Pediatric and adult liver transplantation in Bahrain: The experiences in a country with no available liver transplant facilities.

BACKGROUND: Liver transplantation (LT) is a life-saving procedure for patients with end-stage liver disease and has become the standard and most effective treatment method for these patients. There are many indications for LT that vary between countries and settings. The outcome of LT depends on the available facilities and surgical expertise, as well as the types of liver graft donors available. AIM: To assess the clinical characteristics of patients from Bahrain who underwent LT overseas, and analyze factors affecting their survival. METHODS: In this retrospective cohort study, we reviewed the medical records and overseas committee registry information of all pediatric and adult patients who were sent overseas to undergo LT by the Pediatric and Medical Departments of Salmaniya Medical Complex and Bahrain Defence Force Hospital via the Overseas Treatment Office, Ministry of Health, Kingdom of Bahrain, between 1997 and 2023. Demo graphic data, LT indication, donor-recipient relationship, overseas LT center, graft type, post-LT medications, and LT complications, were collected. Outcomes measured included the overall and 5-year LT survival rate. Fisher's exact, Pearson χ2, and Mann-Whitney U tests were used to compare the pediatric and the adults' group in terms of clinical characteristics, donor-recipient relationship, medication, complications, and outcome. Survival analysis was estimated via the Kaplan-Meier's method. Univariate and multivariate analyses were used to detect predictors of survival. RESULTS: Of the 208 eligible patients, 170 (81.7%) were sent overseas to undergo LT while 38 (18.3%) remained on the waiting list. Of the 170 patients, 167 (80.3%) underwent LT and were included in the study. The majority of the patients were Bahraini (91.0%), and most were males (57.5%). One-hundred-and-twenty (71.8%) were adults and 47 (28.3%) were children. The median age at transplant was 50.0 [interquartile range (IQR): 14.9-58.4] years. The main indication for pediatric LT was biliary atresia (31.9%), while that of adult LT was hepatitis C-related cirrhosis (35.0%). Six (3.6%) patients required re-transplantation. Most patients received a living-related liver graft (82%). Pediatric patients received more living and related grafts than adults (P = 0.038 and P = 0.041, respectively), while adult patients received more cadaveric and unrelated grafts. Most patients required long-term immunosuppressive therapy after LT (94.7%), of which tacrolimus was the most prescribed (84.0%), followed by prednisolone (50.7%), which was prescribed more frequently for pediatric patients (P = 0.001). Most patients developed complications (62.4%) with infectious episodes being the most common (38.9%), followed by biliary stricture (19.5%). Tonsilitis and sepsis (n = 12, 8.1% for each) were the most frequent infections. Pediatric patients experienced higher rates of infection, rejection, and early poor graft function than adult patients (P < 0.001, P = 0.003, and P = 0.025, respectively). The median follow-up time was 6.5 (IQR: 2.6-10.6) years. The overall survival rate was 84.4%, the 5-year survival rate, 86.2%, and the mortality rate, 15.6%. Younger patients had significantly better odds of survival (P = 0.019) and patients who survived had significantly longer follow-up periods (P < 0.001). CONCLUSION: Patients with end-stage liver disease in Bahrain shared characteristics with those from other countries. Since LT facilities are not available, an overseas LT has offered them great hope.

Assessing indicators and clinical differences between functional and organic childhood constipation: a retrospective study in pediatric gastroenterology clinics.

BACKGROUND: Chronic constipation is common among children worldwide. Constipation includes functional constipation (FC) and organic constipation (OC). The early recognition of the causes of childhood constipation and its subsequent complications is important. PURPOSE: This study aimed to evaluate the prevalence and causes of childhood constipation and compare the clinical characteristics, treatment, and outcomes of children with FC versus OC to identify the predictive factors. METHODS: This retrospective cross-sectional study analyzed children with FC or OC diagnosed in pediatric gastroenterology clinics, Salmaniya Medical Complex, Bahrain, 2017-2021. The Rome IV criteria were used to define FC. RESULTS: A total of 7,287 gastroenterology appointments were attended by 4,346 children during the study period. Of the 639 children (14.7%) with constipation, 616 (96.4%) were included in the study. Most patients had FC (n=511, 83%), whereas 17% (n=105) had OC. FC was more common in females than in males. Children with OC were younger (P<0.001) and had lower body weights (P<0.001), more stunted growth (P<0.001), and more associated diseases (P= 0.037) than those with FC. Enuresis was the most associated disease (n=21, 3.4%). Organic causes included neurological, allergic, endocrine, gastrointestinal, and genetic diseases. Allergies to cow milk protein were the most common (n=35, 5.7%). The presence of mucus in the stool was more common in OC than in FC (P=0.041), but no other symptoms or physical findings differed. A total of 587 patients (95.3%) received medication, among which lactulose was commonly prescribed (n=395, 64.1%). There were no intergroup differences in nationality, sex, body mass index, seasonal variation, laxative type, or treatment response. A good response was observed in 114 patients (90.5%). CONCLUSION: Chronic constipation represented a significant proportion of outpatient gastroenterology visits. FC was the most common type. Young children with a low body weight, stunted growth, mucus in the stool, or associated diseases should be assessed for an underlying organic cause.

