A national screening for the prevalence and profile of disability types among Egyptian children aged 6-12 years: a community-based population study.

AIM: This study aimed to determine the prevalence of disability domains among Egyptian children in the age group of 6-12 years as well as assess their socio-demographic, epidemiological, and perinatal predictors. METHODS: A national population-based cross-sectional household survey targeting 20,324 children from eight governorates was conducted. The screening questionnaire was derived from the WHO ten-question survey tool validated for the identification of disabilities. RESULTS: The prevalence of children with at least one type of disability was 9.2%. Learning/ comprehension was the most prevalent type (4.2%), followed by speech/communication (3.7%), physical/ mobility and seizures (2.2% for each), intellectual impairment (1.5%), visual (0.7%), and hearing (0.4%). The commonest predictors for disabilities were children who suffered from convulsions or cyanosis after birth and maternal history of any health problem during pregnancy. However, preterm and low birth weight (LBW) babies or being admitted to incubators for more than two days were strong predictors for all disabilities except hearing disability. A history of jaundice after birth significantly carried nearly twice the odds for seizures (AOR = 2.2, 95% CI:1.5-3.4). History of difficult labor was a predictor of intellectual impairment (AOR = 1.5, 95% CI:1.1-2.0). A disabled mother was a strong predictor for all disabilities except seizures, while a disabled father was a predictor for visual and learning/ comprehension disabilities (AOR = 3.9, 95% CI:2.2-7.1 & AOR = 1.6, 95% CI:1.1-2.4 respectively). Meanwhile, both higher maternal and paternal education decreased significantly the odds to have, physical/ mobility and Learning/ comprehension by at least 30%. CONCLUSION: The study found a high prevalence of disability among Egyptian children aged 6-12 years. It spotted many modifiable determinants of disability domains. The practice of early screening for disability is encouraged to provide early interventions.

Determinants of having severe acute respiratory syndrome coronavirus 2 neutralizing antibodies in Egypt.

BACKGROUND: Reported laboratory-confirmed COVID-19 cases underestimate the true burden of disease as cases without laboratory confirmation, and asymptomatic and mild cases are missed by local surveillance systems. Population-based seroprevalence studies can provide better estimates of burden of disease by taking into account infections that were missed by surveillance systems. Additionally, little is known about the determinants of seroconversion in community settings. METHODS: We conducted a cross-sectional serologic survey among 888 participants in Egypt. RESULTS: Neutralizing antibodies were detected in 30% of study volunteers. Age and educational level were associated with being seropositive as people older than 70 years and people with graduate degrees had lower seroprevalence. Self-reporting cases having COVID-19-related symptoms such as fever, malaise, headache, dyspnea, dry cough, chest pain, diarrhea, and loss of taste or smell were all associated with having antibodies. Fever and loss of taste or smell were strong predictors with odds ratios of 2.1 (95% confidence interval: 1.3-3.5) and 4.5 (95% confidence interval: 2.6-7.8), respectively. CONCLUSIONS: Our results can guide COVID-19 prevention and control policies and assist in determining the immunity level in some Egyptian communities.

Carotid intimal medial thickness and its relation to endothelial dysfunction and echocardiographic changes in adolescents with type 1 diabetes.

AIM: To assess carotid intimal medial thickness (cIMT) in adolescent type 1 diabetic patients and to detect its relation with echocardiographic changes and flow mediated dilatation (FMD) in the brachial artery. METHODS: The study included 62 type 1 diabetic patients and 30 healthy volunteer of the same age and sex. A blood sample was taken for analysis of glycosylated hemoglobin and lipid profile and a urine sample was taken for analysis of albumin/creatinine ratio. cIMT, echocardiography, and FMD via ultrasound were also done; t-test or Mann-Whitney U-test (for non-symmetrically distributed data) for independent variables and Pearson's or Spearman correlation were used. RESULTS: The mean age of patients was 16.3 ± 1.5 years and mean duration of diabetes was 9.4 ± 2.9 years. cIMT (Rt, Lt, and both Rt and Lt) were significantly higher, while FMD and FMD/nitrate mediated dilatation (NMD) ratio was significantly lower in diabetics. Rt cIMT had a significant negative correlation with FMD and FMD/NMD. cIMT had a significant positive correlation with left ventricular end diastolic dimension, inter ventricular septum thickness, peak mitral flow velocity during early diastole/peak mitral flow velocity during late diastole, left ventricular mass, and left ventricular mass index (p<0.05). In addition, cIMT had a significant correlation with waist circumference, waist/height ratio, albumin/ creatinine ratio, total cholesterol, and triglyceride. CONCLUSION: We conclude that alteration in myocardial function and vascular endothelial dysfunction induced by diabetes mellitus may begin early with the association of early atherosclerotic changes.

