Adrian Kark

Royal Brisbane Hospital , Brisbane, Queensland, Australia

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Publications (7)14.79 Total impact

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    ABSTRACT: Background: Renal oncocytosis is a rare histopathological finding which can be the precursor for oncocytoma and chromophobe renal cell cancer, usually presenting as bilateral renal nodules. It has been associated with Birt-Hogg-Dubé syndrome, an autosomal dominant disorder characterised by FLCN gene mutations. Case Report: A 68 year old female presented with progressive decline in renal function over 6 months, to CKD stage IV with no physical symptoms. Past medical history included indeterminate inflammatory arthralgia, left lung adenocarcinoma (T1N2; resected 1999 with durable cure), ischemic heart disease, hypertension and Hashimoto's thyroiditis. There was no personal or family history of pneumothorax, renal lesions or kidney disease. Examination was normal with no cutaneous abnormalities. Investigation showed elevated urine protein: creatinine (37 g/mol), inactive urinary sediment and unremarkable renal ultrasound. Renal biopsy demonstrated acute tubulointerstitial nephritis with mild cortical atrophy. There were also clusters of tubules with renal oncocytosis (expansion of tubules by cells with abundant eosinophilic cytoplasm and nuclear atypia on multiple histological levels). Subsequent bilateral renal MRI showed no renal lesions. FLCN gene analysis revealed a previously unreported rare variant of predicted though invalidated pathogenicity. Renal function has recovered somewhat at 6 months of follow up with last serum creatinine 144umol/L (eGFR 21 mL/min/1.73 m2, CKD-EPI). Genetic counselling has been undertaken. Long term renal follow up and annual screening for development of renal lesions is planned in keeping with standard Birt-Hogg-Dubé Syndrome protocols. Conclusions: This case demonstrates the association between renal oncocytosis and a rare FLCN gene variant. Furthermore this may be a new novel mutation responsible for Birt-Hogg-Dubé syndrome, however further validation is required and protocol screening is indicated in the interim.
    Nephrology 08/2014; 19(S1):94. · 1.69 Impact Factor
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    ABSTRACT: The study objective was to develop and evaluate the feasibility and validity of a self-administered Scored Sodium Questionnaire (SSQ) for use in the routine clinical care of Australian chronic kidney disease (CKD) patients. The study took place in community-based outreach clinics using a multidisciplinary model of care. Assessment of sources of dietary sodium intake in the target population used comprehensive diet history interviews (Phase 1) to inform development of a 10-item food frequency questionnaire that was scored and validated using 24-hour urinary sodium and 2 alternative dietary intake methods (Phase 2). Subjects were adults with CKD Stages 3 to 5 (Phase 1 n = 30; Phase 2 n = 47). On a single day, participants completed the SSQ, feasibility survey, 24-hour urine collection, and 24-hour food record. A diet history interview was also conducted to confirm sodium intake on the day of data collection reflected habitual intake. Validity of the SSQ score was confirmed by correlation with 24-hour urine sodium. Validity of a cutpoint on the SSQ score to correctly identify high- versus low-sodium consumers was confirmed by receiver operating characteristic curve analysis: area under the curve, sensitivity, and specificity. Total SSQ score correlated significantly with 24-hour urine sodium (r = 0.371; P = .031). Correlation between 24-hour food record and diet history sodium confirmed consumption on the data collection day reflected habitual intake (r = 0.701; P ≤ .001). A cutpoint of 65 or greater on the SSQ score was confirmed as valid to identify high-sodium consumers: area under the curve 0.713, sensitivity 61%, and specificity 82%. The SSQ is feasible and valid to assess habitual sodium intake in the Australian CKD population and to identify high-sodium consumers for referral to individualized counseling on a low-sodium diet.
    Journal of Renal Nutrition 01/2014; · 1.75 Impact Factor
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    ABSTRACT: Background: Chronic Kidney Disease (CKD) contributes to 10% of deaths and 15% of hospitalisations, mainly for dialysis. Although the most common chronic disease in Australia, CKD data are minimal and reliant on extrapolations from mortality and end stage kidney disease (ESKD) databases. Aim: To describe how CKD populations differ from ESKD populations and the National Mortality Dataset (NMD). Methods: We extracted demographic and kidney disease specific data for the first 740 RBWH patients entered into the CKD.QLD Registry. Patients were registered if they had at least one biomarker of kidney injury for at least 3-months. Data were cross tabulated with the 2010 ANZDATA, a dataset of treated ESKD, and with the 2006 NMD published by the Australian Institute of Health and Welfare (AIHW). The latter records limited kidney disease data. Findings: The CKD population is older than treated ESKD – mean ages of 64.1 vs 59.6 females and 65.8 vs 61.4 years males. The distribution is similar to the AIHW dataset of CKD, including ESKD irrespective of dialysis treatment. Only 1.8% identified as