Health  Vol.9 No.7 , July 2017
SC Hemoglobinopathy (HbSC) with Osteoarticular Complications: Case Report
Background: Among hemoglobinopathies, the most prevalent in our population are hemoglobins S and C, which are capable of producing disease when homozygous. In cases of double heterozygotes with sickle hemoglobin C (SC), the disease is less expressive in its clinical condition and rarer. Case report: Patient has a previous hospitalization with pain in the joints in knee and hip and several febrile peaks. Upon physical examination, the patient had difficulty in walking, without edema, pedal and tibial posterior pulses present, with no signs of compartment syndrome. Complementary exams revealed anemia, leukocytosis and lymphopenia. The hemoglobin electrophoresis showed the SC Hemoglobinopathy. The treatment with antibiotic therapy according to the protocol (Oxacillin and Ceftriaxone) was restarted and submitted to joint drainage in affected limb. Conclusion: Osteomyelitis and septic arthritis in patients in the pediatric age group should be considered as serious infections that deserve hospitalization and more expressive treatment.
Cite this paper: Macedo, F. , Júnior, J. , Guimarães, F. , Tomaz, R. , Monte Alto, D. , Pinheiro, P. and Coelho, J. (2017) SC Hemoglobinopathy (HbSC) with Osteoarticular Complications: Case Report. Health, 9, 1075-1080. doi: 10.4236/health.2017.97078.

[1]   [1]Freitas, L.G.A., et al. (2011) Alteracoes retinianas apresentadas em pacientes portadores de hemoglobinopatia falciforme atendidos em um Servico Universitário de Oftalmologia. Arquivos Brasileiros de Oftalmologia, 74, 335-337.

[2]   Melo-Reis, P.R., et al. (2006) A importancia do diagnóstico precoce na prevencao das anemias hereditárias. Revista Brasileira de Hematologia e Hemoterapia, 28, 149-152.

[3]   Simon, E., Long, B. and Koyfman, A. (2016) Emergency Medicine Management of Sickle Cell Disease Complications: An Evidence-Based Update. Journal of Emergency Medicine, 51, 370-381.

[4]   Piel, F.B., Simon, I.H., Gupta, S., Weathehall, D. and Williams, T.N. (2013) Global Burden of Sickle Cell Anaemia in Children under Five, 2010-2050: Modelling Based on Demographics, Excess Mortality, and Interventions. PLoS Medicine, 10, e10001484.

[5]   Thame, M.M., Singh-Minott, I., Osmond, C. and Melbourne-Chambers, R.H. (2016) Pregnancy in Sickle Cell-Haemoglobin C (SC) Disease. A Retrospective Study of Birth Size and Maternal Weight Gain. European Journal of Obstetrics, Gynecology, and Reproductive Biology, 203, 16-19.

[6]   Benites, B.D., Benevides, T.C.L., Valente, I.S., Marques, J.F., Gilli, S.C.O. and Saad, S.T.O. (2016) The Effects of Exchange Transfusion for Prevention of Complications during Pregnancy of Sickle Hemoglobin C Disease Patients. Transfusion, 56, 119-124.

[7]   Asnani, M.R., Quimby, K.R., Bennett, N.R. and Francis, D.K. (2016) Interventions for Patients and Caregivers to Improve Knowledge of Sickle Cell Disease and Recognition of Its Related Complications. Cochrane Database of Systematic Reviews, 6, CD011175.

[8]   Oteng-Ntim, E., Ayensah, B., Knight, M. and Howard, J. (2014) Pregnancy Outcome in Patients with Sickle Cell Disease in the UK—A National Cohort Study Comparing Sickle Cell Anaemia (HbSS) with HbSC Disease. British Journal of Haematology, 169, 129-137.

[9]   Sommer, C.K., Stela, G.A., Wagner Sandrine, C. and Castro Simone, M. (2006) Triagem neonatal para hemoglobinopatias: experiência de um ano na rede de saúde pública do Rio Grande do Sul, Brasil. Cadernos de Saúde Pública, 22, 1709-1714.

[10]   Azevedo, E.S., Alves, A.F.P., Silva, M.C.B.O., Souza, M.G.F., Lima, A.M.V.M.D. and Azevedo, W. (1980) Distribution of Abnormal Hemoglobins and Glucose-6-Phosphate Dehydrogenase Variants in 1200 School Children of Bahia, Brazil. American Journal of Physical Anthropology, 53, 509-512.

[11]   Naoum, P.C. (1997) Hemoglobinopatias e Talassemias. Sarvier Ed. Livros Médicos, Sao Paulo.

[12]   Bandeira, F.M.G.C., Leal, M.C., Souza, R.R., Furtado, V.C., Gomes, Y.M. and Marques, N.M. (1999) Características de recém-nascidos portadores de hemoglobina “S” detectados através de triagem em sangue de cordao umbilical. Journal of Pediatrics, 75, 167-171.

[13]   Santos, L.R.O., da Rocha, S.S., Costa, R.d.S., de Araujo, O.D., Oliveira, F.B.M. and Barros, R.S. (2012) Family Care for Children with Sickle Cell Disease. Revista de Enfermagem da UFPI, 1, 124-127.

[14]   Squiers, J.J., Edwards, A.G., Parra, A. and Hofmann, S.L.J. (2016) Acute Splenic Sequestration Crisis in a 70-Year-Old Patient with Hemoglobin SC Disease. Journal of Investigative Medicine High Impact Case Reports, 4, Article ID: 2324709616638363.

[15]   Puccini, P.F., Ferranini, M.A.G. and Iazzetti, A.V. (2012) Osteomielite hematogênica aguda em Pediatria: análise de casos atendidos em hospital universitário. Revista Paulista De Pediatria, 30, 353-358.

[16]   Clark, B.E. and Thein, S.L. (2004) Molecular Diagnosis of Haemoglobin Disorders. Clinical and Laboratory Haematology, 26, 159-176.

[17]   Agência Nacional de Vigilancia Sanitária (ANVISA) (2002) Manual de diagnóstico e tratamento de doencas falciformes. Ministério da Saúde, Brasília.

[18]   Bitaraes, E.L., Oliveira, B.M. and Viana, M.B. (2008) Adesao à antibioticoterapia profilática em criancas com anemia falciforme: Um estudo prospectivo. Journal of Pediatrics, 84, 316-322.

[19]   Silva, W.S., Oliveira, R.F., Ribeiro, S.B., da Silva, I.B., de Araújo, E.M. and Baptista, A.F. (2016) Screening for Structural Hemoglobin Variants in Bahia, Brazil. International Journal of Environmental Research and Public Health, 13, 225.