SCD
MCID: SCK005
MIFTS: 53

Sickle Cell Disease (SCD)

Categories: Blood diseases, Immune diseases, Rare diseases

Aliases & Classifications for Sickle Cell Disease

MalaCards integrated aliases for Sickle Cell Disease:

Name: Sickle Cell Disease 24 25 55 43 3
Sickling Disorder Due to Hemoglobin S 25
Sickle Cell Disorders 25
Hemoglobin S Disease 25
Anemia, Sickle Cell 72
Hbs Disease 25
Scd 25

Classifications:



External Ids:

ICD10 33 D57
UMLS 72 C0002895

Summaries for Sickle Cell Disease

MedlinePlus : 43 What is sickle cell disease? Sickle cell disease (SCD) is a group of inherited red blood cell disorders. If you have SCD, there is a problem with your hemoglobin. Hemoglobin is a protein in red blood cells that carries oxygen throughout the body. With SCD, the hemoglobin forms into stiff rods within the red blood cells. This changes the shape of the red blood cells. The cells are supposed to be disc-shaped, but this changes them into a crescent, or sickle, shape. The sickle-shaped cells are not flexible and cannot change shape easily. Many of them burst apart as they move through your blood vessels. The sickle cells usually only last 10 to 20 days, instead of the normal 90 to 120 days. Your body may have trouble making enough new cells to replace the ones that you lost. Because of this, you may not have enough red blood cells. This is a condition called anemia, and it can make you feel tired. The sickle-shaped cells can also stick to vessel walls, causing a blockage that slows or stops the flow of blood. When this happens, oxygen can't reach nearby tissues. The lack of oxygen can cause attacks of sudden, severe pain, called pain crises. These attacks can occur without warning. If you get one, you might need to go to the hospital for treatment. What causes sickle cell disease? The cause of SCD is a defective gene, called a sickle cell gene. People with the disease are born with two sickle cell genes, one from each parent. If you are born with one sickle cell gene, it's called sickle cell trait. People with sickle cell trait are generally healthy, but they can pass the defective gene on to their children. Who is at risk for sickle cell disease? In the United States, most of the people with SCD are African Americans: About 1 in 13 African American babies is born with sickle cell trait About 1 in every 365 black children is born with sickle cell disease SCD also affects some people who come from Hispanic, southern European, Middle Eastern, or Asian Indian backgrounds. What are the symptoms of sickle cell disease? People with SCD start to have signs of the disease during the first year of life, usually around 5 months of age. Early symptoms of SCD may include Painful swelling of the hands and feet Fatigue or fussiness from anemia A yellowish color of the skin (jaundice) or the whites of the eyes (icterus) The effects of SCD vary from person to person and can change over time. Most of the signs and symptoms of SCD are related to complications of the disease. They may include severe pain, anemia, organ damage, and infections. How is sickle cell disease diagnosed? A blood test can show if you have SCD or sickle cell trait. All states now test newborns as part of their screening programs, so treatment can begin early. People who are thinking about having children can have the test to find out how likely it is that their children will have SCD. Doctors can also diagnose SCD before a baby is born. That test uses a sample of amniotic fluid (the liquid in the sac surrounding the baby) or tissue taken from the placenta (the organ that brings oxygen and nutrients to the baby). What are the treatments for sickle cell disease? The only cure for SCD is bone marrow or stem cell transplantation. Because these transplants are risky and can have serious side effects, they are usually only used in children with severe SCD. For the transplant to work, the bone marrow must be a close match. Usually, the best donor is a brother or sister. There are treatments that can help relieve symptoms, lessen complications, and prolong life: Antibiotics to try to prevent infections in younger children Pain relievers for acute or chronic pain Hydroxyurea, a medicine that has been shown to reduce or prevent several SCD complications. It increases the amount of fetal hemoglobin in the blood. This medicine is not right for everyone; talk to your health care provider about whether you should take it. This medicine is not safe during pregnancy. Childhood immunizations to prevent infections Blood transfusions for severe anemia. If you have had some serious complications, such as a stroke, you may have transfusions to prevent more complications. There are other treatments for specific complications. To stay as healthy as possible, make sure that you get regular medical care, live a healthy lifestyle, and avoid situations that may set off a pain crisis. NIH: National Heart, Lung, and Blood Institute

