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Polysplenia
Polysplenia is a congenital disease manifested by multiple small accessory spleens, rather than a single, full-sized, normal spleen. Polysplenia sometimes occurs alone, but it is often accompanied by other developmental abnormalities. Conditions associated with polysplenia include gastrointestinal abnormalities, such as intestinal malrotation or biliary atresia, as well as cardiac abnormalities, such as dextrocardia. Associated conditions There are frequent associated congenital anomalies all related to deviations in the development of anatomical asymmetries in early embryonic stages. These conditions considered together are called "polysplenia syndrome". Associated conditions include heterotaxy syndrome, intestinal malrotation, situs inversus, biliary atresia, and several cardiac malformations. Associated cardiac conditions include dextrocardia, atrial situs ambiguus, ventricular inversion, and VA concordance with left posterior aorta. Although present, the multiple small spl ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Heterotaxy Syndrome
Situs ambiguus (), or heterotaxy, is a rare congenital defect in which the major visceral Organ (anatomy), organs are distributed abnormally within the chest and abdomen. Clinically, heterotaxy spectrum generally refers to any defect of left-right asymmetry and arrangement of the visceral organs; however, classical heterotaxy requires multiple organs to be affected. This does not include the congenital defect situs inversus, which results when arrangement of all the organs in the abdomen and chest are mirrored, so the positions are opposite the normal placement. Situs inversus is the mirror image of situs solitus, which is normal asymmetric distribution of the abdominothoracic visceral organs. Situs ambiguus can also be subdivided into left-isomerism and right-isomerism based on the defects observed in the spleen, lungs and Atrium (heart), atria of the heart. Individuals with situs inversus or situs solitus do not experience fatal dysfunction of their organ systems, as general ana ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Functional Asplenia
Asplenia is the absence of normal spleen function and is associated with some serious infection risks. Hyposplenism is the condition of reduced ('hypo-'), but not absent, splenic functioning. ''Functional'' asplenia occurs when splenic tissue is present but does not work well (e.g. sickle-cell disease, polysplenia) such patients are managed as if asplenic while in ''anatomic'' asplenia, the spleen itself is absent. Causes Congenital * Congenital asplenia is rare. There are two distinct types of genetic disorders: heterotaxy syndromeOnline Mendelian Inheritance in Man. OMIM entry 208530: Right atrial isomerism; RAI. Johns Hopkins University/ref> and isolated congenital asplenia.Online Mendelian Inheritance in Man. Johns Hopkins UniversityOMIM entry 271400: Asplenia, isolated congenital; ICAS. * polysplenia Acquired Acquired asplenia occurs for several reasons: * Following splenectomy due to splenic rupture from trauma or because of tumor * After splenectomy with the ''goal'' ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Spleen
The spleen (, from Ancient Greek '' σπλήν'', splḗn) is an organ (biology), organ found in almost all vertebrates. Similar in structure to a large lymph node, it acts primarily as a blood filter. The spleen plays important roles in regard to red blood cells (erythrocytes) and the immune system. It removes old red blood cells and holds a reserve of blood, which can be valuable in case of Shock (circulatory), hemorrhagic shock, and also Human iron metabolism, recycles iron. As a part of the mononuclear phagocyte system, it metabolizes hemoglobin removed from senescent red blood cells. The globin portion of hemoglobin is degraded to its constitutive amino acids, and the heme portion is metabolized to bilirubin, which is removed in the liver. The spleen houses antibody-producing lymphocytes in its white pulp and monocytes which remove antibody-coated bacteria and antibody-coated blood cells by way of blood and lymph node circulation. These monocytes, upon moving to injured ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Biliary Atresia
Biliary atresia, also known as extrahepatic ductopenia and progressive obliterative cholangiopathy, is a childhood disease of the liver in which one or more bile ducts are abnormally narrow, blocked, or absent. It can be congenital or acquired. Biliary atresia is the most common reason for pediatric liver transplantation in the United States. It has an incidence of one in 10,000–15,000 live births in the United States, and a prevalence of one in 16,700 in the British Isles. Globally, biliary atresia cases are most common in East Asia, with a frequency of one in 5,000. The cause of biliary atresia in Egyptian infants has been proven to be as a result of aflatoxin induced cholangiopathy acquired prenatally in infants who have glutathione S transferase M1 deficiency. The biliary atresia phenotype caused by congenital aflatoxicosis in GST M1 deficient neonates is named Kotb disease. Syndromic biliary atresia (e.g. Biliary Atresia Splenic Malformation (BASM)) has been associated ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Medical Genetics
Medical genetics is the branch of medicine that involves the diagnosis and management of hereditary disorders. Medical genetics differs from human genetics in that human genetics is a field of scientific research that may or may not apply to medicine, while medical genetics refers to the application of genetics to medical care. For example, research on the causes and inheritance of genetic disorders would be considered within both human genetics and medical genetics, while the diagnosis, management, and counselling people with genetic disorders would be considered part of medical genetics. In contrast, the study of typically non-medical phenotypes such as the genetics of eye color would be considered part of human genetics, but not necessarily relevant to medical genetics (except in situations such as albinism). ''Genetic medicine'' is a newer term for medical genetics and incorporates areas such as gene therapy, personalized medicine, and the rapidly emerging new medical specia ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Congenital Disease
