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BACKGROUND: Despite the extensive reporting of pediatric ulcerative colitis (UC) from industrialized developed countries, reports from developing countries are limited to small-case series from single centers. The objective of our large multicenter study was to determine the clinical, laboratory, endoscopic characteristics of UC in children from a developing country, Saudi Arabia. DESIGN AND SETTINGS: A retrospective study of children diagnosed with UC under the age of 18 years during the period from 2003 to 2012. METHODS: Patients enrolled from 15 medical centers from different regions in Saudi Arabia. A unified database collection form specifically designed for this study was completed by all participating centers. RESULTS: A total of 188 children were diagnosed with UC during the study period (97 males [51.6%] and 91 females [48.4%]). The mean age at diagnosis was 9.1 years, and the mean duration of symptoms before diagnosis was 8.7 months. Consanguinity was present in 57 cases (32.6%), and the family history of inflammatory bowel disease (IBD) was noted in 16 cases (9%). The most common clinical presentation was blood in stool (90%), followed by diarrhea (86%) and abdominal pain (62%). Laboratory investigations revealed elevated erythrocyte sedimentation rate (82%), anemia (75%), thrombocytosis (72%), and hypoalbuminemia (33%). The extent of the disease was pan colonic in 46.1%, and confined to left side of colon and rectum in 23% and 9.6% of the cases, respectively. CONCLUSION: This demographically pediatric IBD retrospective study revealed age-related variation in the distribution of IBD. Clinical presentation, with a high prevalence of positive consanguinity and positive family history, was noted in young patients with UC. The data from this study indicate that UC is increasingly recognized in Saudi Arabia and show many similarities to data from North America and Europe.

Angiodysplasia is a term used to describe distinct gastrointestinal mucosal ectasias that are not associated with cutaneous lesions, systemic vascular disease or a familial syndrome. Seventy-seven percent of angiodysplasia are located in the cecum and/or ascending colon. Fifteen percent are located in the jejunum and/or ileum and the remainder are distributed throughout the alimentary tract. Most commonly, the angiodysplastic lesions are typically seen in elderly patients of both genders, although gastric and duodenal lesions have been reported occasionally in subjects within the third decade of life. However, data on infants and children are scarce. We describe three cases (ages 7 days, 2 years, and 5 years) who presented to our unit with gastrointestinal bleeding. One of these patients developed moderate-to-severe symptoms and was blood-transfusion dependent. She was misdiagnosed as having inflammatory bowel disease and underwent a total colectomy and ileoanal anastomosis. The other two patients were managed conservatively for up to 5 years with no further bleeding.

Aim: The aim of this study is to determine the frequency of celiac disease (CD) in a group of children with juvenile rheumatoid arthritis (JRA) and determine the correlation between the presence of the serologic markers and the histological diagnosis of CD. Patients and Methods : Forty-two children (24 females) with JRA, aged between 5-15 years underwent study of serologic markers for CD (gliadin-IgA, gliadin-IgG, reticulin and endomysium-IgA antibodies). Endoscopic intestinal biopsy was performed in patients who had positive serologic markers for CD. The diagnosis of CD was based on the classic finding of villous atrophy and crypt hypertrophy. Results: Eighteen patients (42.8%) had serologic markers for CD; ten of them with a systemic form, five with a polyarticular form and three with a pauciarticular form of JRA. Levels of AGA -IgG were high in 14 patients (77.8%), four patients (22.2%) had high levels of AGA-IgA and seven patients (38.9%) had anti-endomysium antibodies (AEA). One patient had anti-reticulin antibodies (ARA) 5.5%. Sixteen patients underwent intestinal biopsy; in only one patient with AEA antibodies (2.38%), biopsy revealed typical finding of CD. The patient with CD showed improvement in both growth parameter as well as articular symptoms after starting gluten-free diet Conclusion: Our study shows that the screening for silent CD among children with JRA may be useful. Those patients with AEA need further follow up since these antibodies are quite sensitive and specific for CD

