Med Bull Sisli Etfal Hosp. 2016; 50(3): 171-180 | DOI: 10.5350/SEMB.20160724041035

Primary hyperparathyroidism: Current situation in the clinical and biochemical presentation

Mehmet Uludað, Nurcihan Aygün
Þiþli Hamidiye Etfal Eðitim ve Araþtýrma Hastanesi, Genel Cerrahi Klinði, Ýstanbul - Türkiye

Primary hyperparathyroidism (pHPT) is the third most common endocrine disease and the most common cause of outpatient hypercalcemia. Historically, most patients presented with overt symptoms and signs of pHPT. With the advent and widespread use of automated blood analyzers, currently the majority of patients are diagnosed through routine biochemical laboratory testing done for other aims. The majority of patients are minimally symptomatic or asymptomatic. The clinical presentation of pHPT varies from asymptomatic disease to classic symptomatic disease in which renal and/or skeletal complications are observed. The term “mild pHPT” has been interpreted in many different ways over the past 30 years. Mild pHPT was clearly defined as a disease in asymptomatic patients who do not meet surgical criteria set out by the updated International Guidelines. Normocalcemic pHPT was first defined formally at the time of the ‘’Third International Workshop on the Management of Asymptomatic pHPT’’ in 2008. Normocalcemic pHPT, a variant of the traditional hypercalcemic presentation of pHPT, is characterized by consistently elevated PTH concentrations with normal total and ionized serum calcium (Ca) concentration in the absence of secondary causes for elevated PTH concentrations. Primary hyperparathyroidism may present as classic pHPT (in which both Ca and PTH are high), nonclassic (normohormonal or nonsuprese) pHPT (in which Ca is high and PTH is normal), normocalcemic pHPT (in which Ca is normal and PTH is high) biochemically. The only curative therapy for pHPT is parathyroidectomy. Parathyroidectomy is clearly indicated in all symptomatic patients. ‘’Fourth International Workshop on the Management of Asymptomatic pHPT’’ had been performed in 2013. Surgical indications for pHPT are determined according to the age, Ca level, skeletal and renal findings. In some patients with asymptomatic disease, surgery is not mandatory. On the other hand, even in these patients who don’t meet any criteria for parathyroidectomy, surgery is always an option because it is the only exact treatment for pHPT. The optimal treatment strategy for patients with normocalcemic pHPT has not yet been proved. Experts advise that patients be referred for parathyroidectomy if they have or progress complications of pHPT such as osteoporosis, fragility fractures or kidney stones, even if normocalcemia continues. Patients without complications at the time of diagnosis could be monitored for disease progression.

Keywords: Asymptomatic primary hyperparathyroidism, mild primary hyperparathyroidism, nonclassicprimary hyperparathyroidism, normocalcemic primary hyperparathyroidism, primary hyperparathyroidism, symptomatic primary hyperparathyroidism

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Primer Hiperparatiroidi: Klinik ve biyokimyasal bulgularýn güncel durumu

Mehmet Uludað, Nurcihan Aygün
Þiþli Hamidiye Etfal Eðitim ve Araþtýrma Hastanesi, Genel Cerrahi Klinði, Ýstanbul - Türkiye

Primer hiperparatiroidizm (pHPT) en sýk 3. endokrin hastalýktýr ve ayaktan hastalarda hiperkalseminin en sýk sebebidir. Geçmiþte hastalarýn çoðu belirgin pHPT semptom ve bulgularý ile baþvurmaktaydýlar. Otomatik kan analizörlerinin geliþmesi ve yaygýn kullanýlmasý ile günümüzde hastalarýn büyük bölümü diðer nedenlerle yapýlan rutin biyokimyasal testlerle teþhis edilmektedir. Hastalarýn çoðunluðu minimal semptomatik veya asemptomatiktir. pHPT’nin klinik prezentasyonu, asemptomatik hastalýktan renal ve iskelet komplikasyonlarýnýn gözlendiði semptomatik hastalýða kadar deðiþir. Hafif pHPT terimi 30 yýldýr birçok farklý durum için kullanýlmýþtýr. Hafif pHPT terimi net olarak, güncellenen uluslararasý kýlavuzlara göre cerrahi endikasyonu olmayan asemptomatik hastalarý belirtmek için tanýmlanmýþtýr. Normokalsemik pHPT ilk olarak 2008’de asemptomatik pHPT’nin tedavisi ile ilgili düzenlenen 3. uluslararasý çalýþtayda resmen kabul edilmiþtir. Normokalsemik pHPT klasik hiperkalsemik pHPT’nin bir varyantýdýr. Normokalsemik pHPT paratiroid hormon (PTH) artýþýna neden olabilecek sekonder bir sebep olmaksýzýn, normal iyonize ve total kalsiyum (Ca) düzeyleri ile birlikte yüksek PTH düzeyi ile karakterizedir. pHPT biyokimyasal olarak klasik pHPT (hem Ca hem PTH yüksek), nonklasik (normohormonal veya nonsuprese) pHPT (yüksek Ca, normal PTH), normokalsemik pHPT (normal Ca, yüksek PTH) olarak prezente olabilir. pHPT’nin tek küratif tedavisi cerrahidir. Paratiroidektomi bütün semptomatik hastalarda endikedir. Asemptomatik pHPT’nin tedavisi ile ilgili 4. uluslararasý çalýþtay 2013’te yapýldý. pHPT’nin cerrahi endikasyonlarý yaþ, Ca düzeyi, iskelet ve böbrek bulgularýna göre belirlenir. Bazý asemptomatik hastalarda cerrahi zorunlu deðildir. Diðer yandan, paratiroidektomi için cerrahi kriterleri olmayan bu hastalarda bile cerrahi daima bir seçenektir. Çünkü cerrahi, pHPT’nin tek kesin tedavisidir. Normokalsemik pHPT’li hastalarýn optimal tedavi stratejisi halen ortaya koyulamamýþtýr. Bu konunun deneyimli uzmanlarý Ca düzeyi normal devam etse bile osteoporoz, fraktür, böbrek taþý gibi pHPT kopmplikasyonlarýnýn varlýðýnda veya bunlarýn ortaya çýkmasýnda cerrahi önermektedirler. Taný anýnda komplikasyonsuz hastalar hastalýk ilerlemesi için takip edilebilir

Anahtar Kelimeler: Asemptomatik primer hiperparatiroidizm, hafif primer hiperparatiroidizm, nonklasikprimer hiperparatiroidizm, normokalsemik primer hiperparatiroidizm, primer hiperparatiroidizm, semptomatik primer hiperparatiroidizm

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