Outcome Analysis of Colostomy Closure in Different Pediatric Surgical Conditions: A Pediatric Tertiary Care Hospital Study in Bangladesh

Background: A Colostomy is a revocable surgical procedure an incision in the anterior abdominal wall and suturing it into place in which a stoma is formed by drawing the healthy end of the large intestine or colon through. This opening, in conjunction with the attached stoma appliance, provides an alternative channel for feces to leave the body. Due to anatomical defects frequently referred for closure colostomy, colostomies are often used in cases of imperforate anus and other conditions. The neonatal & children who are unable to pass feces normally and safely. pediatrics colostomy. Some neonates require emergency surgery on their tummy in the first few months of life. It is most commonly due to being born prematurely and developing a bowel problem or a blockage of the bowel. As part of this surgery, the ends of the bowel may be brought to the skin surface to divert stool into a bag called a colostomy. Intended to be temporary with reversal, later on, the stoma allows time for the bowel to rest and recover. Aim of the study: To find out the outcome analysis of colostomy closure in different pediatric surgical conditions and were reviewed to look for complications following closure colostomy.Material & Methods:This prospective was conducted in the department of Pediatrics Surgery Bangladesh Shishu Hospital & Institute, Dhaka, and Lubana General Hospital & Cardiac Center, Dhaka, Bangladesh from July 2014 to June 2021. A total of 86 patients who underwent colostomy closure were enrolled in this prospective study as the study population. Data including age, gender, surgical conditions, complications of the patients and oral feeding, and bowel preparation were all collected from the patients’ parents or hospital admission files.Results:Out of 86 cases included the age range from 8 months-10 years. There were 37(43.0%) females and 49(57.0%) males and there were more difficulties with Anorectal malformation (43.02%) than with Hirschsprung disease (40.7%). There was no record of using Necrotizing Enterocolitis, in surgical patients. The risk of wound infection and leakage was greater than any other consequence in the patients. The illness known as colostomy was predominantly encountered in men. No morbidity was recorded in this study. Conclusions:Proper stoma care, the use of well-fitting colostomy bags, and early colostomy closure enhance the prognosis. Prior to surgery, encourage thorough mechanical bowel preparation and antibiotic use. The key to a successful colostomy closure for anti-surgical diseases is appropriate IV feeding after surgery.

Urodynamic assessment of voiding dysfunction associated with anorectal malformation / 中华实用儿科临床杂志

Anorectal malformation (ARM) accounts for the first place of digestive tract malformations in children, with the incidence of 1/5 000-1/1 500 in newborns.ARM is typically manifested as anal stenosis or anal atresia with or without fistulas and persistent cloaca.Genitourinary malformation is the most common malformation associated with ARM, with the incidence ranging 26%-55%.There were 35%-50% of ARM children develop spinal cord peripheral abnormalities or neurogenic lower urinary tract dysfunction, which usually causes voiding dysfunction associated with ARM.Urodynamic study is the best method to analyze the changes of lower urinary tract function, which can provide an objective classification for bladder and urethral function and guide the formulation of precision treatment plan and therapeutic efficacy during the follow-up period.This study aims to review the cause of voiding dysfunction asso-ciated with ARM and its urodynamic changes.

Anal ultrasonography in monitoring and postoperative evaluation of anorectal malformations

Objectives: We aimed to describe anal ultrasound for monitoring anorectal malformations.Methods: A descriptive and retrospective study of patients diagnosed and/or intervened with anorectal malformations rated by anal ultrasonography, between 2016-2017, was performed. We employed a transducer of 10 megahertz to identify the anatomy of the anal channel.Results: Eight patients, 5 men and 3 women were included, with a mean of 8.37 years old (range, 3-11). Three patients had a diagnosis of an anterior anus (not operated). Surgery was performed in 5 patients: 2 had a rectourethral fistula, 1 had a rectovesical fistula, and 1 had a rectovestibular and a perineal fistula. The most significant ultrasound findings were partial absence of the anterior part of the ex-ternal sphincter, internal sphincter hypertrophy in the middle channel, and absence of internal sphincter in the median and high channel plus the absence of external sphincter in the an-terior region. These results belonged to a patient with clinical incontinence and to 2 patients that were previously diagnosed with an anterior anus.Conclusions: Anal ultrasonography is a non-invasive method, economic, and feasible to be performed without anesthesia. It offers useful anatomical information to establish the best therapeutic option. Its main limitation is the unfeasibility of carrying it out on patients under 3 years old.

