World journal of clinical pediatrics最新文献

{"title":"Gastrointestinal and nutritional care in pediatric neuromuscular disorders.","authors":"Valeria Dipasquale,&nbsp;Rossella Morello,&nbsp;Claudio Romano","doi":"10.5409/wjcp.v12.i4.197","DOIUrl":"10.5409/wjcp.v12.i4.197","url":null,"abstract":"<p><p>Neuromuscular diseases (NMDs) affect the development and growth of the neuromuscular system in children. The pathology can occur anywhere along the neuromuscular pathway, from the brain to the nerves to the muscle fibers. These diseases have a profound impact on the quality of life not only of children but also of their families. The predominant manifestation in NMDs is hypotonia, which leads to muscle weakness and fatigue, reduced mobility, and decreased physical performance. However, multiple organ systems can be affected, with resulting orthopedic, cardiac, infectious, respiratory, and nutritional problems. Children with NMD present an increased risk for several dietary and feeding difficulties because of their neuromuscular diagnosis, presentation, and severity. These problems include chronic gastrointestinal issues (constipation, dysphagia, gastroesophageal reflux, and diarrhea), dysphagia, malnutrition, and body composition alterations. As a result, compared to the overall pediatric population, infants and children with NMD are more likely to be malnourished, ranging from failure to thrive to overweight or obesity. Disease-specific guidelines vary in level of detail and recommendations for dietary management. Overall, nutritional data available are sparse, with the exception of Duchenne muscular dystrophy, spinal muscular atrophy, and congenital muscular dystrophy. The purpose of this review is to describe the spectrum of nutritional challenges in children with NMD and to summarize the main dietary and gastrointestinal recommendations for each neuromuscular disorder to provide guidance for daily clinical practice.</p>","PeriodicalId":75338,"journal":{"name":"World journal of clinical pediatrics","volume":"12 4","pages":"197-204"},"PeriodicalIF":0.0,"publicationDate":"2023-09-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/20/50/WJCP-12-197.PMC10518748.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41123989","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Safety and efficacy of intravitreal anti vascular endothelial growth factor for severe posterior retinopathy of prematurity with flat fibrovascular proliferation.","authors":"Puja Maitra,&nbsp;Subramaniam Prema,&nbsp;Venkatapathy Narendran,&nbsp;Parag K Shah","doi":"10.5409/wjcp.v12.i4.220","DOIUrl":"https://doi.org/10.5409/wjcp.v12.i4.220","url":null,"abstract":"<p><strong>Background: </strong>Intravitreal anti-vascular endothelial growth factor (IVA) injection is known to cause contraction of fibrovascular proliferation (FVP), when present in severe retinopathy of prematurity (ROP).</p><p><strong>Aim: </strong>To assess the structural outcomes of IVA injection in the treatment of severe posterior ROP with significant FVP.</p><p><strong>Methods: </strong>It was a retrospective study in which 36 eyes of 18 preterm babies who developed > 4 clock hours of FVP in zone I or posterior zone II, were treated with either intravitreal 0.625 mg bevacizumab or intravitreal 0.2 mg of ranibizumab. Favorable structural outcome included resolution of plus disease and FVP without the development of tractional retinal detachment. Secondary outcome measure included either full retinal maturation at follow-up or development of recurrent disease requiring additional treatment. Adverse outcomes included progression to retinal detachment.</p><p><strong>Results: </strong>The mean gestational age of the 18 preterm babies was 30 wk (range 27-36), and mean birth weight was 1319 g (range 650-1980 g). Mean post-menstrual age (PMA) at the time of primary treatment was 35.5 wk (range 31-41 wk). All eyes showed regression of plus disease and FVP. 5 eyes of 3 babies showed reactivation of disease and were treated with repeat IVA (<i>n</i> = 2 eyes) or peripheral laser photocoagulation (<i>n</i> = 3 eyes) respectively. 16 out of 36 (44%) reached retinal vascular maturation at final follow up at 5 years.</p><p><strong>Conclusion: </strong>There was good resolution of severe posterior ROP with FVP with IVA, with retinal maturity of 44% at 5 year follow-up and a reactivation rate of 13.8%. When the IVA injection is given prior to 37 wk PMA, while disease is in phase 2, it is less likely to cause contracture of pre-existing FVP.