Determinants of Cardiovascular Risk in 7000 Youth With Type 1 Diabetes in the Australasian Diabetes Data Network.

CONTEXT: Cardiovascular disease occurs prematurely in type 1 diabetes. The additional risk of overweight is not well characterized. OBJECTIVE: The primary aim was to measure the impact of body mass index (BMI) in youth with type 1 diabetes on cardiovascular risk factors. The secondary aim was to identify other determinants of cardiovascular risk. DESIGN: Observational longitudinal study of 7061 youth with type 1 diabetes followed for median 7.3 (interquartile range [IQR] 4-11) years over 41 (IQR 29-56) visits until March 2019. SETTING: 15 tertiary care diabetes centers in the Australasian Diabetes Data Network.Participants were aged 2 to 25 years at baseline, with at least 2 measurements of BMI and blood pressure. MAIN OUTCOME MEASURE: Standardized systolic and diastolic blood pressure scores and non-high-density lipoprotein (HDL) cholesterol were co-primary outcomes. Urinary albumin/creatinine ratio was the secondary outcome. RESULTS: BMI z-score related independently to standardized blood pressure z- scores and non-HDL cholesterol. An increase in 1 BMI z-score related to an average increase in systolic/diastolic blood pressure of 3.8/1.4 mmHg and an increase in non-HDL cholesterol (coefficient + 0.16 mmol/L, 95% confidence interval [CI], 0.13-0.18; P < 0.001) and in low-density lipoprotein (LDL) cholesterol. Females had higher blood pressure z-scores, higher non-HDL and LDL cholesterol, and higher urinary albumin/creatinine than males. Indigenous youth had markedly higher urinary albumin/creatinine (coefficient + 2.15 mg/mmol, 95% CI, 1.27-3.03; P < 0.001) and higher non-HDL cholesterol than non-Indigenous youth. Continuous subcutaneous insulin infusion was associated independently with lower non-HDL cholesterol and lower urinary albumin/creatinine. CONCLUSIONS: BMI had a modest independent effect on cardiovascular risk. Females and Indigenous Australians in particular had a more adverse risk profile.

Reversal of end-stage heart failure in juvenile hemochromatosis with iron chelation therapy: a case report.

BACKGROUND: Juvenile hemochromatosis is the most severe form of iron overloading phenotype. Although rare, it should be suspected in patients who present with hypogonadotropic hypogonadism, diabetes mellitus, or cardiomyopathy without a clear cause. CASE PRESENTATION: A young Serbian male presenting with end-stage heart failure was referred for extracorporeal membrane oxygenation. An endomyocardial biopsy revealed cytoplasmic iron deposits in myocytes. His condition was stabilized with biventricular assist devices and he was listed for heart transplantation. Iron chelation therapy was commenced and resulted in rapid removal of iron burden. Serial outpatient echocardiograms demonstrated myocardial recovery such that a successful biventricular assist device explant occurred 131 days after initial implant. Targeted gene sequencing revealed a loss-of-function mutation within the HJV gene, which is consistent with juvenile hemochromatosis. CONCLUSIONS: This rare case of a patient with juvenile hemochromatosis associated with a HJV mutation provides histologic evidence documenting the reversal of associated end-stage heart failure, requiring emergent mechanical circulatory support, with iron chelation therapy.

The high burden of inpatient diabetes mellitus: the Melbourne Public Hospitals Diabetes Inpatient Audit.

OBJECTIVE: To determine the prevalence of diabetes in inpatients in Melbourne hospitals. DESIGN: Point prevalence survey of all inpatients in each hospital on a single day between 30 November 2010 and 22 November 2012. SETTING: 11 hospitals in metropolitan Melbourne including community, secondary and tertiary hospitals and one aged care and rehabilitation centre. PARTICIPANTS: 2308 adult inpatients in all wards apart from intensive care, emergency, obstetrics and psychiatry. MAIN OUTCOME MEASURES: Point prevalence of self-reported diabetes, details of current medication, self-reported frequency of complications. RESULTS: Diabetes status was obtained in 2273 of 2308 inpatients (98.5%). Of these, 562 (24.7%) had diabetes (95% CI, 22.9%-26.5%). Diabetes prevalence ranged from 15.7% to 35.1% in different hospitals (P < 0.001). Patients with diabetes were older, heavier and more likely to be taking lipid-lowering, antihypertensive and blood-thinning medications. Of 388 patients with complete medication information, 270 (69.6%) were taking oral hypoglycaemic agents alone or in combination with insulin, 158 (40.7%) were treated with insulin (67 [17.3%] with insulin alone) and 51 (13.1%) were not taking medication for diabetes. The frequency of diabetes complications was very high: 207/290 (71.4%) for any microvascular complication, 275/527 (52.2%) for any macrovascular complication and 227/276 (82.2%) for any complication. CONCLUSION: The high burden of diabetes in Melbourne hospital inpatients has major implications for patient health and health care expenditure. Optimising care of these high-risk patients has the potential to decrease inpatient morbidity and length of stay as well as preventing or delaying future complications. A formal Australian national audit of inpatient diabetes would determine its true prevalence and consequences, allowing rational planning to deal with shortcomings in its management.

