Pediatrics international : official journal of the Japan Pediatric Society最新文献_第10页

Case series of arginase 1 deficiency: Expanding the spectrum in hyperargininemia. 精氨酸酶1缺乏的病例系列:扩大了高精氨酸血症的频谱。

IF 1.4

Pediatrics international : official journal of the Japan Pediatric Society Pub Date : 2022-01-01 DOI: 10.1111/ped.14945

Sevil Dorum, Cengiz Havalı

{"title":"Case series of arginase 1 deficiency: Expanding the spectrum in hyperargininemia.","authors":"Sevil Dorum, Cengiz Havalı","doi":"10.1111/ped.14945","DOIUrl":"https://doi.org/10.1111/ped.14945","url":null,"abstract":"Background: Arginase-1 deficiency is a rare, autosomal recessively inherited disorder of the urea cycle. In this study, we describe the clinical and molecular details of six patients who were diagnosed with argininemia, and we describe two of the patients with hyperargininemia who carried two novel variations of the Arginase-1 gene.Methods: The clinical and demographic characteristics of the patients were retrospectively evaluated.Results: The ages of the six patients ranged from 1 day to 20 years, and each patient had consanguineous parents. Neuromotor retardation and spastic paraparesis were found in all patients except one, who was diagnosed prenatally. Hyperargininemia was present in all patients. Urinary orotic acid excretion was increased in four of the six patients. The diagnosis was confirmed by genetic analysis in all the patients. Elevated liver enzymes were detected in three patients and blood urea nitrogen levels were normal in each of the six patients.Conclusions: In this study, we describe the two patients with hyperargininemia who carried two novel variations of the ARG1 gene. Also, we present a patient with normal neurodevelopment who was diagnosed prenatally and treated at an early stage of the disease.","PeriodicalId":206308,"journal":{"name":"Pediatrics international : official journal of the Japan Pediatric Society","volume":" ","pages":"e14945"},"PeriodicalIF":1.4,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1111/ped.14945","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39271801","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 1

Invasive candidiasis in a neonatal intensive care unit in Fukuoka. 在福冈新生儿重症监护病房侵袭性念珠菌病。

IF 1.4

Pediatrics international : official journal of the Japan Pediatric Society Pub Date : 2022-01-01 DOI: 10.1111/ped.14949

Eiji Ohta, Takashi Setoue, Kazutoshi Ito, Tatsurou Kodera, Yasuhiro Onda, Hiroyasu Kawano, Toshikazu Niimi, Hiroya Kakura, Mariko Morii, Tatsuki Miyamoto, Chizuru Hashiguchi, Masatoshi Nakamura, Shinichi Hirose

{"title":"Invasive candidiasis in a neonatal intensive care unit in Fukuoka.","authors":"Eiji Ohta, Takashi Setoue, Kazutoshi Ito, Tatsurou Kodera, Yasuhiro Onda, Hiroyasu Kawano, Toshikazu Niimi, Hiroya Kakura, Mariko Morii, Tatsuki Miyamoto, Chizuru Hashiguchi, Masatoshi Nakamura, Shinichi Hirose","doi":"10.1111/ped.14949","DOIUrl":"https://doi.org/10.1111/ped.14949","url":null,"abstract":"Background: Invasive candidiasis (IC) is a leading cause of morbidity and mortality in preterm infants. The objective of this study was to determine the prevalence of IC infection in newborns in the neonatal intensive care unit (NICU) of a tertiary hospital in Japan, and to identify specific predisposing factors for IC.Methods: We retrospectively collected data on demographics, clinical characteristics, and outcomes of infants with IC, who were discharged from a tertiary NICU in Japan between January 2009 and December 2020. We compared predisposing factors associated with the occurrence of early-onset IC (EOIC < 72 h) and late-onset IC (LOIC ≥ 72 h) with those of early-onset and late-onset bacterial sepsis.Results: Between January 2009 and December 2020, 3,549 infants were admitted to the NICU, including 344 extremely-low birthweight (ELBW) infants. Eleven infants (including nine ELBW infants) had IC (incidence 0.31%), and the mortality rate of IC was 0%. Four (36%) infants had EOIC and seven (64%) had LOIC. All those with EOIC presented with skin lesions and 86% with LOIC had thrombocytopenia. Maternal vaginal Candida colonization was a more specific predisposing factor for EOIC, while gestational age <26 weeks, broad-spectrum antibiotic use, prior bacterial infection, prior gastrointestinal (GI) surgery, and GI diseases were more specific predisposing factors for LOIC.Conclusions: The findings suggest that maternal vaginal Candida colonization and skin lesions in ELBW infants may contribute to early recognition of EOIC. LOIC should be suspected if ELBW infants with several predisposing factors of LOIC have thrombocytopenia.","PeriodicalId":206308,"journal":{"name":"Pediatrics international : official journal of the Japan Pediatric Society","volume":" ","pages":"e14949"},"PeriodicalIF":1.4,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1111/ped.14949","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39309769","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 1

