RESUMEN
Biliary atresia (BA) is a progressive inflammatory fibrosclerosing disease of the biliary system and a major cause of neonatal cholestasis. It affects 1:5,000-20,000 live births, with the highest incidence in Asia. The pathogenesis is still unknown, but emerging research suggests a role for ciliary dysfunction, redox stress and hypoxia. The study of the underlying mechanisms can be conceptualized along the likely prenatal timing of an initial insult and the distinction between the injury and prenatal and postnatal responses to injury. Although still speculative, these emerging concepts, new diagnostic tools and early diagnosis might enable neoadjuvant therapy (possibly aimed at oxidative stress) before a Kasai portoenterostomy (KPE). This is particularly important, as timely KPE restores bile flow in only 50-75% of patients of whom many subsequently develop cholangitis, portal hypertension and progressive fibrosis; 60-75% of patients require liver transplantation by the age of 18 years. Early diagnosis, multidisciplinary management, centralization of surgery and optimized interventions for complications after KPE lead to better survival. Postoperative corticosteroid use has shown benefits, whereas the role of other adjuvant therapies remains to be evaluated. Continued research to better understand disease mechanisms is necessary to develop innovative treatments, including adjuvant therapies targeting the immune response, regenerative medicine approaches and new clinical tests to improve patient outcomes.
Asunto(s)
Atresia Biliar , Atresia Biliar/fisiopatología , Atresia Biliar/diagnóstico , Atresia Biliar/terapia , Atresia Biliar/epidemiología , Atresia Biliar/complicaciones , Humanos , Portoenterostomía Hepática/métodos , Trasplante de Hígado/métodos , Trasplante de Hígado/estadística & datos numéricosRESUMEN
BACKGROUND & AIMS: We have previously reported on the potential pathogenic role of neutrophils in biliary atresia (BA). Herein, we aimed to delineate the role of CD177+ neutrophils in the pathogenesis of BA. METHODS: Immune cells from the livers of mice with rhesus rotavirus-induced BA were analysed. Single-cell RNA-sequencing was performed to specifically analyse Gr-1+ (Ly6C/Ly6G+) cells in the liver. Gene expression profiles of CD177+ cells were analysed using the Smart-Seq RNA-sequencing method, and the pathogenesis of BA was examined in Cd177-/- mice. Neutrophil extracellular trap (NET) inhibitors were used to determine the role of CD177+ cell-derived NETs in BA-associated bile duct damage, and a pilot clinical study evaluated the potential effects of N-acetylcysteine on NET release in BA. RESULTS: Increased levels of Gr-1+ cells were observed in the livers of mice with rhesus rotavirus-induced BA. RNA-sequencing analysis revealed that CD177+ cells were the main population of Gr-1+ cells and expressed elevated levels of both interferon-stimulated and neutrophil degranulation genes. Cd177-/- BALB/c mice exhibited delayed disease onset and reduced morbidity and mortality. High numbers of mitochondria were detected in CD177+ cells derived from mice with BA; these cells were associated with increased levels of reactive oxygen species and increased NET formation, which induced the apoptosis of biliary epithelial cells in cocultures. In a pilot clinical study, the administration of N-acetylcysteine to patients with BA reduced CD177+ cell numbers and reactive oxygen species levels, indicating a potential beneficial effect. CONCLUSIONS: Our data indicate that CD177+ cells play an important role in the initiation of BA pathogenesis via NET formation. CLINICAL TRIAL REGISTRATION: The pilot study of N-acetylcysteine treatment in patients with BA was registered on the Chinese Clinical Trial Registry (ChiCTR2000040505). LAY SUMMARY: Neutrophils (a type of innate immune cell, i.e. an immune cell that doesn't target a specific antigen) are thought to play a role in the development of biliary atresia (a rare but potentially lethal condition of the bile ducts that occurs in infants). Herein, we found that neutrophils expressing a particular protein (CD177) played an important role in bile duct damage by releasing a special structure (NET) that can trap and kill pathogens but that can also cause severe tissue damage. A pilot study in patients with biliary atresia showed that inhibiting NETs could have a beneficial effect.