Linear Growth Impairment in Patients With Pediatric Inflammatory Bowel Disease.

Introduction Linear growth impairment (LGI) is one of the complications of pediatric inflammatory bowel disease (IBD). This study aimed to assess the linear growth of patients with pediatric IBD and to detect the frequency and the possible risk factors of LGI. Methods A retrospective cross-sectional review of medical records of patients with pediatric IBD was conducted in the pediatrics department, Salmaniya Medical Complex, Bahrain, from 1984 to 2019. Demographic and anthropometric data were gathered. World Health Organization (WHO) standards and references were used to define LGI. According to WHO, stunting and severe stunting were defined as length/height for age of <-2 standard deviations and <-3 standard deviations from age and sex-specific reference means, respectively. To determine the possible risk factors for LGI, stunted patients were compared with normal height patients in regard to demographic data, clinical presentations, and treatment used. Results Out of 130 patients with pediatric IBD, 88 (67.7%) had anthropometric data available. Fifty-five (62.5%) were males. Forty-seven (53.4%) had Crohn's disease and 41 (46.6%) had ulcerative colitis. The mean age at presentation was 10.7±3.8 years. The median age at the time of growth measurement was 14.2 (interquartile range=12.1-24.4) years. Fifteen (17%) patients were stunted, and seven (46.7%) of those stunted patients were severely stunted. Weight at presentation was lower in stunted patients (21.6±5.9 kilograms) compared to normal height patients (31±13.4 kilogram) (p=0.048). Sex, delivery type, birth weight, height at presentation, age at presentation, age at growth measurements, IBD type, disease duration, presence of extraintestinal manifestations, and prednisolone and biologic therapy use were not significant factors of stunting. Conclusion Patients with pediatric IBD have a high prevalence of LGI compared to the general population. Low weight at disease presentation is the only significant risk factor for LGI. This might indicate that IBD as a disease by itself is having the main negative impact on linear growth.

Efficacy and Safety of Endoscopic Esophageal Dilatation in Pediatric Patients with Esophageal Strictures.

MATERIALS AND METHODS: In this retrospective cross-sectional single center study, records of patients with esophageal strictures presented to the pediatric department, Salmaniya Medical Complex, Bahrain, in the period between 1995 and 2019 were reviewed. Demographic data, indications of endoscopic dilatations, the procedure success rate, and possible complications were assessed. RESULTS: Forty-six children were found to have esophageal strictures. Twenty-five (54.3%) patients were males. Most patients presented during infancy (86.5%, 32/37 patients). Twenty-six (56.5%) patients required 88 dilatation sessions, while the remaining 20 (43.5%) patients did not require dilatations. The median number of dilatation sessions per patient was three (interquartile range = 2-5). Savary-Gilliard bougienages were the main dilators used (80.8%, 21/26 patients). Anastomotic stricture (post esophageal atresia/tracheoesophageal fistula repair) was the main cause of esophageal strictures and was found in 35 (76.1%) patients. Patients with nonanastomotic strictures had more frequent dilatations compared to those with anastomotic strictures (P = 0.007). The procedure success rate was 98.8%. Yet, it was operator dependent (P = 0.047). Complete response to dilatation was found in 18 (69.2%) patients, satisfactory in seven (26.9%), and an inadequate response in one (3.9%). Those with satisfactory responses still require ongoing dilatations based on their symptoms and radiological and endoscopic findings. No perforation or mortality was reported. Patients with dilatations had more recurrent hospitalization (P < 0.0001), more dysphagia (P = 0.001), but shorter hospital stay (P = 0.046) compared to those without dilatations. Surgical intervention was required in one patient with caustic strictures. The median follow-up period was six years (interquartile range = 2.25-9.0). CONCLUSIONS: Endoscopic esophageal dilatation in children with esophageal strictures is effective and safe. Yet, it was operator dependent. Nonanastomotic strictures require more dilatations compared to anastomotic strictures. Findings of this study are comparable to those reported worldwide.

