Cystic fibrosis is a risk factor for celiac disease.

BACKGROUND: The coexistence of cystic fibrosis (CF) and celiac disease (CD) has been reported. To our knowledge there is no study directly comparing the incidence of CD in CF patients to that in the general population at the same time. There is no published data on genetic predisposition to CD in CF patients either. Therefore, in the present study we aimed to assess the genetic predisposition to CD and its incidence in CF patients comparing it to data from the general population. PATIENTS AND METHODS: Two hundred eighty-two CF patients were enrolled in the study. In 230 CF patients the genetic predisposition to CD (the presence of HLA-DQ2/ DQ8) was assessed. In all CF patients, serological screening for CD was conducted. In patients with positive antiendomysial antibodies (EMA) gastroduenoscopy was offered. Intestinal histology was classified according to modified Marsh criteria. The results of serological CD screening in 3235 Polish schoolchildren and HLA-DQ typing in 200 healthy subjects (HS) were used for comparison. RESULTS: Positive EMA was found in 2.84% of the studied CF patients. The incidence of proven CD was 2.13%. The incidence of CD as well as positive serological screening were significantly more frequent in the CF group than in the general population. The frequency of CD-related HLA-DQ alleles in CF and HS did not differ. CONCLUSIONS: Genetic predisposition to celiac disease in cystic fibrosis patients is similar to that of the general population. However, our results suggest that cystic fibrosis is a risk factor for celiac disease development.

Acid steatocrit determination is not helpful in cystic fibrosis patients without or with mild steatorrhea.

BACKGROUND: Most methods used for the assessment of severe steatorrhea in cystic fibrosis (CF) are sensitive. In fact, the tests show their usefulness in a borderline zone of the results. Yet, the existing data related to acid steatocrit (AS) are still contradictory. Therefore, in the present study we have aimed to assess CF patients without or with mild steatorhea (<10 g/day) and evaluate the applicability of AS in such a subset of patients. PATIENTS AND METHODS: In fifty-five CF patients, AS, fecal fat concentration (FFC) and fecal fat excretion (FFE) in 1-day stool collection were assessed from one to three times (149 samples). In 50 subjects, FFC, FFE, and AS were available for 3 subsequent days. It allowed for the calculations based upon 3-day fecal fat balance study. RESULTS: The correlations between FFE/FFC and AS based upon 1-day stool collection, although statistically significant, were rather weak (r = 0.208, P < 0.011; r = 0.362, P < 0.000006, respectively). The correlations between FFE/FFC and AS based upon the 3-day stool collection, although stronger, did not show values a linear relationship (r = 0.394, P < 0.005; r = 0.454, P < 0.001, respectively). With no regard to the cut-off level for AS (10% and 20%), sensitivity, specificity, negative, and positive predictive values in the determination of abnormal FFC and FFE were not satisfactory. The flow charts describing the accuracy of AS to determine FFE and FC revealed a high level of uncertainty. CONCLUSIONS: AS does not reflect in a reliable way FFE in CF patients without or mild steatorrhea. Its applicability in the assessment of FFC in such patients has therefore limited practical value.