Statin-Induced Myopathy in a Patient with Schwartz-Jampel Syndrome.

Schwartz Jampel syndrome (SJS) is a genetic disorder characterized by myotonia and chondrodysplasia. Mutations of the Perlecan gene (HSPG2), which encodes a key component of the extracellular matrix of muscle, bone, and cartilage is cause for the characteristic dysmorphisms of SJS. Clinically remarkable creatinine phosphokinase (CPK) levels are typical and can be associated with myotonia as an underlying cause in SJS patients. We report a unique case of a symptomatic adverse event of statin use in a SJS patient who demonstrated heightened levels of CPK to baseline following a statin induced myopathy. Discontinuation of the statin and administration of a PCSK-9 inhibitor revealed a return to baseline CPK. This case challenges the current lipid treatment algorithm as it pertains to SJS patients. Further investigation into treatment is required in this special population.

A First Report of HCTZ and Dicyclomine Induced Uncharacteristic Contraction Alkalosis.

Background: Contraction alkalosis is characterized by low serum sodium and chloride and high serum carbon dioxide and bicarbonate levels. Case Report: A 28-year-old Caucasian active-duty male with a history of autosomal dominant polycystic kidney disease and diarrhea-predominant Irritable Bowel Syndrome (D-IBS) presented to his primary care provider (PCP) with elevated blood pressure (136/96 mmHg), was diagnosed with stage-2 hypertension, and started oral HCTZ (25 mg/day). His medications included dicyclomine (10 mg oral three times daily). Subsequently, (Visit 1), his blood pressure was 130/91 mmHg and he was started on telmisartan (20 mg/day). At Visit 2, 4 weeks later, his blood pressure improved (121/73 mmHg); however, blood chemistry revealed elevated serum CO2 (32 mEq/L) and chloride (94 mmol/L). Four days later, the patient presented to the Emergency Department with dyspnea and swallowing difficulty. The patient returned to his PCP 3 days later complaining of cough, congestion, vomiting, and mild dyspnea, blood pressure of 124/84 mmHg. Two months later, sudden onset of projectile vomiting and abdominal pain while running was reported, resolved by rehydration and a single oral dose of prochlorperazine 25 mg. Three months later, (Visit 3), he complained of lightheadedness and cloudy judgment, suggesting contraction alkalosis. HCTZ was discontinued and telmisartan was increased to 20 mg twice daily. A follow-up blood chemistry panel 2 weeks later revealed serum chloride and CO2 levels within normal limits and blood pressure under 130/80 mmHg. Conclusion: This is the first known report of contraction alkalosis driven by drug-drug interaction between dicyclomine and HCTZ.

Effects of oral Aloe vera on electrocardiographic and blood pressure measurements.

PURPOSE: The effects of oral aloe vera on electrocardiographic and blood pressure measurements were evaluated. METHODS: In this double-blind, placebo-controlled, crossover study, healthy volunteers over age 18 years received either 1200 mg of oral aloe vera powder or matching placebo on day 1 of the study and the treatment not received during the first phase on day 8. In each phase, electrocardiographic variables, systolic blood pressure, and diastolic blood pressure were evaluated at baseline and one, three, five, and eight hours after treatment. The primary endpoint was the maximum posttreatment Q-Tc interval over eight hours in both groups. RESULTS: Sixteen participants were enrolled in the study, with a mean ± S.D. age of 25 ± 5 years. No significant differences in electrocardiographic or blood pressure measurements were observed. The maximum Q-Tc interval was 419 ± 17 milliseconds in the placebo group and 422 ± 17 milliseconds in the aloe-treated group. The maximum P-R intervals in the placebo- and aloe-treated groups were 166 ± 22 and 169 ± 25 milliseconds, respectively. The maximum QRS complex duration did not significantly differ between the placebo- and aloe-treated groups (89.4 ± 9 and 89.3 ± 9 milliseconds, respectively). The maximum systolic blood pressures in the placebo- and aloe-treated groups were 120 ± 16 and 120 ± 14 milliseconds, respectively. The maximum diastolic blood pressures in the placebo- and aloe-treated groups were 74 ± 10 and 75 ± 9 milliseconds, respectively. CONCLUSION: A single dose of oral aloe vera had no effect on electrocardiographic or blood pressure measurements in young healthy volunteers.