Circulating fibrocyte level in children with cystic fibrosis. 囊性纤维化患儿的循环纤维细胞水平。

IF 1.4

Pediatrics international : official journal of the Japan Pediatric Society Pub Date : 2022-01-01 DOI: 10.1111/ped.15058

Pınar Aslan Yaşar, Mehmet Köse, Serife Erdem, Melih Hangül, Zehra Filiz Karaman, Ahmet Eken

{"title":"Circulating fibrocyte level in children with cystic fibrosis.","authors":"Pınar Aslan Yaşar, Mehmet Köse, Serife Erdem, Melih Hangül, Zehra Filiz Karaman, Ahmet Eken","doi":"10.1111/ped.15058","DOIUrl":"https://doi.org/10.1111/ped.15058","url":null,"abstract":"Background: This study aimed to evaluate circulating fibrocyte levels in cystic fibrosis (CF) patients during stable and exacerbation periods of the condition.Methods: The study group consisted of 39 patients diagnosed with CF and 20 healthy controls. Individuals included in the study were divided into three groups: CF, CF exacerbated, and a healthy control group. Their circulating fibrocyte levels were compared. Findings from a pulmonary function test and high-resolution computed tomography of the lung were evaluated and compared.Results: The circulating fibrocyte count was found to be significantly higher in patients with CF compared with the exacerbated and control groups. No correlation was found between the forced expiratory volume in 1 s and forced vital capacity values in the pulmonary function test and the circulating fibrocyte count. The circulating fibrocyte count in patients (in the CF group) with positive findings in the high-resolution computed tomography was statistically significantly lower.Conclusions: The circulating fibrocyte level in the peripheral blood of the patients with CF was increased.","PeriodicalId":206308,"journal":{"name":"Pediatrics international : official journal of the Japan Pediatric Society","volume":" ","pages":"e15058"},"PeriodicalIF":1.4,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39733112","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Nurse-driven early rehabilitation protocol for critically ill children. 护士驱动的危重儿童早期康复方案。

IF 1.4

Pediatrics international : official journal of the Japan Pediatric Society Pub Date : 2022-01-01 DOI: 10.1111/ped.15048

