Her urine sediment at presentation showed no haematuria and her serum complement concentrations and blood smear were normal. [adc.bmj.com]

Patients usually present in the sixth or seventh decade of life, and 5-7% present with carcinoid syndrome. [emedicine.medscape.com]

There are many variations on this way of communication presenting the letters in frequency of use in the English language (.. .. .. .. .. .. .. .. .. .. .. ... [rarediseases.org]

Media [ edit ] In the TV show House, Season 2 episode 17, "All In", the final diagnosis of a 6-year-old boy who presents with bloody diarrhea and ataxia is Erdheim–Chester disease. [en.wikipedia.org]

• Asymptomatic

  The disease usually affects persons in the sixth decade of life and most commonly presents as rectal bleeding, pain, or constipation, while 50% of patients are asymptomatic, with tumors found during routine endoscopy. [emedicine.medscape.com]

• Dyspnea

  […] necropsy with considerable left ventricular hypertrophy (heart weight >750 g) and then reviewed their medical records to determine whether signs and symptoms of right-sided heart failure were isolated or simply preceded signs of left-sided heart failure (dyspnea [ncbi.nlm.nih.gov]

  […] review of 59 case studies by Veyssier-Belot, C et al. in 1996 reported the following symptoms in order of frequency of occurrence: [6] Bone pain Retroperitoneal fibrosis Diabetes insipidus Exophthalmos Xanthomas Neurological signs ( including ataxia ) Dyspnea [en.wikipedia.org]

• Regurgitation

  During subsequent years he developed evidence of severe heart failure, runs of ventricular tachycardia, atrial fibrillation, and a murmur consistent with mitral regurgitation. An intracardiac defibrillator was inserted when he was 63 years old. [ncbi.nlm.nih.gov]

• Nausea

  In 1955, Selzer and colleagues ( 6 ) ( Figure 1 ) described a 62-year-old man with severe hypertension (blood pressure 260/140 mm Hg) hospitalized because of nausea, hepatomegaly, distended neck veins, and clear lung fields. [ncbi.nlm.nih.gov]

• Diarrhea

  Carcinoid diarrhea has been correlated with circulating tachykinins. [14] Symptoms may also relate to overproduction of PPs in the pro-opiomelanocortin family (eg, endorphin, enkephalin). [emedicine.medscape.com]

  Media [ edit ] In the TV show House, Season 2 episode 17, "All In", the final diagnosis of a 6-year-old boy who presents with bloody diarrhea and ataxia is Erdheim–Chester disease. [en.wikipedia.org]

• Heart Disease

  Palliation of carcinoid heart disease. Arch Surg. 1987 Sep. 122(9):1076-7. [Medline]. Bhattacharyya S, Davar J, Dreyfus G, Caplin ME. Carcinoid heart disease. Circulation. 2007 Dec 11. 116(24):2860-5. [Medline]. [emedicine.medscape.com]

  Braunwald's Heart Disease: a Textbook of Cardiovascular Medicine. 7th ed. Philadelphia: Elsevier; 2005. p. 1587. [ Google Scholar ] [ncbi.nlm.nih.gov]

• Hypertension

  In 1955, Selzer and colleagues ( 6 ) ( Figure 1 ) described a 62-year-old man with severe hypertension (blood pressure 260/140 mm Hg) hospitalized because of nausea, hepatomegaly, distended neck veins, and clear lung fields. [ncbi.nlm.nih.gov]

  Hokkaido Perinatal Epidemiology Study Group. 4G/5G variant of plasminogen activator inhibitor-1 gene and severe pregnancy-induced hypertension: subgroup analyses of variants of angiotensinogen and endothelial nitric oxide synthase. J. [en.aig-journal.ru]

• Heart Failure

  […] isolated or simply preceded signs of left-sided heart failure (dyspnea). [ncbi.nlm.nih.gov]

• Tachycardia

  During subsequent years he developed evidence of severe heart failure, runs of ventricular tachycardia, atrial fibrillation, and a murmur consistent with mitral regurgitation. An intracardiac defibrillator was inserted when he was 63 years old. [ncbi.nlm.nih.gov]

• Hepatomegaly

  In 1955, Selzer and colleagues ( 6 ) ( Figure 1 ) described a 62-year-old man with severe hypertension (blood pressure 260/140 mm Hg) hospitalized because of nausea, hepatomegaly, distended neck veins, and clear lung fields. [ncbi.nlm.nih.gov]

