Frontal bossing, and Ichthyosis

Diseases related with Frontal bossing and Ichthyosis

In the following list you will find some of the most common rare diseases related to Frontal bossing and Ichthyosis that can help you solving undiagnosed cases.

Top matches:

Rhizomelic chondrodysplasia punctata (RCDP) is a peroxisomal disorder characterized by disproportionately short stature primarily affecting the proximal parts of the extremities, a typical facial appearance including a broad nasal bridge, epicanthus, high-arched palate, dysplastic external ears, and micrognathia, congenital contractures, characteristic ocular involvement, dwarfism, and severe mental retardation with spasticity. Biochemically, plasmalogen synthesis and phytanic acid alpha-oxidation are defective. Most patients die in the first decade of life. RCDP1 is the most frequent form of RCDP (summary by Wanders and Waterham, 2005).Individuals with RCDP1, carrying mutations in the PEX7 gene, have cells of peroxisome biogenesis disorder (PBD) complementation group 11 (CG11, equivalent to CGR). For information on the history of PBD complementation groups, see {214100}. Genetic Heterogeneity of Rhizomelic Chondrodysplasia PunctataRCDP2 (OMIM ) is caused by mutation in the gene encoding acyl-CoA:dihydroxyacetonephosphate acyltransferase (GNPAT ) on chromosome 1q42. RCDP3 (OMIM ) is caused by mutation in the gene encoding alkyldihydroxyacetonephosphate synthase (alkyl-DHAP synthase) (AGPS ) on chromosome 2q31. RCDP5 (OMIM ) is caused by mutation in the gene encoding peroxisomal biogenesis factor-5 (PEX5 ) on chromosome 12p13.Whereas RCDP1 is a peroxisomal biogenesis disorder (PBD), RCDP2 and RCDP3 are classified as single peroxisome enzyme deficiencies (Waterham and Ebberink, 2012).

RHIZOMELIC CHONDRODYSPLASIA PUNCTATA, TYPE 1; RCDP1 Is also known as pbd9|chondrodystrophia calcificans punctata|chondrodysplasia punctata, rhizomelic form|peroxisome biogenesis disorder 9|cdpr

Related symptoms:

  • Intellectual disability
  • Seizures
  • Global developmental delay
  • Short stature
  • Microcephaly


SOURCES: OMIM ORPHANET MENDELIAN

More info about RHIZOMELIC CHONDRODYSPLASIA PUNCTATA, TYPE 1; RCDP1

Linear nevus sebaceous syndrome (LNSS) is characterized by the association of a large sebaceous nevus, usually appearing on the face or on the scalp, with a broad spectrum of abnormalities that may affect every organ system, including the central nervous system (brain neoplasms, hemimegalencephaly and lateral ventricle enlargement).

LINEAR NEVUS SEBACEUS SYNDROME Is also known as linear sebaceous nevus syndrome|jnp|sfm syndrome|solomon syndrome|epidermal nevus syndrome, formerly|sebaceous nevus syndrome, linear|nevus sebaceus of jadassohn|jadassohn nevus phakomatosis|nevus sebaceus syndrome|schimmelpenning syndrome|organoid nevus

Related symptoms:

  • Intellectual disability
  • Seizures
  • Global developmental delay
  • Short stature
  • Growth delay


SOURCES: ORPHANET OMIM MENDELIAN

More info about LINEAR NEVUS SEBACEUS SYNDROME

X-linked dominant chondrodysplasia punctata (CDPX2) is a rare genodermatosis with great phenotypic variation and characterized most commonly by ichthyosis, chondrodysplasia punctata (CDP), asymmetric shortening of the limbs, cataracts and short stature.

X-LINKED DOMINANT CHONDRODYSPLASIA PUNCTATA Is also known as x-linked chondrodysplasia punctata type 2|cpxd|cdpxd|conradi-hunermann-happle syndrome|happle syndrome|conradi-hunermann syndrome|cdpx2|chondrodystrophia calcificans congenita|conradi-h√únermann-happle syndrome

Related symptoms:

  • Short stature
  • Hearing impairment
  • Scoliosis
  • Nystagmus
  • Failure to thrive


SOURCES: OMIM ORPHANET MENDELIAN

More info about X-LINKED DOMINANT CHONDRODYSPLASIA PUNCTATA

Other less relevant matches:

High match CHIME SYNDROME

CHIME syndrome is a rare ectodermal dysplasia syndrome characterized by ocular colobomas, cardiac defects, ichthyosiform dermatosis, intellectual disability, conductive hearing loss and epilepsy.