Asunto(s)
Atresia Biliar , Trampas Extracelulares , Rotavirus , Acetilcisteína , Animales , Atresia Biliar/patología , Modelos Animales de Enfermedad , Interferones , Ratones , Ratones Endogámicos BALB C , Proyectos Piloto , ARN , Especies Reactivas de Oxígeno , Rotavirus/genéticaRESUMEN
AIM: Due to the paucity of data and controversy regarding the etiology and surgical approach for managing anorectal prolapse (ARP) after anorectoplasty, we sought to investigate the underlying anatomic disorder and the surgical outcome in managing this challenging complication. METHODS: We performed a retrospective study on 83 patients with ARP related to anorectal malformations (ARM). Logistic regression analyses were performed to detect the risk factors for the ARP severity. Surgical procedures were stratified according to identified anatomical abnormalities and surgical outcomes were analyzed. RESULTS: 50 patients (62.7%) had high-type ARM. The original anorectoplasty had a higher rate of ARP in laparoscopic-assisted anorectoplasty (n = 49, 59.0%) versus posterior sagittal anorectoplasty (n = 11, 13.3%). ARP was associated with rectal fat hyperplasia (67.5%), dilated muscular tunnel (79.5%), longitudinal muscle (LM) discontinuity (16.9%), rectal dilation (22.9%), mislocated anus (7.2%), and excessive mobile mesorectum (3.6%). Based on the ARP severity, the patients were divided into a severe group (Group 1, n = 38) and a moderate group (Group 2, n = 45). Binary logistic regression analysis showed that hyperplasia rectal fat (OR 4.55, 95% CI 1.16-17.84), rectal dilation (OR 4.21, 95% CI 1.05-16.94), and high-type ARM (OR 2.90, 95% CI 1.14-7.39) were independent risk factors for the development of severe ARP. Complications after stratified surgical repair included wound infection in six patients (7.2%), anal stenosis in one patient (1.2%), and ARP recurrence in two patients (2.4%). Twenty-six patients without colostomy before prolapse repair were followed up for 2 to 12 years. All the patients maintained voluntary bowel movements. Following ARP repair, there was an overall higher rate of no soiling or grade 1 soiling (88.5 vs. 65.4%), but 3 of 12 patients with grade 2 constipation were upgraded to grade 3. CONCLUSION: Our study shows that ARM-related anorectal prolapse is associated with excessive rectum, hyperplasia of rectal fat, mobile mesorectum, loose muscular tunnel, LM discontinuity, and anal mislocation. Surgical repair with techniques stratified according to the patients' underlying risk factors is effective to prevent recurrence and improve the soiling continence.
Asunto(s)
Malformaciones Anorrectales , Procedimientos de Cirugía Plástica , Prolapso Rectal , Canal Anal/cirugía , Malformaciones Anorrectales/complicaciones , Malformaciones Anorrectales/cirugía , Humanos , Hiperplasia/complicaciones , Lactante , Procedimientos de Cirugía Plástica/efectos adversos , Prolapso Rectal/etiología , Prolapso Rectal/cirugía , Recto/cirugía , Estudios RetrospectivosRESUMEN
BACKGROUND: The coronavirus disease 2019 (COVID-19) pandemic is having a profound impact on the health and development of children worldwide. There is limited evidence on the impact of COVID-19 and its related school closures and disease-containment measures on the psychosocial wellbeing of children; little research has been done on the characteristics of vulnerable groups and factors that promote resilience. METHODS: We conducted a large-scale cross-sectional population study of Hong Kong families with children aged 2-12 years. Parents completed an online survey on family demographics, child psychosocial wellbeing, functioning and lifestyle habits, parent-child interactions, and parental stress during school closures due to COVID-19. We used simple and multiple linear regression analyses to explore factors associated with child psychosocial problems and parental stress during the pandemic. RESULTS: The study included 29,202 individual families; of which 12,163 had children aged 2-5 years and 17,029 had children aged 6-12 years. The risk of child psychosocial problems was higher in children with special educational needs, and/or acute or chronic disease, mothers with mental illness, single-parent families, and low-income families. Delayed bedtime and/or inadequate sleep or exercise duration, extended use of electronic devices were associated with significantly higher parental stress and more psychosocial problems among pre-schoolers. CONCLUSIONS: This study identifies vulnerable groups of children and highlights the importance of strengthening family coherence, adequate sleep and exercise, and responsible use of electronic devices in promoting psychosocial wellbeing during the COVID-19 pandemic.