IntroductionThe diagnosis and management of cow's milk allergy (CMA) is a topic of debate and controversy. Our aim was to compare the opinions of expert groups from the Middle East (n = 14) and the European Society of Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) (n = 13).MethodsThese Expert groups voted on statements that were developed by the ESPGHAN group and published in a recent position paper. The voting outcome was compared.ResultsOverall, there was consensus amongst both groups of experts. Experts agreed that symptoms of crying, irritability and colic, as single manifestation, are not suggestive of CMA. They agreed that amino-acid based formula (AAF) should be reserved for severe cases (e.g., malnutrition and anaphylaxis) and that there is insufficient evidence to recommend a step-down approach. There was no unanimous consensus on the statement that a cow's milk based extensively hydrolysed formula (eHF) should be the first choice as a diagnostic elimination diet in mild/moderate cases. Although the statements regarding the role for hydrolysed rice formula as a diagnostic and therapeutic elimination diet were accepted, 3/27 disagreed. The votes regarding soy formula highlight the differences in opinion in the role of soy protein in CMA dietary treatment. Generally, soy-based formula is seldom available in the Middle-East region. All ESPGHAN experts agreed that there is insufficient evidence that the addition of probiotics, prebiotics and synbiotics increase the efficacy of elimination diets regarding CMA symptoms (despite other benefits such as decrease of infections and antibiotic intake), whereas 3/14 of the Middle East group thought there was sufficient evidence.DiscussionDifferences in voting are related to geographical, cultural and other conditions, such as cost and availability. This emphasizes the need to develop region-specific guidelines considering social and cultural conditions, and to perform further research in this area.

Background and Aims. Crohn’s disease (CD) is an evolving disease in KSA. Little is known about its characteristics in the Saudi population. The aims of this study were to describe the characteristics of Saudi children with CD and to determine whether the characteristics of CD in KSA are different from those seen in Western countries. Methods. In this study, children younger than eighteen years of age diagnosed with CD between January 2003 and December 2012 were included. Results. Of 330 patients identified, 186 (56.4%) were males. The median age at diagnosis was 15.8 years. A positive family history for IBD in first-degree relatives occurred in 13.6% of patients. The most common symptoms were abdominal pain (84.2%), weight loss (75.2%), and diarrhea (71.8%). The main disease location was ileocolonic (42.1%) and the main disease behavior was nonstricturing and nonpenetrating (63.6%). Perianal involvement was seen in 60 (18.2%) patients. Laboratory findings revealed anemia in 57.9% of patients, low albumin in 34.5%, and high CRP in 39.4%. Conclusions. Saudi children with CD have lower frequency of first-degree relatives with IBD, lower prevalence of early onset disease, longer diagnostic delay, higher prevalence of growth failure, and greater frequency of stricturing and penetrating disease behavior compared to Western patients. [ABSTRACT FROM AUTHOR]

Background: Cow's milk allergy (CMA) and cow's milk intolerance (CMI) are the major cow's milk disorders observed in infants and young children. This study investigates, for the first time, physician knowledge regarding CMA and CMI prevalence, diagnosis, and management in the Middle East and North Africa (MENA) region. In addition, we explore the role of goat milk-based formula as an alternative in infants suffering from CMI. Method: This cross-sectional survey was conducted from December 2020 to February 2021. A convenience sample of 2500 MENA-based physicians received the questionnaire, developed by a working group of pediatric experts. Results: 1868 physicians completed the questionnaire, including pediatric specialists (80.8%), training physicians (0.2%), dermatologists (0.1%), family/general physicians (12.9%), neonatologists (3.6%), neurosurgeons (0.2%), allergy nurse specialists (0.3%), pharmacists (2.1%), and public health workers (0.1%). Differentiation between CMA and CMI was recognized by the majority of respondents (80.7%), for which the majority of respondents (35.4%) identified that the elimination and challenge test was the best test to differentiate CMA from CMI, whereas 30.7% and 5.4% preferred the immunoglobulin E (IgE) test and skin prick test, respectively. In addition, 28.5% of respondents reported that there is no confirmatory test to differentiate CMA from CMI. The majority of respondents (47.3%) reported that amino acid-based formula (AAF)/ extensively hydrolyzed formula (EHF) is the cornerstone for the management of CMA. However, most respondents (33.7%) reported that lactose avoidance was best for the management of CMI. Overall, 65% of the respondents were aware of nutritionally adapted goat's milk formula as an alternative to cow's milk products and 37% would recommend its routine use in infants (≤2 years of age). Conclusion: The results of this survey demonstrate that the majority of physicians are aware of the underlying pathophysiology and management of CMA and CMI. However, a significant proportion of physicians do not follow the clinical guidelines concerning CMA/CMI diagnosis and management. Notably, this survey identified that goat's milk formulas may offer a suitable alternative to AAF/EHF in infants with CMI as they contain β-casein protein which is easily digestible. In addition, goat's milk formulas contain higher levels of oligosaccharides and medium-chained fatty acids compared with standard cow's milk formulas, yet further clinical trials are warranted to support the inclusion of goat's milk formulas in clinical guidelines. [ABSTRACT FROM AUTHOR]

