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Aims: To identify (a) determinants of glycated haemoglobin (HbA1c) at 18 and 30 months following transition in young people with Type 1 diabetes mellitus (T1DM) to a youth-specific diabetes service; and to (b) evaluate the impact of the service on acute admissions with diabetic ketoacidosis (DKA) over a 14-year period., Methods: An audit of records of youth with T1DM referred from paediatric services to the multidisciplinary transition service at Westmead Hospital, from 2001 to 2012, and followed-up to 2014., Results: Data from 439 adolescents and young adults (Median age: 18) were analysed. The recommended standard of glycaemic control, HbA1c < 7.5% (58 mmol/mol), was achieved by 23% at baseline, 22% at 18-months, and 20% at 30-month. After adjusting for lag time (>3 months) and diabetes duration (>7 years), glycaemic control at first visit predicted subsequent glycaemic control at 18-month and 30-month follow-up. From 2001 to 2014, only 8.6% were lost to follow-up; admissions and readmissions for DKA reduced from 72% (32/47) to 4% (14/340) (p < 0.001). Furthermore, mean length of stay (LOS) significantly decreased from 6.56 to 2.36 days (p < 0.001)., Conclusions: Continuing engagement with the multidisciplinary transition service prevented deterioration in HbA1c following transition. Age-appropriate education and regular follow-up prevents DKA admissions and significantly reduced admission LOS., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Aim: To determine whether cardiovascular outcomes in type 2 diabetes (T2D) differ according to ethnicity, and whether ethnicity influences the effect of gender on these outcomes in Caucasians, East-Southeast-Asians, Middle-Easterners, South-Asians and Pacific-Islanders., Methods: We compared demographics, HbA1c, lipid profile, renal function markers, and prevalence of macrovascular and microvascular complications between ethnic groups. Cross-sectional data was prospectively collected from 204 consecutive patients at Westmead Hospital's T2D clinic from April-October 2015. Univariate analysis was performed using chi-squared test for categorical data, and Mann-Whitney-U or Kruskal-Wallis test for continuous data., Results: Compared to Caucasians, South-Asians were diagnosed younger, were currently younger, had lower body-mass-index (BMI) and better renal function but higher rates of non-ST-elevation myocardial infarction (STEMI, 21.7% versus 3.5%, p<0.05). East-Southeast-Asians had lower BMI but more nephropathy than Caucasians (59% versus 39%, p<0.05). East-Southeast-Asian males had fewer CVD than Caucasians, but this protection was absent in East-Southeast-Asian females. Middle-Easterners had more non-STEMI than Caucasians (5.3% vs 3.5%, p<0.05). Middle-Eastern females were not at lower CVD risk than males. Caucasians had most PVD (20% versus 6%, p<0.05)., Conclusions: Ethnicity influences rates of diabetes-related complications. Female CVD protection is altered in some groups. Ethnicity should be considered in assessing CVD and complications risk., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Two types of retinopathy, diabetic and pigmentary, may be seen in subjects with maternal inheritance diabetes and deafness. The potential for interactions between the two retinopathies has not been explored. The mitochondrial mutation may affect development of diabetic retinopathy in subjects with MIDD by altering normal pathways of glucose metabolism. We identified five unrelated MIDD kindreds with 61 living maternal line family members. Twenty-three of the family members, 12 with diabetes mellitus and 11 without volunteered to be studied. Subjects were graded for severity of diabetic retinopathy and presence or absence of pigmentary retinopathy after slit lamp biomicroscopy, retinal photography of seven standard fields and fluorescein angiography. Blood was taken, in the fasted state, from MIDD subjects (duration of diabetes 17.0+/-6.9 yr) and non-diabetic subjects with the mutation, for assay of sorbitol and glucose and values compared with diabetic and non-diabetic control subjects without the mutation. Diabetic retinopathy was absent in 9/12 subjects (75%), with 3 having mild non-proliferative retinopathy. No one had cataract. Red blood cell sorbitol levels, adjusted for ambient blood glucose, were significantly lower in MIDD subjects compared with diabetic subjects (1.16+/-0.5 cf. 2.03+/-1.1, x 10(-3) g mmol(-1), p=0.04). Pigmentary retinopathy was present in 15 of 23 subjects, of whom 13 had some abnormality of glucose tolerance. Abnormal glucose tolerance was strongly associated with the development of pigmentary retinopathy (odds ratio 19.5, p=0.008). In conclusion, there appears to be a decreased prevalence of diabetic retinopathy and cataract in MIDD, which we propose is due to reduced glucose metabolism by the polyol pathway. Abnormal glucose tolerance increases the clinical expression of pigmentary retinopathy in subjects with a mitochondrial genome mutation. A greater understanding of the metabolic effects of mitochondrial DNA mutations has the potential to give insight into the mechanisms of diabetic retinopathy and other complications of diabetes mellitus.

Objective: While menstrual disturbance is often quoted as a feature of congenital adrenal hyperplasia (CAH), little is known about the mechanism of this symptom. We set out to determine the relationship between menstrual pattern and biochemical characteristics of women with CAH due to 21-hydroxylase deficiency., Patients and Design: All 21 female patients with classic CAH attending the adult endocrinology clinics at The Middlesex Hospital were reviewed. Their ages at menarche and menstrual pattern were recorded and blood samples were taken in the follicular phase of the menstrual cycle when on their usual maintenance therapy., Measurements: Measurements of serum LH, FSH, progesterone, 17 alpha-hydroxyprogesterone, testosterone, androstenedione and plasma renin activity were recorded. Urinary steroid profiles were obtained by gas chromatography and mass spectrometry. Molecular genetic analysis of the 21-hydroxylase gene was performed on leucocyte DNA., Results: In the 18 patients who had spontaneous menarche the degree of menstrual disturbance and progesterone excess was related to the effectiveness of adrenal suppressive therapy. Three out of 21 patients, however, failed to experience menarche on standard medical therapy. These patients with primary amenorrhoea were characterized by reduced endometrial thickening, by non-suppressible serum progesterone concentrations despite suppression of 17 alpha-hydroxyprogesterone levels and by the presence of progesterone metabolites in urinary steroid profiles. Molecular genetic analysis did not differentiate between patients with raised progesterone concentrations and those without., Conclusion: A subgroup of women with congenital adrenal hyperplasia have the triad of non-suppressible serum progesterone of adrenal origin, primary amenorrhoea and infertility due to failure of endometrial thickening. The characteristic urinary steroid profile best distinguishes this subgroup of women from others with congenital adrenal hyperplasia and menstrual disturbance due to inadequate adrenal suppression.