</p>","PeriodicalId":75338,"journal":{"name":"World journal of clinical pediatrics","volume":"12 4","pages":"220-229"},"PeriodicalIF":0.0,"publicationDate":"2023-09-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/e2/48/WJCP-12-220.PMC10518743.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41163814","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Use of endolumenal functional lumen imaging probe in investigating paediatric gastrointestinal motility disorders.","authors":"Emily White,&nbsp;Mohamed Mutalib","doi":"10.5409/wjcp.v12.i4.162","DOIUrl":"10.5409/wjcp.v12.i4.162","url":null,"abstract":"<p><p>Investigating gastrointestinal (GI) motility disorders relies on diagnostic tools to assess muscular contractions, peristalsis propagation and the integrity and coordination of various sphincters. Manometries are the gold standard to study the GI motor function but it is increasingly acknowledged that manometries do not provide a complete picture in relation to sphincters competencies and muscle fibrosis. Endolumenal functional lumen imaging probe (EndoFLIP) an emerging technology, uses impedance planimetry to measure hollow organs cross sectional area, distensibility and compliance. It has been successfully used as a complementary tool in the assessment of the upper and lower oesophageal sphincters, oesophageal body, the pylorus and the anal canal. In this article, we aim to review the uses of EndoFLIP as a tool to investigate GI motility disorders with a special focus on paediatric practice. The majority of EndoFLIP studies were conducted in adult patients but the uptake of the technology in paediatrics is increasing. EndoFLIP can provide a useful complementary data to the existing GI motility investigation in both children and adults.</p>","PeriodicalId":75338,"journal":{"name":"World journal of clinical pediatrics","volume":"12 4","pages":"162-170"},"PeriodicalIF":0.0,"publicationDate":"2023-09-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/f0/68/WJCP-12-162.PMC10518749.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41175539","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Transient hyperphosphatasemia in a toddler with COVID-19 infection: A case report and literature review.","authors":"Pemiga Sukhupanyarak,&nbsp;Voraluck Phatarakijnirund","doi":"10.5409/wjcp.v12.i4.237","DOIUrl":"10.5409/wjcp.v12.i4.237","url":null,"abstract":"<p><strong>Background: </strong>Transient hyperphosphatasemia (TH) is a condition characterized by elevated serum alkaline phosphatase (ALP) in the clinical setting with no evidence of bone or liver disease among children under the age of 5. Typically, it will resolve spontaneously in a few months in the majority of cases. TH has been found to be associated with viral infections. Two cases of TH associated with coronavirus disease 2019 (COVID-19) infection in toddlers have been previously reported.</p><p><strong>Case summary: </strong>A previously healthy 2-year-old boy presented with fever and positive real-time polymerase chain reaction for COVID-19. Prior to his illness, the patient had been in close contact with his grandfather, who later developed COVID-19. The physical examination on admission was unremarkable. He remained asymptomatic throughout 7 d of hospitalization. On the 5^th day of his illness, blood tests showed markedly elevated serum ALP (4178 U/L). Results from the simultaneous testing of the remaining liver profiles and metabolic bone panels were normal. Two months after discharge from the hospital, the patient continued to thrive well. The skeletal surveys revealed no significant abnormalities. The serum ALP declined into the normal range adjusted for his age. This evidence is consistent with the diagnosis of TH.</p><p><strong>Conclusion: </strong>TH can occur in COVID-19-infected toddlers. Serial measurements of ALP levels have been shown to gradually decline into the normal range within a few months. Therefore, being aware of this transient abnormality will help clinicians to avoid additional unnecessary investigations.</p>","PeriodicalId":75338,"journal":{"name":"World journal of clinical pediatrics","volume":"12 4","pages":"237-243"},"PeriodicalIF":0.0,"publicationDate":"2023-09-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/fd/ac/WJCP-12-237.PMC10518746.