ObjectiveTo investigate the clinical and pathological features of Hirschprung disease (HD), intestinal neuro-nal dysplasia (IND) and hypoganglionosis (IH) in children.MethodsThe clinical data and pathologic slices from 238 children with intestinal dysganglionosis were retrospectively analyzed. The age, sex, involved intestinal length of children and prognosis were compared.ResultsIn 238 patients, 138 (58.0%) were diagnosed by rectal mucosal biopsies. There were 122 HD patients whose median age at diagnosis was 9 months and the ratio of male to female was 4.3:1, without involvement of whole colon. There were 45 IND patients whose median age at diagnosis was 14 months and the ratio of male to female was 1.05:1, and the whole colon of 33.3% patients was involved. There were two male IH patients whose ages at diagnosis were 12 years and 18 years respectively, and their whole colon was involved. There were 59 patients with HD complicated by IND whose median age at diagnosis was 13 months and the ratio of male to female was 5.56:1 and the whole colon of 16.9% patients was involved. There were 10 male patients with HD complicated by IH whose median age at diagnosis was 11.5 months and the whole colon of 80.0% patients was involved. The ages at diagnosis, the sex ratio, the rates of whole colon involved, and the cure rates among 5 groups were signiifcantly different (allP<0.01).ConclusionsThe rectal mucosal biopsy was the main method in diagnosis of intestinal dysganglionsis in children. Patients with HD had higher incidence and mild condition and favorable prognosis. Patients with IH or patients with HD complicated by IH had lower incidence rates and severe condition and poor prognosis, followed by patients with IND or patients with HD complicated by IND.

Objective To evaluate the efficacy and safety of different antimicrobial regimens in patients with bloodstream infections caused by carbapenem-resistant Klebsiella pneumoniae (CRKP).Methods The clinical date of patients with CRKP bloodstream infections were retrospectively analyzed at the First Affiliated Hospital of Zhejiang University Medical College between January 2017 and January 2018.All subjects were separated into three groups based on antibiotics regimens over 72 hours,including meropenem 2.0 g every 8 hours,tigecycline 200 mg as initial dose and 100 mg every 12 hours,and polymyxin B 1.25 mg/kg every 12 hours as salvage treatment of tigecycline.Results A total of 86 patients were finally recruited,including 14,52 and 20 patients in groups of meropenem,tigecycline and polymyxin B salvage,respectively.All of the strains were resistant to meropenem and susceptible to tigecycline and polymyxin B initially,while 2 of them became resistant to tigecycline during treatment.The 28-day mortality was significantly higher in meropenem group (13/14) than that in tigecycline group and polymyxin B salvage group (61.5％,32/52) and (12/20),respectively (P＜0.01),while as no significant difference was seen in the last two groups (x2=0.014,P＞0.05).The incidences of hepatic impairment [3.8％(2/52) vs.1/20] and renal dysfunction (0 vs.1/20) between tigecycline group and polymyxin B salvage group were both comparable (P＞0.05).Conclusion The meropenem-based therapy is not recommended for CRKP-related bloodstream infections.Tigecycline-based therapy is still disappointing despite salvage use of polymyxin B after 72 hours.Hepatic and nephretic toxicities caused by additional polymyxin B are acceptable.