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718614004: Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)


Status: current, Primitive. Date: 31-Jan 2017. Module: SNOMED CT core

Descriptions:

Id Description Lang Type Status Case? Module
3312944013 Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder) en Fully specified name Active Case insensitive SNOMED CT core
3312945014 Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome en Synonym Active Case insensitive SNOMED CT core
3312946010 TEMPI syndrome en Synonym Active Case sensitive SNOMED CT core
3312947018 A rare multi-systemic disease with less than 10 cases described in literature. Manifests in mid-adulthood with the development of telangiectasia mostly on the face, trunk and arms, as well as with erythrocytosis that may cause a red facies and occasionally, headaches. The increased serum erythropoietin levels precede the intrapulmonary shunting. The intrapulmonary shunts cause hypoxia which slowly progresses until the person needs continuous supplemental oxygen. Blood clots, probably due to erythrocytosis, and bleeding in the brain have also been reported in some affected individuals. Monoclonal gammopathy and perinephric fluid collections are usually found incidentally and do not seem to cause any complications. The syndrome has a slow and regular progression. The cause of TEMPI syndrome is currently unknown. The abnormal plasma-cell clone and/or the monoclonal gammopathy are suggested to be triggers of the disease. en Definition Active Case sensitive SNOMED CT core


0 descendants.

Expanded Value Set


Outbound Relationships Type Target Active Characteristic Refinability Group Values
TEMPI syndrome Pathological process Abnormal immune process true Inferred relationship Some 8
TEMPI syndrome Is a Telangiectasia of skin true Inferred relationship Some
TEMPI syndrome Is a Monoclonal gammopathy true Inferred relationship Some
TEMPI syndrome Is a Erythrocytosis true Inferred relationship Some
TEMPI syndrome Is a Telangiectasia disorder false Inferred relationship Some
TEMPI syndrome Is a Multisystem disorder false Inferred relationship Some
TEMPI syndrome Finding site Erythrocyte true Inferred relationship Some 4
TEMPI syndrome Has definitional manifestation Immune system finding false Inferred relationship Some
TEMPI syndrome Has definitional manifestation Increased immunoglobulin false Inferred relationship Some
TEMPI syndrome Has definitional manifestation RBC count raised false Inferred relationship Some
TEMPI syndrome Has definitional manifestation Serum gamma globulin raised false Inferred relationship Some
TEMPI syndrome Has definitional manifestation Monoclonal band present false Inferred relationship Some
TEMPI syndrome Associated morphology Telangiectasis true Inferred relationship Some 7
TEMPI syndrome Finding site Microscopic skin vascular structure true Inferred relationship Some 7
TEMPI syndrome Has interpretation Above reference range true Inferred relationship Some 1
TEMPI syndrome Interprets Serum globulin measurement true Inferred relationship Some 1
TEMPI syndrome Interprets Paraprotein measurement true Inferred relationship Some 5
TEMPI syndrome Has interpretation Present true Inferred relationship Some 5
TEMPI syndrome Has interpretation Above reference range true Inferred relationship Some 3
TEMPI syndrome Interprets Haematology procedure true Inferred relationship Some 6
TEMPI syndrome Interprets Immunoglobulin measurement true Inferred relationship Some 3
TEMPI syndrome Interprets Red blood cell count true Inferred relationship Some 2
TEMPI syndrome Has interpretation Above reference range true Inferred relationship Some 2

Inbound Relationships Type Active Source Characteristic Refinability Group

Reference Sets

Australian emergency department reference set

Clinical finding foundation reference set

Cardiovascular finding reference set

Problem/Diagnosis reference set

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