Chanapai Chaiyakulsil, Panitnard Thippanate

{"title":"Nurse-driven early rehabilitation protocol for critically ill children.","authors":"Chanapai Chaiyakulsil, Panitnard Thippanate","doi":"10.1111/ped.15048","DOIUrl":"https://doi.org/10.1111/ped.15048","url":null,"abstract":"Background: Physical impairment is a major morbidity in children surviving intensive care. The main objective of this study was to evaluate the effectiveness of a nurse-driven protocol in the early mobilization of critically ill children in terms of reduction of motor dysfunction, pediatric intensive care unit stays, and ventilator days. The secondary objective was to evaluate safety, in terms of injury, dislodgement of medical devices, and cardiorespiratory instability attributable to the intervention.Methods: The early rehabilitation intervention was initiated in July 2020. This retrospective interrupted time-series study was divided into the pre-implementation phase (January-June 2020) and the post-implementation phase (July-December 2020). The motor function domain of the Functional Status Scale was used to define the motor dysfunction after pediatric intensive care unit discharge.Results: Twenty-five children were allocated in each group. The median age of the whole cohort was 11.5 months and approximately 58% of the population was male. The baseline characteristics of both groups were not statistically significant. There was a statistically significant reduction in motor dysfunction after protocol implementation (64.0% vs 36.0%; P = 0.044) with an absolute risk reduction of 28%. The number needed to treat was 3.6 children. There were no statistically significant differences in the median ventilator days, length of stay in the intensive care unit, and hospital length of stay. No complications were found.Conclusions: A nurse-driven protocol for the early mobilization of critically ill children was feasible and could be effective in reducing post-intensive care motor dysfunction.","PeriodicalId":206308,"journal":{"name":"Pediatrics international : official journal of the Japan Pediatric Society","volume":" ","pages":"e15048"},"PeriodicalIF":1.4,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39851017","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Pernicious anemia associated with Kabuki syndrome. 与歌舞伎综合症相关的恶性贫血。

IF 1.4

Pediatrics international : official journal of the Japan Pediatric Society Pub Date : 2022-01-01 DOI: 10.1111/ped.14960

Satoru Matsushima, Keisuke Kato, Ai Yoshimi, Koh-Ichiro Yoshiura, Masahiro Tsuchida

引用次数: 1

Conservative treatment for duodenal perforation after blunt trauma in a child. 儿童钝性创伤后十二指肠穿孔的保守治疗。

IF 1.4

Pediatrics international : official journal of the Japan Pediatric Society Pub Date : 2022-01-01 DOI: 10.1111/ped.14965

Reina Hoshi, Shuichiro Uehara, Takeshi Furuya, Hide Kaneda, Tsugumichi Koshinaga

引用次数: 2

Coagulation potential in pediatric patients with immunoglobulin A nephropathy. 儿童免疫球蛋白A肾病患者的凝血电位。

IF 1.4

Pediatrics international : official journal of the Japan Pediatric Society Pub Date : 2022-01-01 DOI: 10.1111/ped.15042

Takashi Omae, Tomoaki Ishikawa, Yuto Nakajima, Keiji Nogami

{"title":"Coagulation potential in pediatric patients with immunoglobulin A nephropathy.","authors":"Takashi Omae, Tomoaki Ishikawa, Yuto Nakajima, Keiji Nogami","doi":"10.1111/ped.15042","DOIUrl":"https://doi.org/10.1111/ped.15042","url":null,"abstract":"Background: Immunoglobulin A nephropathy (IgAN) is the most prevalent primary chronic glomerular disease in children. Understanding the changes in coagulability caused by IgAN is important for clarifying pathophysiology and choice of treatment. The coagulation potential in patients with IgAN remains to be investigated, however. We aimed to assess comprehensive coagulation potential in pediatric patients with IgAN and explore its relationship with pathological disease severity.Methods: Fourteen children with IgAN diagnosed by renal biopsy, who were admitted at Nara Medical University Hospital between 2015 and 2020, were analyzed. Rotational thromboelastometry was used to evaluate coagulation potential. Values of rotational thromboelastometry parameters in patients with IgAN were compared with those in control children.Results: In patients with IgAN (aged median 9.5 year), clotting time plus clot formation time (CT + CFT) was shortened (P = 0.003) and α angle was greater (P < 0.001) than those in controls, indicating a hypercoagulable state. The rate of mesangial hypercellularity of glomeruli correlated with CT + CFT, α, and maximum clot firmness (MCF) (rs = -0.79, 0.56, and 0.37). The rate of cellular/fibrocellular crescent of glomeruli correlated with CT + CFT, α, and MCF (rs = -0.41, 0.60, and 0.50). Patients with mesangial hypercellularity ≥80% of glomeruli showed reduced CT + CFT and increased α angle (P = 0.007 and 0.03). Patients with cellular/fibrocellular crescent ≥10% of glomeruli showed decreased CT + CFT and increased α angle (both P = 0.02).Conclusions: The hypercoagulable state in pediatric patients with IgAN may be associated with the pathological severity of their disease.","PeriodicalId":206308,"journal":{"name":"Pediatrics international : official journal of the Japan Pediatric Society","volume":" ","pages":"e15042"},"PeriodicalIF":1.4,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39559255","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 2

