effect of high caloric diet on nutritional parameters of children with thalassemia major

Impaired growth involving both height and weight accompanying thalassemia major poses diagnostic and therapeutic problems. We undertook this study to test the hypothesis that this impaired growth might be corrected, partially or totally, by increasing caloric intake of these patients to compensate for their hypermetabolic status accompanying their anemia and bone marrow hyperactivity. Thirty randomly selected children with thalassemia major were studied. The dietary intake of the thalassemic patients, evaluated by the recall method, appeared to be adequate compared to 30 normal age-matched children. Nutritional status was assessed by measuring the weight, body mass index [BMI], mid-arm circumference [MAC], triceps skin fold thickness [SFT] and serum albumin and insulin-like growth factor-I [IGF-I] concentrations of before and after 8 weeks of high-caloric diet [130-150% of the caloric recommendation for age and sex]. The BMI, triceps SFT and MAC of children with thalassemia were significantly decreased compared to those for the normal control group. IGF-I and albumin concentrations of thalassemic children [69 +/- 20.5 ng/m and 3.65 +/- 0.67 g/dl respectively] were significantly lower than those for normal age-matched children [162.5 +/- 24 ng/ml and 4.29 +/- 0.66 g/dl respectively]. After nutritional supplementation for 8 weeks the MAC, SFT and BMI of the thalassemic children increased significantly [p < 0.05]. Serum IGF-I [84.3 +/- 27.3 ng/ml] and albumin concentrations [3.85 +/- 0.85 g/dl] increased significantly in the thalassemic group after versus before nutritional supplementation however, they were still lower than those for normal children. It appears that the hypermetabolic status of children with thalassemia contributes to their decreased IGFúI synthesis in these patients with subsequent slowing of linear growth and weight gain. Increased caloric dietary intake increased significantly IGF-I synthesis in these patients. This was accompanied with increased BMI, mid-arm circumference and skin fold thickness. Growth impairment of children with thalassemia major, without endocrinopathy and/or cardiomyopathy, can be partially correctable by increasing caloric intake to compensate for their hypermetabolic status

surgical management of ambiguous genitalia secondary to congenital adrenal hyperplasia Alexandria experience

Congenital adrenal hyperplasia is the most common cause of female pseudohermaphroditism all-over the world. The excessive androgenic intermediates released by the diseased adrenal gland virilize the external female genitalia during the early in-utero life to a variable degree. Medical treatment is mandatory to correct the life-threatening metabolic derangement leading to salt-losing crises and surgical correction of the genitalia is essential for the psychologic and functional integrity of the female patient We operated on 12 girls with CAH to correct their genitalia. The surgical procedure included: [1]. after complete degloving of the hypertrophied phallus, mobilization of the roots of the corpora to their attachment at the pubic arch. The neurovascular bundle is dissected and preserved to keep supply of the glans. The corporal bodies are dissected from their attachment to the pubic bone to their termination at the glans. Adequate hemostasis is performed and the glans is fixed with sutures to the roots of the corpora. [2]. A posterior perineal flap is fashioned by drawing an inverted u incision centered on anal verge and reaching to the posterior limit of urogenital sinus anteriorly. [3]. the skin of the phallus, after degloving, is used for replacing the labia minora after division in the midline. The results of this operative procedure proved very effective with excellent cosmetic appearance of the genitalia and good vaginal patency in all the cases followed-up for three years. Mild vaginal stenosis was encountered only in three girls but none had tight stenosis. In conclusion, our data proved that complete correction in one stage is the treatment of choice. Reduction clitoroplasty is the best procedure for the treatment of phallic enlargement. Posterior perineal flap vaginoplasty is highly successful in preventing later vaginal stenosis. However, regular follow up is recommended for calibration and dilatation of the vagina and adjustment of the doses of steroid to assure good suppression of adrenal androgens in these girls

Childhood diabetes in alexandria: mode of presentation and progress

This study was designed to characterize clinical and autoimmune features in all children with newly diagnosed IDDM diagnosed between 1994 and 1998 as well as to follow up their clinical progress. The study was performed on all newly diagnosed children with IDDM presented to the Alexandria University Children's Hospital between 1994 and 1998 [60 children]. After the primary diagnosis and management, the patients were followed up every 2-3 months in the IDDM clinic. The details of any medical problem during the period of study were recorded during the visits. A questionnaire was completed for each newly diagnosed child. This questionnaire was divided into 4 sections and focused on demographic and family characteristics, family history of diabetes, previous medical history of the child and manifestations before diagnosis. Details of clinical and laboratory data at diagnosis were recorded. The clinical features evaluated were the duration of symptoms before admission to the hospital. All blood samples were obtained before initiation of insulin therapy to measure blood glucose, hemoglobin A[1c], venous blood gases, serum electrolytes and ketones. During each clinic visit, the children were examined with emphasis on nutritional and growth data and their anthropometric measurements recorded. The height standard deviation score, body mass indices and growth velocity per year were calculated and recorded. Hemoglobin A[1c] was estimated every clinic visit. Hormonal evaluation for all children included measurement of free thyroxine [FT4], and IGF-1. The results included family history of autoimmune diseases, symptoms occurring within 3 months prior to diagnosis. Polyuria was the most common presenting symptom present in 87%, followed by fatigue/lethargy/malaise [53%], febrile illness [23%], nausea and vomiting [20%], and abdominal pain [15%]. Secondary nocturnal enuresis occurred in [23%]. Mumps was reported in 2 children while chicken pox was reported in 1 child 3-5 weeks before diabetes was diagnosed. The plasma glucose level was 22.3 +/- 5.6 mmol/L, it was related neither to age nor to duration of symptoms. The HbA[1c] concentration was 15.5 +/- 2.9%. Levels of HbA[1c] were correlated with serum glucose concentration. The frequency of DKA, its degree at presentation and associated biochemical changes is presented. As regards the progress of the disease, insulin was withdrawn in two of the 60 children in whom remission lasted 6 and 14 weeks respectively. The risk of readmission was 0.2 per patient during the first year and 0.12 during the second year. Re-admission for poor glycemic control was more common in young children. Children with good glycemic control had better linear growth compared to those with bad glycemic control. With follow up, 3 children developed hypothyroidism, 1 developed vitiligo, 1 developed chronic active hepatitis, 2 developed significant proteinuria. One child died of hepatorenal failure at the age of 5 years. In conclusion, children diagnosed as having diabetes below 5 years seem to present acutely and have highest risk of developing hypoglycemia during the first year after diagnosis