MalaCards based summary : Sickle Cell Disease, also known as sickling disorder due to hemoglobin s, is related to hereditary persistence of fetal hemoglobin-sickle cell disease syndrome and acute chest syndrome, and has symptoms including angina pectoris, abdominal pain and edema. An important gene associated with Sickle Cell Disease is MIR144 (MicroRNA 144), and among its related pathways/superpathways are Factors involved in megakaryocyte development and platelet production and Erythrocytes take up carbon dioxide and release oxygen. The drugs Peginterferon alfa-2a and Ribavirin have been mentioned in the context of this disorder. Affiliated tissues include bone, bone marrow and endothelial, and related phenotypes are Increased shRNA abundance and cardiovascular system

Genetics Home Reference : 25 Sickle cell disease is a group of disorders that affects hemoglobin, the molecule in red blood cells that delivers oxygen to cells throughout the body. People with this disorder have atypical hemoglobin molecules called hemoglobin S, which can distort red blood cells into a sickle, or crescent, shape. Signs and symptoms of sickle cell disease usually begin in early childhood. Characteristic features of this disorder include a low number of red blood cells (anemia), repeated infections, and periodic episodes of pain. The severity of symptoms varies from person to person. Some people have mild symptoms, while others are frequently hospitalized for more serious complications. The signs and symptoms of sickle cell disease are caused by the sickling of red blood cells. When red blood cells sickle, they break down prematurely, which can lead to anemia. Anemia can cause shortness of breath, fatigue, and delayed growth and development in children. The rapid breakdown of red blood cells may also cause yellowing of the eyes and skin, which are signs of jaundice. Painful episodes can occur when sickled red blood cells, which are stiff and inflexible, get stuck in small blood vessels. These episodes deprive tissues and organs of oxygen-rich blood and can lead to organ damage, especially in the lungs, kidneys, spleen, and brain. A particularly serious complication of sickle cell disease is high blood pressure in the blood vessels that supply the lungs (pulmonary hypertension). Pulmonary hypertension occurs in about one-third of adults with sickle cell disease and can lead to heart failure.

CDC : 3 SCD is a group of inherited red blood cell disorders. Healthy red blood cells are round, and they move through small blood vessels to carry oxygen to all parts of the body. In someone who has SCD, the red blood cells become hard and sticky and look like a C-shaped farm tool called a "sickle". The sickle cells die early, which causes a constant shortage of red blood cells. Also, when they travel through small blood vessels, they get stuck and clog the blood flow. This can cause pain and other serious problems such infection, acute chest syndrome and stroke.

Wikipedia : 75 Sickle cell disease (SCD) is a group of blood disorders typically inherited from a person's parents. The... more...

GeneReviews: NBK1377

Related Diseases for Sickle Cell Disease

Diseases in the Sickle Cell Disease family:

Sickle Cell Disease and Related Diseases

Diseases related to Sickle Cell Disease via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 849)
# Related Disease Score Top Affiliating Genes
1 hereditary persistence of fetal hemoglobin-sickle cell disease syndrome 33.7 HBG2 HBG1 HBB BCL11A
2 acute chest syndrome 33.2 VCAM1 HBB
3 splenic sequestration 32.2 HBB BCAM
4 hemoglobinopathy 31.8 HBG1 HBD HBB HBA2 EPO
5 sickle cell anemia 31.4 VCAM1 HBS1L HBG2 HBG1 HBB HBA2
6 hemoglobin c disease 31.2 HBD HBB
7 splenomegaly 31.2 HBA2 EPO
8 glucosephosphate dehydrogenase deficiency 30.7 HBG2 HBB G6PD
9 hereditary spherocytosis 30.7 HBG1 HBB G6PD
10 malaria 30.7 VCAM1 SELP HBB HBA2 G6PD EPO
11 erythroleukemia, familial 30.7 HBG1 HBB EPO
12 hemolytic anemia 30.5 HBG2 HBB HBA2 G6PD EPO
13 retinal vascular occlusion 30.1 SELP PGF
14 hemoglobin d disease 30.0 HBD HBB
15 deficiency anemia 30.0 HBG2 HBB HBA2 G6PD EPO
16 hemoglobin e disease 30.0 HBS1L HBD HBB
17 arteries, anomalies of 29.8 VCAM1 SELP PGF
18 thalassemia 29.6 MIR144 HBS1L HBG1 HBD HBB HBA2
19 alpha-thalassemia 29.4 VCAM1 HBG2 HBG1 HBB HBA2 G6PD
20 fetal hemoglobin quantitative trait locus 1 29.3 HBG2 HBG1 HBD HBB
21 congenital hemolytic anemia 28.8 HBG1 HBD HBB HBA2 G6PD EPO
22 beta-thalassemia 28.7 HBS1L HBG2 HBG1 HBD HBB HBA2
23 sickle cell disease associated with an other hemoglobin anomaly 12.4
24 sickle cell disease and related diseases 12.3
25 schnyder corneal dystrophy 12.1
26 ehlers-danlos syndrome, spondylodysplastic type, 3 11.8
27 sickle beta thalassemia 11.7
28 moyamoya disease 1 11.7
29 endosteal hyperostosis, autosomal dominant 11.5
30 fetal hemoglobin quantitative trait locus 5 11.5
31 qt interval, variation in 11.5
32 abdominal obesity-metabolic syndrome 1 11.4
33 diabetes mellitus, noninsulin-dependent 11.3
34 fanconi anemia, complementation group d2 11.3
35 body mass index quantitative trait locus 11 11.3
36 lipodystrophy, congenital generalized, type 3 11.3
37 lipodystrophy, congenital generalized, type 4 11.3
38 reye syndrome 11.3
39 carnitine deficiency, systemic primary 11.3
40 spondylocostal dysostosis 3, autosomal recessive 11.2
41 osteomesopyknosis 11.2
42 sickle cell - hemoglobin d disease 11.2
43 fanconi anemia, complementation group a 11.2
44 superior semicircular canal dehiscence 11.2
45 subacute cerebellar degeneration 11.2
46 semicircular canal dehiscence syndrome 11.2
47 obsolete: secondary ciliary dyskinesia 11.2
48 pulmonary hypertension 10.9
49 hemosiderosis 10.9
50 rare hereditary hemochromatosis 10.9

