A nationwide survey on Hirschsprung's disease was conducted to clarify the recent trends of diagnosis and treatment of Hirschsprung's disease seen in Japan from 1978 to 1982. A total number of 1628 cases were collected from 135 medical institutions. Estimated incidence of Hirschsprung's disease was 1/4697. In diagnosis, anorectal manometry was carried out in 64.7% of the patients and histochemistry in 28.7%. Barium enema with manometry was most frequently used in combination of the methods. A total of 48.7% of the patients were diagnosed in the first month of life. The overall rate of creation of colostomy was 61.3% in aganglionosis extended to the sigmoid colon. Retrorectal transanal pull-through was used most frequently at 57.4%, including Z-shaped anastomosis at 30.4%, then endorectal pull-through at 27.6% as the definitive operation. Considerably high incidence (12.1%-33.7%) of postoperative enterocolitis was noted after major operative procedures, but the mortality rate was low (1.8%-2.4%) except total colonic with or without small bowel aganglionosis. The large number of patients studied in the present survey reveals that there still is much room for improvement in the diagnosis and treatment of Hirschsprung's disease, especially in total colonic with or without small bowel aganglionosis.
Statins and/or PCSK9 inhibitors cause the regression of coronary atheroma and reduce clinical events. However, it currently remains unclear whether these drugs modulate coronary atheroma calcification in vivo. Coronary artery calcium (CAC) scores (Agatston Units, AUs) were estimated in 120 patients receiving coronary computed tomographic angiography (CCTA) (63% males; median age 56 years). The CAC scores were compared among the three groups: (1) neither statin nor PCSK9 inhibitor therapy, (2) statin monotherapy, and (3) statin and PCSK9 inhibitor combination therapy in an unpaired cross-sectional study. Additionally, CCTA was performed twice at an interval in 15 patients undergoing statin monotherapy to compare the previous (baseline) and subsequent (follow-up) CAC scores in a paired longitudinal study. In addition, a PCSK9 inhibitor was administered to 16 patients undergoing statin therapy. Before and after that, CCTA was performed twice to compare the previous and subsequent CAC scores in a paired longitudinal study. The unpaired cross-sectional study and paired longitudinal study consist of completely different patients. Among 120 patients, 40 (33%) had a CAC score >100 AUs. The median CAC score increased in the following order: statin group, statin and PCSK9 group, and no-statin-no-PCSK9 group. Annual CAC score progression was 29.7% by statin monotherapy and 14.3% following the addition of the PCSK9 inhibitor to statin therapy. The annual rate of CAC with the combination therapy with a PCSK9 inhibitor and a statin is lower than that with statin monotherapy. CAC may be prevented with PCSK9 Inhibitor.
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