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A Turner Syndrome Case with Primary Amenorrhea Detected to Have Distal Radius Fracture and Severe Osteoporosis
2021
Journal:  
İstanbul Kanuni Sultan Süleyman Tıp Dergisi
Author:  
Abstract:

Turner Sendromu (TS) 2500/1 sıklıkta görülen kısmi veya total monozomi X olup, erken overyen yetmezlik, kısa boy, multipl iskelet anomalileri ile karakterizedir ve doğumsal over dis-plazisi olarak da adlandırılır. Gecikmiş ergenlik ve östrojen eksikliği TS’de osteoporoz (OP) oluşumunda belirleyici faktörlerden birkaçıdır. Kemik mineral yoğunluğu (KMY) ölçümü kemik mineral durumunu değerlendirmek için en iyi parametrelerden biridir ve OP tespiti için yüksek bir prediktif değeri vardır. TS olan kızların çoğu feminizasyonu uyarmak, sürdürmek ve osteoporozu önlemek için östrojen replasman tedavisine (ERT) gereksinim duyar. Kırk iki yaşında TS olan hasta, sağ elinden destek alarak yerden kalmak isterken gelişen sağ distal radius fraktürü sonrası rehabilitasyon amacıyla polikliniğimize başvurdu. Primer amenoresi olan hasta hiç hastaneye başvurmamış olup, 20 yaşında amenore nedeniyle hastaneye başvurmuş ve hastaya TS tanısı konmuştur. Bakılan kemik mineral yoğunluğu (KMY) ölçümünde; L2-L4 vertebra KMY T-skoru -4,0 (0,719 g/cm), Z skoru -3,8, total femur T- skoru 1,7 (0,788 g /cm), femur boynu T skoru – 1,7 (0,799) olarak saptandı. Hastada patolojik vertebra fraktürü belirlenmedi. Osteoporoz saptadığımız hastaya, oral risedronat sodyum 35 mg ve kalsiyum 1.200 mg vitD3 880 iu/gün tedavisi verilerek takibe alındı. Bu olgu sunumu ile TS olan primer amenoreik bir hastada erken dönemde hormon replasman tedavisi başlanmadığı takdirde şiddetli osteoporoz ve fraktür gelişebileceğini ortaya koymayı amaçladık.

Keywords:

A Turner Syndrome Case with Primary Amenorrhea Detected to Have Distal Radius Fracture and Severe Osteoporosis
2021
Author:  
Abstract:

Turner syndrome (TS) is a partial or total monosome X that is often seen in 2500/1 and is characterized by early overing insufficiency, short-sized, multi-skeletal abnormalities and is also referred to as parental over dis-plastics. Delayed adolescence and estrogen deficiency are some of the determining factors in the formation of osteoporosis (OP) in TS. The bone mineral intensity (KMY) measurement is one of the best parameters for evaluating the bone mineral state and has a high predictive value for OP detection. Most girls with TS need estrogen replacement therapy (ERT) to stimulate, maintain feminization and prevent osteoporosis. The patient, who was 42 years old, applied to our clinic for rehabilitation after the developing right distal radius fractures while he wanted to stay from the ground with the right hand support. The patient with primary amenorrhea has never been hospitalized, at the age of 20 he has been hospitalized due to amenorrhea and has been diagnosed with TS. In the measurement of bone mineral intensity (KMY), the L2-L4 vertebral KMY T score -4,0 (0,719 g/cm), Z score -3,8, total femur T score 1,7 (0,788 g/cm), femur neck T score - 1,7 (0,799). The patient has no pathological vertebral fractures. The patient we diagnosed osteoporosis was followed by oral risedronate sodium 35 mg and calcium 1,200 mg vitD3 880 iU/day treatment. With the presentation of this phenomenon, we aimed to show that a primary amenorrheic patient with TS may develop severe osteoporosis and fractures if hormone replacement treatment is not started in the early period.

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İstanbul Kanuni Sultan Süleyman Tıp Dergisi

Field :   Sağlık Bilimleri

Journal Type :   Ulusal

Metrics
Article : 386
Cite : 360
2023 Impact : 0.026
İstanbul Kanuni Sultan Süleyman Tıp Dergisi