52618001: Structure of pars distalis of pituitary (body structure)

SNOMED CT Concept\Body structure (body structure)\Anatomical or acquired body structure (body structure)\Anatomical structure\...

\Anatomical structure which has as its parts a heterogeneous collection of organs, organ parts, cells, cell parts or body part subdivisions that are adjacent to, or continuous with one another; does not constitute a cell part, cell, tissue, organ, or body part subdivision.\Structure of anatomical compartment (body structure)\Intracranial structure (body structure)\Brain structure\Brain part\Brain region\Supratentorial brain\Supratentorial brain part\The cerebrum is the regional structure of the brain, which is the adult equivalent of the forebrain or prosencephalon. It is constituted by the structural derivatives of the telencephalon and diencephalon including the cerebral hemispheres, epithalamus, thalamus, hypothalamus, lateral ventricles and third ventricle. This definition is harmonious with the Federation of Association of Anatomist Second Edition (2019) Part V Terminologia Anatomica.\Structure of diencephalon\Diencephalon part\Pituitary and/or pineal structures (body structure)\Pituitary structure\Pituitary part (body structure)\Adenohypophysis structure\Structure of distal part of pituitary

\Gland structure\Endocrine gland structure (body structure)\...

\Pituitary and/or pineal structures (body structure)\Pituitary structure\Pituitary part (body structure)\Adenohypophysis structure\Structure of distal part of pituitary

\Endocrine gland part (body structure)\Pituitary part (body structure)\Adenohypophysis structure\Structure of distal part of pituitary

\Body region structure\Body part structure\Upper body structure\Upper body part structure\Structure of head and/or neck (body structure)\Head structure\Head part\Intracranial structure (body structure)\Brain structure\Brain part\Brain region\Supratentorial brain\Supratentorial brain part\The cerebrum is the regional structure of the brain, which is the adult equivalent of the forebrain or prosencephalon. It is constituted by the structural derivatives of the telencephalon and diencephalon including the cerebral hemispheres, epithalamus, thalamus, hypothalamus, lateral ventricles and third ventricle. This definition is harmonious with the Federation of Association of Anatomist Second Edition (2019) Part V Terminologia Anatomica.\Structure of diencephalon\Diencephalon part\Pituitary and/or pineal structures (body structure)\Pituitary structure\Pituitary part (body structure)\Adenohypophysis structure\Structure of distal part of pituitary

Status: current, Not sufficiently defined by necessary conditions definition status (core metadata concept). Date: 31-Jan 2002. Module: SNOMED CT core

Descriptions:

Expanded Value Set

Outbound Relationships	Type	Target	Active	Characteristic	Refinability	Group	Values
Structure of distal part of pituitary	Is a	Pituitary part (body structure)	false	Inferred relationship	Some
Structure of distal part of pituitary	partie de	Entire pituitary gland	false	Additional relationship	Some
Structure of distal part of pituitary	Is a	Adenohypophysis structure	true	Inferred relationship	Some

