European consensus meeting of ARM-Net members concerning diagnosis and early management of newborns with anorectal malformations.

The ARM-Net (anorectal malformation network) consortium held a consensus meeting in which the classification of ARM and preoperative workup were evaluated with the aim of improving monitoring of treatment and outcome. The Krickenbeck classification of ARM and preoperative workup suggested by Levitt and Peña, used as a template, were discussed, and a collaborative consensus was achieved. The Krickenbeck classification is appropriate in describing ARM for clinical use. The preoperative workup was slightly modified. In males with a visible fistula, no cross-table lateral X-ray is needed and an anoplasty or (mini-) posterior sagittal anorectoplasty can directly be performed. In females with a small vestibular fistula (Hegar size 5 mm, and in the meantime, gentle painless dilatations can be performed. In both male and female perineal fistula and either a low birth weight (<2,000 g) or severe associated congenital anomalies, prolonged preoperative painless dilatations might be indicated to decrease perioperative morbidity caused by general anesthesia. The Krickenbeck classification is appropriate in describing ARM for clinical use. Some minor modifications to the preoperative workup by Levitt and Peña have been introduced in order to refine terminology and establish a comprehensive preoperative workup

Hypothermia for encephalopathy in low and middle-income countries (HELIX): Study protocol for a randomised controlled trial

BACKGROUND: Therapeutic hypothermia reduces death and disability after moderate or severe neonatal encephalopathy in high-income countries and is used as standard therapy in these settings. However, the safety and efficacy of cooling therapy in low- and middle-income countries (LMICs), where 99% of the disease burden occurs, remains unclear. We will examine whether whole body cooling reduces death or neurodisability at 18-22 months after neonatal encephalopathy, in LMICs. METHODS: We will randomly allocate 408 term or near-term babies (aged ≤ 6 h) with moderate or severe neonatal encephalopathy admitted to public sector neonatal units in LMIC countries (India, Bangladesh or Sri Lanka), to either usual care alone or whole-body cooling with usual care. Babies allocated to the cooling arm will have core body temperature maintained at 33.5 °C using a servo-controlled cooling device for 72 h, followed by re-warming at 0.5 °C per hour. All babies will have detailed infection screening at the time of recruitment and 3 Telsa cerebral magnetic resonance imaging and spectroscopy at 1-2 weeks after birth. Our primary endpoint is death or moderate or severe disability at the age of 18 months. DISCUSSION: Upon completion, HELIX will be the largest cooling trial in neonatal encephalopathy and will provide a definitive answer regarding the safety and efficacy of cooling therapy for neonatal encephalopathy in LMICs. The trial will also provide important data about the influence of co-existent perinatal infection on the efficacy of hypothermic neuroprotection. TRIAL REGISTRATION: ClinicalTrials.gov, NCT02387385. Registered on 27 February 2015

Treatments for intractable constipation in childhood

Objectives This is a protocol for a Cochrane Review (intervention). The objectives are as follows: To evaluate the efficacy and safety of treatments used for intractable constipation in children

How do we define therapy-resistant Constipation in Children 4-18 years old? A systematic review with meta-narrative synthesis Data extraction

Background Therapy resistant constipation often is a frustrating clinical entity recognised by the persistence of infrequent and painful bowel movements fecal incontinence and abdominal pain despite intensive treatment. It is important to clearly define therapy resistant constipation before children are subjected to invasive diagnostic and therapeutic procedures. Aim To conduct a systematic review determining how pediatric interventional studies define therapy resistant constipation. Method We searched CENTRAL, MEDLINE, Embase, WHO ICTR and ClinicalTrials.gov. Studies that included patients with therapy resistant constipation were identified. Data were extracted on criteria used for defining therapy resistant constipation and reported using meta-narrative approach highlighting areas of convergence and divergence in the findings. Results A total of 1553 abstracts were screened in duplicate, and 47 studies were included in the review. There were at least 7 definitions used in the paediatric literature to define medically resistant constipation. The term intractable was used in 24 articles and 21 used the term refractory to describe therapy resistant constipation. Out of them only 14 articles have attempted to provide an explicit definition including a predefined time and prior therapy. There were 10 studies without a clear definition for therapy resistant constipation. The duration before being diagnosed as therapy resistant constipation varied from 1 months to 2 years among studies. Seven studies employed the Rome criteria (Rome III or Rome IV) to characterising constipation, while 5 adopted the Rome III and European and North American paediatric societies definition of paediatric gastroenterology, hepatology and nutrition guideline of management of constipation in children. Conclusion The current literature has no explicit definition for therapy resistant constipation in children. There is a need for a detailed consensus definition to ensure consistency of future research and to avoid unnecessary, and maybe even harmful, invasive diagnostic and therapeutic interventions

