A person may also be a carrier of the disease and show no signs or symptoms of the disease. Late complications may result in the development of pigmented gallstones, which is secondary to the detritus of the broken-down blood cells (unconjugated or indirect bilirubin) accumulating within the gallbladder. Also, patients who are heterozygous for a hemochromatosis gene may exhibit iron overload, despite the hemochromatosis genes being recessive. In chronic patients, an infection or other illness can cause an increase in the destruction of red blood cells, resulting in the appearance of acute symptoms, a hemolytic crisis. On a blood smear, Howell-Jolly bodies may be seen within red blood cells. Primary treatment for patients with symptomatic HS has been total splenectomy, which eliminates the hemolytic process, allowing normal hemoglobin, reticulocyte and bilirubin levels. The resultant asplenic patient is susceptible to encapsulated bacterial infection, and prevented with vaccination. If other symptoms, such as abdominal pain persist, the removal of the gallbladder may be warranted for symptomatic cholelithiasis. Epidemiology
Hereditary spherocytosis is the heritable hemolytic disorder, affecting 1 in 2,000 people of Northern European ancestry. According to Harrisons Principles of Internal Medicine, the frequency is at least 1 in 5,000 within the United States of America. While HS is most commonly (though not exclusively) found in Northern European and Japanese families, an estimated 25% of cases are due to spontaneous mutations.
Stupor is the lack of critical mental function and a level of consciousness, in which an affected person is almost entirely unresponsive and responds only to intense stimuli such as pain. The word derives from the Latin stupor ("numbness, insensibility"). Signs and symptoms
Stupor is characterised by impaired reaction to external stimuli. Those in a stuporous state are rigid, mute and only appear to be conscious, as the eyes are open and follow surrounding objects. If not stimulated externally, a patient with stupor will appear to be in a sleepy state most of the time. In some extreme cases of severe depressive disorders the patient can become motionless, lose their appetite and become mute. Short periods of restricted responsivity can be achieved by intense stimulation (e.g. pain, bright light, loud noise, shock). Causes
Stupor is associated with infectious diseases, complicated toxic states (e.g. heavy metals), severe hypothermia, mental illnesses (e.g. schizophrenia, major depressive disorder), epilepsy, vascular illnesses (e.g. hypertensive encephalopathy), acute stress reaction, neoplasms (e.g. brain tumors), brain disorders (e.g. alzheimers, dementia, fatal insomnia), B12 deficiency affects mental abilities, major trauma, alcohol poisoning, vitamin D excess and other maladies.Lesions of the ascending reticular activation system on height of the pons and metencephalon have been shown to cause stupor. The incidence is higher after left-sided lesions. Management
Because stupors are caused by another health condition, treatment focuses on uncovering and treating the cause. Doctors may administer IV antibiotics or fluids to treat infections and nutritional deficits, or conduct an MRI to check for lesions on the brain.