A birth defect is an abnormal condition that is present at birth, regardless of its cause. Birth defects may result in disabilities that may be physical, intellectual, or developmental. The disabilities can range from mild to severe. Birth defects are divided into two main types: structural disorders in which problems are seen with the shape of a body part and functional disorders in which problems exist with how a body part works. Functional disorders include metabolic and degenerative disorders. Some birth defects include both structural and functional disorders. Birth defects may result from genetic or chromosomal disorders, exposure to certain medications or chemicals, or certain infections during pregnancy. Risk factors include folate deficiency, drinking alcohol or smoking during pregnancy, poorly controlled diabetes, and a mother over the age of 35 years old. Many birth defects are believed to involve multiple factors. Birth defects may be visible at birth or diagn ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Accessory Spleen
An accessory spleen is a small nodule of splenic tissue found apart from the main body of the spleen. Accessory spleens are found in approximately 10 percent of the population and are typically around 1 centimetre in diameter. They may resemble a lymph node or a small spleen. They form either by the result of developmental anomalies or trauma. They are medically significant in that they may result in interpretation errors in diagnostic imaging or continued symptoms after therapeutic splenectomy. Polysplenia is the presence of multiple accessory spleens rather than one normal spleen. Causes and locations Accessory spleens may be formed during embryonic development when some of the cells from the developing spleen are deposited along the path from the midline, where the spleen forms, over to its final location on the left side of the abdomen by the 9th–11th ribs. The most common locations for accessory spleens are the hilum of the spleen and adjacent to the tail of the pancrea ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Intestinal Malrotation
Intestinal malrotation is a congenital anomaly of rotation of the midgut. It occurs during the first trimester as the fetal gut undergoes a complex series of growth and development. Malrotation can lead to a dangerous complication called volvulus, in which cases emergency surgery is indicated. Malrotation can refer to a spectrum of abnormal intestinal positioning, often including: * The small intestine found predominantly on the right side of the abdomen * The cecum displaced from its usual position in the right lower quadrant into the epigastrium or right hypochondrium * An absent or displaced ligament of Treitz * Fibrous peritoneal bands called bands of Ladd running across the vertical portion of the duodenum * An unusually narrow, stalk-like mesentery The position of the intestines, narrow mesentery and Ladd's bands can contribute to several severe gastrointestinal conditions. The narrow mesentery predisposes some cases of malrotation to midgut volvulus, a twisting of the enti ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Dextrocardia
Dextrocardia () is a rare congenital condition in which the apex of the heart is located on the right side of the body, rather than the more typical placement towards the left. There are two main types of dextrocardia: dextrocardia of embryonic arrest (also known as isolated dextrocardia) and dextrocardia ''situs inversus''. Dextrocardia ''situs inversus'' is further divided. Classification Dextrocardia of embryonic arrest In this form of dextrocardia, the heart is simply placed further right in the thorax than is normal. It is commonly associated with severe defects of the heart and related abnormalities including pulmonary hypoplasia. Dextrocardia situs solitus Dextrocardia refers to a heart positioned in the right side of the chest. Situs solitus describes viscera that are in the normal position, with the stomach on the left side. Dextrocardia situs inversus Dextrocardia situs inversus refers to the heart being a mirror image situated on the right side. For all visceral ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Situs Inversus
''Situs inversus'' (also called ''situs transversus'' or ''oppositus'') is a Congenital disorder, congenital condition in which the major Organ (anatomy), visceral organs are reversed or mirror image, mirrored from their normal positions. The normal arrangement of internal organs is known as ''situs solitus''. Although cardiac problems are more common, many people with ''situs inversus'' have no medical symptoms or complications resulting from the condition, and until the advent of modern medicine, it was usually undiagnosed. ''Situs inversus'' is found in about 0.01% of the population, or about 1 person in 10,000. In the most common situation, ''situs inversus totalis'', it involves complete transposition (right to left reversal) of all of the viscera. The heart is not in its usual position in the left chest, but is on the right, a condition known as ''dextrocardia'' (). Because the relationship between the organs is not changed, most people with ''situs inversus'' have no associa ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Ventricular Inversion
Ventricular inversion is a condition in which the anatomic right ventricle of the heart is on the left side of the interventricular septum and the anatomic left ventricle A ventricle is one of two large chambers located toward the bottom of the heart that collect and expel blood towards the peripheral beds within the body and lungs. The blood pumped by a ventricle is supplied by an atrium, an adjacent chamber in t ... is on the right. References External links Congenital heart defects {{circulatory-disease-stub ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Splenosis
Splenosis is the result of spleen tissue breaking off the main organ and implanting at another site inside the body. This is called '' heterotopic autotransplantation'' of the spleen. It most commonly occurs as a result of traumatic splenic rupture or abdominal surgery. Depending on the location of the spleen, the new piece usually implants in another part of the abdominal cavity (including the pelvic cavity). Single case reports also describe splenosis in the thoracic cavity, in subcutaneous tissue, in the liver or in the cranial cavity. Splenosis must be distinguished from the presence of additional spleens, which are innate and are the result of differences in embryological development. Additionally, splenosis must be differentiated from malignant tumors which may look similar when imaged.R. D. Fremont, T. W. Rice: ''Splenosis: A Review.'' History Ectopic splenic tissue was first described in 1896 by Albrecht in Germany, whereas the term “splenosis” was first used by Buc ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
