Selective immunoglobulin A deficiency (SIgAD) is a primary immunodeficiency disease and is the most common of the primary antibody deficiencies.[1] Total immunoglobulin A deficiency (IgAD) is defined as an undetectable serum immunoglobulin A (IgA) level at a value < 5 mg/dL (0.05 g/L) in humans. Partial IgAD refers to detectable but decreased IgA levels that are more than 2 standard deviations below normal age-adjusted means.[2, 3] IgAD is commonly associated with normal B lymphocytes in peripheral blood, normal CD4+ and CD8+ T cells, and, usually, normal neutrophil and lymphocyte counts. Anti-IgA autoantibodies of the IgG and/or IgE isotype may be present. Peripheral blood may also be affected by autoimmune cytopenias, eg, autoimmune thrombocytopenia,[4, 5] and patients may have other autoimmune phenomena. IgA was first identified by Graber and Williams in 1952; ten years later, the first patients with IgAD were described. IgAD is a heterogeneous disorder, and the results of intensive study are beginning to elucidate genetic loci and molecular pathogenesis that contribute to various subtypes of this disorder. Several lines of evidence suggest that, in many cases, IgAD and common variable immunodeficiency (CVID) have a common pathogenesis, which is discussed further in Pathophysiology. Other data indicate different genetic risk factors. Family studies show variable inheritance patterns. Familial inheritance of IgAD occurs in approximately 20% of cases,[6] and, within families, IgAD and CVID are associated.[7, 8] Many IgAD patients are asymptomatic (ie, "normal" blood donors) and are identified by finding a laboratory abnormality, without any apparent associated clinical disease. Some patients with IgAD may have the following associated conditions: (1) deficits in one or more immunoglobulin G (IgG) subclasses (this accounts for 20-30% of IgA-deficient patients, many of whom may have total IgG levels within the normal range) or (2) a deficient antibody response to pneumococcal immunization (specific polysaccharide antibody deficiency [SPAD]). Some patients with IgAD later develop CVID, and family members of patients with CVID may have only selective IgAD. Characterization of the receptor for the transmembrane activator and calcium-modulator and cyclophilin ligand interactor (TACI), encoded by the gene TNFRSF13B ( tumor necrosis factor receptor superfamily member 13B), suggests that people with the C104, A181E, and ins204A variants may be at risk for IgAD that progresses to CVID.[9] Primary IgAD is permanent, and below-normal levels have been noted to remain static and persist after 20 years of observation.[10] A recent report documents a rare case of reversion.[11] Environmental factors such as drugs or infections can cause IgAD, but this form is reversible in more than half the cases (see Causes). Although individuals with IgAD have largely been considered healthy, recent studies indicate a higher rate of symptoms. A 20-year follow-up study that compared 204 healthy blood donors with incidentally identified IgAD to 237 healthy subjects with normal IgA levels demonstrated that 80% of IgAD donors and 50% of control subjects had episodes of infections, drug allergy, or autoimmune or atopic disease. Severe respiratory tract infections occurred in 26% of IgAD subjects, in 24% of subjects with decreased IgA levels, and in 8% of control subjects; however, the incidence of life-threatening infections was not increased. IgAD is more common in adult patients with chronic lung disease than in healthy age-matched control subjects.[12] Patients with IgAD are at some increased risk of developing severe reactions after receiving blood products.[13, 14, 15] IgG anti-IgA antibodies may cause severe transfusion reactions if patients with IgAD are given whole blood; therefore, IgA-poor blood or washed red cells are preferred for those patients. IgA-deficient patients with immunoglobulin E (IgE)–class anti-IgA antibodies are at risk for anaphylaxis if they receive blood or intravenous immunoglobulin, but this situation is extremely rare. Individuals with such an unusual profile should receive only low IgA intravenous immunoglobulin preparations. However, caution must be used when administering IGIV to patients with IgAD if their anti-IgA status is unknown. A history devoid of previous blood product administration does not exclude the possibility of anti-IgA antibodies or adverse reactions. Fortunately, appropriate precautions can significantly reduce morbidity (see Treatment). Blood banks can use a simple ELISA screening approach to establish an IgAD blood donor poo