CHIME SYNDROME Is also known as coloboma-congenital heart disease-ichthyosiform dermatosis-intellectual disability-ear anomalies syndrome|zunich-kaye syndrome|zunich neuroectodermal syndrome|neuroectodermal syndrome, zunich type|chime syndrome|gpibd5|pigl-cdg|neuroectodermal dysplasia,

Related symptoms:

  • Intellectual disability
  • Seizures
  • Global developmental delay
  • Short stature
  • Generalized hypotonia


SOURCES: OMIM ORPHANET MESH MENDELIAN

More info about CHIME SYNDROME

Ichthyosis follicularis - alopecia - photophobia (IFAP) is a rare genetic disorder characterized by the triad of ichthyosis follicularis, alopecia, and photophobia from birth.

ICHTHYOSIS FOLLICULARIS-ALOPECIA-PHOTOPHOBIA SYNDROME Is also known as ichthyosis follicularis-atrichia-photophobia syndrome|ifap syndrome|ichthyosis follicularis, atrichia, and photophobia with or without brain anomalies, retardation, ectodermal dysplasia, skeletal malformations, hirschsprung disease, ear/eye anomalies, cle

Related symptoms:

  • Intellectual disability
  • Seizures
  • Global developmental delay
  • Short stature
  • Hearing impairment


SOURCES: ORPHANET OMIM MENDELIAN

More info about ICHTHYOSIS FOLLICULARIS-ALOPECIA-PHOTOPHOBIA SYNDROME

Medium match H SYNDROME

H syndrome is a systemic inherited histiocytosis, with characteristic cutaneous findings accompanying systemic manifestations. H syndrome refers to the major clinical findings of hyperpigmentation, hypertrichosis, hepatosplenomegaly, heart anomalies, hearing loss, hypogonadism, low height, and occasionally, hyperglycemia/diabetes mellitus. Due to overlapping clinical features, H syndrome is now considered to include pigmented hypertrichosis with insulin dependent diabetes mellitus syndrome (PHID), Faisalabad histiocytosis (FHC) and familial sinus histiocytosis with massive lymphadenopathy (FSHML).

H SYNDROME Is also known as sinus histiocytosis and massive lymphadenopathy|hjcd|shml|hyperpigmentation, cutaneous, with hypertrichosis, hepatosplenomegaly, heart anomalies, and hypogonadism with or without hearing loss|h syndrome|faisalabad histiocytosis|pigmented hypertrichosis wi

Related symptoms:

  • Global developmental delay
  • Short stature
  • Generalized hypotonia
  • Hearing impairment
  • Growth delay


SOURCES: MESH ORPHANET OMIM MENDELIAN

More info about H SYNDROME

Cardiofaciocutaneous (CFC) syndrome is a RASopathy characterized by craniofacial dysmorphology, congenital heart disease, dermatological abnormalities (most commonly hyperkeratotic skin and sparse, curly hair), growth retardation and intellectual disability.

CARDIOFACIOCUTANEOUS SYNDROME Is also known as cfcs|cfc syndrome

Related symptoms:

  • Intellectual disability
  • Seizures
  • Global developmental delay
  • Short stature
  • Generalized hypotonia


SOURCES: ORPHANET OMIM MENDELIAN

More info about CARDIOFACIOCUTANEOUS SYNDROME

X-linked dominant chondrodysplasia Chassaing-Lacombe type is a rare genetic bone disorder characterized by chondrodysplasia, intrauterine growth retardation (IUGR), hydrocephaly and facial dysmorphism in the affected males.