Asunto(s)
COVID-19 , Niño , Preescolar , Estudios Transversales , Humanos , Pandemias , Padres , SARS-CoV-2RESUMEN
BACKGROUND AND AIMS: The enteric nervous system, which regulates many gastrointestinal functions, is derived from neural crest cells (NCCs). Defective NCC migration during embryonic development may lead to enteric neuropathies such as Hirschsprung's disease (hindgut aganglionosis). Sox10 is known to be essential for cell migration but downstream molecular events regulating early NCC migration have not been fully elucidated. This study aimed to determine how Sox10 regulates migration of sacral NCCs toward the hindgut using Dominant megacolon mice, an animal model of Hirschsprung's disease with a Sox10 mutation. METHODS: We used the following: time-lapse live cell imaging to determine the migration defects of mutant sacral NCCs; genome-wide microarrays, site-directed mutagenesis, and whole embryo culture to identify Sox10 targets; and liquid chromatography and tandem mass spectrometry to ascertain downstream effectors of Sox10. RESULTS: Sacral NCCs exhibited retarded migration to the distal hindgut in Sox10-null embryos with simultaneous down-regulated expression of cadherin-19 (Cdh19). Sox10 was found to bind directly to the Cdh19 promoter. Cdh19 knockdown resulted in retarded sacral NCC migration in vitro and ex vivo, whereas re-expression of Cdh19 partially rescued the retarded migration of mutant sacral NCCs in vitro. Cdh19 formed cadherin-catenin complexes, which then bound to filamentous actin of the cytoskeleton during cell migration. CONCLUSIONS: Cdh19 is a direct target of Sox10 during early sacral NCC migration toward the hindgut and forms cadherin-catenin complexes which interact with the cytoskeleton in migrating cells. Elucidation of this novel molecular pathway helps to provide insights into the pathogenesis of enteric nervous system developmental defects.
Asunto(s)
Cadherinas/metabolismo , Movimiento Celular , Sistema Nervioso Entérico/metabolismo , Enfermedad de Hirschsprung/metabolismo , Cresta Neural/metabolismo , Células-Madre Neurales/metabolismo , Neurogénesis , Factores de Transcripción SOXE/metabolismo , Citoesqueleto de Actina/genética , Citoesqueleto de Actina/metabolismo , Citoesqueleto de Actina/patología , Animales , Cadherinas/genética , Células Cultivadas , Modelos Animales de Enfermedad , Técnicas de Cultivo de Embriones , Sistema Nervioso Entérico/anomalías , Regulación del Desarrollo de la Expresión Génica , Enfermedad de Hirschsprung/genética , Enfermedad de Hirschsprung/patología , Ratones Endogámicos C3H , Ratones Endogámicos C57BL , Ratones Noqueados , Cresta Neural/anomalías , Células-Madre Neurales/patología , Unión Proteica , Factores de Transcripción SOXE/genética , Transducción de Señal , Factores de TiempoRESUMEN
Biliary Atresia is a devastating pediatric cholangiopathy affecting the bile ducts of the liver. In this review, we describe recent progress in the understanding of liver development with a focus on cholangiocyte differentiation and how use of technical platforms, including rodent, zebrafish and organoid models, advances our understanding of Biliary Atresia. This is followed by a description of potential pathomechanisms, such as autoimmune responses, inflammation, disturbed apical-basal cell polarity, primary cilia dysfunction as well as beta-amyloid accumulation. Finally, we describe current and emerging diagnostic opportunities and recent translation breakthroughs for Biliary Atresia in the area of emerging therapy development, including immunomodulation and organoid-based systems for liver and bile duct repair.