Inbound Relationships	Type	Active	Source	Characteristic	Refinability	Group
Gonadotroph hyperplasia	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	1
Lactotroph hyperplasia	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	1
Simpson-Golabi-Behmel syndrome (disorder)	Finding site	False	Structure of distal part of pituitary	Inferred relationship	Some
Proopiomelanocortin deficiency causes severe obesity beginning at an early age. Affected individuals also have low levels of adrenocorticotropic hormone (ACTH) and tend to have red hair and pale skin.	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	2
Partial growth hormone deficiency	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	1
X-linked intellectual disability-acromegaly-hyperactivity syndrome is characterized by severe intellectual deficit, acromegaly and hyperactivity. The syndrome has been described in two half-brothers. Dysarthria, aggressive behavior, a characteristic facies (an acromegalic and triangular face with a long nose) and macroorchidism were also present. The mother displayed moderate intellectual deficit and milder facial anomalies. Central nervous system anomalies were identified in the two boys: subarachnoid cysts and hyperdensity in the pontine region.	Finding site	False	Structure of distal part of pituitary	Inferred relationship	Some
This syndrome is characterized by the association of hypogonadotropic hypogonadism and frontoparietal alopecia.	Finding site	False	Structure of distal part of pituitary	Inferred relationship	Some
Hypomyelination, hypogonadotropic hypogonadism, hypodontia syndrome (disorder)	Finding site	False	Structure of distal part of pituitary	Inferred relationship	Some
Hypogonadism with anosmia	Finding site	False	Structure of distal part of pituitary	Inferred relationship	Some
Kallmann syndrome with cardiopathy is characterized by hypogonadotropic hypogonadism associated with gonadotropin-releasing hormone (GnRH) deficiency, anosmia or hyposmia (with hypoplasia or aplasia of the olfactory bulbs) and complex congenital cardiac malformations (double-outlet right ventricle, dilated cardiomyopathy, right aortic arch). It represents a distinct clinical entity from Kallmann syndrome.	Finding site	False	Structure of distal part of pituitary	Inferred relationship	Some
This syndrome is characterized by the association of intellectual deficit, congenital cataract, and hypogonadotropic hypogonadism.	Finding site	False	Structure of distal part of pituitary	Inferred relationship	Some
Congenital hypogonadotropic hypogonadism (disorder)	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	3
This syndrome is characterized by the association of severe nasal hypoplasia, hypoplasia of the eyes, hyposmia, hypogeusia and hypogonadotropic hypogonadism.	Finding site	False	Structure of distal part of pituitary	Inferred relationship	Some	8
A rare syndromic neurological disorder characterized by the association of Möbius syndrome (congenital facial palsy with impaired ocular abduction) with peripheral axonal neuropathy and hypogonadotropic hypogonadism. There have been no further reports since 1996.	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	7
A rare endocrine syndrome characterized by the association of hypogonadotropic hypogonadism (with primary amenorrhea and lack of secondary sexual development) and retinitis pigmentosa.	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	5
Sotos' syndrome	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	1
Hypogonadism with anosmia	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	2
X-linked intellectual disability-acromegaly-hyperactivity syndrome is characterized by severe intellectual deficit, acromegaly and hyperactivity. The syndrome has been described in two half-brothers. Dysarthria, aggressive behavior, a characteristic facies (an acromegalic and triangular face with a long nose) and macroorchidism were also present. The mother displayed moderate intellectual deficit and milder facial anomalies. Central nervous system anomalies were identified in the two boys: subarachnoid cysts and hyperdensity in the pontine region.	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	1
This syndrome is characterized by the association of hypogonadotropic hypogonadism and frontoparietal alopecia.	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	4
Hypomyelination, hypogonadotropic hypogonadism, hypodontia syndrome (disorder)	Finding site	False	Structure of distal part of pituitary	Inferred relationship	Some	2
Kallmann syndrome with cardiopathy is characterized by hypogonadotropic hypogonadism associated with gonadotropin-releasing hormone (GnRH) deficiency, anosmia or hyposmia (with hypoplasia or aplasia of the olfactory bulbs) and complex congenital cardiac malformations (double-outlet right ventricle, dilated cardiomyopathy, right aortic arch). It represents a distinct clinical entity from Kallmann syndrome.	Finding site	False	Structure of distal part of pituitary	Inferred relationship	Some	1
