4. Introduction
G6PD deficiency, an X-linked recessive
disorder, is the most common enzymatic
disorder of red blood cells in humans,
There are around 400 biochemically
characterized variants
G6PD-Mediterranean - is a variant resulting
in
acute hemolytic anemia under oxidative
stress. It is also the variant producing favism.
5. Objective
To evaluate and outline some
characteristics of cases of
acute hemolytic anemia
(AHA) in children , living in
ZARKA district – JORDAN
and their relation to G6PD
deficiency .
6. 1- By direct interview with the
parents
2- from patient’s medical file.
7.
8. Setting and
participants
All children admitted to pediatric
department (Zarka government
hospital) with acute hemolytic anemia
in a period of two years (between first
January 2014 and 31 December 2015).
9. clinical criteria for acute
hemolytic anemia were
sudden onset of pallor, jaundice
and red urine, associated with Hb
and PCV values less than two
standard deviations below the
mean values for age.
11. Statistical analysis
Descriptive in addition to some
inferential statistics such as t-test and
odds ratio calculations were used to
analyze the results.
12. RESULTS
49 patients with acute hemolytic anemia were
admitted during the two years period of the study
36 males and 13 females
( M : F ratio = 2.8 : 1 )
14. RESULTS (Continue)
There was no significant
difference in the age of males and
females
(mean age of males was 3.8 and
females 4.6 years .
p-value = 0.4
15. RESULTS (Continue)
Mean Hb level on admission in both
males and females did not differ
significantly
(mean Hb 6.12 , 6.85 gm/dl) respectively
p-value = 0.29
18. RESULTS (Continue)
Only 13 patients (27% ) had positive family
history of AHA.
But positve family history was more prominent
in girls than boys
( odds ratio 3.55 95% CI (0.9-13.9 )
25. Number of cases correlates
strongly with the presence of
fresh fava beans in the market
26. Conclusion 1
ALL cases of AHA were
provoked by
fava beans ingestion
( fresh 86% , dried 14% )
27. Conclusion 2
The strong correlation seen in this study
between cases of AHA and bulk
presence of fresh fava beans in the
market confirms this conclusion .
28. Conclusion 3
No statistically significant difference In
clinical severity between males and
females (Hb 6.12 vs. 6.85 gm /dl
( P value 0.29 )
29. Conclusion 4
The number of males with low
G6PD level is significantly more
than number of females odds
ratio10.6 (CI 95% 1.7- 65 ) .
30. Conclusion 5
G6PD levels on admission do not
correlate with the clinical
severity of AHA. This may be due to
the fact that G6PD levels in patient with
favism are mainly reduced in old RBC's
which already underwent hemolysis .
32. FINAL CONCLUSIONS
• ALL cases of AHA were provoked by
fava beans ingestion
Positive history of fava bean ingestion&strong
correlation seen in this study between cases
of AHA and bulk presence of fresh fava
beans in the market
• No statistically significant difference In
clinical severity between males and females
• G6PD levels on admission do not correlate
with the clinical severity of AHA.
33. We have two studies about G6PD
variants and molecular mutations
among Jordanian population
performed by
Dr. Naif Karadsheh
university of Jordan
34. First prformed 1985
revealed two G6PD variants associated
with hemolysis
G6PD AMMAN-1( SEVER
HEMOLYSIS)
G6PD AMMAN-2 ( MILD HEMOLYSIS
35. The second performed in Amman &
jordan valley in nineties, revealed six
missense mutations
• AMMAN
• G6PD MED. 53.6 %
• G6PD A- 14.3 %
• G6PD VALAD. 7.1%
• G6PD Chatham 3.6%
• G6PD Aures 3.6 %
• G6pd Asahi 3.6%
• Unknown 14.2%
• JORDAN VALLEY
• G6PD MED. 33.3%
• G6PD A- 33.3 %
G6PD Chatham 33.3%
36. We need further studies to detect
the genetic VARIANTS of G6PD
deficiency which most commonly
found ALL OVER Jordan and
which we expect to be the type
G6PD Mediterranean