Background/Aim: Linear growth impairment (LGI) is one of the most important features peculiar to children with inflammatory bowel disease (IBD). The aim of this report is to define the impact of IBD on the linear growth of children in the Kingdom of Saudi Arabia (KSA). Setting and Design: Multicenter retrospective study. Patients and Methods: Data from a cohort of newly- diagnosed children with IBD from 2003 to 2012 were analyzed retrospectively. The diagnosis of IBD was confirmed in accordance with the published criteria. Length/height for age was measured at diagnosis. The World Health Organization (WHO) reference was used and LGI was defined by length/height for age <-2 standard deviation. Chi-square test was used to test the significance of estimates and a P < 0.05 was considered significant. Results: There were 374 children from 0.33 to 16 years of age, including 119 ulcerative colitis (UC) (32%), and 255 Crohn's disease (CD) (68%) patients. The prevalence of LGI was 26%, 28%, and 21% in IBD, CD, and UC, respectively. In children below 10 years, LGI was significantly more common in CD (P = 0.010), while in UC children, it was more common in older children (P = 0.011). Conclusion: This study demonstrates a prevalence of LGI consistent with that reported in the literature, but higher in CD children with early onset (<10 years) and in older children with UC, underscoring the importance of monitoring growth in children with IBD in the Saudi population. Prospective studies are needed to define the impact of IBD on growth velocity, puberty, and final adult stature. [ABSTRACT FROM AUTHOR]

AA descriptive study was undertaken to characterize the cystic fibrosis transmembrane regulator gene mutations (CFTR) in the Saudi Arabian cystic fibrosis (CF) population in relation to clinical presentation and demographic and ethnic origin. During the period October 1992 to September 1997, 70 patients from 46 families were diagnosed as having CF, based on a typical clinical picture and sweat chloride levels > 60 mmol/l and were screened for CFTR mutations. Twelve mutations were identified in 34 families, which constitutes 70% of the CF alleles in the study group. Pancreatic insufficiency (PI) was found in the following mutations: 1548delG in exon 10 (15%) which occurred mainly in native Saudi patients in the central province; 3120+1G A in intron 16 (10%) and H139L in exon 4 (7%), found mainly in native Saudis from the eastern province; Delta F508 mutation (13%) which occurred mainly in expatriates of Middle Eastern origin from different provinces; L117X in exon 19 (2%); G115X in exon 4 (2%); 711+1G A in intron 5 (2%); N 1303K in exon 21(2%) and 425del42 in exon 4 (1%); I1234V in exon 19 (13%) with a predominance of nasal polyps and a variable degree of PI and lung disease; R553X in exon 11 (1%), with electrolyte imbalance; and S549R in 11 (2%) with pancreatic sufficiency and minimal pulmonary disease. The clinical picture did not differ significantly between patients of different ethnic origins with the same CFTR mutation. [ABSTRACT FROM AUTHOR]

