ORIGINAL ARTICLE

Patients with Ectopic Posterior Pituitary: Report of 6 Patients
Ektopik Posterior Hipofizi Olan Hastalar: 6 Olgunun Sunumu
Received Date : 11 Sep 2020
Accepted Date : 23 Nov 2020
Available Online : 11 Jan 2021
Doi: 10.25179/tjem.2020-78916 - Makale Dili: EN
Turk J Endocrinol Metab. 2021;25:39-45
Bu makale, CC BY-NC-SA altında lisanslanmış açık erişim bir makaledir.
ABSTRACT
Objective: Ectopic posterior pituitary (EPP) can occur because of a migration defect or neurodegeneration of the hypothalamic nuclei. EPP is typically rarely diagnosed. Therefore, we aimed to report our patients with EPP. Material and Methods: This is a retrospective study (approved by the Ege University Ethical Committee, protocol 20-7T/49) that included 6 patients with EPP who were followed up between 2012 and 2019. We collected information on age, sex, height, weight, body mass index, age at the diagnosis, history of traumatic delivery, consanguinity, multiple hormone deficiency and treatment. We examined laboratory levels and medical records, and, magnetic resonance imaging (MRI) reports. Results: The mean age of patients was 25.83 years, and the age at diagnosis was 11.16 years. One patient was female, and the others were male. They were receiving hormone replacement treatment. The patients were diagnosed with EPP during their childhood. All patients, except 2, were taking growth hormone replacement therapy. Only one patient had a history of consanguinity. Additional information about the patients is described in the patient sections. Conclusion: Patients with EPP are rarely seen, and this rare condition should be considered when a patient has panhypopituitarism. MRI is the gold standard imaging modality for hypophysis to identify this condition. In addition, patients who have EPP in MRI should be screened for hypopituitarism.
ÖZET
Amaç: Ektopik posterior hipofiz [ectopic posterior pituitary (EPP)] migrasyon defektinden ya da hipotalamik nükleusun nörodejenerasyonundan kaynaklanabilir. EPP, genellikle nadiren teşhis edilir. Bu nedenle EPP'li hastalarımızı paylaşmak istedik. Gereç ve Yöntemler: Bu çalışma, (Ege Üniversitesi Etik Komitesi tarafından onaylanan, protokol 20-7T/49) 2012 ile 2019 arasında takip edilen 6 EPP hastasını içeren retrospektif bir çalışmadır. Yaş, cinsiyet, boy, kilo, beden kitle indeksi, tanı yaşı, travmatik doğum öyküsü, akrabalık, çoklu hormon eksikliği ve tedavisi hakkında bilgi topladık. Laboratuvar değerlerini ve tıbbi kayıtları, manyetik rezonans görüntüleme (MRG) raporlarını inceledik. Bulgular: Hastalarımızın yaş ortalaması 25,83 ve tanı yaşı ortaması ise 11,16’ydı. Hastalarımızdan 1’i kadın, diğerleri erkekti. Hipofizer yetmezlikleri olması nedeniyle hormon replasman tedavisi alıyorlardı ve çocukluk çağında tanı almışlardı. İki hasta dışında diğerleri büyüme hormonu tedavisi almaktaydı. Sadece 1 hastamızın öyküsünde akraba evliliği vardı. Hastaların yarısında, travmatik doğum öyküsü vardı. Hastalar hakkında ek bilgiler, hasta bölümlerinde anlatılmıştır. Sonuç: EPP'li hastalar nadiren görülür ve bir hastada panhipopituitarizm olduğunda bu nadir durumu dikkate almalıyız. MRG, bu durumu tanımlamak için hipofiz için altın standart görüntüleme yöntemidir. Ek olarak, MRG'de EPP'si olan hastalar hipopituitarizm açısından taranmalıdır.
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