Prevalence of Acanthosis Nigricans in an urban population in Sri Lanka and its utility to detect metabolic syndrome

<p>Abstract</p> <p>Background</p> <p>Insulin resistance (IR) plays a major role in the pathogenesis of metabolic syndrome. Acanthosis nigricans (AN) is an easily detectable skin condition that is strongly associated with IR. The aims of this study were, firstly, to investigate the prevalence of AN among adults in an urban Sri Lankan community and secondly, to describe its utility to detect metabolic syndrome.</p> <p>Findings</p> <p>In a community based investigation, 35-64 year adults who were selected using stratified random sampling, underwent interview, clinical examination, liver ultrasound scanning, and biochemical and serological tests. Metabolic syndrome was diagnosed on revised ATP III criteria for Asian populations. AN was identified by the presence of dark, thick, velvety skin in the neck.</p> <p>2957 subjects were included in this analysis. The prevalence of AN, metabolic syndrome and type 2 diabetes mellitus were 17.4%, 34.8% and 19.6%, respectively. There was a strong association between AN and metabolic syndrome. The sensitivity, specificity, positive predictive value and negative predictive value of AN to detect metabolic syndrome were 28.2%, 89.0%, 45.9% and 79.0% for males, and 29.2%, 88.4%, 65.6% and 62.3% for females, respectively.</p> <p>Conclusions</p> <p>AN was common in our study population, and although it did not have a high enough sensitivity to be utilized as a screening test for metabolic syndrome, the presence of AN strongly predicts metabolic syndrome.</p

Prevalence of inflammatory bowel disease in two districts of Sri Lanka: a hospital based survey

<p>Abstract</p> <p>Background</p> <p>Inflammatory bowel disease (IBD) is being increasingly diagnosed in Asia. However there are few epidemiological data from the region.</p> <p>Methods</p> <p>To determine prevalence and clinical characteristics of IBD, a hospital-based survey was performed in the Colombo and Gampaha districts (combined population 4.5 million) in Sri Lanka. Patients with established ulcerative colitis (UC) and Crohn's disease (CD), who were permanent residents of these adjoining districts, were recruited from hospital registries and out-patient clinics. Clinical information was obtained from medical records and patient interviews.</p> <p>Results</p> <p>There were 295 cases of IBD (UC = 240, CD = 55), of which 34 (UC = 30, CD = 4) were newly diagnosed during the study year. The prevalence rate for UC was 5.3/100,000 (95% CI 5.0-5.6/100,000), and CD was 1.2/100,000 (95% CI 1.0-1.4/100,000). The incidence rates were 0.69/100,000 (95% CI 0.44-0.94/100,000) for UC and 0.09/100,000 (95% CI 0.002-0.18/100,000) for CD. Female:male ratios were 1.5 for UC and 1.0 for CD. Mean age at diagnosis was (males and females) 36.6 and 38.1y for UC and 33.4 and 36.2y for CD. Among UC patients, 51.1% had proctitis and at presentation 58.4% had mild disease. 80% of CD patients had only large bowel involvement. Few patients had undergone surgery.</p> <p>Conclusions</p> <p>The prevalence of IBD in this population was low compared to Western populations, but similar to some in Asia. There was a female preponderance for UC. UC was mainly mild, distal or left-sided, while CD mainly involved the large bowel.</p

Outcome of pediatric inflammatory bowel disease in Asian children: a multinational 1-year follow-up study

