A clinical case of liver iron overload in a child with liver cavernoma and extrahepatic portal hypertension
- Authors: Belykh N.A.1, Kotova P.O.1, Piznyur I.V.1, Gavrilova V.V.1
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Affiliations:
- Ryazan State Medical University, Ryazan
- Issue: Vol 15, No 2 (2024)
- Pages: 85-91
- Section: Clinical observation
- URL: https://journals.eco-vector.com/pediatr/article/view/636251
- DOI: https://doi.org/10.17816/PED15285-91
- ID: 636251
Cite item
Abstract
Iron overload syndrome is a pathological condition that occurs as a result of congenital disorders of its regulation, as well as due to repeated hemotransfusions, hemolytic anemia or excessive intake of iron preparations. Accurate data on the prevalence of secondary liver iron overload are not available in the literature. Liver biopsy remains the gold standard for assessing iron overload, however, non-invasive methods, in particular magnetic resonance relaxometry, also allow for an accurate quantitative assessment of the body’s iron overload. In secondary iron overload, chelation therapy is the only therapeutic option to prevent excessive accumulation of iron in tissues, which reduces complications and improves patient survival. The presented clinical case in a 5-year-old child is interesting for a combination of factors contributing to the formation of liver iron overload, as well as the rapid development of hyperferritinemia. The boy has a history of prolonged neonatal jaundice. At 6 months old, the child was first diagnosed with grade 2 iron deficiency anemia, splenomegaly and liver cavernoma were detected during abdominal ultrasound, and therefore the child was referred for consultation to a surgeon. At the age of 1.5 years old, the boy underwent surgical treatment — the formation of splenic-renal anastomosis due to the danger of bleeding from dilated veins of the esophagus. However, in the postoperative period, the failure of the anastomosis was noted, and therefore an H-shaped mesenteric-caval anastomosis was performed. At 2 years old of age, a planned examination revealed an increase in ferritin to 1149.2 ng/ml. During the molecular genetic study, hereditary hemochromatosis was excluded. After the start of chelation therapy, there was a decrease in ferritin levels in dynamics and with its normalization in October 2023, therapy was canceled. The boy is currently under the supervision of a hematologist, a pediatric surgeon and a pediatrician. The clinical case demonstrates the peculiarities of the course of liver iron overload in a child with liver cavernoma and extrahepatic portal hypertension, which required a personalized approach to diagnosis and treatment.
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About the authors
Natalia A. Belykh
Ryazan State Medical University, Ryazan
Author for correspondence.
Email: nbelyh68@mail.ru
ORCID iD: 0000-0002-5533-0205
SPIN-code: 2199-6358
MD, PhD, Dr. Sci. (Medicine), Associate Professor, Head, Head of the Department of faculty and polyclinic Pediatrics with the course of Pediatrics of the Faculty of Additional Professional Education
Russian Federation, 9 Vysokovoltnaya str., Ryazan, 390026
Polina O. Kotova
Ryazan State Medical University, Ryazan
Email: polina.iertskina@mail.ru
ORCID iD: 0000-0002-0792-3233
SPIN-code: 1289-0102
Student VI courses, Head of the Department of faculty and polyclinic Pediatrics with the course of Pediatrics of the Faculty of Additional Professional Education
Russian Federation, 9 Vysokovoltnaya str., Ryazan, 390026Inna V. Piznyur
Ryazan State Medical University, Ryazan
Email: innaabramova@yandex.ru
Assistant Professor, Head of the Department of faculty and polyclinic Pediatrics with the course of Pediatrics of the Faculty of Additional Professional Education
Russian Federation, 9 Vysokovoltnaya str., Ryazan, 390026
Valeria V. Gavrilova
Ryazan State Medical University, Ryazan
Email: valvad66@bk.ru
ORCID iD: 0009-0001-6841-036X
Pediatrician, Head of the Pediatric Department No. 2
Russian Federation, 9 Vysokovoltnaya str., Ryazan, 390026References
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