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

  title={Hutchinson-Gilford Progeria Syndrome: Its Presentation in F. Scott Fitzgerald’s Short Story ‘The Curious Case of Benjamin Button’ and Its Oral Manifestations},
  author={William James Maloney},
  journal={Journal of Dental Research},
  pages={873 - 876}
  • W. Maloney
  • Published 1 October 2009
  • Medicine
  • Journal of Dental Research
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… 
Hutchinson-Gilford Progeria Syndrome: Case Report
A 17-years-old male patient with diagnosis of Progeria sent to the clinic for dental treatments by genetic consultation from Department of Pediatric Endocrine of Istanbul Faculty of Medicine with features of highly suggestive HGPS is monitorized both clinically and radiographgically.
Hutchinson-Gilford Progeria Syndrome (HGPS): relevant aspects of a rare syndrome diagnosed in a Brazilian child
Among the oral and facial findings is remarkable the delay in tooth eruption, the micrognathia and the disproportion between the skull and face in Hutchinson-Gilford Progeria Syndrome.
Hutchinson-Gilford progeria syndrome - A brief introduction
The symptoms of progeria may include alopecia (hair loss), low body weight, decrease joint motility, facial appearance that are similar to old age person and accelerated cardiovascular disease.
A Case Report of Hutchinson-Gilford Progeria Syndrome
The studied patient was older than the global average with no cardiovascular disease and suffered from appendicitis, so scientists are interested in identifying this syndrome, as it may reveal the signs of a natural aging process at an early age.
Dental and craniofacial characteristics in a patient with Hutchinson–Gilford progeria syndrome
The dental and craniofacial characteristics of a 9-year-old girl with HGPS are presented, the first report addressing orthodontic tooth movement and microbiological features in a HGPS patient, and pathologic findings are described and discussed.
Simultaneous shoulder and hip dislocation in a 12-year-old girl with Hutchinson-Gilford progeria syndrome.
A patient with Hutchinson-Gilford progeria syndrome had simultaneous shoulder and hip dislocation associated with a low energy trauma and treatment accomplished as close reduction under general anesthesia and immobilization.
Talking About Rare Diseases
This chapter discusses rare (and orphan) diseases, what has been reported about them, and how they appear to the general public.


Progeria (Hutchinson-Gilford Syndrome)
There has been no typical case of progeria reported in British literature since the original two cases, although Parsons (1949) and IsIIt IC 4 LLc 4JJ ACJ (YI ) l& Usc a typical progerian skull.
Hutchinson–Gilford progeria syndrome: Review of the phenotype
  • R. Hennekam
  • Medicine
    American journal of medical genetics. Part A
  • 2006
Patients can be subdivided in patients with classical HGPS, which follows an autosomal dominant pattern of inheritance, all cases representing spontaneous mutations, and in non‐classical progeria, in whom growth can be less retarded, scalp hair remains present for a longer time, lipodystrophy is more slowly progressive, osteolysis is more expressed except in the face, and survival well into adulthood.
A metabolic study was undertaken on a 6 year old boy with characteristic progeria, a member of an essentially healthy family comprised of the parents.
The Hutchinson-Gilford Progeria Syndrome: a case report.
The HGPS (Hutchinson Gilford Progeria Syndrome) is a rare genetic disorder with an incidence of 1 per 8 million live births and the cause is identified as a defect in the LMNA gene of chromosome 1.
Research on Hutchinson-Gilford progeria syndrome.
  • H. Warner
  • Biology, Medicine
    The journals of gerontology. Series A, Biological sciences and medical sciences
  • 2008
It is argued that progeroid syndromes, including HGPS, ‘‘might have partial mechanistic overlap with normal aging and therefore might provide uniquely informative opportunities to formulate and test hypotheses regarding the biology of aging and age-dependent disease’’.
Recurrent de novo point mutations in lamin A cause Hutchinson–Gilford progeria syndrome
Evidence of mutations in lamin A (LMNA) as the cause of Hutchinson–Gilford progeria syndrome is presented, and the discovery of the molecular basis of this disease may shed light on the general phenomenon of human ageing.
The rate of growth in progeria
LMNA is mutated in Hutchinson-Gilford progeria (MIM 176670) but not in Wiedemann-Rautenstrauch progeroid syndrome (MIM 264090)
The strong association of rare LMNA coding sequence mutations with HGPS implicates this syndrome as a laminopathy, while WRS is most probably due to mutations in another gene.
Reversible phenotype in a mouse model of Hutchinson–Gilford progeria syndrome
In inducible transgenic animal model of HGPS, expression of the progeria mutation does not cause irreversible damage and that reversal of disease phenotype is possible, which gives promise for a treatment for this disease.