Graphical network of the top 20 diseases related to Sickle Cell Disease:



Diseases related to Sickle Cell Disease

Symptoms & Phenotypes for Sickle Cell Disease

UMLS symptoms related to Sickle Cell Disease:


angina pectoris, abdominal pain, edema, chest pain

GenomeRNAi Phenotypes related to Sickle Cell Disease according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased shRNA abundance GR00327-A 8.92 EPO G6PD HBD HBG2

MGI Mouse Phenotypes related to Sickle Cell Disease:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.23 BCAM EPO G6PD KCNN4 PGF SELP

Drugs & Therapeutics for Sickle Cell Disease

Drugs for Sickle Cell Disease (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 386)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Peginterferon alfa-2a Approved, Investigational Phase 4 198153-51-4 5360545
2
Ribavirin Approved Phase 4 36791-04-5 37542
3
Peginterferon alfa-2b Approved Phase 4 99210-65-8, 215647-85-1
4
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 4 437-38-7 3345
5
Riociguat Approved Phase 4 625115-55-1
6
Atorvastatin Approved Phase 4 134523-00-5 60823
7
Proguanil Approved Phase 4 500-92-5 4923
8
Ketamine Approved, Vet_approved Phase 4 6740-88-1 3821
9
Acetaminophen Approved Phase 4 103-90-2 1983
10
Amodiaquine Approved, Investigational Phase 4 86-42-0 2165
11
Sulfadoxine Approved, Investigational Phase 4 2447-57-6 17134
12
Pyrimethamine Approved, Investigational, Vet_approved Phase 4 58-14-0 4993
13
Tocopherol Approved, Investigational Phase 4 1406-66-2, 54-28-4 14986
14
Desogestrel Approved Phase 4 54024-22-5 40973
15
Etonogestrel Approved, Investigational Phase 4 54048-10-1 6917715 40976
16
Deferiprone Approved Phase 4 30652-11-0 2972
17
Ketorolac Approved Phase 4 74103-06-3, 66635-83-4 3826
18
Vitamin E Approved, Nutraceutical, Vet_approved Phase 4 59-02-9 14985
19
Calcium Approved, Nutraceutical Phase 4 7440-70-2 271
20
Vitamin D3 Approved, Nutraceutical Phase 4 67-97-0 6221 5280795
21
Ergocalciferol Approved, Nutraceutical Phase 4 50-14-6 5280793
22
Vitamin D Approved, Nutraceutical, Vet_approved Phase 4 1406-16-2
23
Calcifediol Approved, Nutraceutical Phase 4 19356-17-3 6433735 5283731
24
Dihydroartemisinin Experimental, Investigational Phase 4 71939-50-9 6918483
25
Piperaquine Experimental, Investigational Phase 4 4085-31-8 5079497
26 Tocotrienol Investigational Phase 4 6829-55-6
27 Interferon alpha-2 Phase 4
28 Interferon-alpha Phase 4
29 interferons Phase 4
30 Anti-Inflammatory Agents Phase 4
31 Lipid Regulating Agents Phase 4
32 Hypolipidemic Agents Phase 4
33 Hydroxymethylglutaryl-CoA Reductase Inhibitors Phase 4
34 Anticholesteremic Agents Phase 4
35 Performance-Enhancing Substances Phase 4
36 Anti-Bacterial Agents Phase 4
37 Anesthetics Phase 4
38 Anesthetics, General Phase 4
39 Anesthetics, Intravenous Phase 4
40 Antiparasitic Agents Phase 4
41 Antimalarials Phase 4
42 Antiprotozoal Agents Phase 4
43 Antimetabolites Phase 4
44 Anesthetics, Dissociative Phase 4
45 Antipyretics Phase 4
46 Folic Acid Antagonists Phase 4
47 Renal Agents Phase 4
48 Anti-Infective Agents, Urinary Phase 4
49 Artemisinins Phase 4
50 Artemisinine Phase 4

