Hasil untuk "Pediatrics"

Nanjun Zhang, Bowen Li, Yu Yan et al.

Abstract Background The occurrence of arrhythmias as a complication of Kawasaki disease (KD) is extremely rare. Moreover, previous literature showed a low incidence of arrhythmias during the acute phase of KD, and the majority occurred in the subacute and chronic phases. To date, we have found only 17 sporadically reported global cases in the available literature. Case presentation We present the first documented case of an infant with KD complicated with supraventricular tachycardia (Atrioventricular reentrant tachycardia) during the acute phase. The arrhythmia resolved promptly after the combination therapy of intravenous Immunoglobulin (IVIG) and steroids during the acute phase since the inflammation subsided. Additionally, we conducted a review and summary of cases involving KD-related arrhythmias. Conclusions KD rarely causes arrhythmias, which might be associated with myocarditis and myocardial ischemia attributed to scar formation and/or excessive inflammatory factors damaging the conduction system. Strengthening the early identification and management of complications in patients with KD and personalized follow-up strategies for high-risk children during the chronic phase can enhance patients’ prognosis.

Astha Poudel, Gaurav Goel, K.S. Reghu et al.

Chanisra Suebbook, Raiwada Sanguantrakul Teeracharoensub, Pongtong Puranitee et al.

Purpose To develop a culturally and locally validated and reliable questionnaire for clinical teacher evaluation containing constructs specific to the Thai resident learning context. Methods We followed seven steps for developing questionnaires for educational research. We generated a list of good clinical teacher attributes from a literature review and focus groups. The Delphi procedure was employed to identify the desirable characteristics for residents, involving three stakeholder groups. The content validity index (CVI) of each item was calculated. The average CVI across the items was greater than 0.8, indicating an acceptable level of reliability. Residents then underwent cognitive interviews before pilot testing of the questionnaire. Construct validity was examined using exploratory factor analysis. Reliability was measured using Cronbach’s alpha analysis. Results We identified 44 key clinical teacher characteristics through a literature review and focus groups. After two rounds of the Delphi procedure (35 panelists), 23 characteristics were selected. An initial 23-item questionnaire was developed with a high CVI score. A total of 216 completed questionnaires evaluating 36 clinical teachers were analyzed. Exploratory factor analysis yielded a two-factor model within a 20-item questionnaire. The clinical facilitator domain contained 14 items. The professional identity support domain included six items. Cronbach’s alpha of the model was 0.976. Conclusion A clinical teacher evaluation questionnaire for Thai residents was developed with robust validity and reliability. This validated tool not only allows systematic assessment and improvement of clinical teaching but also provides a replicable framework for developing culturally adapted teacher evaluation instruments in other settings.

Xuejie Gao, Xuejie Gao, Yuyun Chen et al.

ObjectiveThis study examines the association between serum vitamin D levels and the prevalence of metabolic dysfunction-associated fatty liver disease (MAFLD) in adolescents, along with potential modifying factors.MethodsData from 950 adolescents aged 12–18 years in the National Health and Nutrition Examination Survey (NHANES) 2017–2018 were analyzed. MAFLD was defined using hepatic steatosis and metabolic dysfunction criteria. Serum 25(OH)D levels were measured, and weighted logistic regression and restricted cubic spline models were applied to assess their association with MAFLD risk. Stratified analyses were also conducted.ResultsLower serum 25(OH)D levels were significantly associated with higher MAFLD risk (p < 0.001), showing a nonlinear dose-response relationship. Adolescents with 25(OH)D ≥ 75 nmol/L had a 57% lower risk of MAFLD compared to those with levels < 50 nmol/L. Stratified analysis indicated that the protective effect of vitamin D was more evident in individuals with higher retinol levels, though retinol alone was not significantly associated with MAFLD.ConclusionVitamin D deficiency is significantly associated with MAFLD in adolescents, with a nonlinear dose-response relationship modulated by retinol status. These findings underscore the potential role of vitamin D in MAFLD prevention and provide a basis for further prospective or intervention studies.

