Prevalence of functional gastrointestinal disorders in Saudi infants and toddlers: A cross-sectional multicenter study.

BACKGROUND: Functional gastrointestinal disorders (FGIDs) are common pediatric problems, but their prevalence in Saudi Arabia is unknown. We aimed to assess the prevalence of FGIDs and risk factors among children in six regions of Saudi Arabia. METHODS: This was a cross-sectional multicenter study enrolling children aged 0-48 months, attending pediatric clinics. Questionnaires evaluated the clinical history, symptoms, and sociodemographic information. FGIDs were defined according to Rome IV criteria. RESULTS: The study involved 1011 infants and toddlers (mean [standard deviation (SD)] aged, 21.7 [19.4] months; FGIDs and mean [SD] age 17.4 [16.4] months; controls). FGIDs were diagnosed in 483 (47.7%) of all infants and toddlers. The prevalence of FGIDs was significantly higher in children aged 0-12 months than in those aged 13-48 months ( P < 0.001). The most common disorders were functional regurgitation (13.8%) in infants and functional constipation (9.6%) in toddlers. Univariate regression analysis confirmed that the rate of FGIDs was higher in term gestational age infants (odds ratio (OR) 2.7; 95% confidence interval (CI), 1.76-4.17, P < 0.001), in partial breastfeeding (OR 0.58; 95% CI, 0.40-0.84, P = 0.003), in formula feeding (OR 2.25; 95% CI, 1.51-3.35, P < 0.001), and in subjects with no history of food allergy (OR 2.40; 95% CI, 1.58-3.64, P < 0.001). CONCLUSION: FGIDs are common in Saudi infants and toddlers (47.7%). Regurgitation is most prevalent in infants, and functional constipation is most common in toddlers. Term gestational age infant, partial breastfeeding, formula feeding, and subjects with no history of food allergy are associated with the prevalence of FGIDs.

Environmental Risk Factors for Childhood Inflammatory Bowel Diseases: A Multicenter Case-Control Study.

Objective: Multiple environmental factors can be linked to the development of inflammatory bowel disease (IBD).With an increase in the cases of IBD, the objective of this research is to investigate environmental risk factors for IBD in the Saudi population. Methods: A retrospective multicenter case−control study was performed among IBD children from 2009 to 2021.The variables analyzed to be the possible risk factors included their socioeconomic status, living and demographic characteristics, and lifestyle related to IBD. The questionnaire included a list of IBD risk factors that was given to the control and the patient group. For every variable, the 95% confidence interval (CI) and odds rations were also estimated. Results: There were 335 individuals considered in this study: 168 controls (50.1%) and 167 IBD patients (49.9%). Of these, 93 IBD patients (56%) had CD and 74 patients (44%) had UC. Most of participants were female (72.1%) and were aged above 10 years (51.5%). Vaginal delivery (OR 0.551, 95% CI: 1.59−4.14), age above 10 years (OR 1.040, 95% CI: 1.012−1.069), deficient fruit intake (OR 2.572, 95% CI: 1.59−4.14), no exposure to antibiotics (OR 2.396, 95% CI: 1.51−3.81), appendectomy (OR 2.098, 95% CI: 1.87−2.35), less physical activity (OR 2.033, 95% CI: 1.05−3.93) and gastroenteritis admissions > 2 times/year (OR 0.107, 95% CI: 0.037−0.311) were the risk factors for IBD. These factors depicted a more significant link with CD than UC (p < 0.05). Interestingly, sleep disturbance was estimated to be a CD risk factor (adjusted OR: 3.291, 95% CI = 0.97−11.22). Pets in house was risk factor for UC (p < 0.001). Conclusions: This study highlights association between vaginal delivery, age above 10 years, deficient fruit intake, low physical activity, exposure to antibiotics, appendectomy, and frequent gastroenteritis admissions as risk factors for IBD. Knowledge of these risk factors can help pediatricians to prospectively identify patients at risk of environmental exposure.

Consensus statement on the epidemiology, diagnosis, prevention, and management of cow's milk protein allergy in the Middle East: a modified Delphi-based study.