Assessment of human cartilage glycoprotein 39 (YKL-40), preptin, and nitric oxide in adolescent patients with type 1 diabetes and its relation to cardiorenal affection.

AIM: To evaluate new biomarkers such as YKL-40, preptin, and nitric oxide (NO) in patients with diabetes and to assess its relation to cardiorenal injury. PATIENTS AND METHODS: The study included 62 patients with type 1 diabetes and 30 healthy volunteers. Blood sample was taken for assessment of glycosylated hemoglobin, lipid profile, YKL-40, preptin, and NO. Also, urine sample was taken for analysis of albumin/creatinine ratio. Echocardiography was also done. RESULTS: NO was lower, whereas YKL-40, preptin, and albumin/creatinine ratio were significantly higher in patients with diabetes. NO had a significant negative correlation with LVEDD, LVESD, PWT, LV mass, YKL-40, preptin, and albumin/creatinine ratio. YKL-40 had a significant positive correlation with waist/height ratio, preptin and negative correlation with E/A ratio. Stepwise multiple regression revealed that E/A ratio is the only parameter related to YKL-40. On the contrary, NO and systolic blood pressure are related to preptin. CONCLUSION: A significant reduction of NO and elevation of YKL-40 and preptin was found in patients with diabetes. A decrease in NO is associated with diastolic dysfunction, LV hypertrophy, and renal impairment, whereas YKL-40 is associated with diastolic dysfunction. An increase in preptin level was associated with hypertension.

Evaluation of fetuin-A and carotid intima-media thickness in adolescent type 1 diabetic patients.

OBJECTIVE: To evaluate fetuin-A level and carotid intima-media thickness (CIMT) in adolescent type 1 diabetics. PATIENTS AND METHODS: The study included 62 type 1 diabetic patients and 30 healthy volunteers of the same age and sex. Blood sample was taken for assessment of glycosylated hemoglobin (HbA1), lipid profile, and fetuin-A. Urine sample was also taken for assessment of albumin/creatinine ratio. Anthropometric measurements were taken, including weight, height, and waist and hip circumference. CIMT was assessed for all patients and controls. RESULTS: Serum fetuin-A, Rt., Lt. and both CIMT were significantly higher in diabetics. Fetuin-A had a significant positive correlation with duration of disease, waist and hip circumference, BMI, BMI SDS, waist/height ratio, Rt., Lt. and both CIMT. Stepwise multiple regression analysis revealed that the duration of disease, waist/height ratio, and HDL-c were the factors related to fetuin-A. CONCLUSION: Adolescent type 1 diabetic patients have high fetuin-A levels and increased CIMT, with the latter representing the development of early atherosclerosis. In this light, adolescents with type 1 diabetes require frequent follow up for early detection of atherosclerosis.

Assessment of hearing in children with type 1 diabetes mellitus.

OBJECTIVE: To evaluate auditory function in a group of Egyptian type 1 diabetic children. METHODS: This was a cross sectional observational study, which included 40 patients with type 1 diabetes and 40 controls. HbA1, urinary albumin/creatinine ratio, and auditory assessments (including dizziness questionnaire, pure tone audiometry, speech audiometry, tympanometry, and auto-acoustic cochlear emission) were completed for all patients and controls. Mann-Whitney U-test, χ2-test and Spearman's correlation were used for statistical analyses. RESULTS: Assessment of pure tone audiometry revealed that the diabetics had a significantly higher reading in high frequency at 8000 Hz, 16,000 Hz, 17,000 Hz, and 18,000 Hz on the right side and at 4000 Hz, 8000 Hz, 16,000 Hz, 17,000 Hz, and 18,000 Hz on the left side. There was a significantly lower level in speech reception threshold, repetition of words, and masking level of diabetics on the left side. Evaluation of transient otoacoustic emission revealed that diabetics recorded significantly lower signal to noise ratio at 4000 Hz on the right side and at 1000, 1500, 4000, and all Hz on left side. There was significant lower emission amplitude in the right side of the diabetics group at 1500 and 4000 Hz and at 1000, 1500, and 4000 Hz on the left side. Patients with failed otoacoustic emission were significantly higher in disease duration >10 years. CONCLUSION: Type 1 diabetes is associated with high/extended high frequency hearing loss, more prominent on the left side and with longer disease duration.