Asunto(s)
Conductos Biliares/citología , Atresia Biliar/diagnóstico , Organoides/patología , Animales , Atresia Biliar/patología , Diferenciación Celular , Modelos Animales de Enfermedad , Células Epiteliales/citología , HumanosRESUMEN
Objectives: This study aimed to identify key factors affecting Healthcare workers (HCWs) perceived stress and risk of contracting COVID-19 among themselves and their family members during the pandemic. Methods: A cross-sectional online questionnaire study was conducted between 19 March and April 5, 2020 in Hong Kong. HCWs from public hospitals and private dentists, and their family members participated. Results: A total of 747 HCWs and 245 family members participated. Higher perceived stress in HCWs was associated with more negative changes in family relationship (p = 0.025). The HCWs' perceived stress, however, was positively associated with family cohesion (p = 0.033) and stress levels of family members (p < 0.001). The level of HCWs' satisfaction toward the hospital policies in response to the COVID-19 outbreak was associated with lower levels of perceived stress and risk of themselves or their family members contracting COVID-19. HCWs' previous frontline experience of SARS was significantly associated with less perceived risk of themselves or their family members contracting COVID-19. Conclusion: Hospital policies addressing HCWs' needs, frontline experience of SARS, and family relationship influenced psychological wellbeing of HCWs during the COVID-19 outbreak.
Asunto(s)
COVID-19 , Personal de Salud , Pandemias , Estrés Psicológico , COVID-19/epidemiología , COVID-19/psicología , Estudios Transversales , Personal de Salud/psicología , Humanos , Análisis Multinivel , Medición de Riesgo , Estrés Psicológico/psicologíaRESUMEN
Hirschsprung disease (HSCR) is a complex genetic disease characterized by absence of ganglia in the intestine. HSCR etiology can be explained by a unique combination of genetic alterations: rare coding variants, predisposing haplotypes and Copy Number Variation (CNV). Approximately 18% of patients have additional anatomical malformations or neurological symptoms (HSCR-AAM). Pinpointing the responsible culprits within a CNV is challenging as often many genes are affected. Therefore, we selected candidate genes based on gene enrichment strategies using mouse enteric nervous system transcriptomes and constraint metrics. Next, we used a zebrafish model to investigate whether loss of these genes affects enteric neuron development in vivo. This study included three groups of patients, two groups without coding variants in disease associated genes: HSCR-AAM and HSCR patients without associated anomalies (HSCR-isolated). The third group consisted of all HSCR patients in which a confirmed pathogenic rare coding variant was identified. We compared these patient groups to unaffected controls. Predisposing haplotypes were determined, confirming that every HSCR subgroup had increased contributions of predisposing haplotypes, but their contribution was highest in isolated HSCR patients without RET coding variants. CNV profiling proved that specifically HSCR-AAM patients had larger Copy Number (CN) losses. Gene enrichment strategies using mouse enteric nervous system transcriptomes and constraint metrics were used to determine plausible candidate genes located within CN losses. Validation in zebrafish using CRISPR/Cas9 targeting confirmed the contribution of UFD1L, TBX2, SLC8A1, and MAPK8 to ENS development. In addition, we revealed epistasis between reduced Ret and Gnl1 expression and between reduced Ret and Tubb5 expression in vivo. Rare large CN losses-often de novo-contribute to HSCR in HSCR-AAM patients. We proved the involvement of six genes in enteric nervous system development and Hirschsprung disease.
