SC Hemoglobinopathy (HbSC) with Osteoarticular Complications: Case Report
- 1 Department of Pediatric, Hospital Maternidade Sao Vicente de Paulo (HMSVP), Barbalha, Brazil
- 2 School of Medicine, Federal University of Cariri (UFCA), Barbalha, Brazil
- 3 Residence in Pediatrics, Federal University of Cariri (UFCA), Barbalha, Brazil
- 4 Residence in Pediatrics, Federal University of Cariri (UFCA), Barbalha, Brazil
- 5 School of Medicine, Federal University of Cariri (UFCA), Barbalha, Brazil
- 6 School of Medicine, Federal University of Cariri (UFCA), Barbalha, Brazil
- 7 Department of Pediatric, Hospital Maternidade Sao Vicente de Paulo (HMSVP), Barbalha, Brazil
Abstract
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.
- [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. https://doi.org/10.1590/S0004-27492011000500005
- 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. https://doi.org/10.1590/S1516-84842006000200017
- 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. https://doi.org/10.1016/j.jemermed.2016.05.042
- 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. https://doi.org/10.1371/journal.pmed.1001484
- 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. https://doi.org/10.1016/j.ejogrb.2016.05.002
- 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. https://doi.org/10.1111/trf.13280
- 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. https://doi.org/10.1002/14651858.cd011175.pub2
- 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. https://doi.org/10.1111/bjh.13270
- 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. https://doi.org/10.1590/S0102-311X2006000800019