Asymmetric dimethyl L-arginine, nitric oxide and cardiovascular disease in adolescent type 1 diabetics.

OBJECTIVE: To evaluate asymmetric dimethyl L-arginine (ADMA), nitric oxide (NO) and cardiovascular disease in adolescent type 1 diabetics. METHODS: The study included 62 type 1 diabetic patients and 30 healthy volunteers of the same age and sex. Blood samples were taken for assessment of ADMA, NO, oxidized low density lipoprotein (OxLDL), glycosylated hemoglobin, and lipid profile. Urine samples were taken for assessment of albumin/creatinine ratio. M mode echocardiography and flow mediated dilatation (FMD) via ultrasound were completed; t-test for independent variables, Pearson's correlation, and stepwise multiple regression analysis were used. RESULTS: The mean age of patients was 16.3±1.5 years and mean duration of diabetes was 9.4±2.9 years. Nitric oxide, ADMA and FMD were significantly lower, while OxLDL and the albumin/creatinine ratio were significantly higher in diabetics. Nitric oxide had a significant negative correlation with left ventricular end-diastolic dimension, left ventricular end-systolic dimension, posterior wall thickness, left ventricular mass, albumin/creatinine ratio, and OxLDL, as well as a positive correlation with ADMA. Albumin/creatinine ratio had a significant positive correlation with OxLDL and negative correlation with ADMA. Stepwise multiple regression analysis revealed that ADMA is the only parameter related to NO, however, albumin/creatinine ratio and OxLDL are related to ADMA. CONCLUSIONS: Type 1 diabetic patients had endothelial and diastolic dysfunction. The reduction in NO, ADMA, and elevation of OxLDL, and its relation to echocardiographic data and albumin/creatinine ratio, may reflect their role in cardiac and renal affection.

Evaluation of the policy of secondary prevention against rheumatic fever among Egyptian children.

OBJECTIVE: We evaluated the effectiveness of long acting penicillin (LAP) as a 2-weekly regimen in winter and a 3-weekly regimen in summer for prevention of streptococcal colonization and also studied the common complaints of patients during the period of compliance for LAP administration. We also attempted to determine the incidence of relapses or recurrence of rheumatic fever (RF) after the onset of first episode of RF. PATIENTS & METHODS: 210 rheumatic patients with good compliance to LAP (for at least one year) were included in the study. Demographic, clinical information, patients' complaints and echocardiographic data of rheumatic patients were collected both retrospectively and prospectively. Anti-streptolysin O titre (ASOT) and throat swab culture were done at the end of the study (on Day 14 in the 2-weekly regimen and on Day 21 in the 3-weekly regimen). RESULTS: The age of onset of rheumatic fever was mostly between 5 and 15 years and the youngest patient was 2 years old. Subclinical carditis (SCC) was present in 79 (37%) of all the patients in the study population. Only 7 patients (3.3%) had a relapse within 2 years of the acute episode of RF. At the end of the study, ASOT was found to be high only in 11 patients (5.2%) and throat swab cultures were found negative in all patients CONCLUSION: LAP regimen is fairly effective in eradicating streptococcal colonization. The incidence of relapse of RF within 2 years of the acute episode of RF is relatively low.

Predicting growth response among Egyptian prepubertal idiopathic isolated growth hormone deficient children.

