The main complaint at presentation was bone or joint pains. Symptoms involved bone or joint pains in 72% of the patients, while 59% presented hepatomegaly and 50% splenomegaly. [ncbi.nlm.nih.gov]

Therefore, the present study is an attempt in this direction. The present study attempts to evaluate varied manifestations of SCD in a tertiary care center in north India, which is a nonendemic belt. [doi.org]

• Splenomegaly

  We report here our experience with an adult patient with severe SC disease who developed symptomatic splenomegaly requiring splenectomy while being treated with hydroxyurea. [ncbi.nlm.nih.gov]

  Homozygous hemoglobin C disease is generally a benign condition associated with mild hemolytic anemia and splenomegaly. [nejm.org]

• Anemia

  Natural history of sickle cell anemia in Saudi Arabs: a study of 270 subjects. Ann Intern Med. 1978; 88 (1):1–6. [ PubMed ] [ Google Scholar ] 14. Padmos MA, Roberts GT, Sackey K, et al. [ncbi.nlm.nih.gov]

  In the United States, it's estimated that sickle cell anemia affects 70,000–100,000 people, mainly African Americans. The most common signs and symptoms of sickle cell anemia are linked to anemia and pain. [web.archive.org]

  anemia is a disease in which your body produces abnormally shaped red blood cells. [icd9data.com]

  Sickle cell anemia, hereditary disease that destroys red blood cells by causing them to take on a rigid “sickle” shape. [britannica.com]

• Pain

  Our patient no longer requires narcotic analgesics for pain, has resolution of constitutional symptoms, and has not required further hospitalizations for vaso-occlusive pain crises. [ncbi.nlm.nih.gov]

  Painful events were reduced with hydroxyurea, more in patients >15 years old. [miami.pure.elsevier.com]

  Clinical Scenario A 39 year old man with sickle cell disease presents to the emergency department with a 24hr history of pain in his arms and legs. His pain is consistent with his usual painful crisis. [bestbets.org]

• Fatigue

  People who have sickle cell disease may also have trouble coping with pain and fatigue, as well as with frequent medical visits and hospitalizations. [nhlbi.nih.gov]

• Falling

  Acute splenic sequestration, a well recognized complication of the various sickle cell syndromes, is characterized by increasing splenomegaly and a sudden fall in hemoglobin concentration. [ncbi.nlm.nih.gov]

  The duty on ensuring inclusiveness in school falls to the school authorities who must make arrangements for supporting pupils at school with medical conditions. Pupils with sickle cell disorder fall under this legislation and guidance. [sicklecellanaemia.org]

  Haemoglobin disorders fall into two main categories: sickle-cell disease and thalassaemias. Sickle-cell disease is characterized by a modification in the shape of the red blood cell from a smooth, donut-shape into a crescent or half moon shape. [web.archive.org]

  Public Library (Idaho Falls) Idaho State University (Pocatello) Marshall Public Library (Pocatello) Meridian Library District (Meridian) Nampa Public Library (Nampa) Twin Falls Public Library (Twin Falls) University of Idaho Library (Moscow, and other [tools.wmflabs.org]

• Disability

  Policy Interpretation We consider all medical evidence when we evaluate a claim for disability benefits. [ssa.gov]

  We provide this guidance to help adjudicators consistently apply our policies in disability claims involving SCD. [gpo.gov]

  Please enable JS and disable any ad blocker [cairn.info]

  When we think of inclusiveness, the 2006 United Nations Convention on the Rights for Persons with Disabilities is also applicable and school authorities are responsible for ensuring inclusion of a child with health condition who may further have acquired [sicklecellanaemia.org]

  Complications of sickle cell anemia are a result of sickle cells blocking blood flow to specific organs, and include stroke, acute chest syndrome (a condition that lowers the level of oxygen in the blood), organ damage, other disabilities, and in some [web.archive.org]

• Cough

  Common side effects of Endari include constipation, nausea, headache, abdominal pain, cough, pain in the extremities, back pain and chest pain. [fda.gov]

  Symptoms may include: Chest pain Cough Fever Rapid breathing Shortness of breath Brain complications CLINICAL STROKE A stroke occurs when blood flow is blocked to a part of the brain. When this happens, brain cells can be damaged or can die. [nhlbi.nih.gov]

• Nausea

  After surgery, the patient’s pain improved and he experienced resolution of nausea. The patient was vaccinated against S. pneumoniae, N. meningitides, and H. influenza per CDC guidelines. [eposters.net]

  You should get medical care right away if you have A sudden high fever A severe headache A stiff neck Nausea or vomiting Early treatment can help prevent serious problems, including death. [nlm.nih.gov]

  Common side effects of Endari include constipation, nausea, headache, abdominal pain, cough, pain in the extremities, back pain and chest pain. [fda.gov]

