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255314001: Progressive (qualifier value)

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

Descriptions:

Id Description Lang Type Status Case? Module
380497019 Progressive en Synonym (core metadata concept) Active Entire term case insensitive (core metadata concept) SNOMED CT core
646336011 Progressive (qualifier value) en Fully specified name Active Entire term case insensitive (core metadata concept) SNOMED CT core
138851000077114 progressif fr Synonym (core metadata concept) Active Entire term case insensitive (core metadata concept) SNOMED CT Switzerland NRC maintained Module

0 descendants.

Expanded Value Set

Outbound Relationships Type Target Active Characteristic Refinability Group Values
Progressive Is a Behaviour descriptors true Inferred relationship Some
Progressive Is a Chronic true Inferred relationship Some
Inbound Relationships Type Active Source Characteristic Refinability Group
Acquired progressive lymphangioma (disorder) Clinical course True Progressive Inferred relationship Some 2
Progressive angina (disorder) Clinical course True Progressive Inferred relationship Some 2
Acquired progressive kinking of hair Clinical course True Progressive Inferred relationship Some 2
Cirrhosis of liver due to chronic hepatitis C Clinical course True Progressive Inferred relationship Some 3
Intrahepatic phlebosclerosis and fibrosis Clinical course False Progressive Inferred relationship Some 3
Hepatic fibrosis with hepatic sclerosis Clinical course False Progressive Inferred relationship Some 3
Cirrhosis of liver co-occurrent and due to primary sclerosing cholangitis (disorder) Clinical course True Progressive Inferred relationship Some 5
Capsular portal cirrhosis Clinical course True Progressive Inferred relationship Some 3
Fatty portal cirrhosis Clinical course True Progressive Inferred relationship Some 3
Posthepatitic cirrhosis Clinical course True Progressive Inferred relationship Some 3
Micronodular cirrhosis Clinical course True Progressive Inferred relationship Some 4
Cardiac cirrhosis Clinical course True Progressive Inferred relationship Some 4
Unilobular portal cirrhosis Clinical course True Progressive Inferred relationship Some 3
Syphilitic cirrhosis Clinical course True Progressive Inferred relationship Some 3
Parasitic cirrhosis Clinical course True Progressive Inferred relationship Some 3
A rare early childhood onset progressive encephalopathy characterized by extreme cerebellar atrophy, infantile onset hypotonia, infantile spasms with hypsarrhythmia, profound intellectual disability, and optic atrophy. PEHO stands for the main features of the syndrome: Progressive encephalopathy with Edema, Hypsarrhythmia and Optic atrophy. Clinical course True Progressive Inferred relationship Some 3
Mixed micro and macronodular cirrhosis (disorder) Clinical course True Progressive Inferred relationship Some 5
Idiopathic copper-associated cirrhosis is a rare copper-overload liver disease characterized by a rapidly progressive liver cirrhosis from the first few years of life leading to hepatic insufficiency and harboring a specific pathological aspect: pericellular fibrosis, inflammatory infiltration, hepatocyte necrosis, absence of steatosis, poor regeneration and histochemical copper staining. Clinical course True Progressive Inferred relationship Some 6
Glissonian cirrhosis Clinical course True Progressive Inferred relationship Some 4
Nutritional cirrhosis Clinical course True Progressive Inferred relationship Some 3
Cirrhosis of liver caused by methotrexate (disorder) Clinical course True Progressive Inferred relationship Some 4
Cirrhosis secondary to cholestasis Clinical course True Progressive Inferred relationship Some 4
Infectious cirrhosis Clinical course True Progressive Inferred relationship Some 3
Juvenile portal cirrhosis Clinical course True Progressive Inferred relationship Some 3
Early cirrhosis (disorder) Clinical course True Progressive Inferred relationship Some 3
Cryptogenic cirrhosis Clinical course True Progressive Inferred relationship Some 3
Florid cirrhosis Clinical course True Progressive Inferred relationship Some 3
Cirrhosis resulting from accumulation of iron in the liver. Clinical course True Progressive Inferred relationship Some 3
Macronodular cirrhosis Clinical course True Progressive Inferred relationship Some 4
Toxic cirrhosis Clinical course True Progressive Inferred relationship Some 3
Cirrhosis - non-alcoholic Clinical course True Progressive Inferred relationship Some 3
Cirrhosis of liver caused by methyldopa (disorder) Clinical course True Progressive Inferred relationship Some 4
Drug-induced cirrhosis of liver (disorder) Clinical course True Progressive Inferred relationship Some 4
