Delayed peak response of cortisol to insulin tolerance test in patients with Prader-Willi syndrome

2018 ◽  
Vol 176 (6) ◽  
pp. 1369-1374 ◽  
Author(s):  
Yuji Oto ◽  
Keiko Matsubara ◽  
Tadayuki Ayabe ◽  
Masahisa Shiraishi ◽  
Nobuyuki Murakami ◽  
...  
2002 ◽  
pp. 667-672 ◽  
Author(s):  
S Petersenn ◽  
R Jung ◽  
FU Beil

OBJECTIVE: The diagnosis of GH deficiency in adults should be made using provocative testing of GH secretion. The insulin tolerance test (ITT) is recommended as the gold standard investigation. Because of the risk of serious complications, patients with epilepsy or known ischemic heart disease should not undergo this test. GHRP-6 is a synthetic hexapeptide that releases GH by binding to specific hypothalamic and pituitary receptors. We assessed the diagnostic capability of GH stimulation by GHRP-6 alone or in combination with GHRH in comparison to the results of an ITT. DESIGN: Twenty patients underwent an ITT for suspected pituitary or adrenal disease. Either GHRP-6 (1 microg/kg) alone, or GHRP-6 in combination with GHRH (1 microg/kg) were administered on different days. Blood samples were obtained during a subsequent 90-min period for measurement of GH. RESULTS: Ten patients had a GH peak response of less than 3 microg/l during ITT and were considered growth hormone deficient (GHD). The GH mean peak (+/-S.E.M., range) in this group was 0.7 microg/l (+/-0.3, 0.1-2.9) compared with 14.5 microg/l (+/-3.5, 3.8-40.8) in the group of patients with a GH peak response of more than 3 microg/l (growth hormone sufficient (GS)). For the GHRP-6 test, the GH mean peak was 1.3 microg/l (+/-0.6, 0.1-6.7) in the GHD group versus 25.7 microg/l (+/-5.5, 7.7-54.2) in the GS group. After GHRP-6+GHRH, the GH mean peaks were 4.0 microg/l (+/-1.3, 0.2-11.9) versus 54.7 microg/l (+/-11.1, 13.9-136.0) respectively. During administration of GHRP-6, the only side effects observed were flush symptoms. CONCLUSIONS: Peak GH levels below 7 microg/l for the GHRP-6 test and below 13 microg/l for the combined GHRP-6+GHRH test identified all patients with GH deficiency correctly as defined by ITT. The results suggest that testing with GHRP-6 or GHRP-6+GHRH is as sensitive and specific as an ITT for the diagnosis of adult GH deficiency.


2011 ◽  
Vol 96 (1) ◽  
pp. E173-E180 ◽  
Author(s):  
Stense Farholt ◽  
Rasmus Sode-Carlsen ◽  
Jens Sandahl Christiansen ◽  
John R. Østergaard ◽  
Charlotte Høybye

Context: Prader-Willi syndrome (PWS) is a genetic disease associated with hypogonadism and partial GH insufficiency, possibly explained in part by a hypothalamic dysfunction. Partial insufficiency of the hypothalamic-pituitary-adrenal (HPA) axis has recently been suggested. Objective: The objective of the study was to further explore the HPA axis in PWS by use of routine tests. Design: Nonselected PWS patients were examined with a standard high-dose synacthen test or the insulin tolerance test (ITT). A random serum (s) cortisol was measured in case of acute illness. Setting: The study was conducted at university hospitals in Denmark and Sweden. Patients: Sixty-five PWS patients with a confirmed genetic diagnosis participated in the study. Main Outcome Measures: A s-cortisol value above 500 nmol/liter as well as an increase of 250 nmol/liter or greater was considered a normal response. Results: Fifty-seven PWS patients (median age 22 yr, total range 0.5–48 yr) were examined with the high-dose synacthen test. The median s-cortisol at the time of 30 min was 699 (474–1578) nmol/liter. Only one patient had a s-cortisol level below 500 nmol/liter but an increase of 359 nmol/liter. This patient subsequently showed a normal ITT response. Two patients had increases less than 250 nmol/liter but a time of 30-min s-cortisol values of 600 nmol/liter or greater. These three patients were interpreted as normal responders. Eight patients [aged 26 (16–36) yr] examined with the ITT had a median peak s-cortisol of 668 (502–822) nmol/liter. Four children admitted for acute illnesses had s-cortisol values ranging from 680 to 1372 nmol/liter. Conclusion: In this PWS cohort, the function of the HPA axis was normal, suggesting that clinically significant adrenal insufficiency in PWS is rare.


1998 ◽  
Vol 18 (02) ◽  
pp. 74-79
Author(s):  
K.-H. Zurborn ◽  
H. D. Bruhn ◽  
H. Mönig

SummaryIn order to study the acute and prolonged effects of hypoglycemia on the hemostatic and fibrinolytic system we measured prothrombin fragment (F1+2), thrombin-antithrombin III complex (TAT), platelet factor 4 (PF4), β-thromboglobulin (âTG), factor VIII antigen (F VIII antigen), D-dimer, tissue-type plasminogen activator (t-PA) antigen, and plasminogen activator inhibitor (PAI-1) in 22 patients during insulin tolerance test. F1+2 and TAT increased significantly 15 and 90 minutes after administration of insulin, as did PF4 and âTG. At 4 and 24 hours, these parameters were not different from baseline. Factor VIII antigen was not significantly altered. D-dimer concentration did not change. However, the D-dimer/TAT ratio significantly decreased at 15 and 90 minutes but increased markedly above baseline at 4 and 24 hours. t-PA antigen was also found to be elevated at 15 and 90 minutes but had returned to baseline at 4 and 24 hours. PAI-1 concentration did not change. We conclude from these data that both coagulation and fibrinolysis are activated in the short-term response to acute insulin-induced hypoglycemia, followed by a prolonged activation of fibrinolysis. Our study may explain why patients undergoing insulin tolerance test, despite marked clotting and platelet activation, almost never develop thromboembolic complications.


1993 ◽  
Vol 10 (9) ◽  
pp. 839-842 ◽  
Author(s):  
S. Hirst ◽  
D.I.W. Phillips ◽  
S.K. Vines ◽  
P.M. Clark ◽  
C.N. Hales

2021 ◽  
Author(s):  
Sahar Abidi ◽  
Wafa Grira ◽  
Nadia Khessairi ◽  
Ibtissem Oueslati ◽  
Meriem Yazidi ◽  
...  

1975 ◽  
Vol 80 (1) ◽  
pp. 1-13 ◽  
Author(s):  
Robert Fiedler ◽  
Dorothy T. Krieger

ABSTRACT Congenital stenosis of the aqueduct of Sylvius is reported to be associated with sella turcia enlargement and clinical and laboratory abnormalities of the hypothalamic-pituitary-target-organ axis. It is a surgically reversible lesion. In the present report, 3 female patients with this lesion were studied with tests of basal endocrine function, as well as insulin tolerance tests, response to metyrapone and determination of circadian periodicity of plasma cortisol levels. In one patient all testing was normal and no surgery was performed. In 2 other patients the insulin tolerance test revealed either abnormal cortisol or growth hormone responses and in one patient urinary gonadotrophins were absent. All tests became normal post-operatively although in one instance not completely so until 5 years after surgery.


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