Objetivos: Describir la ecografía anal en el seguimiento de malformaciones anorrectales.Material y Métodos: estudio descriptivo y retrospectivo de pacientes diagnosticados y/o in-tervenidos de malformaciones anorrectales, evaluados por ecografía anal, entre 2016-2017. Empleamos un transductor de 10 megahercios, identificando la anatomía del canal anal alto, medio y bajo.Resultados: Se incluyeron ocho pacientes, 5 hombres y 3 mujeres, con una media de 8,37 años (rango, 3-11). Tres pacientes tenían diagnóstico de ano anterior (no operados previa-mente). Se intervinieron 5 pacientes: 2 presentaban fístula recto-uretral, 1 fístula recto-vesi-cal, 1 recto-vestibular y 1 fístula perineal.Los hallazgos ecográficos más significativos fueron: ausencia parcial de la parte anterior del esfínter externo, hipertrofia del esfínter interno en el canal medio en un paciente; y ausencia de esfínter interno en el canal medio y alto más ausencia de esfínter externo en la región anterior, en un paciente con incontinencia clínica, ambos pacientes previamente diagnosticados de ano anterior que posteriormente fueron intervenidos.Conclusiones: La ecografía anal es un método no invasivo, económico, factible de realizarse sin anestesia y ofrece una información anatómica útil para establecer la mejor opción terapéutica. Su principal limitación es la imposibilidad de realizarlo en pacientes menores de 3 años.

Pattern of anorectal malformation in a tertiary care hospital of Bihar

Background: Anorectal malformation (ARM) is common cause of neonatal intestinal obstruction and is one of the most common neonatal surgical emergency encountered by paediatric surgeons. There is no published study about the prevalence of ARM cases within the referral zone of the hospital. This study was done to demonstrate the burden of Anorectal malformation and their surgical intervention as seen in Indira Gandhi Institute of medical science, Patna, Bihar.Methods: Data were retrospectively collected over a period of four years between January 2015 and December 2018. The data collected were demographic status of the patient, diagnosis, associated anomaly, duration of hospital stay and their surgical intervention.Results: There were 395 cases of anorectal malformation out of 93203 OPD patients (0.42%). Male to female ratio of 3.01:1.  ARM also represented 25.3%   of neonatal emergency surgical procedures. The majority of the patient had presented in the neonatal period (51.6%) with mean weight of 2.6 kg. The most common type of ARM found in male patient was rectourethral fistula n=215 (54.4%) in which 164 (76.2%) patients were with rectobulbar fistula and 51 (23.8%) patients had rectoprostatic fistula. Sixty nine (17.4%) female patients presented with perineal fistula.Conclusions: We found a clear male preponderance of anorectal malformation in our study. Patients affected were mostly from poor socioeconomic strata of the society. Present study confirms huge work load of anorectal malformation in this region. This will be helpful in future planning and effective management including financial constraints of the treatment for our poor patients.

A Study of Gastrointestinal Tract Obstruction in Neonates and Its Management

Gastrointestinal tract obstructions are the most common surgical emergencies in neonatal period. The aetiology of these disorders is diverse and mostly the consequences prenatal developmental malformations. The management and survival are still a challenge, especially in developing countries like India.METHODSA prospective observational study was conducted in a tertiary care paediatric institute from October 2016 to September 2019. Newborns in the age group of one to 28 days, who were operated in the hospital for gastrointestinal tract obstruction were analysed. Institutional Ethics Committee approval was taken. Data with regard to demographic patterns, clinical profile, management approach and outcome, were collected and analysed.RESULTSOut of 531 newborns operated for gastrointestinal obstruction, 80% cases presented within first week of life. Male neonates were more commonly affected than females (M: F=2.2:1) and 58% cases were having low birth weight. Anorectal malformation was the commonest cause of obstruction (40.7% cases) followed by intestinal atresia (18% cases). Hirschsprung’s disease, malrotation, meconium ileus and hypertrophic pyloric stenosis were among the important aetiologies. The overall mortality in this study was 13% and septicaemia was the leading cause.CONCLUSIONSAetiology of gastrointestinal obstruction in newborn is diverse ranging from oesophageal atresia to anorectal malformations. Low birth weight and other co-morbidities are associated in many cases. The overall outcome is in improving trend due to gradual understanding about the pathology and advancement of neonatal care. Early diagnosis, surgical intervention and availability of well-equipped neonatal intensive care unit facility are essential for better survival.