X-LINKED DOMINANT CHONDRODYSPLASIA, CHASSAING-LACOMBE TYPE Is also known as x-linked dominant chondrodysplasia-hydrocephaly-microphthalmia syndrome

Related symptoms:

  • Intellectual disability
  • Short stature
  • Growth delay
  • Micrognathia
  • Low-set ears


SOURCES: OMIM ORPHANET MENDELIAN

More info about X-LINKED DOMINANT CHONDRODYSPLASIA, CHASSAING-LACOMBE TYPE

Some ectodermal dysplasias are here classified as congenital disorders characterized by abnormal development in 2 or more ectodermal structures (hair, nails, teeth, and sweat glands) without other systemic findings.Hypohidrotic, or anhidrotic, ectodermal dysplasia is characterized by a triad of signs comprising sparse hair (hypotrichosis), abnormal or missing teeth (anodontia or hypodontia), and inability to sweat (anhidrosis or hypohidrosis). Typical clinical manifestations also include dryness of the skin, eyes, airways, and mucous membranes presumably due to the defective development of several exocrine glands. Hypohidrotic ectodermal dysplasia can be associated with dysmorphic features (forehead bumps, rings under the eyes, everted nose, and prominent lips) and occasionally with absent nipples (summary by Cluzeau et al., 2011).For a discussion of genetic heterogeneity of hypohidrotic/anhidrotic ectodermal dysplasia, see {305100}.

ECTODERMAL DYSPLASIA 10B, HYPOHIDROTIC/HAIR/TOOTH TYPE, AUTOSOMAL RECESSIVE; ECTD10B Is also known as eda|ectodermal dysplasia, hypohidrotic|hed|ectodermal dysplasia, anhidrotic

Related symptoms:

  • Depressed nasal bridge
  • Hypertension
  • Frontal bossing
  • Hyperhidrosis
  • Hyperkeratosis


SOURCES: OMIM MENDELIAN

More info about ECTODERMAL DYSPLASIA 10B, HYPOHIDROTIC/HAIR/TOOTH TYPE, AUTOSOMAL RECESSIVE; ECTD10B

JACKSON-WEISS SYNDROME; JWS Is also known as craniosynostosis, midfacial hypoplasia, and foot abnormalities

Related symptoms:

  • Intellectual disability
  • Hypertelorism
  • Strabismus
  • Cleft palate
  • Ptosis


SOURCES: MESH OMIM MENDELIAN

More info about JACKSON-WEISS SYNDROME; JWS

Top 5 symptoms//phenotypes associated to Frontal bossing and Ichthyosis

Symptoms // Phenotype % cases
Short stature Common - Between 50% and 80% cases
Intellectual disability Common - Between 50% and 80% cases
Hyperkeratosis Common - Between 50% and 80% cases
Global developmental delay Common - Between 50% and 80% cases
Alopecia Common - Between 50% and 80% cases

Other less frequent symptoms

Patients with Frontal bossing and Ichthyosis. may also develop some of the following symptoms:

Common Symptoms - More than 50% cases

Growth delay

Uncommon Symptoms - Between 30% and 50% cases

Depressed nasal bridge Seizures Hearing impairment Hypotrichosis Ptosis Epicanthus Cerebral cortical atrophy Abnormality of the kidney Hypertelorism Sparse hair Hydrocephalus Microphthalmia Ventriculomegaly Hernia Muscular hypotonia Malar flattening Failure to thrive Scoliosis Abnormality of the dentition Flexion contracture Cleft palate Erythema Hydronephrosis Nystagmus Plagiocephaly Hepatomegaly Edema Clinodactyly of the 5th finger Hyperhidrosis Abnormality of the nervous system Delayed skeletal maturation Corneal opacity Talipes Palmoplantar hyperkeratosis Depressed nasal ridge Ectodermal dysplasia Erythroderma Abnormality of the skeletal system Thick vermilion border Macrocephaly Camptodactyly Abnormal heart morphology Sparse eyelashes Ventricular septal defect Feeding difficulties Strabismus Epidermal acanthosis Generalized hypotonia Scaling skin Kyphosis Severe short stature Wide nasal bridge Kyphoscoliosis Rhizomelia Dry skin Abnormality of epiphysis morphology Intellectual disability, severe Atrial septal defect Flat face Proptosis Micrognathia Absent eyebrow Sensorineural hearing impairment Cataract

