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Partially responsive celiac disease resulting from small intestinal bacterial overgrowth and lactose intolerance.

BACKGROUND: Celiac disease is a common cause of chronic diarrhea and malabsorption syndrome all over the world. Though it was considered uncommon in India in past, it is being described frequently recently. Some patients with celiac disease do not improve despite gluten free diet (GFD). A study described 15 cases of celiac disease unresponsive to GFD in whom small intestinal bacterial overgrowth (SIBO) or lactose intolerance was the cause for unresponsiveness. CASE PRESENTATION: During a three-year period, 12 adult patients with celiac disease were seen in the Luminal Gastroenterology Clinic in a tertiary referral center in northern India. Two of these 12 patients (16.6%), who did not fully respond to GFD initially, are presented here. Unresponsiveness resulted from SIBO in one and lactose intolerance in the other. The former patient responded to antibiotics and the latter to lactose withdrawal in addition to standard GFD. CONCLUSION: In patients with celiac disease partially responsive or unresponsive to GFD, SIBO and lactose intolerance should be suspected; appropriate investigations and treatment for these may result in complete recovery.

Adult↗

A reliable screening test for childhood celiac disease: fluorescent immunosorbent test for gliadin antibodies. A prospective multicenter study.

The diagnostic value of gliadin antibody determination using the fluorescent immunosorbent test was examined in a prospective multicenter study comprising 251 children with malabsorptive disorders. Antibodies to gliadin were found in all 72 patients (100%) with active celiac disease (29 children with celiac disease proved by challenge, 43 with probable celiac disease). All children up to the age of 7 years had antibodies in high titers. By contrast, 96 (84%) of 114 children with other malabsorptive disorders and a normal mucosa or with partial villous atrophy had no gliadin antibodies, 14 (12%) had a low titer, and only four (3.5%) showed moderate to high titers. Four children with gastrointestinal tract symptoms of cow milk intolerance and a flat mucosa also showed no antibodies. In 24 of 29 children (83%) with cystic fibrosis and six of seven children with Crohn disease (biopsies not performed in either group), no antibodies could be detected. The others had low or elevated titers. In 25 children with acute gastroenteritis (not biopsied) antibodies were not found at hospital admission nor six weeks later after reintroduction of gluten. The determination of antibodies to gliadin with the fluorescent immunosorbent test is a reliable screening test for childhood celiac disease. In our series there were no false negative results in children with untreated celiac disease. A positive gliadin antibody titer is not proof of celiac disease. In each child the diagnosis must be confirmed by small intestinal biopsy even if the gliadin antibody titer is high. The detection of high titers of cow milk antibodies in 27% of patients with celiac disease is of no value.

Adolescent↗

Celiac disease: how common is jejunoileal fold pattern reversal found at small-bowel follow-through?

OBJECTIVE: Celiac disease, or nontropical sprue, is a cause of mucosal malabsorption. A decreased number of jejunal folds and an increased number of ileal folds (jejunoileal fold pattern reversal) found at small-bowel follow-through have been reported for patients with celiac disease. We asked three questions regarding jejunoileal fold pattern reversal found at small-bowel follow-through in patients with celiac disease. (1) How often is it present, either partially or completely? (2) How often is it associated with other findings of malabsorption? (3) How reliably can it be distinguished from the normal pattern? MATERIALS AND METHODS: Twenty-eight small-bowel follow-through examinations performed on 25 adult patients with celiac disease (confirmed by characteristic small-bowel biopsy and clinical response to a gluten-free diet) were reviewed retrospectively by two authors, who agreed by consensus on partial or complete jejunoileal fold pattern reversal and on other findings of malabsorption. Two methods were used to control for retrospective bias. (1) The prospective and retrospective readings of fold pattern reversal were compared for agreement. (2) The author who had not participated in the retrospective review was asked to distinguish, on the basis of the presence or absence of fold pattern reversal, 24 cases of celiac disease (all of which showed partial or complete fold pattern reversal on retrospective review) from 25 normal control cases (patients with diarrhea) (conformed by normal small-bowel biopsy). RESULTS: Partial or complete jejunoileal fold pattern reversal was identified retrospectively in 24 of the 28 small-bowel examinations (86%) performed on patients with celiac disease. One-half lacked other findings of malabsorption. The prospective and retrospective readings of fold pattern reversal agreed in 21 of the 28 examinations (75%). Forty-four of 49 examinations (90%) were correctly identified by the third author on the basis of fold pattern reversal. CONCLUSION: In patients with celiac disease, partial or complete jejunoileal fold pattern reversal discovered at small-bowel follow-through is common, is often not associated with other findings of malabsorption, and can be reliably distinguished from the normal pattern. Identification of jejunoileal fold pattern reversal found at small-bowel follow-through should prompt an appropriate clinical evaluation for celiac disease.