BACKGROUND: This study aimed to develop an expert consensus regarding the epidemiology, diagnosis, and management of cow's milk protein allergy (CMPA) in the Middle East. METHODS: A three-step modified Delphi method was utilized to develop the consensus. Fifteen specialized pediatricians participated in the development of this consensus. Each statement was considered a consensus if it achieved an agreement level of ≥ 80%. RESULTS: The experts agreed that the double-blind placebo-controlled oral challenge test (OCT) should be performed for 2-4 weeks using an amino acid formula (AAF) in formula-fed infants or children with suspected CMPA. Formula-fed infants with confirmed CMPA should be offered a therapeutic formula. The panel stated that an extensively hydrolyzed formula (eHF) is indicated in the absence of red flag signs. At the same time, the AAF is offered for infants with red flag signs, such as severe anaphylactic reactions. The panel agreed that infants on an eHF with resolved symptoms within 2-4 weeks should continue the eHF with particular attention to the growth and nutritional status. On the other hand, an AAF should be considered for infants with persistent symptoms; the AAF should be continued if the symptoms resolve within 2-4 weeks, with particular attention to the growth and nutritional status. In cases with no symptomatic improvements after the introduction of an AAF, other measures should be followed. The panel developed a management algorithm, which achieved an agreement level of 90.9%. CONCLUSION: This consensus document combined the best available evidence and clinical experience to optimize the management of CMPA in the Middle East.

PLACK syndrome is potentially treatable with intralipids.

We describe an 11-year-old girl with PLACK Syndrome (peeling skin, leukonychia, acral punctate keratosis, cheilitis, and knuckle pads), who was found to have a novel homozygous variant in CAST, the pathogenicity of which was confirmed using blood-derived RNA. There is no established treatment for PLACK syndrome. However, we demonstrate for the first time that this condition is associated with low levels of vitamin A and essential fatty acids, which prompted us to consider a potential treatment strategy. Indeed, we initiated this patient on intravenous lipid infusion (Vitalipid®; an emulsion of fat-soluble vitamins and lipofundin-MCT/LCT 20%) and the response was dramatic. Following the fourth monthly course of treatment, pruritis disappeared and the skin lesions showed remarkable objective improvement. PLACK syndrome is a very rare genodermatosis and only six families have been described to date with pathogenic CAST variants. This is the first report of an objective response to a therapeutic agent, which suggests that PLACK is a potentially treatable condition. The remarkable response we report and the relative safety of the intervention should prompt healthcare providers who care for PLACK syndrome patients to explore this as a potential treatment strategy in future studies.

Natural history and outcome of inflammatory bowel diseases in children in Saudi Arabia: A single-center experience.

Background/Aim: Inflammatory bowel disease (IBD) is a chronic gastrointestinal disorder which includes ulcerative colitis (UC), Crohn's disease (CD), and indeterminate colitis (IC). The natural history of pediatric IBDs is poorly understood and generally unpredictable. We aim to study the natural history of IBD in Saudi children including the extraintestinal manifestations, changes in diagnosis, disease behavior, medical management, and surgical outcome. Patients and Methods: A retrospective review of all the charts of children less than 14 years of age who were diagnosed as IBD and followed up in King Faisal Specialist Hospital and Research Center (KFSH and RC) from January 2001 to December 2011 was performed. Results: Sixty-six children were diagnosed with IBD, 36 patients (54.5%) had CD, 27 patients (41%) had UC, and 3 patients (4.5%) had IC. Change in the diagnosis from UC to CD was made in 5 patients (7.6%). Extraintestinal manifestations were documented in 32% of all patients, and the most common was bone involvement (osteopenia/osteoporosis) in 16.7% of the patients. Arthritis (13.6%) was the second most common manifestation. Sclerosing cholangitis was reported in 2.8% in CD compared to 14.8% in UC. At the time of data collection, 8 patients (12%) were off therapy, 38 patients (57.6) were on 5-ASA, 31 patients (47%) were on azathioprine, and 12 patients (18.2%) were receiving anti-TNF. Of the children with CD, 10 patients (27.8%) underwent 1 or more major operations. Of the children with UC, 18.5% underwent 1 or more major intraabdominal procedures. Conclusions: Many issues in pediatric IBD can predict the natural history of the disease including growth failure, complications, need for more aggressive medical treatment, and/or surgery. More studies are needed from the region focusing on factors that may affect the natural history and disease progression.

Prevalence of celiac disease in Saudi children with Down syndrome: A retrospective study.