Indications and complications of colostomy in newborn: our experience

Background: The aim of the present study was to evaluate the indications, complications and outcome of colostomy in newborn patients.Methods: This is a descriptive, retrospective study carried out in the Department of Pediatric surgery, Gandhi Medical College Bhopal to evaluate acceptance and outcome of colostomy in newborn over a one year period from March 2018 to March 2019. All newborn with diagnosis of anorectal malformation, Hirshsprungs disease and other emergency conditions underwent transverse loop colostomy by expert and trained pediatric surgeon.Results: Out of 40 patients operated complications of colostomy was seen in 18 (45%) patients. Most common complication was skin excoriation in 08 patients (20%), bleeding from stoma site in 02 (05%) cases. Stoma prolapse occurred in 02 (05%) patients. Wound infection, dehiscence, stomal stenosis and parastomal hernia was seen in one patient each 2 (2.5%). 2 (0.5%) patient died due to multiple congenital anomalies.Conclusions: Colostomy in children is associated with significant morbidity in developing country. To minimize these problems, the pediatric surgeon should focus on the management and early closure of a colostomy.

Functional Outcomes in Anorectal Malformation Patients Following Definitive Surgery

@#Introduction: Recently, management of anorectal malformation (ARM) emphasis on good intestinal functional outcomes after definitive procedure. This study analyzed the patients’ outcomes following operation related with the predictive variables. Methods: We applied the Krickenbeck classification and Rintala scoring system to define ARM type and functional outcomes, respectively. Results: This study ascertained 72 patients: 38 males and 34 females. According to Rintala scoring system, 94.4%, 90.2%, 60%, 83.3%, and 60% patients showed no soiling, no constipation, ability to hold back defecation, defecation frequency of every other day to twice a day, and feels/reports the urge to defecate, respectively. In addition, none of patients had either accident or social problem. Rintala score of normal and good have been shown in 14 (19.4%) and 55 (76.4%) patients, respectively. Female patients had a 4.2-times higher risk for showing a more/less often frequency of defecation compared with male patients (95% confidence interval (CI)=1.03-17.1; p=0.035). Conclusions: ARM patients’ functional outcomes after procedure in our institution are considered relatively good. In addition, the frequency of defecation in male patients after definitive surgery is better than female patients.

The analysis of continence after posterosagital anorectoplasty in anorectal malformation patients in Wahidin Sudirohusodo hospital of Makassar

Background: The management of anorectal malformations universally uses posterior sagittal anorectoplasty (PSARP) as standard surgery. The aim of this research was to determine continence in patients with anorectal malformation after PSARP action.Methods: This study was a descriptive analytical categorical research with cross sectional design. The data were obtained from patients’ medical records and questionnaire given to patients underwent PSARP in Wahidin Sudirohusodo Hospital and network hospitals from June 1 to December 1, 2018. The analysis was done to 60 children ranging from 5 to 8 years old. Most of them were male (56.7%) ranging from 5 to 6 years old (53.3%).Results: The results of the research indicated that based on malformation subtype, most of them suffered from rectourethra fistula (40.0%) with continence problem (61.7%). There was no significant correlation between sex and continence problem (p >0.05). However, it was seen that the percentage of continence subjects was higher in female (65.4%) than in male (58.8%), while the percentage of soiling and constipated subjects was higher in male than in female. Rectourethra fistula was found more frequently in male than in female (70.6%), while vestibuler fistula was found more frequently in female (69.2%). There was no significant correlation between age and defecation problem (p >0.05). However, the percentage of continence and soiling subjects was higher in 5-6 years children then the one for 7-8 year children, while the percentage of constipated subjects was higher in 7-8 year children (21.4%) than 5-8 year children (18.8%).Conclusions: Gender differences in the outcome of children with anorectal malformations must be considered. Men with perineal fistula were likely to experience continence and constipation than women with perineal fistulas. Women with perineal fistulas and vestibular fistulas had almost similar outcome.

