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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
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Vol.125, No.5, May 2021
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Original Article
Title
Analysis of Children with Severe Motor and Intellectual Disabilities Who Required Consultations with Physicians during Short Stay and Occurrence of Incidents
Author
Kiyoshi Takemoto Masahisa Funato Yoshitaka Iijima Waka Hatano Hiroshi Wada Tamami Katayama Masumi Fujiwara and Atsuko Kashiwagi
Department of Pediatrics, Osaka Developmental Rehabilitation Center
Abstract
A retrospective study was conducted on cases of children with severe motor and intellectual disabilities who required consultations with physicians during short stay at our facility for disabled children, in addition to the occurrence of incidents of those cases using medical records. Subjects and methods: Assessments were made on a total of 326 cases whereby patients used short-stay services at our facility between April 2018 and March 2019, regarding reasons and details of consultation, details of incidents, and associations between consultation needs or occurrence of incidents and patients' backgrounds, including medical care. Results: Out of 326 cases, 56 (17%) had received some sort of medical treatment. The most common reasons were fever and tachycardia (11 cases), respiratory symptoms (11 cases), and eye or skin symptoms (11cases); these accounted for 59% of the entire cases. Incidents had occurred in 26 out of 326 cases (8%), and the great majority of cases (20 cases [77%]) were associated with trauma. The need for consultation increased significantly in patients with tube-feeding (adjusted odds ratio: 2.53, p value: 0.025). However, our model did not detect any significant association with sex, age (< 18 years old), first-time users, complication with epilepsy, or presence or absence of respiratory support. Our model also did not detect any association between occurrence of incidents and any of these factors. Conclusion: It is important to pay attention to traumatic incidents as well as changes in physical conditions during short stay in children with severe conditions.
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Original Article
Title
Effects of Positive Parenting Programs on Children's Psychosocial Problems
Author
Ichiro Fujita1) Machiko Shirayama2) Naoko Kajihara3) Kanoko Hashino4) and Kenichi Akama1)
1)Department of Child Development, Faculty of Human Relations, Fukuoka Jo Gakuin University
2)Furaha Osaka Psychological Development Office
3)Saga City Kouno After-school Children's Club
4)Hashino Child Clinic
Abstract
We examined the significance of parents' experiences with the Triple P-Positive Parenting Program on children's feelings and behaviors. Triple P is based on cognitive behavioral therapy and instructs parents on parenting skills such as setting a good example and focusing on the child's preferred behavior. The PSC (Pediatric Symptom Checklist) Japanese version health questionnaire was used to screen children for psychosocial problems. The number of children with a PSC score of 17 or above (with psychosocial problems) prior to group-work was 49.6% among 137 participants, which decreased to 35.0% in the ex-post survey. When the McNemar test was performed on the ratio of the number of people with a score of 17 or above, the posterior ratio was significantly lower compared to the prior ratio. Infants with a score of 17 or above decreased from 44.4% to 38.3% and school children from 57.1% to 30.4%. The mean value of PSC scores±standard deviation was significantly reduced from 17.24±9.06 to 14.64±8.56. Further analysis revealed significant improvements in anxiety/disorders of human relations and school truancy/indefinite complaints. It was found that children's psychosocial problems could be improved by parents taking the triple P for about 2 months.
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Original Article
Title
Major Inflammatory Markers Do Not Distinguish Urinary Tract Infections from Post-immunization Fever
Author
Kentaro Kogawa1)2) Yoshiki Kusama1)3) Kunio Muraki1)2) and Nobuaki Matsunaga3)
1)Department of Pediatrics, Fuji City General Hospital
2)Department of Pediatrics, The Jikei University School of Medicine
3)AMR Clinical Reference Center, Disease Control and Prevention Center, National Center for Global Health and Medicine Hospital
Abstract
Background: No study has been conducted to determine whether inflammatory markers are useful for distinguishing urinary tract infections (UTI) from post-immunization fever (PIF).
Methods: A retrospective, single-center, observational study was conducted. We included febrile infants younger than 6 months who presented to our hospital between September 2011, and August 2016. We analyzed whether clinically useful cut-off levels of white blood cell count (WBC) and C-reactive protein (CRP) can be determined by receiver operating characteristics (ROC). Continuous febrile states at 24 and 48 hours were also analyzed.
Results: We identified 33 cases of PIF and 36 cases of UTI. The area-under-ROC curve for WBC and CRP were 0.65 and 0.73. The best cutoff-score was 16,500/μL for WBC and 3.45 mg/dL for CRP. False positives for the best cut-off scores were 44% for WBC and 50% for CRP. Fever continued in 69.4% and 33.3% of UTI cases for 24 hours and 48 hours, respectively. In PIF cases, however, fever only continued in 13.8% and 0.0% of cases for 24 hours and 48 hours, respectively.
Conclusions: Since a clear cut-off point could not be established by ROC, neither WBC nor CRP were useful in distinguishing PIF and UTI.
