Cholestasis syndrome in a patient with hereditary spherocytosis

Abstract

Introduction: Among hereditary hemolytic anemias, hereditary spherocytosis (HS) is the one that occurs most frequently. Autosomal dominant (AD) and autosomal recessive (AR) inheritance patterns account for 75% and 25% of all HS cases, respectively. The prevalence of HS varies among different racial and ethnic regions, affecting approximately 1 in 2,000 people in Northern Europe, North America, and Japan, but is less common in African Americans and Southeast Asia. Cholestatic syndrome is usually one of the most common complications and occurs in patients between 10 and 30 years of age, although younger patients can also present it and cholecystectomy is a safe and effective radical treatment for this pathology. Objective: To describe the clinical evolution and the surgical management of the patient with spherocytosis who develops a cholestatic syndrome as one of the most frequent complications of this pathology. Materials and methods: The theoretical support of this clinical case, corresponding to the most updated information up to 5 years ago, was obtained through the literature search and access to scientific articles, books, magazines, and other physical and virtual documents through of professional seekers in the field of health. Conclusions: The evolution of the complications of hereditary spherocytosis, especially cholestatic syndrome, led to several days of hospital stay, but in most cases, they are usually resolved favorably in this case by means of laparoscopic cholecystectomy, satisfactory results were obtained and discharge. 22 days after admission.