confirmed the clinical diagnosis and in imaging studies. Although osteomyelitis is rarely associated with KD, his osteomyelitis is considered related to several of the drugs, theefore osteomyelitis and other deep infections as well as relapse or recurrence of KD should be taken into account, especially in patients with KD who have fever or inflammatory response after the use of immunosuppressive agents for refractory KD.
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Case Report
Title
Pediatric Cardiopulmonary Arrests at Nursery and Junior High School
Author
Yuichiro Hirata1)2) SooYoung Lee1)2) Noriyuki Kaku1)2) Kenichiro Yamamura2) Haruhisa Baba1) Tomoo Kanna1)3) Hiroshi Sugimori1) and Toshiro Hara2)
1)Emergency and Critical Care Center, Kyushu University Hospital
2)Department of Pediatrics, Kyushu University Hospital
3)Committee of Consideration for BLS Training at School, Japanese Society for Emergency Medicine
Abstract
We report two cases of pediatric cardiopulmonary arrest that occurred at a nursery and junior high school, wherein teachers performed successful resuscitation that eventually resulted in a good clinical outcome. Case 1 was a 2-year-old boy who suddenly collapsed during playtime at a seasonal event program at a nursery. Because he was unresponsive, the teachers immediately initiated cardiopulmonary resuscitation (CPR) and called an ambulance. Twenty-six minutes after the collapse, the patient arrived at our clinic and an ECG showed both torsade de pointes and an alternating sinus rhythm. Eighty-five minutes after the collapse, spontaneous circulation was restored and he was treated with intensive care, including therapeutic hypothermia therapy. He was discharged from our hospital on day 41 without any aftereffects.
Case 2 was a 14-year-old girl who underwent ventricular septal defect closure at 5 months old. After the operation, the patient had complications of sick sinus syndrome and aortic valve stenosis, and thus, was prohibited from intense sports activity. At a later time, she collapsed after running 2,000 meters at her school. She was unresponsive and teachers called an ambulance while they began administering CPR according to the BLS. Fourteen minutes after the collapse, her spontaneous circulation returned. After treatment in an intensive care unit, she was transferred to another hospital on day 13 to continue the evaluation and treatment of cardiac diseases that had no neurological complications.
The desirable outcomes in both cases were a consequence of quickly performed CPR by the teachers. The importance of the BLS was reinforced through these cases and we hope that pediatric BLS can thus be disseminated to schools nationwide.
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Brief Report
Title
Investigation of Hospitalized Cases due to Rotavirus Gastroenteritis for Fiscal Years 2010-2014
Author
Tamaki Nakamura Satoko Kobayashi Taira Ariyoshi Fumiko Okazaki Noriko Hotta Satoko Itoh Hiroshi Tateishi and Masashi Uchida
Department of Pediatrics, Japan Community Healthcare Organization Tokuyama Central Hospital
Abstract
We admitted 191 children aged 1 month to 8 years of age to our pediatric department with rotavirus gastroenteritis between January 2010 and December 2014. The number of hospitalized cases under 1 year of age decreased significantly since rotavirus vaccines have been approved. No child under 1 year of age was admitted due to rotavirus gastroenteritis in 2014. Fifty-four children were admitted due to intussusception and only 1 patient suffered from rotavirus gastroenteritis. Rotavirus vaccines were not associated with intussusception and other severe adverse reactions.
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