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1.
Cureus ; 13(1): e12877, 2021 Jan 23.
Artigo em Inglês | MEDLINE | ID: mdl-33633906

RESUMO

Introduction Excess iron accumulation occurs mainly in organs such as reticuloendothelial cells, the pituitary gland, and the pancreas in beta-thalassemia because of blood transfusions. In the present study, it was aimed to investigate the relationship between T2* values on magnetic resonance imaging (MRI) and clinically diagnosed pituitary endocrinological disorders in children with thalassemia major. Methods This study enrolled patients diagnosed with beta-thalassemia at pediatric hematology outpatient clinics. In the study, in addition to the medical history of the patients, routinely performed tests, including hemoglobin electrophoresis, routine biochemical tests, and tests for pubertal development (follicle-stimulating hormone (FSH), luteinizing hormone (LH), estradiol, testosterone, etc.), as well as iron deposition measured by hepatic MRI T2* (STAR) sequence, were retrospectively assessed. A total of 29 patients were enrolled. Results Hypothyroidism was detected in 34.6% (9/26) of patients, short stature in 37% (10/27), and pubertal retardation in 50% (14/28) of the patients. There was no significant correlation between hypothyroidism and pituitary MRI T2* values. No significant correlation was found between laboratory parameters and pituitary MRI examination. Although the sensitivity of T2* levels could rise above 80%, their specificity remained low. This is one of the major limitations of the pituitary MR T2* study for the prediction of short stature. The best lower cut-off level of MR T2* to predict short stature was found 14.6 ms. Conclusion The diagnostic specificity pituitary MR examination levels for short stature were detected as low. Thus, the clinical standardization and validation of pituitary MR T2* values examination are needed before clinical follow-up and multifaceted studies are needed.

2.
Turk J Pediatr ; 63(6): 1103-1107, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-35023663

RESUMO

BACKGROUND: Based on the information obtained so far, COVID- 19 is relatively mild in children. We will present a 6-month-old male patient infected with COVID -19 in April 2020, while receiving HLH 2004 chemotherapy protocol with the diagnosis of familial (Genetic / Primary) Hemophagocytic Lymphohistiocytosis (HLH). CASE: Herein we present a case accompanied by a defective perforin gene defect in the primary HLH pathogenesis, Covid-19 infection with the presence of fever and hyperferritinemia, which was evaluated in favor of reactivation and the patient was given both the HLH-2004 chemotherapy protocol treatment and COVID -19 therapy as recommended by the guidelines. Our patient improved clinically and in terms of laboratory test results at the end of the 15 < sup > th < /sup > day of hospitalization and was discharged. CONCLUSIONS: It should be remembered that COVID-19 can be seen with different clinical manifestations in the pediatric age group, and COVID-19 tests should be recommended, especially in children with immunosuppression and fever.


Assuntos
COVID-19 , Linfo-Histiocitose Hemofagocítica , Criança , Febre , Humanos , Lactente , Linfo-Histiocitose Hemofagocítica/diagnóstico , Masculino , SARS-CoV-2
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