scholarly journals Coexistence of Relapsing Polychondritis and Sickle Cell Disease in a Child

2021 ◽  
Vol 2021 ◽  
pp. 1-7
Author(s):  
Bernard Ofoe Tetteh ◽  
Florence-Barbara Yebuah ◽  
Maame-Boatemaa Amissah-Arthur ◽  
Dzifa Dey
Keyword(s):  
Sickle Cell Disease ◽  
Clinical Features ◽  
Sickle Cell ◽  
Clinical Symptoms ◽  
Relapsing Polychondritis ◽  
Complex Case ◽  
Unknown Etiology ◽  
Cell Disease ◽  
Systemic Autoimmune Rheumatic Disease ◽  
Anti Inflammatory

Relapsing polychondritis (RP) is a rare, severe connective tissue disease of unknown etiology affecting cartilaginous and proteoglycan-rich structures in an episodic and inflammatory manner. Approximately a third of RP cases occur in conjunction with another disease usually systemic autoimmune rheumatic disease, or myelodysplastic syndrome. Sickle cell disease (SCD) is a common inherited hematologic condition characterized by the inheritance of two abnormal hemoglobins, of which one is a hemoglobin S, presenting with severe acute and chronic complications from vaso-occlusive phenomena, which can be difficult to differentiate from RP. The pathogenesis of RP is poorly understood but suggests an autoimmune mechanism with a link to sickle cell disease yet to be established. Treatment is empiric with steroids, anti-inflammatory, and disease-modifying antirheumatic drugs being the mainstay of therapy. Severe complications occur despite treatment, with respiratory involvement being the most catastrophic. This case report reviews a complex case of RP in an 11-year-old girl with sickle cell disease (SF genotype) presenting with bilateral red painful eyes, a painful swollen left ear, and knee pain. Laboratory findings revealed elevated inflammatory markers with negative immune serology. A diagnosis of RP was made based on the patient's symptomatology, presentation, and fulfillment of 5 out of the 6 clinical features using McAdam’s criteria. Management was instituted with a myriad of conventional and biologic DMARDs and other anti-inflammatory medications with no significant improvement and the development of complications of airway obstruction from disease activity and osteoporotic fracture from steroid therapy and underlying hemoglobinopathy. In children, the diagnosis of RP is delayed or overlooked due to its low incidence, variability in clinical symptoms, or sharing similar clinical features with other coexisting disease entities. This article reports its occurrence in the pediatric population and highlights the difficulty in managing such cases as there are no defined standard treatment protocols.

scholarly journals Use of anti-inflammatory analgesics in sickle-cell disease

2017 ◽  
Vol 42 (5) ◽  
pp. 656-660 ◽  
Author(s):  
J. Han ◽  
S. L. Saraf ◽  
J. P. Lash ◽  
V. R. Gordeuk
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Anti Inflammatory

The painful crisis of homozygous sickle cell disease: clinical features

1994 ◽  
Vol 87 (3) ◽  
pp. 586-591 ◽  
Author(s):  
Graham R. Serjeant ◽  
Charles D. E. Ceulaer ◽  
Rosemary Lethbridge ◽  
Joanne Morris ◽  
Atul Singhal and ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Clinical Features ◽  
Sickle Cell ◽  
Cell Disease ◽  
Homozygous Sickle Cell Disease ◽  
Painful Crisis

Nitrated Lipids in a Murine Model of Sickle Cell Disease at Baseline.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 3758-3758
Author(s):  
Jeffrey Schwartz ◽  
Paul R.S. Baker ◽  
Francisco J. Schopfer ◽  
Bruce A. Freeman
Keyword(s):  
Linoleic Acid ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Ion Trap ◽  
Internal Standard ◽  
Wild Type ◽  
Cell Disease ◽  
Inflammatory Signaling ◽  
No Bioavailability ◽  
Anti Inflammatory