Marwa Mohamed Farag, Mohamed Alaa Eldin Hassan Thabet, Ahmed Adel Hassan El Beheiry et al.

Abstract Background To transfuse or not to transfuse premature infants” is still one of debatable issues in neonatal care that has not been completely solved. Recognizing hemodynamically significant (HS) anemia might be part of the solution. Our purposes were to investigate the hemodynamic effects of late onset anemia and red blood cells (RBCs) transfusion on premature neonates with gestational age 28–32 weeks and to tailor transfusion needs based on hemodynamic variables and Hb/HCT level. Methods In the current study, 36 anemic preterm infants with a gestational age of 28–32 weeks and a postnatal age of 3–9 weeks, all having a hematocrit (HCT) level below 30% and being candidates for red blood cell (RBC) transfusions, were compared to 36 non-anemic infants with HCT levels of 30% or higher in terms of hemodynamic parameters during their initial scans. Each anemic infant underwent a second scan 24 h after receiving RBC transfusions. The hemodynamic parameters assessed included left ventricular cardiac output (LVO) and the flow velocities in the renal (RA), anterior cerebral (ACA), and celiac (CA) arteries, measured using functional echocardiography and Doppler imaging. The hemodynamic changes were related to the presence of clinical signs in the anemic infants. Results LVO, stroke volume (SV), heart rate (HR), and ACA-peak systolic velocity (PSV) were significantly increased in anemia of prematurity and significantly decreased after RBCs transfusions. With an area under the ROC curve of 0.862, LVO displayed the highest diagnostic performance for HS-anemia of prematurity. Conclusions LVO, SV, HR, ACA-PSV, hemodynamic parameters can be used for diagnosing HS-anemia and can provide objective criteria for identifying patients in need of RBCs-transfusions. They also help in monitoring response of RBCs-transfusion in anemic preterm infants. Those cut off measures require validation by future studies.

Melissa Blum, Donato DeIngeniis, Daniela K. Shill et al.

Introduction: Rising ambient temperatures threaten vulnerable populations such as pregnant women, with urban populations bearing a greater risk due to the urban heat island effect. Here, we assessed the independent effects of trimester-specific warm season exposure during pregnancy and neighborhood heat vulnerability on maternal outcomes, including gestational diabetes, hypertensive disorders of pregnancy, genitourinary infections, and operative delivery. Methods: This retrospective study analyzed 819 participants from the Stress in Pregnancy Study (2009–2014), a longitudinal birth cohort study in New York City. Generalized linear models examined associations between trimester-specific warm season exposure, New York City Heat Vulnerability Index (ranging 1-5), and adverse maternal outcomes, adjusting for demographics, parity, and substance use. Results: First trimester warm season exposure was associated with increased odds of gestational hypertension (adjusted odds ratio [AOR] 4.50, 95%CI 1.17-17.27), preeclampsia (AOR 4.38, 95%CI 1.51-12.75), and genitourinary infection (AOR 2.27, 95%CI 1.14-4.51). Each unit increase in heat vulnerability index was associated with increased odds of preeclampsia (AOR 1.38, 95%CI 1.05-1.81) and genitourinary infection (AOR 1.32, 95%CI 1.11-1.57). Conclusions: Both early pregnancy warm weather exposure and neighborhood vulnerability independently increased the risk of adverse maternal complications. Our findings provide evidence in support of targeted heat mitigation strategies to limit heat exposure in at-risk communities as climate change progresses.

Paris Kantaras, Niki Mourouti, Theodora Mouratidou et al.

