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Redalyc.Hepatobiliary Abnormalities in Pediatric Patients with Sickle Cell Acta Gastroenterológica Latinoamericana ISSN: 0300-9033 [email protected] Sociedad Argentina de Gastroenterología Argentina Almeida, Roberto Paulo; Dantas Ferreira, Cibele; Conceição, Joseni; Franca, Rita; Lyra, Isa; Rodrigues Silva, Luciana Hepatobiliary abnormalities in pediatric patients with sickle cell disease Acta Gastroenterológica Latinoamericana, vol. 39, núm. 2, junio, 2009, pp. 112-117 Sociedad Argentina de Gastroenterología Buenos Aires, Argentina Available in: http://www.redalyc.org/articulo.oa?id=199317359008 How to cite Complete issue Scientific Information System More information about this article Network of Scientific Journals from Latin America, the Caribbean, Spain and Portugal Journal's homepage in redalyc.org Non-profit academic project, developed under the open access initiative N MANUSCRITO ORIGINAL ACTA GASTROENTEROL LATINOAM - JUNIO 2009;VOL 39:Nº2 Hepatobiliary abnormalities in pediatric patients with sickle cell disease Roberto Paulo Almeida,1 Cibele Dantas Ferreira,2 Joseni Conceição,1,2 Rita Franca,1,2 Isa Lyra,1 Luciana Rodrigues Silva 2 1 Postgraduate Program in Medicine and Health, Federal University of Bahia. Brasil. 2 Department of Gastroenterology and Pediatric Hepatology, Federal University of Bahia. Brasil. Acta Gastroenterol Latinoam 2009;39:112-117 Summary medad de células falciformes en la ciudad de Salvador, Objetive: to describe clinical, laboratory and ultraso- Brasil. Material y métodos: los pacientes pediátricos nographic abnormalities in the hepatobiliary system of con enfermedad de células falciformes fueron evaluados pediatric patients with sickle cell disease in the city of clínicamente. Se revisaron sus historias clínicas y se exa- Salvador, Brazil. Material and Methods: pediatric minaron los hallazgos de las pruebas complementarias patients with sickle cell disease were clinically evalua- para identificar las anormalidades hepatobiliares. Re- ted, their charts were reviewed and findings of supple- sultados: se evaluó un total de 134 pacientes, 65 mentary tests were examined to identify hepatobiliary (48.9%) de los cuales presentaron hepatomegalia. La abnormalities. Results: a total of 134 patients were elevación de las transaminasas estuvo presente en el evaluated, 65 of whom (48.9%) presented hepatome- 42.2% de los casos y el 11.4% presentaron colelitiasis. galia. Elevated transaminases were present in 42.2% Hubo una asociación estadísticamente significativa en- and 11.4% presented cholelithiasis. There was a statis- tre la presencia de hepatomegalia y la homocigosis SS, y tically significant association between the presence of entre la colelitiasis y los pacientes mayores de 10 años de hepatomegalia and SS homozygotes and between cho- edad (p=0.001 y p=0.00, respectivamente). Conclu- lelithiasis and patients over 10 years of age (p=0.01 sión: fueron frecuentes las anormalidades hepatobilia- and p=0.00, respectively). Conclusion: hepatobiliary res en los pacientes con enfermedad de células falcifor- abnormalities in patients with sickle cell disease were mes, particularmente en los portadores de hemoglobina common, particularly in patients with hemoglobin SS SS y los adolescentes. and in adolescent patients. Palabras claves: enfermedad de células falciformes, pa- Key words: sickle cell disease, pediatric patients, liver cientes pediátricos, anormalidades hepáticas. abnormalities. Sickle cell anemia (HbSS) is caused by a point Alteraciones hepatobiliares en mutation (GAG " GTG) in the ß-globin gene, pacientes pediátricos con la which defines the substitution of glutamic acid by enfermedad de células falciformes valine in position 6 of the ß chain of amino-acids, Resumen resulting in abnormal hemoglobin, hemoglobin S Objetivo: describir las anormalidades clínicas, analíti- (HbS). This hemoglobin presents physical and che- cas y ecográficas de los pacientes pediátricos con enfer- mical abnormalities and a tendency to polymeriza- tion under certain circumstances. The presence of HbS associated with other hereditary variants of he- Correspondencia: Cibele Dantas Ferreira moglobin such as hemoglobin C, D and S/ß thalas- Centro de Estudos em Gastroenterologia e Hepatologia Pediátricas Centro Pediátrico Professor Hosannah de Oliveira – 4º andar semia, among others, characterizes sickle cell disea- Rua Padre Feijó, s/n, Canela, 40110-170, Salvador, BA, Brazil. se.1-3 It is considered the most common monogenic Telephone: +55 71 3339-6139 / 8201-2945 disease in Brazil. Around 3,500 children are estima- FAX: +55 71 3339-6100 E-mail: [email protected] ted to be born with this disease annually and anot- 112" Alteraciones hepatobiliares en el paciente pediátrico Roberto Paulo Almeida y col her 200,000 with the sickle cell trait.4 Reflecting the The patients in the study were then divided into ethnic characteristics of this region, the data from two groups: HbSS patients and non-HbSS patients. the Bahia State Program for Neonatal Screening in- Student’s t-test was used to compare the mean va- dicate a prevalence rate for this