We report on a patient with protein-losing enteropathy (PLE) after undergoing total cavopulmonary connection (TCPC) operation who was introduced to subcutaneous immunoglobulin (SCIG) home therapy as immunoglobulin G (IgG) replacement therapy. The patient was a 21-year-old man with hypoplastic left heart syndrome who developed edema and ascites secondary to PLE 5 years after the TCPC operation. When he was 15-yearsold, intravenous immunoglobulin (IVIG) was administered for the treatment of hypogammaglobulinemia. SCIG home therapy was introduced when he was 20 years old. The serum IgG level did not increase with the initial SCIG dose (8 g/week); therefore, we increased the SCIG dose (16 g/week). After SCIG administration, the serum albumin level was reduced from 2.7 to 2.4 g/dL, IgG level increased from 370 to 484 mg/dL, and the total duration of the hospital stay was shortened from 4.7 to 1.2 d/month. With regard to adverse events, a local reaction was observed at the subcutaneous infusion site, but this improved with time. These findings suggest that SCIG home therapy is effective in increasing and maintaining serum IgG levels and avoiding hospitalization. The appropriate SCIG dose for patients with PLE is unknown. We believe that this should be considered on a case-by-case basis depending on the severity of each case.
Intracardiac thrombosis may occur in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). Most thrombi are treated with warfarin, but some are treated with novel oral anticoagulants (NOAC). We describe a case of a 30-year-old man with a thrombus that was successfully dissolved by treatment with dabigatran etexilate. The patient was diagnosed with ARVC at the age of 15 years. At the age of 16, he was prescribed carvedilol and enalapril for the treatment of heart failure. However, at the age of 30, echocardiography detected a thrombus in the right ventricle. Although oral warfarin and intravenous unfractionated heparin were initiated, the size of the thrombus did not change, and on day 3 of therapy initiation, we switched from warfarin to dabigatran etexilate. On day 35, the thrombus was eliminated. Dabigatran etexilate may be effective in the treatment of intracardiac thrombosis in patients with ARVC. Adequate anticoagulation is needed to prevent severe complications such as thromboembolism or sudden death.
The aim of this study was to investigate whether the different effect on sympathetic activity between nisoldipine, long‐acting L‐type calcium channel blocker, and cilnidipine, L‐ and N‐type calcium channel blocker, using an identical group of healthy subjects.
Eight healthy men (22 to 28 years) were given 10mg nisoldipine or 10mg cilnidipine in a randomized crossover design. In each trial, without medication on day 1 (control) and with medication on day 2, subjects were measured heart rate(HR), high frequency(HF)/low frequency(LF) of HR variability and plasma noradrenaline(NA) during head‐up tilt test and palmer sweating during mental arithmetic test before and 1,2,4,6, and 8 h after administration time. Plasma concentration profiles were similar between both drugs. Two‐occasional controls showed no significant difference in these parameters. Head‐up tilt increased HR, LF/HF and plasma NA. There were no significant difference in HR and plasma NA in supine and head‐up positions at any measured times between both drugs. LF/HF (3.3±1.1) in head‐up position at 4 h during cilnidipine was significantly lower than that (6.2±1.6) during nisoldipine (p=0.012). None caused postural hypotension. While there was no difference in mental arithmetic induced sweating secretion between both drugs. Cilnidipine might have a weak inhibitory effect on reflex sympathetic activity induced by head‐up tilt compared to nisoldipine.
Clinical Pharmacology & Therapeutics (2004) 75, P6–P6; doi:
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