• Stroke

  An infarct can be caused by several different conditions such as a blood clot (thrombosis) or stroke. [rarediseases.org]

• Kidney Failure

  Kidney failure Hypopituitarism Liver failure Diagnosis [ edit ] Radiologic osteosclerosis and histology are the main diagnostic features. [en.wikipedia.org]

• Ventricular Hypertrophy

  In 1915, Bernheim published a similar article ( 2 ), this time titling it “Right ventricular stenosis caused by displacement of the septum in eccentric hypertrophy of the left ventricle and resulting venous asystole.” [ncbi.nlm.nih.gov]

[…] failure after about 20 months of treatment. [adc.bmj.com]

In some, there is histiocyte proliferation, and on staining, the section is CD68 + and CD1a-. [ citation needed ] Treatment [ edit ] Current treatment options include: Surgical debulking High-dose corticosteroid therapy Ciclosporin Interferon -α [8] Chemotherapy [en.wikipedia.org]

Complete surgical removal of all tumor tissues, when feasible, is the best treatment. Systemic therapy should be used to control humorally mediated symptoms when the cancer spreads elsewhere. See Treatment and Medication. [emedicine.medscape.com]

Standard Therapies Treatment Treatment should first be aimed at the underlying cause of the disorder. [rarediseases.org]

Journal of Pediatrics, 64(5): 740-745 [ CR ] [ EČ ][ 1 ][ GS ] Gellermann, J., Stefanidis, C.J., Mitsioni, A., Querfeld, U. (2010) Successful treatment of steroid-resistant nephrotic syndrome associated with WT1 mutations. [scindeks.ceon.rs]

Improved prognosis for congenital nephrotic syndrome of the Finnish type in Irish families. Arch Dis Child 1999;80:466-9. Koziell A, Iyer VK, Moghul NE, Ramani P, Taylor CM. Congenital nephrotic syndrome. Pediatr Nephrol ;16:185-9. Ljungberg P. [saripediatri.org]

The vinca alkaloids and anthracyclines have been used most commonly in ECD treatment. [12] Prognosis [ edit ] Erdheim–Chester disease is associated with high mortality rates. [9] [13] In 2005, the survival rate was below 50% at three years from diagnosis [en.wikipedia.org]

Prognosis of carcinoid heart disease: analysis of 200 cases over two decades. Circulation. 2005 Nov 22. 112(21):3320-7. [Medline]. Rorstad O. Prognostic indicators for carcinoid neuroendocrine tumors of the gastrointestinal tract. [emedicine.medscape.com]

Epidemiology and survival in patients with carcinoid disease in The Netherlands. An epidemiological study with 2391 patients. Ann Oncol. 2001 Sep. 12(9):1295-300. [Medline]. [Full Text]. Berge T, Linell F. Carcinoid tumours. [emedicine.medscape.com]

Hokkaido Perinatal Epidemiology Study Group. 4G/5G variant of plasminogen activator inhibitor-1 gene and severe pregnancy-induced hypertension: subgroup analyses of variants of angiotensinogen and endothelial nitric oxide synthase. J. [en.aig-journal.ru]

[…] diagnosis. [14] More recent reports of patients treated with Interferon therapy describe an overall 5-year survival of 68%. [15] Long term survival is currently even more promising, although this impression is not reflected in the recent literature. [16] Epidemiology [en.wikipedia.org]

Reciprocal relationships between insulin resistance and endothelial dysfunction: molecular and pathophysiological mechanisms. Circulation. 2006; 113(15): 1888-904. 3. [en.aig-journal.ru]

We suggest that the precise pharmacological actions of captopril and indometacin are unknown, but we speculate that activity is effected through microvascular pathophysiological features shared by both Finnish-type congenital nephrotic syndrome and diffuse [adc.bmj.com]

Pathophysiology Pathophysiology is closely related to the sites of the primary tumors. When these tumors spread to the liver, patients usually begin to develop malignant carcinoid syndrome. [emedicine.medscape.com]

E-mail: [email protected] Makatsariya Aleksandr Davidovich, MD, corresponding member of the Russian Academy of Sciences, Professor, Head of the Department of Obstetrics and Gynecology, Faculty of Medical and Preventive, I.M. [en.aig-journal.ru]