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Polycythemia Type Chuvash

Chuvash Polycythemia


Presentation

  • The rationales for these trials are discussed and resulting data from the completed trials are presented. Chapter 55: Cytokines Research on cytokines is another area that is exploding in magnitude.[books.google.com]
  • Increased levels of HIFs lead to an upregulation of the body’s hypoxic response, even though there is no hypoxia present. H&O How is Chuvash polycythemia currently managed? VG The optimal management of the condition is not known.[hematologyandoncology.net]
  • Improve your interpretation of presenting symptoms with 38 new topics and 40 new images in the Differential Diagnosis section, and optimize patient care with more than 250 new figures and tables.[books.google.de]
  • Polyakova, one of the authors of this report. 1, 2 The median age at diagnosis of Chuvash polycythemia was 16 years (interquartile range, 6-22 years), and patients typically presented with plethora, headache, and fatigue. 1, 2 We were able to visit or[bloodjournal.org]
Plethora
  • Signs of polycythemia include the following: * Rubor, especially facial rubor * Skin plethora * Hypertension, both systolic and diastolic * Hepatomegaly * Splenomegaly * Conjunctival plethora (engorged vessels in the bulbar conjunctiva) * Ecchymosis *[findmeacure.com]
  • Facial, conjunctival and mucous membrane plethora, and palmar erythema are other nonspecific consequences of erythrocytosis. Spider angiomata suggest that the erythrocytosis is due to the hepatopulmonary syndrome.[oncologynurseadvisor.com]
  • Polyakova, one of the authors of this report. 1, 2 The median age at diagnosis of Chuvash polycythemia was 16 years (interquartile range, 6-22 years), and patients typically presented with plethora, headache, and fatigue. 1, 2 We were able to visit or[bloodjournal.org]
Rigor
  • Nevertheless, that we used adefined set of subjects diagnosed before 1977 as patients, studied the patients and matched cohorts in a narrow time frame, and used both a spouse cohort and a rigorously matched community cohort to test study hypotheses strengthens[bloodjournal.org]
Infertility
  • A32-year-old Punjabi-speaking female (B1, Figure 1B ) from the Toba Tek Singh district of Pakistan attending an infertility clinic was found to have an Hb of 207 g/L and PCV of 0.66 with a normal P50 value.[bloodjournal.org]
Pneumonia
  • Consult new chapters covering Ultrasound, Innate Immunity, Adaptive Immunity, Deposition and Clearance, Ventilator-Associated Pneumonia.[books.google.de]
  • Hypoxic hypoxia is a job in ordinary persons at high heights and is a complication of pneumonia and a scope of other diseases of the respiratory system ( 1,2,15 ).[thepoetrytrust.org]
  • Death from pneumonia, measles, and varicella was 16.0% by age 3 years in community members, reflecting high infant and childhood mortality in Chuvashia in the mid-20th century, and this high early mortality in community members appeared to bias the analysis[bloodjournal.org]
Hypotension
  • KDR (VEGF receptor 2) is the major mediator for the hypotensive effect of VEGF. Hypertension. 2002 ; 39 : 1095 -1100. Henry TD, Rocha-Singh K, Isner JM, et al.[bloodjournal.org]
  • Vitals signs are monitored during and after phlebotomy, and the patient is provided with oral fluids and protected from orthostatic hypotension.[medical-dictionary.thefreedictionary.com]
  • The flow of blood to lung is on the whole really big, and it takes drawn-out hypotension to bring forth important harm. However, acute respiratory distress syndrome can develop when there is a lingering circulatory prostration ( 1,2,15 ).[thepoetrytrust.org]
Cesarean Section
  • Three days later, fetal heart monitoring showed an abnormal pattern requiring an emergency cesarean section. Early institution of heparin is beneficial in the management of Chuvash-type polycythemia in pregnancy as the condition is thrombogenic.[ncbi.nlm.nih.gov]
  • Three days later, fetal heart monitoring showed an abnormal pattern requiring an emergency cesarean section.[reproductivemedicine.com]
Headache
  • At times the phlebotomy is performed in response to a symptom such as headache. Sometimes patients request phlebotomy to ameliorate the ruddy complexion.[hematologyandoncology.net]
  • Common Symptoms: pruritus, headache, fatigue, difficulty thinking or sleeping, dizziness, hyperuricemia, and bleeding or thrombotic complications.[emdocs.net]
  • He presented with a 1-year history of epigastric discomfort, pain in the left hypochondrium, easy fatigability, intermittent headache and dizziness.[sjmms.net]
  • This may cause sluggishness, irritability, headache, dizziness, fainting, disturbances of sensation in the hands and feet, and a feeling of fullness in the head. There may be episodes of acute pain as spontaneous clots occur in the blood vessels.[medical-dictionary.thefreedictionary.com]
  • Primary polycythemia symptoms, when they occur, include itching, especially after exposure to warm water, joint pain, headache, fatigue, and an inability to concentrate.[wisegeek.com]
Peripheral Neuropathy
  • A Bengali-speaking family from Bangladesh ( Figure 1A ) was investigated following the identification of the propositus (A1) who presented at the age of 21 years with peripheral neuropathy. A computed tomographic (CT) scan of his head was normal.[bloodjournal.org]
  • XL019 was terminated after all patients developed central and/or peripheral neuropathy in clinical phase 1 [ 50 ].[link.springer.com]

