A single measurement of serum 17alpha-hydroxyprogesterone (17OHP) level can be unreliable because of its marked diurnal variation. We investigated the relationship of serum level of 17OHP with that of androstenedione (AD), which shows a smaller diurnal variation. And we tested whether the responses of these two hormones to low-dose ACTH stimulation are correlated in patients with 21-hydroxylase deficiency. Baseline serum 17OHP and AD levels were measured in 87 patients and a low-dose ACTH stimulation test was performed in 41 patients. The basal 17OHP level correlated positively with the basal AD level independently of sex, type of 21-hydroxylase deficiency, and the time of day of blood sampling (n = 87, R2 = 0.75, P < 0.001). The area under the curve of 17OHP and AD correlated positively with their respective basal levels. The fold-change increase in 17OHP after ACTH injection correlated negatively with the basal 17OHP level, but that of AD did not correlate with the basal AD level. The random serum 17OHP level, used in the clinic, is a reliable guide and a low-dose ACTH stimulation test provides no extra benefit for assessing the treatment adequacy in patients with 21-hydroxylase deficiency.
17-alpha-Hydroxyprogesterone/*blood ; Adolescent ; Adrenal Hyperplasia, Congenital/*diagnosis/drug therapy ; Adrenocorticotropic Hormone/*diagnostic use ; Androstenedione/*blood ; Circadian Rhythm ; Female ; Humans ; Male ; Steroid 21-Hydroxylase/metabolism ; Young Adult

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

OBJECTIVETo investigate the basic clinical characteristics of paraneoplastic neurological syndrome (PNS) in children.

METHODTo retrospectively analyze the clinical data of 12 PNS children who were hospitalized in neurology department in Beijing Children's Hospital from 2010 to 2011. Some patients were followed up after surgery.

RESULTIn 12 patients with PNS, 11 were male and 1 was female. The mean onset age were (30.5 ± 15.3) months. The mean duration from neurological symptom onset to finding out of tumor was (112.7 ± 154.4) days. The onset of the disease in 2 patients was acute, in 3 was subacute and in the other 7 was chronic (2 of 7 had 2 to 3 relapses). Of 12 patients, 11 had symptoms of ataxia (3 patients also had opsoclonus and myoclonus, OMS), 1 had weakness of limbs at onset and then had ataxia. Nine of 12 patients had surgery, and pathologic diagnosis was neuroblastoma and ganglioneuroma. Six patients were followed-up for 8 to 21 months. One patient had a little improvement and 5 almost recovered.

CONCLUSIONThe PNS children can have neurological symptoms only at the onset and there were no particular evidence of tumor. It is prone to misdiagnosis. The prognosis of PNS in children was poor.

Adrenocorticotropic Hormone ; therapeutic use ; Biomarkers, Tumor ; analysis ; Brain ; diagnostic imaging ; pathology ; Child, Preschool ; Female ; Ganglioneuroma ; diagnosis ; pathology ; therapy ; Humans ; Immunoglobulins, Intravenous ; therapeutic use ; Infant ; Magnetic Resonance Imaging ; Male ; Neuroblastoma ; diagnosis ; pathology ; therapy ; Opsoclonus-Myoclonus Syndrome ; diagnosis ; pathology ; therapy ; Paraneoplastic Syndromes, Nervous System ; diagnosis ; pathology ; therapy ; Prognosis ; Radiography ; Retrospective Studies