Adult↗

Prevalence of celiac disease in Tunisian blood donors.

OBJECTIVES: Prevalence of adult celiac disease is unknown in Tunisia. Symptomatic forms are less frequent than silent forms, which, according to recent serological screening in Europe and the United States, have an estimated prevalence of 1/100 to 1/500. We aimed to determine the prevalence of celiac disease in healthy blood donors in Tunisia. METHODS: Between November 2002 and March 2004, 1 418 sera from blood donors were tested for IgA anti-endomysium antibodies (EMA) by indirect immuno-fluorescence on monkey esophagus cryosections. RESULTS: The sample population included 1090 men and 328 women: mean age 29 and 26 years respectively. Three sera from two men and one woman were positive for IgA EMA. ELISA search for anti-tissue tranglutaminase antibodies (ATG) in these three sera was positive in two. Upper gastrointestinal endoscopy with proximal intestinal biopsies was performed in the three patients. Subtotal or total villous atrophy was observed in the two ATG-positive patients, confirming the diagnosis of celiac disease. In the third patient, histologic examination did not show any abnormality. CONCLUSION: Adult celiac disease is considered relatively rare in Tunisia. In fact, our study revealed a prevalence of about 1/700 among blood donors.

Adolescent↗

Celiac disease in children with Down syndrome: importance of follow-up and serologic screening.

BACKGROUND: Celiac disease, also known as gluten-sensitive enteropathy, is a chronic inflammation disease of the small intestinal mucosa. Detection of Ig-A antigliadin antibodies (AGA) and antiendomysial antibodies (EMA) in serum is important in the diagnosis and screening for celiac disease. Antiendomysial antibodies have greater sensitivity compared to antigliadin antibodies. It has been reported that the prevalence of celiac disease is higher in children with Down syndrome than the other autoimmune conditions. The aim of the present study was to investigate the incidence of celiac disease in children with Down syndrome, to assess the availability of Ig-A AGA and EMA for serologic screening, and to highlight the importance of follow-up for children with Down syndrome. METHODS: Forty-seven children with Down syndrome without known celiac disease were tested for total blood count, thyroid function tests, immunoglobulin values, Ig-A AGA and EMA. Duodenal biopsy was performed on eight patients who showed at least one serologically positive marker. RESULTS: The ages of the children with Down syndrome ranged from 2 to 18 years (30 boys/17 girls). The mean age was 6.55 +/- 3.88. Total blood count and immunoglobulin values were normal. Eleven of the 47 patients (23.40%) were found to be serologically positive, 10 (21.28%) having antigliadin antibody concentrations above normal; and six (12.77%) being positive for antiendomysial antibody. In five patients (10.64%), both Ig-A AGA and EMA concentrations were high and positive. Duodenal biopsies of three of eight cases (37.50%) revealed villous atrophy, lymphocyte infiltration and crypt hyperplasia. Three cases with abnormal biopsy results (100%) were below the 10th percentile for weight and height. Hypo-thyroidism was detected in one of 11 cases where at least one serologic marker was positive. CONCLUSION: Children with Down syndrome should be carefully examined in their follow up, and celiac disease should be considered in cases with growth retardation. Ig-A antigliadin antibodies and EMA are non-invasive, cheap and readily available serologic screening tests for celiac disease, and the positivity of both markers gives the most reliable result.

Adolescent↗

Gliadin challenge-induced jejunal prostaglandin E2 secretion in celiac disease.

