RESUMEN
BACKGROUND: We utilized the data from the Saudi national biliary atresia (BA) study (2000-2018) to describe the clinical, biochemical, imaging, and histopathological features of BA and the perioperative clinical practices among local pediatric gastroenterologists. METHODS: This is a retrospective, multicenter, nationwide study that included 10 tertiary care governmental hospitals including the four liver transplant (LT) centers in different regions across Saudi Arabia. RESULTS: BA was diagnosed in 204 infants (106 females; 10% preterm). The median age at referral was 65 days. Congenital anomalies were present in 68 patients (33%); 22 were splenic malformation (10.8%). The medians of laboratory investigations were total bilirubin (189 µmol/l), direct bilirubin (139 µmol/l), ALT (164 u/l), and GGT (472 u/l). The level of serum GGT was normal in 26 cases (12.7%). The ultrasound findings included hypoplastic or atrophic gall bladder (GB) (65%), normal GB (30%), and cord sign (5%). A HIDA scan was performed in 99 cases (48.52%). Magnetic resonance cholangiopancreatography (MRCP) was performed in 27 cases (13%). A total of 179 liver biopsies (88%) were obtained. The most common histopathologic findings were bile duct proliferation (92%), canalicular cholestasis (96%), bile plugs (84%), and portal fibrosis (95%). Cholangiography was performed in 139 cases (68%): operative in 122 (60%) and percutaneous in 17 (8%). A total of 143 children (70%) underwent Kasai portoenterostomy (KPE) at a median age of 70 days. After KPE, steroid was used in 37% of the cases and 100 cases (70%) were prescribed prophylactic antibiotics for variable duration (ranging between 3 and 12 months). CONCLUSION: Our data show marked variation in the diagnostic evaluation and perioperative management of BA cases among the different tertiary centers. There is a need to establish a national BA registry in Saudi Arabia aiming to standardize pre- and postoperative clinical practices. Additionally, normal serum GGT level, normal GB size on ultrasound, and being a premature baby should not preclude the diagnostic workup for BA.
Asunto(s)
Enfermedades de los Conductos Biliares , Atresia Biliar , Lactante , Niño , Femenino , Recién Nacido , Humanos , Atresia Biliar/diagnóstico , Atresia Biliar/cirugía , Estudios Retrospectivos , Arabia Saudita/epidemiología , BilirrubinaRESUMEN
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.