Workup

  • Renal angiomyolipoma can be found incidentally during workup for suspected renal disease and is one of the few lesions which can be specifically diagnosed by radiological findings.[jmedicalcasereports.biomedcentral.com]
Hemoglobin Increased
  • increase of 4% for each 1000-m increase in altitude), congenital methemoglobinemia, and other high–oxygen affinity hemoglobinopathies stimulating increased Epo production.[findmeacure.com]

Treatment

  • The main goal of treatment is to prevent thrombosis and minimize the complications of therapy. A 20-year-old, Asian woman with her first pregnancy was admitted to the hospital at 16 weeks' gestation.[ncbi.nlm.nih.gov]
  • There are simply not enough patients to make the development of a targeted treatment worthwhile for pharmaceutical companies," he added. The study results were published earlier this week in the journal Nature Medicine.[news-medical.net]
  • The book is a testament to the editorial panel's creed that innovative, comprehensive, multidisciplinary treatment of cancer patients must be grounded in a fundamental understanding of cancer biology.[books.google.com]
  • The main goal of treatment is to prevent thrombosis and minimize the complications of therapy. CASE: A 20-year-old, Asian woman with her first pregnancy was admitted to the hospital at 16 weeks' gestation.[reproductivemedicine.com]
  • Demographic characteristics and causes of death of patients with Chuvash polycythemia and matched cohorts Treatment of patients with Chuvash polycythemia Of the 96 patients with Chuvash polycythemia ( Table 1 ), we were able to obtain information on treatment[bloodjournal.org]

Prognosis

  • For example, untreated, polycythemia vera (PV) was initially thought to have a poor prognosis with a life expectancy of one to two years from the time of diagnosis.[rxlist.com]
  • Prognosis and treatment of polycythemia vera (S. Schrier, Ed.). Retrieved April 5, 2017, from Tefferi, A., & Barbui, T. (2017). Polycythemia vera and essential thrombocythemia: 2017 update on diagnosis, risk-stratification, and management.[emdocs.net]
  • Survival and prognosis among 1545 patients with contemporary polycythemia vera: an international study. Leukemia. 2013 Sep. 27(9):1874-81. [Medline]. [Full Text]. Tefferi A, Vardiman JW.[emedicine.medscape.com]

