dos Santos, Ana Luiza Melo;de Melo Santos, Helen;Nogueira, Marina Bettiol;Tavora, Hugo Tadashi Oshiro;da Cunha, Maria de Lourdes Jaborandy Paim;de Melo Seixas, Renata Belem Pessoa;Monte, Luciana de Freitas Velloso;de Carvalho, Elisa
Purpose: The objective of this study was to describe the clinical phenotypes of children and adolescents with cystic fibrosis (CF); and to assess the role of pancreatic insufficiency and neonatal screening in diagnosis. Methods: A cross-sectional study was conducted, which included 77 patients attending a reference center of CF between 2014 and 2016. Epidemiological data, anthropometric measurements, and the presence of pulmonary, pancreatic, gastrointestinal and hepatobiliary manifestations were evaluated based on clinical data and complementary examinations. Results: Of the 77 patients, 51.9% were male, with a median age of 147 months (7.0-297.0 months), and the majority showed adequate nutritional status. The most common phenotype was pulmonary (92.2%), followed by pancreatic (87.0%), with pancreatic insufficiency in most cases. Gastrointestinal manifestation occurred in 46.8%, with constipation being the more common factor. Hepatobiliary disease occurred in 62.3% of patients. The group with pancreatic insufficiency was diagnosed earlier (5.0 months) when compared to the group with sufficiency (84.0 months) (p=0.01). The age of diagnosis was reduced following implementation of neonatal screening protocols for CF (6.0 months before vs. 3.0 months after, p=0.02). Conclusion: The pulmonary phenotype was the most common, although extrapulmonary manifestations were frequent and clinically relevant, and should mandate early detection and treatment. Neonatal screening for CF led to earlier diagnosis in patients with pancreatic failure, and therefore, should be adopted universally.
Purpose: Recently well-developed anorectal function tests have revealed that there is an obvious pelvic floor dyssynergia (PFD) pattern in pediatric patients with constipation, as well as in adult's. The use of biofeedback therapy (BT) has been widely implemented in adult PFD patients; however, this approach has only rarely been considered for pediatric PFD patients. Therefore, we assessed the effectiveness of BT in children with PFD. Methods: We studied 70 children with PFD, who were referred to the department of pediatrics at the Asan Medical Center for the management of soiling or chronic constipation from September 2002 to February 2005. Diagnosis of PFD and assessment of the efficacy of BT for PFD treatment were carried out along with several ano-rectal function tests (cine-defecography, ano-rectal manometry, balloon expulsion test and intra-anal EMG); in addition, a questionnaire was administered. The BT based intra-anal EMG was performed. A follow-up telephone interview was performed more than 6 months later. Results: Most of the symptoms and results of the ano-rectal function tests were statistically improved after BT. In comparisons between the BT and control groups (BT refusal group due to poor compliance), the symptoms were statistically improved at follow-up. Therefore, for the shortterm improvement of symptoms, BT was better than conservative therapy alone. The negative feelings associated with ano-rectal function testing and BT were directly associated with failure or success of therapy. Conclusion: Pediatric patients with constipation or soiling that presented with an obvious PFD pattern showed that BT was a useful therapeutic tool for rapid improvement of symptoms.
We present a case of a colonic involvement associated with necrotizing pancreatitis, with a review of the literature. A 10 year old boy had an appendectomy at the local clinic ten days ago. On admission, he complained nausea, vomiting and severe constipation. His abdomen was distended and he had tenderness on the left abdomen. Laboratory and radiologic studies revealed findings consistent with acute pancreatitis with colonic complication. He was treated conservatively for 30 days but did not improve. On hospital 30th day, abdominal pain developed and his vital sign changed. Abdominal CT suggested ischemic change of the transverse colon. At laparotomy, the left colon showed stenosis. The greatly distended transverse colon was resected and a transverse end colostomy was done. He was discharged at postoperative 45th day with improvement and colostomy closure was performed 8 months later.