In total, 3274 adults (65.2% females) from six European countries were included in this cross-sectional analysis using data from the baseline assessment of the Feel4Diabetes study. Anthropometric, sociodemographic, dietary and behavioral data were assessed, and the existence of metabolic syndrome (MetS) was recorded. Beverage consumption patterns (BCPs) were derived via principal component analysis. Three BCPs were derived explaining 39.5% of the total variation. BCP1 was labeled as “Alcoholic beverage pattern”, which loaded heavily on high consumption of beer/cider, wine and other spirits; BCP2 was labeled as “High in sugars beverage pattern” that was mainly characterized by high consumption of soft drinks with sugar, juice containing sugar and low consumption of water; and BCP3 was labeled as “Healthy beverage pattern” that was mainly characterized by high consumption of water, tea, fruit juice freshly squeezed or prepacked without sugar and low consumption of soft drinks without sugar. After adjusting for various confounders, BCP2 was positively associated with elevated triglycerides (<i>p</i> = 0.001), elevated blood pressure (<i>p</i> = 0.001) elevated fasting glucose (<i>p</i> = 0.008) and the existence of MetS (<i>p</i> = 0.006), while BCP1 was inversely associated with reduced HDL-C (<i>p</i> = 0.005) and BCP3 was inversely associated with elevated blood pressure (<i>p</i> = 0.047). The establishment of policy actions as well as public health nutritional education can contribute to the promotion of a healthy beverage consumption.

V.P. Prytula, O.O. Kurtash, V.F. Rybalchenko et al.

The clinical course of Hirschsprung’s disease (HD) in newborns and infants depends on the extent of сolonic aganglionosis, the age of patient, the presence of associated malformations and complications. The aim - to study the peculiarities of the clinical course of HD in newborns and infants. Materials and methods. An analysis of the clinical course of HD in 483 in newborns and infants. There were 100 (20.71%) patients with the rectal, 192 (39.75%) with rectosigmoid, 150 (31.05%) with subtotal, and 41 (8.49%) with total form of HD. 98 (20.29%) patients were found to have associated malformations. Results. 64 (13.25%) patients with a rectal form, 72 (14.91%) - with rectosigmoid, 150 (31.05%) - with subtotal, and 41 (8.49%) with total form had an acute clinical course and subacute clinical course was observed in 36 (7.45%) patients with rectal and 120 (24.85%) with rectosigmoid forms of HD. 327 (67.70%) infants with HD were diagnosed with varying degrees of hypotrophy, 315 (65.22%) had HD-associated enterocolitis, 16 (3.31%) had toxic megacolon, and 241 (49.89%) patients had varying degrees of anemia. Conclusions. In the presence of associated malformations in newborns and infants, in 8.07% of cases, clinical symptoms may prevail over the classic signs of HD. The severity of the course and late diagnosis are the main reasons for the appearance of severe complications of HD in newborns and infants - hypotrophy (67.70%), HD-associated enterocolitis (65.22%), toxic megacolon (3.31%) and anemia (49.89%). The research was carried out in accordance with the principles of the Declaration of Helsinki. The research protocol was approved by the Local Ethics Committee of all institutions mentioned in the work. Informed consent of the children’s parents was obtained for the research. No conflict of interests was declared by the authors.

article Editorial

Департамент медицинской помощи детям, службы родовспоможения и общественного здоровья Минздрава России включил заболевание spina bifida в перечень редких (орфанных) заболеваний.

Keisuke Watanuki, Hiroshi Koga

IntroductionAnti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) myopathy is a recently recognized pathology, but appears less common in children and the characteristics of pediatric cases remain unclear.Case reportWe report a pediatric case of anti-HMGCR myopathy accompanied by skin rash. Motor function and serum creatine kinase level normalized after combinational treatment including early intravenous immunoglobulin, methotrexate, and corticosteroid.Literature reviewWe searched PubMed and identified reports with detailed clinical information of 33 pediatric patients &lt;18 years old with anti-HMGCR myopathy. Among these 33 patients and our own case, skin rash and maximum serum creatine kinase level &gt;5,000 IU/L were observed in 44% (15 patients) and 94% (32 patients), respectively. Skin rash was present in 15 of the 22 patients (68%) ≥7 years old and none of the 12 patients (0%) &lt;7 years old. Among the 15 patients with skin rash, 12 (80%) presented with erythematous rash.ConclusionErythematous skin rash may offer a clue to the diagnosis of anti-HMGCR myopathy in children with muscle weakness and serum creatine kinase level &gt;5,000 IU/L in the absence of other myositis-specific antibodies, particularly in patients ≥7 years old. Our results suggest the importance of early anti-HMGCR testing in pediatric patients with these manifestations.