Cirrhosis of liver caused by amiodarone (disorder) Clinical course True Progressive Inferred relationship Some 4
Advanced cirrhosis Clinical course True Progressive Inferred relationship Some 3
Primary biliary cholangitis Clinical course False Progressive Inferred relationship Some 4
Cholangiolitic cirrhosis (disorder) Clinical course True Progressive Inferred relationship Some 4
PEHO-like syndrome is a rare, genetic neurological disease characterized by progressive encephalopathy, early-onset seizures with a hypsarrhythmic pattern, facial and limb edema, severe hypotonia, early arrest of psychomotor development and craniofacial dysmorphism (evolving microcephaly, narrow forehead, short nose, prominent auricles, open mouth, micrognathia), in the absence of neuro-ophthalmic or neuroradiologic findings. Poor visual responsiveness, growth failure and tapering fingers are also associated. Clinical course True Progressive Inferred relationship Some 3
Portal cirrhosis (disorder) Clinical course True Progressive Inferred relationship Some 3
Syphilitic portal cirrhosis Clinical course True Progressive Inferred relationship Some 4
Toxic portal cirrhosis Clinical course True Progressive Inferred relationship Some 3
Pigmentary portal cirrhosis Clinical course True Progressive Inferred relationship Some 3
Postnecrotic cirrhosis Clinical course True Progressive Inferred relationship Some 4
Cruveilhier-Baumgarten syndrome Clinical course True Progressive Inferred relationship Some 4
Diffuse nodular cirrhosis Clinical course True Progressive Inferred relationship Some 3
Multilobular portal cirrhosis Clinical course True Progressive Inferred relationship Some 3
Secondary biliary cirrhosis Clinical course True Progressive Inferred relationship Some 4
Indian childhood cirrhosis Clinical course True Progressive Inferred relationship Some 4
Clonorchiasis with biliary cirrhosis Clinical course True Progressive Inferred relationship Some 4
Cirrhosis and chronic liver disease Clinical course False Progressive Inferred relationship Some 3
Hepatic sclerosis Clinical course False Progressive Inferred relationship Some 3
Cirrhosis of liver due to hepatitis B Clinical course True Progressive Inferred relationship Some 4
Hypoxia-associated cirrhosis (disorder) Clinical course True Progressive Inferred relationship Some 4
Laennec's cirrhosis, non-alcoholic Clinical course True Progressive Inferred relationship Some 3
Decompensated cirrhosis of liver (disorder) Clinical course True Progressive Inferred relationship Some 3
Distal hereditary motor neuropathy type 1 is a rare neuromuscular disease characterized by slowly progressive lower limb muscular weakness and atrophy, without sensory impairment. Additional clinical features may include pes cavus, hammertoe and increased muscle tone. Clinical course True Progressive Inferred relationship Some 1
Reynolds syndrome (RS) is an autoimmune disorder characterized by the association of primary biliary cirrhosis (PBC) with limited cutaneous systemic sclerosis (lcSSc). Clinical course True Progressive Inferred relationship Some 4
Cardiac portal cirrhosis Clinical course True Progressive Inferred relationship Some 3
Latent cirrhosis Clinical course True Progressive Inferred relationship Some 3
Obstructive biliary cirrhosis Clinical course True Progressive Inferred relationship Some 4
Cirrhotic cardiomyopathy is the term used to describe a constellation of features indicative of abnormal heart structure and function in patients with cirrhosis. These include systolic and diastolic dysfunction, electrophysiological changes, and macroscopic and microscopic structural changes. Clinical course True Progressive Inferred relationship Some 5
Biliary cirrhosis Clinical course True Progressive Inferred relationship Some 4
Alcoholic cirrhosis (disorder) Clinical course True Progressive Inferred relationship Some 4
Biliary cirrhosis of children Clinical course True Progressive Inferred relationship Some 4
Bacterial portal cirrhosis Clinical course True Progressive Inferred relationship Some 4
Cirrhosis of liver Clinical course True Progressive Inferred relationship Some 3
Occult macular dystrophy is a rare, genetic retinal dystrophy disease characterized by bilateral progressive decline of visual acuity, due to retinal dysfunction confined only to the macula, associated with normal fundus and fluorescein angiograms and severly attenuated focal macular and multifocal electroretinograms. Clinical course True Progressive Inferred relationship Some 2
A rare, genetic neuromuscular disease characterized by progressive external ocular, facial and pharyngeal muscle weakness, leading to variable degrees of ptosis, ophthalmoparesis, facial muscle atrophy, dysarthria and dysphagia, as well as distal muscle weakness and atrophy of lower and upper extremities. Respiratory muscle involvement is common, but sensorineural hearing loss, asymmetrical extremity weakness and severe proximal weakness are rare. Clinical course True Progressive Inferred relationship Some 1
