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

• SNOMED CT Concept\Clinical finding (finding)\...
• \Skin AND/OR mucosa finding (finding)\Skin or mucosa lesion\Lesion of skin and/or skin-associated mucous membrane\Skin lesion\Vascular lesion of skin\Telangiectasia of skin (disorder)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Skin AND/OR mucosa finding (finding)\Skin finding\Disorder of skin (disorder)\Skin lesion\Vascular lesion of skin\Telangiectasia of skin (disorder)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Skin AND/OR mucosa finding (finding)\Skin finding\Disorder of skin (disorder)\Vascular disease of the skin\Vascular lesion of skin\Telangiectasia of skin (disorder)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Finding of blood, lymphatics and immune system\Disorder of cellular component of blood (disorder)\Red blood cell disorder\Erythrocytosis\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \General finding of soft tissue\Skin finding\Disorder of skin (disorder)\Skin lesion\Vascular lesion of skin\Telangiectasia of skin (disorder)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \General finding of soft tissue\Skin finding\Disorder of skin (disorder)\Vascular disease of the skin\Vascular lesion of skin\Telangiectasia of skin (disorder)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \General finding of soft tissue\Blood vessel finding\Vascular disorder\Telangiectasia disorder\Telangiectasia of skin (disorder)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \General finding of soft tissue\Blood vessel finding\Vascular disorder\Vascular disease of the skin\Vascular lesion of skin\Telangiectasia of skin (disorder)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \General finding of soft tissue\Disorder of soft tissue\Soft tissue lesion\Telangiectasia disorder\Telangiectasia of skin (disorder)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \General finding of soft tissue\Disorder of soft tissue\Soft tissue lesion\Skin lesion\Vascular lesion of skin\Telangiectasia of skin (disorder)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \General finding of soft tissue\Disorder of soft tissue\Disorder of skin and/or subcutaneous tissue (disorder)\Disorder of skin (disorder)\Skin lesion\Vascular lesion of skin\Telangiectasia of skin (disorder)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \General finding of soft tissue\Disorder of soft tissue\Disorder of skin and/or subcutaneous tissue (disorder)\Disorder of skin (disorder)\Vascular disease of the skin\Vascular lesion of skin\Telangiectasia of skin (disorder)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \General finding of soft tissue\Disorder of soft tissue\Vascular disorder\Telangiectasia disorder\Telangiectasia of skin (disorder)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \General finding of soft tissue\Disorder of soft tissue\Vascular disorder\Vascular disease of the skin\Vascular lesion of skin\Telangiectasia of skin (disorder)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Procedure related finding\Evaluation finding\...
• \Finding of cellular component of blood (finding)\Red blood cell finding\Erythrocytosis\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Measurement finding\Finding of substance level (finding)\Finding of blood substance level\Blood substance level above reference range\Gammopathy\Disorder of increased production of immunoglobulin protein (disorder)\Monoclonal gammopathy (clinical)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Measurement finding\Finding of substance level (finding)\Finding of blood substance level\Finding of protein in serum or plasma\Gammopathy\Disorder of increased production of immunoglobulin protein (disorder)\Monoclonal gammopathy (clinical)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Measurement finding\Finding of substance level (finding)\Protein level - finding\Finding of paraprotein measurement (finding)\Paraprotein detected (finding)\Monoclonal gammopathy (clinical)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Measurement finding\Finding of substance level (finding)\Protein level - finding\Finding of protein in serum or plasma\Gammopathy\Disorder of increased production of immunoglobulin protein (disorder)\Monoclonal gammopathy (clinical)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Measurement finding\Finding of substance level (finding)\Protein level - finding\Globulin level - finding\Globulin level outside reference range (finding)\Globulin above reference range\Gammopathy\Disorder of increased production of immunoglobulin protein (disorder)\Monoclonal gammopathy (clinical)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Measurement finding\Finding of substance level (finding)\Protein level - finding\Immunoglobulin level - finding\Immunoglobulins outside reference range\Immunoglobulin above reference range (finding)\Disorder of increased production of immunoglobulin protein (disorder)\Monoclonal gammopathy (clinical)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Measurement finding\Measurement finding outside reference range\Blood cell count outside reference range\Red blood cell count outside reference range (finding)\Red blood cell count above reference range (finding)\Erythrocytosis\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Measurement finding\Measurement finding outside reference range\Measurement finding above reference range\Blood substance level above reference range\Gammopathy\Disorder of increased production of immunoglobulin protein (disorder)\Monoclonal gammopathy (clinical)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Measurement finding\Measurement finding outside reference range\Measurement finding above reference range\Red blood cell count above reference range (finding)\Erythrocytosis\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Measurement finding\Measurement finding outside reference range\Measurement finding above reference range\Immunoglobulin above reference range (finding)\Disorder of increased production of immunoglobulin protein (disorder)\Monoclonal gammopathy (clinical)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Measurement finding\Measurement finding outside reference range\Measurement finding above reference range\Globulin above reference range\Gammopathy\Disorder of increased production of immunoglobulin protein (disorder)\Monoclonal gammopathy (clinical)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Measurement