Celiac disease in children: Increasing prevalence and changing clinical presentations.

BACKGROUND: Celiac disease (CD) is a chronic autoimmune enteropathy. It results from genetic predisposition and exposure to gluten-containing food. The prevalence and presentation of CD vary among populations. PURPOSE: This study aimed to describe the prevalence and clinical characteristics of CD in children in Bahrain. METHODS: We retrospectively reviewed the medical records of children diagnosed with CD in the pediatric department, Salmaniya Medical Complex, Bahrain, in 1988-2018. Their clinical, biochemical, serological, and histopathological findings were documented. Adherence to the recommended gluten-free diet (GFD) was assessed. RESULTS: Of 86 patients with CD, 67 were included. The CD prevalence was 0.02%. A significant increase in prevalence in the last decade was observed (P<0.0001). Thirty-eight patients (56.7%) were males. The median (interquartile range) age at presentation was 4.45 (1.5-7.3) years. A family history of CD was positive in 13 out of 43 patients (30.2%). Pallor and failure to thrive were the most common presentations. The most frequent associated disease was iron-deficiency anemia in 23 patients (69.7%). Positive serology was found in 32 of 45 patients (71.1%). Marsh-Oberhuber type III was found in 16 of 35 patients (45.7%). Seropositive patients were significantly older (P=0.025) and had more severe duodenal histology (P=0.002). Adherence to GFD was poor in 27 patients (64.3%). CONCLUSION: This study revealed a significant increase in CD prevalence over the last decade. Atypical presentations were frequent. Most patients had poor adherence to GFD.

Pediatric Crohn's Disease in Bahrain.

OBJECTIVES: Our study aimed to report the epidemiology, clinical presentations, diagnostic and therapeutic approaches, and outcomes of Crohn's disease (CD) in pediatric patients in Bahrain. METHODS: We conducted a retrospective review of the medical records of patients with CD diagnosed in the pediatric department, Salmaniya Medical Complex, Bahrain, between 1984 and 2017. We used the data to calculate the annual incidence and cumulative prevalence. Data about gender, nationality, clinical presentation, age at presentation and diagnosis, duration of illness, consanguinity, family history, contact with smokers, and comorbidities were gathered. Results of hematological, biochemical, and serological tests were also collected. All radiological, endoscopic, and histopathological findings were reviewed. Data about medical therapy, relapse episodes, hospital admissions, complications, and outcomes were collected. RESULTS: Of 108 pediatric patients diagnosed with inflammatory bowel disease (IBD), 51 (47.2%) patients had CD. The annual incidence was 1 in 100 000 per year (range = 0-5 patients/year) with significant rise on comparing the three decades (p = 0.0001). Prevalence was 9.32 patients per 100 000 pediatric populations. Thirty-four patients (66.7%) were males, and the median age was 18.5 years (range = 6.4-35.0). Common clinical presentations were recurrent abdominal pain and weight loss. Family history of IBD was found in 10 patients. One patient had positive antineutrophil cytoplasmic antibody. The terminal ileum was involved in 68.1%, colon in 63.8%, and perianal area in 17.0% patients. Biological therapy was used in five patients. Surgical intervention was required in six patients. The mean follow-up period was 9.2±5.6 years. CONCLUSIONS: The clinical characteristics of our population are comparable to that reported in neighboring countries and worldwide.

Neonatal indirect hyperbilirubinemia and glucose-6-phosphate dehydrogenase deficiency.

PURPOSE: This study aimed to determine the prevalence of glucose-6-phosphate dehydrogenase (G6PD) deficiency among infants with neonatal indirect hyperbilirubinemia (NIH); compare G6PD-deficient and G6PD-normal patients regarding hyperbilirubinemia and need for exchange transfusions (ET); and assess risk factors for ET and kernicterus. METHODS: This is a case-control retrospective study. Medical records of NIH patients admitted to the Pediatric Department, Salmaniya Medical Complex, Bahrain, between January 2007 and June 2010 were reviewed. Data on sex, age at presentation, hospitalization duration, need for ET, hemoglobin (Hb) level, reticulocyte count, direct Coombs test, serum total and indirect bilirubin levels, thyroid function, blood and urine cultures, G6PD status, and blood groups were collected and compared between the G6PD-deficent and G6PD-normal patients. RESULTS: Of 1,159 NIH patients admitted, 1,129 were included, of whom 646 (57%) were male. Among 1,046 patients tested, 442 (42%) were G6PD deficient, 49 (4%) needed ET, and 11 (1%) had suspected Kernicterus. The G6PD-deficient patients were mainly male (P<0.0001), and had lower Hb levels (P<0.0001) and higher maximum bilirubin levels (P=0.001). More G6PD-deficient patients needed ET (P<0.0001). G6PD deficiency (P=0.006), lower Hb level (P=0.002), lower hematocrit count (P=0.02), higher bilirubin level (P<0.0001), higher maximal bilirubin level (P<0.0001), and positive blood culture result (P<0.0001) were significant risk factors for ET. Maximal bilirubin level was a significant risk factor for kernicterus (P=0.021) and independently related to ET (P=0.03). CONCLUSION: G6PD deficiency is an important risk factor for severe NIH. In G6PD-deficent neonates, management of NIH should be hastened to avoid irreversible neurological complications.

