Progressive Osteolysis in Progeria

Ozonoff, M. B.; Clemett, Arthur R.

doi:10.2214/ajr.100.1.75

Cited by 35 publications

(17 citation statements)

References 0 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The commonest cause of death is myocardial infarction and survival beyond the second decade is rare. The radiological features, which consist of hypoplastic facial bones, delay of cranial suture and fontanelle closure, thin short clavicles, coxa valgus, and progressive acro-osteolysis of the terminal phalanges have been recently reviewed by Margolin and Steinbach (1968), Ozonoff and Clemett (1967), and Macleod (1966).…”

mentioning

confidence: 99%

Progeria: Case Report with Long-term Studies of Serum Lipids

Machamara¹,

Farn²,

Mitra³

et al. 1970

Archives of Disease in Childhood

View full text Add to dashboard Cite

A. S. (1970). Archives of Disease in Childhood, 45, 553. Progeria: case report with long-term studies of serum lipids. A girl with the typical features of progeria died at the age of 10 years from cardiac failure following repeated myocardial infarctions. Serial studies of the serum lipids were made from the age of 2 years 3 months. Variable hyperlipidaemia, increased f,-and pre-fl-ipoproteins, and impaired clearing of absorbed dietary fat were shown. A polyunsaturated fat diet, which was started at the age of 2 years 5 months and continued throughout the rest of her life, controlled the hyperlipidaemia and the clearing of fat became normal. Pre-,B-lipoprotein could still, however, be seen occasionally. The dietary regimen did not delay the development of atherosclerosis, the pathogenesis of which, in progeria, may be due to factors other than hyperlipidaemia.Progeria was first described by Hutchinson in 1886 and named by Hastings Gilford in 1897. The term means prematurely old. Individuals appear normal at birth and weight gain is usually satisfactory during the first year of life, but thereafter virtually ceases. Growth in height is retarded and rarely exceeds that of a normal 4-or 5-year-old. Intelligence is normal. The appearance has been likened to a 'plucked bird' or very old man because of baldness and lack of subcutaneous fat; the skin is atrophic and often shows brown pigmentation. The nails are atrophic or absent. The eyes are prominent and the nose beaked; the chin recessive and the maxilla hypoplastic. The chest is narrow and the abdomen protrudes. Clinical manifestations of osteoarthritis and arteriosclerosis may appear before the sixth year. The commonest cause of death is myocardial infarction and survival beyond the second decade is rare. The radiological features, which consist of hypoplastic facial bones, delay of cranial suture and fontanelle closure, thin short clavicles, coxa valgus, and progressive acro-osteolysis of the terminal phalanges have been recently reviewed by Margolin and Steinbach (1968), Ozonoff and Clemett (1967), and Macleod (1966.

show abstract

mentioning

confidence: 99%

Progeria: Case Report with Long-term Studies of Serum Lipids

Machamara¹,

Farn²,

Mitra³

et al. 1970

Archives of Disease in Childhood

View full text Add to dashboard Cite

show abstract

“…One form presents with thinning and disappearance of bone (11) and must be differentiated from other types of vanishing bone disease such as the disappearing ribs in recumbent patients (12,13) and the disappearing bones of progeria (14), hemangiomatosis, or nephropathy (15). The thin bones of neurofibromatosis or congenital pseudarthrosis must also be differentiated.…”

Section: Discussionmentioning

confidence: 99%

Lymphangiomatosis of Bone

Steiner¹,

Farman²,

Lawson³

1969

Radiology

View full text Add to dashboard Cite

“…These children possess various physical characteristics which resemble those of an elderly individual. The general physical characteristics are agedlooking skin, conspicuous veins running through the head, short stature, alopecia, craniofacial disproportion, high-pitched voice, micrognathia, dystropic nails, skeletal hypoplasia and dysplasia, beak-like nose, coxa valga, decreased subcutaneous fat, thin limbs with prominent stiff joints, atrophic skin, horse-riding stance, sclerodermoid lesions, delayed dentition, mottling hyperpigmentation, prominent eyes, delayed closure of fontanelles and sutures, protruding ears with absence of earlobes, faint midfacial cyanosis, flares metaphyses, hypoplastic nipples, decreased sweating, and easy bruising (Hutchinson, 1886;Talbott et al, 1945;Manschot, 1950;Thomson and Forfar, 1950;Cooke, 1953;Album and Hope, 1958;Ozonoff and Clemett, 1967;Fleischmajer and Nedwich, 1973;Csoka et al, 2004;Gordon et al, 2007;Progeria Research Foundation, 2009;Surjushe et al, 2009). There is a rate of > 2 SD below normal weight gain for individuals with HGPS at any corresponding age (Gordon et al, 2007).…”

Section: Traits Of Hgpsmentioning

confidence: 99%

Hutchinson-Gilford Progeria Syndrome: Its Presentation in F. Scott Fitzgerald’s Short Story ‘The Curious Case of Benjamin Button’ and Its Oral Manifestations

Maloney

2009

J Dent Res

View full text Add to dashboard Cite

Hutchinson-Gilford Progeria Syndrome (HGPS) was first documented in the medical literature in 1886. A HGPS patient has the physical characteristics and appearances of an elderly individual. In 1921, F. Scott Fitzgerald published a short story entitled 'The Curious Case of Benjamin Button'. The main character of Fitzgerald's fictional work is born with a very rare condition in which he looks like an elderly person. The main difference between the fictional individual and individuals with HGPS is that Fitzgerald's character becomes younger as the years go by. This paper serves three purposes. The first purpose is to scientifically present the possibility that Fitzgerald consciously based his character, Benjamin Button, upon individuals with HGPS. The second purpose is to describe the rare condition of HGPS, along with its many manifestations in the head and neck region. The third purpose is to postulate that HGPS individuals might not only have the appearance of an aged person, but also might actually undergo true physical aging, which would enable researchers to gain valuable information into the treatment of ailments commonly associated with the natural process of aging.

show abstract

Progressive Osteolysis in Progeria

Cited by 35 publications

References 0 publications

Progeria: Case Report with Long-term Studies of Serum Lipids

Progeria: Case Report with Long-term Studies of Serum Lipids

Lymphangiomatosis of Bone

Hutchinson-Gilford Progeria Syndrome: Its Presentation in F. Scott Fitzgerald’s Short Story ‘The Curious Case of Benjamin Button’ and Its Oral Manifestations

Contact Info

Product

Resources

About