Background/Aim: Linear growth impairment (LGI) is one of the most important features peculiar to children with inflammatory bowel disease (IBD). The aim of this report is to define the impact of IBD on the linear growth of children in the Kingdom of Saudi Arabia (KSA). Setting and Design: Multicenter retrospective study. Patients and Methods: Data from a cohort of newly- diagnosed children with IBD from 2003 to 2012 were analyzed retrospectively. The diagnosis of IBD was confirmed in accordance with the published criteria. Length/height for age was measured at diagnosis. The World Health Organization (WHO) reference was used and LGI was defined by length/height for age

Gastrointestinal basidiobolomycosis (GIB) is an unusual fungal infection that manifests in the skin and rarely involves other systems. All of the few cases of GIB reported so far were diagnosed with difficulty, necessitating laparotomy and resection of the inflamed part of the bowel. We report a child with GIB who was successfully diagnosed endoscopically without surgical intervention.

Background and Aims. Crohn’s disease (CD) is an evolving disease in KSA. Little is known about its characteristics in the Saudi population. The aims of this study were to describe the characteristics of Saudi children with CD and to determine whether the characteristics of CD in KSA are different from those seen in Western countries. Methods. In this study, children younger than eighteen years of age diagnosed with CD between January 2003 and December 2012 were included. Results. Of 330 patients identified, 186 (56.4%) were males. The median age at diagnosis was 15.8 years. A positive family history for IBD in first-degree relatives occurred in 13.6% of patients. The most common symptoms were abdominal pain (84.2%), weight loss (75.2%), and diarrhea (71.8%). The main disease location was ileocolonic (42.1%) and the main disease behavior was nonstricturing and nonpenetrating (63.6%). Perianal involvement was seen in 60 (18.2%) patients. Laboratory findings revealed anemia in 57.9% of patients, low albumin in 34.5%, and high CRP in 39.4%. Conclusions. Saudi children with CD have lower frequency of first-degree relatives with IBD, lower prevalence of early onset disease, longer diagnostic delay, higher prevalence of growth failure, and greater frequency of stricturing and penetrating disease behavior compared to Western patients.

Background/Aims: The objective of this study was to describe patients with chronic diarrhea and abnormal skin hyperpigmentation with distinct distribution. Methods: This is a retrospective review of children who presented with diarrhea and skin hyperpigmentation. The clinical presentation, laboratory investigations as well as endoscopic and histological data were reviewed. Results: Seven patients with chronic diarrhea had abnormal skin hyperpigmentation with distinct distribution and presented in the first two months of life. Six patients had other features such as abnormal hair and facial dysmorphism. Mental retardation was reported in one patient. Consanguinity was positive in six patients, and there was family history of consanguinity in four patients, with two patients being siblings. No significant immunodeficiency was reported. Intestinal biopsies were obtained in six patients and showed active chronic inflammation in three patients, partial villous atrophy in two patients, and eosinophilic infiltrate with mild villous atrophy in one patient. Colonic biopsies showed mild focal colitis in three patients and mild colitis with eosinophilic infiltrate in one patient. Skin biopsies showed a greater number of melanophagies with fibrosis of papillary derma in two patients but skin biopsy was normal in one patient. The hair of two patients was analyzed by electron microscopy, which showed an abnormal pattern with decreased pigmentation and diameter; however, its chemical analysis was normal. Two other patients had trichorrhexis nodosa, but no abnormalities were seen in one patient. Chromosomal number was normal in three patients. One patient died because of sepsis, and only one patient was dependent on total parenteral nutrition. Conclusions: We believe that this association might represent a new syndrome with an autosomal recessive inheritance that warrants further studies.

