5300004: Hemoglobin Bart's hydrops syndrome (disorder)

• SNOMED CT Concept\Clinical finding\...
• \Evaluation finding\Measurement finding\Finding of substance level\Protein level - finding\Haemoglobin finding\Haemoglobin level outside reference range\Haemoglobin low\Congenital anaemia\Haemoglobin Bart's hydrops syndrome
• \Evaluation finding\Measurement finding\Finding of substance level\Protein level - finding\Haemoglobin finding\Haemoglobin level outside reference range\Haemoglobin low\Anaemia due to disturbance of haemoglobin synthesis\Thalassaemia\Alpha thalassaemia\Haemoglobin Bart's hydrops syndrome
• \Evaluation finding\Measurement finding\Measurement finding outside reference range\Abnormal blood cell count\Red blood cell count outside reference range\RBC count low\Congenital anaemia\Haemoglobin Bart's hydrops syndrome
• \Evaluation finding\Measurement finding\Measurement finding outside reference range\Abnormal blood cell count\Cytopenia\RBC count low\Congenital anaemia\Haemoglobin Bart's hydrops syndrome
• \Evaluation finding\Measurement finding\Measurement finding outside reference range\Measurement finding below reference range\Haemoglobin low\Congenital anaemia\Haemoglobin Bart's hydrops syndrome
• \Evaluation finding\Measurement finding\Measurement finding outside reference range\Measurement finding below reference range\Haemoglobin low\Anaemia due to disturbance of haemoglobin synthesis\Thalassaemia\Alpha thalassaemia\Haemoglobin Bart's hydrops syndrome
• \Evaluation finding\Measurement finding\Measurement finding outside reference range\Measurement finding below reference range\Cytopenia\RBC count low\Congenital anaemia\Haemoglobin Bart's hydrops syndrome
• \Evaluation finding\Measurement finding\Measurement finding outside reference range\Haemoglobin level outside reference range\Haemoglobin low\Congenital anaemia\Haemoglobin Bart's hydrops syndrome
• \Evaluation finding\Measurement finding\Measurement finding outside reference range\Haemoglobin level outside reference range\Haemoglobin low\Anaemia due to disturbance of haemoglobin synthesis\Thalassaemia\Alpha thalassaemia\Haemoglobin Bart's hydrops syndrome
• \Evaluation finding\Measurement finding\Red blood cell count - finding\Red blood cell count outside reference range\RBC count low\Congenital anaemia\Haemoglobin Bart's hydrops syndrome
• \Evaluation finding\Haematopoietic system finding\Haematology test outside reference range\Haemoglobin level outside reference range\Haemoglobin low\Congenital anaemia\Haemoglobin Bart's hydrops syndrome
• \Evaluation finding\Haematopoietic system finding\Haematology test outside reference range\Haemoglobin level outside reference range\Haemoglobin low\Anaemia due to disturbance of haemoglobin synthesis\Thalassaemia\Alpha thalassaemia\Haemoglobin Bart's hydrops syndrome
• \Evaluation finding\Haematopoietic system finding\Haemoglobin finding\Haemoglobin level outside reference range\Haemoglobin low\Congenital anaemia\Haemoglobin Bart's hydrops syndrome
• \Evaluation finding\Haematopoietic system finding\Haemoglobin finding\Haemoglobin level outside reference range\Haemoglobin low\Anaemia due to disturbance of haemoglobin synthesis\Thalassaemia\Alpha thalassaemia\Haemoglobin Bart's hydrops syndrome
• \Finding of blood, lymphatics and immune system\Blood disease\...
• \Hereditary disorder of cellular element of blood\Hereditary red blood cell disorder\Hereditary haemoglobinopathy\Thalassaemia\Alpha thalassaemia\Haemoglobin Bart's hydrops syndrome
• \Anaemia\Fetal anaemia\Haemoglobin Bart's hydrops syndrome
• \Anaemia\Congenital anaemia\Haemoglobin Bart's hydrops syndrome
• \Anaemia\Anaemia due to disturbance of haemoglobin synthesis\Thalassaemia\Alpha thalassaemia\Haemoglobin Bart's hydrops syndrome
• \Red blood cell disorder\Hereditary red blood cell disorder\Hereditary haemoglobinopathy\Thalassaemia\Alpha thalassaemia\Haemoglobin Bart's hydrops syndrome
• \Red blood cell disorder\Haemoglobinopathy\Hereditary haemoglobinopathy\Thalassaemia\Alpha thalassaemia\Haemoglobin Bart's hydrops syndrome
• \Oedema\Disorder characterised by oedema\Hydrops fetalis\Haemoglobin Bart's hydrops syndrome
• \Fetal finding\Fetal disorder\...
• \Fetal anaemia\Haemoglobin Bart's hydrops syndrome
• \Hydrops fetalis\Haemoglobin Bart's hydrops syndrome
• \Fetus with hereditary disease\Haemoglobin Bart's hydrops syndrome
• \Disease\Genetic disease\Hereditary disease\Hereditary disorder by system\Hereditary disorder of cellular element of blood\Hereditary red blood cell disorder\Hereditary haemoglobinopathy\Thalassaemia\Alpha thalassaemia\Haemoglobin Bart's hydrops syndrome
• \Disease\Genetic disease\Hereditary disease\Fetus with hereditary disease\Haemoglobin Bart's hydrops syndrome
• \Disease\Genetic disease\Hereditary disease\Autosomal hereditary disorder\Autosomal recessive hereditary disorder\Haemoglobin Bart's hydrops syndrome
• \Disease\Disorder of fetus or newborn\Fetal disorder\Fetal anaemia\Haemoglobin Bart's hydrops syndrome
• \Disease\Disorder of fetus or newborn\Fetal disorder\Hydrops fetalis\Haemoglobin Bart's hydrops syndrome
• \Disease\Disorder of fetus or newborn\Fetal disorder\Fetus with hereditary disease\Haemoglobin Bart's hydrops syndrome
• \Disease\Blood disease\Hereditary disorder of cellular element of blood\Hereditary red blood cell disorder\Hereditary haemoglobinopathy\Thalassaemia\Alpha thalassaemia\Haemoglobin Bart's hydrops syndrome
• \Disease\Blood disease\Anaemia\Fetal anaemia\Haemoglobin Bart's hydrops syndrome
• \Disease\Blood disease\Anaemia\Congenital anaemia\Haemoglobin Bart's hydrops syndrome
• \Disease\Blood disease\Anaemia\Anaemia due to disturbance of haemoglobin synthesis\Thalassaemia\Alpha thalassaemia\Haemoglobin Bart's hydrops syndrome
• \Disease\Blood disease\Red blood cell disorder\Hereditary red blood cell disorder\Hereditary haemoglobinopathy\Thalassaemia\Alpha thalassaemia\Haemoglobin Bart's hydrops syndrome
• \Disease\Blood disease\Red blood cell disorder\Haemoglobinopathy\Hereditary haemoglobinopathy\Thalassaemia\Alpha thalassaemia\Haemoglobin Bart's hydrops syndrome
• \Disease\Disorder of body system\Hereditary disorder by system\Hereditary disorder of cellular element of blood\Hereditary red blood cell disorder\Hereditary haemoglobinopathy\Thalassaemia\Alpha thalassaemia\Haemoglobin Bart's hydrops syndrome
• \Disease\Disorder of body system\Red blood cell disorder\Hereditary red blood cell disorder\Hereditary haemoglobinopathy\Thalassaemia\Alpha thalassaemia\Haemoglobin Bart's hydrops syndrome
• \Disease\Disorder of body system\Red blood cell disorder\Haemoglobinopathy\Hereditary haemoglobinopathy\Thalassaemia\Alpha thalassaemia\Haemoglobin Bart's hydrops syndrome
• \Disease\Disorder characterised by oedema\Hydrops fetalis\Haemoglobin Bart's hydrops syndrome
• \Disease\Congenital disease\Hereditary haemoglobinopathy\Thalassaemia\Alpha thalassaemia\Haemoglobin Bart's hydrops syndrome
• \Disease\Congenital disease\Congenital anaemia\Haemoglobin Bart's hydrops syndrome