Interventional clinical trials:

(show top 50) (show all 765)
# Name Status NCT ID Phase Drugs
1 Effect of Mobile-Directly Observed Therapy (DOT) on Adherence to Hydroxyurea Treatment in Adult HbSS Patients at Muhimbili National Hospital (MNH) in Tanzania: a Pilot Study Unknown status NCT02844673 Phase 4 Hydroxyurea
2 Phase IV Study of Effectiveness of Interferon and Ribavirin Treatment in Thalassemia Major Patients With Chronic Viral Hepatitis C Unknown status NCT00887081 Phase 4 PEG-IFN alpha2a or PEG-IFN alpha2b and Ribavirin
3 Evaluation of the Impact of Renal Function on the Pharmacokinetics of Hydroxyurea (SIKLOS ®) in Normal-renal Function, Hyperfiltrating and Renal Failure Sickle Cell Disease Patients (DARH) Completed NCT02522104 Phase 4 Siklos
4 Intranasal Fentanyl for Initial Treatment of a Vaso-occlusive Crisis: A Randomized, Double Blind Placebo Controlled Trial Completed NCT01482091 Phase 4 Fentanyl Citrate;Normal Saline
5 Rejuvesol® Washed RBC in Sickle Cell Patients Requiring Frequent Transfusions Completed NCT02731157 Phase 4 Rejuvesol
6 Future of Spermatogenesis in Men With Sickle Cell Disease Medically Treated. Completed NCT01609192 Phase 4 Hydrea® (hydroxyurea )
7 Comparing Acute Pain Management Protocols for Patients With Sickle Cell Disease Completed NCT02222246 Phase 4 Hydromorphone (Standardized, weight-based dosing);Morphine Sulfate (Standardized, weight-based dosing);Hydromorphone (Patient Specific dosing);Morphine Sulfate (Patient Specific dosing)
8 Intranasal Fentanyl Versus Intravenous Morphine in the Emergency Department Treatment of Severe Painful Sickle Cell Crises in Children Completed NCT03682211 Phase 4 Fentanyl Citrate;Morphine sulphate
9 Pilot Pharmacokinetic Study In Patients With Inadequate Response To Deferasirox (Exjade) Completed NCT00749515 Phase 4 Deferoxamine;Deferasirox
10 Low-dose Ketamine Versus Morphine for Severe Painful Sickle Cell Crises in Children at Mulago Hospital: A Randomised Controlled Trial Completed NCT02434939 Phase 4 Low dose ketamine;Morphine
11 A Randomised Controlled Trial of Atorvastatin as an Anti-Inflammatory Agent in Non-Cystic Fibrosis Bronchiectasis Completed NCT01299181 Phase 4 Atorvastatin
12 A Randomised Controlled Trial of Atorvastatin as an Anti-Inflammatory Agent in Non-Cystic Fibrosis Bronchiectasis in Patients With Pseudomonas Aeruginosa Completed NCT01299194 Phase 4 ATORVASTATIN
13 The Effect of Riociguat on Gas Exchange, Exercise Performance, and Pulmonary Artery Pressure During Acute Altitude Exposure Completed NCT02024386 Phase 4 Riociguat
14 Does IV Acetaminophen Reduce Opioid Requirement in Pediatric Emergency Department Patients With Acute Sickle Cell Crises? Recruiting NCT03541980 Phase 4 Acetaminophen;Normal saline
15 The Efficacy and Safety of Ferriprox® for the Treatment of Transfusional Iron Overload in Patients With Sickle Cell Disease or Other Anemias Recruiting NCT02041299 Phase 4 Deferiprone;Deferoxamine
16 Enhancing Preventive Therapy of Malaria In Children With Sickle Cell Anemia in East Africa (EPiTOMISE) Recruiting NCT03178643 Phase 4 Proguanil Oral Tablet;Sulfadoxine/Pyrimethanine-Amodiaquine (SP-AQ);Dihydroartemisinin-Piperaquine (DP)