Patients presenting with severe hypotonic hyponatremia: etiological factors, assessment, and outcomes.

BACKGROUND: Although hospital-acquired hyponatremia is well described, severe community-acquired hyponatremia has been studied less extensively. AIM: To assess characteristics and outcomes of patients admitted with severe hypotonic hyponatremia (SHH) (defined as serum sodium ≤ 120 mmol/L). METHODS: All patients with serum sodium of ≤ 120 mmol/L who were admitted to 2 large teaching hospitals from January 2000 to August 2007 were identified, and data were obtained from medical records. Main outcome measures were incidence of osmotic demyelination and mortality. RESULTS: Two hundred fifty-five patients were admitted who had SHH (female to male ratio 2:1), and the mean age was 72 ± 14 years. The most common etiological factors were thiazide/indapamide diuretics (41%), syndrome of inappropriate antidiuretic hormone secretion (38%), and hypovolemia (24%). Inappropriately rapid correction of serum sodium (> 12 mmol/L over the first 24 hours) occurred in 37 patients (15%), with 4 patients (11%) developing osmotic demyelination. Patients who developed osmotic demyelination were more likely to be younger, abuse alcohol (3 of 4 patients), and have lower serum potassium levels. One patient had a hypoxic-anoxic episode at presentation. The patients also had a mean serum sodium increase in the first 24 and 48 hours of 21 ± 5 mmol/L and 28 ± 8 mmol/L, respectively. None of the patients with osmotic demyelination received hypertonic saline. None of the patients in whom the serum sodium increment was limited to ≤ 12 mmol/L developed osmotic demyelination. Overall, mortality was 10% and was not related to sodium level at presentation. CONCLUSIONS: Patients treated with thiazide or indapamide (particularly elderly women) may benefit from monitoring of serum sodium levels. Inappropriately rapid serum sodium correction is associated with osmotic demyelination, particularly in patients with risk factors for this condition. In contrast to what has been reported for hyponatremia in hospitalized patients, severity of hyponatremia on admission did not predict increased mortality in our patient population.

Gonadotroph adenoma in multiple endocrine neoplasia type 1.

OBJECTIVE: To describe a case of an in vivo follicle-stimulating hormone (FSH)-secreting gonadotroph adenoma in a man with multiple endocrine neoplasia type 1 (MEN 1) syndrome. METHODS: We present a retrospective case description and a discussion of the related literature. RESULTS: A 48-year-old man had progressively deteriorating visual acuity and bitemporal hemianopia, found to be attributable to a macroadenoma encircling both carotid arteries and compressing the optic chiasm. His libido and erectile function had been reduced for 2 years. The serum FSH level was substantially elevated at 31.1 IU/L (reference range, 1 to 10); alpha-subunit was elevated at 2.25 IU/L (reference range, 0.09 to 0.4); luteinizing hormone was normal at 4 IU/L (reference range, 1 to 10); total testosterone was low at 6.8 nmol/L (reference range, 9.5 to 35); and prolactin was slightly increased at 433 mU/L (reference range, 50 to 300). Transsphenoidal hypophysectomy was performed. Pituitary histopathologic examination showed a tumor with cytoplasmic granular FSH immunoreactivity. The patient had undergone parathyroidectomy for primary hyperparathyroidism 2 years before the current intervention. A family history of endocrine neoplasia was obtained of one sibling with a nonfunctioning pituitary adenoma, another sibling who had died of pancreatic carcinoma, and a third sibling, along with her son, who has primary hyperparathyroidism. Performance of genetic testing for MEN 1 revealed a nonsense mutation--R460X (nt7605C>T)--located on exon 10 of the MEN1 gene. CONCLUSION: We report an unusual case of clearly high circulating immunoreactive FSH due to a functioning FSH-secreting gonadotroph adenoma in a man with the MEN 1 syndrome.