Status: current, Primitive. Date: 31-Jan 2002. Module: SNOMED CT core

Descriptions:

Id	Description	Lang	Type	Status	Case?	Module
2841557010	Haemoglobin Bart hydrops syndrome	en	Synonym	Active	Initial character case insensitive	SNOMED CT core
2841558017	Hemoglobin Bart hydrops syndrome	en	Synonym	Active	Initial character case insensitive	SNOMED CT core
496516017	Alpha thalassemia major	en	Synonym	Active	Case insensitive	SNOMED CT core
496517014	Hemoglobin Barts hydrops	en	Synonym	Active	Initial character case insensitive	SNOMED CT core
496518016	Haemoglobin Bart's hydrops syndrome	en	Synonym	Active	Initial character case insensitive	SNOMED CT core
496519012	Alpha thalassaemia major	en	Synonym	Active	Case insensitive	SNOMED CT core
496520018	Haemoglobin Bart's disease	en	Synonym	Active	Initial character case insensitive	SNOMED CT core
496521019	Haemoglobin Barts hydrops	en	Synonym	Active	Initial character case insensitive	SNOMED CT core
791071016	Hemoglobin Bart's hydrops syndrome (disorder)	en	Fully specified name	Active	Initial character case insensitive	SNOMED CT core
9888011	Hemoglobin Bart's hydrops syndrome	en	Synonym	Active	Initial character case insensitive	SNOMED CT core
9889015	Hemoglobin Bart's disease	en	Synonym	Active	Initial character case insensitive	SNOMED CT core
4590277018	A severe form of alpha-thalassemia that is mostly lethal, and associated with severe long-term outcome and lifelong transfusions in survivors. It is characterized by fetal onset of generalized edema, pleural and pericardial effusions, and severe hypochromic anemia. Caused by deletion or inactivation of all four alpha-globin alleles leading to a severe deficiency in alpha-globin chains of Hb, and to the production of gamma-4 tetramers (Hb Bart's) during fetal life, and beta-4 tetramers (HbH) postnatally. Hb Bart's and HbH have increased oxygen affinity resulting in ineffective tissue oxygen delivery. The disease is mostly the result of combined, biallelic deletions in the HBA1 and HBA2 genes (16p13.3). The pattern of inheritance is autosomal recessive.	en	Definition	Active	Case sensitive	SNOMED CT core
4590278011	A severe form of alpha-thalassaemia that is mostly lethal, and associated with severe long-term outcome and lifelong transfusions in survivors. It is characterized by fetal onset of generalised oedema, pleural and pericardial effusions, and severe hypochromic anaemia. Caused by deletion or inactivation of all four alpha-globin alleles leading to a severe deficiency in alpha-globin chains of Hb, and to the production of gamma-4 tetramers (Hb Bart's) during fetal life, and beta-4 tetramers (HbH) postnatally. Hb Bart's and HbH have increased oxygen affinity resulting in ineffective tissue oxygen delivery. The disease is mostly the result of combined, biallelic deletions in the HBA1 and HBA2 genes (16p13.3). The pattern of inheritance is autosomal recessive.	en	Definition	Active	Case sensitive	SNOMED CT core

Expanded Value Set

Reference Sets

Australian emergency department reference set

Clinical finding foundation reference set

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Problem/Diagnosis reference set

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