aboriginal. Mean eGFR was 37 mls/min/1.73 m2 (IQR 24.9-51.2). Stage 3 was most common (47.2%), 33.4% stages 4 and 5 and 19.5% stages 1 and 2. Renovascular and renovascular (HTN) were the most common primary renal diseases (27.8%), followed by glomerulonephritis (10.14%) compared to treated ESKD where diabetic nephropathy (38%) was the most common diagnosis and renovascular disease 13%. Primary diagnosis could not be extracted in 31% compared to 5% in treated ESKD. Hypertension and diabetes were comorbidities in 49% and 27.4% in the CKD.QLD dataset. Conclusions: The CKD.QLD Registry is the first practice glimpse into the large CKD population who are not on ESKD treatment. They differ from the latter in several important respects and the longitudinal monitoring functionality of CKD.QKD will greatly supplement information in existing datasets.
    Internal Medicine Journal 05/2013; 43(S3):42. · 1.82 Impact Factor
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    ABSTRACT: Aim: To demonstrate the utility of the CKD.QLD Registry to answer clinical questions. Background: CKD.QLD is a research collaborative studying chronic kidney disease (CKD) patients in the public renal system in Queensland. Funded by Amgen, Queensland Health and the NHRMC, this is the fi rst such systematic study in Australia. Methods: CKD.QLD established a Registry of data recorded during usual care of consenting patients. The fi rst data sampling was with the 1,000th patient consented. 560 were from the Royal Brisbane and Women’s Hospital (RBWH), the fi rst centre to participate. We report preliminary analyses of these data and comparison with external datasets. Results: Of the fi rst 560 RBWH patients, 267 (47.7%) were female with mean (median) ages of 64.1 (68.5) for females and 65.8 (70.1) for males. These are older than patients recorded in ANZDATA 2010 (59.6 and 61.4 yrs respectively). Their age distribution is comparable to those recently described by AIHW in the estimated total incidence of end stage kidney disease in Australia, whether they started renal replacement therapy (RRT) or died with renal failure without RRT. This is the fi rst practice glimpse into the large CKD population who do not receive RRT. Only 1.8% identifi ed as aboriginal. Mean eGFR was 37 mls/ min/1.73 m2 (IQR 24.9–51.2). Stage 3 was the most common (47.2%), 33.4% stages 4 and 5 and 19.5% stages 1 and 2. Renovascular and renovascular (HTN) was the most common primary renal disease (27.8%) and glomerulonephritis was 10.14%. Primary diagnosis could not be extracted in 31%. Hypertension and diabetes were comorbidities in 49% and 27.4%. Conclusions: The CKD.QLD Registry provides characteristics of the CKD patient population in renal care, which greatly supplement information in existing datasets.
    Nephrology 01/2012; 17(S2):79. · 1.69 Impact Factor
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    ABSTRACT: Calciphylaxis continues to present a clinical challenge for patient management. As in this case, it can be associated with connective tissue disease (CTD) such as systemic lupus erythematosus (SLE). Unlike previous reported cases, long-term remission has been attained. This provides some insight into methods of therapy as well as potential pathogenic models for this disease.
    Lupus 10/2011; 21(4):441-4. · 2.78 Impact Factor
  • Nephrology 01/2011; 16(S1):44-69. · 1.69 Impact Factor
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    ABSTRACT: An optimal haemoglobin (Hb) response to erythropoietin requires elevated iron indices in dialysis patients; however, it is unknown if the same applies in chronic kidney disease (CKD). One hundred patients [CKD Stages 3-5, Hb >or= 110 g/L, iron replete, erythropoietin-stimulating agent (ESA)-naive, 47% diabetic, median age 69.5 years] were block-randomized in an open-label study to receive up to 200 mg intravenous iron sucrose (Group A, n = 52) bimonthly or oral iron sulphate (Group B) to maintain raised and normal iron indices (respectively) over 12 months. The primary endpoint was the change in Hb concentration at 12 months or at termination after at least 6 months of treatment. Eighty-five patients reached the primary endpoint (43, Group A; 42, Group B). Initial Hb was 119 +/- 7 vs 116 +/- 12 g/L (mean +/- standard deviation); ferritin 122 (71-176), median (inter-quartile range), vs 90 microg/L (58-150); transferrin saturation (TSat) 22 (18-26) vs 21% (15-24); and creatinine 240 (195-313) vs 230 micromol/L (184-352). Ferritin and TSat differed by month 2 [157 (103-220) vs 96 microg/L (73-162), P = 0.003] and month 6 [25 (20-31) vs 21% (17-27), P = 0.02], respectively. At study end, Hb did not differ between groups (121 +/- 10 vs 117 +/- 13 g/L). Ferritin was 362 (310-458) vs 125 microg/L (84-190), P < 0.001; TSat 30 (23-34) vs 21% (18-24), P < 0.001; and creatinine 229 (188-326) vs 272 micromol/L (195-413), P = NS. For patients (Groups A and B, n = 27 in each group) whose creatinine regression slope increased (indicating worsening function), the fall in Hb over 12 months also did not differ between groups despite adequate separation in iron indices. Serious adverse events overall did not differ between groups. Elevated iron indices did not increase Hb synthesis in ESA-naive, iron replete, pre-dialysis patients with Hb >110 g/L.
    Nephrology Dialysis Transplantation 11/2009; 25(3):920-6. · 3.37 Impact Factor