Study of Neuro- Psychological Complications of Children with Beta-Thalassemia: Relation to Growth Pattern and Hematological Parameters
Hanan Galal Azouz;
Abstract
Beta-thalassemia represents a group of recessively inherited hemoglobin disorders first described by Cooley and Lee and characterized by reduced synthesis of β-globin chain. The homozygous state results in severe anemia, which needs regular blood transfusion.
Beta thalassemia is the most common chronic hemolytic anemia in Egypt (85.1%). It has a high carrier rate that has been estimated in normal random subjects from different geographical areas of Egypt.
Present transfusion protocols have increased the life expectancy of patients with beta-thalassemia major, but hemosiderosis is a major clinical complication of the treatment. Short stature and hypogonadism are extremely frequent in patients with thalassemia. Neurological complications have been attributed to various factors such as chronic hypoxia, bone marrow expansion, iron overload, and desferrioxamine (DFO) neurotoxicity. In most cases, neurological involvement does not initially present with relevant signs or symptoms (i.e., is subclinical) and can only be detected during neurophysiological or neuroimaging evaluation.
Beta-thalassemia major has received little investigation on the psychological aspects of the disease and the psychosocial adjustment of patients with this anemia. Many causes, including the chronicity of disease, burden of treatment modalities, morbidities, and the expectation of early death resulting from the disease complications, may lead to psychosocial burden in Thalassemia Major patients.
The aim of the present study was:
- To detect neuro-psychological disturbances in beta-thalassemic children.
- To correlate between these disturbances and hematological parameters, growth pattern and pubertal staging of these patients.
This cross sectional descriptive study was conducted at the outpatient hematology clinic and neurology units of Alexandria University Children’s hospital at El-Shatby in the period from May 2009 till May 2011. It included 40 children aged 6-18 years who had clinical and laboratory diagnosis of beta-thalassemia with no other chronic diseases as heart failure, renal failure, hepatic failure or diabetes mellitus and no family history of any psychiatric illness. Ethical consent was taken from parents or guardians of the patients [written form] after explanation of the aim of the study and its benefits for their children and other children who have the same disease.
All patients enrolled in the study were subjected to:
1- History taking: Stressing on patient age, gender, patient's age at the first transfusion, and chelation therapy (type, dose, route of administration, age at onset of chelation therapy and duration of therapy).
2- Socio – economic evaluation which is questionnaire to parents of children about occupation of father, education of mother, and monthly income. (El-Shakhs, 1995)
3- Clinical examination: with special emphasis on anthropometric measurements, pubertal development, abdominal examination for hepatosplenomegaly and neurological Examination.
4- Laboratory investigations included: complete blood count, Serum ferritin, Serum iron, Serum zinc, total iron binding capacity.
5- Electroencephalogram (EEG) recording.
6- Measurement of intelligences quotients (IQ) by Standford-Binet scale, Arabic Version.
7- Assessment of child behavior: we used the Arabic translated form of Child behavior check list (CBCL) 6-18yr.
8- The Egyptian version of child manifest anxiety scale (CMAS).
9- The Arabic version of child depression inventory (CDI)
Results
I. Descriptive data of the studied patients
The current study included 19 beta thalassemic boys and 21 beta thalassemic girls. It was found that the mean age in patients was 10.58 ± 2.7 years. The disease duration ranged between 4.0 – 15.0 years and mean was 8.43 ± 2.64 years. The age of starting blood transfusion was 1.0 – 10.0 years and the mean was 3.65 ± 2.86 years.19 patients (47.5%) had splenectomy (9 boys and 10 girls). Regarding the weight, it ranged from 16.0 – 49.0 kg and mean was 28.71 ± 6.73kg.The weight-z score ranged -4.60 – 0.29. The height ranged from 104.0 – 164.0 cm and mean was 132.48 ± 13.90 cm. The z scores of height ranged -4.32 – 1.23 with 70% of patients were <1 or less and only 5% of patients had z-score >1. The body mass index ranged 12.80 – 20.30 and BMI- z score ranged -3.31 – 1.63 with 32.5% of patients had z-score of <1 or less and only 10% of patients had z-score >1. The level of hemoglobin (Hb) in patients ranged from 4.60 – 9.40 gm/dl with a mean 6.90 ± 1.07 gm/dl. As regard mean serum zinc was93.62 ± 25.19ug /dl, iron was 180.68 ± 33.84 ug/dl and ferritin level was 2942.90 ± 1920.94ugldl with 85% of patients had serum ferritin above 1000 ug/dl as well as mean total iron binding capacity was 93.53 ± 30.65.
52% of studied patients had normal motor system and 48% had abnormal motor system examination in the form of either decreased muscle power and/or bulk. None of our patients had sensory abnormalities or cranial nerves abnormailities. 22.5% of patients had immature EEG background activity and 5% had abnormal epileptiform activity. 50% of studied patients had subaverage IQ, 15 % of them had slow learnr and 35% had normal IQ.
CBCL syndrome items T-score categories in the studied patients showed that boys had a statistically significant higher withdrawn and somatic behavior than girls. Somatic problems had highest percentage of clinical range was (47.5%), followed by anxious behavior (20.0%) then attention problem (17.5%). 14 (35%) of thalassemic children had clinically abnormal high CBCL total scores indicating the presence of behavioral problems in them. Abnormal CBCL Internalization problem scores were seen in 55% of children, indicating symptoms of social withdrawal, somatic complaints, and anxiety/depression. Clinically significant, abnormal CBCL Externalization score was seen in 11(27.5%) of thalassemic children indicating rule breaking and aggressive behavior symptoms. Thought problems mean score was significantly higher in boys of older age group compared to younger age group (p 0.040*).
