Introduction:. Patients with Down syndrome (DS) have an increased risk of developing autoimmune diseases. This is a rare case of a pediatric patient with DS with an initial clinical profile of diabetic ketoacidosis.Case presentation:. 6-year-old male patient with symptoms suggestive of diabetes mellitus type 1 (DM1) of 15 days of evolution (polyuria, polydipsia, polyphagia and loss of 2 kilos of weight), who was admitted to the emergency department of the Hospital de San José, in Bogotá, Colombia, with uncontrollable vomiting and dehydration. The tests performed confirmed moderate ketoacidosis: glycometry: 592 mg/dL, pH: 7.19, HCO3: 10 mmol/L, PCO2: 45, PO2: 95 and lactic acid: 1.4 mmol/L. Management with isotonic fluids and intravenous insulin therapy was initiated and the patient was transferred to the pediatric intensive care unit, where ketoacidosis was controlled in approximately 10 hours. Subcutaneous insulin schedule was initiated without complications.Discussion:. This case highlights the importance of monitoring possible autoimmune complications in patients with DS, since the risk of developing them is 4.2 times higher than in the general population.Conclusion:. This case calls on to contemplate autoimmune complications in patients with DS during clinical practice. Although they are not part of the most frequent reasons for consultation, they cannot be underestimated and should be suspected and treated in a timely manner.
INTRODUCTION: Gastric varices are often associated with formation of spontaneous left-sided porto-systemic shunts, which arise to relieve portal hypertension or bypass obstruction. Gastro-renal shunts are the most common (80%), followed by gastro-caval shunts. Balloon Retrograde Transvenous Obliteration remains one of the primary measures to prevent re-bleeding from gastric varices. This procedure consists of inserting a balloon into the outflow vein of a shunt, allowing injection of a sclerosant for occlusion. It has the advantage of not diverting blood to bypass the liver, but it can worsen portal pressure and increase risk for ascites or esophageal variceal bleeding. CASE DESCRIPTION/METHODS: 67-year-old male with past medical history of untreated Hepatitis C was admitted after syncope. He also had nausea, lightheadedness and hematemesis. In the ED, his blood pressure was stable with an Hb of 9.5 mg/dL. Ceftriaxone, an octreotide drip and PPI were promptly started. 3 units of blood were transfused. Urgent endoscopy did not reveal esophageal varices, but profuse active bleeding in the fundus was seen. Attempts to control the hemorrhage were ineffective due to lack of visibility. CT scan of the abdomen showed a cirrhotic liver with a 6 cm mass in the right lobe and a large gastric varix with a gastrorenal shunt. Importantly, there was no evidence of portal or splenic vein thrombosis. Left-sided TIPS was tried, but attempts to pass from left portal vein to left hepatic vein were futile due to challenging anatomy. After multiple transfusions the patient eventually underwent successful BRTO. He had no further bleeding and was ultimately diagnosed with HCC. DISCUSSION: BRTO is indicated primarily for prevention of recurrent gastric variceal bleeding. It is useful in patients with elevated MELD score, right-sided heart failure or hepatic encephalopathy, who are at high risk for TIPS. Patients with gastric variceal bleeding and concomitant HCC usually have more advanced cirrhosis and lower possibility of HCC treatment. This is due to bleeding-related hepatic decompensation and overall poorer liver functional reserve. Although BRTO has its disadvantages, its ability to divert blood flow towards the liver is thought to possibly preserve hepatic function. This might make patients with HCC and gastric varices good candidates for BRTO, allowing better possibility for treatment and increased survival. Further long-term studies are needed to evaluate prognosis and complications of BRTO in these patients.
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