Secular changes in Paget's disease: contrasting changes in the number of new referrals and in disease severity in two neighboring regions of Spain.

SUMMARY: We studied the changes in the number of new referrals with Paget's disease of bone (PDB) and severity of PDB in a high prevalence focus and its neighboring region. Referral of patients changed only in the high prevalence focus. The severity of PDB decreased in both regions. These results could suggest the effects of an environmental influence on disease activity. INTRODUCTION: The prevalence and severity of PDB have decreased in several countries over recent years. We previously reported a high radiological prevalence of PDB in Vitigudino. Here we sought to determine if secular changes in the number of new referrals and severity of PDB had occurred over recent years. METHODS: We studied 280 patients with clinically diagnosed PDB who were evaluated at a regional referral center for metabolic bone disease between 1986 and 2009. Changes in the number of new referrals were calculated by relating these data to the number of subjects at risk as determined by population registers. Trends in disease severity were analyzed with alkaline phosphatase (ALP) activity and disease extent on scan. RESULTS: Referrals from the Vitigudino region increased substantially between 1986 and 2003 but fell markedly between 2004 and 2009, although by this time there had been depopulation of the region due to emigration. No significant changes in the rates of referral occurred in the remainder of Salamanca. ALP activity and disease extent decreased in Salamanca, but only ALP activity decreased in Vitigudino. Referrals rate and severity of PDB in Vitigudino were greater than in the remainder of Salamanca. CONCLUSIONS: Referral of patients with clinically diagnosed PDB has remained stable for most of Salamanca during the past 24 years, but substantial changes have been observed in Vitigudino. In agreement with other reports, the severity of PDB has decreased in both regions consistent with the effects of an environmental influence on disease activity.

[The role of bone scintigraphy in two cases of hereditary multiple exostoses]. / Papel de la gammagrafía ósea en dos casos de exóstosis múltiple hereditaria.

We present the cases of two patients aged 18 and 40 years, with no previous personal history of interest. The only symptoms reported by the first patient in the previous year were episodes of pain and stiffness without inflammation in the right knee and third finger of the right hand. These episodes lasted two to three weeks. Our second patient presented numbness in the lower left limb over the previous year. The analytical study was normal. The patients were diagnosed with hereditary multiple exostoses, based on the clinical, radiological and scintigraphic findings. The bone scintigraphy enabled whole body images to be obtained in a single examination and may be used for the differential diagnosis of other osteoarticular conditions, as well as for the follow-up and assessment of the response to treatment.

Papel de la gammagrafía ósea en dos casos de exóstosis múltiple hereditaria / The role of bone scintigraphy in two cases of hereditary multiple exostoses

Se presentan los casos de dos pacientes de 18 y 40 años sin antecedentes personales de interés. Como única sintomatología, el primer paciente refería en el último año episodios de dolor y sensación de rigidez sin inflamación en la rodilla derecha y el tercer dedo de la mano derecha. Dichos episodios fueron de dos y tres semanas de evolución. Nuestro segundo paciente presentaba sensación de adormecimiento del miembro inferior izquierdo de 1 año de evolución. El estudio analítico fue normal. Se llegó al diagnóstico de exóstosis múltiple hereditaria basándose en los hallazgos clínicos, radiológicos y gammagráficos. La gammagrafía ósea permite obtener en una sola exploración imágenes de cuerpo entero y puede emplearse para el diagnóstico diferencial de otros procesos osteoarticulares, así como para el control evolutivo y la valoración de la respuesta al tratamiento

We present the cases of two patients aged 18 and 40 years, with no previous personal history of interest. The only symptoms reported by the first patient in the previous year were episodes of pain and stiffness without inflammation in the right knee and third finger of the right hand. These episodes lasted two to three weeks. Our second patient presented numbness in the lower left limb over the previous year. The analytical study was normal. The patients were diagnosed with hereditary multiple exostoses, based on the clinical, radiological and scintigraphic findings. The bone scintigraphy enabled whole body images to be obtained in a single examination and may be used for the differential diagnosis of other osteoarticular conditions, as well as for the follow-up and assessment of the response to treatment

[SAPHO syndrome]. / Síndrome SAPHO.

We report two cases, one 23 and one 27 year old man, with several years background of sternal and sternoclavicular joints pain, respectively. The laboratory tests were normal. Based on the clinical, radiologic and scintigraphic findings, SAPHO syndrome was diagnosed. Bone scintigraphy makes it possible to obtain a whole body scan in a single whole body scan, and can be used for differential diagnosis of other osteoarticular diseases and for evolutive control and therapeutic response.

Síndrome SAPHO / SAPHO syndrome

Se presentan los casos de dos pacientes de 23 y 27 años sin antecedentes personales de interés. Como única sintomatología referían dolor esternal y en articulaciones esternoclaviculares, respectivamente, de varios años de evolución. El estudio analítico era normal. Se llegó al diagnóstico de síndrome SAPHO basándose en los hallazgos clínicos, radiológicos y gammagráficos. La gammagrafía ósea permite obtener en una sola exploración imágenes de cuerpo entero y puede emplearse para el diagnóstico diferencial de otros procesos osteoarticulares, así como para el control evolutivo y valoración de la respuesta al tratamiento

We report two cases, one 23 and one 27 year old man, with several years background of sternal and sternoclavicular joints pain, respectively. The laboratory tests were normal. Based on the clinical, radiologic and scintigraphic findings, SAPHO syndrome was diagnosed. Bone scintigraphy makes it possible to obtain a whole body scan in a single whole body scan, and can be used for differential diagnosis of other osteoarticular diseases and for evolutive control and therapeutic response