Background and Objectives: Current practices and available resources for nutrition therapy in paediatric intensive care units (PICUs) in the Asia Pacific-Middle East region are expected to differ from western countries. Existing guidelines for nutrition management in critically ill children may not be directly applicable in this region. This paper outlines consensus statements developed by the Asia Pacific-Middle East Consensus Working Group on Nutrition Therapy in the Paediatric Critical Care Environment. Challenges and recommendations unique to the region are described.Methods and Study Design: Following a systematic literature search from 2004-2014, consensus statements were developed for key areas of nutrient delivery in the PICU. This review focused on evidence applicable to the Asia Pacific-Middle East region. Quality of evidence and strength of recommendations were rated according to the Grading of Recommendation Assessment, Development and Evaluation approach.Results: Enteral nutrition (EN) is the preferred mode of nutritional support. Feeding algorithms that optimize EN should be encouraged and must include: assessment and monitoring of nutritional status, selection of feeding route, time to initiate and advance EN, management strategies for EN intolerance and indications for using parenteral nutrition (PN). Despite heterogeneity in nutritional status of patients, availability of resources and diversity of cultures, PICUs in the region should consider involvement of dieticians and/or nutritional support teams.Conclusions: Robust evidence for several aspects of optimal nutrition therapy in PICUs is lacking. Nutritional assessment must be implemented to document prevalence and impact of malnutrition. Nutritional support must be given greater priority in PICUs, with particular emphasis in optimizing EN delivery. [ABSTRACT FROM AUTHOR]

Background and Aims. Crohn's disease (CD) is an evolving disease in KSA. Little is known about its characteristics in the Saudi population. The aims of this study were to describe the characteristics of Saudi children with CD and to determine whether the characteristics of CD in KSA are different from those seen in Western countries. Methods. In this study, children younger than eighteen years of age diagnosed with CD between January 2003 and December 2012 were included. Results. Of 330 patients identified, 186 (56.4%) were males. The median age at diagnosis was 15.8 years. A positive family history for IBD in first-degree relatives occurred in 13.6% of patients. The most common symptoms were abdominal pain (84.2%), weight loss (75.2%), and diarrhea (71.8%). The main disease location was ileocolonic (42.1%) and the main disease behavior was nonstricturing and nonpenetrating (63.6%). Perianal involvement was seen in 60 (18.2%) patients. Laboratory findings revealed anemia in 57.9% of patients, low albumin in 34.5%, and high CRP in 39.4%. Conclusions. Saudi children with CD have lower frequency of first-degree relatives with IBD, lower prevalence of early onset disease, longer diagnostic delay, higher prevalence of growth failure, and greater frequency of stricturing and penetrating disease behavior compared to Western patients.

Background: Pediatric inflammatory bowel disease (IBD) is increasingly recognized in developing countries; however, the incidence and trend over time have not been reported., Methods: This retrospective study included children diagnosed with IBD in gastroenterology centers in the Kingdom of Saudi Arabia between 2003 and 2012. The date of birth, date and age at diagnosis, gender, and final diagnosis were collected on special forms. Clinical, laboratory, imaging, endoscopy, and histopathology results were reviewed to confirm the final diagnosis. Descriptive statistics were used to compare ulcerative colitis and Crohn's disease in different age groups, and significance was assessed by the chi-square test. Incidence rates and trend over time were analyzed with the assumption of Poisson distribution. The incidence rate over time was compared in 2 periods (2003-2007 and 2008-2012). A P value of <0.05 and 95% confidence intervals were used to assess the significance and precision of the estimates., Results: A total of 340 Saudi Arabian children aged 0 to 14 years were diagnosed. The mean incidence rate per 100,000 individuals was 0.2, 0.27, and 0.47 for ulcerative colitis, Crohn's disease, and IBD, respectively. Except for the 0- to 4-year age group, there was a significant increase in incidence over time., Conclusions: Although the incidence of pediatric IBD in Saudi Arabian children is lower than suggested in the Western literature, there is a significantly increasing trend over time. However, decreased trend in the younger age group over time is identified. Prospective studies will be important to identify the risk factors for IBD in different age groups.

Gastrointestinal basidiobolomycosis (GIB) is an unusual fungal infection that manifests in the skin and rarely involves other systems. All of the few cases of GIB reported so far were diagnosed with difficulty, necessitating laparotomy and resection of the inflamed part of the bowel. We report a child with GIB who was successfully diagnosed endoscopically without surgical intervention.