Intestinal lymphangiectasia in children. A favorable response to dietary modifications.

Intestinal lymphangiectasia (IL) is a rare disease characterized by dilatation of intestinal lymphatics. It can be classified as primary or secondary according to the underlying etiology. The clinical presentations of IL are pitting edema, chylous ascites, pleural effusion, acute appendicitis, diarrhea, lymphocytopenia, malabsorption, and intestinal obstruction. The diagnosis is made by intestinal endoscopy and biopsies. Dietary modification is the mainstay in the management of IL with a variable response. Here we report 2 patients with IL in Bahrain who showed positive response to dietary modification.

Growth assessment and risk factors of malnutrition in children with cystic fibrosis.

OBJECTIVES: To evaluate the nutritional status, to screen for the presence of malnutrition, and to study the possible risk factors associated with malnutrition in patients with cystic fibrosis (CF). METHODS: A retrospective cross-sectional review of medical records of all diagnosed CF patients in the Pediatric Department, Salmaniya Medical Complex, Manama, Kingdom of Bahrain, between January 1984 and May 2015 was conducted. Demographic and anthropometric data were collected from records of last visit to CF clinic. Nutritional status and risk factors of malnutrition were assessed. RESULTS: All records of 109 CF patients were reviewed. Forty-seven pediatric patients were included in the study. All included patients were on pancreatic enzyme replacement and 42 (89%) received high-calorie supplementation. Growth failure was noted in 34 (72%) patients, 19 (56%) were wasted and stunted, 8 (23.5%) were wasted only, and 7 (20.5%) were stunted. Low birth weight (p=0.032), and the presence of gastroesophageal reflux disease (GERD) (p=0.039) were the significant risk factors for malnutrition. CONCLUSIONS: Most CF patients in Bahrain (72%) are malnourished. Low birth weight and the presence of GERD are risk factors.

Effect of pediatric liver transplantation on renal function.

The aim of this review is to define the incidence of renal dysfunction among pediatric liver transplant (LT) survivors, to identify the associated risk factors and to outline the therapeutic options. Renal dysfunction is a common problem after pediatric LT. The measured glomerular filtration rate is considered the "gold-standard" for assessment of renal function. Renal dysfunction in pediatric LT recipients is multifactorial. Renal-sparing immunosuppressive strategies are essential to reverse renal dysfunction and to prevent end-stage renal disease.

Neonatal hemochromatosis. Case series from Bahrain.

OBJECTIVE: To review clinical presentations, diagnosis, response to treatment, and outcome of infants with neonatal hemochromatosis (NH). METHODS: This is a retrospective review of all cases admitted to the Pediatric Department at Salmaniya Medical Center, Manama, Bahrain between March 2008 and May 2011. The diagnosis was based on serum iron and ferritin, alpha-fetoprotein levels (AFP), liver and buccal biopsies, and abdominal MRI scan. RESULTS: Ten patients (8 males and 2 females) were diagnosed with NH. Two patients were intrauterine growth restriction (IUGR) and 6 were preterm. The median birth weight was 1.700 grams. The median age at presentation was 16 days, and at diagnosis was 23 days. Two patients had positive consanguinity. Clinical presentations of the infants were hepatosplenomegaly (n=5), ascites (n=3), and hypoglycemia (n=6). All patients had raised ferritin levels, prolonged prothrombin time, and 9 patients had high serum iron and serum AFP. Abdominal MRI showed iron overload in the liver (n=8). Liver biopsies showed evidence of hemochromatosis (n=3). Buccal biopsies stained positive for iron (n=1). Eight patients received antioxidant therapy and survived. Two patients passed away. CONCLUSION: Neonatal hemochromatosis is a rare liver disease of newborns with a spectrum of clinical severity. Elevated serum ferritin and AFP support the diagnosis after excluding other causes of neonatal liver failure. The use of antioxidant therapy helps to improve the outcome.