Impacto de la enfermedad de Legg-Calvé-Perthes en un área de salud mediterránea / Impact of Legg-Calve-Perthes disease in a Mediterranean health area

Introducción: Se han relacionado varios factores poblacionales socioeconómicos en la etiología de la enfermedad de Legg-Calvé-Perthes (ELCP), como son su mayor incidencia en población urbana, vivir en la periferia de núcleos urbanos o residir a una determinada latitud con respecto al ecuador terráqueo. Es conocida la incidencia en algunos otros países, pero se desconoce la incidencia del proceso en nuestro medio, hecho importante para acomodar los recursos sociosanitarios necesarios para el tratamiento de la enfermedad. Por todo ello, el objetivo del presente estudio es determinar la incidencia de la ELCP en el Área de Salud 2 de Madrid. Material y métodos: Se analiza la incidencia de ELCP en el Área de Salud 2 de Madrid por ser el área mediterránea una zona poco estudiada. Se trata de una zona con muy escasa movilidad geográfica, de nivel socioeconómico medio-bajo, e integrada en su mayoría por trabajadores de empresas del sector secundario y terciario. Los datos se han obtenido a partir de las bases de datos hospitalarias del Área 2 entre los años 1994 y 2010. Los criterios de inclusión fueron padecer la ELCP y la distribución territorial de la población en el Área 2 de Madrid. Conclusiones: La incidencia de ELCP en esta población mediterránea de un área industrial fue de 1,59 casos por 100.000 habitantes-año, próxima a la encontrada en poblaciones de latitudes similares; pero, sin embargo, se trata de un área industrial con un nivel socioeconómico parecido al de las poblaciones de Reino Unido con una incidencia mucho mayor.(AU)

Introduction: Several socioeconomic population factors have been related to the aetiology of Legg-Calve-Perthes disease (LCPD), such as its higher incidence in the urban population, living on the periphery of urban centres or residing at a certain latitude with respect to the world equator. The incidence in some other countries is known but the incidence of the process in our environment is unknown an important fact to allocate the social and health resources necessary for the treatment of the disease. Therefore, the aim of the present study is to determine the incidence of LCPD in Health Area 2 of Madrid. Material and methods: The incidence of LCPD is analysed in Healthcare Area 2 of Madrid because it is the Mediterranean area that has been scarcely studied. It is an area with very low geographic mobility, a medium-low socioeconomic level, and mostly composed of workers in companies in the secondary and tertiary sectors. The data were obtained from the hospital databases of Area 2 between the years 1994 and 2010. The inclusion criteria were the presence of LCPD and the territorial distribution of the population in Area 2 of Madrid. Conclusions: The incidence of LCPD in this Mediterranean population of an industrial area was 1.59 cases/year and 100,000 inhabitants, close to that found in populations of similar latitudes, but nevertheless it is an industrial area and socioeconomic level similar to United Kingdom populations with a much higher incidence.(AU)

Impact of Legg-Calve-Perthes disease in a Mediterranean health area / Impacto de la enfermedad de Legg-Calvé-Perthes en un área de salud mediterránea

Introduction: Several socioeconomic population factors have been related to the aetiology of Legg-Calve-Perthes disease (LCPD), such as its higher incidence in the urban population, living on the periphery of urban centres or residing at a certain latitude with respect to the world equator. The incidence in some other countries is known but the incidence of the process in our environment is unknown an important fact to allocate the social and health resources necessary for the treatment of the disease. Therefore, the aim of the present study is to determine the incidence of LCPD in Health Area 2 of Madrid. Material and methods: The incidence of LCPD is analysed in Healthcare Area 2 of Madrid because it is the Mediterranean area that has been scarcely studied. It is an area with very low geographic mobility, a medium-low socioeconomic level, and mostly composed of workers in companies in the secondary and tertiary sectors. The data were obtained from the hospital databases of Area 2 between the years 1994 and 2010. The inclusion criteria were the presence of LCPD and the territorial distribution of the population in Area 2 of Madrid. Conclusions: The incidence of LCPD in this Mediterranean population of an industrial area was 1.59 cases/year and 100,000 inhabitants, close to that found in populations of similar latitudes, but nevertheless it is an industrial area and socioeconomic level similar to United Kingdom populations with a much higher incidence.(AU)

