The Journal of clinical endocrinology and metabolism · 2019
Prevalence and Course of Endocrinopathy in POEMS Syndrome.
СтатьяПоддержка исследования: вне правительства СШАНазвание и аннотация: Перевод в очереди
Обработка статьи · этапы, ошибки и управление
Загрузка состояния…
Ошибки и ограничения
История · было → стало
Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.
Caimari Francisca, Keddie Stephen, Lunn Michael P, D'Sa Shirley, Baldeweg Stephanie E
Аннотация
CONTEXT: POEMS syndrome is a rare multisystem disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma-proliferative disorder, and skin changes, among other features.
OBJECTIVE: To describe the prevalence and course of endocrine dysfunction in POEMS.
DESIGN: Cohort study with systematic review of the endocrinopathy in POEMS.
SETTING: Seventy-five patients with POEMS were evaluated by the multidisciplinary team at our tertiary specialist center.
PATIENTS: Endocrine data were available for 59 patients who attended the clinic from June 1999 to May 2018.
INTERVENTIONS: All patients had regular endocrine screening, including testing for diabetes, pituitary and thyroid dysfunction and assessment of bone metabolism.
MAIN OUTCOME MEASURE: Prevalence and survival time to develop endocrinopathy in POEMS.
RESULTS: Thirty-four (63%) patients presented with an endocrinopathy at POEMS diagnosis and 54 (92%) had at least one endocrine abnormality at follow-up. The median follow-up was 4.4 (interquartile range, 1.5, 7.9) years. The most common endocrine abnormality was hypogonadism in 68%, followed by hyperprolactinemia (56%), hypothyroidism (54%), abnormal glucose metabolism (24%), adrenal insufficiency (17%), and high IGF-1 levels (15%). Spontaneous resolution of endocrine abnormalities at the end of follow-up was observed: 14% of patients with hypogonadism; 42%, hyperprolactinemia; 34%, hypothyroidism; and 38%, high IGF-1 levels.
CONCLUSIONS: Endocrinopathy was found in 63% of patients at diagnosis and in 92% of patients during follow-up in our cohort. Therefore, patients with POEMS should be systematically assessed for endocrinopathy. The most common deficiencies were hypogonadism and hypothyroidism; however, but endocrinopathy can normalize, so ongoing treatment should remain under review.
OBJECTIVE: To describe the prevalence and course of endocrine dysfunction in POEMS.
DESIGN: Cohort study with systematic review of the endocrinopathy in POEMS.
SETTING: Seventy-five patients with POEMS were evaluated by the multidisciplinary team at our tertiary specialist center.
PATIENTS: Endocrine data were available for 59 patients who attended the clinic from June 1999 to May 2018.
INTERVENTIONS: All patients had regular endocrine screening, including testing for diabetes, pituitary and thyroid dysfunction and assessment of bone metabolism.
MAIN OUTCOME MEASURE: Prevalence and survival time to develop endocrinopathy in POEMS.
RESULTS: Thirty-four (63%) patients presented with an endocrinopathy at POEMS diagnosis and 54 (92%) had at least one endocrine abnormality at follow-up. The median follow-up was 4.4 (interquartile range, 1.5, 7.9) years. The most common endocrine abnormality was hypogonadism in 68%, followed by hyperprolactinemia (56%), hypothyroidism (54%), abnormal glucose metabolism (24%), adrenal insufficiency (17%), and high IGF-1 levels (15%). Spontaneous resolution of endocrine abnormalities at the end of follow-up was observed: 14% of patients with hypogonadism; 42%, hyperprolactinemia; 34%, hypothyroidism; and 38%, high IGF-1 levels.
CONCLUSIONS: Endocrinopathy was found in 63% of patients at diagnosis and in 92% of patients during follow-up in our cohort. Therefore, patients with POEMS should be systematically assessed for endocrinopathy. The most common deficiencies were hypogonadism and hypothyroidism; however, but endocrinopathy can normalize, so ongoing treatment should remain under review.