Background

1
(No Transcript)
2
ASSOCIATION OF GROWTH FAILURE AND LOW SERUM IRON
IN G6PD DEFICIENT CHILDREN

• Y K Abu-Osba, L B El-Asir,
• D A Al-Masri, H I Dawood
• Neonatal Medicine, Jordan Hospital, Amman, Jordan

3
G6PD DEFICIENCY, GROWTH FAILURE AND LOW SERUM
IRON Background

• G6PD deficiency (G6PD-D) is not uncommon in
  Jordan as well as in many Arab World countries.
  Also failure to thrive and iron deficiency is
  common in developing countries especially in low
  income sectors of the population .
• About 400 million people worldwide have G6PD
  deficiency, making it the most common inherited
  enzyme deficiency. In those who are born with it,
  either the red blood cells do not make enough of
  the enzyme or the G6PD produced is altered and
  cannot function properly

4
G6PD DEFICIENCY, GROWTH FAILURE AND LOW SERUM
IRON Background-2

• G6PD, is just one of the many enzymes that affect
  cell metabolism. G6PD is produced by red blood
  cells and helps the body process carbohydrates,
  turning them into energy .
• Normal functioning of G6PD is also important to
  the body because it protects the red blood cells
  from the stress of oxidizing substances. These
  are potentially harmful byproducts that are
  created when certain medications are taken or
  when the body's immune system is working hard to
  fight infection.

5
G6PD DEFICIENCY, GROWTH FAILURE AND LOW SERUM
IRON Aim of the study

• Association of G6PD-D and growth failure had not
  been described to our knowledge. Also association
  of low serum iron and G6PD-D has not been
  reported.  
• The aim of this report is to draw attention to
  the combination of these findings G6PD-D, low
  serum iron and failure to thrive.  

6
G6PD DEFICIENCY, GROWTH FAILURE AND LOW SERUM
IRON Setting and Methods

• We conducted the observations at Jordan
  Hospital Medical Center and Clinics .
• Patients seen in the outpatient clinic
  suffering from failure to thrive (FTT) were
  screened for common causes of FTT as indicated by
  the clinical presentation .

7
G6PD DEFICIENCY, GROWTH FAILURE AND LOW SERUM
IRON Methods

• Investigations included complete blood count,
  urine and stool analysis, kidney function,
  serum iron, total iron binding capacity,
  ferritin, growth hormone, IGF, G6PD
  level in red blood cells.
• G6PD determinations were done by the Trinity
  Biotech procedure which is a modification
  of the spectrophotometric methods of
  Komberg and Horecker and of Lohr and
  Waller method .
• Some of the investigations were not done for
  clinical or financial reasons .

8
G6PD DEFICIENCY, GROWTH FAILURE AND LOW SERUM
IRON Results

• Thirteen children were investigated for poor
  weight gain or decelaration of their physical
  growth.
• Age ranged between 3 months to 11years.
• Weight was below 10th centile in 11 children, and
  two had steady decrease in their weight centile
  for age.

9
G6PD Def, Growth Failure and Low Serum
IronGrowth Parameters

10
G6PD DEFICIENCY, GROWTH FAILURE AND LOW SERUM
IRON Results

• G6PD levels range between 20 to 155, average 114,
  SD 46.55 mU/109 erythrocytes , normal for newborn
  219-564 and for children (1m to 1year of age) and
  adults 186-473 and146-376 mU/109 respectively.

11
??G6PD LEVELS
12
G6PD DEFICIENCY, GROWTH FAILURE AND LOW SERUM
IRON Results

• None of the patients were anemic except one ,
  hemoglobin range 8.6-12.8 g/dL, average 11.4, and
  SD 0.67 gm (normal Hgb 10.3-14.9).
• Serum iron was measured in 11 patients, 8
  children had lower serum iron than normal, range
  14 to 32, average 21.75, SD 7.2 (normal 37-158
  ug/dl). Serum iron was not done in two patients .
  Total iron binding capacity was within normal in
  8 patients, high in one patient, and not done in
  two.
• Other tests results were unremarkable.

13
Hematocrit levels
14
Serum iron levels
15
G6PD DEFICIENCY, GROWTH FAILURE AND LOW SERUM
IRON Conclusions

• We described an association of weight gain
  failure / deceleration and low serum iron in
  thirteen children with G6PD-D.
• We did not establish a cause and effect
  findings.
• We want to draw the attention of the possible
  presence of these findings together although
  we expect normal or high serum iron with
  possible chronic / acute hemolysis in
  G6PD-deficient patients.

16
G6PD DEFICIENCY, GROWTH FAILURE AND LOW SERUM
IRON Recommendations

• We recommend a longitudinal controlled double
  blind study to clarify these findings, and screen
  all newborns for G6PD deficiency in high risk
  areas.

17
(No Transcript)