Asunto(s)
Variaciones en el Número de Copia de ADN , Sistema Nervioso Entérico/crecimiento & desarrollo , Redes Reguladoras de Genes , Enfermedad de Hirschsprung/genética , Animales , Estudios de Casos y Controles , Modelos Animales de Enfermedad , Sistema Nervioso Entérico/química , Epistasis Genética , Predisposición Genética a la Enfermedad , Haplotipos , Humanos , Ratones , Pez CebraRESUMEN
Biliary atresia (BA) is an immune-related disorder and signal transducer and activator of transcription 3 (STAT3) is a key signalling molecule in inflammation. The present study was designed to clarify the function of STAT3 in BA. STAT3 expression was examined in patients and a mouse BA model in which STAT3 levels were further altered with a specific inhibitor or activator. Neutrophil accumulation and the levels of the neutrophil chemoattractants (C-X-C motif) ligand 1 (CXCL1) and IL-8 were determined. The effects of STAT3 inhibition on IL-8 expression were examined in human biliary epithelial cell (BEC) cultures. Functional changes in liver STAT3+ neutrophils in the mouse model were analysed with 10× single cell RNA-seq methods. Results showed STAT3 and p-STAT3 expression was reduced in BA liver tissue compared with control samples. Administration of a STAT3 inhibitor increased jaundice and mortality and reduced body weight in BA mice. In contrast, the STAT3 activator ameliorated BA symptoms. Extensive neutrophil accumulation together with CXCL1 up-regulation, both of which were suppressed by an anti-CXCL1 antibody, were observed in the STAT3 inhibitor-treated group. Recombinant IL-8 administration increased disease severity in BA mice, and the STAT3 activator had the reverse effect. Inhibiting STAT3 increased apoptosis of human BECs together with up-regulated IL-8 expression. RNA-seq analysis revealed reduced the numbers of STAT3 expressing neutrophil in BA which was accompanied by marked enhanced interferon-related antiviral activities. In conclusion, STAT3 reduction, enhanced IL-8 and CXCL1 expression and promoted the accumulation of interferon-responsive neutrophils resulting in BEC damage in BA.
Asunto(s)
Atresia Biliar/patología , Quimiocina CXCL1/metabolismo , Interleucina-8/metabolismo , Infiltración Neutrófila , Factor de Transcripción STAT3/metabolismo , Animales , Atresia Biliar/metabolismo , Quimiocina CXCL1/genética , Modelos Animales de Enfermedad , Células Epiteliales , Humanos , Lactante , Hígado/metabolismo , Ratones Endogámicos BALB C , Rotavirus , Infecciones por Rotavirus , Factor de Transcripción STAT3/genéticaRESUMEN
Persistence of protective immunity for SARS-CoV-2 is important against reinfection. Knowledge on SARS-CoV-2 immunity in pediatric patients is currently lacking. We opted to assess the SARS-CoV-2 adaptive immunity in recovered children and adolescents, addressing the pediatrics specific immunity towards COVID-19. Two independent assays were performed to investigate humoral and cellular immunological memory in pediatric convalescent COVID-19 patients. Specifically, RBD IgG, CD4+, and CD8+ T cell responses were identified and quantified in recovered children and adolescents. SARS-CoV-2-specific RBD IgG detected in recovered patients had a half-life of 121.6 days and estimated duration of 7.9 months compared with baseline levels in controls. The specific T cell response was shown to be independent of days after diagnosis. Both CD4+ and CD8+ T cells showed robust responses not only to spike (S) peptides (a main target of vaccine platforms) but were also similarly activated when stimulated by membrane (M) and nuclear (N) peptides. Importantly, we found the differences in the adaptive responses were correlated with the age of the recovered patients. The CD4+ T cell response to SARS-CoV-2 S peptide in children aged <12 years correlated with higher SARS-CoV-2 RBD IgG levels, suggesting the importance of a T cell-dependent humoral response in younger children under 12 years. Both cellular and humoral immunity against SARS-CoV-2 infections can be induced in pediatric patients. Our important findings provide fundamental knowledge on the immune memory responses to SARS-CoV-2 in recovered pediatric patients.