A rare mitochondrial oxidative phosphorylation disorder characterized by myoclonic seizures, ataxia, generalized epilepsy, muscle weakness and ragged red fibers in the muscle biopsy. Clinical course True Progressive Inferred relationship Some 1
A rare, genetic, vitreoretinal degeneration characterized by a slowly progressive vitreoretinopathy with onset during the second or third decade of life. The disease initially presents as autoimmune uveitis with reduction in the b-wave on electroretinography, and progresses with development of photoreceptor degeneration, vitreous hemorrhage, cystoid macular edema, retinal neovascularization, intraocular fibrosis, secondary glaucoma, and retinal detachment leading to phthisis and complete blindness. Clinical course True Progressive Inferred relationship Some 3
Recessive intellectual disability-motor dysfunction-multiple joint contractures syndrome is a rare, genetic, syndromic intellectual disability disorder characterized by severe intellectual disability, progressive, postnatal, multiple joint contractures and severe motor dysfunction. Patients present arrest and regression of motor function and speech acquisition, as well as contractures which begin in lower limbs and slowly progress in an ascending manner to include spine and neck, resulting in individuals presenting a specific fixed position. Clinical course True Progressive Inferred relationship Some 3
Huntington disease-like 3 is a rare Huntington disease-like syndrome characterized by childhood-onset progressive neurologic deterioration with pyramidal and extrapyramidal abnormalities, chorea, dystonia, ataxia, gait instability, spasticity, seizures, mutism, and (on brain MRI) progressive frontal cortical atrophy and bilateral caudate atrophy. Clinical course True Progressive Inferred relationship Some 1
A rare, slowly progressive genetic peripheral neuropathy characterized by distal atrophy and weakness affecting the upper limbs (with a predilection for the thenar eminence) and subsequently the lower limbs, associated with uni- or bilateral vocal cord paresis leading to hoarse voice and breathing difficulties, and facial weakness. Clinical course True Progressive Inferred relationship Some 2
Hereditary motor and sensory neuropathy type 5 is a rare axonal hereditary motor and sensory neuropathy characterized by slowly progressive distal muscle weakness and atrophy with or without sensory loss resulting in difficulty in walking, foot drop and pes cavus, that may be associated with pyramidal signs (extensor plantar responses, mild increase in tone, brisk tendon reflexes), muscle cramps, pain and spasticity. Clinical course True Progressive Inferred relationship Some 1
Hereditary motor and sensory neuropathy with acrodystrophy is a rare axonal hereditary motor and sensory neuropathy characterized by progressive axonal neuropathy with limb weakness and severe distal sensory loss in all limbs and acrodystrophic changes leading to painless non-healing ulcers, osteomyelitis, contractures and mutilating lesions with loss of terminal phalanges. One family with three affected siblings is described and there have been no further descriptions in the literature since 1999. Clinical course True Progressive Inferred relationship Some 1
Progressive polyneuropathy with bilateral striatal necrosis is a rare, genetic disorder of thiamine metabolism and transport characterized by the childhood-onset of recurrent episodes of flaccid paralysis and encephalopathy, associated with bilateral striatal necrosis and chronic progressive axonal polyneuropathy with proximal and distal muscle weakness, areflexia, contractures and foot deformities. Clinical course True Progressive Inferred relationship Some 2
Hereditary fibrosing poikiloderma-tendon contractures-myopathy-pulmonary fibrosis syndrome is a rare, genetic, hereditary poikiloderma syndrome characterized by early-onset poikiloderma (mainly on the face), hypotrichosis, hypohidrosis, muscle and tendon contractures with varus foot deformity, progressive proximal and distal muscle weakness in all extremities, and progressive pulmonary fibrosis. Mild lymphedema of the extremities, growth retardation, liver impairment, exocrine pancreatic insufficiency and hematologic abnormalities are additional variable features. Clinical course True Progressive Inferred relationship Some 4
A rare axonal hereditary motor and sensory neuropathy characterized by infantile onset of slowly progressive distal motor weakness and atrophy (more severe in legs and moderate in arms) with mildly delayed motor development, hypotonia, and distal sensory impairment of all sensory modalities. Clinical course True Progressive Inferred relationship Some 2