This syndrome is characterized by the association of intellectual deficit, congenital cataract, and hypogonadotropic hypogonadism.	Finding site	False	Structure of distal part of pituitary	Inferred relationship	Some	2
Growth delay due to insulin-like growth factor I deficiency is characterized by the association of intrauterine and postnatal growth retardation with sensorineural deafness and intellectual deficit.	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	1
Beckwith-Wiedemann syndrome	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	6
Hypomyelination, hypogonadotropic hypogonadism, hypodontia syndrome (disorder)	Finding site	False	Structure of distal part of pituitary	Inferred relationship	Some	1
This syndrome is characterized by the association of intellectual deficit, congenital cataract, and hypogonadotropic hypogonadism.	Finding site	False	Structure of distal part of pituitary	Inferred relationship	Some	1
Isolated follicle stimulating hormone deficiency	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	1
Hypothalamic hypothyroidism	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	2
Transient infantile hyperthyrotropinemia (disorder)	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	3
Familial hyperprolactinemia is a rare, genetic endocrine disorder characterized by persistently high prolactin serum levels (not associated with gestation, puerperium, drug intake or pituitary tumor) in multiple members of a family. Clinically it manifests with signs usually observed in hyperprolactinemia, which are: secondary medroxyprogesterone acetate (MPA)-negative amenorrhea and galactorrhea in female patients, and hypogonadism and decreased testosterone level-driven sexual dysfunction in male patients. Oligomenorrhea and primary infertility have also been reported in some female patients.	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	1
X-linked acrogigantism	Finding site	False	Structure of distal part of pituitary	Inferred relationship	Some	1
A rare, genetic, neuro-endocrino-cutaneous disorder characterised by highly variable degrees of alopecia, moderate to severe intellectual disability, progressive, late-onset motor deterioration and combined anterior pituitary hormone deficiency, manifesting with central hypogonadotropic hypogonadism, delayed or absent puberty, growth hormone deficiency (resulting in short stature), progressive central adrenal insufficiency and a hypoplastic anterior pituitary gland. Additional features include hypodontia, flexural reticulate hyperpigmentation, gynaecomastia, microcephaly and kyphoscoliosis.	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	2
A rare group of multiple congenital anomalies/dysmorphic syndrome characterised by autism spectrum disorder, developmental delay, intellectual disability, hyperphagia/obesity, and short stature (clinical features overlapping with Prader-Willi syndrome). However, it is a clinically and genetically heterogeneous group where patients may completely lack or manifests in minority some classical clinical features of Prader-Willi syndrome such as short stature, hypotonia, hypogonadism, hyperphagia and morbid obesity.	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	2
Kallmann syndrome with cardiopathy is characterized by hypogonadotropic hypogonadism associated with gonadotropin-releasing hormone (GnRH) deficiency, anosmia or hyposmia (with hypoplasia or aplasia of the olfactory bulbs) and complex congenital cardiac malformations (double-outlet right ventricle, dilated cardiomyopathy, right aortic arch). It represents a distinct clinical entity from Kallmann syndrome.	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	3
This syndrome is characterized by the association of severe nasal hypoplasia, hypoplasia of the eyes, hyposmia, hypogeusia and hypogonadotropic hypogonadism.	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	3
Hypogonadotropic hypogonadism-severe microcephaly-sensorineural hearing loss-dysmorphism syndrome is a rare, non-acquired pituitary hormone deficiency syndrome characterized by severe, congenital microcephaly, facial dysmorphism (highly arched eyebrows, hypertelorism, convex nasal ridge, protruding ears with underdeveloped superior antihelix crus, micrognathia), bilateral sensorineural deafness and hypogonadotropic hypogonadism, in association with early feeding problems, myopia, moderate intellectual disability and moderate short stature.	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	2
A rare, genetic pituitary disease characterized by infantile-onset, rapid and excessive acceleration of linear growth and body size due to mixed growth hormone (GH)- and prolactin-secreting adenomas and/or pituitary hyperplasia. Patients present with gigantism and may have associated acromegalic features (e.g. coarse facial features, frontal bossing, prognathism, increased interdental space) as well as marked enlargement of hands and feet, soft tissue swelling, increased appetite and acanthosis nigricans.	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	1