Asunto(s)
Inmunidad Adaptativa/inmunología , Anticuerpos Neutralizantes/inmunología , Anticuerpos Antivirales/inmunología , COVID-19/inmunología , Convalecencia , SARS-CoV-2/inmunología , Adolescente , Linfocitos T CD4-Positivos/inmunología , Linfocitos T CD4-Positivos/metabolismo , Linfocitos T CD4-Positivos/virología , Linfocitos T CD8-positivos/inmunología , Linfocitos T CD8-positivos/metabolismo , Linfocitos T CD8-positivos/virología , COVID-19/virología , Niño , Preescolar , Femenino , Humanos , Inmunidad Humoral/inmunología , Inmunoglobulina G/inmunología , Masculino , SARS-CoV-2/metabolismo , SARS-CoV-2/fisiología , Glicoproteína de la Espiga del Coronavirus/inmunología , Glicoproteína de la Espiga del Coronavirus/metabolismoRESUMEN
Haematological and immunological data of children with COVID-19 infection is lacking. Between 21st January and 20th March 2020, 244 children who were confirmed to have COVID-19 infection and admitted to the Wuhan Children's Hospital, China were retrospectively reviewed. 193 children were considered as symptomatic, which was defined as having either the presence of clinical symptoms or the presence of CT thorax abnormalities. Their haematological and immunological profiles, including complete blood counts, lymphocyte subsets (T, B and NK cell counts), immunoglobulin (Ig) profiles (IgG, IgA and IgM) and cytokine profiles were analysed and compared between the symptomatic and asymptomatic groups. The median values and the interquartile ranges were calculated. Comparison was made using the Mann-Whitney U test. Children with symptomatic COVID-19 infection had significantly lower haemoglobin levels, but higher absolute lymphocyte and monocyte counts, IgG and IgA levels, as well as interleukin 6 (IL-6), IL-10, tumour necrosis factor alpha and interferon gamma levels. The obtained data will be utilized for further studies in comparing children and adults with COVID-19 infections in other parts of the world and with different severity .
RESUMEN
OBJECTIVES: To compare the clinical and laboratory features of severe acute respiratory syndrome 2003 (SARS) and coronavirus disease 2019 (COVID-19) in 2 Chinese pediatric cohorts, given that the causative pathogens and are biologically similar. STUDY DESIGN: This is a cross-sectional study reviewing pediatric patients with SARS (n = 43) and COVID-19 (n = 244) who were admitted to the Princess Margaret Hospital in Hong Kong and Wuhan Children's Hospital in Wuhan, respectively. Demographics, hospital length of stay, and clinical and laboratory features were compared. RESULTS: Overall, 97.7% of patients with SARS and 85.2% of patients with COVID-19 had epidemiologic associations with known cases. Significantly more patients with SARS developed fever, chills, myalgia, malaise, coryza, sore throat, sputum production, nausea, headache, and dizziness than patients with COVID-19. No patients with SARS were asymptomatic at the time of admission, whereas 29.1% and 20.9% of patients with COVID-19 were asymptomatic on admission and throughout their hospital stay, respectively. More patients with SARS required oxygen supplementation than patients with COVID-19 (18.6 vs 4.7%; P = .004). Only 1.6% of patients with COVID-19 and 2.3% of patients with SARS required mechanical ventilation. Leukopenia (37.2% vs 18.6%; P = .008), lymphopenia (95.4% vs 32.6%; P < .01), and thrombocytopenia (41.9% vs 3.8%; P < .001) were significantly more common in patients with SARS than in patients with COVID-19. The duration between positive and negative nasopharyngeal aspirate and the length in hospital stay were similar in patients with COVID-19, regardless of whether they were asymptomatic or symptomatic, suggesting a similar duration of viral shedding. CONCLUSIONS: Children with COVID-19 were less symptomatic and had more favorable hematologic findings than children with SARS.