Beta thalassemia is the most common chronic hemolytic anemia in Egypt (85.1%). It has a high carrier rate that has been estimated in normal random subjects from different geographical areas of Egypt.
Present transfusion protocols have increased the life expectancy of patients with beta-thalassemia major, but hemosiderosis is a major clinical complication of the treatment. Short stature and hypogonadism are extremely frequent in patients with thalassemia. Neurological complications have been attributed to various factors such as chronic hypoxia, bone marrow expansion, iron overload, and desferrioxamine (DFO) neurotoxicity. In most cases, neurological involvement does not initially present with relevant signs or symptoms (i.e., is subclinical) and can only be detected during neurophysiological or neuroimaging evaluation.
Beta-thalassemia major has received little investigation on the psychological aspects of the disease and the psychosocial adjustment of patients with this anemia. Many causes, including the chronicity of disease, burden of treatment modalities, morbidities, and the expectation of early death resulting from the disease complications, may lead to psychosocial burden in Thalassemia Major patients.
The aim of the present study was:
- To detect neuro-psychological disturbances in beta-thalassemic children.
- To correlate between these disturbances and hematological parameters, growth pattern and pubertal staging of these patients.
This cross sectional descriptive study was conducted at the outpatient hematology clinic and neurology units of Alexandria University Children’s hospital at El-Shatby in the period from May 2009 till May 2011. It included 40 children aged 6-18 years who had clinical and laboratory diagnosis of beta-thalassemia with no other chronic diseases as heart failure, renal failure, hepatic failure or diabetes mellitus and no family history of any psychiatric illness. Ethical consent was taken from parents or guardians of the patients [written form] after explanation of the aim of the study and its benefits for their children and other children who have the same disease.
All patients enrolled in the study were subjected to:
1- History taking: Stressing on patient age, gender, patient's age at the first transfusion, and chelation therapy (type, dose, route of administration, age at onset of chelation therapy and duration of therapy).
2- Socio – economic evaluation which is questionnaire to parents of children about occupation of father, education of mother, and monthly income. (El-Shakhs, 1995)
3- Clinical examination: with special emphasis on anthropometric measurements, pubertal development, abdominal examination for hepatosplenomegaly and neurological Examination.
4- Laboratory investigations included: complete blood count, Serum ferritin, Serum iron, Serum zinc, total iron binding capacity.
5- Electroencephalogram (EEG) recording.
6- Measurement of intelligences quotients (IQ) by Standford-Binet scale, Arabic Version.
7- Assessment of child behavior: we used the Arabic translated form of Child behavior check list (CBCL) 6-18yr.
8- The Egyptian version of child manifest anxiety scale (CMAS).
9- The Arabic version of child depression inventory (CDI)
Results
I. Descriptive data of the studied patients
The current study included 19 beta thalassemic boys and 21 beta thalassemic girls. It was found that the mean age in patients was 10.58 ± 2.7 years. The disease duration ranged between 4.0 – 15.0 years and mean was 8.43 ± 2.64 years. The age of starting blood transfusion was 1.0 – 10.0 years and the mean was 3.65 ± 2.86 years.19 patients (47.5%) had splenectomy (9 boys and 10 girls). Regarding the weight, it ranged from 16.0 – 49.0 kg and mean was 28.71 ± 6.73kg.The weight-z score ranged -4.60 – 0.29. The height ranged from 104.0 – 164.0 cm and mean was 132.48 ± 13.90 cm. The z scores of height ranged -4.32 – 1.23 with 70% of patients were <1 or less and only 5% of patients had z-score >1. The body mass index ranged 12.80 – 20.30 and BMI- z score ranged -3.31 – 1.63 with 32.5% of patients had z-score of <1 or less and only 10% of patients had z-score >1. The level of hemoglobin (Hb) in patients ranged from 4.60 – 9.40 gm/dl with a mean 6.90 ± 1.07 gm/dl. As regard mean serum zinc was93.62 ± 25.19ug /dl, iron was 180.68 ± 33.84 ug/dl and ferritin level was 2942.90 ± 1920.94ugldl with 85% of patients had serum ferritin above 1000 ug/dl as well as mean total iron binding capacity was 93.53 ± 30.65.
52% of studied patients had normal motor system and 48% had abnormal motor system examination in the form of either decreased muscle power and/or bulk. None of our patients had sensory abnormalities or cranial nerves abnormailities. 22.5% of patients had immature EEG background activity and 5% had abnormal epileptiform activity. 50% of studied patients had subaverage IQ, 15 % of them had slow learnr and 35% had normal IQ.
CBCL syndrome items T-score categories in the studied patients showed that boys had a statistically significant higher withdrawn and somatic behavior than girls. Somatic problems had highest percentage of clinical range was (47.5%), followed by anxious behavior (20.0%) then attention problem (17.5%). 14 (35%) of thalassemic children had clinically abnormal high CBCL total scores indicating the presence of behavioral problems in them. Abnormal CBCL Internalization problem scores were seen in 55% of children, indicating symptoms of social withdrawal, somatic complaints, and anxiety/depression. Clinically significant, abnormal CBCL Externalization score was seen in 11(27.5%) of thalassemic children indicating rule breaking and aggressive behavior symptoms. Thought problems mean score was significantly higher in boys of older age group compared to younger age group (p 0.040*).
Other data
| Title | Study of Neuro- Psychological Complications of Children with Beta-Thalassemia: Relation to Growth Pattern and Hematological Parameters | Other Titles | دراسة المضاعفات العصبية و النفسية فى الأطفال المصابين بأنيميا البحر المتوسط : علاقته بنمط النمو و مقاييس الدم | Authors | Hanan Galal Azouz | Issue Date | 2015 |
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