Spectrum of Celiac disease in Paediatric population: Experience of Tertiary Care Center from Pakistan

Abstract

Objective:To determine clinical features and relevant laboratory investigations of patient with celiac disease (CD) and comparing classical celiac disease (CCD) with Non-diarrheal celiac disease (NDCD).Methods:This is a five years retrospective study conducted at The Aga Khan University Hospital Karachi, Pakistan from January 2010 to December 2015, enrolling children from one year to 15 years of either gender diagnosed as celiac disease in accordance with revised ESPGHAN criteria. Biopsy samples with grade 2 or more on Modified Marsh Classification were considered as consistent with celiac disease. Celiac patients were categorized into Classical celiac disease (with Chronic Diarrhea) and non-diarrheal celiac disease (Atypical celiac) and their clinical features and relevant laboratory investigations were documented.Results:Total 66 patients were selected with celiac disease according to inclusion criteria, 39 (59.09%) patients were labeled as CCD and 27 (40.91%) patients were labeled as NDCD. Marsh grading 3a and above were more marked in CCD as compared to NDCD. Mean titer for Tissue transglutaminase antibodies (TTG) were higher in CCD group in comparison to NDCD group. In CCD, the most common clinical presentations were abdominal distension whereas in NDCD, the most remarkable features were recurrent abdominal pain (62.9%). Frequency of failure to thrive is significantly high in CCD (82.05%) but patients merely with short stature were more common in NDCD (33.3%). Refractory anemia was present in 66.6% patients in NDCD group and 41.1% patients in CCD group. 74.3% patients in CCD group were vitamin D deficient whereas 85% patient had vitamin D deficiency in NDCD group (p= 0.03).Conclusion:NDCD is not uncommon in our population. Recurrent abdominal pain, failure to thrive or patients only with short stature and refractory anemia are prominent features in NCDC group whereas abdominal distension, failure to thrive and recurrent abdominal pain were noticeable features in CCD. High grade histopathology and raised antibodies titer is hallmark of CCD. Vitamin D deficiency is almost equally present in both groups.

Spectrum of Clinical Presentation of Celiac Disease in Pediatric Population

“…Marsh 3b and advance stage were more commonly present in classic celiac disease as compare to non-classic variety, in which early grade was more common. This is in accordance with multiple studies that show a high grade of histopathological findings in patients with gastrointestinal symptoms [ 13 ]. Although villous atrophy is the hallmark of CD.…”

“…In our study, 30% of NCCD presented with short stature only. This is quite similar to the study performed by Aziz et al [ 13 ]. Therefore many studies recommend screening for CD in severely short stature.…”

“…This may in part be related to under diagnosis of non-celiac disease in our community that may be related to high threshold of physicians for investigating patients with non-classic presentations of CD. The mean age at diagnosis of CCD is 5.6 years which is similar to the study Aziz et al [ 13 ]. The early presentation of CCD and late presentation of NCCD is documented in many studies.…”

“…Therefore many studies recommend screening for CD in severely short stature. Anemia is more commonly reported in CD throughout the globe and it has been observed that low hemoglobin may not improve even after one year of gluten-free diet [ 13 ]. In our study 84% of patients had anemia.…”

Frequency of autoimmune thyroiditis in children with Celiac disease and effect of gluten free diet

“…In a study by Aziz DA et al on 66 children with classic and non-classic CD found that only 2 (3.03%) had autoimmune thyroiditis. 12 However, the studies conducted by Meloni A et al and Kalyoncu D et al found higher percentage of AT (10.5%) among 324 patients with CD and 16.4% (11/67) of children with CD, respectively. 13 , 14 The high risk of CD patients to develop AT is explained by the fact that the Celiac disorder carries one or more MHC or other immune related genes supporting AT.…”