disease of 1:650 li- lues of laboratory tests, and the chi-square test was veborn infants, which makes sickle cell disease a sig- used for comparisons between proportions, correc- nificant public health issue in this region.5,6 tions being made using Fisher’s exact test. p-values Sickle cell disease is a multisystemic disease with a <0.05 were considered significant. great variability of clinical manifestations modula- Prior to initiation, the project had been submitted ted by genetic and environmental factors. Liver in- to and approved by the Internal Review Board of volvement is common in patients with sickle cell di- the Professor Edgard Santos Teaching Hospital, Fe- sease, principally in homozygotic patients, and oc- deral University of Bahia. curs as a result of multiple transfusions, of the supe- rimposition of hepatitis by hepatotropic or non-he- patotropic viruses, by other infections, cholelithiasis Results or as a result of vascular phenomena related to the During the study period, 134 patients with sickle primary disease itself. Liver dysfunction may vary cell disease were evaluated. The mean age of patients from a self-limiting cholestasis to cases of cirrhosis was 7.09 ± 4.36 years (range 0.38 – 18.39 years). and liver failure; however, severe liver disease rarely The following phenotypes were found: SS in 100 occurs in these patients unless other concomitant patients (74.6%); SC in 29 (21.6%) and Sβ-thalas- pathologies, such as infection with hepatitis C virus semia in 5 (3.8%). The mean age of the children at (HCV) or human immunodeficiency virus (HIV) the time of diagnosis was around two years. Table 1 are present.7-9 describes the signs and symptoms that led to the Although sickle cell disease has been studied in- diagnostic investigation of sickle cell disease. No- tensely, there is a scarcity of data in the literature on tably, skin or mucosal pallor were reported in 41 pa- the liver abnormalities associated with this patho- tients (22.4%), neonatal screening test in 18 logy, particularly in the pediatric age-group. The (9.84%), fever in 17 (9.29%), joint swelling in 15 objective of the present study was to describe clini- (8.2%), painful crises in 10 (5.46%) and pain in cal, laboratory and ultrasonographic abnormalities limbs in 10 (5.46%). in the hepatobiliary system of pediatric patients The principal findings of the physical and supple- with sickle cell disease in the city of Salvador.7-14 mentary examinations are described in Table 1. He- patomegaly was often noted, being found in 65 pa- tients (48,9%), and jaundice in 81 (60,4%). Patients and Methods With respect to the laboratory tests, aspartate The present descriptive, cross-sectional study was aminotransferase (AST) was above the normal ran- carried out in the pediatric outpatient hematology clinics of the Bahia Hematology Foundation and ge in 64 patients (47.8%), while alanine amino- the Professor Edgard Santos Teaching Hospital of the transferase (ALT) was high in 19 (14.2%). Results Federal University of Bahia between July 2002 and of bilirubin had been recorded in 101 patients. In- October 2003, with the objective of evaluating chil- direct bilirubin was elevated in 60 (59.4%) and di- dren and adolescents attending these clinics who rect bilirubin in 65 (64.4%). had been diagnosed with sickle cell disease. Results of serum ferritin levels had been recorded All patients of 0-18 years of age with sickle cell di- in 73 patients. They were found elevated in 40 sease who attended the clinic for consultation du- (54.8%), with a mean value of 256.2 ± 278.2 ring this period were invited to participate in the ng/ml. In these patients, the mean number of trans- study. If they agreed to participate, the patients an- fusions reported was 5.59, while it was 2.5 in the d/or their guardians signed an informed consent group of patients with normal ferritin (p=0.022). form. Then, an interview and a physical examina- Of the interviewed children, 80 (59.7%) had un- tion were performed by one of the investigators and dergone a blood transfusion at some time, with a clinical and laboratory results, biopsy findings and mean of 4.11 ± 4.57 transfusions per patient, ran- results of ultrasonography scans of the liver and bi- ging from none to 22 transfusions in one case. liary tracts were recorded. Mean hemoglobin level was 8.49 ± 3.45 mg/dL. #113 Acta Gastroenterológica Latinoamericana – Vol 39 / N° 2 / Junio 2009 Tabla 1. Characteristics of pediatric patients with sickle 0.6 doses per patient). None of the children had cell disease. been vaccinated against hepatitis A. Clinical and laboratory characteristics n (%) χ2 P Only two patients had been submitted to a liver biopsy, one of whom had positive serology for hepa- titis C. In both cases, sinusoidal dilatation and peri- sinusoidal fibrosis were present. Only the patient with HCV infection had lymphocyte infiltration and a diagnosis suggestive of hepatitis. Data were summarized in table 1. Statistically sig- nificant correlations were found between lithiasis and age and between hepatomegaly and type of he- moglobinopathy.
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