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Case Report
Title
A Patient with Perinatal Lethal Hypophosphatasia Showing Good Prognosis for Respiratory Function Due to Early Enzyme Replacement Therapy
Author
Akihiro Takase1) Tadamune Kinjo1) Shuichi Suzuki2) Yushi Noguchi1) Takashi Shima1) Takeshi Kusuda1) Nobuhiko Kan1) Yasushi Takahata1) and Kenichi Miyako2)
1)Department of Neonatology, Fukuoka Children's Hospital
2)Department of Endocrinology and Metabolism, Fukuoka Children's Hospital
Abstract
Hypophosphatasia (HPP) is a rare bone disease caused by defects of the gene ALPL, which encodes the tissue nonspecific alkaline phosphatase. Previously, perinatal lethal form of HPP was fatal due to respiratory failure, but survival has improved with the advent of enzyme replacement therapy (ERT) in recent years. However, previous reports demonstrated that patients receiving ERT required tracheostomy during the course of treatment despite ventilator weaning. Here, we report a female perinatal lethal HPP infant treated with ERT initiated on postnatal day 4. She suffered from severe respiratory failure at birth, and was immediately intubated. Radiographic examination showed hypoplasia of the whole body bone, and serum alkaline phosphatase was extremely low at 6 IU/L. Genetic analysis identified a compound heterozygous mutation (c.1130C>T/c.1559delT) of the ALPL gene. The patient required high frequency oscillation ventilation and inhaled nitric oxide therapy early after birth. We administered ERT from day 4, and she was successfully weaned from mechanical ventilation without tracheostomy on day 74 and discharged on day 130. Her growth and development was normal at 1 year and 6 months of age. Early initiation of enzyme replacement therapy in perinatal lethal HPP infants might improve the prognosis of respiratory function.
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Case Report
Title
Infectious Pulmonary Artery Aneurysm Secondary to Infective Endocarditis Successfully Treated with Conservative Therapy
Author
Mai Yamada1)2) Hiroshi Ono1) Taiyu Hayashi1) Yasushi Misaki1) and Hitoshi Kato1)
1)Division of Cardiology, National Center for Child Health and Development
2)Department of Pediatrics and Adolescent Medicine, Tokyo Medical University
Abstract
A 5-year-old boy with 22q11.2 deletion syndrome, pulmonary atresia, underlying major aortopulmonary collateral arteries, status post Rastelli surgery, and unifocalization developed an infected pulmonary aneurysm 4 years after surgery due to infective endocarditis of the pulmonary valve. As the aneurysm was located in a low-pressure pulmonary circulation region and the diameter of the aneurysm was relatively small, intervention for the aneurysm was not necessary, and it was relieved by conservative treatment with only antibacterial agents. Later, however, an operation was performed due to severe pulmonary stenosis and reflux. There are reports that surgical intervention is necessary because infectious pulmonary aneurysms are associated with rupture or dissection and have a poor prognosis. However, in some cases, such as this one, patients can be followed up conservatively as long as enhanced CT is performed regularly and there is no expansion of the aneurysm.
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Case Report
Title
A Case of Severe Motor and Intellectual Disabilities Developing Intractable Pneumonia Due to Zinc Supplementation-induced Copper Deficiency and Neutropenia
Author
Ryosuke Kanamori1) Kosuke Miyawaki1) Atsushi Arai1) Kota Sasaki1) Akira Kumakura1) Masato Sato2) Daisuke Hata1) and Mitsutaka Shiota1)
1)Department of Pediatrics, Kitano Hospital, The Tazuke Kofukai Medical Research Institute
2)Department of Pediatric Surgery, Kitano Hospital, The Tazuke Kofukai Medical Research Institute
Abstract
Copper deficiency can present as anemia, neutropenia and myelopathy. Although it is rare, case of copper deficiency due to inappropriate nutrition therapy including excessive zinc administration have been reported.
A 21-year-old man with severe motor and intellectual disabilities received zinc supplementation for 8 months. Ten days before admission, he developed a fever. The examination revealed anemia, neutropenia and pneumonia, and he was admitted to our hospital for further examination and treatment.
The blood examination after admission revealed low levels of copper, and he was diagnosed with copper deficiency caused by excessive zinc administration. Cessation of zinc supplementation and initiation of copper supplementation normalized the copper level on day 37 of admission. However, his pneumonia did not improve for a long time and he required respiratory support even after the pneumonia improved. After aggressive copper replacement therapy, he was given 18 mg of zinc and 1.3 mg of copper daily. One year after discharge, his serum zinc and copper levels remain normal and he has not suffered from pneumonia.
Zinc-induced copper deficiency is a preventable disease, but once it occurs, it can create great disadvantage for patients. All medical staff involved with patients with severe motor and intellectual disabilities should understand this disease.
