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Down Syndrome and Hirschsprung Disease Hirschsprung disease, also known as congenital intestinal aganglionosis or aganglionic megacolon, is a congenital disorder characterized by absence of enteric ganglia along a variable length of the intestine, named after Harald Hirschsprung (1830-1916), a Danish physician who first described the disease in 1886. Hirschsprung disease is a lack of ganglion cells in the wall of the bowel. Hirschsprung disease was first described in a constipated infant with Down syndrome in Cuba Vacher et al. www.he.net /~altonweb/cs/downsyndrome/hirschin.html   (1252 words)

Hirschsprung's disease - Wikipedia, the free encyclopedia Hirschsprung's disease, or congenital aganglionic megacolon, involves an enlargement of the colon, caused by bowel obstruction resulting from an aganglionic section of bowel (the normal enteric nerves are absent) that starts at the anus and progresses upwards. This disease is named for Harald Hirschsprung, the Danish physician who first described the disease in 1886, describing two infants who had died with swollen bellies. Hirschsprung’s disease is a congenital disorder of the colon in which certain nerve cells, known as ganglion cells, are absent, causing chronic constipation (Worman and Ganiats 487). en.wikipedia.org /wiki/Hirschsprung's_disease   (1534 words)

Harald Hirschsprung (www.whonamedit.com) Harald Hirschsprung was born in Copenhagen in 1830. In 1870 Hirschsprung was appointed to a hospital for neonates, thus becoming the first paediatrician in Denmark and was subsequently the obvious choice as chief physician of the Queen Louisa Hospital for Children, which was opened in 1879. Hirschsprung was a withdrawn, difficult man, with a streak of determination which alienated many of his peers. www.whonamedit.com /doctor.cfm/1137.html   (998 words)

IPEG: Guidelines for Surgical Treatment of Hirschsprung's Disease Hirschsprung’s disease (HD), or colonic aganglionosis, was first described clinically by Harald Hirschsprung and later precisely defined by Ovar Swenson. Hirschsprung’s disease is a rare congenital disease of the intestine, characterized by the absence of autonomic ganglion cells in the wall of the colon. Hirschsprung's Disease and Related Neuromuscular Disorders of the Intestine. www.ipeg.org /guidelines/hirschsprungs.html   (1636 words)

Med-Lib - Medical Online Library - English Articles - Oxford Textbook of Surgery - Hirschsprung's disease Hirschsprung's disease has an incidence of 1 in 5000 births and is the most common cause of intestinal obstruction in the neonate. The pathological hallmark of Hirschsprung's disease is absence of ganglion cells in the submucosal plexus of Meissner and aganglionosis or hypoganglionosis in the intermyenteric plexus of Auerbach. Ultrashort-segment Hirschsprung's disease is a disorder of the internal sphincter complex. med-lib.ru /english/oxford/hirsch_dis.shtml   (2210 words)

Hirschsprung's disease Symptoms  Information Hirschsprung's disease, or aganglionic megacolon, involves an enlargement of the colon, caused by bowel obstruction resulting from an aganglionic section of bowel (the normal enteric nerves are absent) that starts at the anus and progresses upwards. A barium enema is the mainstay of diagnosis of Hirschsprung’s. Since Down’s Syndrome occurs in two percent of Hirschsprung’s cases, there is a likelihood that RET is involved heavily in both diseases. www.nextweb.com.br /medical_dictionary/hirschsprungs_disease_symptoms_information.htm   (1445 words)

UNSW Embryology-OMIM HIRSCHSPRUNG DISEASE Hirschsprung disease, with major and modifying sequence variants in a variety of genes, may serve as a model for other complex disorders for which the search for defective genes has begun. The disorder described by Hirschsprung (1888) and known as Hirschsprung disease or aganglionic megacolon is a congenital disorder characterized by absence of enteric ganglia along a variable length of the intestine. Passarge (1993) stated that Harald Hirschsprung (1830-1916) was a Danish physician in Copenhagen and that his family lent its name to the Hirschsprung Art Gallery of Copenhagen. embryology.med.unsw.edu.au /OMIMfind/git/OMIM-142623.htm   (2652 words)

