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Table 1 Clinical manifestations of Chinese CF patients

From: Clinical and genetic characteristics of cystic fibrosis in CHINESE patients: a systemic review of reported cases

CF N = 71  
Age at onset(y)(n = 63) Median age 1y Pulmonary function  
0.1–6.0y* FEV1(n = 38) 67.8% ± 20.9%
Age at diagnosis(y)(n = 69) Median age 10y FVC(n = 20) 80.7% ± 13.8%
4.6–13.3y* FEV1/FVC (n = 19) 71.2% ± 12.3%
Sex(F/M) (n = 69) 37/32 Sweat chloride test (n = 52) Median value 118.0 mmol/L
Family history(n = 71) 17/71   104.0–135.0 mol/L*
Clinical manifestations Sputum or BALF culture
Underweight (n = 25) (n = 59)  
normal 8/25 PA 46/59
-1 SD - 0 2/25 MSSA 13/59
-2 SD - (−1 SD) 6/25 Aspergillus 4/59
−3 SD - (− 2 SD) 3/25 KP 3/59
<-3 SD 6/25 Other 3/59
Respiratory system (n = 71)   MRSA 2/59
bronchiectasis 67/71 E.Coli 1/59
ABPA 15/71 MRSE 1/59
sinusitis 33/71 MSSE 1/59
Digestive system (n = 71) n = 71 SP 1/59
icterus 0 BC 1/59
diarrhea 15/71 HI 1/59
hepatomegaly 1/71 AC 1/59
hypersplenotrophy 1/71 MC 1/59
ileus 3/71 NTM 1/59
hepatolienomegaly 2/71 Misdiagnosis (n = 29)  
positive fecal Sudan III 10/71 bronchiectasis 12/29
hepatocirrhosis 2/71 pneumonia 9/29
Reproductive system (n = 38)   Batter syndrome 5/29
  ABPA 5/29
CBAVD (adult male, n = 1) 1 other 3/29
TB 4/29
upper respiratory tract infection 2/29
  1. *:IQR
  2. ABPA allergic bronchopulmonary aspergillosis, Ac Acinetobacter. BALF bronchial alveolar lavage fluid, BC Burkholderiacepaci, CBAVD congenital bilateral absence of the vas deferens, CF cystic fibrosis, E.coli Escherichia coli, FEV1 forced expiratory volume in the first second, FVC forced vital capacity, HI Haemophilus influenza, KP Klebsiella pneumoniae, MC Moraxella catarrhalis, MRSA methicillin-resistant Staphylococcus aureus, MRSE methicillin-resistant Staphylococcus epidermidis, MSSA methicillin-sensitive Staphylococcus aureus, MSSE methicillin-sensitive Staphylococcus epidermidi, NTM non-tuberculosis Mycobacterium, PA Pseudomonas aeruginosa, PI pancreatic insufficiency, SD standard deviation, SP Streptococcus pneumoniae. TB tuberculous bacillus