Characteristics of fulminant type 1 diabetes mellitus.

BACKGROUND: Fulminant type 1 diabetes is characterized by acute onset and rapid development of ketoacidosis. CASE REPORT: We present three cases of fulminant type 1 diabetes we experienced at our hospital. All three patients showed extremely high levels of plasma glucose, low HbA1c levels, positivity for urinary ketone bodies, ketoacidosis, and low C-peptide excretion. Flu-like symptoms were noted a few days before hospitalization, and increases in the WBC and CRP levels were observed. Interestingly, case 1 was negative for anti-GAD antibody at the time of hospitalization but became positive 22 days later. Moreover, this patient reverted to being negative for anti-GAD antibody 93 days later. Cases 1 and 3 were positive for HLA-DR4 and HLA-DR9, and Case 2 was positive for HLA-DR4. All three individuals had flu-like symptoms and showed inflammatory markers in the blood. CONCLUSIONS: Although autoimmune abnormalities are not usually linked with fulminant type 1 diabetes, the conclusion drawn from the present study is that patients with this disease should be monitored on several different occasions for autoimmune antibodies.

A trial of intranasal ACTH(1-24) administration to a patient with isolated ACTH deficiency.

BACKGROUND: Isolated adrenocorticotropic hormone (ACTH) deficiency is a rare cause of secondary adrenocortical insufficiency. Normally it could be used therapeutically as an alternative to glucocorticoid treatment in these patients. We investigated the possibility of therapeutic approach as intranasal ACTH replacement therapy in patients with ACTH deficiency. CASE REPORT: A 32-year-old woman with general fatigue, weakness of legs and loss of consciousness due to severe hyponatremia was admitted to our hospital. Endocrinological studies showed low levels of plasma ACTH and serum cortisol with the loss of circadian rhythm. Plasma ACTH and serum cortisol levels failed to respond after intravenous injection of human corticotropin releasing hormone (hCRH), however, serum cortisol showed a blunted response to ACTH(1-24) stimulation test. She was diagnosed isolated ACTH deficiency. We performed continuous intranasal administration of ACTH(1-24) to the patient. There were no cortisol, aldosterone and dehydroepiandrosterone (DHEA) responses to a single intranasal ACTH(1-24) administration while these levels increased 6 days after intranasal treatment of ACTH(1-24). CONCLUSIONS: These data demonstrate that adrenocortical steroids production are stimulated by intranasal administration of ACTH(1-24) in this patient with isolated ACTH deficiency. We suggest that intranasal administration of ACTH offers a therapeutic approach as ACTH replacement therapy in patients with ACTH deficiency. The latter may be more physiologic than glucocorticoid replacement.