Three-dimensional ultrasonography for evaluation of development of fetal anal sphincter / 中华医学超声杂志（电子版）

Objective To investigate the capability oftomography ultrasound (TUI) in evaluating the development of the normal anorectal structure to provide a reference for prenatal diagnosis of imperforate anus.Methods From June 2017 to July 2018,315 singleton pregnant women at 19-40 weeks of gestational age were selected from Beijing Tiantan Hospital,and they ranged in age from 23 to 40 years,with an average age of 29±3 years.The TUI technique was applied to measure the fetal anal sphincter diameter (ASD),anal canal diameter (ACD),and anal canal length (ACL).The correlation between ASD,ACD,or ACL and gestational age was analyzed by Pearson correlation analysis,and the regression equation and curve were established.The intraclass correlation coefficient (ICC) and 95％ confidence interval (CI) were used to evaluate the repeatability of the measurement ofASD,ACD,and ACL.Results The display rate of fetal anorectum in all fetuses by the TUI technique was 97.8％ (308/315).Pearson correlation analysis showed that the normal fetal ASD,ACD,and ACL were positively correlated with the gestational age of the fetus,and the regression equations are:ASD,Y=-0.001X2 + 0.421X-1.726 (r=0.83,P ＜ 0.01);ACD,Y=0.418X-4.207 (r=0.89,P ＜ 0.01);and ACL,Y=0.526X-1.062 (r=0.87,P ＜ 0.01).Conclusion TUI for assessment of fetal anorectum is feasible.This method can be applied to improve the display rate of fetal anorectum.

Currarino syndrome in an adult woman

Currarino syndrome is a hereditary disease characterized by the triad of sacral agenesis, anorectal malformation, and presacral mass. Most patients are diagnosed in childhood, and this condition rarely manifests in adulthood. In women, gynecological malformations associated with Currarino syndrome have been reported, such as bicornuate uterus, rectovaginal fistula, and septate uterus. We present a rare case of a 29-year-old woman with a suspected pelvic mass who was diagnosed with Currarino syndrome.

Anorectal Malformation with Associated Duodenal Obstruction and Uterus Didelphys / Malformación anorrectal asociada con obstrucción duodenal y útero didelfo

ABSTRACT Anorectal malformations have long been associated with multiple other anomalies, commonly referred to as the VACTERL complex. We present a case of a newborn baby girl with an unusually high number of associated anomalies, including the rarely encountered condition of uterus didelphys.

RESUMEN Las malformaciones anorrectales se han asociado por mucho tiempo con otras anomalías, conocidas comúnmente como asociación VACTERL. Presentamos el caso de una niña recién nacida con un número inusualmente alto de anomalías asociadas, incluyendo una condición raramente encontrada conocida como útero didelfo.

Study of MRI in congenital anorectal malformation / 实用放射学杂志

Objective To investigate the value of MRI on the perianal muscles developmental situation and associated malformations in congenital anorectal malformation.Methods The MRI features of 37 cases with congenital anorectal malformation were analyzed retrospectively.All of them were performed pelvic MRI,and 28 cases were underwent lumbosacral MRI additionally.Results Of the 37 cases,there were 9 cases with muscle belly slim and 6 cases with bilateral asymmetry of the levator ani muscle;12 cases with muscle belly slim,5 cases with bilateral asymmetry and 6 cases with irregular shape of anal sphincter.There was 1 case of ectopic kidney and fused kidney,8 cases of sacrum and coccyx vertebra deformation,4 cases of lipoma of filum terminale,3 cases of low cone,1 case of tethered cord,2 cases of cyst of coni medullaris and 3 cases of syringomyelus.There were 1 7 cases with fistula in congenital anorectal malformation proved by surgery,including 10 cases of urethral fistula,3 cases of bladder fistula,2 cases of perineal fistula,2 cases of vaginal fistula.9 cases with fistula could be clearly demonstrated on MRI,the others could not be visualized clearly. Conclusion MRI can clearly show the perianal muscles developmental situation and associated malformations in congenital anorectal malformation.MRI has high clinical value in congenital anorectal malformations.