  When symptoms develop, they may include: Nausea Right-side upper belly pain Vomiting If problems continue or recur, a person may need surgery to remove the gallbladder. [nhlbi.nih.gov]

• Abdominal Pain

  We present a case of fulminant ASSC in an adult with hemoglobin SC who developed high fever, intense abdominal pain, leukocytosis, and jaundice. [ncbi.nlm.nih.gov]

  pain, moderate splenomegaly; unique to SC disease is an ↑ risk of retinal disease–proliferative retinopathy, retinal vascular disease, aseptic necrosis of femoral head, acute chest syndrome after fat embolism due to bone infarction. [medical-dictionary.thefreedictionary.com]

  Common side effects of Endari include constipation, nausea, headache, abdominal pain, cough, pain in the extremities, back pain and chest pain. [fda.gov]

• Vascular Disease

  Although many retinal vascular diseases may cause exudative detachments, to the authors' knowledge, this is the first definite case of exudative retinal detachment in a patient with a sickling hemoglobinopathy. [ncbi.nlm.nih.gov]

  […] may be ↑ Clinical Similar to, but less severe than, sickle cell anemia; by preadolescence, episodic skeletal or abdominal pain, moderate splenomegaly; unique to SC disease is an ↑ risk of retinal disease–proliferative retinopathy, retinal vascular disease [medical-dictionary.thefreedictionary.com]

  Bridgers WA Cerebral vascular disease accompanying sickle cell anemia. Am J Pathol 1939;15353- 362 Google Scholar 5. Steinberg MHBrugnara C Pathophysiological-based approaches to treatment of sickle cell disease. [doi.org]

• Skin Ulcer

  Skin ulcers Skin ulcers in the legs can occur if small vessels there are blocked. [healthline.com]

  However, it is a medicine that was originally designed for certain types of cancer and has many side effects, including suppression of white blood cells that fight infection, skin ulcers, stomach problems and anemia. It can also cause birth defects. [news-medical.net]

• Erythema

  Observe for pallor, icterus, and erythema or edema of the extremities or joints. Perform a neurological examination to search for focal neurological deficits. Look for evidence of retinopathy on funduscopy. [web.archive.org]

• Arthralgia

  According to family, she complained of severe arthralgias in her hands and knees for the prior 2 days. Physical examination revealed an obese woman who was unresponsive to verbal stimulus. [cp.neurology.org]

  Fatigue, dyspnea on effort, attacks of mild jaundice (this patient has laboratory features of hemolysis), and arthralgias are seen. Life expectancy is usually only slightly shortened. [path.upmc.edu]

• Fussiness

  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 [nlm.nih.gov]

  Early symptoms of sickle cell disease may include: A yellowish color of the skin, known as jaundice, or whites of the eyes, known as icterus, that occurs when a large number of red cells undergo hemolysis Fatigue or fussiness from anemia Painful swelling [nhlbi.nih.gov]

• Fear

  Anish’s younger brother has gone to India to earn money to pay for his brother’s treatment but his mother fears that even her younger son could be suffering from the same condition, as he complains frequently of joint pains. [nepalitimes.com]

  Life has taken a sharp turn for the better: no more transfusions, no more pain, no more fear. “He said, ‘Mom, I think I want to get me a job,’” said his mother, Leuteresa Roberts. [nytimes.com]

• Irritability

  These include: excessive fatigue or irritability, from anemia fussiness, in babies bedwetting, from associated kidney problems jaundice, which is yellowing of the eyes and skin swelling and pain in hands and feet frequent infections pain in the chest, [healthline.com]

  Signs in infants can include swollen and painful hands and/or feet (dactylitis), irritability, crying, and severe infections. [rarediseases.org]

  In order to prevent problems, call your doctor right away if your child has any of the following: Fever of 101 degrees or higher Signs of infection (chills, lethargy, irritability, poor feeding, vomiting) Severe headache or dizziness Chest pain or trouble [newbornscreening.info]

• Lethargy

  In order to prevent problems, call your doctor right away if your child has any of the following: Fever of 101 degrees or higher Signs of infection (chills, lethargy, irritability, poor feeding, vomiting) Severe headache or dizziness Chest pain or trouble [newbornscreening.info]

Some of the most common causes of nontraumatic spinal cord injury in pediatrics include autoimmune, hypercoagulable, and infections etiologies, necessitating a broad workup. [pediatrics.aappublications.org]

A complete cardiac and neurologic workup, including fasting lipids, serial troponins, and EEG is mandatory. [jscimedcentral.com]

• Reticulocytosis

  […] complication, seen in the first few years of life and resulting in circulatory collapse and death from anemia and hypovolemic shock, is defined as a sudden enlargement of the spleen with a decrease in Hb concentration (2 g/L at least) and substantial reticulocytosis [web.archive.org]