Case Based Pediatrics Chapter Hirschsprung’s disease (also known as congenital megacolon or congenital intestinal aganglionosis) is a disease condition most commonly affecting the rectosigmoid portion of the colon. The severity of Hirschsprung’s disease varies with the length of the involved segment and may be very difficult to diagnose especially in the ultra short segment disease because of the variability of the constipation. Absence of ganglion cells as the cause for uncoordinated peristalsis was correctly identified as the cause for Hirschsprung's disease in infancy in1938 by Robertson and Kernahan. www.hawaii.edu /medicine/pediatrics/pedtext/s09c09.html   (1882 words)

Indian Pediatrics - Editorial   (Site not responding. Last check: 2007-11-05) Hirschsprung’s disease (HSCR) is the main genetic cause of functional intestinal obstruction with an incidence of 1/5000 live births. Hirschsprung’s disease (HSCR, aganglionic megacolon) is the main genetic cause of functional intestinal obstruction with an incidence of 1/5000 live births. A missense mutation of the endothelin-B receptor gene in multigenic Hirschsprung’s disease. www.indianpediatrics.net /nov2004/nov-1115-1123.htm   (3011 words)

(Kapiolani Medical Center for Women and Children - Honolulu, HI) Children's Surgery: Conditions   (Site not responding. Last check: 2007-11-05) Hirschsprung's Disease: Congenital megacolon or congenital intestinal aganglionosis is a condition first recognized by Harald Hirschsprung where a child is born without certain nerves in the intestine (ganglion cells). In young infants with Hirschsprung's Disease, an initial surgery called a colostomy is the safest procedure. Hirschsprung's Disease is one of the most complex pediatric surgical problems in which Dr. Shim has over 30 years of experience. verizonsupersite.com /childrensurgerycom/conditions/view.nhtml?profile=conditions&UID=10017   (391 words)

ABC Sci-Tech - 16/04/02 : Biotech breakthrough as complex gene disease is unravelled Individuals with Hirschsprung lack nerve cells in bowel muscles that push faecal material along the intestine and often suffer from a bloated abdomen, constipation, vomiting and bowel inflammation as a result. In addition to mutations in five genes that cause long-segment Hirschsprung, which affects a long portion of the intestine, they found mutations in three genes that are to blame for short-segment Hirschsprung which affects the rectum and a small portion of the colon. Short-segment Hirschsprung, in which usually only a few centimetres of bowel are affected, is by far the commonest form of the disease, accounting for up to 85 per cent of all cases. www.abc.net.au /news/scitech/2002/04/item20020415094518_1.htm   (436 words)

Page 3   (Site not responding. Last check: 2007-11-05) Hirschsprung's Disease is a motor disorder of the colon thought to be caused by the failure of neural crest cells to migrate to the end of the developing colon early in foetal life. The majority of babies with Hirschsprung's disease are diagnosed in the neonatal period. Hirschsprung's disorder is considered when there is a delay of more than 48 hours in passing the first meconium (first tarry looking bowel motion). www.keea.org.nz /NL15P3.html   (533 words)

Enterocolitis Associated with Hirschsprung's Disease   (Site not responding. Last check: 2007-11-05) Despite the recognition of this process in Harald Hirschsprung's first description of the disease in 1886, little attention was given to this disease process for the next 70 years (1). In their report, they noted that children with Hirschsprung's disease had persistent retention of C difficile beyond 12 months of age, despite the usual loss of this organism in gastrointestinal tracts of normal children. This suggested that patients with Hirschsprung's disease may be more susceptible to infectious agents such as C difficile, and that this may predispose them to a high incidence of infections. pediatric.um-surgery.org /new_070198/new/Library/Hirschsprung/enterocolitis   (5030 words)

Hirschsprung disease, associated syndromes, and genetics: a review -- Amiel and Lyonnet 38 (11): 729 -- Journal of ... Hirschsprung's disease, Ondine's curse, and neuroblastoma - manifestations of neurocristopathy. Hirschsprung disease associated with polydactyly, unilateral renal agenesis, hypertelorism, and congenital deafness: a new autosomal recessive syndrome. Smith-Lemli-Opitz syndrome is caused by mutations in the 7-dehydrocholesterol reductase gene. jmg.bmjjournals.com /cgi/content/full/38/11/729   (5784 words)

