RESUMO
We report a case of severe hypertriglyceridemia (HTG) complicated by hyperviscosity syndrome as a possible adverse reaction to risankizumab-rzaa in a 49-year-old male with a history of longstanding uncontrolled type 2 diabetes, obesity, and coronary artery disease with prior ST-elevation myocardial infarction. On admission, the patient presented with xanthomatous plaques, chest and epigastric discomfort, and headache. Subsequent blood testing revealed severely elevated triglyceride (TG) levels at 7670â mg/dL (86.59â mmol/L) [reference range: <150â mg/dL; 1.69â mmol/L] and total cholesterol at 934â mg/dL (24.14â mmol/L) [reference range: <200â mg/dL; 5.17â mmol/L]. Triglyceride levels decreased and symptoms resolved with dietary restrictions and plasmapheresis. At follow-up, his TG remained elevated but improved, and he was advised to continue lipid-lowering medications as well as cessation of risankizumab. While the patient presented with high risk factors, we posit that the subacute presentation of severe HTG is a possible result of his recent course of risankizumab-rzaa therapy for management of psoriasis. This is noteworthy as pharmaceutical surveys and clinical trials do not list severe HTG as an adverse effect. Postmarketing surveillance studies are essential to confirm this potential association and monitor drug safety. In summary, this case highlights a possible link between risankizumab and severe HTG, emphasizing the importance of ongoing pharmacovigilance to identify and manage unexpected adverse effects associated with new medications.
RESUMO
Background: Abnormal substrate on invasive electroanatomic mapping (EAM) correlates with areas of myocardial thinning and fibrofatty replacement in Arrhythmogenic Cardiomyopathy (ACM). However, EAM parameters are absent from all sets of diagnostic criteria for ACM. Case summary: A 41-year-old female with no significant family history was referred for evaluation of frequent premature ventricular complexes (PVCs). Twelve-lead ECG showed diffuse low-voltage QRS complexes. Holter monitor showed 28% burden of PVCs with various morphologies consistent with right ventricular (RV) inflow and outflow tract exits. Transthoracic echocardiogram revealed normal biventricular function and dimension. Cardiac magnetic resonance revealed a mildly increased indexed RV end-diastolic volume with normal RV systolic function and no dyssynchrony, akinesia, dyskinesia, or late gadolinium enhancement. Electrophysiologic study demonstrated 2 predominant PVC morphologies that were targeted with ablation, in addition to extensive abnormality with low-voltage and fractionated electrograms in the peri-tricuspid and right ventricular outflow tract free wall regions with septal sparing, suggestive of RV cardiomyopathy. Subsequent genetic testing revealed two pathogenic variants in the desmoplakin and plakophilin-2 genes, confirming the diagnosis of ACM. Conclusion: Advanced RV electropathy can precede RV structural changes in ACM. Invasive evaluation of the electroanatomic substrate should be considered in select cases even when imaging findings are not diagnostic. Future iterations of ACM guidelines may need to consider EAM substrate as one of the diagnostic criteria. A high index of diagnostic suspicion for ACM should be maintained in patients with multifocal RV ectopy.
RESUMO
Presympathetic neurons in the rostral ventrolateral medulla (RVLM) including the adrenergic cell groups play a major role in the modulation of several reflexes required for the control of sympathetic vasomotor tone and blood pressure (BP). Moreover, sympathetic vasomotor drive to the kidneys influence natriuresis and diuresis by inhibiting the cAMP/PKA pathway and redistributing the Na+/H+ exchanger isoform 3 (NHE3) to the body of the microvilli in the proximal tubules. In this study we aimed to evaluate the effects of renal afferents stimulation on (1) the neurochemical phenotype of Fos expressing neurons in the medulla oblongata and (2) the level of abundance and phosphorylation of NHE3 in the renal cortex. We found that electrical stimulation of renal afferents increased heart rate and BP transiently and caused activation of tyrosine hydroxylase (TH)-containing neurons in the RVLM and non-TH neurons in the NTS. Additionally, activation of the inhibitory renorenal reflex over a 30-min period resulted in increased natriuresis and diuresis associated with increased phosphorylation of NHE3 at serine 552, a surrogate for reduced activity of this exchanger, in the contralateral kidney. This effect was not dependent of BP changes considering that no effects on natriuresis or diuresis were found in the ipsilateral-stimulated kidney. Therefore, our data show that renal afferents leads to activation of catecholaminergic and non-catecholaminergic neurons in the medulla oblongata. When renorenal reflex is induced, NHE3 exchanger activity appears to be decreased, resulting in decreased sodium and water reabsorption in the contralateral kidney.