Corrected sodium levels for hyperglycemia is a better predictor than measured sodium levels for clinical outcomes among patients with extreme hyperglycemia.

BACKGROUND: Abnormal serum sodium levels in various diseases increase mortality; however, hyperglycemia depresses serum sodium concentration significantly. This study aimed to evaluate the clinical impact of measured serum sodium levels and corrected sodium levels among patients with severe hyperglycemia. METHODS: Patients with blood glucose levels ≥500 mg/dL visiting the emergency department between July 2008 and September 2010 were enrolled retrospectively. The participants were divided into five groups for measured sodium levels and five groups for corrected sodium levels according to blood glucose levels. Multivariate Cox regression was used. The primary outcome was all-cause 90-day mortality. Secondary outcomes included rate of intensive care unit hospitalization, respiratory failure, and renal failure. RESULTS: A total of 755 patients with severe hyperglycemia were enrolled, and the 90-day mortality was 17.2%. Compared with the reference group, the 90-day mortality was higher in the patients with measured hypernatremia (adjusted hazard ratio [HR], 2.86; 95% confidence interval [CI], 1.39-5.87), corrected hyponatremia (adjusted HR, 3.56; 95% CI, 1.44-8.80), and severe corrected hypernatremia (adjusted HR, 2.68; 95% CI, 1.28-5.62). However, patients with severe measured hyponatremia did not show increased mortality (adjusted HR, 1.67; 95% CI, 0.84-3.32). CONCLUSION: Among patients with severe hyperglycemia, corrected sodium level is a better indicator of clinical outcomes compared with measured sodium levels, especially in this population with measured hyponatremia.

A case of adrenal Cushing's syndrome with bilateral adrenal masses.

UNLABELLED: A functional lesion in corticotrophin (ACTH)-independent Cushing's syndrome is difficult to distinguish from lesions of bilateral adrenal masses. Methods for distinguishing these lesions include adrenal venous sampling and (131)I-6ß-iodomethyl-19-norcholesterol ((131)I-NP-59) scintigraphy. We present a case of a 29-year-old Han Chinese female patient with a history of hypercholesterolaemia and polycystic ovary syndrome. She presented with a 6month history of an 8kg body weight gain and gradual rounding of the face. Serial examinations revealed loss of circadian rhythm of cortisol, elevated urinary free-cortisol level and undetectable ACTH level (<5pg/mL). No suppression was observed in both the low- and high-dose dexamethasone suppression tests. Adrenal computed tomography revealed bilateral adrenal masses. Adrenal venous sampling was performed, and the right-to-left lateralisation ratio was 14.29. The finding from adrenal scintigraphy with NP-59 was consistent with right adrenal adenoma. The patient underwent laparoscopic right adrenalectomy, and the pathology report showed adrenocortical adenoma. Her postoperative cortisol level was 3.2µg/dL, and her Cushingoid appearance improved. In sum, both adrenal venous sampling and (131)I-NP-59 scintigraphy are good diagnostic methods for Cushing's syndrome presenting with bilateral adrenal masses. LEARNING POINTS: The clinical presentation of Cushing' syndrome includes symptoms and signs of fat redistribution and protein-wasting features.The diagnosis of patients with ACTH-independent Cushing's syndrome with bilateral adrenal masses is challenging for localisation of the lesion.Both adrenal venous sampling and (131)I-NP-59 scintigraphy are good methods to use in these patients with Cushing's syndrome presenting with bilateral adrenal masses.

Prognostic factors of mortality among patients with severe hyperglycemia.

OBJECTIVES: Severe hyperglycemia is associated with increased morbidity and mortality in a variety of patients. We undertook this study to identify prognostic factors of mortality among patients experiencing severe hyperglycemia in the emergency department (ED). STUDY DESIGN: Longitudinal observation study. METHODS: We recruited patients who visited the ED with blood glucose levels higher than 500 mg/dL between July 2008 and September 2010. The primary outcome was death from any cause within 90 days. Outcome analysis was first performed with Pearson's χ(2) test. Any characteristic with suspected significance (P < .1) was then used in a univariate Cox regression model. The variables found to be statistically significant were then subjected to multivariate analysis for further investigation. RESULTS: Among 733 patients with severe hyperglycemia, the 90-day mortality rate was 14.6% (n = 107). Independent prognostic factors for increasing 90-day mortality included elevated absolute neutrophil count (hazard ratio [HR], 7.34), elevated C-reactive protein (HR, 4.48), elevated blood urea nitrogen (HR, 3.04), elevated respiratory rate (HR, 2.91), decreasing body temperature (HR, 2.68), decreasing systolic blood pressure (HR, 2.65), elevated potassium (HR, 2.54), decreasing blood glucose (HR, 2.46), elevated creatinine (HR, 2.40), elevated white blood cell count (HR, 2.30), and elevated ratio of blood urea nitrogen to creatinine (HR, 2.23). CONCLUSIONS: The 90-day mortality rate among patients with severe hyperglycemia in the ED was 14.6%. Sepsis, renal impairment with electrolyte imbalance, and lower blood pressure were independent prognostic factors.

Novel mutation of RUNX2 gene in a patient with cleidocranial dysplasia.

BACKGROUND: Cleidocranial dysplasia is a rare hereditary skeletal disorder due to heterozygous loss of function mutations in the RUNX2 gene that encodes runt-related transcription factor 2 (RUNX2). Here we report a 52 year-old woman with cleidocranial dysplasia due to a novel RUNX2 mutation. CASE DESCRIPTION: A 52 year-old Han Chinese woman presented with short stature and skeletal dysplasia that was first noted during early childhood. She was 153 cm in height and 40 kg in weight. Her skull was deformed with hypertelorism, midface hypoplasia, protrusion of chin, and dental abnormalities. Radiological examination revealed shortened clavicles and depressed skull bone and that were consistent with the clinical diagnosis of cleidocranial dysplasia. There was no family history of a similar skeletal disorder. We sequenced the RUNX2 gene and discovered a novel heterozygous mutation in exon 3 (c.476 del G, p.G159fs175X) that is predicted to cause a frameshift and premature termination that leads to the loss of the final 347 amino acid residues. This severely truncated protein is expected to be inactive. LITERATURE REVIEW: RUNX2 gene controls osteoblast differentiation and chondrocyte maturation. Around 90 RUNX2 mutations have been discovered in patients with cleidocranial dysplasia. CLINICAL RELEVANCE: We identified a case of cleidocranial dysplasia due to a novel mutation of RUNX2 gene at exon 3 (c.476 del G).