Jejunal prostaglandin E2 secretion in patients with adult celiac disease was studied under basal conditions and after local gliadin challenge. A newly developed tube system with occluding balloons was used to perfuse a jejunal segment. After an initial basal perfusion period of 40 minutes, an average of 12 mg +/- 3 (SEM) of crude gliadin was administered into the perfused segment. Perfusion continued for an additional 100 minutes and prostaglandin E2 was determined in the effluent with a radiometric method. Patients with histologically active celiac disease (n = 7) had higher basal jejunal secretion of prostaglandin E2, 523 +/- 229 pg.cm-1.h-1 (mean +/- SEM), than both celiac patients with normalized small bowel mucosa (n = 6), 160 +/- 44 pg.cm-1.h-1, and healthy controls (n = 18), 184 +/- 38 pg.cm-1.h-1, (P less than 0.05). After gliadin challenge of the jejunal segment, a significant increase (P less than 0.05) in prostaglandin E2 secretion was noted in patients with celiac disease (n = 10). The individual maximum increase was on average fivefold and occurred 60-100 minutes after gliadin administration. In healthy controls (n = 5), no alteration in prostaglandin E2 secretion was detected after gliadin challenge. Enhanced jejunal prostaglandin 2 synthesis may be involved in the pathophysiological processes initiated by gliadin in patients with celiac disease.

Adult↗

Epilepsy, occipital calcifications, and oligosymptomatic celiac disease in childhood.

The association of epilepsy, occipital calcifications, and celiac disease has been recognized as a distinct syndrome. The objective of this study was to present the clinical, electrophysiologic, and neuroradiologic features in a series of patients with this syndrome. Thirty-two patients with the constellation of epilepsy, occipital calcifications, and celiac disease were identified in our epilepsy clinic. The mean age was 11 years and the mean length of follow-up was 7.4 years. The 1990 criteria of the European Society of Pediatric Gastroenterology and Nutrition were used to diagnose celiac disease. The Kruskal-Wallis statistics test was employed with a signficance of P < .05. Thirty-one patients had partial seizures, 21 of them with symptoms related to the occipital lobe. In most patients, the epilepsy was controlled or the seizures were sporadic. Three developed severe epilepsy. Occipital calcifications were present in all cases. Computed tomography in 7 patients showed hypodense areas in the white matter around calcifications, which decreased or disappeared after a period of gluten-free diet in 3 patients. A favorable outcome of epilepsy was detected in patients with the earliest dietary therapy. This study presents the largest series of children with this syndrome outside Italy. White-matter hypodensities surrounding calcifications are rarely reported. A prompt diagnosis of celiac disease might improve the evolution of the epilepsy and may improve cognitive status.

Adolescent↗

Clinical benefit of a gluten-free diet in type 1 diabetic children with screening-detected celiac disease: a population-based screening study with 2 years' follow-up.

OBJECTIVE: This study was performed to 1) determine the prevalence of celiac disease in Danish children with type 1 diabetes and 2) estimate the clinical effects of a gluten-free diet (GFD) in patients with diabetes and celiac disease. RESEARCH DESIGN AND METHODS: In a region comprising 24% of the Danish population, all patients <16 years old with type 1 diabetes were identified and 269 (89%) were included in the study. The diagnosis of celiac disease was suspected in patients with endomysium and tissue transglutaminase antibodies in serum and confirmed by intestinal biopsy. Patients with celiac disease were followed for 2 years while consuming a GFD. RESULTS: In 28 of 33 patients with celiac antibodies, an intestinal biopsy showed villous atrophy. In 5 patients, celiac disease had been diagnosed previously, giving an overall prevalence of 12.3% (95% CI 8.6-16.9). Patients with celiac disease had a lower SD score (SDS) for height (P < 0.001) and weight (P = 0.002) than patients without celiac disease and were significantly younger at diabetes onset (P = 0.041). A GFD was obtained in 31 of 33 patients. After 2 years of follow-up, there was an increase in weight SDS (P = 0.006) and in children <14 years old an increase in height SDS (P = 0.036). An increase in hemoglobin (P = 0.002) and serum ferritin (P = 0.020) was found, whereas HbA(1c) remained unchanged (P = 0.311) during follow-up. CONCLUSIONS: This population-based study showed the highest reported prevalence of celiac disease in type 1 diabetes in Europe. Patients with celiac disease showed clinical improvements with a GFD. We recommend screening for celiac disease in all children with type 1 diabetes.

Adolescent↗

Effect of gluten-free diet on bone mineral content in growing patients with celiac disease.

Osteoporosis is a complication of celiac disease in adulthood, but little is known about the influence of the disease on bone mineralization in children. In the present study we evaluated radial bone mineral content (BMC) in celiac children and adolescents at diagnosis and after they consumed a gluten-free diet (GFD). The BMC values of 33 celiac patients at diagnosis were significantly lower than those of 255 control subjects (P < 0.001). There was no difference between diabetic and non-diabetic celiac patients. In 14 patients the BMC increased significantly (P < 0.05, ANCOVA) after 1.28 y of GFD. In these patients the mean annual BMC increment was 0.07 g/cm, significantly greater (P < 0.05) than the increment of normal growing children (0.05 g.cm-1.y-1). Our data indicate that although osteoporosis complicates celiac disease during childhood and adolescence, GFD alone is able to remarkably improve bone mineralization.

