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

Acute porphyria: an unusual case of quadriparesis, hypertension, recurrent severe cyclic abdominal pain, and seizures

Porphyria refers to a rare group of genetically inherited or acquired disorders that arise due to reduced metabolic activity of any of the enzymes in the haem biosynthetic pathway. Defect in any enzyme causes the presentation of symptoms of porphyria. The epidemiology of Acute Intermittent Porphyria (AIP) is complicated because of its rarity and delay in diagnosis. We present the case of a seven-year-old girl who presented with multisystem involvement; her symptoms were quadriparesis, hypertension, recurrent severe cyclic abdominal pain, and seizures. These symptoms together were not explained by the differentials taken into account. She presented before puberty with no family history of such conditions, while being born of consanguineous marriage. Her symptoms along with urinary porphobilinogen positivity test helped to reach the diagnosis of AIP in the absence of cutaneous manifestations. ---Continu

Idiopathic pulmonary haemosiderosis: an unusual case of anaemia with pulmonary involvement

Idiopathic pulmonary haemosiderosis is a rare disorder, with recurrent life-threatening alveolar haemorrhages and chronic lung parenchymal changes. It is associated with a triad of haemoptysis, iron deficiency anaemia, and diffuse pulmonary infiltrates. Although most cases are idiopathic, secondary haemosiderosis linked to known diseases has also been observed. Most of the cases remain undiagnosed because the disease is very low on the list of differentials. There is no specified age for the disease. The present study reports on an adolescent female patient who presented with microcytic anaemia and bilateral lung infiltrates to the National Institute of Child Health (NICH), Karachi, a tertiary care hospital. She was diagnosed with Idiopathic pulmonary haemosiderosis after ruling out other possibilities. Keywords: Pulmonary haemosiderosis, Alveolar haemorrhages, Lung disease, Alveolar hypoventilation syndrome

Strigolactone (GR24) Application Positively Regulates Photosynthetic Attributes, Stress-Related Metabolites and Antioxidant Enzymatic Activities of Ornamental Sunflower (<i>Helianthus annuus</i> cv. Vincent’s Choice) under Salinity Stress

Strigolactones, a new group of phytohormones, are reported to improve plant tolerance to multiple abiotic stresses. A pot experiment was conducted to investigate the impact of synthetic strigolactone (GR24 at 0.001, 0.01 and 0.1 mg L−1) application on ornamental sunflowers (Helianthus annuus cv. Vincent’s Choice) grown under salt stress (150 mM NaCl). Salt stress was applied after 14 days, and SL was applied 25 days seed sowing. The results showed that amongst various GR24 concentrations, 0.01 mg L−1 proved to be superior, as it enhanced the photosynthetic rate (9.29%), transpiration rate (0.76%), stomatal conductance (77.5%), total soluble protein (0.55%) and K+ (14.63% in roots; 14.87% in shoots) and Ca2+ (12.63% in roots; 11.48% in shoots) contents under control conditions. Similarly, the leaf turgor potential (Ψp), osmotic potential (Ψs) and free proline, glycinebetaine (GB), superoxide dismutase (SOD), catalase (CAT) and peroxide (POD) contents increased by 58.17, 89.95, 159.04, 101.54, 74.42, 175.68 and 53.62%, respectively, under salt stress conditions. The leaf water potential (Ψw) decreased (−0.14%) and the malondialdehyde (MDA) content increased (16.65%) when treated with the 0.001 mg L−1 GR24 level. Meanwhile, hydrogen peroxide (H2O2) and Na+ concentrations in roots and shoots increased by 62.53%, 74.66% and 38.55% under saline conditions with a GR24 level of 0 mg L−1. Regarding the plant biomass, a GR24 level of 0.01 mg L−1 with salt stress greatly decreased the root (−47.27% and −50.45%) and shoot (−44.79% and −59.42%) fresh and dry weights, respectively, compared to control conditions. These results reveal that exogenously applied GR24 might be an effective way to mitigate the perilous impacts of salt stress in ornamental sunflower production. It is suggested that the use of molecular techniques to study different processes in which GR24 could play a vital part in various commercial floricultural crops is extremely imperative and can open novel horizons for future investigations in this exhilarating field of plant hormones