OBJECTIVE: To determine the predictors of growth response to growth hormone treatment in a group of isolated idiopathic growth hormone (GH). PATIENTS AND METHODS: 477 GH deficient (GHD) children with GH therapy were included in the study. Patients were followed up for a minimum of 1 year and up to 6 years. Multiple linear regressions were performed to identify predictors of growth response to rhGH in the first 4 years of treatment. RESULTS: In the first year, three significant predictors of growth were identified: GH peak [ln (ug/L)], age of onset of therapy and target height-height SDS. In the second and third years of therapy, growth velocity (GV) was both significantly and positively correlated to the GV (cm/year) of the previous year. CONCLUSION: Prediction models offer a valuable tool for individualization and assuring adherence to rhGH and thus a cost effective treatment, which is the ultimate goal of GH therapy.

Egyptian growth hormone deficient patients: demographic, auxological characterization and response to growth hormone therapy.

OBJECTIVE: To study growth response to growth hormone (GH) treatment in growth hormone-deficient (GHD) patients. PATIENTS AND METHODS: In total, 477 GHD children were included in the study. Patients were followed up for a minimum of 1 year and up to 6 years, anthropometric assessment was performed every 3 months, while thyroid profile was followed every 6 months. Student's t-test was used for analysis of two quantitative variables. RESULTS: Patients with complete GHD and multiple pituitary hormone deficiency were significantly shorter as expressed in their height standard deviation score (SDS), target height - height (SDS) and a more bone age delay. Patients with complete GHD showed better growth response compared with those with partial GHD in the first 2 years of therapy. CONCLUSION: We conclude that anthropometric assessment is the cornerstone in GHD diagnosis and follow-up, where catch up growth occurs in the first 2 years of therapy followed by a plateau.

Hypertension in type 1 diabetic patients-the influence of body composition and body mass index: an observational study.

OBJECTIVE: To estimate the influence of body composition and body mass index on blood pressure in type 1 diabetic patients. METHODS: This cross-sectional, observational study included 45 type 1 diabetic patients and 30 age and sex matched healthy volunteers. Blood pressure, anthropometric measurements, and body composition by dual X-ray absorptiometry (DXA) were done for all patients and controls. T-test, one way ANOVA and multiple regressions were used for statistical analyses. RESULTS: Twenty diabetic patients (44.4%) had hypertension. Hypertensive diabetic patients had the highest total fat mass %, soft tissue fat mass %, abdomen fat % and fat/lean ratio followed by non hypertensives and the least was the controls (p=0.0001). Abdominal fat % was the only parameter significantly associated with mean arterial blood pressure (ß-5.8, 95% CI: 3.7-8.0, p=0.0001) and systolic blood pressure (ß-8.6, 95% CI: 5.4-11.9, p=0.0001) by stepwise multiple regression analysis in the diabetic patients. In the contrary, abdominal fat % (ß-2.7, 95% CI: 0.9-4.5, p=0.006), duration of diabetes (ß-2.5, 95% CI: 1.4-3.5, p=0.0001) and fat/ lean ratio (ß-11.7, 95% CI: 1.5-21.9, p=0.03) were related to diastolic blood pressure. CONCLUSION: Diabetes is associated with an increase in body fat especially abdominal, which leads to an increase in insulin resistance and decrease in lean mass. In type 1 diabetic patients, blood pressure depends on body mass index SDS and fat mass. Abdominal fat is the only factor related to mean arterial blood pressure and systolic blood pressure.

Bone density, body composition, and markers of bone remodeling in type 1 diabetic patients.

OBJECTIVE: To assess bone mineral density (BMD), body composition by dual X-ray absorptiometry (DXA), and various biochemical markers of bone growth and resorption in a group of children with type 1 diabetes mellitus (T1DM). PATIENTS AND METHODS: The study included 47 patients with T1DM and 30 age- and sex-matched controls. Anthropometric measurements, biochemical markers for bone formation, bone resorption and DXA were done for all patients and controls. RESULTS: Of our diabetes patients, seven (16.7 %), three (7.3 %), and 17 (41.5%) met diagnostic criteria for osteopenia at the right femur, lumbar spine and total body, respectively. On the other hand, osteoporosis as defined by the WHO criteria was diagnosed in 21 patients (51.2%) at the total body by DXA. Lean body mass and lean fat ratio were lower, while, total fat mass, abdominal fat%, soft tissue fat mass%, and fat/lean ratio were higher in diabetics compared to controls. Also, our patients showed lower serum osteocalcin, osteoprotegerin, procollagen type 1, and higher urinary deoxypyridinoline. Pubertal (diabetics and controls) have higher BMD and BMC than prepubertal. CONCLUSION: Diabetic patients had a low BMD after adjustment (Z score), low bone formation and high bone resorption markers. Diabetes control and increase in BMI leads to a decrease in the incidence of low bone mineral density. Diabetes causes an increase in body fat especially abdominal fat which leads to an increase in insulin resistance and decrease in lean mass.

