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1.
Eur J Pediatr ; 182(4): 1749-1754, 2023 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-36763189

RESUMEN

Heart disease is the primary cause of death in patients with beta-thalassemia major. The study aimed to determine the association between vitamin D and left ventricular function in patients with beta-thalassemia major with iron overload. A cross-sectional hospital-based study was conducted, where the vitamin D and ferritin levels of children living with beta-thalassemia major were measured, and left ventricular function was assessed utilizing ejection fraction (EF) and fractional shortening (FS) using 2D echocardiography. The mean serum ferritin was 4622 ± 2289 ng/ml, and the mean serum vitamin D levels were 22 ± 7.7 ng/ml. The mean values of EF were 62.30 ± 6.9%, and FS was 31.21 ± 4.8%. Statistically significant negative correlation (r = -0.447, p < 0.001) was found between vitamin D and serum ferritin values, and a significant positive association was found between vitamin D levels concerning EF and FS with a p-value of 0.034 and 0.014, respectively.Conclusion: It was observed  that increasing ferritin was associated with lower vitamin D levels which in turn influenced fractional shortening /cardiac function in these patients.  What is Known: • Patients with Beta Thalassemia major on long term transfusion are prone to develop heart disease / cardiac failure due to chronic iron overload. What is New: • Patients with beta thalassemia major on long term term transfusions with iron overload who are vitamin D deficient are more prone to the cardiac complications which inturn can be prevented by vitamin D supplementation.


Asunto(s)
Cardiopatías , Sobrecarga de Hierro , Talasemia beta , Niño , Humanos , Talasemia beta/complicaciones , Función Ventricular Izquierda , Vitamina D , Estudios Transversales , Sobrecarga de Hierro/complicaciones , Ferritinas , Vitaminas
2.
J Pediatr Hematol Oncol ; 44(1): e253-e254, 2022 01 01.
Artículo en Inglés | MEDLINE | ID: mdl-33306604

RESUMEN

Hemophagocytic lymphohistiocytosis (HLH) is a multisystem disease wherein there is an exaggerated immune system activation following a trigger such as infection, malignancy, or autoimmune diseases. Here we report a case of a 3-year-old boy who presented to us with fever, was diagnosed with dengue fever, and treatment started for the same. Clinical response was poor to treatment and high-grade fever persisted. Subsequent evaluation showed Plasmodium falciparum malaria and treatment was initiated with antimalarial drugs. Further clinical deterioration with poor trend of laboratory values over the next few days prompted evaluation for HLH; workup was positive satisfying the HLH-2004 criteria and IV dexamethasone was started. The child gradually improved and was discharged with normal counts on follow-up over the next 3 months. This article emphasizes on the importance of high degree of suspicion, early workup, and initiation of treatment for HLH for a better outcome.


Asunto(s)
Virus del Dengue/metabolismo , Dengue , Linfohistiocitosis Hemofagocítica , Malaria Falciparum , Plasmodium falciparum/metabolismo , Preescolar , Dengue/sangre , Dengue/diagnóstico , Dengue/terapia , Humanos , Linfohistiocitosis Hemofagocítica/sangre , Linfohistiocitosis Hemofagocítica/parasitología , Linfohistiocitosis Hemofagocítica/terapia , Linfohistiocitosis Hemofagocítica/virología , Malaria Falciparum/sangre , Malaria Falciparum/diagnóstico , Malaria Falciparum/terapia , Masculino
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