Metabolic Syndrome in Adults Receiving Clozapine; The Need for Pharmacist Support.

People who are diagnosed with treatment resistant schizophrenia (TRS) are likely to have clozapine as a therapeutic management option. There is a high prevalence of metabolic syndrome in patients receiving clozapine. To mitigate against this, monitoring of weight, waist circumference, lipid profile, glycated haemoglobin (HbA1c), fasting blood glucose (FBG) and blood pressure (BP) is recommended. The aims of this study were to examine the prevalence of metabolic syndrome and whether any variables were correlated with its development, and to highlight any opportunities for the pharmacist to offer support. This study was conducted in an urban hospital and its associated Clozapine Clinic in Cork, Ireland. A retrospective audit assessed the prevalence of metabolic syndrome using the International Diabetes Federation (IDF) criteria. Patients were eligible for inclusion if they were aged 18 years or more, registered with the Clozapine Clinic, and had the capacity to provide informed consent. All data were entered into Microsoft® Excel ® (Microsoft Corporation) and further statistical analysis was undertaken using R, t-tests, Fisher's Exact Test and Mann-Whitney U tests as appropriate, and p ≤ 0.05 was considered statistically significant. Of 145 patients (32% female; mean age (SD) 45.3 (±11.7) years; 86.2% living independently/in family home), nearly two thirds (n = 86, 59.3%) were diagnosed with metabolic syndrome. The mean age of participants with metabolic syndrome was 44.4 years (SD = 10.8), similar to the 46.6 years (SD = 12.8) for those without. Variables that were identified to be statistically significantly associated with metabolic syndrome included waist circumference, weight, triglycerides, high density lipoprotein-cholesterol (HDL-C), BP, FBG and HbA1c. The high incidence of metabolic syndrome in this patient population highlights the need for continued physical health monitoring of these patients to ameliorate the risk of developing metabolic syndrome.

Hepatorenal dysfunction assessment with the Model for End-Stage Liver Disease Excluding INR score predicts worse survival after heart transplant in pediatric Fontan patients.

BACKGROUND: Fontan physiology results in multiorgan dysfunction, most notably affecting the liver and kidney. We evaluated the utility of Model for End-Stage Liver Disease Excluding INR (MELD-XI) score, a score evaluating the function of both liver and kidney to identify Fontan patients at increased risk for morbidity and mortality post-heart transplant. METHODS: The Pediatric Heart Transplant Society database was queried to identify Fontan patients listed for heart transplant between January 2005 and December 2018. MELD-XI scores were calculated at listing and heart transplant. A multivariable analysis was conducted to identify risk factors for post-heart transplant mortality. Demographic, clinical characteristics, and survival differences were evaluated and compared between the high and low MELD-XI score cohorts. The impact of changing MELD-XI scores during the waitlist period on post-heart transplant outcomes was also evaluated. RESULTS: Of 565 Fontan patients who underwent transplantation, 524 (93%) had calculable MELD-XI scores at the time of heart transplant: 421 calculable at listing and 392 calculable at listing and at heart transplant. On multivariable analysis, only MELD-XI score (squared) (hazard ratio, 1.007), history of protein-losing enteropathy (hazard ratio, 2.1), and ventricular assist device use at transplant (hazard ratio, 3.4) were risk factors for early phase post-heart transplant mortality. Patients with high MELD-XI scores at heart transplant had inferior survival post-heart transplant (P = .02); those in the high MELD-XI score cohort at wait listing and heart transplant tend to have the worst post-heart transplant survival; however, this was not significant (P = .42). CONCLUSIONS: The MELD-XI, an easily calculated score, serves as a valuable aid in identifying pediatric Fontan patients at increased risk for post-heart transplant mortality.

Evaluation and management of renal tumors in the Birt-Hogg-Dubé syndrome.

PURPOSE: Herein we describe the evaluation and management of renal tumors in Birt-Hogg-Dubé (BHD), an autosomal dominant disorder predisposing to cutaneous fibrofolliculomas, pulmonary cysts, spontaneous pneumothorax and renal tumors. MATERIALS AND METHODS: A total of 124 affected individuals underwent comprehensive clinical evaluation, including body computerized tomography, to determine cutaneous, pulmonary and renal manifestations of BHD. Of these individuals 14 had their renal tumors managed at our institution. RESULTS: Of the 124 BHD affected individuals 34 (27%) had renal tumors of various histologies, most commonly hybrid oncocytic tumor and chromophobe renal carcinoma. Average age at renal tumor detection was 50.4 years and multiple tumors were found in a majority of patients. Some patients with renal tumors were identified that did not have the characteristic cutaneous hallmarks of BHD. In 4 of the 14 patients treated at our institution small (less than 3 cm) renal tumors were observed, while 10 others underwent a total of 12 renal procedures, including 4 radical and 8 partial nephrectomies. At a median of 38 months of followup 5 of these 10 patients remained free of disease, 3 had small renal tumors and 2 died of metastatic renal cancer. CONCLUSIONS: Patients with BHD are at risk for multiple renal tumors that are often malignant and can metastasize. Individuals at risk or affected by BHD should be radiographically screened for renal tumors at periodic intervals and they are best treated with nephron sparing surgical approaches. Genetic testing for this syndrome is now available.