Sept 1997 Hirschsprung's Disease is where a portion of the bowel is missing nerves. Hirschsprung's disease is one of the more common conditions treated by pediatric surgeons. The first report of a patient with Hirschsprung's disease was in 1691 by Frederick Ruysch in an autopsy report of a 5-year-old girl who died with unremitting constipation. www.hirschsprungs.info /newsletter/newsletter_sept97.htm   (4195 words)

Hirschsprungs disease - Thus, the specifics of the origins of the disease hirschsprungs disease are still not completely known. RET codes for proteins that help the neural crest cells (which become ganglion cells) move through the digestive tract during the development of the embryo (Sawin). A disease is any abnormal condition of the body or mind that causes discomfort, dysfunction, or distress to the person affected or those in contact with the person. www.medicalgeo.com /Med-Diseases-H---Hi/Hirschsprungs-disease.html   (1656 words)

Hirschsprung's disease - Patient UK Delayed passage of meconium is very important as nearly half of all infants with Hirschsprung's disease do not pass meconium within 36 hours, and nearly half of infants with delayed first passage of meconium have Hirschsprung's disease. In Hirschsprung's disease there is failure of reflex relaxation of the internal anal sphincter in response to inflation of a rectal balloon. Harald Hirschsprung was a Danish paediatrican who was born in 1830 and died in 1916. www.patient.co.uk /showdoc/40001391   (1516 words)

eMedicine - Hirschsprung Disease : Article Excerpt by: Ciro Yoshida, Jr, MD Background: The first report of a patient with Hirschsprung disease (HD) was made in 1691 by Frederick Ruysch, but it was Harald Hirschsprung who published the classic description of congenital megacolon in 1886. Because of the polygenic nature of HD, the penetrance of the condition is variable; it leads to the variable manifestations of the disease. In the US: In the United States, the incidence of Hirschsprung disease is approximately 1 case per 5,000 live births. www.emedicine.com /radio/byname/hirschsprung-disease.htm   (588 words)

(Kapiolani Medical Center for Women and Children - Honolulu, HI) Children's Surgery: Doctor to Doctor   (Site not responding. Last check: 2007-11-05) Hirschsprung’s Disease or aganglionic megacolon occurs in 1 in 5000 births and has a 5:1 male-to-female ratio. It was first described by Dr. Harald Hirschsprung in 1886 but its patho-physiology and treatment waited until 1948 when they were described by Orvar Swenson who also devised the first successful corrective surgery, the Swenson Procedure. In the newborn and in the young infant its first manifestation is the delayed passage of the first meconium stool beyond 24 hours. verizonsupersite.com /childrensurgerycom/doctortodoctor/view.nhtml?profile=doctortodoctor&UID=10017   (572 words)

Hirschsprung's Disease and Allied Disorders, -- HOWARD 49 (5): 740 -- Gut Hirschsprung's Disease and Allied Disorders, -- HOWARD 49 (5): 740 -- Gut Hirschsprung's disease, or congenital intestinal aganglionosis, was named after Harald Hirschsprung, a Danish paediatrician The book reviewed here is the second edition of a text on Hirschsprung's disease first published by Holschneider in 1982. gut.bmjjournals.com /cgi/content/full/49/5/740d   (649 words)

August 1999 Alex died from undiagnosed Hirschsprung's Disease and because of her death, our members decided to start wearing burgundy ribbons in memory of our children who suffer and sometimes die from being misdiagnosed or undiagnosed and/or rare diseases. Hirschsprung's disease was named after "Harald Hirschsprung's" who took care of two boys that couldn’t have proper "bowel movement" ("Hirschsprung's’s Disease"1). Hirschsprung’s is a severe disease to be infected with. www.hirschsprungs.info /newsletter/newsletter_aug99.htm   (5075 words)

Visual Arts The conflict between the so-called Funen painters and historically inspired artists, such as the married couple, Agnes Slott-Møller (1862-1937) and Harald Slott-Møller (1864-1937), was violent and culminated in the ’peasant painter feud’ of 1907. Although Lundstrøm, in his later development as a painter and decorative artist, turned towards a Neo-Classical celebration of the female body related to that represented in sculpture by Kai Nielsen (1882-1924) and Gerhard Henning (1880-1967), he had paved the way for abstract art. During the first third of the century, artists such as Edvard Weie (1879-1943) and Harald Giersing (1881-1927) continued to cultivate a style which valued the pure, colour intensity of the painting above considerations of resemblance to nature. www.um.dk /publikationer/um/english/factsheetdenmark/visualarts/html/chapter01.htm   (2358 words)

