Self-expanding metal stents for the treatment of malignant colon obstruction from extra-colonic malignancy versus intra-colonic malignancy: a systematic review and meta-analysis.

BACKGROUND AND AIMS: The relative utility of self-expanding metal stent (SEMS) insertion for malignant colon obstruction (MCO) due to extra-colonic malignancy (ECM) versus intra-colonic malignancy (ICM) is understudied. METHODS: A systematic search was done from inception-April 2021 to identify reports of safety and efficacy of SEMS insertion for the treatment of MCO-ECM versus MCO-ICM. A meta-analysis of proportions, comparative meta-analysis to compute relative risks (RR), and mean differences (MD) was performed. Subgroup analyses and influence analyses were conducted. The certainty in estimates of effect(s) was assessed using the GRADE approach. RESULTS: Eight non-randomized studies were identified; 46% (39-53%) and 63% (59-67%) of patients in the ECM and ICM groups were male. Most obstructions were in the rectosigmoid colon in both ECM and ICM groups. SEMS insertion in MCO-ECM was associated with an increased risk of technical failure compared to MCO-ICM (RR 2.92; 1.13-7.54; Certainty: Very Low). Risk of clinical failure of SEMS was higher in MCO-ECM compared to MCO-ICM (RR 2.88; 1.58-2.52; Certainty: Very Low). The risk of clinical failure remained significant throughout the influence analysis, as well as on subgroup analysis. There was no significant difference in the risk of adverse events or luminal perforation with SEMS insertion among patients with MCO-ECM and MCO-ICM. On influence analysis, removal of one study unveiled a significant increase in the risk of luminal perforation in MCO-ECM (RR 3.22; 1.44-7.19; p = 0.004). CONCLUSION: SEMS for MCO-ECM may have a technical success rate comparable to or questionably worse than MCO-ICM, with low certainty in estimate of effects. SEMS deployment in MCO-ECM carries a higher risk of clinical failure, with a questionably higher risk of luminal perforation.

Hereditary Tyrosinemia Compounded With Hyperinsulinemic Hypoglycemia: Challenging Diagnosis of a Rare Case.

Hereditary tyrosinemia type 1 (HT-1) is a rare autosomal recessive disorder caused by a deficiency in the enzyme fumarylacetoacetate hydrolase (FAH), which catalyzes the final step in the tyrosine degradation pathway. Hereditary tyrosinemia is a heterogeneous disease with a wide spectrum of clinical manifestations involving hepatic, renal, or nervous systems. It has grave consequences if left untreated. Some of the late complications of hereditary tyrosinemia include cirrhosis, liver nodules, hepatocellular carcinoma, hypophosphatemic rickets, nephrocalcinosis, glomerulosclerosis, and chronic renal failure. Rarely, infants with hereditary tyrosinemia may present with persistent hypoglycemia, which may be a result of acute liver failure or hyperinsulinism. Hyperinsulinemic hypoglycemia (HH), caused by dysregulation of insulin secretion from pancreatic ß-cells, leads to insulin driven glucose entry into the tissues and inhibits glycolysis, gluconeogenesis, fatty acid release, and ketone body synthesis. Hyperinsulinemic hypoglycemia can cause severe, persistent hypoketotic hypoglycemia. Diagnosing tyrosinemia type 1 can be a challenge as it is a heterogeneous disorder with a wide variety of clinical manifestations and complications. We herein report a rare case of a three-day-old male neonate with HT-1 compounded with HH.