Biography:

Su Zhen has completed her MD from Wenzhou Medical University; PhD from Second Military Medical University and; Post-doctoral studies from Emory University School of Medicine. She is the Vice-Director of Renal Division at First Affiliated Hospital of Wenzhou Medical University. She has published more than 40 papers in reputed journals and had oral presentation in ASN 2005 (American Society of Nephrology) meeting and WCN 2007 (ISN World Congress of Nephrology) meeting.

Abstract:

Background & Aim: There were few related studies aiming to severe IgA nephropathy (IgAN) which could progress rapidly to end stage renal disease (ESRD) within ten years. To find valuable clinical or pathological factors and promising precautions is essential.

Method: A single center case-control study was performed. 50 ESRD patients with the primary cause of IgAN and a short renal survival time of less than ten years after diagnose were enrolled in the case group. 100 IgAN patients with a renal survival time of more than ten years were enrolled in the control group. IgA Oxford classification scores, clinical data at baseline and during the follow-up were collected. Multivariate logistic regression was used to investigate factors associated with the development of ESRD.

Results: There were significant differences in baseline clinical data between these two groups, as well as the constituent ratio of Oxford MEST-score. Distinct differences were observed in time-average uric acid (TA-UA), time-average hemoglobin (TA-Hb), time-average albumin (TA-Alb), time-average total cholesterol (TA-TC) and time-average urinary protein (TA-P) during the follow-up. In multivariate logistic models, IgA oxford score M1 (OR=5.10, P=0.018) and eGFR (OR=0.97, P=0.039) at biopsy, TA-UA (OR=2.06, P=0.026) and TA-Hb (OR=0.53, P=0.022) during the follow-up were identified independent factors for developing ESRD.

Conclusion: IgAN patients with pathological assessment of M1, low baseline eGFR, TA-Hb and high TA-UA were more likely to progress to ESRD, and should be paid more attention. Appropriate regulations of UA, Hb and urine protein after diagnose may be a promising treatment.

Biography:

Surjit Tarafdar is a Nephrologist working at Blacktown Hospital in Sydney since 2014 and has additional position of Conjoint Lecturer at Western Sydney University Medical School since March 2014. He had conceptualized and eventually co-written “Passing the FRACP Written Examination-Questions and Answers” published by Wiley-Blackwell in 2013. He has started the annual “Revise Nephrology”, a weekend nephrology refresher programme for trainees with the aim of helping them to understand nephrology better and pass the FRACP theory examination. In 2017, 182 doctors attended Revise Nephrology. He is currently editing a nephrology book entitled “Lecture Notes in Nephrology” which is due to be published by Wiley-Blackwell publishing house in April 2018.

 

Abstract:

Defect of CYP11B1, or 11-beta-hydroxylase 1(11β-OH 1) deficiency causes 5% of congenital adrenal hyperplasia (CAH). A 27-year-old male with history of hypertension from the age of 21 and non-compliance was found to have hypokalaemia with metabolic alkalosis. Investigations revealed he had primary aldosteronism with an aldosterone/renin ratio of more than 200 (normal <30). CT imaging of the adrenal glands showed hyperplastic right adrenal gland. Given the young age and strong family history of early onset hypertension patient had genetic tests which revealed a large deletion in CYP11B1. No evidence of hyperandrogenism was found. Subsequently patient’s 52 year old father who had been hypertensive since his twenties was investigated and found to have primary hyperaldosteronism with the same genetic defect. Both father and son responded very well to spironolactone. 11β-OH1 associated CAH is characterized by hyperandrogenism along with accumulation of 11-deoxycortisol and 11-deoxycorticosterone. 11-deoxycorticosterone (which has intrinsic mineralocorticoid activity) causes hypokalemic hypertension and suppressed aldosterone production. However, our patient family has a significantly elevated aldosterone to renin ratio suggesting that blockage of the cortisol pathway caused activation of the mineralocorticoid pathway rather than the androgen pathway. This is the first documented case of a deletion in gene CYP11B1 presenting with hypertension and primary aldosteronism rather than hyperandrogenism and hypertension with hypoaldosteronism. This group of patients should be recognized as a new subset of familial hyperaldosteronism rather than CAH.