Abstract Introduction Sickle cell disease (SCD) is increasingly recognized as a disorder of inflammatory homeostasis. One major focus in recent years has been understanding the role of Nitric Oxide (NO) in the pathophysiology of SCD. NO is a critical mediator of inflammatory pathways and current evidence supports the precept that NO bioavailability is decreased in SCD, resulting in normal concentrations of NO at baseline but an inability to increase NO during stress. Nitrated fatty acids, such as Nitrolinoleate (LNO2), have recently been reported as potent and abundant anti-inflammatory signaling mediators with the ability to cause vasorelaxation and inhibition of platelet and neutrophil activation. Evidence supports their anti-inflammatory signaling is mediated through the release of NO and NO-related products. LNO2 has not previously been described in patients with SCD and our objective was to quantify LNO2 in a murine model of SCD at baseline. Methods Whole blood was obtained from transgenic sickle cell and wild type mice (n = 5 and 6, respectively). Blood was centrifuged and separated into plasma and packed red blood cells (RBCs). These biological samples were prepared for lipid analysis by the method of Bligh and Dyer; care was taken so that the pH of the extraction milieu was consistently maintained at 7 so as to avoid artifactual nitration. Samples were analyzed for free LNO2 content by electrospray ionization tandem mass spectrometry. Using a hybrid triple quadrupole ion trap mass spectrometer, MRM transitions were monitored that specifically identified nitrated linoleic acid species; these species were concomitantly confirmed by the qualitative analytical abilities of the ion trap. The presence of nitrated linoleic acid was confirmed by HPLC chromatographic retention times, MS/MS “fingerprints” and was quantitated by the inclusion of a known quantity of 13C-labeled LNO2. Results LNO2 concentration was calculated as a function of the ratio of analyte to internal standard peak areas by using an internal standard curve linear over five orders of magnitude. Free LNO2 in the RBCs and plasma of 5 transgenic sickle cell mice were 3.97 ± 2.56 nM and 12.37 ± 9.83 nM, respectively. Free LNO2 in the RBCs and plasma of 6 wild type mice were 9.49 ± 8.32 nM and 14.91 ± 10.08 nM, respectively. There were no significant differences in LNO2 concentration between any of the groups. Conclusions LNO2 is present in both transgenic sickle cell mice and wild type mice in comparable concentrations at baseline. As a mediator of NO anti-inflammatory signaling, this is consistent with human studies showing comparable concentrations of NO metabolites at baseline between sickle cell patients and healthy controls. Further study of LNO2 in sickle cell disease is warranted to better understand its role in the inflammatory process associated with acute stress, such as vaso-occlusive pain crisis and acute chest syndrome, when NO bioavailability is decreased.

Hypertensive Encephalopathy in Children with Sickle Cell Disease.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 3790-3790
Author(s):  
Caterina P. Minniti ◽  
Steven L. Weinstein ◽  
Zarir Khademian
Keyword(s):  
Magnetic Resonance ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Complete Resolution ◽  
Clinical Symptoms ◽  
Hypertensive Encephalopathy ◽  
Cell Disease ◽  
Acute Infarction ◽  
Cortical Edema