Asplenia is the absence of spleen and/or its functions. Abnormalities of the spleen may be classified on a pattern oriented approach, based on splenic imaging.[1] These include anomalies of the following: Shape (clefts, notches, lobules) Location (wandering spleen) Number (asplenia, polysplenia) Size (splenomegaly, atrophy) Solitary lesions (cysts, lymphangiomas, hemangiomas, hamartomas) Multiple lesions (trauma, infections, neoplasms, storage disorders) Diffuse disease (infarction, heavy metal deposition, peliosis) Absence of splenic tissue can be total (congenital asplenia) or partial (hypoplastic) from birth. Loss of splenic tissue due to surgical removal may occur later in life as a result of trauma that causes rupture of the organ. The spleen may be removed in other conditions (eg, hemoglobinopathies) to improve the red cell life expectancy. Removal of the spleen may be undertaken as a result of being involved in a neoplastic processor as a staging procedure in some cancers. Occasionally, the spleen may be removed to address the sheer mass effect of a massive enlargement (such as in storage disorders), which can cause mass effects. Autosplenectomy is the process where the spleen loses its function due to multiple and repeated infarctive episodes, as in sickle hemoglobinopathies. See the image below.

Sodium levels are tightly controlled in a healthy individual by regulation of urine concentration and an intact thirst mechanism. Hypernatremia (defined as a serum sodium level >145 mEq/L) is rare in patients with preserved thirst mechanism. When hypernatremia does occur, it is associated with a high mortality rate (>50% in most studies). Given this high mortality rate, the emergency physician must be able to recognize and treat this condition. This article discusses the patients in whom hypernatremia should be suspected and how to initiate workup and administer appropriate treatment. In general, hypernatremia can be caused by derangement of the thirst response or altered behavioral response thereto (primarily psychiatric patients, and elderly patients who are institutionalized), impaired renal concentrating mechanism (diabetes insipidus [DI]) secondary to kidney pathology (nephrogenic DI) or difficulty with the neurohormonal control of this concentrating mechanism (central DI), or by losses of free water from other sources.

Breast cancer usually starts off in the inner lining of milk ducts or the lobules that supply them with milk. A malignant tumor can spread to other parts of the body. A breast cancer that started off in the lobules is known as lobular carcinoma, while one that developed from the ducts is called ductal carcinoma. The vast majority of breast cancer cases occur in females. This article focuses on breast cancer in women. We also have an article about male breast cancer. Breast cancer is the most common invasive cancer in females worldwide. It accounts for 16% of all female cancers and 22.9% of invasive cancers in women. 18.2% of all cancer deaths worldwide, including both males and females, are from breast cancer. Breast cancer rates are much higher in developed nations compared to developing ones. There are several reasons for this, with possibly life-expectancy being one of the key factors - breast cancer is more common in elderly women; women in the richest countries live much longer than those in the poorest nations. The different lifestyles and eating habits of females in rich and poor countries are also contributory factors, experts believe. According to the National Cancer Institute, 232,340 female breast cancers and 2,240 male breast cancers are reported in the USA each year, as well as about 39,620 deaths caused by the disease.

How to Treat Cuts & Scrapes | First Aid Training

Thalassemia is an inherited blood disorder characterized by less hemoglobin and fewer red blood cells in your body than normal. Several types of thalassemia exist, including alpha-thalassemia, beta-thalassemia intermedia, Cooley's anemia and Mediterranean anemia. Hemoglobin is the substance in your red blood cells that allows them to carry oxygen. The low hemoglobin and fewer red blood cells of thalassemia may cause anemia, leaving you fatigued. If you have mild thalassemia, you may not need treatment. But, if you have a more severe form of thalassemia, you may need regular blood transfusions. You can also take steps on your own to cope with fatigue, such as choosing a healthy diet and exercising regularly.