17 Comparison of Nebulized Sub-dissociative Dose Ketamine at Three Different Dosing Regimens for Treating Acute Pain in the Pediatric ED: A Prospective, Randomized Double-Blind Trial Recruiting NCT03950817 Phase 4 Nebulized Sub-dissociative Dose Ketamine at either 0.75 mg/kg;Nebulized Sub-dissociative Dose Ketamine at 1 mg/kg;Nebulized Sub-dissociative Dose Ketamine at 1.5 mg/kg.
18 Risk Stratification for Clinical Severity of Sickle Cell Disease in Nigeria and Assessment of Efficacy and Safety During Treatment With Hydroxyurea Active, not recruiting NCT02149537 Phase 4 hydroxyurea
19 Endothelial Monocyte-activating Polypeptide-II as an Endothelial Dysfunction Marker and Its Relation to the Oxidative Stress in Egyptian Sickle Patients Active, not recruiting NCT03903133 Phase 4 Vitamin E
20 Use of Etonogestrel-releasing Contraceptive Implant in Women With Sickle Cell Disease Active, not recruiting NCT02594462 Phase 4 etonogestrel-releasing implant contraceptive
21 Long-term Safety and Efficacy Study of Ferriprox® for the Treatment of Transfusional Iron Overload in Patients With Sickle Cell Disease or Other Anemias Enrolling by invitation NCT02443545 Phase 4 Deferiprone
22 A Randomized Phase IV Control Trial of Single High Dose Oral Vitamin D3 (Stoss Therapy) in Pediatric Patients Undergoing HSCT to Prevent Vitamin D Deficiency and Insufficiency During Transplant Enrolling by invitation NCT03176849 Phase 4
23 Early Low-dose Ketamine Infusion Versus Usual Care for Sickle Cell Pain Crisis: a Randomized, Prospective Study. Not yet recruiting NCT04005209 Phase 4 Ketamine
24 An Open, Multicenter Clinical Trial to Investigate the Immunogenicity and Safety of the Pneumococcal 7-Valent Conjugate Vaccine (PREVENAR) in Sickle Cell Disease Infants. Terminated NCT00368186 Phase 4
25 A Pilot Study on the Effects of Intravenous Ketamine on Acute Pain Crisis in Patients With Sickle Cell Disease Terminated NCT00252122 Phase 4 Ketamine
26 An Evaluation of the Effectiveness of Ibuprofen and Opioid (Morphine or Diamorphine) for Acute Pain in Sickle Cell Disease: a Double-blind, Placebo-controlled Randomised Trial Terminated NCT00880373 Phase 4 Ibuprofen;Placebo;Diamorphine or Morphine
27 Ketorolac Versus Ibuprofen for the Painful Crisis of Sickle Cell Disease - Southwestern Comprehensive Sickle Cell Center Terminated NCT00115336 Phase 4 Intravenous Ketorolac;Ibuprofen
28 Non-Invasive Assessment of Opioid Analgesia in Children With Sickle Cell Disease Withdrawn NCT00513864 Phase 4 Dextromethorphan;Codeine;Morphine
29 Endothelial Function in Patients With Sickle Cell Anemia Before and After Sildenafil Withdrawn NCT00937144 Phase 4 Viagra (Sildenafil);placebo
30 A Randomized, Controlled Trial of Autologous Platelet Gel Treatment in Diabetic Foot Ulcers Withdrawn NCT00338702 Phase 4
31 Transfusion Alternatives Pre-Operatively in Sickle Cell Disease Unknown status NCT00512577 Phase 3
32 Omega 3 Fatty Acid Therapy for Prevention of Vaso-occlusive Crisis and Manifestations in Omani Patients With Sickle Cell Disease Unknown status NCT02525107 Phase 3
33 Ketamine as an Adjuvant Therapy for Acute Vaso Occlusive Crisis in Pediatric Patients With Sickle Cell Disease, a Pilot Study Unknown status NCT02801292 Phase 3 Ketamine