Celiac disease (CD) is an immune-mediated disease affecting the small intestine secondary to gluten exposure. The currently available treatment is lifelong adherence to a gluten-free diet (GFD). Several disorders are known to be associated with celiac disease, including Down syndrome (DS). In several studies, the prevalence of CD in DS ranged between 4 and 17%. CD is prevalent in Arabs; however, few studies have been performed to determine the prevalence of CD in DS patients. Our study aimed to determine the prevalence of CD in Saudi Down syndrome patients using serological markers and small bowel biopsy. This is a retrospective study in which files relating to Down syndrome patients who were followed up in a general pediatric clinic at King Faisal Specialist Hospital and Research Center were reviewed regarding demographic data, serological markers and biopsy results. Of the total number of patients reviewed (91), 7 were excluded because data were missing; the remaining 84 patients included 35 females and 49 males. The age range of the patients at the time of screening was from 1 to 18 years. Patient demographic data are shown in Table 1. Among the studied patients, antigliadin antibody (AGA) IgA was high in 27 patients (32.14%), and AGA IgG was high in 44 patients (52.38%). Twelve patients (14.28%) tested positive and 58 (69.04%) tested negative for anti-endomysial antibodies. Anti-tissue glutaminase antibody IgA was found to be high in 13 patients (15.5%) and normal in 54 patients (64.28%). Serum IgA levels were normal in 36 patients (43%) and low in 1 patient (1.2%). Biopsy was performed in 22 patients who tested positive for anti-endomysial or anti-tissue transglutaminase antibodies. The biopsies provided positive results in 9 patients (10.7%). Our study showed a confirmed prevalence of 10.7% for celiac disease in Saudi patients with Down syndrome based on serology and biopsy; together with previous cases reported in the literature, this result indicates a need to screen these patients for celiac disease.

Middle East Consensus Statement on the Diagnosis and Management of Functional Gastrointestinal Disorders in <12 Months Old Infants.

This paper covers algorithms for the management of regurgitation, constipation and infantile colic in infants. Anti-regurgitation formula may be considered in infants with troublesome regurgitation, while diagnostic investigations or drug therapy are not indicated in the absence of warning signs. Although probiotics have shown some positive evidence for the management of functional gastrointestinal disorders (FGIDs), the evidence is not strong enough to make a recommendation. A partially hydrolyzed infant formula with prebiotics and ß-palmitate may be considered as a dietary intervention for functional constipation in formula fed infants. Lactulose has been shown to be effective and safe in infants younger than 6 months that are constipated. Macrogol (polyethylene glycol, PEG) is not approved for use in infants less than 6 months of age. However, PEG is preferred over lactulose in infants >6 months of age. Limited data suggests that infant formula with a partial hydrolysate, galacto-oligosaccharides/fructo-oligosaccharides, added ß-palmitate may be of benefit in reducing infantile colic in formula fed infants in cases where cow's milk protein allergy (CMPA) is not suspected. Evidence suggests that the use of extensively hydrolyzed infant formula for a formula-fed baby and a cow's milk free diet for a breastfeeding mother may be beneficial to decrease infantile colic if CMPA is suspected. None of the FGIDs is a reason to stop breastfeeding.

Correction: Middle East Consensus Statement on the Diagnosis and Management of Functional Gastrointestinal Disorders in <12 Months Old Infants.

[This corrects the article on p. 153 in vol. 19, PMID: 27738596.].

Familial aggregation of Crohn's disease and necrotizing sarcoid-like granulomatous disease.

Granulomatous inflammatory diseases are disorders of an undetermined etiology, affecting different organs and having a diverse clinical course. Familial aggregation of these disorders is being reported increasingly, most commonly familial Crohn's disease. We described the coexistence of Crohn's disease and necrotizing sarcoid-like granulomatous disease in two siblings from a first-degree consanguineous Saudi family. The first child presented with recurrent abdominal pain associated with bloody stool and arthritis, whereas the second child presented with fever of unknown origin and lymphadenopathy as well as hepatomegaly without gastrointestinal tract disease. They are phenotypically different; however, they share a novel risk locus and allele. This report supports the heritability and familial aggregation of granulomatous inflammatory diseases and suggests that one causal mutation underlies both Crohn's disease and necrotizing sarcoid-like granulomatous disease.

Intestinal lymphangiectasia in a patient with infantile systemic hyalinosis syndrome: a rare cause of protein-losing enteropathy.

Infantile systemic hyalinosis (ISH) is a rare autosomal recessive disease. Typically, ISH patients present with progressive painful joint contractures, intractable diarrhea, hyperpigmented skin lesions, and peri-anal fleshy nodules. We report a case of a 19-month-old male child with atypical ISH presentation. His main clinical finding was protein-losing enteropathy due to intestinal lymphangectasia. This report is intended to enhance awareness about the gastrointestinal tract presentation of ISH.