Silvia Y. Vargas-Roldán, José L. Lezana-Fernández, Jorge F. Cerna-Cortés et al.

La fibrosis quística es una enfermedad hereditaria autosómica recesiva que se origina por mutaciones en el gen regulador de conductancia transmembranal de la fibrosis quística (CFTR, cystic fibrosis transmembrane conductance regulator). El CFTR es una proteína que transporta iones a través de la membrana de las células epiteliales pulmonares. La pérdida de su función conlleva la producción de un moco pegajoso y espeso, donde se pueden establecer y adaptar diversos patógenos bacterianos que contribuyen a la pérdida gradual de la función pulmonar. En este artículo de revisión se dará evidencia de los mecanismos moleculares que utilizan Pseudomonas aeruginosa y Burkholderia cenocepacia para sobrevivir y persistir en el ambiente pulmonar. Adicionalmente, se describirán las nuevas estrategias de terapia a base de moduladores de la función del CFTR.

Yizhou Huang, Han Dai, Gangyi Yang et al.

Abstract Background Congenital disorders of glycosylation (CDG) are a group of metabolic diseases with clinical and genetic heterogeneity, and CDG-IIg is one of the rare reported types of CDG. The aim of this study is to report the clinical manifestations and gene-phenotype characteristics of a rare case of CDG caused by a COG1 gene mutation and review literatures of CDG disease. Case presentation The patient was male, and the main clinical symptoms were developmental retardation, convulsion, strabismus, and hypoglycemia, which is rarely reported in CDG-IIg. We treated the patient with glucose infusion and he was recovered from hypoglycemia. Genetic analysis showed that the patient carried the heterozygous intron mutation c.1070 + 3A > G (splicing) in the coding region of the COG1 gene that was inherited from the mother, and the heterozygous mutation c.2492G > A (p. Arg831Gln) in exon 10 of the COG1 gene that was inherited from the father. The genes interacting with COG1 were mainly involved in the transport and composition of the Golgi. The clinical data and laboratory results from a patient diagnosed with CDG-IIg were analyzed, and the causative gene mutation was identified by high-throughput sequencing. The genes and signal pathways related to COG1 were analyzed by Gene Ontology and Kyoto Encyclopedia of Genes and Genomes enrichment analyses. Conclusions The c.2492G > A (p. Arg831Gln) mutation in exon 10 of the COG1 gene may be a potential pathogenetic variant for CDG-IIg. Because of the various manifestations of CDG in clinical practice, multidisciplinary collaboration is important for the diagnosis and treatment of this disease.

Niranjan Lal Jeswani, Muhammad Faisal Khilji, Syed Rizvi et al.

Objectives: We sought to study the epidemiology of drowning among children reported at Sultan Qaboos University Hospital in Oman. Methods: We conducted a retrospective study of the patients who presented to the emergency department with a history of drowning over 10 years from January 2008 to December 2017. Patients with children aged one to 18 years old were included in the study. The data including demographics, timing and location of drowning, season, adult supervision, swimming ability, medical risk factors, duration of submersion, on spot resuscitation, emergency medicine department assessment, and hospital management and outcome were collected from electronic hospital information system using a preformed proforma. The outcome was categorized into either full recovery, severe neurological injury, or brain death based on the pediatric cerebral performance category (PCPC). A good outcome represents a score of 1–3 points, and a PCPC of 4–6 points corresponds to a poor outcome. We calculated correlation for all variables with the outcome by using chi-square and Fisher’s exact tests. A p-value of < 0.050 is taken as significant value. Results: A total of 74 patients were included in the study; 54 (73.0%) were male, and 47 (63.5%) were aged < 6 years old. More than half (59.4%) of drownings happened in swimming pool, 21 (28.4%) children were unsupervised during the incident, and 39 (52.7%) required cardiopulmonary resuscitation (CPR). Out of all studied subjects, three (4.1%) were brain dead, and two (2.7%) developed severe neurological injury. On univariate analysis, the following variables were statistically significant (p < 0.050), predicting the poor outcome like lack of adult supervision, duration of submersion >10 minutes, asystole, Glasgow Coma Scale < 8, temperature < 35 oC, pH < 7, anion gap > 20, blood glucose > 10 mmol/L, abnormal chest X-ray findings, rewarming, CPR, intubation, inotropic support, and pediatric intensive care unit admission. Conclusions: Our study suggests that children, especially males under the age of six with no swimming ability, need strict supervision next to bodies of water. Furthermore, preventive measures might include raising community awareness about the risk factors of drowning, commencing public CPR lessons, and strict pool safety regulation by related authorities.

