Functional outcome of anorectal malformations and associated anomalies in era of krickenbeck classification

Abstract OBJECTIVE: To describe the management and functional outcome of anorectal malformations and associated anomalies according to Krickenbeck classification. STUDY DESIGN: Case series. PLACE AND DURATION OF STUDY: The Aga Khan University Hospital, Karachi, from January 2002 to December 2012. METHODOLOGY: Anorectal anomalies were classified according to Krickenbeck classification. Data was collected and proforma used regarding the primary disease associated anomalies, its management and functional outcome, according to Krickenbeck classification. Cases included were: all those children with imperforate anus managed during the study period. Qualitative variables like gender and functional outcome were reported as frequencies and percentages. Quantitative variables like age were reported as medians with interquartile ranges. RESULTS: There were 84 children in study group. Most common associated anomaly was cardiac (38%), followed by urological anomaly (33%). All children were treated by Posterior Sagittal Anorectoplasty (PSARP). Fistula was present in 64 out of 84 (76%) cases. The most common fistula was rectourethral (33%), followed by recto vestibular (31%). According to Krickenbeck classification, continence was achieved in 62% children; however 27% children were constipated, followed by 12% children having fecal soiling. CONCLUSION: Functional outcome of anorectal malformation depends upon severity of disease. A thorough evaluation of all infants with ARM should be done with particular focus on cardiovascular (38%) and genitourinary abnormalities (33%)

Mortality from gastrointestinal congenital anomalies at 264 hospitals in 74 low-income, middle-income, and high-income countries: a multicentre, international, prospective cohort study

Summary Background Congenital anomalies are the fifth leading cause of mortality in children younger than 5 years globally. Many gastrointestinal congenital anomalies are fatal without timely access to neonatal surgical care, but few studies have been done on these conditions in low-income and middle-income countries (LMICs). We compared outcomes of the seven most common gastrointestinal congenital anomalies in low-income, middle-income, and high-income countries globally, and identified factors associated with mortality. Methods We did a multicentre, international prospective cohort study of patients younger than 16 years, presenting to hospital for the first time with oesophageal atresia, congenital diaphragmatic hernia, intestinal atresia, gastroschisis, exomphalos, anorectal malformation, and Hirschsprung’s disease. Recruitment was of consecutive patients for a minimum of 1 month between October, 2018, and April, 2019. We collected data on patient demographics, clinical status, interventions, and outcomes using the REDCap platform. Patients were followed up for 30 days after primary intervention, or 30 days after admission if they did not receive an intervention. The primary outcome was all-cause, in-hospital mortality for all conditions combined and each condition individually, stratified by country income status. We did a complete case analysis. Findings We included 3849 patients with 3975 study conditions (560 with oesophageal atresia, 448 with congenital diaphragmatic hernia, 681 with intestinal atresia, 453 with gastroschisis, 325 with exomphalos, 991 with anorectal malformation, and 517 with Hirschsprung’s disease) from 264 hospitals (89 in high-income countries, 166 in middleincome countries, and nine in low-income countries) in 74 countries. Of the 3849 patients, 2231 (58·0%) were male. Median gestational age at birth was 38 weeks (IQR 36–39) and median bodyweight at presentation was 2·8 kg (2·3–3·3). Mortality among all patients was 37 (39·8%) of 93 in low-income countries, 583 (20·4%) of 2860 in middle-income countries, and 50 (5·6%) of 896 in high-income countries (p<0·0001 between all country income groups). Gastroschisis had the greatest difference in mortality between country income strata (nine [90·0%] of ten in lowincome countries, 97 [31·9%] of 304 in middle-income countries, and two [1·4%] of 139 in high-income countries; p≤0·0001 between all country income groups). Factors significantly associated with higher mortality for all patients combined included country income status (low-income vs high-income countries, risk ratio 2·78 [95% CI 1·88–4·11], p<0·0001; middle-income vs high-income countries, 2·11 [1·59–2·79], p<0·0001), sepsis at presentation (1·20 [1·04–1·40], p=0·016), higher American Society of Anesthesiologists (ASA) score at primary intervention (ASA 4–5 vs ASA 1–2, 1·82 [1·40–2·35], p<0·0001; ASA 3 vs ASA 1–2, 1·58, [1·30–1·92], p<0·0001]), surgical safety checklist not used (1·39 [1·02–1·90], p=0·035), and ventilation or parenteral nutrition unavailable when needed (ventilation 1·96, [1·41–2·71], p=0·0001; parenteral nutrition 1·35, [1·05–1·74], p=0·018). Administration of parenteral nutrition (0·61, [0·47–0·79], p=0·0002) and use of a peripherally inserted central catheter (0·65 [0·50–0·86], p=0·0024) or percutaneous central line (0·69 [0·48–1·00], p=0·049) were associated with lower mortality. Interpretation Unacceptable differences in mortality exist for gastrointestinal congenital anomalies between lowincome, middle-income, and high-income countries. Improving access to quality neonatal surgical care in LMICs will be vital to achieve Sustainable Development Goal 3.2 of ending preventable deaths in neonates and children younger than 5 years by 2030

Association of biochemical markers with COVID-19 severity in Pakistan

Objective: To evaluate demographics, biochemical markers and clinical features of patients suffering from coronavirus disease-2019. Method: The cross-sectional study was conducted retrospectively at the Capital Hospital, Islamabad, and the Fauji Foundation Hospital, Rawalpindi, Pakistan, from October 08, 2021 to March 01, 2022 and comprised patients of either gender with coronavirus disease-2019 diagnosed on the basis of reverse transcriptase polymerase chain reaction. Patients’ demographic, clinical and laboratory findings were obtained using patient charge sheets. Coronavirus disease-2019 was categorised as non-severe, severe and critical, according to the World Health Organisation criteria. Data was analysed using SPSS 26. Results: Of the 431 patients, 91(21.1%) were men and 340(78.9%) were women. The overall mean age was 60.75±14.45 years. Of the total, 148(34.3%) had non-severe, 190(44.1%) severe and 93(21.6%) had critical condition at the time of admission. Hypertension 307(71.2%) and diabetes mellitus 249(57.8%) were the most common comorbidities, while fever 353(81.9%), shortness of breath 339(78.7%) and cough 302(70.1%) were the most common symptoms reported. Higher age was significantly associated with coronavirus disease-2019 severity (p<0.001). Among comorbidities, chronic kidney disease (p<0.001) and cancer (p=0.046), and, among signs and symptoms, shortness of breath (p=0.002) and chest pain (p=0.021), were significantly associated with coronavirus disease-2019 severity. Serum total bilirubin, alanine aminotransferase, urea and creatinine levels had significant association with disease severity (p<0.001). Total leukocyte count, neutrophil-to-lymphocyte ratio, prothrombin time, and plasma D-Dimer levels had significant association with disease severity (p<0.001). Serum ferritin, lactate dehydrogenase and interleukin-6 levels were also significantly associated with disease severity (p<0.05). Conclusion: Assessment of biochemical markers was an excellent way to monitor disease progression in coronavirus disease-2019 patients. Key Words: COVID-19, Diabetes, Severity, Hypertension, Clinical features