Cruveilhier–Baumgarten Disease
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Cruveilhier–Baumgarten Disease
Cruveilhier–Baumgarten disease or Pégot-Cruveilhier–Baumgarten disease is a rare medical condition in which the umbilical or paraumbilical veins are distended, with an abdominal wall bruit (the Cruveilhier-Baumgarten bruit) and palpable thrill, portal hypertension with splenomegaly, hypersplenism and oesophageal varices, with a normal or small liver. The presence of the Cruveilhier-Baumgarten venous hum is highly suggestive of portal hypertension, and is never a normal physical examination finding. It was first described by Pégot in 1833, and then by Jean Cruveilhier (1835) and Paul Clemens von Baumgarten (1907). Armstrong ''et al.'' (1942) and Steinburg and Galambos (1967) described two different types of the condition: * Cruveilhier-Baumgarten syndrome: liver cirrhosis or portal hypertension is the cause of the distension of the paraumbilical veins (i.e. an ''acquired'' condition in which the veins reopen due to high portal pressure). * Cruveilhier–Baumgarten ...
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Hepatology
Hepatology is the branch of medicine that incorporates the study of liver, gallbladder, biliary tree, and pancreas as well as management of their disorders. Although traditionally considered a sub-specialty of gastroenterology, rapid expansion has led in some countries to doctors specializing solely on this area, who are called hepatologists. Diseases and complications related to viral hepatitis and alcohol are the main reason for seeking specialist advice. More than two billion people have been infected with hepatitis B virus at some point in their life, and approximately 350 million have become persistent carriers. Up to 80% of liver cancers can be attributed to either hepatitis B or hepatitis C virus. In terms of mortality, the former is second only to smoking among known agents causing cancer. With more widespread implementation of vaccination and strict screening before blood transfusion, lower infection rates are expected in the future. In many countries, however, overall ...
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Hypersplenism
Splenomegaly is an enlargement of the spleen. The spleen usually lies in the left upper quadrant (LUQ) of the human abdomen. Splenomegaly is one of the four cardinal signs of ''hypersplenism'' which include: some reduction in number of circulating blood cells affecting granulocytes, erythrocytes or platelets in any combination; a compensatory proliferative response in the bone marrow; and the potential for correction of these abnormalities by splenectomy. Splenomegaly is usually associated with increased workload (such as in hemolytic anemias), which suggests that it is a response to hyperfunction. It is therefore not surprising that splenomegaly is associated with any disease process that involves abnormal red blood cells being destroyed in the spleen. Other common causes include congestion due to portal hypertension and infiltration by leukemias and lymphomas. Thus, the finding of an enlarged spleen, along with caput medusae, is an important sign of portal hypertension. Defi ...
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Diseases Of Liver
A disease is a particular abnormal condition that negatively affects the structure or function of all or part of an organism, and that is not immediately due to any external injury. Diseases are often known to be medical conditions that are associated with specific signs and symptoms. A disease may be caused by external factors such as pathogens or by internal dysfunctions. For example, internal dysfunctions of the immune system can produce a variety of different diseases, including various forms of immunodeficiency, hypersensitivity, allergies and autoimmune disorders. In humans, ''disease'' is often used more broadly to refer to any condition that causes pain, dysfunction, distress, social problems, or death to the person affected, or similar problems for those in contact with the person. In this broader sense, it sometimes includes injuries, disabilities, disorders, syndromes, infections, isolated symptoms, deviant behaviors, and atypical variations of structure and fu ...
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Splenomegaly
Splenomegaly is an enlargement of the spleen. The spleen usually lies in the left upper quadrant (LUQ) of the human abdomen. Splenomegaly is one of the four cardinal signs of ''hypersplenism'' which include: some reduction in number of circulating blood cells affecting granulocytes, erythrocytes or platelets in any combination; a compensatory proliferative response in the bone marrow; and the potential for correction of these abnormalities by splenectomy. Splenomegaly is usually associated with increased workload (such as in hemolytic anemias), which suggests that it is a response to hyperfunction. It is therefore not surprising that splenomegaly is associated with any disease process that involves abnormal red blood cells being destroyed in the spleen. Other common causes include congestion due to portal hypertension and infiltration by leukemias and lymphomas. Thus, the finding of an enlarged spleen, along with caput medusae, is an important sign of portal hypertension. Definiti ...
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Portal Hypertension
Portal hypertension is abnormally increased portal venous pressure – blood pressure in the portal vein and its branches, that drain from most of the intestine to the liver. Portal hypertension is defined as a hepatic venous pressure gradient greater than 5 mmHg. Cirrhosis (a form of chronic liver failure) is the most common cause of portal hypertension; other, less frequent causes are therefore grouped as non-cirrhotic portal hypertension. When it becomes severe enough to cause symptoms or complications, treatment may be given to decrease portal hypertension itself or to manage its complications. Signs and symptoms Signs and symptoms of portal hypertension include: * Ascites (free fluid in the peritoneal cavity), ** Abdominal pain or tenderness (when bacteria infect the ascites, as in spontaneous bacterial peritonitis). * Increased spleen size (splenomegaly), which may lead to lower platelet counts (thrombocytopenia) * Anorectal varices * Swollen veins on the anterior abdomin ...