Carlos Abril-Pérez, Javier Sánchez-Arráez, Juncal Roca-Ginés et al.

Umut C. Kucuksezer, Cevdet Ozdemir, Lacin Cevhertas et al.

Allergen-specific immunotherapy (AIT) is the mainstay treatment for the cure of allergic disorders, with depicted efficacy and safety by several trials and meta-analysis. AIT impressively contributes to the management of allergic rhinitis, asthma and venom allergies. Food allergy is a new arena for AIT with promising results, especially via novel administration routes. Cell subsets with regulatory capacities are induced during AIT. IL-10 and transforming growth factor (TGF)-β are the main suppressor cytokines, in addition to surface molecules such as cytotoxic T-lymphocyte-associated antigen-4 (CTLA-4) and programmed cell death protein-1 (PD-1) within the micro milieu. Modified T- and B-cell responses and antibody isotypes, increased activity thresholds for eosinophils, basophils and mast cells and consequent limitation of inflammatory cascades altogether induce and maintain a state of sustained allergen-specific unresponsiveness. Established tolerance is reflected into the clinical perspectives as improvement of allergy symptoms together with reduced medication requirements and evolved disease severity. Long treatment durations, costs, reduced patient compliance and risk of severe, even life-threatening adverse reactions during treatment stand as major limiting factors for AIT. By development of purified non-allergenic, highly-immunogenic modified allergen extracts, and combinational usage of them with novel adjuvant molecules via new routes may shorten treatment durations and possibly reduce these drawbacks. AIT is the best model for custom-tailored therapy of allergic disorders. Better characterization of disease endotypes, definition of specific biomarkers for diagnosis and therapy follow-up, as well as precision medicine approaches may further contribute to success of AIT in management of allergic disorders.

Thereza Piloya, Beatrice Odongkara, Edward Maloba Were et al.

Abstract Background There’s abundant sunshine in the tropics but severe rickets is still observed. Nutritional rickets is associated with an increased risk of acute lower respiratory infections. Pneumonia is the leading cause of death in the under 5 -year old children with the highest burden in developing countries. Both Pneumonia and rickets are common in the developing countries and may affect clinical presentation and outcome. This study aimed to determine the prevalence and associated factors of nutritional rickets in children admitted with severe pneumonia. Methods This was a cross-sectional study of children aged 2–59 months presenting with severe pneumonia at an emergency unit. We enrolled 221 children between February and June 2012 after consent. A pre-coded questionnaire was used to collect data on socio-demographic, nutritional and past medical history. Physical exam was done for signs of rickets and anthropometric measurements. Serum calcium, phosphorus, and alkaline phosphatase (ALP) were assessed. Children with any physical signs of rickets or biochemical rickets (ALP > 400 IU); had a wrist x-ray done. Nutritional rickets was defined as the presence of radiological changes of cupping or fraying and/ or metaphyseal thickening. Severe pneumonia was defined using the WHO criteria. Statistical analysis was performed using the Stata 10 statistical package. P- value < 0.05 was significant. Results The prevalence of nutritional rickets among children with severe pneumonia is 9.5%. However, 14.5% had raised ALP (biochemical rickets). The factors independently associated with rickets was an elevated alkaline phosphatase; p-value < 0.001, or 32.95 95% CI (10.54–102.93). Other factors like breastfeeding, big family size, birth order were not significantly associated with rickets. Low serum calcium was detected in 22 (9.9%) of the 221 participants. Overall few children with rickets had typical clinical features of rickets on physical examination. Conclusion Rickets is a common problem in our setting despite ample sunshine. Clinicians should actively assess children for rickets in this setting and screen for rickets in those children at high risk even without clinical features.