34 Development of a Ready-to-use Nutraceutical Food for Patients With Sickle Cell Disease (SCD): Testing of Vascular Support Components Unknown status NCT01718054 Phase 2, Phase 3 Chloroquine
35 A Prospective Randomized Comparative Study of Efficacy and Safety of Combined Deferiprone (DFP) and Deferasirox Versus DFP and Desferrioxamine (DFO) Therapy in Diseases With Severe Iron Overload Unknown status NCT01511848 Phase 2, Phase 3 DFP (ferriprox) and deferasirox (ICL 670);DFP, DFO
36 A PHASE 3, MULTICENTER, RANDOMIZED, DOUBLE-BLIND, PLACEBO-CONTROLLED, PARALLEL-GROUP STUDY TO EVALUATE THE EFFICACY AND SAFETY OF RIVIPANSEL (GMI-1070) IN THE TREATMENT OF VASO-OCCLUSIVE CRISIS IN HOSPITALIZED SUBJECTS WITH SICKLE CELL DISEASE Completed NCT02187003 Phase 3 Rivipansel
37 MAST - Magnesium for Sickle Cell Acute Crisis in Children Completed NCT00313963 Phase 3 Magnesium Sulfate;Normal Saline
38 Evaluation of Repeat Administration of Purified Poloxamer 188 in Vaso-Occlusive Crisis of Sickle Cell Disease (EPIC-E): An Open-Label Safety Extension Trial Assessing Repeat Administration of MST-188 (Purified Poloxamer 188) Injection in Subjects With Sickle Cell Disease Experiencing Vaso Occlusive Crisis Completed NCT02449616 Phase 3 MST-188
39 Evaluation of Purified Poloxamer 188 in Vaso-Occlusive Crisis of Sickle Cell Disease (EPIC): A Phase 3 Randomized, Double-Blind, Placebo-Controlled, Multicenter Clinical Trial of MST-188 (Purified Poloxamer 188) Injection in Subjects With Sickle Cell Disease Experiencing Vaso Occlusive Crisis Completed NCT01737814 Phase 3 Saline;MST-188
40 Phase III Randomized Study of Poloxamer 188 for Vaso-Occlusive Crisis of Sickle Cell Disease Completed NCT00004408 Phase 3 poloxamer 188
41 A Phase 3, Open-Label, Single-Arm Trial Evaluating the Safety, Tolerability, and Immunogenicity of 13-valent Pneumococcal Conjugate Vaccine in Children With Sickle Cell Disease Previously Immunized With 23vPS Vaccine Completed NCT00918580 Phase 3
42 A Randomized, Controlled, Double-blind Clinical Trial of L-arginine as Adjuvant Therapy for Sickle Cell Disease Completed NCT01142219 Phase 3 L-arginine;Placebo
43 N-Acetylcysteine in Patients With Sickle Cell Disease - Reducing the Incidence of Daily Life Pain Completed NCT01849016 Phase 3 N-Acetylcysteine;Placebo
44 A Study to Provide Expanded Access of (Exjade®) Deferasirox to Patients With Congenital Disorders of Red Blood Cells and Chronic Iron Overload From Blood Transfusions Who Cannot Adequately be Treated With Other Locally Approved Iron Chelators Completed NCT00235391 Phase 3 Deferasirox
45 Bicentric Study of the Effect of Inhaled Nitric Oxide Compared to Placebo in Acute Chest Syndrome of Adult Sickle Cell Patients Completed NCT00748423 Phase 2, Phase 3 Nitric Oxide;Placebo
46 Ameliorating Attention Problems in Children With SCD Completed NCT01411280 Phase 3 methylphenidate
47 Intravenous Magnesium for Sickle Cell Vasoocclusive Crisis Completed NCT01197417 Phase 2, Phase 3 Intravenous Magnesium Sulfate;Normal Saline Placebo
48 Evaluation of the Lung Capillary Blood Volume in Children With Sickle Cell Disease Completed NCT00560261 Phase 3
49 Stroke Prevention in Sickle Cell Anemia (STOP 1) Completed NCT00000592 Phase 3
50 Penicillin Prophylaxis in Sickle Cell Disease (PROPS) Completed NCT00000585 Phase 3 penicillin

