eliac disease is characterized by small bowel
villous atrophy and malabsorption. Associated vitamin K deficiency leads
to prolonged prothrombin time [1]. We conducted this study to analyze
the coagulation profile of children with celiac disease and assess its
association with Marsh grading on histology.
Children aged 6 month to 18 years with suspected
celiac disease were included based on symptoms and serology. Children
with prior administration of vitamin K or those already on gluten free
diet (GFD) were excluded. Complete blood count, prothrombin time and
activated partial thromboplastin time (aPTT) were tested prior to
endoscopy. International normalized ratio (INR) <1.4 was considered as
normal [1]. We categorized deranged INR as mild (1.40-2.50), moderate
(2.51-5.0) and severe (>5.0) [2]. Abnormal aPTT was defined as the
difference between test and control of more than 8 seconds. Normal
platelet counts were 1.50-4.50 lakhs/cu.mm. Children with Marsh
histology grade II/ III were started on GFD and favorable responses
confirmed on follow-up to establish definitive diagnosis of Celiac
disease. Children with moderate and severely deranged prothrombin time
were given one dose of parenteral Vitamin K before procedure.
Out of 152 children, 111 (65 boys, 46 girls) were
confirmed to have celiac disease. The mean age at presentation was 5.5
years, the youngest being 7 months and oldest 15 years. Thirty (27%)
children had deranged INR; none had any significant bleeding. The
proportion of deranged INR was found to be increasing with the severity
of histological abnormalities (Table I). Eighty-two
(73.9%) children had normal platelet counts while thrombocytosis was
seen in 26.1% children; 19% children had prolonged aPTT.
TABLE I INR in Different Marsh Grades of Celiac Disease
INR |
Marsh |
Marsh |
Marsh |
Marsh |
Total |
|
grade 2 |
grade 3a |
grade 3b |
grade 3c |
(n=11) |
|
(n=9) |
(n=16) |
(n=29) |
(n=57) |
|
<1.40 |
8 (88.9) |
13 (81.3) |
23 (79.3) |
37 (64.9) |
81 (73.0) |
³1.40 |
1 (11.1) |
3 (18.8) |
6 (20.7) |
20 (35.1) |
30 (27.0)
|
INR: International normalized ratio; Values in No.(%).
|
Mitterstieler, et al. [3] reported four
children with celiac disease who had hemorrhagic diathesis due to a low
"prothrombin complex". After the administration of vitamin K1, there was
an immediate rise in the prothrombin complex and bleeding was quickly
stopped.
Most published guidelines on celiac disease recommend
confirmation of the diagnosis of celiac disease by documenting villous
atrophy on small intestinal biopsy and response to gluten-free diet [5].
In the presence of coagulopathy, thrombocytopenia or portal
hypertension, diagnostic endoscopy and mucosal biopsy can cause
significant bleeding [6]. Our study has a limitation that we could not
demonstrate clinical significance of coagulopathy as none of our patient
developed overt bleeding. This may be due to small sample size, and the
vitamin K received before procedure may have partly corrected the
prothrombin time.
We conclude that almost one-fourth of children with
celiac disease have deranged INR before starting treatment, but they do
not seem to develop bleeding following upper GI endoscopy.
Contributors: SSS: involved in data collection,
manuscript writing, LB: involved in data analysis and manuscript
writing. DS: conceptualized and designed the study, supervised data
collection, and critically reviewed and revised the manuscript. All
authors approved the final manuscript.
Funding: None; Competing interest: None
stated.
References
1. Cavallaro R, Iovino P, Castiglione F, Palumbo A,
Marino M, Di Bella S, et al. Prevalence and clinical associations of
prolonged prothrombin time in adult untreated coeliac disease. Eur J
Gastroenterol Hepatol. 2004;16:219-23.
2. Ertekin V, Selimoglu MA. Prevalence of prolonged
prothrombin time in children with coeliac disease. Eur J Gastroenterol
Hepatol. 2006;18:579-80; (author reply 580).
3. Mitterstieler G, Zieglauer H. Vitamin K deficiency
bleeding as a leading symptom in celiac disease (author’s transl).
Padiatr Padol. 1978;13:175-82.
4. Francavilla R, Cristofori F, Stella M, Borrelli G,
Naspi G, Castellaneta S. Treatment of celiac disease: from gluten-free
diet to novel therapies. Minerva Pediatr. 2014;66:501-16.6.
5. Complications of upper Gastrointestinal Endoscopy. Available from:
http://www.bsg.org.uk/pdf_word_docs/complications.pdf. Accessed
December 10, 2016.