Asunto(s)
Infecciones por Coronavirus/epidemiología , Neumonía Viral/epidemiología , Síndrome Respiratorio Agudo Grave/epidemiología , Adolescente , Infecciones Asintomáticas , Betacoronavirus , COVID-19 , Niño , Preescolar , China/epidemiología , Infecciones por Coronavirus/diagnóstico , Estudios Transversales , Femenino , Hong Kong , Hospitalización , Humanos , Lactante , Tiempo de Internación , Masculino , Pandemias , Neumonía Viral/diagnóstico , Estudios Retrospectivos , Coronavirus Relacionado al Síndrome Respiratorio Agudo Severo , SARS-CoV-2 , Síndrome Respiratorio Agudo Grave/diagnósticoRESUMEN
PURPOSE: Laparoscopic-assisted anorectoplasty (LAARP) is considered to benefit the patients with vesico-prostatic fistula. The aim of this study is to present the details of our LAARP technique for improving the short- and long-term outcomes in the patients with high and intermediate types of anorectal malformations (ARMs). METHODS: 330 patients with high-type (174 cases) and intermediate-type (156 cases) anorectal malformation (aged 8 days to 15 years) underwent LAARP from 2001 to 2019. LAARP was performed for full mobilization and resection of the dilated rectum, intra-rectal closure of the fistula, visualization, and enlargement of the center of the longitudinal muscle tube (LMT) from pelvic and perineal aspects. RESULTS: LAARP was performed in all patients and no patient was converted to open procedure. The urethral diverticulum was found in three patients (1.02%, 3/294) according to postoperative protocol voiding cystourethrogram but was not associated with any symptoms such as urinary tract infection and dysuria. Rectal prolapse requiring surgical intervention developed in 25 (7.6%) of 330 patients. Anal stricture occurred in three patients and re-do anoplasty was performed 5 months after LAARP. Anal retraction occurred in two patients and re-pull-through was conducted at 5 and 6 days, respectively, after LAARP. 228 patients who were older than 3 years were followed up. The median follow-up period was 5.8 years (range 3-15 years). 217 patients (95.2%) had voluntary bowel movements; 202 patients (88.6%) were free from soiling or with grade 1 soiling; 30 patients (13.6%) and 25 patients (11.3%) suffered from grade 1 and grade 2 constipation, respectively, while no patient had grade 3 constipation. CONCLUSION: Our experience demonstrates that the LAARP has advantages on rectal mobilization and resection, intra-rectal fistula closure and accurate tunnel formation in the LMT with minimal trauma. The improvement of the short-term and long-term outcomes after LAARP has been shown not only for high-type ARM but also for intermediate-type ARM.
Asunto(s)
Malformaciones Anorrectales/cirugía , Defecación/fisiología , Procedimientos Quirúrgicos del Sistema Digestivo/métodos , Laparoscopía/métodos , Adolescente , Malformaciones Anorrectales/fisiopatología , Niño , Preescolar , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Periodo Posoperatorio , Estudios RetrospectivosRESUMEN
BACKGROUND: The objective of this study was to determine the most optimal timing of liver transplant (LT) for post-Kasai portoenterostomy (KPE) patients based on disease severity scores. METHODS: This was a retrospective study and the clinical data of all LT recipients aged <18â¯years (nâ¯=â¯89) with a history of KPE were analyzed. They were divided into three groups according to their PELD/MELD scores at the time of LT (A: <15; B: 15-25; C: >25). The effects of LT on the clinical outcomes and hospitalization status were analyzed. RESULTS: There were 33, 34 and 22 patients in group A, B and C, respectively. There was no significant difference in 3-year graft survival rate between the three groups but group C patients had the highest incidence of vascular or biliary complications (pâ¯=â¯0.022). Group C patients had a significantly lower hospital admission frequency (pâ¯=â¯0.036) and shorter hospital stay (pâ¯=â¯0.041) after LT when compared with their pre-LT status and with non-LT patients with similar disease severity scores. On the other hand, the hospitalization frequency and duration were similar in patients with the lowest disease severity score (group A) before, after and without LT. CONCLUSIONS: The benefit of LT was less obvious when the disease severity score is <15. A high complication rate was reported when LT was performed at a scoreâ¯>â¯25. Donor availability, the patient's general condition and parental wish should be considered during individual assessment. TYPE OF STUDY: Clinical research paper. LEVEL OF EVIDENCE: Level III.