Natural progression of cardiac autonomic neuropathy in patients with type 1 diabetes: a four-year follow-up study.

OBJECTIVE: To determine the prevalence and clinical characteristics of cardiac autonomic neuropathy in type 1 diabetic patients who were followed up for 4 years to shed further light on the natural progression of cardiac autonomic neuropathy. METHODS: It is a prospective cohort observational study, consisted of 57 patients who were originally studied using the standard tests proposed by Ewing and Clarke (1985). At two years follow up, 46 patients were reevaluated, 55 patients from the original study were reevaluated after another 2 years for the 3rd time using the same protocol. The control group comprised 30 age and sex matched healthy volunteers. McNemar test, ANOVA for repeated measurements, paired t test and unpaired t test were used for statistical analyses. RESULTS: The prevalence of established cardiac autonomic neuropathy (CAN) at the beginning was 14%. Q-Tc intervals were found to be significantly higher in patients with abnormal cardiovascular reflex (CVRs) in the 2nd examination (0.4±0.04 vs 0.5±0.05 sec, p=0.006). Eighteen patients showed deterioration of their CVRs test between 1st and 3rd examination. There was deterioration of their glycemic control guided by glycosylated hemoglobin (8.5±1.4 vs 9.9±1.5%, p=0.05*) and albumin/creatinine ratio (4.4±4.0 vs 28.2±28.0 mg/g creatinine, p=0.04). On the other hand, 12 patients showed regression of their CVRs test. Only their insulin dose showed significant decrease (1.8±1.3 vs 1.1±0.3 dose/kg, p=0.02). CONCLUSION: The prevalence of established CAN in diabetic patients is high at the beginning of the study. Glycosylated hemoglobin, systolic and diastolic blood pressure were significantly increased in diabetics with deterioration of their CAN. However, the dose of insulin was significantly decreased in diabetics with regression of their CAN.

Screening of growth hormone deficiency in short thalassaemic patients and effect of L-carnitine treatment.

INTRODUCTION: Evaluation of growth hormone (GH) in short thalassaemic patients and effect of L-carnitine therapy in those with hormone deficiency. MATERIAL AND METHODS: The study included 30 ß-thalassaemic patients aged 13.8 ±1.7 years and 30 children with constitutional short stature as controls. Anthropometric measurements (basal and after 6 months), thyroid profile, insulin-like growth factor-1 (IGF-1) and GH provocation by 2 tests were carried out. Eight patients with inadequate GH response to both clonidine and ITT were given L-carnitine treatment for 6 months. They were re-evaluated (clinically, anthropometrically and in the laboratory by doing GH stimulation test) after 6 months of therapy. RESULTS: Twelve (40%) patients had sub-clinical hypothyroidism and 10 (33.3%) had growth hormone deficiency (GHD). Peak GH and growth velocity (cm and standard deviation score [SDS]) were significantly lower while weight (SDS) and weight/height SDS were significantly higher than in patients with constitutional short stature (p < 0.05). A significant positive correlation was found between height and target height (cm). Haemoglobin levels, peak GH, IGF-1 and growth velocity (cm & SDS) were significantly higher and the number of blood transfusions was significantly lower in GH deficiency patients after L-carnitine treatment (p < 0.05). Delta changes were higher in height (cm & SDS), estimated mature height and sitting height and lower in target height - height (SDS and cm) six months after L-carnitine treatment in ß-thalassaemic patients with GHD (p < 0.05). CONCLUSIONS: Growth hormone deficiency is an aetiological factor in thalassaemic patients with short stature. L-carnitine can promote GH secretion and growth.

Bone mineral density, bone turnover markers, lean mass, and fat mass in Egyptian children with congenital adrenal hyperplasia.