Síndrome de Currarino como enfermedad infrecuente dentro del diagnóstico diferencial de la patología anexial oncológica / Currarino Syndrome as a Rare Disease in the Differential Diagnosis of Oncological Adnexal Pathology

El Síndrome de Currarino se define como la presencia de una tríada característica que asocia: estenosis anal, malformación sacrococcígea y masa presacra. La escasa sintomatología, caracterizada además por su inespecificidad, provoca que en muchas ocasiones el diagnóstico se realice durante la edad adulta y pueda confundirse con patologías ginecológicas, de origen predominantemente oncológico. El diagnóstico mediante pruebas de imagen, se realiza fundamentalmente a través de la resonancia magnética nuclear. La extirpación quirúrgica de la masa presacra unida al tratamiento sintomático del resto de la triada son la base terapéutica de esta infrecuente patología. Presentamos el caso de una paciente de 20 años de edad con estreñimiento crónico y dolor abdominal inespecífico. Es enviada al Servicio de Ginecología por la presencia de una masa de gran tamaño que se supone de origen anexial. Tras el estudio exhaustivo de dicha paciente, se llegó a la conclusión diagnóstica de que dicha tumoración corresponde a un meningocele, asociado a agenesia parcial sacra y estenosis anal; tríada que define al Síndrome de Currarino.

Currarino Syndrome is defined as the presence of a characteristic triad that associates anal stricture, sacrococcygeal malformation and presacral mass defines this syndrome. The scarce symptomology, also characterized by nonspecific symptoms, conditions that in many cases the diagnosis is made in adulthood and can be confused with gynecological pathologies. The diagnosis is made by imaging tests, fundamentally the NMR (nuclear magnetic resonance). The main treatment is the surgical excision of the mass presacra together with the symptomatic treatment of the rest of symptoms. We present the case of a 20-year-old patient with chronic constipation and abdominal pain that is referred to Gynecology due to the presence of a mass that is supposed to be adnexal. After the study of this patient is diagnosed a meningocele, associated with partial sacral agenesis and anal stricture, triad that defines Currarino Syndrome.

Advances in nervous system complications and intervention of human and rats anorectal malformation / 国际儿科学杂志

Anorectal malformations (ARM)comprise a broad spectrum of congenital disorders that account for 25 ％ of gastrointestinal malformations.Despite numerous technical advances for treatment of ARM,complications such as fecal incontinence and constipation still occur and can greatly deteriorate patients' quality of life.It is recognized that lumbosacral spinal cord anomalies in ARM has been an important factor affecting the fecal function after procedure.Researchers have found that lumbosacral myelodysplasia is the common seen complication of ARM and neural cells decreased in lumbosacral spinal cord by the study on animals and human.Due to numerous factos affecting nerve innervation on annrectus and pelvic floor muscle,this review summarizes the nervous system complications and abnormal intervention of human and rats ARMs.The developments of study on ARM complications and intervention are detailed.Then the new direction of the research about the anorectal malformation nervous system is put forward,and the new strategy of improving the prognosis of anorectal malformation surgery is explored.

Intestinal Obstruction in Pediatric Age Group: A Clinico- Pathological Study.

Background: Intestinal obstruction is one of the commonest surgical emergencies seen in pediatric patients. In last few decades, the scenario of diagnosis and management of pediatric intestinal obstruction has been changed due to better understanding of pathophysiology, improvement in diagnostic methods, availability of better antibiotics, safer pediatric anesthesia and advanced pre and post operative intensive care. Objectives: To study the epidemiology, clinical features and outcome of intestinal obstruction in paediatric patients. Methods: The present prospective study was conducted in department of General Surgery, Index Medical College, Indore in paediatric patients admitted with clinical features and diagnosis of intestinal obstruction between July 2014 to June 2016 (2 years). Results: Majority of cases of intestinal obstruction in our study were in age group of 0-1 years (70.2%), followed by the age group of 1-5 years (20.2%) and more than 5 years (9.6%). Conclusion: Majority of patients (70.2%) were of less than one year age, and male to female ratio was found to be 3.5:1. Congenial causes of intestinal obstruction were more common (61.7%) than the acquired causes. Intussusception (18.1%) was the commonest cause of intestinal obstruction in this series.

Long-term evaluation of fecal continence and quality of life in patients operated for anorectal malformations / Avaliação tardia da continência fecal e da qualidade de vida em pacientes operados por malformações anorretais

Summary Introduction: Patients operated for correction of anorectal malformations (ARM) can develop fecal incontinence, constipation, and soiling, with loss in quality of life. Objective: To evaluate, through the use of questionnaires, fecal continence, and quality of life of children in the late postoperative follow-up of ARM correction, both high and low. In addition, the levels of fecal continence and quality of life were compared with those of a control group. Method: A Fecal Continence Index Questionnaire (ICF) and a Questionnaire for Assessment of Quality of Life Related to Fecal Continence in Children and Adolescents (QQVCFCA) were administered to 63 patients with ARM, aged from 7 to 19 years, whose surgical treatment had been completed for at least 6 months. The patients were compared to a control group of 59 children. Results: In the control group, 25 (42.4%) patients had good continence and 34 (57.6%), normal continence. We found that the quality of life in children with ARM is compromised globally, in all areas and in the ICF questionnaire, compared to controls (p<0.001). There was no difference between patients with high and low defects. Thirty-two (50.8%) patients had other associated anomalies. Conclusion: In patients operated for ARM correction, quality of life and ICF were compromised, and there was no difference between patients with high-type and low-type of the disease. In about half the cases there are other associated malformations.