Rare Symptoms - Less than 30% cases

Patchy alopecia Posteriorly rotated ears Cerebral atrophy Wide mouth Autism Prominent forehead Brachydactyly Low-set, posteriorly rotated ears Abnormal cardiac septum morphology Pectus carinatum Short philtrum Full cheeks Psoriasiform dermatitis Abnormality of hair texture Polyhydramnios Sleep apnea Polydactyly Type I diabetes mellitus Aspiration Hyperpigmentation of the skin Enlarged kidney Abnormality of the pinna Congenital ichthyosiform erythroderma Cardiomegaly Postaxial polydactyly Abnormal vertebral morphology Sparse and thin eyebrow Hemivertebrae Hip dislocation Growth hormone deficiency Leukemia Short palm Splenomegaly Alopecia of scalp Cerebellar hypoplasia Absent eyelashes Abnormal eyelash morphology Macrotia Depressivity Abnormal palate morphology Respiratory tract infection Scarring Inguinal hernia Nail dystrophy Genu valgum Platyspondyly Submucous cleft hard palate Astigmatism Hydroureter Palmoplantar keratoderma Hypohidrosis Heat intolerance Intrauterine growth retardation Abnormality of the nail Osteolysis Intellectual disability, mild Short foot Hypodontia Anteverted nares Low-set ears Webbed neck Microdontia Fine hair Underdeveloped supraorbital ridges Episcleritis Brittle hair Keratitis Large for gestational age Hypoplastic nipples Aplasia/Hypoplasia of the eyebrow Short nose Cryptorchidism Myopia Umbilical hernia Conductive hearing impairment Midface retrusion Dandy-Walker malformation EEG abnormality Telecanthus Abnormality of the eye Coloboma Recurrent fractures Nevus Overgrowth Agenesis of corpus callosum Coarctation of aorta Aplasia/Hypoplasia of the corpus callosum Hemangioma Abnormality of vision Melanocytic nevus Abnormality of dental morphology Osteopenia Short neck Biparietal narrowing Limb undergrowth Microcephaly High palate Upslanted palpebral fissure Pulmonic stenosis Pulmonary hypoplasia Short distal phalanx of finger Sparse body hair Syndactyly Epiphyseal stippling Concave nasal ridge Delayed CNS myelination Neoplasm Hyperreflexia Talipes equinovarus Blindness Neurofibromas Dilatation Cavernous hemangioma Optic atrophy Downslanted palpebral fissures Abnormal mitral valve morphology Gastroesophageal reflux Dolichocephaly Pruritus Short metatarsal Neurological speech impairment Irritability Feeding difficulties in infancy Hypertrophic cardiomyopathy Aggressive behavior Coarse facial features Constipation High forehead Acanthosis nigricans Encephalopathy Pectus excavatum Thrombocytopenia Abnormality of cardiovascular system morphology Long philtrum 2-3 toe syndactyly Broad hallux Behavioral abnormality Congestive heart failure Hypertonia Vomiting Abnormality of the cerebral white matter Hypermetropia Optic nerve dysplasia Retinal dystrophy Dental malocclusion Headache Premature birth Intestinal malrotation Vesicoureteral reflux Abnormal bleeding Abdominal distention Sleep disturbance High, narrow palate Mandibular prognathia Hepatic steatosis Bruising susceptibility Dysphagia Craniosynostosis Abnormal location of ears Bulbous nose Falls Joint hypermobility Long face Abnormality of skin pigmentation Peripheral axonal neuropathy Abnormality of the hairline Hypoplasia of the maxilla Convex nasal ridge Hyperkeratosis pilaris Cardiomyopathy Delayed speech and language development Dysarthria Abnormal eyebrow morphology Abnormality of fibula morphology Reticulocytopenia Histiocytosis Skin nodule Nasal obstruction Recurrent pharyngitis Anterior plagiocephaly Decreased serum testosterone level Communicating hydrocephalus Varicose veins Aspiration pneumonia Craniofacial dysostosis Abnormality of cardiovascular system physiology Exocrine pancreatic insufficiency Severe sensorineural hearing impairment Laryngeal cleft Episodic fever Broad metatarsal Polycythemia Scleroderma Hallux varus Lipoatrophy Microcytic anemia Elevated erythrocyte sedimentation rate Chronic rhinitis Pancreatic hypoplasia Progressive visual loss Cervical lymphadenopathy Shallow orbits Split foot Hypoplasia of the frontal lobes