Background: Hemolytic uremic syndrome (HUS) is characterized by microangiopathic hemolytic anemia (MAHA), thrombocytopenia, and acute kidney injury. The acute pancreatitis-associated HUS is a rare entity, and this case is one of the few pediatric cases reported. Case presentation: A 17-year-old girl referred to the emergency department with complaints of abdominal pain, fever, and vomiting. The skin and sclera were icteric. Murphy’s sign was positive. Hemogram was normal, biochemical analysis revealed an increase in liver function tests and cholestasis enzymes. Abdominal CT revealed multiple stones in the hydropic gallbladder lumen and the pancreas was edematous. With a diagnosis of acute pancreatitis supportive treatment was started. Acute kidney failure, cholestasis, anemia, and thrombocytopenia developed at the 2nd day of follow-up. Thrombotic thrombocytopenic purpura was excluded with normal ADAMTS-13 level. Intermittent hemodialysis and plasma exchange (PE) treatments were initiated, considering pancreatitis-related HUS. On the 3rd day of PE, the patient’s urine output exceeded 1 cc/kg per hour. No schistocyte was found in the peripheral smear after 7 PE and 5 hemodialysis sessions. Anemia, thrombocytopenia, and kidney functions improved. On the 15th day of the follow-up, endoscopic retrograde cholangiopancreatography performed, and gallbladder stones were removed. Conclusion: The pathogenesis of HUS developing after acute pancreatitis is not fully understood. The mechanism proposed is that acute pancreatitis triggers cytokine release, resulting in endothelial damage and HUS. In conclusion, HUS may rarely develop in patients with acute pancreatitis. With the early diagnosis and appropriate treatment, the kidney functions can be completely normalized.

The Medical Bulletin of Sisli Etfal Hospital

Adenosine deaminase 2 (ADA-2) deficiency is a rare autoinflammatory and immunodeficiency disorder that predisposes affected individuals to severe infections. We report the case of a 13-year-old boy with a homozygous CECR1 (c.11104 del) mutation who developed fatal breakthrough invasive aspergillosis caused by Aspergillus flavus. Despite combination antifungal therapy, multiple surgical interventions, and granulocyte transfusions, the infection progressed, leading to pulmonary dissemination. Fungal culture and polymerase chain reaction (PCR) confirmed A. flavus. This case underscores the aggressive course of fungal infections in ADA-2 deficiency and the limited efficacy of standard treatment strategies in the setting of profound immunosuppression and vascular invasion. Early recognition and novel therapeutic approaches are urgently needed to improve outcomes in this vulnerable population.