The menstrual cycle is the monthly series of changes a woman's body goes through in preparation for the possibility of pregnancy. Each month, one of the ovaries releases an egg — a process called ovulation. At the same time, hormonal changes prepare the uterus for pregnancy.

Keratitis is an inflammation of the cornea — the clear, dome-shaped tissue on the front of your eye that covers the pupil and iris. Keratitis is sometimes caused by an infection involving bacteria, viruses, fungi or parasites. Noninfectious keratitis can be caused by a minor injury, wearing your contact lenses too long or other noninfectious diseases. If you have eye redness or other symptoms of keratitis, make an appointment to see your doctor. With prompt attention, mild to moderate cases of keratitis can usually be effectively treated without loss of vision. If left untreated, or if an infection is severe, keratitis can lead to serious complications that may permanently damage your vision.

The exact cause of schizophrenia isn't known, but genetics, environment, and imbalanced brain chemicals may play a role. Schizophrenia is characterized by abnormal social behavior. In severe cases, patients may see or hear things that aren't real. Treatment is usually lifelong and often involves a combination of medications and psychological and social therapy.

What is idiopathic intracranial hypertension??? Idiopathic intracranial hypertension (IIH) is a disorder that results from an increase in the pressure of the Cerebro-Spinal Fluid (CSF) that cushions and protects the brain and spinal cord. The CSF is constantly produced in the brain and reabsorbed back into the bloodstream at a fairly constant rate. This allows the fluid pressure around the brain to remain constant. What are the symptoms of idiopathic intracranial hypertension? Headaches that are generally nonspecific in location, type and frequency and can be associated with nausea and vomiting. Pulsatile tinnitus is a rhythmic or pulsating ringing heard in one or both ears. Horizontal double vision can be a sign of pressure on the 6th cranial nerve(s). Nonspecific radiating pain in the arms or legs (radicular pain). Transient obscurations of vision (TOV), which are temporary dimming or complete blacking out of vision. Visual field defects. These defects can occur in the central as well as the peripheral vision. Loss of color vision. What causes idiopathic intracranial hypertension? The cause is usually not known. A common explanation for increased pressure is a problem with the reabsorption of this fluid back into the body, which causes the pressure to increase. Sometimes the cause is determined and is referred to as “secondary” intracranial hypertension.

Ventricular tachycardia is a type of heart rhythm disorder (arrhythmia) in which the lower chambers of your heart (ventricles) beat very quickly because of a problem in your heart's electrical system. In ventricular tachycardia, your heart may not be able to pump enough blood to your body and lungs because the chambers are beating so fast that they don't have time to properly fill. Ventricular tachycardia may be brief — lasting for just seconds and often not causing symptoms — or it can last for much longer, and you can develop symptoms such as dizziness or lightheadedness, or you can even pass out. This condition usually occurs in people with other heart conditions, such as coronary artery disease, cardiomyopathy and some types of valvular heart disease. Ventricular tachycardia may lead to a condition in which your lower heart chambers quiver (ventricular fibrillation), which may cause your heart to stop (sudden cardiac arrest) and lead to death if not treated immediately. Ventricular tachycardia can also cause your heart to stop, especially if the heart is beating very quickly, if it's lasting for a long period, and if you have an underlying heart condition.

Pulmonary fibrosis is a lung disease that occurs when lung tissue becomes damaged and scarred. This thickened, stiff tissue makes it more difficult for your lungs to work properly. As pulmonary fibrosis worsens, you become progressively more short of breath. The scarring associated with pulmonary fibrosis can be caused by a multitude of factors. But in most cases, doctors can't pinpoint what's causing the problem. When a cause can't be found, the condition is termed idiopathic pulmonary fibrosis. The lung damage caused by pulmonary fibrosis can't be repaired, but medications and therapies can sometimes help ease symptoms and improve quality of life. For some people, a lung transplant might be appropriate.