Resumo Introdução: os pacientes operados para correção de malformações anorretais (MAR) podem evoluir com incontinência fecal, constipação e soiling, com prejuízo na qualidade de vida. Objetivo: avaliar pela aplicação de questionários a continência fecal e a qualidade de vida de crianças no seguimento pós-operatório tardio de correção de MAR, formas altas e baixas. Também foram comparados os índices de continência fecal e qualidade de vida com um grupo controle de crianças. Método: foram utilizados o Questionário para o Índice de Continência Fecal (ICF) e o Questionário para Avaliar a Qualidade de Vida Relativa à Continência Fecal em Crianças e Adolescentes (QQVCFCA). Os questionários foram aplicados em 63 pacientes de 7 a 19 anos com MAR, com tratamento cirúrgico finalizado há pelo menos 6 meses. Os pacientes foram comparados com um grupo controle de 59 crianças. Resultados: nos pacientes do grupo controle, 25 (42,4%) apresentaram boa continência e 34 (57,6%), normal. A qualidade de vida nas crianças com MAR está comprometida globalmente, em todos os domínios e no ICF, quando comparada com a dos controles (p<0,001). Não houve diferença entre os pacientes com anomalias altas e anomalias baixas. Trinta e dois (50,8%) pacientes apresentaram outras anomalias associadas. Conclusão: nos pacientes operados para correção de MAR, qualidade de vida e ICF foram comprometidos, não havendo diferença entre os pacientes com as formas altas ou baixas da doença. Em cerca de metade dos casos existem outras malformações associadas.

Pitfall in the One-stage Management of Rectovestibular Fistula: A Rare Variant of Congenital Pouch Colon.

The gold standard for management of rectovestibular fistula is a three-stage procedure in many Western countries. This malformation has been corrected successfully in a single-stage in the Indian subcontinent. This report describes a girl with rectovestibular fistula who was later diagnosed to be a case of congenital pouch colon (CPC).

Caudal regression syndrome.

A case of caudal regression syndrome was reported in 20 wks foetus during routine foetal autopsy at GMCH, Chandigarh. The external examination showed 2 vessels in umbilicus. There was anteroposterior lengthening of skull. The anal opening was absent. The lower limbs were fused in thigh region with a small appendages attached to this on left side which also terminated in foot. The right foot had 5 toes and left foot had 3 toes. No external genitalia were seen. On internal examination, the gut was opening in a dilated cloaca like blind chamber. Kidneys were absent on both sides.X ray examination revealed small sacrum, femur, tibia in both the legs. Fibula was absent bilaterally. Caudal dysgenesis syndrome and caudal regression syndrome are broad terms that refer to a constellation of caudal congenital anomalies affecting caudal spine and spinal cord, hind gut, urogenital system, and the lower limbs. The etiology, incidence, causative factors of this case will be discussed in light of available literature

Study on the influencing factors of quality of life in children with congenital anorectal malformation / 国际儿科学杂志