The authors report a case of Currarino triad which had a congenital anorectal stenosis associated with a sacral defect and a presacral mass. A 1-year-old female presented with constipation since birth. Neurological deficits were not found on admission. She had had a diverting colostomy due to anorectal stenosis at another hospital before admission. Lumbar X-ray films showed bony defect caudal to the third sacral vertebra. Magnetic resonance image demonstrated a round cystic pelvic mass which was connected with a dural sac via anterior sacral defect. Posterior approach with Intradural removal of the presacral cystic mass was performed and followed by anoplasty by a pediatric surgeon. The cystic mass was verified histologically as mature teratoma with cystic change. Postoperatively, the urinary function and bowel movement remained intact. Currarino triad should be suspected and evaluated physically and radiographically in a case of congenital anorectal stenosis. Prompt recognition and close cooperation between pediatric surgeons and neurosurgeons is advisable to ensure adequate surgical treatment.
Immature ganglion cell (IGC) is known for its relationship with intestinal motility and its impact on postoperative functional outcomes of Hirschsprung's disease (HD). There are few studies on the relationship between intestinal dysmotility and IGC in HD patients. 67 patients pathologically diagnosed with HD and who received definitive operation in Seoul National University Children's Hospital from 2010 to 2011 were included. 10 patients were excluded due to inadequate immunohistochemical staining results. The proximal end of resected ganglionic segment was evaluated with immunohistochemistry examination with MAP-2, a marker of ganglionic cells and bcl-2, a marker of IGCs The median age at operation was 155 (15-4678) day-old. 55 (96.5%) patients positive for bcl-2, were regarded as having IGC, and 2 (3.5%) patients positive for MAP-2 but negative for bcl-2, were regarded as having only mature ganglion cells. In the bcl-2 positive group, there were 7 patients (12.7%) with constipation, 15 patients (27.3%) with soiling, 3 patients (5.5%) with perianal excoriation and 6 patients (10.9%) with medication use. In bcl-2 negative group, intestinal dysmotility was not seen. There was no statistical significance in the two groups. Considering that HD is diagnosed at a young age, the rate of IGC present is very high and it might be inappropriate to relate IGC to functional outcome at young ages.
Functional gastrointestinal disorders (FGIDs) are classified as a combination of persistent gastrointestinal symptoms. The Rome IV criteria can elucidate several factors in the pathogenesis of FGIDs. The frequency of FGIDs can differ between clinical and nonclinical settings and between geographic regions. To determine the global prevalence of FGIDs in neonates and toddlers according to the Rome IV criteria. We included cohort and descriptive observational studies reporting the prevalence of FGIDs according to the Rome IV criteria in children aged 0-48 months. We searched the Medline, Embase, Lilacs, and CENTRAL databases from May 2016 to the present day. Furthermore, unpublished literature was searched to supplement this information. The Strengthening the Reporting of Observational Studies in Epidemiology statement was used to evaluate the risk of bias. A meta-analysis of the proportions was performed using MetaProp in R. The results are reported in forest plots. We identified and analyzed 15 studies comprising 48,325 participants. Six studies were conducted in Europe, three in Latin America, two in North America, and four in Asia. Most participants were 12-48 months old (61.0%) and were recruited from the community. The global prevalence of FGIDs was 22.0% (95% confidence interval, 15-31%). The most common disorder was functional constipation (9.0%), followed by infant regurgitation syndrome (8.0%). Its prevalence was higher in the Americas (28.0%). FGIDs, as defined by the Rome IV criteria, are present in 22% of children, and the most common primary disorder is functional constipation. A higher prevalence of FGIDs has been reported in America.
The influence of nutrition during early life on physical growth as well as mental development has been thoroughly discussed in the literature. The physical dimensions of the body are greatly influenced by nutrition, particularly during the period of rapid growth in early childhood. Nutritional status affects every pediatric patient's response toillness. Good nutrition is important for achieving normal growth and development. It is indicated that permanent impairment of the central nervous system may result from dietary restriction of imbalance during certain periods of life. If children under 3 years of age show a good nutritional status, it may be assumed that they are well nourished. Several common diseases of children such as iron deficiency, chronic constipation and atopic dermatitis are known food related diseases. Patients with chronic illness and those at risk of malnutrition should have detailed nutritional assessments done. Components of a complete nutritional assessment include a medical history, nutritional history including dietary intake, physical examination, anthropometrics (weight, length or stature, head circumference, midarm circumference, and triceps skinfold thickness), pubertal staging, skeletal maturity staging, and biochemical tests of nutritional status. The use of age, gender, and disease-specific growth charts is essential in assessing nutritional status and monitoring nutrition interventions. Nutrition assessment and dietary counseling is helpful for the cure of disease, and moreover, the prevention of illness.