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41175651","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Radiation dose analysis of computed tomography coronary angiography in Children with Kawasaki disease.","authors":"Mahesh Chandra Bhatt,&nbsp;Manphool Singhal,&nbsp;Rakesh Kumar Pilania,&nbsp;Subhash Chand Bansal,&nbsp;Niranjan Khandelwal,&nbsp;Pankaj Gupta,&nbsp;Surjit Singh","doi":"10.5409/wjcp.v12.i4.230","DOIUrl":"10.5409/wjcp.v12.i4.230","url":null,"abstract":"<p><strong>Background: </strong>There is evolving role of computed tomography coronary angiography (CTCA) in non-invasive evaluation of coronary artery abnormalities in children with Kawasaki disease (KD). Despite this, there is lack of data on radiation dose in this group of children undergoing CTCA.</p><p><strong>Aim: </strong>To audit the radiation dose of CTCA in children with KD.</p><p><strong>Methods: </strong>Study (December 2013-February 2018) was performed on dual source CT scanner using adaptive prospective electrocardiography-triggering. The dose length product (DLP in milligray-centimeters-mGy.cm) was recorded. Effective radiation dose (millisieverts-mSv) was calculated by applying appropriate age adjusted conversion factors as per recommendations of International Commission on Radiological Protection. Radiation dose was compared across the groups (0-1, 1-5, 5-10, and > 10 years).</p><p><strong>Results: </strong>Eighty-five children (71 boys, 14 girls) with KD underwent CTCA. The median age was 5 years (range, 2 mo-11 years). Median DLP and effective dose was 21 mGy.cm, interquartile ranges (IQR) = 15 (13, 28) and 0.83 mSv, IQR = 0.33 (0.68, 1.01) respectively. Mean DLP increased significantly across the age groups. Mean effective dose in infants (0.63 mSv) was significantly lower than the other age groups (1-5 years 0.85 mSv, 5-10 years 1.04 mSv, and > 10 years 1.38 mSv) (<i>P</i> < 0.05). There was no significant difference in the effective dose between the other groups of children. All the CTCA studies were of diagnostic quality. No child required a repeat examination.</p><p><strong>Conclusion: </strong>CTCA is feasible with submillisievert radiation dose in most children with KD. Thus, CTCA has the potential to be an important adjunctive imaging modality in children with KD.</p>","PeriodicalId":75338,"journal":{"name":"World journal of clinical pediatrics","volume":"12 4","pages":"230-236"},"PeriodicalIF":0.0,"publicationDate":"2023-09-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/71/a5/WJCP-12-230.PMC10518747.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41172296","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Evaluation of children and adults with post-COVID-19 persistent smell, taste and trigeminal chemosensory disorders: A hospital based study.","authors":"Sherifa Ahmed Hamed,&nbsp;Eman Bahaa Kamal-Eldeen,&nbsp;Mohamed Azzam Abdel-Razek Ahmed","doi":"10.5409/wjcp.v12.i3.133","DOIUrl":"https://doi.org/10.5409/wjcp.v12.i3.133","url":null,"abstract":"<p><strong>Background: </strong>Smell disorders are the most frequent persistent coronavirus disease 2019 (COVID-19) complications.</p><p><strong>Aim: </strong>To describe the patterns and characteristics of persistent smell and taste disorders in Egyptian patients.</p><p><strong>Methods: </strong>Assessment was done to 185 patients (adults = 150, age: 31.41 ± 8.63 years; children = 35; age: 15.66 ± 1.63 years). Otolaryngology and neuropsychiatric evaluations were done. Measurements included: A clinical questionnaire (for smell and taste); sniffin' odor, taste and flavor identification tests and the Questionnaire of Olfactory Disorders-Negative Statements (sQOD-NS).</p><p><strong>Results: </strong>Duration of disorders was 11.53 ± 3.97 ms (6-24 ms). Parosmia (<i>n</i> = 119; 64.32%) was developed months after anosmia (3.05 ± 1.87 ms). Objective testing showed anosmia in all, ageusia and flavor loss in 20% (<i>n</i> = 37) and loss of nasal and oral trigeminal sensations in 18% (<i>n</i> = 33) and 20% (<i>n</i> = 37), respectively. Patients had low scoring of sQOD-NS (11.41 ± 3.66). There were no specific differences in other demographics and clinical variables which could distinguish post-COVID-19 smell and taste disorders in children from adults.</p><p><strong>Conclusion: </strong>The course of small and taste disorders are supportive of the nasal and oral neuronal compromises. Post-COVID-19 taste and trigeminal disorders were less frequent compared to smell disorders. Post-COVID-19 flavor disorders were solely dependent on taste and not smell disorders. There were no demographics, clinical variables at onset or specific profile of these disorders in children compared to adults.</p>","PeriodicalId":75338,"journal":{"name":"World journal of clinical pediatrics","volume":"12 3","pages":"133-150"},"PeriodicalIF":0.0,"publicationDate":"2023-06-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/04/75/WJCP-12-133.PMC10278074.