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Venous Hum
Venous hum is a benign auscultatory phenomenon caused by the normal flow of blood through the jugular veins. At rest, 20% of cardiac output flows to the brain via the internal carotid and vertebral arteries; this drains via the internal jugular veins. The rush of blood from these veins to the brachiocephalic vein can cause the vein walls to vibrate, creating a humming noise which can often be heard by the subject. Typically, a peculiar humming sound is heard in the upper chest near the clavicle, emanating from the supraclavicular fossa just lateral to the sternocleidomastoid muscle, usually more obviously on the right side than on the left. The sound may radiate to the upper sternal border on either side. Though the exact mechanism is still unclear, it has been suggested that the hum occurs when otherwise silent laminar flow through the internal jugular vein is disturbed by deformation of this vessel at the level of the transverse process of the atlas during head rotation. The ve ...
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Liver
The liver is a major Organ (anatomy), organ only found in vertebrates which performs many essential biological functions such as detoxification of the organism, and the Protein biosynthesis, synthesis of proteins and biochemicals necessary for digestion and growth. In humans, it is located in the quadrant (anatomy), right upper quadrant of the abdomen, below the thoracic diaphragm, diaphragm. Its other roles in metabolism include the regulation of Glycogen, glycogen storage, decomposition of red blood cells, and the production of hormones. The liver is an accessory digestive organ that produces bile, an alkaline fluid containing cholesterol and bile acids, which helps the fatty acid degradation, breakdown of fat. The gallbladder, a small pouch that sits just under the liver, stores bile produced by the liver which is later moved to the small intestine to complete digestion. The liver's highly specialized biological tissue, tissue, consisting mostly of hepatocytes, regulates a w ...
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Esophageal Varices
Esophageal varices are extremely dilated sub-mucosal veins in the lower third of the esophagus. They are most often a consequence of portal hypertension, commonly due to cirrhosis. People with esophageal varices have a strong tendency to develop severe bleeding which left untreated can be fatal. Esophageal varices are typically diagnosed through an esophagogastroduodenoscopy. Pathogenesis The upper two thirds of the esophagus are drained via the esophageal veins, which carry deoxygenated blood from the esophagus to the azygos vein, which in turn drains directly into the superior vena cava. These veins have no part in the development of esophageal varices. The lower one third of the esophagus is drained into the superficial veins lining the esophageal mucosa, which drain into the left gastric vein, which in turn drains directly into the portal vein. These superficial veins (normally only approximately 1 mm in diameter) become distended up to 1–2 cm in diameter in as ...
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Splenomegaly
Splenomegaly is an enlargement of the spleen. The spleen usually lies in the left upper quadrant (LUQ) of the human abdomen. Splenomegaly is one of the four cardinal signs of ''hypersplenism'' which include: some reduction in number of circulating blood cells affecting granulocytes, erythrocytes or platelets in any combination; a compensatory proliferative response in the bone marrow; and the potential for correction of these abnormalities by splenectomy. Splenomegaly is usually associated with increased workload (such as in hemolytic anemias), which suggests that it is a response to hyperfunction. It is therefore not surprising that splenomegaly is associated with any disease process that involves abnormal red blood cells being destroyed in the spleen. Other common causes include congestion due to portal hypertension and infiltration by leukemias and lymphomas. Thus, the finding of an enlarged spleen, along with caput medusae, is an important sign of portal hypertension. Definiti ...
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Jean Cruveilhier
Jean Cruveilhier (; 9 February 1791 – 7 March 1874) was a French anatomist and pathologist. Academic career Cruveilhier was born in Limoges, France. As a student in Limoges, he planned to enter the priesthood. He later developed an interest in pathology, being influenced by Guillaume Dupuytren (1777-1835), a friend of Cruveilhier's father. In 1816 he earned his medical doctorate in Paris, where in 1825 he succeeded Pierre Augustin Béclard (1785–1825) as professor of anatomy. In 1836 he relinquished the chair of anatomy to Gilbert Breschet (1784–1845), and in doing so, became the first occupant of the recently founded chair of pathological anatomy. In 1836 he was elected to the Académie de Médecine, becoming its president in 1839. For over forty years he was president of the ''Société anatomique''. Puerto Rican pro-independence leader, surgeon and Légion d'honneur laureate, Ramón Emeterio Betances, was one of his prominent students. He died, aged 83, ...
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Portal Hypertension
Portal hypertension is abnormally increased portal venous pressure – blood pressure in the portal vein and its branches, that drain from most of the intestine to the liver. Portal hypertension is defined as a hepatic venous pressure gradient greater than 5 mmHg. Cirrhosis (a form of chronic liver failure) is the most common cause of portal hypertension; other, less frequent causes are therefore grouped as non-cirrhotic portal hypertension. When it becomes severe enough to cause symptoms or complications, treatment may be given to decrease portal hypertension itself or to manage its complications. Signs and symptoms Signs and symptoms of portal hypertension include: * Ascites (free fluid in the peritoneal cavity), ** Abdominal pain or tenderness (when bacteria infect the ascites, as in spontaneous bacterial peritonitis). * Increased spleen size (splenomegaly), which may lead to lower platelet counts (thrombocytopenia) * Anorectal varices * Swollen veins on the anterior abdomin ...
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