Objective To analyse the influencing factors of quality of life (QOL)among postoperative children with congenital anorectal malformation (ARM), so as to provide evidence for developing comprehensive interventions and improve QOL of children with ARM.Methods The QOL of 100 children aged at 2 and 5 years old were evaluated by using the Chinese version of PedsQLTM4.0 scale, and the total and 4 dimensions scores of QOL were calculated.Univariate and multiple stepwise regression model was applied to analyse the influencing factors of the QOL.Results The results of univariate analysis among ARM children aged at 2 years old showed that the clinical classification, bowel disorders and family residence have different influence on QOL scores of the 4 dimensions and total scale (P ＜ 0.05).The results of univariate analysis among ARM children aged at 5 years old showed that the clinical classification, bowel disorders and maternal education had different influence on QOL scores of the 4 dimensions and total scale (P ＜ 0.05).The results of multiple stepwise regression model among ARM children aged at 2 years old showed that the major influence factor of QOL scores in physical function was family residence and bowel disorders(P ＜ 0.05), the major influence factor of QOL scores in social functioning was bowel disorders(P ＜ 0.05), the major influence factor of the total scores was bowel disorders and family residence(P ＜0.05).The results of multiple stepwise regression model among ARM children aged at 5 years old showed that the major influence factor of QOL scores in physical function was maternal occupation and clinical classification(P ＜ 0.05), the major influence factor of QOL scores in emotional functioning was bowel disorders(P ＜ 0.05) ,the major influence factor of QOL scores in social functioning was clinical classification and bowel disorders (P ＜ 0.05), the major influence factor of QOL scores in school performance was maternal occupation(P ＜ 0.05), the major influence factor of total scores was bowel disorders and clinical classification(P ＜ 0.05).Conclusion Family residence and bowel disorders have effct on QOL of ARM children aged at 2 years old.Maternal occupation, clinical classification and bowel disorders have effct on QOL of ARM children aged at 5 years old.We should take positive interventions on these influencing factors to improve QOL of children with ARM.

Expressions of glial cell line -derived neurotrophic factor and its tyrosine kinase receptor RET in the terminal rectum of fetal rats with congenital anorectal malformations at different gestational ages / 中华实用儿科临床杂志

Objective To explore the expressions and distributions of glial cell line -derived neurotrophic factor (GDNF)and itstyrosine kinase receptor RET in the terminal rectums of fetal rats with congenital anorectal malfor-mations (ARM)at different gestationalage,and to explore their effects on the enteric nervous system in the terminal rectum of ARMfetal rats.Methods Thirty -five SD pregnancy rats were divided into a saline group (n =1 0)and an ethylenethiourea experiment group (n =25)by simple randomized study.The fetal rats were removed from the pregnant rats at the gestational 1 6 d,1 8 d and 20 d.The fetal rats were divided into the saline control group,the ethylenethiourea control group (fetal rats without ARM)and the ethylenethiourea malformation group (ARM fetal rats)by the naked eye and dissecting microscope.HE staining was used to observe the morphology and the intestinal ganglion cells in the terminal rectum were counted.The immunohistochemical staining and Western blot methods were used to observe the distributions of GDNF and RET in the rectum at the gestational 1 6 d,1 8 d and 20 d.The quantitative real -time poly-merase chain reaction (qRT -PCR)was used to detect the expression of GDNF mRNA in the fetal rats in the terminal rectum at the gestational 1 6 d,1 8 d and 20 d.Results HE staining:the development of anorectal terminal in 3 groups of fetal rats was unclear at the gestational 1 6 d.A small amount of scattered nerve plexuses were observed in the muscu-lar layer.The nuclei were small and sparse.The axons and cytoplasms were less.The serosal layer,muscular layer,sub-mucosa,mucosal layer and glands in the terminal rectum were gradually clear in the saline control group and the ethyle-nethiourea control group at the gestational 1 8 d and 20 d.The intermuscular submucosal nerve plexuses increased gra-dually (1 1 .400 ±3.1 34 and 1 1 .200 ±3.425 at the gestational 1 8 d;66.1 00 ±4.954 and 67.600 ±5.481 at the gesta-tional 20 d).While,the layer was unclear in the ethylenethiourea malformation group and the nerve plexus was less (7.800 ±1 .989 at the gestational 1 8 d,and 25.200 ±3.048 at the gestational 20 d),and the difference was statistical-ly significant compared with 2 control groups (F =7.591 ,271 .833,all P 0.05);the expressions of GDNF and RET protein were 1 03.624 ±27.533 and 1 05.1 84 ±1 9.634 at the ges-tational 1 8 d;1 51 .496 ±33.622 and 1 50.738 ±21 .423 at the gestational 20 d in 2 control groups.Compared with the ethylenethiourea malformation group (79.1 69 ±1 1 .697 at the gestational 1 8 d;94.873 ±1 1 .309 at the gestational 20 d),and the difference were statistically significant (all P <0.05).Conclusions The expressions of GDNF and its tyrosine kinase receptor RET had a certain temporal correlation in the terminal rectum of normal fetal rats at different gestational ages and ARM.Moreover,the abnormal expressions of GDNF and its tyrosine kinase receptor RET in the dis-tal rectum of ARMfetal rats can affect the development of enteric nervous system.