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SLE and Sickle cell disease

Ahmed B Idris, Ala Abdulgayoom, Eman Mudawi, A M El Hassan, Elwaleed Mohamed Elamin, Lamyaa Ahmed Mohamed El Hassan
In spite of the wide distribution of sickle cell disease (SCD) in Africa, an association with systemic lupus erythromatosis (SLE) is seldom reported. This may be due to the poor association between the two diseases or the high prevalence of missed cases. Progressive renal injury is prominent in both SCD and SLE. In this communication, we are presenting a case of an 11-year-old male who presented with sickle cell nephropathy that manifested as nephrotic syndrome with no response to conservative therapy, alongside unexplained massive hemolysis...
May 2015: Saudi Journal of Kidney Diseases and Transplantation
Teresa Cristina M V Robazzi, Crésio Alves, Laís Abreu, Gabriela Lemos
OBJECTIVE: To report a case of coexisting systemic lupus erythematosus (SLE) and sickle cell disease (SCD) with a review of the literature on the topic. METHODOLOGY: Report of case and research of the association between SLE and SCD in literature through scientific articles in health sciences databases, such as LILACS, MEDLINE/Pubmed and Scielo, until May 2012. Descriptors used: 1. Sickle cell anemia; 2. Sickle cell disease; 3. Systemic lupus erythematosus; 4. Hemoglobinopathies...
January 2015: Revista Brasileira de Reumatologia
Babatunde Gbolahan Ogundunmade, Unyime Sunday Jasper
BACKGROUND: Bilateral facial nerve palsy is a relatively rare presentation and often points to a serious underlying medical condition. Several studies have reported presentation of bilateral facial nerve palsy in association with Lyme disease, Guillain-Barre syndrome, systemic lupus erythematosus, human immunodeficiency virus, sarcoidosis, diabetes and Hanson disease. While unilateral facial nerve palsy is sometimes associated with hemiplegia in sickle cell patients, no case of bilateral facial nerve palsy have been reported in the literature...
2014: BMC Research Notes
Vinay Minocha, Fauzia Rana
Introduction. The diagnosis of systemic lupus erythematosus (SLE) in patients with sickle cell disease (SCD) can be difficult to establish because the musculoskeletal, central nervous system, and renal manifestations are similar in both diseases. In the presented case, we highlight the diagnostic challenge that can evolve in patients with a concurrence of both diseases and we establish the importance of early recognition and treatment of lupus nephritis in patients with SCD. Case Presentation. We present a case of a 31-year-old African American female with sickle-C disease (hemoglobin SC) who was admitted to our hospital with complaints of periumbilical abdominal pain associated with intractable nausea and vomiting, abdominal distension, and worsening lower extremity edema...
2013: Case Reports in Hematology
Regina D Crawford, Lindsey Kalhagen, Endi Wang, Marilyn J Telen
Kikuchi-Fugimoto's Disease (KFD), also known as histiocytic necrotizing lymphadenitis, is most frequently seen in young women and has been associated with autoimmune disorders such as polymyositis and systemic lupus erythematosus. It is generally a self-limiting disease with recovery time ranging from weeks to months. A typical presentation of KFD includes painful cervical lymphadenopathy, usually consisting of unilateral involvement of the posterior cervical chain. To date, this condition has not been described in patients with sickle cell disease...
September 2012: Journal of the National Medical Association
Mouna Maamar, Zoubida Tazi-Mezalek, Hicham Harmouche, Wafaa Mounfaloti, Mohammed Adnaoui, Mohammed Aouni
UNLABELLED: HASH(0x4b520b0) INTRODUCTION: The occurrence of systemic lupus erythematosus has been only rarely reported in patients with sickle-cell disease. CASE PRESENTATION: We describe the case of a 23-year-old North-African woman with sickle-cell disease and systemic lupus erythematosus, and discuss the pointers to the diagnosis of this combination of conditions and also present a review of literature. The diagnosis of systemic lupus erythematosus was delayed because our patient's symptoms were initially attributed to sickle-cell disease...
2012: Journal of Medical Case Reports
Geraldo Bezerra da Silva Junior, Elizabeth De Francesco Daher, Francisco Airton Castro da Rocha
The osteoarticular involvement in sickle cell disease has been poorly studied and it is mainly characterized by osteonecrosis, osteomyelitis and arthritis. The most frequent complications and those that require hospital care in sickle cell disease patients are painful vaso-occlusive crises and osteomyelitis. The deoxygenation and polymerization of hemoglobin S, which results in sickling and vascular occlusion, occur more often in tissues with low blood flow, such as in the bones. Bone microcirculation is a common place for erythrocyte sickling, which leads to thrombosis, infarct and necrosis...