Clara Gerosa, Eleonora Obinu, Daniela Fanni et al.

One of the most severe events occurring in critically ill patients admitted to a neonatal intensive care unit (NICU) center is represented by the multiple organ failure (MOF), a systemic inflammatory response leading to a progressive organ dysfunction and mortality in newborns. MOF may occur in newborns primarily affected by multiple single organ diseases, including respiratory distress syndrome neonatal sepsis with acute kidney injury, post-asphyxial hypoxic-ischemic encephalopathy and pandemic influenza A (H1N1) infection. In a previous article from our group, based on the histological examination of all organs at autopsy of newborns affected by MOF, all organs studied did not escape to be damaged, including thymus and pancreas normally not mentioned in the literature of MOF. The aim of this article is to review the most important pathological changes pathologists should look for in every case of MOF occurring in the perinatal period, with particular attention to systemic endothelial changes occurring in blood vessels in all organs and sytems. On the basis of our experience, matching data during the last phases of the clinicopathological diagnosis represents a useful method, much more productive as compared to the method based on giving pathological answers to the clinical questions prospected before autopsy. As for the pathological features observed in neonatal MOF, one of them deserves a particular attention: the vascular lesions, and in particular the multiple changes occurring during MOF development in endothelial cells, ending with the loss of the endothelial barrier, probably the most relevant histological lesion followed by the insurgence of interstitial edema and disseminated intravascular coagulation. Small vessels should be observed at high power, with particular attention to the size and shape of endothelial nuclei, in order to evidence endothelial swelling, probably the initial modification of the endothelial cells leading to their death. Finally, only the clinical pathological discussion may lead to a good diagnosis, correlating the morphological evidences with the clinical history and the sequence of clinical events that, at the best of our experience, are always different in a new case of MOF.   Proceedings of the International Course on Perinatal Pathology (part of the 10th International Workshop on Neonatology · October 22nd-25th, 2014) · Cagliari (Italy) · October 25th, 2014 · The role of the clinical pathological dialogue in problem solving Guest Editors: Gavino Faa, Vassilios Fanos, Peter Van Eyken

David S. Freedman

Elham Bozorgmehr, Tayebeh Malek Mohammadi, Abolghasem Hajizamani et al.

BACKGROUND: Many pediatric oral diseases are preventable if physicians recognize and encourage preventive care and refer patients to dentists whenever necessary. Parents usually visit pediatricians for routine care during the first few years of a child’s life. Therefore, pediatricians have can assist dental professionals by educating parents to maintain their children's oral health. The main objective of this study was to determine knowledge, attitude, and practices of pediatricians about the oral disease prevention. METHODS: A pilot questionnaire was completed by volunteer pediatricians and pediatric residents in Kerman, Iran. It comprised a series of questions including sociodemographic and practice characteristics, knowledge about the risk factors for oral diseases, attitude toward oral disease prevention, practicing preventive care for oral diseases, and information about oral diseases. RESULTS: Overall, 60 subjects participated in the study. Less than half of the respondents knew all the main risk factors of dental caries, gingivitis, and malocclusion. There was also a positive attitude that caries can be prevented (100%). Less than 10% of the participants prescribed dietary fluoride supplements for their patients. CONCLUSIONS: Although we found inadequate knowledge about oral and dental diseases among pediatricians, the majority of our subjects believed that they had an important responsibility in preventing oral diseases.