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Case Report
Title
Acute Rheumatic Fever Initially Presenting with Lumbar Facet Arthritis
Author
Takashi Shinkai Chihiro Iitsuka Hiroomi Nagata Satoshi Ikehara and Yuuji Shinzato
Department of Pediatrics, Chubu Tokushukai Hospital
Abstract
Acute rheumatic fever (ARF) is a systemic non-suppurative inflammatory disease secondary to pharyngitis caused by group A β-hemolytic streptococci. The incidence of ARF has dramatically decreased in developed countries due to the spread of antibacterial drugs, and there are few opportunities to encounter patients with ARF in daily medical practice. Here, we report a case of ARF in which the patient presented with lumbar facet arthritis without an episode of antecedent infection.
The patient was a previously healthy 8-year-old boy. He visited our emergency department with fever and low back pain. Lumbar spine MRI revealed a diagnosis of right lumbar facet arthritis and antibiotic therapy was started, but the symptoms did not improve, and pain appeared in the right ankle and left knee joint after hospitalization. When Ibuprofen was administered orally for polyarthritis, the pains improved promptly, but erythema marginatum appeared on the abdomen and extremities 6 days after the illness. Furthermore, an increase in the antibody titer of group A streptococcus and a prolongation of PR time on electrocardiography were confirmed, and we diagnosed ARF because revised Jones criteria were met.
The spine is not affected in a typical case of ARF, and there have been no reports of ARF with lumbar facet arthritis in Japan. In cases of arthritis of unidentified cause, it is necessary to keep track of progress with ARF in mind.
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Case Report
Title
A Case of Stevens-Johnson Syndrome (SJS) and Vanishing Bile Duct Syndrome (VBDS) Associated with Tranilast
Author
Madoka Fujii1) Tomoyuki Tsunoda2) Chiaki Takahashi3) Sayaka Fukuda1) Soya Kobayashi2) Mari Iwamoto1) and Ayano Inui2)
1)Department of Pediatrics, Saiseikai Yokohamashi Tobu Hospital
2)Department of Pediatric Hepatology and Gastroenterology, Saiseikai Yokohamashi Tobu Hospital
3)Department of Dermatology, Saiseikai Yokohamashi Tobu Hospital
Abstract
Tranilast is a medication used to prevent post-surgical keloid formation and is also an anti-allergic drug. In the pediatric field, it is commonly administered after cleft lip repair surgery. To date, there has been no report regarding serious side effects of tranilast among children. Here, we report a case of an infant with Stevens-Johnson syndrome (SJS) and vanishing bile duct syndrome (VBDS) caused by tranilast. The patient was given tranilast following a cleft lip repair operation, and on the 11th day, he developed fever, systemic erythema, and jaundice with cholestasis. Histopathological features of the skin and liver were compatible with SJS and Drug-Induced Liver Injury (DILI) accompanied by VBDS, and drug-induced SJS and VBDS were thus suspected. Administration of tranilast was discontinued and the patient was treated with steroids and ursodeoxycholic acid. The skin erythema and cholestasis gradually diminished and liver function normalized 7 months after onset. Although a drug lymphocyte stimulation test for tranilast was negative, tranilast was considered to have caused both SJS and VBDS given the clinical course of the patient. This is the first report of severe side effects caused by tranilast. Clinicians should pay careful attention to side effects when administering tranilast.
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Case Report
Title
Acute Laryngeal Edema Caused by Accidental Ingestion of Arisaema serratum Fruit Requiring Endotracheal Intubation for Airway Management
Author
Yusuke Otani1) Yoko Takagi1) Yasuko Kobayashi1) Mariko Takei1) Yuka Ikeuchi1) Satoshi Yamada1) Yutaka Nishida1) Takashi Ishige1) Shunpei Suda1) Miyuki Kohno2) Hirokazu Arakawa1) and Takumi Takizawa1)
1)Department of Pediatrics, Gunma University Graduate School of Medicine
2)Department of Pediatrics, JCHO Gunma Chuo Hospital
Abstract
Acute laryngeal edema may cause life-threatening airway obstruction that eventually requires emergency endotracheal intubation or tracheotomy. We present the case of a 2-year-old boy with acute laryngeal edema and airway obstruction caused by accidental ingestion of Arisaema serratum fruit. He suddenly developed intensive oropharyngeal pain and swelling in his lower lip followed by a persistent vomiting reflex. Although he showed no respiratory symptoms upon presenting to the emergency room, he developed severe laryngeal edema 4 hours after ingestion and underwent endotracheal intubation under elective tracheotomy standby. The edema of the lip and larynx spontaneously regressed over 2 days, and erosion appeared on his lower lip on day 2. He was extubated on day 5 and discharged on day 9 without complications. To the best of our knowledge, this is the first pediatric case of a patient requiring endotracheal intubation for airway management for acute laryngeal edema caused by accidental Arisaema species ingestion to be reported. The toxicity of Arisaema species is due to insoluble, needle-like calcium oxalate crystals, which can physically damage the mucosa once ingested. It is reported that 60% of poisoning cases by natural plant toxins are in patients younger than 5 years old. Therefore, it is important to consider poisoning by natural toxins as one possible cause of pediatric acute laryngeal edema.
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