Hereditary progressive mucinous histiocytosis is a rare, benign, non-Langerhans cell histiocytosis characterized by childhood or adolescence onset of multiple, small, asymptomatic, slowly progressing, skin-colored to red-brown papules with predilection for the face, dorsal hands, forearms and legs, without associated mucosal or visceral involvement. Histologically, papules are well-circumscribed, unencapsulated, nodular aggregates of histiocytes with abundant mucin in the upper and mid dermis. Clinical course True Progressive Inferred relationship Some 2
A rare, genetic, neuromuscular disease characterized by proximal muscle weakness with an early involvement of foot and hand muscles following normal motor development in early childhood, a rapidly progressive disease course leading to generalized areflexic tetraplegia with contractures, severe scoliosis, hyperlordosis, and progressive respiratory insufficiency leading to assisted ventilation. Cranial nerve functions are normal and tongue wasting and fasciculations are absent. Milder phenotype with a moderate generalized weakness and slower disease progress was reported. Clinical course True Progressive Inferred relationship Some 3
Early-onset spastic ataxia-myoclonic epilepsy-neuropathy syndrome is a rare hereditary spastic ataxia disorder characterized by childhood onset of slowly progressive lower limb spastic paraparesis and cerebellar ataxia (with dysarthria, swallowing difficulties, motor degeneration), associated with sensorimotor neuropathy (including muscle weakness and distal amyotrophy in lower extremities) and progressive myoclonic epilepsy. Ocular signs (ptosis, oculomotor apraxia), dysmetria, dysdiadochokinesia, dystonic movements and myoclonus may also be associated. Clinical course True Progressive Inferred relationship Some 4
Young adult-onset distal hereditary motor neuropathy is a rare autosomal recessive distal hereditary motor neuropathy characterized by slowly progressive muscular weakness, hypotonia and atrophy of the lower limbs, more pronounced distally, leading to paralysis, and loss of tendon reflexes. Additional features may include pes cavus and mild dysphonia. The upper limbs are relatively spared. Clinical course True Progressive Inferred relationship Some 2
A rare, genetic, neurodegenerative disease characterized by normal early development followed by childhood onset optic atrophy with progressive vision loss and eventually blindness, followed by progressive neurological decline that typically includes cerebellar ataxia, nystagmus, dorsal column dysfunction (decreased vibration and position sense), spastic paraplegia and finally tetraparesis. Clinical course True Progressive Inferred relationship Some 3
A rare subtype of autosomal recessive intermediate Charcot-Marie-Tooth (CMT) disease characterized by childhood to adulthood-onset of progressive, moderate to severe, predominantly distal, mostly lower limb muscle weakness and atrophy, foot deformities (including pes cavus and hammer toes), absent deep tendon reflexes and distal sensory loss associated with decreased motor and sensory nerve conduction velocities and features of both demyelinating and axonal neuropathy on sural nerve biopsy. Clinical course True Progressive Inferred relationship Some 1
A rare subtype of autosomal dominant Charcot-Marie-Tooth disease type 2, characterized by adolescent to adulthood-onset of symmetrical, slowly progressive distal muscle weakness and atrophy (with a predominant weakness of the distal lower limbs) associated with reduced or absent deep tendon reflexes, pes cavus and mild to moderated deep sensory impairment. Clinical course True Progressive Inferred relationship Some 2
A rare hereditary ataxia characterized by an early onset symptomatic generalized epilepsy, progressive cerebellar ataxia resulting in significant difficulties to walk or wheelchair dependency, and intellectual disability. Clinical course True Progressive Inferred relationship Some 1
A rare, genetic, neurodegenerative disorder characterized by progressive psychomotor and cognitive regression (manifesting with gait ataxia, spasticity, loss of language, mild to severe intellectual disability, pyramidal and extrapyramidal signs and, frequently, development of tetraplegia or tetraparesis) associated with variable degrees of lipodystrophy, hepatomegaly, hypertriglyceridemia and muscular hypertrophy. Hyperactivity, tremor and development of seizures may also be associated. Clinical course True Progressive Inferred relationship Some 3