Turricephaly Tongue thrusting Ataxia Seborrheic keratosis Retroperitoneal fibrosis Generalized ichthyosis Upper eyelid edema Bilateral camptodactyly Preaxial foot polydactyly Broad finger Stiff skin Snoring Panniculitis Facial telangiectasia Functional abnormality of the gastrointestinal tract Myelofibrosis Broad hallux phalanx Generalized lymphadenopathy Symphalangism affecting the phalanges of the hand Corneal arcus Hyperplasia of the maxilla Periorbital hyperpigmentation Abnormality of the cardiovascular system Gastrointestinal dysmotility Hypoplasia of the zygomatic bone Abnormal aortic valve morphology Hyperextensibility of the finger joints Short attention span Thin skin Hoarse voice Oligodontia Abnormality of the gastrointestinal tract Abnormality of the ulna Anhidrosis Abnormality of the pulmonary artery Premature skin wrinkling Arnold-Chiari type I malformation Everted lower lip vermilion Atopic dermatitis Agenesis of permanent teeth Poor appetite Excessive wrinkled skin Anal stenosis Eyelid fasciculation Generalized hyperpigmentation Curly hair Multiple lentigines Multiple cafe-au-lait spots Abnormal heart valve morphology Delayed eruption of teeth Subvalvular aortic stenosis Multiple palmar creases Decreased skull ossification Oral aversion Deep palmar crease Abnormality of refraction Abnormal myocardium morphology Abnormality of the testis Abnormality of the optic nerve Dystrophic fingernails Abnormal hair pattern Thick upper lip vermilion Thin ribs Hypoplastic iliac wing 11 pairs of ribs Hypertension Metaphyseal chondrodysplasia Severe platyspondyly Metaphyseal cupping of metacarpals Distal shortening of limbs Slow-growing hair Woolly hair Multiple plantar creases Hallux valgus Abnormality of the calcaneus Metaphyseal cupping of proximal phalanges Hypoplasia of the calcaneus Thickened helices Soft skin Long palpebral fissure Narrow forehead Abnormality of the optic disc Everted upper lip vermilion Endocarditis Frontal balding Inappropriate crying Cutis laxa Oculomotor apraxia Cutaneous T-cell lymphoma Morphological abnormality of the gastrointestinal tract Narrow palate Cerebral visual impairment Anterior creases of earlobe Abnormal tricuspid valve morphology Delayed gross motor development Puberty and gonadal disorders Lymphedema Cafe-au-lait spot Thickened skin Decreased body weight Open mouth Inflammatory abnormality of the skin Hemiparesis Periorbital wrinkles Myocardial infarction Low posterior hairline Abnormality of the genitourinary system Abnormality of the auditory canal Abnormality of the sternum Pleural effusion Infantile spasms Anodontia Neurodevelopmental delay Anterior hypopituitarism Malnutrition Open bite Obsessive-compulsive behavior Heart murmur Chronic otitis media Cubitus valgus Ectropion Sparse eyebrow Increased nuchal translucency Bilateral ptosis Failure to thrive in infancy Redundant skin Deep philtrum Absent nipple Anhidrotic ectodermal dysplasia Hypohidrotic ectodermal dysplasia Hyperextensible skin Poor suck Relative macrocephaly Optic nerve hypoplasia Sparse or absent eyelashes Increased antibody level in blood Uveitis Leukocytosis Coarse hair Vertebral wedging Hemiatrophy Scarring alopecia of scalp Butterfly vertebrae Dry hair Tracheal stenosis Patellar dislocation Foot polydactyly Aplasia/Hypoplasia of the skin Bilateral talipes equinovarus Abnormality of the thorax Abnormality of pelvic girdle bone morphology Tracheal calcification Abnormality of the fingernails Joint dislocation Hip dysplasia Esotropia Microcornea Postnatal growth retardation Intellectual disability, moderate Skeletal dysplasia Glaucoma Nevus sebaceus Linear nevus sebaceous Odontogenic neoplasm Hyperkeratosis with erythema Punctate vertebral calcifications Abnormality of toe Abnormality of the outer ear Retinal coloboma Long foot Acute leukemia Acute lymphoblastic leukemia Increased number of teeth Transposition of the great arteries Decreased fertility Large hands Overfolded helix Growth abnormality Recurrent skin infections Widely spaced