الفهرس 
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Abstract
In Egypt, β-TM seriously threatens public health, estimated over 1–5 million newborns affected by this disorder; and it is considered the most common genetically determined chronic hemolytic anemia (85.1%) in our region. A high rate of carriers has been reported in Egypt, ranging from 4– 10%. Also, growth failure in BTM is a recognized feature. Its pathogenesis is multifactorial. Nutrition is a significant factor in a child’s growth, development, and overall functioning.
The aim of this study was to assess the impact of nutritional status on insulin-like growth factor-1 (IGF-1) levels among Egyptian children with βTM and to identify Cutoff values of macronutrients associated with stunted growth.
Patients and methods:
This cross-sectional study included 67 children with β-TM aged 2 to 18 years (case group) enrolled from the Pediatric hematological unit at Menoufia University Hospital, in the period between April 2021 to November 2022. Thirty-five age- and sex-matched healthy children without hematologic disorders or a family history of blood disorders served as a control group and were recruited from the general outpatient clinic.
Age, sex, frequency of blood transfusions, type of chelation therapy and compliance with chelation therapy were documented. The chelation therapy used on our patients was either mono or combination. The proper doses taken throughout each day were verified by prescription refills and pill count after reviewing patient self-reports of dose-taking. Puberty was evaluated using Tanner staging.
We recorded daily food intake on three visits using ”24-hour recall” and analyzed the average daily servings of major food groups. We measured the quantity of consumed food in grams or household measures and determined nutrients and calories using tables from the National Nutrition Institute (NNI) Egypt and Michelle Mcquire and KA Beerman.
All anthropometric measurements were taken and plotted on Egyptian Z-score charts.
In Laboratory Investigations, Pretransfusion hemoglobin and ferritin levels were measured in addition to serum IGF-1.
Results:
This study compared children with β-TM to a control group. The mean age of children with β-TM was 10.672±3.75 years, ranging from 1-18 years, while the control group had a mean age of 9.37±3.3 and ranged from 3-18 years. Of the children with β-TM, 36 (53.7%) were male, and 31 (46.3%) were female. In the control group, 16 children (45.7%) were male, and 19 (54.3%) were female. However, there were no significant differences in age, sex, or residence between the two groups.
A significantly higher incidence of positive family history of thalassemia (40.3%) and consanguinity (35.8%) was found among our children with β-TM compared to the control group.
Most β-TM patients were prepubertal and a quarter experienced delayed puberty, which differed significantly from control groups.
Patients with β-TM had an average onset of illness at 80.07±4.12 months and received blood transfusions every 25.6±7 days. 16.4% had undergone splenectomy. 32.8% had regular iron chelation compliance, while 67.2% had intermittent compliance. Chelation was done using Deferasirox (53.7%), Deferoxamine (7.5%), or a combination of both (38.8%).
All anthropometric measurements recorded in the recruited children with β-TM were statistically significantly lower than that recorded in the control group, except for weight was significantly higher than that recorded in the control group. In addition, the median of weight, height, and BMI zscore of β-TM patients were significantly lower than that of controls. Also, mean MUAC was significantly low in cases than in controls.
A significantly high proportion of stunting and wasting was detected among the studied children with β-TM.
Children with β-TM had significantly lower Hb levels and higher ALT, AST, and serum ferritin levels, as well as lower IGF-1 levels compared to healthy controls.
In the current study, children with β-TM consumed fewer macronutrients, energy, and servings food groups than the control group.
β-TM children showed significant positive correlations between IGF1 and weight, height, BMI, MUAC, fruit, orange vegetable intake, protein, carbohydrate, fat, and Energy intakes. However, there was a significant negative correlation between IGF-1 and serum ferritin.
Moreover, height correlates positively with protein intake and negatively with serum ferritin levels.
IGF-1 levels of ≤1.4 ηg /ml and serum ferritin levels of ≥1950 ng/ml can predict stunting in β-TM patients with a sensitivity of 95.5% and 75%, respectively, and a specificity of 91.3% and 60%, respectively. Additionally, macronutrient and energy intake can also predict stunting in children with βTM.
Multivariate linear regression showed that protein was the most potent independent risk factor predicting IGF-1 in children with β-TM. Carbohydrates were the second-strongest independent risk factor of IGF-I in β-TM patients.