finding\Measurement finding outside reference range\Immunoglobulins outside reference range\Immunoglobulin above reference range (finding)\Disorder of increased production of immunoglobulin protein (disorder)\Monoclonal gammopathy (clinical)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Measurement finding\Measurement finding outside reference range\Globulin level outside reference range (finding)\Globulin above reference range\Gammopathy\Disorder of increased production of immunoglobulin protein (disorder)\Monoclonal gammopathy (clinical)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Measurement finding\Detected by measurement (finding)\Paraprotein detected (finding)\Monoclonal gammopathy (clinical)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Measurement finding\Red blood cell count - finding\Red blood cell count outside reference range (finding)\Red blood cell count above reference range (finding)\Erythrocytosis\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Hematopoietic system finding\Red blood cell finding\Erythrocytosis\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Integumentary system finding\Disorder of integument\Disorder of skin and/or subcutaneous tissue (disorder)\Disorder of skin (disorder)\Skin lesion\Vascular lesion of skin\Telangiectasia of skin (disorder)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Integumentary system finding\Disorder of integument\Disorder of skin and/or subcutaneous tissue (disorder)\Disorder of skin (disorder)\Vascular disease of the skin\Vascular lesion of skin\Telangiectasia of skin (disorder)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Integumentary system finding\Skin finding\Disorder of skin (disorder)\Skin lesion\Vascular lesion of skin\Telangiectasia of skin (disorder)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Integumentary system finding\Skin finding\Disorder of skin (disorder)\Vascular disease of the skin\Vascular lesion of skin\Telangiectasia of skin (disorder)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Cardiovascular finding\Disorder of cardiovascular system (disorder)\Vascular disorder\Telangiectasia disorder\Telangiectasia of skin (disorder)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Cardiovascular finding\Disorder of cardiovascular system (disorder)\Vascular disorder\Vascular disease of the skin\Vascular lesion of skin\Telangiectasia of skin (disorder)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Cardiovascular finding\Blood vessel finding\Vascular disorder\Telangiectasia disorder\Telangiectasia of skin (disorder)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Cardiovascular finding\Blood vessel finding\Vascular disorder\Vascular disease of the skin\Vascular lesion of skin\Telangiectasia of skin (disorder)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Disease\Disorder of immune function (disorder)\Gammopathy\Disorder of increased production of immunoglobulin protein (disorder)\Monoclonal gammopathy (clinical)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Disease\Disorder of cellular component of blood (disorder)\Red blood cell disorder\Erythrocytosis\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Disease\Disorder of body system\Skin or mucosa lesion\Lesion of skin and/or skin-associated mucous membrane\Skin lesion\Vascular lesion of skin\Telangiectasia of skin (disorder)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Disease\Disorder of body system\Disorder of integument\Disorder of skin and/or subcutaneous tissue (disorder)\Disorder of skin (disorder)\Skin lesion\Vascular lesion of skin\Telangiectasia of skin (disorder)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Disease\Disorder of body system\Disorder of integument\Disorder of skin and/or subcutaneous tissue (disorder)\Disorder of skin (disorder)\Vascular disease of the skin\Vascular lesion of skin\Telangiectasia of skin (disorder)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Disease\Disorder of body system\Disorder of cardiovascular system (disorder)\Vascular disorder\Telangiectasia disorder\Telangiectasia of skin (disorder)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Disease\Disorder of body system\Disorder of cardiovascular system (disorder)\Vascular disorder\Vascular disease of the skin\Vascular lesion of skin\Telangiectasia of skin (disorder)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Disease\Disorder of body system\Red blood cell disorder\Erythrocytosis\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Disease\Disorder of soft tissue\Soft tissue lesion\Telangiectasia disorder\Telangiectasia of skin (disorder)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Disease\Disorder of soft tissue\Soft tissue lesion\Skin lesion\Vascular lesion of skin\Telangiectasia of skin (disorder)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Disease\Disorder of soft tissue\Disorder of skin and/or subcutaneous tissue (disorder)\Disorder of skin (disorder)\Skin lesion\Vascular lesion of skin\Telangiectasia of skin (disorder)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Disease\Disorder of soft tissue\Disorder of skin and/or subcutaneous tissue (disorder)\Disorder of skin (disorder)\Vascular disease of the skin\Vascular lesion of skin\Telangiectasia of skin (disorder)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Disease\Disorder of soft tissue\Vascular disorder\Telangiectasia disorder\Telangiectasia of skin (disorder)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)
• \Disease\Disorder of soft tissue\Vascular disorder\Vascular disease of the skin\Vascular lesion of skin\Telangiectasia of skin (disorder)\Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome (disorder)

Status: current, Not sufficiently defined by necessary conditions definition status (core metadata concept). 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	Entire term case insensitive (core metadata concept)	SNOMED CT core
3312945014	Telangiectasia, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunting syndrome	en	Synonym (core metadata concept)	Active	Entire term case insensitive (core metadata concept)	SNOMED CT core
3312946010	TEMPI syndrome	en	Synonym (core metadata concept)	Active	Entire term case sensitive (core metadata concept)	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	Entire term case sensitive (core metadata concept)	SNOMED CT core

Expanded Value Set

This concept is not in any reference sets

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