Because high levels of cortisol are frequently observed in patients with septic shock, low levels of serum cortisol are considered indicative of relative adrenal insufficiency (RAI). This study was performed to investigate whether pretest clinical characteristics, including basal serum cortisol levels, are predictive of serum cortisol response to corticotropin and whether basal cortisol levels have a prognostic significance in patients with septic shock. We performed a retrospective analysis of 68 patients with septic shock who underwent short corticotropin stimulation testing. RAI was defined as an increase in cortisol level <9 microgram/dL from baseline, and results showed that 48 patients (70.6%) had this insufficiency. According to the univariate analysis, the RAI group had significantly higher simplified acute physiology score II (SAPS II) and sequential organ failure assessment (SOFA) scores than the non-RAI group. The incidence of RAI was the same regardless of the basal serum cortisol level (p=0.447). The hospital mortality rate was 58.8% and was not significantly different between the RAI and non-RAI groups. However, a high basal serum cortisol level (> or =30 microgram/dL) was significantly associated with in-hospital mortality. In conclusion, our data suggest that basal serum cortisol levels are not predictive of serum cortisol response to corticotropin but have a significant prognostic value in patients with septic shock.
Adrenal Insufficiency/blood/diagnosis ; Adrenocorticotropic Hormone/*pharmacology ; Aged ; Female ; Humans ; Hydrocortisone/*blood ; Incidence ; Male ; Middle Aged ; Prognosis ; Reference Values ; Shock, Septic/*blood/*diagnosis/mortality ; Time Factors ; Treatment Outcome

Allgrove syndrome is a rare autosomal recessive disorder. It is also known as the 3A syndrome and characterised by the triad of achalasia, alacrima and adrenal insufficiency. The AAAS gene is encoded on chromosome 12q13. We report the case of a 23-year-old woman who presented at the hospital with adrenal crisis that was triggered by infection of the urinary system and gastrointestinal bleeding. She had a known diagnosis of achalasia for eight years, and ophthalmologic examination revealed alacrima. Based on our findings, the patient was diagnosed with Allgrove syndrome.
Adrenal Insufficiency ; blood ; diagnosis ; genetics ; Adrenocorticotropic Hormone ; blood ; Diagnosis, Differential ; Diagnostic Techniques, Ophthalmological ; Endoscopy, Gastrointestinal ; Esophageal Achalasia ; blood ; diagnosis ; genetics ; Female ; Humans ; Mutation ; Nerve Tissue Proteins ; blood ; genetics ; Nuclear Pore Complex Proteins ; blood ; genetics ; Young Adult

An obese, 65 years old woman admitted to the department of orthopedics for total knee replacement arthroplasty under the diagnosis of severe degenerative arthritis and pain of both knee joints. Incidentally, an asymptomatic huge hepatic cyst (size 25 x 27 cm) was found in the pre-operative check ups During the induction of general anesthesia, severe hypotension and intractable atrial fibrillation with RVR suddenly developed. Initially, various tilting positions together with rapid fluid replacement were tried immediately considering the clinical features as supine hypotensive episode, but with little improvement of clinical situation. Then, a bolus of intravenous hydrocortisone succinate 100 mg was tried empirically, resulting in the immediate normalization of blood pressure. Her laboratory findings after the incidence showed decreased blood cortisol level, but ACTH stimulation test was within normal limits. We concluded the case, clinically, as functional adrenal insufficiency and report the case with the reviews of related articles
Adrenal Insufficiency* ; Adrenocorticotropic Hormone ; Aged ; Anesthesia* ; Anesthesia, General ; Arthroplasty ; Arthroplasty, Replacement, Knee ; Atrial Fibrillation ; Blood Pressure ; Diagnosis ; Female ; Humans ; Hydrocortisone ; Hypotension ; Incidence ; Knee Joint ; Orthopedics ; Osteoarthritis ; Succinic Acid