Septic arthritis of the pubic symphysis in a patient with SLE. 系统性红斑狼疮患者的耻骨联合脓毒性关节炎。

IF 1.4

Pediatrics international : official journal of the Japan Pediatric Society Pub Date : 2022-01-01 Epub Date: 2021-12-14 DOI: 10.1111/ped.14875

Masaki Shimizu, Asami Shimbo, Susumu Yamazaki, Yuko Segawa, Masaaki Mori

引用次数: 0

Two cases of Kleine-Levin syndrome with good responses to lithium treatment at low serum levels. 两例Kleine-Levin综合征患者在低血清水平下对锂治疗反应良好。

IF 1.4

Pediatrics international : official journal of the Japan Pediatric Society Pub Date : 2022-01-01 DOI: 10.1111/ped.15103

Tomoko Uchida, Daisuke Matsuzawa, Daisuke Sawada, Takashi Kanbayashi, Katsunori Fujii

引用次数: 0

Bifidobacterium breve during infancy attenuates mobility in low birthweight rats. 婴儿期短双歧杆菌降低低出生体重大鼠的活动能力。

IF 1.4

Pediatrics international : official journal of the Japan Pediatric Society Pub Date : 2022-01-01 DOI: 10.1111/ped.15209

Ayaka Itoh, Nao Tanaka, Sachiko Fukunaga, Akiko Nakano-Doi, Tomohiro Matsuyama, Takayuki Nakagomi, Masahiro Tsuji

{"title":"Bifidobacterium breve during infancy attenuates mobility in low birthweight rats.","authors":"Ayaka Itoh, Nao Tanaka, Sachiko Fukunaga, Akiko Nakano-Doi, Tomohiro Matsuyama, Takayuki Nakagomi, Masahiro Tsuji","doi":"10.1111/ped.15209","DOIUrl":"https://doi.org/10.1111/ped.15209","url":null,"abstract":"Background: Children with low birthweight (LBW) have a higher risk for developing attention-deficit/hyperactivity disorder, for which no prophylactic measure exists. The gut microbiota in infants with LBW is different from that in infants with normal birthweight and is associated with attention-deficit/hyperactivity disorder. Oral supplementation with Bifidobacterium has several health benefits, such as suppressing inflammation.Methods: We examined the effect of gavage supplementation with Bifidobacterium breve M-16V from postnatal days 1-21 in a rat model of intrauterine hypoperfusion.Results: The open-field test at 5 weeks of age (equivalent to human pubertal age) showed that rats in the LBW-vehicle group were marginally hyperactive compared with rats in the sham group, while rats in the LBW-B.breve group were significantly hypoactive compared with rats in the LBW-vehicle group. The gut microbiota in the LBW-vehicle group exhibited a profile significantly different from that in the sham group, whereas the gut microbiota in the LBW-B.breve group did not exhibit a significant difference from that in the sham group. Anatomical/histological evaluation at 6 weeks of age demonstrated that the brain weight and the cerebral areas on coronal sections were reduced in the LBW groups compared with the sham group. Probiotic supplementation did not ameliorate these morphological brain anomalies in LBW animals. The percentage of Iba-1+ cells in the brain was not different among the LBW-B.breve, LBW-vehicle, and sham groups.Conclusion: Bifidobacterium breve supplementation during early life is suggested to have the potential to help children with LBW attenuate hypermobility in adolescence.","PeriodicalId":206308,"journal":{"name":"Pediatrics international : official journal of the Japan Pediatric Society","volume":" ","pages":"e15209"},"PeriodicalIF":1.4,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40609594","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 2