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9709213","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Prevalence of gastroesophageal reflux disease in children with extraesophageal manifestations using combined-video, multichannel intraluminal impedance-pH study.","authors":"Sutha Eiamkulbutr,&nbsp;Termpong Dumrisilp,&nbsp;Anapat Sanpavat,&nbsp;Palittiya Sintusek","doi":"10.5409/wjcp.v12.i3.151","DOIUrl":"https://doi.org/10.5409/wjcp.v12.i3.151","url":null,"abstract":"<p><strong>Background: </strong>Gastroesophageal reflux disease (GERD) might be either a cause or comorbidity in children with extraesophageal problems especially as refractory respiratory symptoms, without any best methods or criterion for diagnosing it in children.</p><p><strong>Aim: </strong>To evaluate the prevalence of extraesophageal GERD using conventional and combined-video, multichannel intraluminal impedance-pH (MII-pH), and to propose novel diagnostic parameters.</p><p><strong>Methods: </strong>The study was conducted among children suspected of extraesophageal GERD at King Chulalongkorn Memorial Hospital between 2019 and 2022. The children underwent conventional and/or combined-video MII-pH. The potential parameters were assessed and receiver operating characteristic was used for the significant parameters.</p><p><strong>Results: </strong>Of 51 patients (52.9% males), aged 2.24 years were recruited. The common problems were cough, recurrent pneumonia, and hypersecretion. Using MII-pH, 35.3% of the children were diagnosed with GERD by reflux index (31.4%), total reflux events (3.9%), and symptom indices (9.8%) with higher symptom recorded in the GERD group (94 <i>vs</i> 171, <i>P</i> = 0.033). In the video monitoring group (<i>n</i> = 17), there were more symptoms recorded (120 <i>vs</i> 220, <i>P</i> = 0.062) and more GERD (11.8% <i>vs</i> 29.4%, <i>P</i> = 0.398) by symptom indices<i>.</i> Longest reflux time and mean nocturnal baseline impedance were significant parameters for diagnosis with receiver operating characteristic areas of 0.907 (<i>P</i> = 0.001) and 0.726 (<i>P</i> = 0.014).</p><p><strong>Conclusion: </strong>The prevalence of extraesophageal GERD in children was not high as expected. The diagnostic yield of symptom indices increased using video monitoring. Long reflux time and mean nocturnal baseline impedance are novel parameters that should be integrated into the GERD diagnostic criteria in children.</p>","PeriodicalId":75338,"journal":{"name":"World journal of clinical pediatrics","volume":"12 3","pages":"151-161"},"PeriodicalIF":0.0,"publicationDate":"2023-06-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/3b/5f/WJCP-12-151.PMC10278077.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10086649","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"COVID-19-induced liver injury in infants, children, and adolescents.","authors":"Rana Bitar,&nbsp;Ahmed A Elghoudi,&nbsp;David Rawat,&nbsp;Amer Azaz,&nbsp;Mohamad Miqdady,&nbsp;Hassib Narchi","doi":"10.5409/wjcp.v12.i3.57","DOIUrl":"https://doi.org/10.5409/wjcp.v12.i3.57","url":null,"abstract":"<p><p>Coronavirus disease 2019 (COVID-19) typically presents with fever and respiratory symptoms in children. Most children develop an asymptomatic and mild illness, with a minority requiring specialist medical care. Gastrointestinal manifestations and liver injury can also occur in children following infection. The mechanisms of liver injury may include infection following direct viral hepatic tissue invasion, immune response, or medication effects. Affected children might develop mild liver dysfunction which has a benign course in most children with no pre-existing liver disease. However, the presence of non-alcoholic fatty liver disease or other pre-existing chronic liver disorders is associated with a higher risk of developing severe COVID-19 illness with poor outcomes. On the other hand, the presence of liver manifestations is associated with the severity of COVID-19 disease and is considered an independent prognostic factor. Respiratory, hemodynamic, and nutritional supportive therapies are the mainstay of management. Vaccination of children at increased risk of developing severe COVID-19 disease is indicated. This review describes the liver manifestations in children with COVID-19, detailing its epidemiology, basic mechanisms, clinical expression, management, and prognosis in those with and without pre-existing liver disease and also children who have had earlier liver transplantation.