INTRODUCTION: Aim of this paper is to assess bone mineral density (BMD) and body composition, by dual energy X-ray absorptiometry (DXA), and various markers of bone growth, in a group of children with congenital adrenal hyperplasia (CAH) on long-term glucocorticoid therapy. MATERIAL AND METHODS: A case-control study included thirty patients with CAH with different states of metabolic control. Their mean age was 7.5 ±4.2 years. All patients are subjected to BMD using DXA at the neck of the femur and lumbar spine. A blood sample was taken for assessment of osteocalcin, osteoprotegerin, and procollagen type 1, as markers of bone formation, as well as RANKL and urinary deoxypyridinoline (DPD), as markers of bone resorption. RESULTS: We found no difference in BMD in patients and control subjects; however, patients showed significantly lower serum osteocalcin (p = 0.008) and osteoprotegerin (p = 0.0001) and significantly higher serum RANKL levels (p = 0.0001). Our results show that patients had significantly lower lean body mass (p = 0.005) and fat/lean ratio (p = 0.008) compared to matched controls. The duration of treatment showed a significant negative correlation with procollagen type 1 (r = -0.49, p = 0.02) and lean mass % (r = -0.43, p = 0.04); however, it showed a significant positive correlation with total fat mass % (r = 0.6, p = 0.0006), and fat/lean ratio (r = 0.43, p = 0.04). Dose of steroid had a significant positive correlation with BMI SDS (r = 0.4, p = 0.02). CONCLUSIONS: Bone mineral density is normal but bone turnover is low in patients with CAH. There is an increase in fat/lean mass in patients with CAH.

Screening for coeliac disease among Egyptian children.

INTRODUCTION: To screen for coeliac disease in Egyptian children with non-endocrinal short stature, refractory iron deficiency anaemia and type 1 diabetes. Also, to evaluate the sensitivity and specificity of different serological tests for diagnosis of coeliac disease (CD). MATERIAL AND METHODS: The study included 292 patients with clinical risk of CD. Testing for coeliac antibodies was performed, together with upper gastrointestinal endoscopy and small intestinal biopsy. RESULTS: Eleven patients (44%) among 25 patients with refractory iron deficiency anaemia, 23 patients (34.3%) among 67 patients with non-endocrinal short stature, and 6 patients (3%) among 200 patients with type I diabetes mellitus were diagnosed by jejunal biopsy as having coeliac disease. AGA (IgG) had the highest sensitivity for diagnosing CD (80.0%) followed by the TTG (72.7%) antibody, while ARA had the highest specificity (95.9%) followed by anti-EMA (94.7%). CONCLUSIONS: Coeliac disease is more common in Egyptian children with refractory iron deficiency anaemia, non-endocrinal short stature and type 1 diabetes than was previously thought; therefore it is mandatory to screen such patients for CD. Serological tests showed fairly good sensitivity and specificity for the diagnosis; however, intestinal biopsy remains the cornerstone for definitive diagnosis of patients with immunological reaction to gluten.

Bone age is the best predictor of growth response to recombinant human growth hormone in Turner's syndrome.

BACKGROUND AND OBJECTIVES: Recombinant human growth hormone (rhGH) is approved for use in children with Turner's syndrome (TS) in most industrialized countries and is recommended in the recently issued guidelines. We determined the growth responses of girls who are treated with rhGH for TS, with an aim to identify the predictors of growth response. MATERIALS AND METHODS: Fifty-six prepubertal girls with TS, documented by peripheral blood karyotype, were enrolled. All the patients received biosynthetic growth hormone therapy with a standard dose of 30 IU/m(2)/week. The calculated dose per week was divided for 6 days and given subcutaneously at night. RESULTS: This study showed that rhGH therapy provides satisfactory auxological results. Bone age delay is to be considered as a predictive factor which may negatively influence the effect of rhGH therapy on final height. The growth velocity in the preceding year is the most important predictor of rhGH therapy response. CONCLUSION: These observations help us to guide rhGH prescription, to reduce the risks and costs.

Assessment of puberty in relation to L-carnitine and hormonal replacement therapy in beta-thalassemic patients.