Conventional treatment of Hirschsprung's disease consists of initial colostomy followed by pull-through operation. But, the treatment of Hirschsprung's disease has been changed along with the development of new surgical technique. Since 1995, endo-GIA has been available at our hospital and one stage Duhamel operation has been performed for neonatal Hirschsprung's disease. Between May 1995 and April 2006, 26 neonates have been treated with one stage pull-through operation by one pediatric surgeon at HanYang University Hospital. The sex ratio was 4.2:1 with male predominance. Clinical findings included abdominal distension (96.2 %), vomiting (50.0 %), delayed passage of meconium (46.2 %), constipation (23.1 %), and enterocolitis (15.4 %). Twenty two cases (84.6 %) were short-segment and 4 cases (15.4 %) were long-segment disease, of which 2 cases were total colon aganglionosis. One of the two patients with total colonic aganglionosis had double transition zones - distal ileum and hepatic flexure of the colon. The average age at operation was $14.56{\pm}8.77$ days and the average weight at operation was $3.26{\pm}0.66kg$. Primary Duhamel operations were performed in 25 patients and Soave-Boley operations was performed in one patient. The endo-GIA 35 (Ethicon, USA) was used from 1995 until 1997, and after that endo-GIA 60 (USSC, USA) was used. The average Duhamel operation time was $88.57{\pm}22.80$ minutes. Wound abscess (n = 2) and septum formation (n =1) occurred after Duhamel operation. Bowel function was normalized in 59 % within 3 months and in 95% within 1 year after operation. There was no mortality after one stage pull-through operation in neonate.
Purpose: The aim of this study was to evaluate the clinical features and factors contributing to treatment outcome for chronic functional constipation in children. Methods: We analyzed the medical records of patients with constipation, who visited the inpatient or outpatient clinic of the Department of Pediatrics of Pusan National University Hospital, between January 1998 and December 2007. The clinical features, outcomes, and factors affecting the treatment response according to the main drug (lactulose vs. PEG 4000) were analyzed retrospectively. Results: Two hundred forty children (142 males and 98 females) were enrolled in this study. The mean age was 51.2${\pm}$37.9 months. The duration of symptoms was 32.6${\pm}$33.7 months. The accompanying symptoms were as follows: encopresis, 91 (30.4%); abdominal pain, 76 (31.6%); and blood-tinged stool, 37 (15.4%). The treatment response was achieved earlier in females (p<0.001), patients with accompanying symptoms (p<0.05), and patients treated with PEG 4000 (p=0.001). The duration of symptoms (p<0.05) and stool frequency before treatment (p<0.05) were related to a delayed treatment response. Relapse occurred in 7 children, all of whom were treated successfully later. Conclusion: Factors contributing to treatment response are female gender, accompanying symptoms, duration of symptoms, and stool frequency before treatment. PEG 4000 is superior to lactulose in response time and taken into consideration as a primary drug for the treatment of functional constipation of children. Early treatment and sufficient treatment time may also be important factors to achieve an early response and prevent relapse.
Objective : In order to review the studies related to the treatment of children by aupuncture or mo ibustion Methods : We referred to a Pubmed site by using searching word of "the terms of pediatrics(for e ample child children infant baby pediatrics etc and acupuncture" Results : 40 journals with 63 papers were searched 50 papers were reported Conclusion : 1 We found 63 articles in 40 journals which were Pediatrics J Tradit chin Med Am Fam Physician Anaesthesia linical research was the most of these studies and the pattern of the rest were review or clinical conferences 3 We analy ed articles and found many articles of nocturnal enuresis asthma bronchitis postoperative vomitting constipation etc 4 The result of clinical studies represented the significant cure rate owever the cases of sensorineural hearing loss vomitting after tonsillectomy e ercise induced asthma were not 5 It was ta en to research for laser acupressure bloodletting ear acupuncture etc as well as body acupuncture in the area of pediatric acupuncture treatment as alternative therapy which was composed of the lower stimulation than adult.
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