2012: Revista Brasileira de Hematologia e Hemoterapia
N P Shilkina
From June 10th -14th, 2011, Yaroslavl played host to the traditional VIII International Conference <Systemic Circulation, Microcirculation and Haemorheology,> (from angiogenesis to central circulation) with a school for young scientists. The reports discussed cellular and molecular mechanisms of changes in the microrheological properties of RBCs and WBCs. as well as a role of a series of signal compounds and their receptors in regulation of angiogenesis.Also presented were the results of using new methods of investigations such as atomic-power microscopy, computer-assisted video-biomicroscopy of vessels of bulbar conjunctiva, bioimpedance spectroscopy...
2012: Angiologii︠a︡ i Sosudistai︠a︡ Khirurgii︠a︡, Angiology and Vascular Surgery
Marios G Lykissas, Ioannis D Gelalis, Ioannis P Kostas-Agnantis, Georgios Vozonelos, Anastasios V Korompilias
Despite the large number of the outstanding researches, pathogenesis of osteonecrosis remains unknown. During the last decades the hypothesis that increased intravascular coagulation may be the pathogenetic mechanism which leads to osteonecrosis is gaining constantly support. Both primary factors of hyper-coagulability, such as resistance to activated protein C, protein C and protein S deficiency, low levels of tissue plasminogen activator, high levels of plasminogen activator inhibitor, von Willebrand factor, lipoprotein (a), and secondary factors of hypercoagulability with factors potentially activating intravascular coagulation, such as pregnancy, antiphospholipid antibodies, systemic lupus erythematosus, hemoglobinopathies and sickle cell disease, and hemato-oncologic diseases are discussed in this article...
May 9, 2012: Orthopedic Reviews
C Marrec, G Picherot, C Kandel, M Caquard
Kikuchi disease is a histiocytic necrotizing lymphadenitis revealed by cervical lymphadenopathy and prolonged fever. It occurs predominantly in young Asian women. The pathophysiology is unknown; the main hypothesis is an autoimmune process. Although the disease usually resolves spontaneously in a few weeks or months, it may progress to systemic lupus erythematosus. Kikuchi disease is rare in children, but it should be considered a potential diagnosis in case of polyadenopathy. We relate a case of Kikuchi-Fujimoto disease in a 13-year-old boy with sickle-cell anemia...
January 2012: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
Caroline B Long, Irene Ramos, Deepa Rastogi, Deepa Manwani, Ginger Janow, Marcela Del Rio, Marguerite Mayers, Betsy C Herold, Ana Fernandez-Sesma, Rebecca Pellett Madan
OBJECTIVE: Humoral and cell-mediated immune responses to monovalent 2009 pandemic influenza A (H1N1/2009) and seasonal trivalent influenza (TIV) vaccines were evaluated in healthy children and children with asthma, sickle cell disease (SCD), systemic lupus erythematosus (SLE), and solid organ transplantation (SOT). STUDY DESIGN: Blood was collected from 112 subjects at the time of H1N1/2009 vaccination and 46 ± 15 days later for hemagglutination inhibition titers and γ-interferon ELISPOT responses to H1N1/2009 vaccine and TIV; unvaccinated children also received TIV at enrollment...
January 2012: Journal of Pediatrics
Gordan Grahovac, Milorad Vilendecic, Darko Chudy, Dubravka Srdoc, Jasenka Skrlin
STUDY DESIGN: Case report. OBJECTIVE: The nontraumatic cranial epidural hematoma is an extremely rare clinical entity and it commonly occurs in the presence of infectious diseases as complication of sinusitis, coagulopathy, vascular malformation of the dura, hemorrhagic tumor, in patients with sickle cell disease, systemic lupus erythematosus, after open heart surgery, and in patients on hemodialysis. SUMMARY OF BACKGROUND DATA: We reported a case of spontaneous epidural hematoma that occurred after herniated disc surgery...
December 15, 2011: Spine
W Drescher, T Pufe, R Smeets, R V Eisenhart-Rothe, M Jäger, M Tingart
Femoral head necrosis is an ischaemic bone necrosis of traumatic or nontraumatic pathogenesis which can lead to hip joint destruction in young age. It is today the indication for 10 % of all the total hip joint replacements. Known aetiologies of nontraumatic femoral head necrosis are alcoholism, steroids, sickle cell anaemia, caisson, and Gaucher's disease. Further risk factors are chemotherapy, chronic inflammatory bowel disease, systemic lupus erythematosus, and multiple sclerosis, in which also steroids are involved...
