Diabetes mellitus with Laron syndrome: case report

被引:10
作者
Agladioglu, Sebahat Yilmaz [1 ]
Cetinkaya, Semra [1 ]
Erdeve, Senay Savas [1 ]
Onder, Asan [1 ]
Kendirci, Havva Nur Peltek [1 ]
Bas, Veysel Nijat [1 ]
Aycan, Zehra [1 ]
机构
[1] Dr Sami Ulus Training & Res Childrens Hosp, Clin Pediat Endocrinol, TR-06080 Ankara, Turkey
关键词
diabetes mellitus; IGF-1; treatment; Laron syndrome; obesity; GROWTH-HORMONE RESISTANCE; IGF-I DEFICIENCY; PITUITARY DWARFISM; CHILDREN;
D O I
10.1515/jpem-2012-0411
中图分类号
R5 [内科学];
学科分类号
100201 [内科学];
摘要
There are different opinions concerning changes in glucose metabolism in patients with Laron syndrome. In this paper we discuss the treatment results of our patient with Laron syndrome who developed diabetes during late adolescence. A 19-year-old boy with Laron syndrome was referred to our clinic for follow-up. He had been diagnosed with Laron syndrome (LS) at 4 years old and rIGF-1 therapy was initiated. After 4 months the treatment was discontinued. At the age of 17, rIGF-1 therapy was restarted. A height gain of 8.8 cm. was observed during the 2-year treatment period. He was admitted to our hospital at the age of 19 years following discontinuation of the therapy. At that time, his height was 142 cm, and weight for height was 136%. His blood glucose was 85 mg/dL (4.72 mmol/L), insulin was 26.39 pmol/L, and HbA(1c) was 5.4%. At the age of 20, when he has not been receiving IGF-1 therapy for 1 year, his weight for height was 143 cm. Laboratory evaluation revealed that fasting blood glucose was 176 mg/dL (9.77 mmol/L), fasting insulin was 29.86 pmol/L, and HbA(1c) was 7.5%. Primary insulin therapy was then initiated. His parents both had a diagnosis of type 2 diabetes. Insulin therapy was switched to oral antidiabetic (OAD) therapy at the end of the second year because of a normal C-peptide level of 0.8 nmol/L under insulin therapy. After 6 months of OAD, HbA(1c) was 5.7%. The treatment was then switched to IGF-1 therapy, but his blood glucose profile was impaired and OAD therapy was restarted. In conclusion, we observed that genetic susceptibility and abdominal obesity caused type 2 diabetes in this patient. We believe that oral antidiabetic agents and life-style changes may be the appropriate approach when diabetes is developed in LS patients.
引用
收藏
页码:955 / 958
页数:4
相关论文
共 16 条
[1]
Long-term treatment with recombinant insulin-like growth factor (IGF)-I in children with severe IGF-I deficiency due to growth hormone insensitivity [J].
Chernausek, Steven D. ;
Backeljauw, Philippe F. ;
Frane, James ;
Kuntze, Joyce ;
Underwood, Louis E. .
JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM, 2007, 92 (03) :902-910
[2]
Growth Hormone Receptor Deficiency Is Associated with a Major Reduction in Pro-Aging Signaling, Cancer, and Diabetes in Humans [J].
Guevara-Aguirre, Jaime ;
Balasubramanian, Priya ;
Guevara-Aguirre, Marco ;
Wei, Min ;
Madia, Federica ;
Cheng, Chia-Wei ;
Hwang, David ;
Martin-Montalvo, Alejandro ;
Saavedra, Jannette ;
Ingles, Sue ;
de Cabo, Rafael ;
Cohen, Pinchas ;
Longo, Valter D. .
SCIENCE TRANSLATIONAL MEDICINE, 2011, 3 (70)
[3]
Poor reproducibility of IGF-I and IGF binding protein-3 generation test in children with short stature and normal coding region of the GH receptor gene [J].
Jorge, AA ;
Souza, SC ;
Arnhold, IJ ;
Mendonca, BB .
JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM, 2002, 87 (02) :469-472
[4]
BODY-FAT IN LARON SYNDROME PATIENTS - EFFECT OF INSULIN-LIKE GROWTH FACTOR-I TREATMENT [J].
LARON, Z ;
KLINGER, B .
HORMONE RESEARCH, 1993, 40 (1-3) :16-22
[5]
Diabetic retinopathy in two patients with congenital IGF-I deficiency (Laron syndrome) [J].
Laron, Z ;
Weinberger, D .
EUROPEAN JOURNAL OF ENDOCRINOLOGY, 2004, 151 (01) :103-106
[6]
CARBOHYDRATE-METABOLISM IN PRIMARY GROWTH-HORMONE RESISTANCE (LARON SYNDROME) BEFORE AND DURING INSULIN-LIKE GROWTH-FACTOR-I TREATMENT [J].
LARON, Z ;
AVITZUR, Y ;
KLINGER, B .
METABOLISM-CLINICAL AND EXPERIMENTAL, 1995, 44 (10) :113-118
[7]
LARON Z, 1968, ISRAEL J MED SCI, V4, P883
[8]
LARON Z, 1966, ISRAEL J MED SCI, V2, P152
[9]
GROWTH-CURVES FOR LARON SYNDROME [J].
LARON, Z ;
LILOS, P ;
KLINGER, B .
ARCHIVES OF DISEASE IN CHILDHOOD, 1993, 68 (06) :768-770
[10]
Extensive personal experience - Laron syndrome (primary growth hormone resistance or insensitivity): The personal experience 1958-2003 [J].
Laron, Z .
JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM, 2004, 89 (03) :1031-1044