OBJECTIVE: To investigate puberty in a group of thalassemic patients with delayed or arrested pubertal development and to compare the effects of hormonal and L-carnitine therapy on puberty in those patients. PATIENTS: Thirty-two -thalassemic patients with arrested or failure of puberty were enrolled for 1 year in this study. METHOD: Clinical pubertal assessment and laboratory investigations were done for all patients at the beginning, 6 months later clinical pubertal assessment was done. Patients were divided into two groups (16 each): first group received L-carnitine therapy, while the second group received hormonal therapy. Pubertal and laboratory assessment were done 6 months after hormonal and L-carnitine therapy. RESULTS: Failure of puberty was confirmed in 71.4% of boys and 33.3% of girls, while arrested puberty was observed in 28.6% of boys and 66.7% of girls. All girls had amenorrhea, primary amenorrhea in 88.9% and secondary amenorrhea in 11.1%. Menses occurred in 20% of female patients after L-carnitine therapy and in 37.5% of them after hormonal therapy. Improvement of pubertal staging was observed in 50% of males after L-carnitine therapy compared to 75% of them after hormonal therapy. While improvement of pubertal staging was seen in 90% of females after L-carnitine therapy compared to 100% of females after hormonal treatment. However, these results showed no significant difference between both groups. CONCLUSION: Delayed puberty in beta-thalassemia major is either due to failure of gonads or failure of the whole hypothalamic pituitary gonadal axis. L-carnitine as well as hormonal replacement therapy had a positive effect on puberty in the thalassemic patients. Further studies are needed to clarify the role of L-carnitine on puberty in these patients.

Prevalence of celiac disease, Helicobacter pylori and gastroesophageal reflux in patients with refractory iron deficiency anemia.

OBJECTIVE: The aim of this article is to determine the prevalence of celiac disease (CD), Helicobacter pylori (H. pylori) and gastroesophageal reflux (GER) in patients with resistant iron deficiency anemia (IDA). PATIENTS: The study included 25 patients <18 years of age with refractory IDA (not responding to iron therapy for 3 months in a dose of 6 mg elemental iron/kg/day). METHODS: All patients included in the study were subjected to careful history taking and thorough clinical examination. Blood sample was taken for analysis of antibodies for CD including: antigliadin antibody (AGA), antiendomysial antibody (EMA), antireticulin antibody (ARA) and antitissue Transglutaminase (tTg) IgG antibody. Anti-H. pylori IgG antibodies and a (13)C-urea breath test (UBT) was done to all patients to diagnose H. pylori. Upper gastrointestinal tract endoscopy was done for all patients to evaluate for the presence of some etiologies of intractable anemia as chronic blood loss. These included: CD, H. pylori infection and GER. The upper gastrointestinal tract endoscopy was also done to evaluate the presence of bleeding spots, ulcers or angiomatous malformations. In addition, gastric antral biopsies were taken for diagnosis of H. pylori infection by the following tests: rapid urease test, histopathological examination and culture. RESULTS: CD was positive in 11 out of 25 patients (44%), H. pylori infection in 12 out of 25 patients (48%), while GER was diagnosed in 11 out of 25 patients (44%). Patients with CD had age of presentation < or =2 years in two patients (18.2%) while the remaining nine patients (81.8%) had age of presentation >2 years and it was statistically significant (p = 0.05*). Also patients with H. pylori had age of presentation < or =4 years in five patients (41.7%) and the remaining seven patients (81.8%) had age of presentation >4 years and it was statistically significant (p = 0.03*). Logistic regression analysis demonstrated that the risk factors for severity of anemia were age of patients and duration of anemia. On the other hand, other parameters have no significant influence on the severity of anemia. Also risk factors of short stature were age of presentation of anemia, degree of anemia and H. pylori infection. AGA had the highest sensitivity (100%) followed by antiendomysium antibody (81.8%) while the tTG antibody had the highest specificity (85.7%) for diagnosis of CD. UBT and histopathology had the highest sensitivity (100%) for diagnosis of H. pylori while rapid urease test, culture, H. pylori stool antigen and anti-H. pylori IgG antibody had the highest specificity (100%). In conclusion, refractory IDA may be due to clinically unapparent H. pylori gastritis and CD. CD is one of the most common causes of intestinal malabsorption during childhood which leads to impairment of iron absorption. Apart from offering them gluten-free diet rich in iron, early detection and treatment of IDA and prophylactic iron and folic acid supplementation will go a long way to optimize their mental and psychological functions. Eradication of H. pylori infection with concomitant iron therapy should correct the anemia.