ODCs

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Buschke-Ollendorff syndrome

1 OMIM reference -
1 associated gene
33 signs/symptoms

PROTEIN INTERACTIONS: 1
COMMON SIGNS: 4

Hutchinson-Gilford progeria syndrome

1 OMIM reference -
2 associated genes
43 signs/symptoms

Buschke-Ollendorff syndrome

Hutchinson-Gilford progeria syndrome

LEMD3	(UniProt - OMIM)		LMNA	(UniProt - OMIM)
			ZMPSTE24	(UniProt - OMIM)


INTERACTOME ASSOCIATIONS (click on a score value to see the evidence)	LEMD3	(0.49)	LMNA

Citations in the biomedical literature:

Buschke-Ollendorff syndrome		Hutchinson-Gilford progeria syndrome
	Synonym(s): - Disseminated dermatofibrosis with osteopoikilosis		Synonym(s): - Progeria

	Classification (Orphanet): - Rare bone disease - Rare developmental defect during embryogenesis - Rare genetic disease - Rare skin disease		Classification (Orphanet): - Rare bone disease - Rare developmental defect during embryogenesis - Rare eye disease - Rare genetic disease - Rare skin disease

	Classification (ICD10): - Congenital malformations, deformations and chromosomal abnormalities -		Classification (ICD10): - Endocrine, nutritional and metabolic diseases -

	Epidemiological data: Class of prevalence: 1-9 / 100 000 Average age onset: variable Average age of death: normal Type of inheritance: autosomal dominant		Epidemiological data: Class of prevalence: <1 / 1 000 000 Average age onset: neonatal/infancy Average age of death: - Type of inheritance: autosomal dominant

	External references: 1 OMIM reference - 1 MeSH reference: C537415		External references: 1 OMIM reference - 1 MeSH reference: D011371


COMMON SIGNS	- Abnormal cry / voice / phonation disorder / nasal speech - Articular / joint pain / arthralgia - Dysostosis / chondrodysplasia / osteodysplasia / osteochondrosis / skeletal dysplasia - Short stature / dwarfism / nanism

Buschke-Ollendorff syndrome		Hutchinson-Gilford progeria syndrome
	Very frequent - Abnormal dentition / dental position / implantation / unerupted / dental ankylosis - Anomalies of bones / skeletal anomalies - Autosomal dominant inheritance - Bone pain - Diabetes mellitus - Diffuse / generalised skin hypopigmentation / cutaneous albinism - Epiphyseal anomaly - Follicular / erythematous / edematous papules / milium - Hearing loss / hypoacusia / deafness - Hyperostosis - Hypoplastic aorta / coarctation / stenosis / anomaly / aortic arch interruption - Metaphyseal anomaly - Nasal congestion / sinusitis / rhinitis / rhinorrhea - Osteosclerosis / osteopetrosis / bone condensation - Sarcoma - Visual loss / blindness / amblyopia Frequent - Mediastinal / hilar adenopathies - Strabismus / squint Occasional - Abnormal scarring / cheloids / hypertrophic scars - Arthritis / synovitis / synovial proliferation - Contractures / cramps / trismus / tetania / claudication / opisthotonos - Diaphyseal anomaly - Insulin-dependent / type 1 diabetes - Lateral cleft lip / gingival cleft / paramedian nasal cleft - Mutiple fractures / bone fragility - Myalgia / muscular pain - Palmoplantar hyperkeratosis / keratoderma - Pigmented naevi / naevus pigmentosus / lentigo - Subcutaneous nodules / lipomas / tumefaction / swelling		Very frequent - Abnormal fat distribution / lipodystrophy - Alopecia - Anodontia / oligodontia / hypodontia - Asthenia / fatigue / weakness - Decreased body hair / axillar / pubic hairlessness - Delayed dentition / eruption of teeth / lack of eruption of teeth - Early death / lethality - Failure to thrive / difficulties for feeding in infancy / growth delay - Hypotrichosis / atrichia / atrichiasis / scalp hairlessness - Late puberty / hypogonadism / hypogenitalism - Micrognathia / retrognathia / micrognathism / retrognathism - Narrow face - Premature ageing - Proptosis / exophthalmos - Skull / cranial anomalies - Terminal / third phalangeal bone of fingers hypoplasia - Thin skin - Thin / hypoplastic toenails - Thin / hypoplastic / hyperconvex fingernails - Weight loss / loss of appetite / break in weight curve / general health alteration Frequent - Abnormal gait - Abnormal pigmentary skin changes / skin pigmentation anomalies - Acrocyanosis / Raynaud's phenomenon / vasomotor disorders - Angor pectoris / myocardial infarction - Anomalies of eyelids, eyelashes and lacrimal system - Anomalies of teeth and dentition - Arterial atheroma / precocious atherosclerosis / arteriosclerosis - Beaked nose - Clavicle absent / abnormal - External ear anomalies - Global upper and lower limbs anomalies - Hip dislocation / dysplasia / coxa valga / coxa vara / coxa plana - Large fontanelle / delayed fontanelle closure - Osteolysis / osteoclasia / bone destruction / erosions - Osteoporosis / osteopenia / demineralisation / osteomalacia / rickets - Rippled skin - Thin / retracted lips - Tight skin / lack of elasticity Occasional - Nephrosclerosis