  Reticulin and Howell Jolly bodies do not have the distinctive pattern of Hb H inclusions; however, a brisk reticulocytosis can make identification of a rare H-containing cell difficult. electrophoresis Traditionally, electrophoresis has been the method [clinchem.org]

  Laboratory variables at baseline revealed anemia with expected leukocytosis and reticulocytosis ( Table 2 ). [doi.org]

• Reticulocytes Increased

  With each course, fetal reticulocytes increased within 48-72 h, peaked in 7-11 d, and fell by 18-21 d. [doi.org]

Effects of hydroxyurea treatment for patients with hemoglobin SC disease. [miami.pure.elsevier.com]

To date, only 5 publications have reported short-term, incomplete, or conflicting laboratory and clinical outcomes of hydroxyurea treatment in a total of 71 adults and children with HbSC. [ncbi.nlm.nih.gov]

During the treatment phase, 20.6 dose-limiting toxic effects per 100 patient-years occurred, as compared with 20.7 events per 100 patient-years before treatment. [doi.org]

Prognosis The prognosis is difficult to predict. Serious VOA or organ failure can be a cause of death. The documents contained in this web site are presented for information purposes only. [orpha.net]

Despite these problems, the prognosis is generally good Hb C Disease may lower the red blood cell count and make the body susceptible to opportunistic infections, which could further lead to more severe hemolytic anemia Additional and Relevant Useful [dovemed.com]

It is associated with a poorer prognosis. Hemoglobin SD, hemoglobin SE, and hemoglobin SO These types of sickle cell disease are more rare and usually don’t have severe symptoms. [healthline.com]

Folic acid supplementation may help produce normal red blood cells and improve the symptoms of anemia [7] Prognosis [ edit ] Overall, hemoglobin C disease is one of the more benign hemoglobinopathies. [5] Mild-to-moderate reduction in RBC lifespan may [en.wikipedia.org]

These data suggest that prognosis for lifetime severe SS is initially manifested as an increased risk of septicemia during childhood. Citing Literature Number of times cited according to CrossRef: 39 A. Raffetin, G. Melica, E. Audureau, A. [doi.org]

Careful evaluation of CBCs and peripheral blood smears is required in cases of worsening anemia among sickle cell patients and consideration should be given to cold hemagglutinin disease as an etiology. [ncbi.nlm.nih.gov]

In this paper, the etiology, diagnosis, treatment, and prevention of clinical stroke, as well as so-called “silent stroke,” are examined. [doi.org]

Etiology Sickle cell anemia is determined by combinations of two abnormal alleles of the beta globin gene among which at least one carries the beta 6 glu-val mutation (Hb S). [orpha.net]

Abstract Although the epidemiology and pathophysiology of serious bacterial infection in homozygous sickle cell anemia (SS disease) have become increasingly well understood, information about infection risk and splenic reticuloendothelial function in [ncbi.nlm.nih.gov]

Summary Epidemiology The prevalence of sickle cell carriers in 25 European states is estimated at about 1/150. [orpha.net]

B.Modell et al., Epidemiology of haemoglobin disorders in Europe: an overview. Scand J Clin Lab Invest. 67, 39 (2007) R. Herklotz et al., Hämoglobinopathien ? Klinik und Diagnostik von Thalassämien und anomalen Hämoglobinen. Therap. [charite.de]

Hemoglobin SC Disease Inheritance Hb C Dieases and Trait Symptoms: Hemoglobin C Trait (Hb AC) – no anemia develops (no symptoms) Hemoglobin C Disease (Hb CC) – mild hemolytic anemia, mild spleenomegaly, jaundice and pigmented gallstones might develop Epidemiology [medical-labs.net]

Clinical epidemiology of sickle cell disease in Saudi Arabia The clinical manifestations of SCD are unpredictable and variable. [doi.org]

There seem to be pathophysiological correlations for the observed clinical findings, and there are potential beneficial effects of partial exchange transfusion. [ncbi.nlm.nih.gov]

Gladwin, Intravascular hemolysis and the pathophysiology of sickle cell disease, Journal of Clinical Investigation, 10.1172/JCI89741, 127, 3, (750-760), (2017) ., (2017) ., (2017) ., (2017). [doi.org]

Haemoglobin disorders can be effectively reduced through a strategic balance of disease management and prevention programmes. [web.archive.org]

"Genetic Disorders and the Fetus: Diagnosis, Prevention and Treatment.6th ed". Wiley-Blackwell. 2010 6th ed. 2. "WHO. [doi.org]

Erythrocytapheresis before G-CSF administration may help prevent these complications. [ncbi.nlm.nih.gov]

Prevent infection Infection is the leading cause of death in children with SCD. It is very important that children with SCD take penicillin daily to prevent infection. [newbornscreening.info]