Bone Density↗

Relation between cigarette smoking and celiac disease: evidence from a case-control study.

OBJECTIVES: It has been suggested that environmental factors other than gliadin might play a role in pathogenesis of celiac disease. Cigarette smoking was reported to exert a protective effect against the development of symptomatic celiac disease; however, this relationship was not confirmed. The aim of this study was to determine the effect of cigarette smoking on celiac disease. METHODS: A cohort of 87 consecutive celiac disease patients attending the clinic of Malabsorption and 174 age- and sex-matched individuals diagnosed with functional GI disorder were included in the study. Clinical information was obtained both at the time of diagnosis and at follow-up by reviewing the clinical history. Smoking information was obtained through an in-person interview using a questionnaire. RESULTS: Although 33% of controls were current smokers at the time of the study, only 16% of celiac patients were smokers at diagnosis (odds ratio, 0.39; 95% confidence interval 0.19-0.79; p < 0.006). The proportion of nonsmokers among patients (84%) was significantly greater than that among controls (67%; odds ratio, 2.54; 95% confidence interval 1.27-5.16; p < 0.007). Current smoker patients had a lower baseline BMI (p < 0.05) and body weight (p < 0.05) compared to former smokers. Compared with nonsmokers, control individuals who were active smokers at entry in the study were younger (p < 0.02) and had lower body weight (p < 0.03) and BMI (p < 0.03). Interestingly, positive lineal correlation was observed between age at diagnosis and daily cigarette consumption (r = 0.72; p < 0.004) in active smokers. We did not detect any relationship either between causes for cessation of smoking and clinical symptoms or between differences in the proportions of smoking habits when patients were stratified according to their clinical status at diagnosis (symptomatic vs subclinical/asymptomatic cases). CONCLUSIONS: This study provides evidence that, compared with control subjects, a significantly lower proportion of patients with celiac disease were current smokers at the time of diagnosis, and that cigarette smoking delayed diagnosis of celiac disease. Our study suggests that the nutritional compromise of patients with celiac disease who smoked resulted from the summation of the effect of celiac disease per se and that produced by the smoking habit. Further studies are necessary to identify whether the relationship between smoking and celiac disease is causal or incidental.

Adult↗

Detection of autoantibodies against tissue transglutaminase in patients with celiac disease and dermatitis herpetiformis.

OBJECTIVE: Endomysial autoantibodies (EmA) are specific for celiac disease. The target antigen has been identified as tissue tranglutaminase (tTG). Our aim was to study the accuracy of a newly developed enzyme-linked immunosorbent assay (ELISA) for easy detection of tTG autoantibodies. METHODS: Thirty-one sera from patients with histologically proven celiac disease and 23 healthy controls were examined for EmA using monkey esophagus and human umbilical cord as substrate. IgA-tTG autoantibodies were determined by newly developed ELISA. Additionally, sera from patients with dermatitis herpetiformis (n = 20), inflammatory bowel disease (IBD; n = 32), chronic liver disease (n = 36), and diabetes mellitus (n = 19) were tested. RESULTS: The sensitivity of the tTG autoantibody ELISA accounted for 90% detection in patients with untreated celiac disease. The specificity was 76% owing to positive values in the lower range in patients with IBD (15%), chronic liver disease (36%), and diabetes (22%), all of whom were negative for EmA. In dermatitis herpetiformis patients 90% were EmA-positive. Of these, only 47% showed elevated tTG autoantibodies. Preincubation of sera from dermatitis patients with tTG abolished immunofluorescent staining of endomysial structures. CONCLUSION: Detection of mid- to high-titer tTG autoantibodies is highly specific for celiac disease. However, in the low-titer range, overlap exists with liver disease, IBD, and diabetes. Tissue transglutaminase autoantibodies may evolve as a new screening and follow-up method for celiac disease. Although tTG seems to be a major autoantigen in dermatitis herpetiformis, the low sensitivity of both tTG ELISA and immunofluorescence using human umbilical cord suggests differential involvement of tTG in this disease.

Animals↗

Endoscopic features of celiac disease in adults and their correlation with age, histological damage, and clinical form of the disease.