Danish Museums Online At the age of 22 in 1891 his first sculpture was acquired by the collector Heinrich Hirschsprung and cast in bronze (it is today exhibited in the garden by the Hirschsprung Collection, Copenhagen). His most important patron was the brewer Carl Jacobsen (Carlsberg), who among other pieces ordered "Thor Driving Across the Arch of the Sky" (in copper it stands on top of the brewery buildings at Carlsberg). Bonnesens last great patron was Harald Plum, who had the huge sculpture group "Thors Fighting the Giants" placed on his private island, Thorø (it now stands by the Haustrup Plast factory on the outskirts of Odense). www.dmol.dk /engelsk/billede_info.asp?genst_id=19250   (318 words)

eMedicine - Hirschsprung Disease : Article by Ciro Yoshida, Jr, MD Oestreich AE: Ultrasound diagnosis of Hirschsprung disease in the infant with distended abdomen. Rosenfield NS, Ablow RC, Markowitz RI, et al: Hirschsprung disease: accuracy of the barium enema examination. Torfs CP: An epidemiological study of Hirschsprung disease in a multiracial California population. www.emedicine.com /radio/topic343.htm   (2099 words)

Hirschsprung's disease and allied disorders. 2nd edition. -- STRINGER 85 (1): 80 -- Archives of Disease in Childhood ISBN 90 5702 263 X. In 1887, Harald Hirschsprung published the first detailed description of congenital megacolon, but it was not until 1948 that This multiauthor text on Hirschsprung's disease contains contributions from an array of world experts. Recent developments are discussed, including the obscure relationship between Hirschsprung's disease and intestinal neuronal adc.bmjjournals.com /cgi/content/full/85/1/80d   (486 words)

E-cards E-cards - with motifs from The Hirschsprung Collection Select af motif in the menu to the right and fill in the cells with your name, recipient and message. You can buy these postcards and many more at The Hirschsprung Collection, by phone +45 35 42 03 36 or at dhs@hirschsprung.dk hirschsprung.dynamicweb.dk /Default.asp?ID=218   (66 words)

Colon Cleanse - The Truth About Colon Cleansing and Detoxification - Foot Detox Machine Hirschsprung’s disease involves an enlargement of the colon, caused by bowel obstruction resulting from an aganglionic section of bowel (the normal enteric nerves are absent) that starts at the anus and progresses upwards. The length of bowel that is affected varies but seldom stretches for more than a foot or so. This disease is named for Harald Hirschsprung, the Danish physician who first described the disease in 1886. www.colon-cleansing.net /hirschsprungs-disease   (777 words)

[No title] Hirschsprung, Harald (1830-1916), Danish physician who in 1887 described congenital dilatation of the colon. Although there were earlier references to this condition, Hirschsprung gave the first complete description, hence the eponym by which it is now known. Hodgkin, Thomas (1798-1866), English physician who was singularly unsuccessful in his practice, so that he gave up medicine altogether. www.lakesidepress.com /pulmonary/books/house/history-m.html   (6262 words)

Abstracts of Lectures and Posters of VIII DSPS Introduction: Hirschsprung's disease has been known since its first description by Harald Hirschsprung in 1887 and aganglionosis was accepted as the sole cause of bowel motility disorders in children. Material and methods: Between 1997 and 2001, 144 children suffering from constipation due to failure to pass stool were investigated and treated at the Department of Pediatric Surgery, University Children's Hospital in Bratislava. Out of remaining forty-eight patients, eighteen (38%) had NID type B, ten (21%) had Hirschsprung's disease combined with NID type B, eight (17%) were diagnosed with hypoganglionosis, five (10%) with heterotopy, five (10%) with hypoganglionosis of the submucosus plexus and in two (4%) immaturity of ganglion cells were found. www.kdch.sk /8dsps/ab_vsetky.html   (13853 words)

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