A middle-aged male patient with recurrent hypoglycemia was diagnosed as insulinoma according to the symptoms and the laboratory tests. Laboratory tests also showed low cortisol and adrenocortico-tropic hormone (ACTH) levels, therefore adrenal insufficiency was diagnosed and hydrocortisone was given. The hormone replacement therapy was terminated after surgery, and ACTH and cortisol levels returned to normal.
Adrenal Insufficiency ; drug therapy ; etiology ; Adrenocorticotropic Hormone ; blood ; Anti-Inflammatory Agents ; therapeutic use ; Humans ; Hydrocortisone ; therapeutic use ; Insulinoma ; complications ; diagnosis ; Male ; Middle Aged ; Treatment Outcome

This study was undertaken to evaluate the incidence and risk factors associated with relative adrenal insufficiency (RAI) in Korean critically-ill patients. All patients who were admitted to the Medical Intensive Care Unit (MICU) of Samsung Medical Center between January 1, 2006 and April 30, 2007 were prospectively evaluated using a short corticotropin stimulation test on the day of admission. RAI was defined as an increase in the serum cortisol level of <9 microgram/dL from the baseline after administration of 250 microg of corticotropin. In all, 123 patients were recruited and overall the incidence of RAI was 44% (54/123). The presence of septic shock (P=0.001), the Simplified Acute Physiology Score (SAPS) II (P=0.003), the Sequential Organ Failure Assessment (SOFA) score (P=0.001), the mean heart rate (P=0.040), lactate levels (P=0.001), arterial pH (P=0.047), treatment with vasopressors at ICU admission (P=0.004), and the 28-day mortality (P=0.041) were significantly different between patients with and without RAI. The multivariate analysis showed that the SOFA score was an independent predictor of RAI in critically-ill patients (odd ratio=1.235, P=0.032). Our data suggest that RAI is frequently found in Korean critically-ill patients and that a high SOFA score is an independent predictor of RAI in these patients.
Adrenal Insufficiency/diagnosis/*epidemiology/mortality ; Adrenocorticotropic Hormone/diagnostic use ; Adult ; Aged ; *Critical Illness ; Female ; Humans ; Hydrocortisone/blood ; Intensive Care Units ; Korea ; Male ; Middle Aged ; Multivariate Analysis ; Predictive Value of Tests ; Prospective Studies ; ROC Curve ; Risk Factors ; Severity of Illness Index ; Survival Rate

Congenital adrenal insufficiency is caused by specific genetic mutations. Early suspicion and definite diagnosis are crucial because the disease can precipitate a life-threatening hypovolemic shock without prompt treatment. This study was designed to understand the clinical manifestations including growth patterns and to find the usefulness of ACTH stimulation test. Sixteen patients with confirmed genotyping were subdivided into three groups according to the genetic study results: congenital adrenal hyperplasia due to 21-hydroxylase deficiency (CAH, n=11), congenital lipoid adrenal hyperplasia (n=3) and X-linked adrenal hypoplasia congenita (n=2). Bone age advancement was prominent in patients with CAH especially after 60 months of chronologic age (n=6, 67%). They were diagnosed in older ages in group with bone age advancement (P<0.05). Comorbid conditions such as obesity, mental retardation, and central precocious puberty were also prominent in this group. In conclusion, this study showed the importance of understanding the clinical symptoms as well as genetic analysis for early diagnosis and management of congenital adrenal insufficiency. ACTH stimulation test played an important role to support the diagnosis and serum 17-hydroxyprogesterone levels were significantly elevated in all of the CAH patients. The test will be important for monitoring growth and puberty during follow up of patients with congenital adrenal insufficiency.
17-alpha-Hydroxyprogesterone/blood ; 46, XY Disorders of Sex Development/drug therapy/*genetics ; Adolescent ; Adrenal Hyperplasia, Congenital/drug therapy/*genetics ; Adrenal Insufficiency/*congenital/diagnosis/drug therapy/genetics ; Adrenocorticotropic Hormone/*metabolism ; Bone Development/genetics ; Child ; Child, Preschool ; DAX-1 Orphan Nuclear Receptor/genetics ; Female ; Genetic Diseases, X-Linked/drug therapy/*genetics ; Genotype ; Glucocorticoids/therapeutic use ; Humans ; Intellectual Disability/complications ; Male ; Mineralocorticoids/therapeutic use ; Obesity/complications ; Phosphoproteins/genetics ; Puberty, Precocious/complications ; Retrospective Studies ; Steroid 21-Hydroxylase/genetics