April 2011: Zeitschrift Für Orthopädie und Unfallchirurgie
Michael A Mont, Michael G Zywiel, David R Marker, Mike S McGrath, Ronald E Delanois
BACKGROUND: An asymptomatic hip with osteonecrosis is typically discovered as the contralateral hip of a patient with one symptomatic joint. Treatment of the asymptomatic hip is controversial. While some authors claim a benign natural history, others have reported a rate of femoral head collapse exceeding 50%. The purpose of this report was to systematically review the published literature regarding asymptomatic osteonecrosis of the femoral head to evaluate the overall prevalence of progression to symptomatic disease and/or femoral head collapse as well as to determine whether various radiographic and demographic factors influence progression of the disorder...
September 15, 2010: Journal of Bone and Joint Surgery. American Volume
O O Adelowo, S Oguntona
BACKGROUND: Avascular Necrosis (AVN) or osteonecrosis has been frequently reported among Nigerians with sickle cell disease. Other known aetiologies include connective tissue diseases, alcohol, fat embolism, juvenile arthritis and pregnancy. Connective tissue disease (CTD) are uncommonly reported among Nigerians. OBJECTIVE: To report the cases of three Nigerian female patients with radiological evidence of AVN associated with connective tissue diseases and inflammatory arthritis who presented to a rheumatology hospital in Lagos, Nigeria...
July 2009: West African Journal of Medicine
Henning R Johannson, Michael G Zywiel, David R Marker, Lynne C Jones, Mike S McGrath, Michael A Mont
The primary goals of this critical literature review were to determine whether revision rates of primary total hip arthroplasty in patients with osteonecrosis differ based on the underlying associated risk factors and diagnoses, whether the outcomes of this procedure have improved over the past two decades, and to compare outcomes based on study level of evidence. A systematic literature review yielded 67 reports representing 3,277 hips in 2,593 patients who had a total hip arthroplasty for osteonecrosis of the femoral head...
April 2011: International Orthopaedics
M Cherner, D Isenberg
Two cases of Afro-Caribbean women with an association of sickle cell disease (SCD) and systemic lupus erythematosus (SLE) are presented. We discuss the clues to the diagnosis of this combination with a review of the literature. Owing to a substantial overlap between the clinical manifestations of the two disorders, the diagnosis of SLE in patients with SCD may be difficult to establish and is often delayed.
June 2010: Lupus
Wasiu A Olowu, Kayode A Adelusola, Olufemi Adefehinti, Tajudeen G Oyetunji
BACKGROUND: The study determined (i) whether or not quartan malaria nephropathy (QMN) is still a major cause of childhood nephrotic syndrome (CNS) in Nigeria, (ii) secondary causes other than QMN and their associated glomerular pathology and (iii) renal and patient outcome. METHODS: The study was a prospective non-randomized study of consecutive cases of secondary CNS. Patients with idiopathic CNS were excluded. RESULTS: Twenty-four of 78 (30...
March 2010: Nephrology, Dialysis, Transplantation
Zakiya Al-Mosawi, Badriya E Al-Hermi, Khulood K Al-Saad, Eman M Farid, Hussain A Makki
OBJECTIVE: To analyze the clinical and serological features of children with systemic lupus erythematosus (SLE) in a major referral center in Bahrain and to assess the comorbidity, its morbidity, and mortality. METHODS: We retrospectively reviewed the medical charts of children with SLE treated in the Pediatric Rheumatology Clinic at Salmaniya Medical Complex, Kingdom of Bahrain from 1998 to 2007. The ethical approval for the study was obtained from the Research Health Committee, Ministry of Health, Kingdom of Bahrain...
May 2009: Saudi Medical Journal
Pierre Cochat, Sonia Fargue, Guillaume Mestrallet, Therese Jungraithmayr, Paulo Koch-Nogueira, Bruno Ranchin, Lothar Bernd Zimmerhackl
Renal transplantation (Tx) is the treatment of choice for end-stage renal disease. The incidence of acute rejection after renal Tx has decreased because of improving early immunosuppression, but the risk of disease recurrence (DR) is becoming relatively high, with a greater prevalence in children than in adults, thereby increasing patient morbidity, graft loss (GL) and, sometimes, mortality rate. The current overall graft loss to DR is 7-8%, mainly due to primary glomerulonephritis (70-80%) and inherited metabolic diseases...
November 2009: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
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