Background Epidemiological data on pediatric inflammatory bowel disease (PIBD) have been reported in Asian countries. However, short-term follow-up data, especially in Southeast Asian countries, are limited. Purpose Analyze and compare the baseline and 1-year follow-up (1FU) data for PIBD in Asian children. Methods The multinational network included patients with PIBD (aged <19 years) in 5 Asian countries (Malaysia, Philippines, Singapore, Sri Lanka, and Thailand). The diagnosis of PIBD requires gastrointestinal endoscopy. The patients' demographics, clinical information, disease- related outcomes, and treatment data at 1FU were collected. Results In 1995–2021, 368 patients were enrolled (Crohn disease [CD], 56.8%; ulcerative colitis [UC], 38%; and inflammatory bowel disease [IBD]-unclassified, 5.2%). At 1FU, symptoms including diarrhea, bloody stools, and nausea/vomiting subsided in <3%, while abdominal pain persisted in 10.5% of patients with CD and 7.1% of patients with UC. Assessment endoscopy was performed at 1FU in 38% of CD and 31% of UC cases, of which 21% and 23% showed mucosal healing, respectively. Oral prednisolone was administered to 55.3% of patients at diagnosis and 26.8% at 1FU, while infliximab was administered to 2.5% and 7.2% of patients at diagnosis and 1FU, respectively. Independent factors of 1-year clinical remission for CD were oral prednisolone (odds ratio [OR], 0.20; 95% confidence interval [CI], 0.06–0.68), antibiotic use (OR, 0.09; 95% CI, 0.01–0.54), and immunomodulator use (OR, 5.26; 95% CI, 1.52–18.22). A history of weight loss at diagnosis was the only independent risk factor of an IBD flare by 1FU (OR, 2.01; 95% CI, 1.12–3.63). Conclusion The proportion of children with PIBD and abdominal pain at 1FU remained high. The rates of repeat endoscopy and infliximab use were suboptimal with high rates of systemic corticosteroid use. Quality improvement based on the aforementioned predictors may enhance PIBD care in this geographic region or similar settings

Disease phenotypic and outcome of very-early onset inflammatory bowel disease in Asian children: an understudied population

BackgroundThere is a paucity of knowledge on disease phenotype and outcome of very early-onset (VEO) inflammatory bowel disease (VEO-IBD) from recently developed and developing countries, including from Southeast Asia. We studied disease phenotype, clinical characteristics, management and outcome of VEO-IBD in South and Southeast Asian children.Materials and methodsWe extracted data from a multicentre Asian pediatric (onset &lt;18 years) IBD registry. VEO- and later-onset pediatric (LO-p) IBD were defined as onset of disease &lt;6 years and ≥6 years, respectively. We excluded monogenic IBD.ResultsOf 440 children with IBD cases; 112 (25.5%) were VEO-IBD; Crohn's disease (CD) 36 (32.1%); ulcerative colitis (UC) 68 (60.7%), and IBD-unspecified 7 (7.1%). UC was more common in VEO-IBD while CD more common in LO-pIBD (CD = 68.9% vs. UC = 25.9%; p &lt; 0.001). Disease location/extent of disease and disease severity were similar in both age groups for both CD and UC. For CD, inflammatory disease behavior was equally common in both age group (77.8% in VEO-IBD vs. 76.6% of LO-pIBD), majority had isolated colonic disease (27.8% VEO-IBD vs. 36.3% LO-pIBD), while stricturing and penetrating diseases were not observed in VEO-CD, but noted in 4.9% and 8.4% of LO-pCD, respectively. Among UC cases, pancolitis was observed in 60.3% of VEO-IBD vs. 65.9% of LO-pIBD. Most UC never had severe disease regardless of age group. Five years after diagnosis, VEO-IBD were more likely to have corticosteroids, immunomodulators or biologics than LO-pIBD. Despite this, inactive/mild disease activity was the predominant outcome at 5 year follow up for both VEO-CD (98.2%) and VEO- UC (96.1%). Bowel surgery rate was 2.4% and 1.7% for VEO- and LO-IBD at 5 years, respectively.ConclusionsDespite differences in disease phenotype at diagnosis, disease behaviour, location/extent and disease severity were similar between VEO- and LO-IBD, with a comparable overall clinical remission rates between both age groups at 5 years after diagnosis

Constipation in Children: Novel Insight Into Epidemiology, Pathophysiology and Management

Constipation in children is a common health problem affecting 0.7% to 29.6% children across the world. Exact etiology for developing symptoms is not clear in children and the majority is considered to have functional constipation. Alteration of rectal and pelvic floor function through the brain-gut axis seems to play a crucial role in the etiology. The diagnosis is often a symptom-based clinical process. Recently developed Rome III diagnostic criteria looks promising, both in clinical and research fields. Laboratory investigations such as barium enema, colonoscopy, anorectal manometry and colonic transit studies are rarely indicated except in those who do not respond to standard management. Treatment of childhood constipation involves several facets including education and demystification, toilet training, rational use of laxatives for disimpaction and maintenance and regular follow-up. Surgical options should be considered only when medical therapy fails in long standing constipation. Since most of the management strategies of childhood constipation are not evidence-based, high-quality randomized controlled trials are required to assess the efficacy of currently available or newly emerging therapeutic options. Contrary to the common belief that children outgrow constipation as they grow up, a sizable percentage continue to have symptoms beyond puberty