Scientists don't know what causes canker sores. Most believe that there is a problem with the body's immune system. Emotional stress, menstruation or injury to the mouth are common triggers for simple canker sores. Certain foods such as citrus or acidic foods may trigger a canker sore or make one more uncomfortable.

Lymphoma is cancer of the lymph system (or lymphatic system), which is part of our immunity. It is characterized by the formation of solid tumors in the immune system.1 The cancer affects immune cells called lymphocytes, which are white blood cells. Diagram of the lymphatic system The lymphatic system is a system of vessels that branch back from virtually all our tissues to drain excess fluids and present foreign material to the lymph nodes. Learn more about the lymphatic system here. Statistics from the US National Cancer Institute estimate that there are nearly 20 cases of non-Hodgkin's lymphoma for every 100,000 people in the American population.2 Hodgkin's lymphoma, meanwhile, is relatively rare, with around three cases in every 100,000 people.3

In lumbar stenosis, the spinal nerve roots in the lower back become compressed and this can produce symptoms of sciatica—tingling, weakness or numbness that radiates from the low back and into the buttocks and legs—especially with activity. Lumbar spinal stenosis often mimics symptoms of vascular insufficiency. Both conditions can cause claudication, which means leg pain with walking. If vascular studies identify normal blood flow, and there is confirmation of spinal stenosis on diagnostic testing, the symptoms are then called neurogenic claudication. In the classic description, people with spinal stenosis will describe an onset of leg pain, or weakness with walking, but with relief of symptoms with sitting. Many will also describe increased tolerance to walking when flexed forward, such as when walking while leaning forward on a shopping cart.

Acute respiratory distress syndrome (ARDS) occurs when fluid builds up in the tiny, elastic air sacs (alveoli) in your lungs. More fluid in your lungs means less oxygen can reach your bloodstream. This deprives your organs of the oxygen they need to function. ARDS typically occurs in people who are already critically ill or who have significant injuries. Severe shortness of breath — the main symptom of ARDS — usually develops within a few hours to a few days after the original disease or trauma. Many people who develop ARDS don't survive. The risk of death increases with age and severity of illness. Of the people who do survive ARDS, some recover completely while others experience lasting damage to their lungs.

Heart sounds are the noises generated by the beating heart and the resultant flow of blood through it. Specifically, the sounds reflect the turbulence created when the heart valves snap shut. In cardiac auscultation, an examiner may use a stethoscope to listen for these unique and distinct sounds that provide important auditory data regarding the condition of the heart. In healthy adults, there are two normal heart sounds often described as a lub and a dub (or dup), that occur in sequence with each heartbeat. These are the first heart sound (S1) and second heart sound (S2), produced by the closing of the atrioventricular valves and semilunar valves, respectively. In addition to these normal sounds, a variety of other sounds may be present including heart murmurs, adventitious sounds, and gallop rhythms S3 and S4.

Near the end of diastole, the ventricles nearly fill with blood, and then the atria contract, adding even more volume to the ventricles. The volume of blood in the ventricles at the end of diastole is referred to as the end-diastolic volume. The other phase of the cardiac cycle is called systole.

Cardiac arrest is the abrupt loss of heart function in a person who may or may not have diagnosed heart disease. The time and mode of death are unexpected. It occurs instantly or shortly after symptoms appear. Each year, more than 350,000 emergency medical services-assessed out-of-hospital cardiac arrests occur in the United States

People with panic disorder have sudden and repeated attacks of fear that last for several minutes or longer. These are called panic attacks. Panic attacks are characterized by a fear of disaster or of losing control even when there is no real danger. A person may also have a strong physical reaction during a panic attack. It may feel like having a heart attack. Panic attacks can occur at any time, and many people with panic disorder worry about and dread the possibility of having another attack.