</p>","PeriodicalId":75338,"journal":{"name":"World journal of clinical pediatrics","volume":"12 3","pages":"57-67"},"PeriodicalIF":0.0,"publicationDate":"2023-06-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/84/01/WJCP-12-57.PMC10278079.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9709209","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinical characteristics of community-acquired pneumonia in children caused by mycoplasma pneumoniae with or without myocardial damage: A single-center retrospective study.","authors":"Shukri Omar Yusuf, Peng Chen","doi":"10.5409/wjcp.v12.i3.115","DOIUrl":"10.5409/wjcp.v12.i3.115","url":null,"abstract":"<p><strong>Background: </strong>Mycoplasma pneumoniae (MP) is a prevalent pathogen that causes respiratory infections in children and adolescents.</p><p><strong>Aim: </strong>To assess the differences in the clinical features of MP-associated community-acquired pneumonia (CAP) in children who presented with mild or severe mycoplasma pneumoniae pneumonia (MPP); to identify the incidence of myocardial damage between the two groups.</p><p><strong>Methods: </strong>This work is a retrospective study. We identified children between 2 mo and 16 years of age with clinical and radiological findings consistent with CAP. We admitted patients to the inpatient department of the Second Hospital of Jilin University, Changchun, China, from January 2019 to December 2019.</p><p><strong>Results: </strong>A total of 409 hospitalized patients were diagnosed with MPP. Among them were 214 (52.3%) males and 195 (47.7%) females. The duration of fever and cough was the longest in severe MPP cases. Similarly, plasma levels of highly sensitive C-reactive protein (<i>t</i> = -2.834, <i>P</i> < 0.05), alanine transaminase (<i>t</i> = -2.511, <i>P</i> < 0.05), aspartate aminotransferase (<i>t</i> = -2.939, <i>P</i> < 0.05), and lactate dehydrogenase (LDH) (<i>t</i> = -2.939, <i>P</i> < 0.05) were all elevated in severe MPP cases compared with mild MPP cases, and these elevations were statistically significant (<i>P</i> < 0.05). Conversely, the neutrophil percentage was significantly lower in severe MPP cases than in mild MPP cases. The incidence of myocardial damage was significantly higher in severe MPP cases than in mild MPP cases (<i>χ</i>² = 157.078, <i>P</i> < 0.05).</p><p><strong>Conclusion: </strong>Mycoplasma pneumoniae is the main cause of CAP. The incidence of myocardial damage was higher and statistically significant in severe MPP cases than in mild MPP cases.</p>","PeriodicalId":75338,"journal":{"name":"World journal of clinical pediatrics","volume":"12 3","pages":"115-124"},"PeriodicalIF":0.0,"publicationDate":"2023-06-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/9c/8a/WJCP-12-115.PMC10278075.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9709211","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Hirschsprung's disease associated enterocolitis: A comprehensive review.","authors":"Eric M Gershon, Leonel Rodriguez, Ricardo A Arbizu","doi":"10.5409/wjcp.v12.i3.68","DOIUrl":"10.5409/wjcp.v12.i3.68","url":null,"abstract":"<p><p>Hirschsprung's disease (HSCR) is a congenital disorder characterized by failure of the neural crest cells to migrate and populate the distal bowel during gestation affecting different lengths of intestine leading to a distal functional obstruction. Surgical treatment is needed to correct HSCR once the diagnosis is confirmed by demonstrating the absence of ganglion cells or aganglionosis of the affected bowel segment. Hirschsprung's disease associated enterocolitis (HAEC) is an inflammatory complication associated with HSCR that can present either in the pre- or postoperative period and associated with increased morbidity and mortality. The pathogenesis of HAEC remains poorly understood, but intestinal dysmotility, dysbiosis and impaired mucosal defense and intestinal barrier function appear to play a significant role. There is no clear definition for HAEC, but the diagnosis is primarily clinical, and treatment is guided based on severity. Here, we aim to provide a comprehensive review of the clinical presentation, etiology, pathophysiology, and current therapeutic options for HAEC.</p>","PeriodicalId":75338,"journal":{"name":"World journal of clinical pediatrics","volume":"12 3","pages":"68-76"},"PeriodicalIF":0.0,"publicationDate":"2023-06-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/fa/50/WJCP-12-68.PMC10278080.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10086644","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}