AI Summary & Key Takeaways
AI InsightAfter six months, the findings of physical examination and MR imaging showed that the volume of testes had been decreased and nodular structures had regressed evidently.
📌 3 Key Takeaways
Abstract
The patient with congenital adrenal hyperplasia (CAH)who had been diagnosed at 1,3 years of age by another center, applied to our hospital at 2 years old. He had been receiving therapy with 10 mg/m2 of hydro-cortisone daily. In his anthropometrical evaluations; he was 2 years old, his bone age was 4 years, height standard deviation (HSDS) was +1,3 SD. His pubertal maturation was stage I and his physical examination was normal. In his follow up examinations we continued hydrocortisone therapy and doses were regulated. When he was 10 years old, his blood pressure was measured high (BP: 140/100 mm Hg) and according to the levels of 11-deoxycortisol; 13,0 ng/mL (< 8), rennin activity of plasma; < 0,16 ng/mL/hr (1,9-6) and aldosterone; < 25 pg/mL (29-161), the diagnosis of 1ip-hydroxylase deficiency was considered. Antihy-pertensive therapy was added to the hydrocortisone. By the time he reached the final height, he was 16,9 years old and the epiphysis had been closed. At that time he presented with testicular enlargement for 1 month. On physical examination nodular structure was palpable and bilateral testes volumes were over 25 ml. On sonography, both testes parenchyma were heterogenic and multiple, different sized hypo echoic areas were seen. On MR imaging, a lesion filling the scrotum with the dimensions of 10x8x8cm was seen. On T1A and T2A images, the mass was hypo intense. It was described as rest tumor. Because of the rest tu¬mor, the dexamethasone dose was increased to a higher dose (1,5 mg/24 hr). After six months, the findings of physical examination and MR imaging showed that the volume of testes had been decreased and nodular structures had regressed evidently.
Özet
1.3 yaşında başka bir merkezde konjenital adrenal hiperplazi (KAH) tanısı alan ve 10 mg/mVgün hidrokortizon kullanan hasta 2 yaşında hastanemize başvurdu. Antropometrik değerlendirmede; takvim yaşı: 2, kemik yaşı: 4 yaş, boy standart sapması (BSDS): +1,3 SD,'puberte evre I, fizik muayene bulguları normaldi. Kullanmakta olduğu hidrokortizon tedavisine doz ayarlamaları ile devam edilen hastanın 10 yaşında" tansiyon arteriyeli yüksek saptandı (TA: 140/100 mm Hg). Bu dönemde yapılan hormonal değerlendirmede; 11-eoksikortizol; 13,0 ng/mL (< 8), plazma renin aktivitesi; (0,16 ng/mL/ saat (1,9-6) ve aldosteron (25 pg/mL (29-161) bulunması nedeni ile hastada 11(3-hidroksilaz eksikliği düşünül¬dü. Hidrokortizon tedavisine antihipertansif eklenerek izleme devam edildi.Takvim yaşı 16,9 olduğunda, epifizleri kapanan, 160.5 cm (BSDS: -2,07) ile final boya ulaşan olgunun 1 aydır testiste büyüme şikayeti olduğu öğrenildi. Fizik incelemede her iki testis 25 mL üzerinde ve nodüler yapıda palpe edildi. Ultrasonografik değerlendirmede her iki testis parankimi heterojendi ve çok sayıda değişik boyutlarda yer yer düzensiz sınırlı hipoekojen alanlar izlendi. Testis manyetik rezonans görüntülemede (MRG) skrotumu dolduran yaklaşık 10x8x8 cm boyutlu multi lobule, T1A ve T2A görüntülerde hipointens özellikte kitle lezyonu adrenal rest tümörü olarak yorumlandı. Final boya ulaşan olgunun almakta olduğu deksametazon rest tümörü nedeniyle günde 1,5 mg'a yükseltildi. Altı ay sonraki kontrolünde fizik muayene ve MRG bulguları olarak testis volümünün küçüldüğü, nodüler yapıların öncekine göre belirgin düzeldiği görüldü.