BACKGROUND AND STUDY AIMS: Although earlier studies have focused on endoscopic markers as predictors of celiac disease, there are still no certainties about the value of these markers. The aim of this study was to consider first, specific endoscopic features as predictors of specific histological damage; secondly, whether there is an association between the endoscopic features of celiac disease and the age of patients at the time of diagnosis; and thirdly, whether particular endoscopic features of celiac disease are associated with the clinical form of the disease. PATIENTS AND METHODS: We studied the endoscopic features of celiac disease in 144 consecutive adult patients (52 had the classical form of the disease, 64 the subclinical and 28 the silent form). The histopathological findings were expressed according to the Marsh classification. RESULTS: Slight/mild damage seen at endoscopy was associated with a Marsh II-IIIa grading (P < 0.005), while severe endoscopic damage was related to a Marsh IIIb-IIIc grading (P < 0.0005). Younger patients showed slighter damage at endoscopy (P < 0.001), while older patients showed more severe damage (P < 0.005). Finally, the classical form of celiac disease showed more severe damage at endoscopy, while the subclinical/silent forms of celiac disease showed slighter damage endoscopically (P < 0.001). CONCLUSIONS: This study showed that the endoscopic appearance of the duodenum may be predictive of histological damage grading. Moreover, we showed that in young patients with subclinical/silent celiac disease there is a greater probability of finding slight/mild endoscopic abnormalities associated with slight/mild histological damage.

Adolescent↗

Mortality and causes of death in celiac disease in a Mediterranean area.

No data on mortality in celiac disease are currently available in southern Europe. Our aim was to evaluate mortality and the cause of death in adult celiac disease in a Mediterranean area. In all, 228 adults with celiac disease were histologically diagnosed in our department from 1980 to 1997. Full information on their state of health was obtained in 216 of 228 patients. A tabulation of patient-years at risk was constructed in terms of age at diagnosis and the interval from diagnosis. Standardized mortality ratio was calculated by dividing the number of observed deaths by the number of expected deaths. Twelve deaths were observed, whereas 3.12 deaths were expected (SMR = 3.8; 95% CI 2-7). The increased mortality was mainly observed within four years from diagnosis (8 observed; 1.4 expected) (SMR = 5.8; 95% CI 2.5-11.5). Twelve tumors were observed (six lymphomas). In conclusion, mortality from adult celiac disease in our geographical area is increased compared with the general population, and this increased risk seems due to non-Hodgkin's lymphoma.

Adult↗

Endomysial antibodies in the diagnosis of celiac disease and the effect of gluten on antibody titers.

Celiac disease, a common chronic gastrointestinal disorder, is gluten induced and is controlled with a gluten-free diet. While the management of CD with a gluten-free diet is quite effective, the diagnosis is rather difficult. The ESPGAN criteria for the diagnosis of CD seems to be tedious and time-consuming. Serological tests for IgA class endomysial antibodies, as detected by indirect immunofluorescence, on human and primate smooth muscles are specific and sensitive markers of celiac disease. Of all the specimens examined, endomysial antibodies were present in patients with gluten-sensitive enteropathy. These antibodies occurred in all active cases of celiac disease, in 90 percent suspected celiac patients where all the ESPGAN criteria has not been fulfilled. This contrasts to the presence of endomysial antibodies in 46 percent of confirmed and 17 percent of suspected celiac patients maintained on a gluten-free diet for various time intervals. Endomysial antibodies also occurred in all cases with chronic diarrhea and gut histology consistent with CD and 8% of asymptomatic family members of CD patients. None of the patients with other gastrointestinal and liver diseases had endomysial antibodies. These studies thus emphasize the specificity and sensitivity of endomysial antibodies for celiac disease.

Animals↗

Use of HLA typing in diagnosing celiac disease in patients with type 1 diabetes.