Childhood-onset spasticity with hyperglycinemia is a rare neurometabolic disease characterized by a childhood onset of progressive spastic ataxia associated with gait disturbances, hyperreflexia, extensor plantar responses and non-ketotic hyperglycinemia typically revealed by biochemical analysis. Additional signs of upper extremity spasticity, dysarthria, learning difficulties, poor concentration, nystagmus, optic atrophy and reduced visual acuity may also be associated. Clinical course True Progressive Inferred relationship Some 4
Progressive retinal dystrophy due to retinol transport defect is a rare, genetic, metabolite absorption and transport disorder characterized by progressive rod-cone dystrophy, usually presenting with impaired night vision in childhood, progressive loss of visual acuity and severe retinol deficiency without keratomalacia. Association with ocular colobomas, severe acne and hypercholesterolemia has been reported. Clinical course True Progressive Inferred relationship Some 2
A rare progressive muscular dystrophy characterized by an adult-onset scapulo-axio-peroneal myopathy. Clinical presentation includes shoulder girdle atrophy, scapular winging, axial muscular atrophy of postural muscles combined with a generalized hypertrophy. Typically, neck rigidity, rigid spine, Achilles tendon shortening, and respiratory insufficiency later in disease course are present. Clinical course True Progressive Inferred relationship Some 2
A rare neuroinflammatory disease characterized by the onset of ataxia, dysarthria and cerebral white matter changes which are triggered by viral infection. Episodic progressive neurodegeneration (manifesting with loss of motor and verbal skills, muscle weakness, further cerebral white matter degeneration and, eventually, death) is observed in the absence of hematopathology, cytokine overproduction, fever, hypertriglyceridemia, hypofibrinogenemia and hyperferritinemia. Clinical course True Progressive Inferred relationship Some 4
A rare, genetic, mitochondrial disease characterized by early-onset progressive renal failure, manifesting with hyperuricemia, hyponatremia, hypomagnesemia, hypochloremic metabolic alkalosis, elevated BUN and polyuria, associated with systemic manifestations which include pulmonary hypertension, failure to thrive, global developmental delay, hypotonia and ventricular hypertrophy. Additional features include prematurity, elevated serum lactate, diabetes mellitus and, in some, pancytopenia. Clinical course True Progressive Inferred relationship Some 2
A rare, acquired motor neuron disease characterized by a slowly progressive, unilateral, ascending or descending hemiplegia, associated to unilateral or asymmetrical pyramidal signs and no sensory loss. It is a diagnosis of exclusion and controversy exists regarding whether the presence of bulbar symptoms, sphincter disturbances, fasciculations or cognitive manifestations characterize the disease. Clinical course True Progressive Inferred relationship Some 2
Hyperphosphatasemia tarda Clinical course True Progressive Inferred relationship Some 5
A rare primary bone dysplasia characterized by the association of multiple epiphyseal dysplasia with macrocephaly and dysmorphic facial features (such as frontal bossing, hypertelorism, flat malar region, low-set ears, and short neck). Patients are of normal stature and present with joint swelling and genu valgum. Additional reported manifestations include clinodactyly, spindle-shaped fingers, and pectus excavatum. Clinical course True Progressive Inferred relationship Some 2
Pyknodysostosis Clinical course True Progressive Inferred relationship Some 2
Osteopetrosis - delayed type Clinical course True Progressive Inferred relationship Some 3
A rare congenital disorder of bone resorption characterised by generalised skeletal densification. Clinical course True Progressive Inferred relationship Some 3
Sclerosteosis Clinical course True Progressive Inferred relationship Some 2
A rare congenital muscular alpha-dystroglycanopathy with brain and eye anomalies disease characterized by a severe muscle-eye-brain disease-like phenotype associated with intellectual disability, muscular dystrophy, macrocephaly and extended bilateral multicystic white matter disease. Clinical course True Progressive Inferred relationship Some 6
A rare, genetic, neurological disorder characterized by early-onset, progressive ataxia associated with myoclonic seizures (frequently associated with other seizure types such as generalized tonic-clonic, absence and drop attacks), scoliosis of variable severity, areflexia, elevated creatine kinase serum levels, and relative preservation of cognitive function until late in the disease course. Clinical course True Progressive Inferred relationship Some 1

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