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1.
Eur J Haematol ; 94(2): 157-61, 2015 Feb.
Article in English | MEDLINE | ID: mdl-25039473

ABSTRACT

BACKGROUND: Sickle cell disease (SCD) has extremely variable phenotypes, and several factors have been associated with the severity of the disease. OBJECTIVES: To analyze the chronic complications of SCD and look for predictive risk factors for increased severity and number of complications. METHODS: Retrospective study including all children followed for SCD in the Paediatric Haematology Unit of a tertiary hospital in Portugal, who completed 17 yr old between the years 2004 and 2013. RESULTS: We identified 44 patients, 55% female and 98% black. Chronic complications occurred in 80% of cases. Slight dilatation of the left ventricle was the most frequent complication (47.7%), followed by respiratory function disturbs (43.2%), microlithiasis or cholelithiasis (40.9%), increased flow velocity of cerebral arteries (31.8%), enuresis, delayed puberty and bone abnormalities (6.8% each), sickle cell retinopathy and leg ulcer (4.6% each) and recurrent priapism (2.3%). We identified a statistically significant association between leukocytes >15 000/µL and a higher number of hospitalizations (P < 0.001) and chronic complications of the disease (P = 0.035). The occurrence of dactylitis in first year of life was also significantly associated with a higher number of hospitalizations (P = 0.004) and chronic complications (P = 0.018). The presence of α-thalassemia was associated with a lower number of chronic complications (P = 0.036). CONCLUSIONS: Leucocytosis and dactylitis in the first year of life can be predictors of SCD severity, while the presence of α-thalassemia can be protective. The determination of early predictors of chronic complications of SCD may improve the comprehensive care of these patients.


Subject(s)
Anemia, Sickle Cell/epidemiology , Adolescent , Anemia, Sickle Cell/blood , Child , Child, Preschool , Chronic Disease , Comorbidity , Female , Humans , Infant , Infant, Newborn , Male , Portugal/epidemiology , Prevalence , Prognosis , Retrospective Studies , alpha-Thalassemia
2.
BMJ Case Rep ; 20132013 Dec 16.
Article in English | MEDLINE | ID: mdl-24343806

ABSTRACT

Epstein-Barr virus infection is common in children, usually presenting as infectious mononucleosis, including fever, tonsillitis and lymphadenopathy associated with self-resolving increase in transaminases. Cholestasis is rare in children with only a few cases reported but it was described in up to 55% of the adult population affected. We present a case of a 6-year-old boy with fever, vomiting and choluria. The physical examination showed hepatomegaly and jaundice and was otherwise unremarkable. The laboratory studies revealed increased transaminases (aspartate aminotransferase 97 U/L, alanine aminotransferase 166 U/L), hyperbilirubinaemia (total bilirubin 3.2 mg/dL, direct bilirubin 2.89 mg/dL) and increased γ-glutamyl transpeptidase (114 mg/dL). Urine urobilinogen was increased. The abdominal ultrasound showed hepatomegaly. Epstein-Barr viral capsid antibody IgM was positive and IgG was negative. Serological studies for other viruses were negative. We underline the need to consider Epstein-Barr virus in the cholestatic hepatitis differential diagnosis, in order to avoid unnecessary investigations.


Subject(s)
Cholestasis/diagnosis , Epstein-Barr Virus Infections/diagnosis , Hepatitis, Viral, Human/virology , Herpesvirus 4, Human , Liver/pathology , Transaminases/blood , Bilirubin/blood , Capsid/immunology , Child , Cholestasis/etiology , Cholestasis/virology , Diagnosis, Differential , Epstein-Barr Virus Infections/complications , Epstein-Barr Virus Infections/immunology , Epstein-Barr Virus Infections/virology , Hepatitis, Viral, Human/complications , Hepatitis, Viral, Human/diagnosis , Hepatitis, Viral, Human/metabolism , Hepatomegaly/diagnosis , Hepatomegaly/etiology , Hepatomegaly/virology , Herpesvirus 4, Human/immunology , Humans , Hyperbilirubinemia/diagnosis , Hyperbilirubinemia/etiology , Hyperbilirubinemia/virology , Immunoglobulin G/metabolism , Immunoglobulin M/metabolism , Jaundice/diagnosis , Jaundice/etiology , Jaundice/virology , Liver/enzymology , Liver/virology , Liver Function Tests , Male , Urobilinogen/urine , gamma-Glutamyltransferase/blood
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