Vol 44 # 4 December 2012 - Kma.org.kw

December 2012
KUWAIT MEDICAL JOURNAL 336
and C, antinuclear antibodies and anti-double strand
antibodies were negative. α -1 antitrypsin and serum
ceruloplasmin were within normal limits. Serum
protein electrophoresis was normal. Further enquiry
into patient’s history, revealed that the patient had
frequent attacks of diarrhea over the previous three
months.
The common association of CD with type 1 diabetes
mellitus attracted our attention to the possibility of
this disorder as a cause of the presenting picture of
this patient. Anti-gliadin antibodies were evaluated
together with anti-endomysial antibodies and tissue
transglutaminase antibodies (tTGA). Anti-gliadin
antibodies were elevated (IgA 62 u/l, normal < 5 u/l)
IgG was 69 u/l (normal < 7 u/l), anti-endomysial
antibodies were positive and tTGA were elevated (IgA
was > 120 u/l, normal < 7 u/l). These findings were
highly suggestive of CD. We proceeded for upper
gastrointestinal endoscopy with duodenal biopsy for
histopathology which showed nearly complete villous
atrophy with intraepithelial lymphocytic infiltration.
The patient was diagnosed to have CD with elevated
liver enzymes as a result. Further investigations showed
decreased level of vitamin D (41.45 nmol/l, normal
50 - 80 nmol/l) and serum iron (3 μmol/l, normal 6
– 7 μmol/l). A dietitian and gastroenterologist were
consulted and gluten free diet (GFD) was started for the
patient with multivitamin supplementation including
vitamin D, K, folic acid, and iron.
Four weeks later, although liver enzymes became
normal, we were informed by the mother that her
daughter was poorly compliant for GFD.
Five weeks after diagnosis, the patient was admitted
to the hospital because of urinary tract infection,
diarrhea and increasing lower limb edema. Clinical
examination was unremarkable except for bilateral
lower limb pitting edema and brownish patchy scaly
skin pigmentation on the extremities. Plasma albumin
was as low as 15g/dl. Random blood sugar was 12.7
mmol/l with no acetone in urine and blood pH was
7.39. CBC showed Hb of 12.1g/dl WBCs 15,000/mm 3
(85% were polymorhs) and platelets were 235,000/mm 3 .
Urine culture and sensitivity were sent. Empirical
antibiotic ciprofloxacin 500 mg BD was started and this
choice was confirmed by the result of urine culture and
sensitivity test. IV albumin 20 g twice daily was started
with intensive insulin regimen.
The patient became distressed after six days of
admission with a temperature of 38.1 ºC and hypoxia.
X-Ray chest showed bilateral bronchopneumonia.
Patient was shifted to the ICU and ventilated as her
respiratory rate had reached 35/min. Antibiotics
were modified to Tazocin 4.5 g/6 hourly and IV
clarithromycin 500 mg BD. Tracheal aspirate was
sent for culture and sensitivity test along with blood
culture and sensitivity. The patient did not respond
well to antibiotics, and deteriorated within two days
and died. Result of tracheal aspirate and blood culture
showed Klebsiella pneumoniae.
DISCUSSION
CD is triggered by ingestion of wheat gluten
and related other cereal proteins, particularly
those in rye and barley. These molecules induce an
inflammatory response in the small intestine, resulting
in villous atrophy, crypt hyperplasia and lymphocytic
infiltration [1] . CD is sometimes divided into clinical
subtypes. The terms classic apply to cases which
meet the classic features of CD, which include chronic
diarrhea, abdominal distension and pain, weakness
and sometimes malabsorption. In contrast in atypical
asymptomatic form, no gastrointestinal manifestations
are present, but features such as anemia, osteoporosis,
short stature, infertility and neurological problems are
common, and a case may be discovered on duodenal
biopsy for any other reason or immunology screening.
Nevertheless, the disease remains widely underrecognized
[2] .
Studies in the United States and Europe show the
prevalence of the disease approaching 1% [3,4] . Based
on small bowel biopsy diagnosis, the prevalence of
CD in type 1 diabetes in children is 1:6 to 1:103 and in
adults is 1:16 to 1:76 [5] . CD is most often present before
the onset of diabetes [6] . The association between the
development of both diseases may be explained by
the inheritance of common major histocompatibility
complex immunogenotypes that influence the
presentation of auto antigens to CD4 + T-Cells [7] .
In adults with CD, hypertransaminasemia is
frequent and normalizes with gluten free diet. If not,
liver biopsy should be done to rule out autoimmune
hepatitis and primary billiary cirrhosis [8] . Isolated
elevation of ALP (alkaline phosphatase) is less
common and may reflect presence of secondary
hyperparathyroidism (bone-specific form).
Hypoalbuminemia and prolonged prothrombin time
may indicate severe form of malabsorption [9] .
The American Gastroenterological Association
(AGA) Institute recommended that testing for CD
should be considered in symptomatic patients who are
at particular risk. These include those with unexplained
iron deficiency anemia, premature osteoporosis, Down
syndrome, unexplained hypertransaminasemia,
autoimmune hepatitis and primary billiary cirrhosis.
The diagnostic tests for CD widely used now
include IgA antiendomysial antibodies and IgA tTGA
(sensitivity - 90% and specificity - 95%), but distal
duodenal biopsy remains the gold standard diagnostic
method (showing total or partial villous atrophy,
crypt lengthening and increased lymphocytes in
lamina propria and intraepithelial region). Biopsy is
indicated even if serology is negative and CD is still