Etiology

  • In this new 8th edition an outstanding editorial team from world-renowned medical centers continues to hone the leading edge forged in previous editions, with timely information on biology, immunology, etiology, epidemiology, prevention, screening, pathology[books.google.com]
  • There are two distinct forms of the disease: Primary polycythemia (polycythemia vera) is a myeloproliferative disorder of unknown etiology.[medical-dictionary.thefreedictionary.com]
  • […] phases in this disease i) Initial polycythemic phase – Increase in red cell mass ii) Post polycythemic / spent phase- All cytopenias with extramedullary erythropoiesis Epidemiology - Incidence – 8-12 cases / 1 million population - Mean age – 60 years Etiology[sites.google.com]
  • It was suggested that Chuvash polycythemia represents a secondary form of familial and congenital erythrocytosis of as yet unknown etiology.[sjmms.net]
  • […] dysfunction, the decrease in cerebral blood flow seen in newborns with polycythemia is a physiologic response and does not appear to cause cerebral ischemia. [6] Epidemiology The incidence, morbidity, and mortality of polycythemia depends on the underlying etiology[emedicine.medscape.com]

Epidemiology

  • In this new 8th edition an outstanding editorial team from world-renowned medical centers continues to hone the leading edge forged in previous editions, with timely information on biology, immunology, etiology, epidemiology, prevention, screening, pathology[books.google.com]
  • The epidemiology specifically of polycythemia vera has been studied extensively and is reviewed below.[emedicine.medscape.com]
  • There are two phases in this disease i) Initial polycythemic phase – Increase in red cell mass ii) Post polycythemic / spent phase- All cytopenias with extramedullary erythropoiesis Epidemiology - Incidence – 8-12 cases / 1 million population - Mean age[sites.google.com]
  • Limitations to our study include that: (1) data on mortality were obtained retrospectively from a variety of sources; (2) historical findings of the living participants were subject to recall bias; and (3) the sample size is rather small for an epidemiologic[bloodjournal.org]
Sex distribution
Age distribution

Pathophysiology

  • In keeping with the existence of cross-regulatory interactions between Fe homeostasis and the pathophysiology of these diseases, further investigations on the mechanisms that provide cellular and systemic adaptation to tissue Fe overload are instrumental[books.google.de]
  • Pathophysiologic ClassificationRELATIVE Reduced plasma volume (hemoconcentration)ABSOLUTEPrimary (Erythropoietin normal or low) Polycythemia vera Inherited erythropoietin receptor mutationsSecondary (High erythropoietin) Compensatory Lung disease High-altitude[slideshare.net]
  • The molecular pathophysiology underlying the Chuvash polycythaemia has recently been elucidated and is ascribed to dysregulation of oxygen homeostasis caused by mutations in the von Hippel-Lindau ( VHL ) gene, a key player in the hypoxia-sensing pathway[onlinelibrary.wiley.com]
  • It also has activity against cells expressing JAK2(V617F), a mutated form of the janus kinase 2 (JAK2) enzyme that is implicated in the pathophysiology of many myeloproliferative diseases, including polycythaemia vera.[diki.pl]
  • Until recently, the pathophysiology of polycythemia vera was unclear.[findmeacure.com]

Prevention

  • The main goal of treatment is to prevent thrombosis and minimize the complications of therapy. A 20-year-old, Asian woman with her first pregnancy was admitted to the hospital at 16 weeks' gestation.[ncbi.nlm.nih.gov]
  • Rapidly find the answers you need with separate sections on diseases and disorders, differential diagnosis, clinical algorithms, laboratory results, and clinical preventive services, plus an at-a-glance format that uses cross-references, outlines, bullets[books.google.de]
  • The main goal of treatment is to prevent thrombosis and minimize the complications of therapy. CASE: A 20-year-old, Asian woman with her first pregnancy was admitted to the hospital at 16 weeks' gestation.[reproductivemedicine.com]
  • In this new 8th edition an outstanding editorial team from world-renowned medical centers continues to hone the leading edge forged in previous editions, with timely information on biology, immunology, etiology, epidemiology, prevention, screening, pathology[books.google.com]
  • Further research into this area might shed light into the associated coagulation pathways and present new targets to prevent thromboses in at-risk patients or to develop new anticoagulants based on the pathways that are observed.[hematologyandoncology.net]

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