Introducción: Se han relacionado varios factores poblacionales socioeconómicos en la etiología de la enfermedad de Legg-Calvé-Perthes (ELCP), como son su mayor incidencia en población urbana, vivir en la periferia de núcleos urbanos o residir a una determinada latitud con respecto al ecuador terráqueo. Es conocida la incidencia en algunos otros países, pero se desconoce la incidencia del proceso en nuestro medio, hecho importante para acomodar los recursos sociosanitarios necesarios para el tratamiento de la enfermedad. Por todo ello, el objetivo del presente estudio es determinar la incidencia de la ELCP en el Área de Salud 2 de Madrid. Material y métodos: Se analiza la incidencia de ELCP en el Área de Salud 2 de Madrid por ser el área mediterránea una zona poco estudiada. Se trata de una zona con muy escasa movilidad geográfica, de nivel socioeconómico medio-bajo, e integrada en su mayoría por trabajadores de empresas del sector secundario y terciario. Los datos se han obtenido a partir de las bases de datos hospitalarias del Área 2 entre los años 1994 y 2010. Los criterios de inclusión fueron padecer la ELCP y la distribución territorial de la población en el Área 2 de Madrid. Conclusiones: La incidencia de ELCP en esta población mediterránea de un área industrial fue de 1,59 casos por 100.000 habitantes-año, próxima a la encontrada en poblaciones de latitudes similares; pero, sin embargo, se trata de un área industrial con un nivel socioeconómico parecido al de las poblaciones de Reino Unido con una incidencia mucho mayor.(AU)

Is VEGF under-expressed in Indian children with Perthes disease?

BACKGROUND: The role of vascular endothelial growth factor (VEGF) after ischaemic necrosis of the femoral head in Legg-Calve-Perthes disease (LCPD) has not been adequately studied in humans, especially in Indian population. Therefore, we aimed to evaluate the serum levels of VEGF-A in Indian children with various stages of LCPD and compare them with those of an age- and sex-matched control group of healthy children. METHODS: In this case-control study, we enrolled 42 children (below 14 years age) suffering from LCPD and 21 age- and sex-matched healthy controls. Patients were classified radiographically according to Waldenstrom's classification. Serum VEGF-A was estimated by sandwich enzyme-linked immunosorbent assay technique. The serum values were compared between the patient group and the control group, as well as between the Waldenstrom subgroups. Results were expressed as means with ranges or median with interquartile range. RESULTS: The mean age in the patient as well as the control group was 9 years (range 4-13 years). The median value (interquartile range) of serum VEGF-A was 162.5 pg/ml (673.75 pg/ml) in the patient group and 652 pg/ml (190.5 pg/ml) in the control group (p = 0.013). When compared between lower Waldenstrom stages (initial stage + stage of fragmentation) and higher Waldenstrom stages (re-ossification stage + stage of healing), the mean values of serum VEGF-A were 464.7 pg/ml (range 0-2211 pg/ml) and 301.1 pg/ml (range 0-1910 pg/ml), respectively (p = 0.305). CONCLUSIONS: VEGF is under-expressed in Indian children suffering from LCPD. As VEGF acts as a key regulator of endochondral ossification, our finding may open new therapeutic approaches to the disease. Also, serum VEGF may act as a valuable marker for the follow-up of the disease. Our study also provides baseline data about serum VEGF-A levels in Indian cohort of LCPD patients. Future multi-centre studies are warranted with a larger sample size to fully appreciate the patho-physiological changes in VEGF occurring in LCPD.

Racial and geographic factors in the incidence of Legg-Calvé-Perthes' disease: a systematic review.

Legg-Calvé-Perthes' disease (Perthes' disease) is a childhood osteonecrosis of the hip for which the disease determinants are poorly understood. In this review, the authors identify studies of Perthes' disease incidence published up to December 2010 and make denominator populations comparable in order to allow meaningful between-study evaluation. Incidence rates and confidence intervals were determined, and, where appropriate, denominator populations were obtained from national statistical offices. Poisson regression was used to determine the influence of race and geography. The review included 21 studies that described 27 populations in 16 countries, with 124 million person-years of observation. The annual incidence among children under age 15 years ranged from 0.2 per 100,000 to 19.1 per 100,000. Race was a key determinant, with East Asians being least affected and whites most affected, though data were insufficient to consider incidence among blacks (for South Asians vs. East Asians, incidence rate ratio = 2.9, 95% confidence interval (CI): 2.4, 3.5; for whites vs. East Asians, incidence rate ratio = 8.8, 95% CI: 8.2, 9.6). Latitude was a strong predictor of disease, even after adjustment for race. Each 10° increase in latitude was associated with an incidence increase of 1.44 (95% CI: 1.30, 1.58) times. While much of the international variation appears to be a function of race, latitude demonstrates a strong association. This observation may offer new epidemiologic insights into the determinants of Perthes' disease.