Prevalence and significance of acanthosis nigricans in children and adolescents

Acanthosis nigricans [AN] develops commonly in obese adults, yet its prevalence and metabolic significance in children and adolescents have not been determined. To address these issues, 100 obese children and adolescents enrolled in the obesity clinic at the Royal Hospital, Muscat, Oman, were chosen at random and examined. AN was observed in 43 of the study children [43%]. The frequency and severity of AN increased significantly with increasing body mass index [BMI] in these children. Twenty patients with obesity and AN and 20 age-matched nonacanthotic obese children, randomly selected from the study children, were investigated. Their oral glucose tolerance and serum C-peptide responses to IV glucagon were evaluated.- Circulating concentrations of free thyroxine [FT4], TSH, basal and ACTH-stimulated cortisol, testosterone, leutinizing hormone[LH], follicle-stimulating hormone [FSH], and prolactin were measured by radioimmunoassay [RIA]. Children with AN exhibited higher basal and glucagon-stimulated C-peptide concentrations than the nonacanthotic obese group. Two hours after the oral load of glucose [1.75 g/kg], serum glucose concentration [6.3 +/- 1.4 mmol/L] was higher in the acanthotic group versus the nonacanthotic group [5.2 +/- 0.8 mmol/L]. Impaired glucose tolerance was detected in two children with AN [10%], and in none of the nonacanthotic controls. Hypothyroidism was diagnosed in two [10%] children with AN [TSH = 109 and 18 mIU/mL and FT 4 = 4.6 and 13.5 pmol/L respectively], while all the nonacanthotic children were euthyroid. Serum testosterone concentration was insignificantly lower in the acanthotic group [6.5 +/- 3.9 ng/dL] versus the nonacanthotic children [8.3 +/- 4.5 ng/dL]. Basal serum LH, FSH and prolactin concentrations and basal and ACTH-stimulated cortisol levels did not differ between the two study groups. Plasma triglyceride concentration was significantly higher in the acanthotic group [1.43 +/- 0.5 mmol/L] versus the nonacanthotic group [1.05 +/- 0.45 mmol/L], and was correlated significantly with BMI [r = 0.466, P < 0.05]. In conclusion, obesity is a significant risk factor for the development of AN in children. AN is a reliable skin marker of hyperinsulinemia in obese children and adolescents. The prevalence of impaired glucose tolerance [10%] and primary hypothyroidism [10%] appears to be higher in obese acanthotic children than in those without AN

Evaluation of therapeutic value of human chorionic gonadotrophin [HCG] in children with unilateral undescended testes [Cryptorchidism]

The present study was undertaken to determine the therapeutic effect of HCG [1500 U/M, IM, twice weekly 4 weeks] on 50 children with unilateral cryptorchidism, inguinal [n=36], high scrotal [n=6] and intraabdominal [n=8]. Testicular descent has been accomplished, completely in 5.6% and partially in 22.2%, of children with unilateral inguinal testis. Eighty three% of high scrotal testes descended to the bottom of scrotum after HCG therapy, whereas none of the intraabdominal testes did change their position after hormonal therapy. Significant enlargement of testicular and penile size was observed in all the study children after HCG treatment. This finding proved normal Lydig cell function and sensitive androgenic receptors in patients with unilateral crytorchidism

Defective arginine-induced insulin secretion in children with nutritional rickets

We evaluated arginine-induced insulin and growth hormone secretion in 10 children with proved vitamin-D-deficiency rickets and compared these values with those of eight normal age-matched control children. Thirty minutes after an intravenous infection of arginine hydrochloride [10% solution, 0.5 g/kg], the mean serum insulin level was 159.3 +/- 122.7 pmol/L for the rachitic children, which was significantly lower than that for the normal children. Neither the fasting insulin level nor any others after the arginine infusion differed significantly from those the control children. There were no significant differences in the fasting or the arginine-stimulated growth hormone levels between the rachitic and control children. The concentrations of insulinlike growth factor I and II did not differ between the two groups. These data suggest that there is not a major role for circulating growth hormone [and by implication somatomedin] on bone mineralization

Serum cortisol concentrations in children with protein energy malnutrition

We measured basal corisol level in 15 children with kwashiorkor, 15 children marasmic kwashiorkor, and 21 children with marasmus, before and after nutritional rehabilitation, as well as in 10 underweight and 8 normal Egyptian children. Cortisol levels are elevated in kwashiorkor, maramic kwashiorkor, and maramus groups, but returned to normal after nutritional rehabilitation. No differences in basal cortisol were detected between the three severely malnourished groups before refeeding. The percent body weight deficit and the calculated muscle diameter correlated significantly with cortisol levels in all the children before nutritional rehabilitation, but there was no significant correlation between serum albumin or glucose and cortisol levels. Increased cortisol levels appeared not to be related to the type of protein-energy malnutrition, but represent an attempt of the rganism to adapt to decreased diatary protein and/or energy supply through breakdown of muscle protein to provide the liver with the necessary amino acids for gluconeogenesis and albumin synthesis