Search NIH Clinical Center for Sickle Cell Disease

Inferred drug relations via UMLS 72 / NDF-RT 51 :


Hydroxyurea
Sodium phenylbutyrate

Genetic Tests for Sickle Cell Disease

Anatomical Context for Sickle Cell Disease

MalaCards organs/tissues related to Sickle Cell Disease:

41
Bone, Bone Marrow, Endothelial, Testes, Lung, Kidney, Heart

Publications for Sickle Cell Disease

Articles related to Sickle Cell Disease:

(show top 50) (show all 13507)
# Title Authors PMID Year
1
Lactate dehydrogenase as a biomarker of hemolysis-associated nitric oxide resistance, priapism, leg ulceration, pulmonary hypertension, and death in patients with sickle cell disease. 9 38 4
16291595 2006
2
A Phase 3 Randomized Trial of Voxelotor in Sickle Cell Disease. 38 17
31199090 2019
3
Reduced peripheral blood superoxide dismutase 2 expression in sickle cell disease. 38 17
31098737 2019
4
Sickle cell disease: Reducing the global disease burden. 38 17
31069977 2019
5
Sickle cell disease. 38 4
28159390 2017
6
How we manage iron overload in sickle cell patients. 38 4
28295188 2017
7
Gene Therapy for β-Hemoglobinopathies. 38 4
28377044 2017
8
Interventions for preventing silent cerebral infarcts in people with sickle cell disease. 38 4
28500860 2017
9
Intravascular hemolysis and the pathophysiology of sickle cell disease. 38 4
28248201 2017
10
Gene Therapy in a Patient with Sickle Cell Disease. 38 4
28249145 2017
11
Crizanlizumab for the Prevention of Pain Crises in Sickle Cell Disease. 38 4
27959701 2017
12
Prophylactic versus selective blood transfusion for sickle cell disease in pregnancy. 38 4
28005272 2016
13
New developments in anti-sickling agents: can drugs directly prevent the polymerization of sickle haemoglobin in vivo? 38 4
27605087 2016
14
The potential of gene therapy approaches for the treatment of hemoglobinopathies: achievements and challenges. 38 4
27695619 2016
15
Haematopoietic stem cell transplantation for sickle cell disease - current practice and new approaches. 38 4
27255787 2016
16
Fetal haemoglobin in sickle-cell disease: from genetic epidemiology to new therapeutic strategies. 38 4
27353686 2016
17
New insights into sickle cell disease: mechanisms and investigational therapies. 38 4
27055046 2016
18
Alternative donor allogeneic hematopoietic cell transplantation for hemoglobinopathies. 38 4
27000737 2016
19
Central nervous system complications and management in sickle cell disease. 38 4
26758917 2016
20
Neutrophils, platelets, and inflammatory pathways at the nexus of sickle cell disease pathophysiology. 38 4
26758915 2016
21
Indications and Results of HLA-Identical Sibling Hematopoietic Cell Transplantation for Sickle Cell Disease. 38 4
26500093 2016
22
2015 Clinical trials update in sickle cell anemia. 38 4
26178236 2015
23
The LSD1 inhibitor RN-1 induces fetal hemoglobin synthesis and reduces disease pathology in sickle cell mice. 38 4
26031919 2015
24
Update of hematopoietic cell transplantation for sickle cell disease. 38 4
25767957 2015
25
Variation in pediatric emergency department care of sickle cell disease and fever. 38 4
25779022 2015
26
Pregnancy in patients with sickle cell disease: maternal and perinatal outcomes. 38 4
24866352 2015
27
Phytomedicines (medicines derived from plants) for sickle cell disease. 38 4
25844571 2015
28
Elevated tricuspid regurgitant velocity as a marker for pulmonary hypertension in children with sickle cell disease: less prevalent and predictive than previously thought? 38 4
24942020 2015
29
Immunologic effects of hydroxyurea in sickle cell anemia. 38 4
25180279 2014
30
Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members. 38 4
25203083 2014
31
The ethics of a proposed study of hematopoietic stem cell transplant for children with "less severe" sickle cell disease. 38 4
24963044 2014
32
Pulmonary hypertension of sickle cell disease beyond classification constraints. 38 4
24794120 2014
33
Sickle cell disease and pregnancy outcomes: population-based study on 8.8 million births. 38 4
24344096 2014
34
Evidence-based focused review of the status of hematopoietic stem cell transplantation as treatment of sickle cell disease and thalassemia. 38 4
24511087 2014
35
Red blood cell alloimmunization in sickle cell disease and in thalassaemia: current status, future perspectives and potential role of molecular typing. 38 4
24117723 2014
36
Targeted fetal hemoglobin induction for treatment of beta hemoglobinopathies. 38 4
24589264 2014
37
Management of sickle cell disease in the community. 38 4
24613806 2014
38
Hydroxyurea is associated with lower costs of care of young children with sickle cell anemia. 38 4
23999955 2013
39
The natural history of sickle cell disease. 38 4
23813607 2013
40
A randomized, placebo-controlled trial of arginine therapy for the treatment of children with sickle cell disease hospitalized with vaso-occlusive pain episodes. 38 4
23645695 2013
41
Bacteremia risk and outpatient management of febrile patients with sickle cell disease. 38 4
23669523 2013
42
Stroke in patients with sickle cell disease. 38 4
23782084 2013
43
Sickle cell vaso-occlusion causes activation of iNKT cells that is decreased by the adenosine A2A receptor agonist regadenoson. 38 4
23377438 2013
44
Lysine-specific demethylase 1 is a therapeutic target for fetal hemoglobin induction. 38 4
23416702 2013
45
Sickle cell disease, vasculopathy, and therapeutics. 38 4
23190149 2013
46
Hydroxyurea for the treatment of sickle cell disease: efficacy, barriers, toxicity, and management in children. 38 4
22517797 2012
47
Red blood cell alloimmunization in sickle cell disease: pathophysiology, risk factors, and transfusion management. 38 4
22563085 2012
48
Novel approaches to the treatment of sickle cell disease: the potential of histone deacetylase inhibitors. 38 4
22780210 2012
49
A biophysical indicator of vaso-occlusive risk in sickle cell disease. 38 4
22378926 2012
50
Markers of severe vaso-occlusive painful episode frequency in children and adolescents with sickle cell anemia. 38 4
21890147 2012