The genesis of Perthes' disease: does it lie in hips with reduced ranges of movement?

Noting that Perthes' disease is relatively common in Caucasian British children compared with black African children, this study was carried out to compare the range of hip movements in a series of 100 normal Ghanaian children and 182 normal British children, between 5 and 6 years of age. A significantly increased range of movement was found in the African group in all parameters, (P<0.001). The changes were even more marked when sex was taken into consideration, female children having a much bigger range of movement than the males in both racial groups. The unaffected hips in a series of 51 Caucasian children presenting with unilateral Perthes' disease were then analysed and were found to have a reduced range of movement compared with normal, in flexion, abduction, internal rotation in flexion and external rotation in extension (P<0.001). It is suggested that one of the reasons why some hips are vulnerable to Perthes' disease could be a preexisting restricted range of movement, which renders them more susceptible to strain injuries.

The incidence of Perthes' disease in Korea: a focus on differences among races.

The purpose of this study was to determine the annual incidence of Perthes' disease in Korea and compare this with other populations. A survey identified all newly diagnosed children with Perthes' disease aged 14 years or younger in South Honam, Korea, between January 1999 and December 2001. A total of 84 children were included: 29 in 1999, 28 in 2000 and 27 in 2001. The mean annual incidence was 3.8 per 100 000. This is similar to that reported in other Asian countries, but higher than in black populations and lower than in Caucasians.

The 1226G (N370S) Gaucher mutation among patients with Legg-Calve-Perthes disease.

Legg-Calve-Perthes disease (LCPD) is an avascular necrosis of the femoral head with an annual incidence of 5-15/100,000. The estimated incidence of Gaucher disease, a lysosomal recessive storage disease, is 1:850, with a carrier rate of 1:17.5 for the 1226G (N370S) mutation among Ashkenazi Jews in whom there is a predilection. Since clinical and radiological findings of avascular hip necrosis due to either Gaucher disease or LCPD may be indistinguishable, misdiagnosis may occur. The purpose of this study was to evaluate the incidence of 1226G Gaucher mutation in a cohort of radiologically confirmed LCPD patients (diagnosed 1986-2000) in Israel. Enzyme assay was performed for confirmation of affected versus carrier status in patients with the 1226G mutation. In all, 78 LCPD patients, 86% males, 51% with severe bone disease, were studied. Family history was negative for Gaucher disease. Ethnic origin was 39% Ashkenazi Jewish, 6% Arab, and 55% other ethnicities. One Ashkenazi Jewish LCPD patient was homozygous for the 1226G mutation, and 4 LCPD patients were carriers: 3 Ashkenazi Jewish and 1 Arab patient. The frequency of the 1226G mutation among the LCPD patients was increased relative to historical Ashkenazi Jewish Israeli controls (P = 0.01). Since Gaucher disease may be misdiagnosed as LCPD, glucocerebrosidase enzyme testing is recommended among Ashkenazi Jewish children diagnosed with LCPD.

The incidence and distribution of Legg-Calvé-Perthes' disease in Liverpool, 1982-95.

AIMS: To determine the incidence and distribution of Legg-Calvé-Perthes' disease in Liverpool, in the period 1982-95. METHODS: Examination of information in a register, analysing the patients' addresses by indices of deprivation. RESULTS: A total of 122 white children were diagnosed as having Perthes' disease during the study, whereas black and minority groups form 5.8% of the population. The incidence rate in inner Liverpool had decreased to 10.5 in the period 1990-95. Simple Spearman correlations revealed an association between the disease incidence in electoral wards and deprivation. Regression analysis showed that for the period 1990-95 the most powerful effects on incidence were increases in ward deprivation since 1976, the percentage free school meals in 1986, the ward Health Index in 1981, and the percentage low birth weight in 1981. CONCLUSIONS: We suggest that environmental influences may come into play some years before a child presents with pain in the hip. There may be a genetic predisposition to the disease.

Ethnic factors in Perthes disease: a retrospective study among white and Asian population living in the same environment.

The aim of this study was to find out about the incidence of Perthes disease in different ethnic groups living in similar environments. Among the child population of Bradford, between 1991-1997, we identified from the file notes twenty-five cases of Perthes disease among the white European population (with an incidence of 5:54500 for the year 1997) and one case of Perthes disease among Asians (with an incidence of 0:16100 for the year 1997). There were two positive family histories of Perthes disease and only a case of bilateral Perthes disease. There was no significant difference in the incidence of Perthes disease among the rural and urban population. We conclude that under similar environmental circumstances the genetic factor plays an important role in the predisposition to Perthes disease.