Abstract Cerebrovascular (CVA) accidents are a serious complication of Sickle Cell Disease (SCD). Acute cerebral infarction occurs in approximately 10% of children with SCD under the age of 16 years, with silent infarcts identified in an additional 22%. Children however can present with acute neurologic deterioration mimicking a CVA but demonstrate a picture consistent with hypertensive encephalopathy (HTNE). The incidence of HTNE in children with SCD is unknown with few annedoctal reports available in the literature. We retrospectively identified 83 children with SCD, who received their care at Children’s National Medical Center in Washington, DC, from 1992 to 2005, who presented with neurologic complaints that prompted Magnetic Resonance Imaging (MRI). There were 37 females and 46 males, age 13 months to 17 years, with mean age of 5 years and 8 months. Clinical and neuro-imaging data identified 8 children (7 females and 1 male) with clinical picture compatible with HTNE (BP > 2 std deviation for age), prior to neuroimaging. At the time of the hypertensive episode, the ages ranged from 6 to 16 years and 8 months, with an average of 14 years and 2 months. All patients had Hb SS. Neurologic complaints included seizure, sudden onset headache, confusion, loss of consciousness, and urinary retention. Elevated blood pressures were aggressively treated and all patient received an exchange transfusion. No specific etiology for the hypertension was determined. Initial MRI, obtained within 24–48 hours of presentation, did not show acute or prior infarction in this group. However, there was absence of diffusion restriction on T1 and T2 weighted images, and presence of cerebral cortical edema, which differentiate HTNE from acute infarction. The magnetic resonance angiogram (MRA) did not demonstrate evidence of arteriopathy. The hallmark of HTNE is a complete resolution of abnormalities of both the neurologic exam and the imaging studies at follow up. There was complete resolution of cerebral edema in all patients and mild interval prominence of the cerebral sulci, which can indicate cerebral volume loss, in three out of the 8 patients, in follow up studies obtained 10 days to 4 months later. No recurrences of the symptoms have been reported, with follow up ranging from 5 months to 8 years. None of the patients with HTNE has received chronic transfusions. We conclude that HTNE should be considered in the differential diagnosis of an acute neurologic event in a child with SCD. Accurate recording of vital signs and prompt correction of hypertension is indicated. MRI can accurately distinguish between HTNE and acute infarction, even when clinical symptoms are similar. This distinction helps physicians to establish proper treatment such as chronic transfusion following infarction.

Clinical Features of Sickle Cell Disease in Eastern Saudi Arab Children

1990 ◽  
Vol 12 (1) ◽  
pp. 51-55 ◽  
Author(s):  
Mohamed I. El Mouzan ◽  
Baker H. Al Awamy ◽  
Mohammed T. Al Torki
Keyword(s):  
Sickle Cell Disease ◽  
Clinical Features ◽  
Sickle Cell ◽  
Cell Disease ◽  
Arab Children

Pneumonia in young children with homozygous sickle cell disease: risk and clinical features

1985 ◽  
Vol 144 (3) ◽  
pp. 255-258 ◽  
Author(s):  
K. De Ceulaer ◽  
K. W. McMullen ◽  
G. H. Maude ◽  
R. Keatinge ◽  
G. R. Serjeant
Keyword(s):  
Sickle Cell Disease ◽  
Young Children ◽  
Clinical Features ◽  
Sickle Cell ◽  
Disease Risk ◽  
Cell Disease ◽  
Homozygous Sickle Cell Disease

scholarly journals Mortality of covid19 in sickle cell disease systematic review

2021 ◽  
Vol 12 (2) ◽  
pp. 151-156
Author(s):  
Waseem Bader Al Talalwah ◽  
Shorok Ali Al Dorazi
Keyword(s):  
Sickle Cell Disease ◽  
Clinical Features ◽  
Sickle Cell ◽  
Pulmonary Arteries ◽  
Beta Thalassemia ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Fourth Decade ◽  
Radiological Changes

The covid19 infection is pandemic disease are more commonly in chronic diseases. As covid19 case pulmonary infection, the current study focuses on sickle cell disease patients are suitable to acute chest syndrome. It will clarify the risk factors for covid19 infection and its clinical features in sickle cell disease. It also reviews the radiological findings to gain more data on this pandemic disease. The current study includes 44 cases of sickle cell disease having covid19 infection. The entire cases include sickle cell disease only in 89% whereas the other sickle cell disease cases are coexisted with beta- thalassemia in 9.1%. The fourth decade is the highest peak incidence. There are several complications found to be in sickle cell disease are acute chest syndrome, chronic leg ulcer, renal failure exceeding 10%. The bronchial asthma found to be in 6.9% and avascular necrosis found to be 11.4% whereas the cerebrovascular accident found to be in 13.8%. The radiological changes include lung tissues, alveoli, pleural cavities and pulmonary arteries in different rate. The incidence of recovery found to be in 93% whereas the death found to be in 7%. It found to be involving in respiratory and gastrointestinal systems result in different clinical features in different rate. This study compares the clinical features, findings investigation and complications between sex and decades. Further, this study clarifies recovery and mortality rate between sex and decades. Knowing and understanding covid19 infection in sickle cell disease, physicians will be able to provide high quality of medical services.

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