Section I - The DiseaseThe Epidemiology and Etiology of Perthes Disease
Section snippets
Geography
There is a marked geographic variation in the frequency of Perthes disease. Studies of incidence are difficult to directly compare, as inappropriate population denominators have sometimes been used. Table 1 shows those studies where the annual incidence rates have been calculated using a denominator of 0- to 14-year-old child years.
There is a significant geographic variation in disease incidence between countries, within countries, and even between small local areas. Equatorial regions have a
Time trends
Few studies have sought to measure the influence of time on disease incidence. Between 1924 and 1960, Peic18 reviewed outpatient data from an orthopedic clinic in Dortmund, Germany. He noted that the incidence of cases tended to increase 5 years after a period of economic recession. However, his method of case ascertainment and definition of source population raises doubts as to the robustness of this association. A Scottish study similarly noted an association between “gross value added” (a
Stature
The most detailed observation of growth in Perthes disease was published in 1978.25 This large, cross-sectional, anthropometric study involved 232 children with Perthes disease from 3 centers in the United Kingdom. A control group was drawn from primary schools in 2 of the 3 regions. The results demonstrated a subtle global growth disturbance in affected individuals with the demonstration of a normal head size, yet increasing growth restriction as one progressed distally along limb units, such
Hypotheses
The clear association with growth and the potential for an intrauterine effect have led to two specific hypotheses: nutritional deficiency and parental smoking.
Manganese deficiency has so far been investigated because of the known consequences of growth abnormalities in chickens, and epiphyseal dysplasia in rats. An initial case-control study amongst children with Perthes disease found an association,31 but this was not confirmed in a second smaller study nor by supplementation after disease
Summary
Perthes disease, despite a century of research, remains of unknown etiology. However, any hypothesis must fit into the clear epidemiologic framework that we have. The disease is associated with deprived populations, and is much more common in white children than in Asians or Africans. It is associated with a global growth disorder characterized by delayed and disproportionate growth. The causal insult appears to occur very early in a child’s life, with early changes in skeletal development
References (35)
- et al.
Multicenter study for Legg-Calvé-Perthes disease in Japan
J Orthop Sci
(2006) - et al.
Association of Perthes’ disease with congenital anomalies of genitourinary tract and inguinal region
Lancet
(1971) - et al.
The epidemiology of Perthes’ disease in south India
Int J Epidemiol
(1988) - et al.
Incidence of Legg-Perthes disease (osteochondritis deformans)
N Engl J Med
(1966) - et al.
Incidence and genetics of Legg-Perthes disease (osteochondritis deformans) in British Columbia: evidence of polygenic determination
J Med Genet
(1972) - et al.
Perthes’ disease of the hip in three regions of England
J Bone Joint Surg Br
(1978) The incidence of Perthes’ disease in three population groups in the Eastern Cape region of South Africa
J Bone Joint Surg Br
(1982)- et al.
Perthes’ disease of the hip in Liverpool
Br Med J (Clin Res Ed)
(1983) - et al.
Perthes’ disease in Yorkshire
J Bone Joint Surg Br
(1989) - et al.
Incidence of Perthes’ disease in Uppsala, Sweden
Acta Orthop Scand
(1992)
Deprivation, urbanisation and Perthes’ disease in Northern Ireland
J Bone Joint Surg Br
The incidence and distribution of Legg-Calvé-Perthes’ disease in Liverpool, 1982–95
Arch Dis Child
The incidence of Perthes’ disease in Korea: a focus on differences among races
J Bone Joint Surg Br
The incidence of Perthes’ disease in Southwest Scotland
J Bone Joint Surg Br
The epidemiology and aetiology of Perthes’ disease in Norway. A nationwide study of 425 patients
J Bone Joint Surg Br
Legg-Calve-Perthes disease: a statistical evaluation of 358 cases
Mt Sinai J Med
The social origins of Perthes’ disease of the hip
Paediatr Perinat Epidemiol
Cited by (51)
Towards a definition of Ancient Rare Diseases (ARD): Presenting a complex case of probable Legg-Calvé-Perthes Disease from the North Caucasian Bronze Age (2200-1650 cal BCE)
2021, International Journal of PaleopathologyCitation Excerpt :A similar trend has been discovered for Scotland and Britain (Perry et al., 2012). A gradual decline through time is thought to be associated with a general improvement in wellbeing that seems to be especially distinct in regions that were socioeconomically deprived (Perry, 2014), or in times where exposure to passive smoke inhalation was more common. Hence, in general, LCPD prevalence must be understood as a dynamic phenomenon.
The diagnosis and management of common childhood orthopedic disorders: An update
2020, Current Problems in Pediatric and Adolescent Health CareCitation Excerpt :The reported annual incidence ranges between 0.2 – 32 per 100,000. The incidence is higher in males and in the Caucasian population.139-141 Patients generally present between 4 and 8 years of age with pain and or with limp or abnormal gait.
Interleukin-6 deletion stimulates revascularization and new bone formation following ischemic osteonecrosis in a murine model
2018, BoneCitation Excerpt :LCPD affects 1 in 1200 children and has a regional annual incidence ranging from 5 to 15 per 100,000 children. It is 4–5 times more common in boys than in girls with an occurrence rate of 1 in 740 boys and 1 in 3500 girls [1,2]. Patients with LCPD suffer from hip pain and stiffness, permanent femoral head deformity, and early osteoarthritis.
Case study of Legg-Calvé-Perthes disease observed in Radom (Poland, 18th–19th century) with literature review
2023, Anthropological Review
The authors have nothing to disclose.