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Dergiler / Journal of Clinical Research in Pediatric Endocrinology / 2017 / Cilt: 9 - Sayı: 3

Long-Term Follow-up of a Case with Proprotein Convertase 1/3 Deficiency: Transient Diabetes Mellitus with Intervening Diabetic Ketoacidosis During Growth Hormone Therapy

E. Nazlı GÖNÇ
ZEYNEP ALEV ÖZÖN
AYFER ALİKAŞİFOĞLU
Nurgün KANDEMİR
Sayfa
283–287
DOI
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⚡ TL;DR(1 Cümlede Hızlı Çıkarım)

The findings of this patient show that diabetes mellitus can develop at any time during follow-up of cases with proportein convertase 1/3 deficiency especially under GH therapy.

📌 3 Maddede Bu Makale

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Amaç & Hipotez:Proprotein convertase 1/3 (PC1/3) deficiency is a very rare disease characterized by severe intractable diarrhea in the first years of life, followed by obesity and several hormonal deficiencies later.
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Temel Bulgular & Veri:We herein present a girl with PC1/3 deficiency who has been followed from birth to 17 years of age.
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Sonuç & Katkı:The findings of this patient show that diabetes mellitus can develop at any time during follow-up of cases with proportein convertase 1/3 deficiency especially under GH therapy.
🔬Tür:Araştırma Makalesi
📍Kapsam:Genişletilmiş Akademik Örneklem
⚙️Değişkenler:Alan Parametreleri

Abstract

Proprotein convertase 1/3 (PC1/3) deficiency is a very rare disease characterized by severe intractable diarrhea in the first years of life, followed by obesity and several hormonal deficiencies later. Diabetes mellitus requiring insulin treatment and diabetic ketoacidosis have not been reported in this disorder. We herein present a girl with PC1/3 deficiency who has been followed from birth to 17 years of age. She developed deficiencies of all pituitary hormones over time as well as diabetes mellitus while receiving growth hormone (GH) therapy. She was complicated with diabetic ketoacidosis during dietary management of diabetes mellitus, thus insulin treatment was initiated. Insulin requirement to regulate hyperglycemia was short-lived. Repeat oral glucose tolerance test five years later was normal. The findings of this patient show that diabetes mellitus can develop at any time during follow-up of cases with proportein convertase 1/3 deficiency especially under GH therapy.

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Bu Makaleye Atıf Yap

GÖNÇ, E. N., ÖZÖN, Z. A., ALİKAŞİFOĞLU, A., KANDEMİR, N. (2017). Long-Term Follow-up of a Case with Proprotein Convertase 1/3 Deficiency: Transient Diabetes Mellitus with Intervening Diabetic Ketoacidosis During Growth Hormone Therapy. Journal of Clinical Research in Pediatric Endocrinology, Cilt: 9 - Sayı: 3, 283–287. https://ajindex.com/articles/210448

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Kaynakça (7)

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