teeth Elevated 8-dehydrocholesterol Abnormal dermatoglyphics Skin ulcer Joint contracture of the hand Tall stature Broad-based gait Thick lower lip vermilion Tetralogy of Fallot Bifid uvula Brachycephaly Stippled calcification in carpal bones Tarsal stippling Elevated 8(9)-cholestenol Odontoma Nevus sebaceous Aplasia/Hypoplasia of the nipples Congenital contracture Ophthalmoplegia Attention deficit hyperactivity disorder Carcinoma Pregnancy exposure Calcific stippling of infantile cartilaginous skeleton Bilateral cleft palate Coronal cleft vertebrae Multiple epiphyseal dysplasia Polysplenia Severe failure to thrive Flared metaphysis Epiphyseal dysplasia Iris coloboma Spina bifida occulta Abnormality of the metaphysis Congenital diaphragmatic hernia Limitation of joint mobility Polymicrogyria Congenital cataract Severe global developmental delay Cleft lip Abnormality of metabolism/homeostasis Respiratory insufficiency Spasticity Pain Facial asymmetry Hypopigmentation of the skin Epidermal nevus Dilatation of the cerebral artery Hemimegalencephaly Cranial asymmetry Abnormality of dental color Adenoma sebaceum Asymmetric growth Porencephalic cyst Hypophosphatemic rickets Hyperphosphaturia Hemihypertrophy Abnormality of finger Gangrene Genu recurvatum Irregular hyperpigmentation Generalized muscle weakness Osteomalacia Basal cell carcinoma Vertebral segmentation defect Prominent occiput Aplasia/Hypoplasia of the cerebellum Rickets Precocious puberty Reduced tendon reflexes Horseshoe kidney Exotropia Pachygyria Cerebral calcification Peripheral pulmonary artery stenosis Ureteropelvic junction obstruction Hyperglycemia Ichthyosis follicularis Dyspnea Hypothyroidism Micropenis Diabetes mellitus Hypogonadism Pneumonia Patent ductus arteriosus Clinodactyly Fever Anemia Unilateral chest hypoplasia Abnormal pelvis bone morphology Hepatosplenomegaly Thin fingernail Scleritis Hypoplasia of the bladder Cerebral cortical hemiatrophy Paronychia Hyperconvex fingernails Abnormality of temperature regulation Abnormal nasolacrimal system morphology Subcortical cerebral atrophy Corneal scarring Abnormality of the upper urinary tract Cheilitis Pes planus Apnea Oxycephaly Hypertriglyceridemia Stridor Lipodystrophy Azoospermia Elbow flexion contracture Hypergonadotropic hypogonadism Gynecomastia Gingival overgrowth Telangiectasia Bronchiectasis Primary amenorrhea Hypertrichosis Blue sclerae Retinopathy Epistaxis Bilateral sensorineural hearing impairment Mitral valve prolapse Amenorrhea Decreased testicular size Wide intermamillary distance Polyneuropathy Cleft upper lip Lymphadenopathy Abnormality of the foot Delayed puberty Malabsorption Alopecia universalis Thin eyebrow Aplastic clavicle Photophobia Abnormality of the ribs Oligohydramnios Eczema Postaxial hand polydactyly Specific learning disability Nail dysplasia Brain atrophy Papule Camptodactyly of finger Developmental regression Mental deterioration Dementia Omphalocele Recurrent respiratory infections Recurrent infections Immunodeficiency Hypoplasia of the corpus callosum Respiratory distress Low-set nipples Aplasia/Hypoplasia of the phalanges of the toes Violent behavior Pulmonary valve atresia Aplasia/Hypoplasia of the phalanges of the hand Clubbing of toes Duplicated collecting system Choanal atresia Aganglionic megacolon Alopecia totalis Absent septum pellucidum Olivopontocerebellar atrophy Recurrent corneal erosions Hypoplastic fingernail Follicular hyperkeratosis Corneal erosion Abnormal eyelid morphology Blepharitis Parakeratosis Oligodactyly Ectrodactyly Atonic seizures Abnormality of the vertebral column Mixed hearing impairment Renal dysplasia Intestinal obstruction Bifid scrotum Unilateral renal agenesis Urticaria Abnormality of the hand Opacification of the corneal stroma Abnormality of dental enamel Conjunctivitis Recurrent bacterial infections Multicystic kidney dysplasia Abnormality of the hair Hypoplasia of dental enamel Calcaneonavicular fusion


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