Variations for Sickle Cell Disease

Expression for Sickle Cell Disease

Search GEO for disease gene expression data for Sickle Cell Disease.

Pathways for Sickle Cell Disease

Pathways related to Sickle Cell Disease according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 11.65 HBG2 HBG1 HBD HBB
2
Show member pathways
10.8 HBB HBA2
3 10.7 VCAM1 HBB HBA2
4 10.61 VCAM1 SELP HBB HBA2

GO Terms for Sickle Cell Disease

Cellular components related to Sickle Cell Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.87 VCAM1 SELP PGF MIR144 HBB HBA2
2 blood microparticle GO:0072562 9.46 HBG2 HBD HBB HBA2
3 hemoglobin complex GO:0005833 9.35 HBG2 HBG1 HBD HBB HBA2
4 endocytic vesicle lumen GO:0071682 9.26 HBB HBA2
5 haptoglobin-hemoglobin complex GO:0031838 9.02 HBG2 HBG1 HBD HBB HBA2

Biological processes related to Sickle Cell Disease according to GeneCards Suite gene sharing:

(show all 12)
# Name GO ID Score Top Affiliating Genes
1 response to hypoxia GO:0001666 9.69 VCAM1 PGF EPO
2 response to lipopolysaccharide GO:0032496 9.67 VCAM1 SELP EPO
3 cellular oxidant detoxification GO:0098869 9.65 HBG2 HBG1 HBD HBB HBA2
4 blood coagulation GO:0007596 9.62 HBG2 HBG1 HBD HBB
5 leukocyte cell-cell adhesion GO:0007159 9.48 VCAM1 SELP
6 cell volume homeostasis GO:0006884 9.46 SLC12A4 KCNN4
7 calcium-mediated signaling using intracellular calcium source GO:0035584 9.43 VCAM1 SELP
8 leukocyte tethering or rolling GO:0050901 9.4 VCAM1 SELP
9 erythrocyte maturation GO:0043249 9.37 G6PD EPO
10 hydrogen peroxide catabolic process GO:0042744 9.35 HBG2 HBG1 HBD HBB HBA2
11 protein heterooligomerization GO:0051291 9.16 HBB HBA2
12 oxygen transport GO:0015671 9.02 HBG2 HBG1 HBD HBB HBA2

Molecular functions related to Sickle Cell Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 heme binding GO:0020037 9.77 HBG2 HBG1 HBD HBB HBA2
2 oxygen binding GO:0019825 9.72 HBG2 HBG1 HBD HBB HBA2
3 peroxidase activity GO:0004601 9.65 HBG2 HBG1 HBD HBB HBA2
4 oxygen carrier activity GO:0005344 9.55 HBG2 HBG1 HBD HBB HBA2
5 hemoglobin alpha binding GO:0031721 9.4 HBD HBB
6 organic acid binding GO:0043177 9.35 HBG2 HBG1 HBD HBB HBA2
7 haptoglobin binding GO:0031720 9.02 HBG2 HBG1 HBD HBB HBA2

Sources for Sickle Cell Disease

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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