Late-onset isolated ACTH deficiency is a rare, acquired, pituitary hormone deficiency characterized by secondary adrenal insufficiency, with normal secretion of anterior pituitary hormones, except for ACTH. Patients present with weakness, fatigue, weight loss, anorexia, vomiting/nausea, hypoglycemia, and abnormally low serum ACTH and cortisol levels. Association with autoimmune disease such as Hashimoto's thyroiditis has been described.	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	1
A rare, genetic, non-syndromic, obesity disease characterized by severe, early-onset obesity, associated with major hyperphagia and endocrine abnormalities, resulting from leptin receptor deficiency.	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	2
A rare endocrine disease characterized by a miniature adult type of congenital adrenal hypoplasia (residual adrenal cortex is composed of a small amount of permanent adult cortex with normal structural organization), selective absence of pituitary luteinizing hormone in otherwise normal brain, and neonatal demise. Patients present with hypogonadotropic hypogonadism, hypoglycemia, seizures, encephalopathy and diabetes insipidus. There have been no further descriptions in the literature since 1988.	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	3
Sotos' syndrome	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	4
This syndrome is characterized by the association of intellectual deficit, congenital cataract, and hypogonadotropic hypogonadism.	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	3
Prader-Willi syndrome	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	3
A type of familial infantile gigantism caused by microduplication of Xq26.3. Onset usually occurs in the first year of life in previously normal infants. Patients present with gigantism and may associate acromegalic features (e.g. coarse facial features, frontal bossing, prognathism, increased interdental space) as well as marked enlargement of hands and feet, soft tissue swelling, appetite increase and acanthosis nigricans. May present as a sporadic condition or as familial isolated pituitary adenomas.	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	4
Hypersomatotropic gigantism	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	3
Beckwith-Wiedemann syndrome	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	5
Intellectual disability-myopathy-short stature-endocrine defect syndrome is a rare congenital myopathy syndrome characterized by nonprogressive myopathy (manifesting with mild facial and generalized weakness, bilateral ptosis, and severe lumbar lordosis), severe intellectual disability, short stature, and sexual infantilism (due to hypogonadotropic hypogonadism). The presence of a small pituitary fossa was also noted. There have been no further descriptions in the literature since 1985.	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	3
Functional hypogonadotropic hypogonadism (disorder)	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	1
A very rare autosomal recessive, slowly progressive neurodegenerative disorder characterized by the triad of cerebellar ataxia (that generally manifests at adolescence or early adulthood), chorioretinal dystrophy, which may have a later onset (up to the fifth-sixth decade) leading to variable degrees of visual impairment, and hypogonadotropic hypogonadism (delayed puberty and lack of secondary sex characteristics). Ataxia-hypogonadism-choroidal dystrophy syndrome belongs to a clinical continuum of neurodegenerative disorders along with the clinically overlapping cerebellar ataxia-hypogonadism syndrome.	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	4
6q16 microdeletion syndrome	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	5
Progressive cerebellar ataxia with hypogonadism	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	3
Hereditary growth hormone deficiency (disorder)	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	1
Hyperprolactinemia due to hypothyroidism	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	2
A rare hypomyelinating leukodystrophy disorder characterized by the association of dental abnormalities (delayed dentition, abnormal order of dentition, hypodontia), hypogonadotropic hypogonadism, and hypomyelinating leukodystrophy manifesting with neurodevelopmental delay or regression and/or progressive cerebellar symptoms.	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	4
Odontoleukodystrophy (disorder)	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	5
Leukoencephalopathy, ataxia, hypodontia, hypomyelination syndrome (disorder)	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	6
