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Celiac crisis with ataxia in a child.

Oba J, Escobar AM, Schvartsman BG, Gherpelli JL - Clinics (Sao Paulo) (2011)

View Article: PubMed Central - PubMed

Affiliation: Hospital Israelita Albert Einstein, São Paulo, São Paulo, Brazil. janeoba@gmail.com

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This clinical case illustrates a rare and life‐threatening presentation of celiac disease (CD), a celiac crisis with neurological symptoms... The diagnosis of celiac crisis should be included in the differential diagnosis of some types of diarrhea with serious metabolic disorders to prevent serious future complications... Serology tests for antigliadin (AGA) and antiendomysium antibodies (EMA) IgA and IgG were negative for CD at ages 2 and 4... Laboratory test results showed hyponatremia (133 mEq/L), hypokalemia (3.1 mEq/L), hyperchloremic metabolic acidosis (pH 7.2; bicarbonate: 7 mmol/L; BE: –19.9)... CD was suspected... The mucosa magnified by endoscope showed microhemorrhage, reduction in duodenal folds and multiple erosions (Figure 1A and B)... Some authors believe that there is a positive correlation between gluten sensitivity and cerebellar disease because of the presence of AGA in patients with neurological symptoms of unknown etiology... Vitamin E and vitamin B12 deficiencies, due to chronic malabsorption, may also cause spine cerebellar degeneration and peripheral neuropathies... The abrupt increase in gluten ingestion and the recovery from ataxia and diarrhea by reducing gluten ingestion may be strongly associated with dosage... Therefore, under normal conditions, the juxtaposed enterocytes would limit the passage of macromolecules (including gluten peptides) and avoid the crisis... This explains the child's recovery after the reduction in gluten ingestion at the hospital, without any specific neurological treatment; metabolic acidosis, however, persisted... Later, a large amount of gluten led to uncontrollable diarrhea, and hypoalbuminemia created conditions for low tolerance to fluid loss, metabolic acidosis and hypokalemia, all characteristics of celiac crisis... The development of serological markers for CD screening has facilitated its early diagnosis in risk populations... The sensitivity of serological EMA and anti‐tTG antibodies is greater than 90%, and these test markers are the best diagnostic options for CD.

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(A) Endoscopic image: Loss of duodenal folds. (B) Mucosa (100× magnification): villous atrophy, microhemorrhage with multiple erosions. (C) Histology: total villous atrophy, Marsh 3C (1).
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f1-cln_66p173: (A) Endoscopic image: Loss of duodenal folds. (B) Mucosa (100× magnification): villous atrophy, microhemorrhage with multiple erosions. (C) Histology: total villous atrophy, Marsh 3C (1).

Mentions: Fecal analysis revealed an increased loss of fats. Serum IgA EMA and IgA anti‐tissue transglutaminase antibody (anti‐tTG) was reagent, over 100 U/mL. CD was suspected. The mucosa magnified by endoscope showed microhemorrhage, reduction in duodenal folds and multiple erosions (Figure 1A and B). Histological examination showed partial and total villous atrophy, intraepithelial lymphocytes and increased length crypts (Marsh 3C) (Figure 1C).


Celiac crisis with ataxia in a child.

Oba J, Escobar AM, Schvartsman BG, Gherpelli JL - Clinics (Sao Paulo) (2011)

(A) Endoscopic image: Loss of duodenal folds. (B) Mucosa (100× magnification): villous atrophy, microhemorrhage with multiple erosions. (C) Histology: total villous atrophy, Marsh 3C (1).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3044576&req=5

f1-cln_66p173: (A) Endoscopic image: Loss of duodenal folds. (B) Mucosa (100× magnification): villous atrophy, microhemorrhage with multiple erosions. (C) Histology: total villous atrophy, Marsh 3C (1).
Mentions: Fecal analysis revealed an increased loss of fats. Serum IgA EMA and IgA anti‐tissue transglutaminase antibody (anti‐tTG) was reagent, over 100 U/mL. CD was suspected. The mucosa magnified by endoscope showed microhemorrhage, reduction in duodenal folds and multiple erosions (Figure 1A and B). Histological examination showed partial and total villous atrophy, intraepithelial lymphocytes and increased length crypts (Marsh 3C) (Figure 1C).

View Article: PubMed Central - PubMed

Affiliation: Hospital Israelita Albert Einstein, São Paulo, São Paulo, Brazil. janeoba@gmail.com

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

This clinical case illustrates a rare and life‐threatening presentation of celiac disease (CD), a celiac crisis with neurological symptoms... The diagnosis of celiac crisis should be included in the differential diagnosis of some types of diarrhea with serious metabolic disorders to prevent serious future complications... Serology tests for antigliadin (AGA) and antiendomysium antibodies (EMA) IgA and IgG were negative for CD at ages 2 and 4... Laboratory test results showed hyponatremia (133 mEq/L), hypokalemia (3.1 mEq/L), hyperchloremic metabolic acidosis (pH 7.2; bicarbonate: 7 mmol/L; BE: –19.9)... CD was suspected... The mucosa magnified by endoscope showed microhemorrhage, reduction in duodenal folds and multiple erosions (Figure 1A and B)... Some authors believe that there is a positive correlation between gluten sensitivity and cerebellar disease because of the presence of AGA in patients with neurological symptoms of unknown etiology... Vitamin E and vitamin B12 deficiencies, due to chronic malabsorption, may also cause spine cerebellar degeneration and peripheral neuropathies... The abrupt increase in gluten ingestion and the recovery from ataxia and diarrhea by reducing gluten ingestion may be strongly associated with dosage... Therefore, under normal conditions, the juxtaposed enterocytes would limit the passage of macromolecules (including gluten peptides) and avoid the crisis... This explains the child's recovery after the reduction in gluten ingestion at the hospital, without any specific neurological treatment; metabolic acidosis, however, persisted... Later, a large amount of gluten led to uncontrollable diarrhea, and hypoalbuminemia created conditions for low tolerance to fluid loss, metabolic acidosis and hypokalemia, all characteristics of celiac crisis... The development of serological markers for CD screening has facilitated its early diagnosis in risk populations... The sensitivity of serological EMA and anti‐tTG antibodies is greater than 90%, and these test markers are the best diagnostic options for CD.

Show MeSH
Related in: MedlinePlus