OBJECTIVE: This study examines the use of HLA typing for the diagnosis of celiac disease in a group of Australians with type 1 diabetes. RESEARCH DESIGN AND METHODS: Subjects included 131 sequential patients with type 1 diabetes (mean age 17 years [range 10-37]), 77 patients with biopsy-proven celiac disease (mean age 52 years [range 12-84]), and 162 healthy control subjects (mean age 17 years [range 2 months to 56 years]). Subjects were prospectively screened for celiac disease using endomysial antibodies (EMAs), tissue transglutaminase antibodies (TTGAs), and celiac disease-specific HLA typing. RESULTS: Celiac disease was diagnosed in 11 subjects after an intestinal biopsy (prevalence 8.4%). There was 95% agreement between TTGA and EMA for positive results and 100% for negative results. There was no significant difference for HLA DQ2 and DR4 among patients with type 1 diabetes with or without celiac disease. CONCLUSIONS: The prevalence of celiac disease among patients with type 1 diabetes is higher than previously estimated in Australia. TTGA is a valuable diagnostic tool that can be used for screening celiac disease in patients with type 1 diabetes. HLA typing should not be used in the diagnosis of celiac disease in patients with type 1 diabetes because of the similarities of HLA types between patients with type 1 diabetes and those with celiac disease.

Adolescent↗

Primary sclerosing cholangitis and celiac disease. A novel association.

The association of primary sclerosing cholangitis and celiac disease was observed in three patients, an association not previously reported. All three patients were men who presented with chronic cholestatic liver disease at ages 32, 46, and 62 years, respectively. In each patient, endoscopic retrograde cholangiography showed the typical findings of primary sclerosing cholangitis. Histologic features of liver biopsy were compatible with the diagnosis. Two patients had associated chronic ulcerative colitis. All three patients complained of frequent loose stools and weight loss; subsequent testing showed severe steatorrhea (204 to 323 mmol/d of fecal fat on a 100 g fat diet). Total villous atrophy was found in all three patients on histologic examination of the small bowel. Celiac disease was diagnosed at the time of presentation in two patients who had primary sclerosing cholangitis and was diagnosed three years after the onset of primary sclerosing cholangitis in the third patient. The celiac disease responded to a gluten-free diet in each patient whereas the primary sclerosing cholangitis was not affected by dietary treatment. The possibility of a chance association of primary sclerosing cholangitis and celiac disease cannot be accurately assessed but seems unlikely given the rarity of both diseases. The relationship between the two diseases remains unknown, although an immunologic connection is suspected. Celiac disease should be considered in the differential diagnosis of severe steatorrhea in patients with primary sclerosing cholangitis.

Adult↗

Comparison of IgA endomysium antibody and IgA tissue transglutaminase antibody in celiac disease.

The antigen for immunoglobulin (Ig) A endomysium antibody (EmA), a sensitive and specific serological marker for celiac disease, has recently been described as tissue transglutaminase (tTG). The aim of this study was to compare the assays used to measure IgA EmA and IgA tTG antibody in patients with celiac disease and disease control subjects. Sera from 21 patients with untreated celiac disease, 48 patients with treated celiac disease and 128 disease control subjects were tested both for IgA EmA with the use of indirect immunofluorescence against human umbilical cord and for IgA tTG antibody with the use of ELISA. Titres of IgA tTG antibody were significantly higher in both the untreated and treated celiac groups than in the disease control group. Titres in the treated group were, however, significantly lower than in the untreated group. A reference range was calculated to include 99.8% of the disease control group in whom small bowel biopsy showed no evidence of celiac disease. One patient from the disease control group with raised IgA tTG antibody titres and positive IgA EmA was found to have celiac disease on small bowel biopsy. The sensitivity, specificity, and positive and negative predictive values of the IgA EmA assay were all 100%. The sensitivity of the IgA tTG antibody assay was 95%, specificity 100%, positive predictive value 100% and negative predictive value 97.7%. An ELISA used to measure IgA tTG antibody is an excellent tool to screen for celiac disease and may prove useful for monitoring response to treatment.

Case-Control Studies↗

Intestinal malignant lymphoma in a child with familial celiac disease.

A case of intestinal malignant lymphoma occurring in a 10-year-old North African girl with celiac disease is reported. Celiac disease was diagnosed at age 4. A gluten-free diet was started and was maintained for the next 6 years. Seven months after the patient returned to a normal diet, clinical symptoms and subtotal villous atrophy relapsed. Within the next month an abdominal malignant lymphoma was diagnosed. After chemotherapy the tumor disappeared but recurred 6 months later, involving the colon and the liver. Death occurred shortly thereafter. A brother, two years older, also with proven celiac disease, is presently on a strict gluten-free diet. Both children shared the same histocompatibility antigens (HLA A2-B17/A29-B12, identity at the D locus). Whether or not relapse of celiac disease played a role in the appearance of the tumor remains highly speculative; however, the case of our patient suggests that familial involvement and, in some instances, ethnic origin may constitute risk factors predisposing to malignancy.

Celiac Disease↗