Hypomyelination, hypogonadotropic hypogonadism, hypodontia syndrome (disorder)	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	8
A rare mitochondrial disease characterized by a highly variable phenotypic spectrum comprising delayed motor development, peripheral neuropathy, cataract, short stature due to growth hormone deficiency, nystagmus, sensorineural hearing loss, dysmorphic facial features, and skeletal abnormalities consistent with spondyloepimetaphyseal dysplasia. Hyperextensible joints, achalasia, and telangiectasia have also been described. Cognition is normal. Atrophy of the pituitary gland has been observed in brain imaging.	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	6
A rare Prader-Willi-like syndrome characterized by severe obesity due to SIM1 mutation, in addition to some clinical features of Prader-Willi- syndrome including intellectual disability, developmental delay, behavior problems and facial dysmorphism. Unlike Prader-Willi syndrome, short stature, hypotonia and hypogonadism may not be observed.	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	3
A rare Prader-Willi-like syndrome characterized by arthrogryposis, including contractures of the proximal and distal interphalangeal joints, and autism spectrum disorder due to MAGEL2 mutation. Overlapping phenotypes with Prader-Willi syndrome include hypotonia, feeding difficulties, weight gain, developmental delay, intellectual disability and hypogonadism. Minority of patients manifest hyperphagia and morbid obesity in contrast to patients with Prader-Willi syndrome.	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	3
A rare endocrine disease characterized by neonatal hypoglycemia, prolonged cholestatic jaundice, and seizures. Typical are low plasma ACTH and cortisol levels in the absence of structural pituitary defects, and sometimes low partial growth hormone deficiency is associated.	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	2
A form of monogenic obesity with characteristics of severe early-onset obesity and marked hyperphagia. Patients with congenital leptin deficiency are severely hyperphagic from early infancy and, although birthweight is normal, they rapidly become obese during early childhood. An increased susceptibility to infections has also been reported in these infants and appears to be associated with reduced numbers of circulating CD4+ T cells, and impaired T cell proliferation and cytokine release. Absence of serum leptin is caused by homozygous frameshift or missense mutations in the ob gene (7q31.3) and is inherited as an autosomal recessive trait.	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	2
A rare genetic disease characterized by childhood onset of multiple endocrine manifestations in combination with central and peripheral nervous system abnormalities. Reported signs and symptoms include postnatal growth retardation, moderate intellectual disability, hypogonadotropic hypogonadism, insulin-dependent diabetes mellitus, central hypothyroidism, demyelinating sensorimotor polyneuropathy, and cerebellar and pyramidal signs. Progressive hearing loss and a hypoplastic pituitary gland have also been described. Brain imaging shows moderate white matter abnormalities.	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	3
A rare Prader-Willi-like syndrome with characteristics of intellectual disability, morbid obesity, hypogonadotrophic hypogonadism, hyperphagia and developmental delay. Endocrine disorders including hypothyroidism and insulin resistance can be observed. Unlike Prader-Willi syndrome, profound muscular hypotonia, feeding difficulties in neonates, short stature and growth hormone deficiency are not observed.	Finding site	True	Structure of distal part of pituitary	Inferred relationship	Some	3

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Reference Sets

Anatomy structure and entire association reference set (foundation metadata concept)

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Id	Description	Lang	Type	Status	Case?	Module
87573014	Distal part of pituitary	en	Synonym (core metadata concept)	Active	Entire term case insensitive (core metadata concept)	SNOMED CT core
496383018	Structure of distal part of pituitary	en	Synonym (core metadata concept)	Active	Entire term case insensitive (core metadata concept)	SNOMED CT core
2638822012	Structure of pars distalis of pituitary (body structure)	en	Fully specified name	Active	Entire term case insensitive (core metadata concept)	SNOMED CT core
2647113014	Pars distalis of adenohypophysis	en	Synonym (core metadata concept)	Active	Entire term case insensitive (core metadata concept)	SNOMED CT core
2647114015	Pituitary pars distalis	en	Synonym (core metadata concept)	Active	Entire term case insensitive (core metadata concept)	SNOMED CT core
2670903014	Structure of pars distalis of pituitary	en	Synonym (core metadata concept)	Active	Entire term case insensitive (core metadata concept)	SNOMED CT core