Predictors of glycemic control in children with Type 1 diabetes mellitus in Assiut-Egypt

Background : Type 1 diabetes mellitus (T1DM) may lead to severe long-term health consequences, such as renal failure, blindness, as well as heart and cerebrovascular disease. Although a direct relationship between blood glucose control and diabetes complications remains to be established beyond doubt, most diabetologists aim to achieve the best possible glucose control in their patients with T1DM. The aim of this study was to detect the predictors of glycemic control among children with T1DM in Assiut Governorate-Egypt. Materials and Methods : We enrolled 415 children aged 2 to 18 years with type 1 diabetes of >1-year duration. They were subjected to full history including demographic factors and disease-related factors. Examination was done with determination of the body mass index, and assessment of stage of maturity. Investigations included hemoglobin A1c (HbA1c) and lipid profile. Patients with HbA1c above the recommended values for age by the American Diabetes Association were considered as poor glycemic control group. Results : Of the studied cases, 190 cases (45.8%) were of poor glycemic control. Patients with poor control had significantly higher mean age (16.83 ± 3.3 vs 9.77 ± 3.7, P<0.000). Girls aged 15 years or more had significantly higher prevalence of poor glycemic control than males of the same age group. As regard the disease-related factors, patients with poor control had significantly longer duration of disease (7.94 ± 2.6 vs 2.40 ± 2.0, P<0.000) and were older in age at onset of disease. Insulin regimen which consists of basal bolus insulin plus three injections of regular insulin was associated with more frequency of good glycemic control than other regimens. Patients with poor control had significantly higher mean of cholesterol, triglyceride (TG), high-density lipoprotein cholesterol, and low-density lipoprotein cholesterol than patients with good control. Adjusting for other variables, age of the patients, duration of disease, and serum TG level were significant independent risk factors of poor glycemic control. Conclusions : This study concluded that children more than 15 years, duration of disease more than 5 years, and high serum TG level are the predictors of poor glycemic control of children with T1DM in Assiut - Egypt. Pediatricians need to be aware of factors associated with poor glycemic control in children with T1DM, so that more effective measures can be implemented to prevent deterioration in diabetes control

X-linked congenital adrenal hypoplasia associated with hypospadias in an Egyptian baby: a case report

<p>Abstract</p> <p>Introduction</p> <p>X-linked congenital adrenal hypoplasia is a rare developmental disorder of the human adrenal cortex and is caused by deletion or mutation of the <it>dosage-sensitive sex reversal adrenal hypoplasia congenita critical region of the X chromosome, gene 1</it> (<it>DAX-1</it>) gene. Most affected children present with failure to thrive, salt wasting and hypoglycemic convulsions in the first months of life. Hypospadias affects approximately one in 250 live male births. Mutations in the <it>mastermind-like domain-containing 1</it> (<it>MAMLD1</it>) gene have been implicated as one of the causes of hypospadias in children. To the best of our knowledge, an association between congenital adrenal hypoplasia due to a <it>DAX-1</it> mutation and hypospadias due to mutation of the <it>MAMLD1</it> gene has not previously been reported in the literature.</p> <p>Case presentation</p> <p>A 35-day-old male Egyptian baby was referred to our institution for the evaluation of a two-week history of recurrent vomiting associated with electrolyte imbalance. On examination, our patient was found to have hypotension and dehydration. A genital examination showed distal penile hypospadias with chordee and normal testes. He had hyponatremia, hyperkalemia, hypoglycemia and metabolic acidosis. Endocrinological investigations revealed low levels of cortisol, 17-hydroxyprogesterone and aldosterone, with a high level of adrenocorticotrophic hormone. A provisional diagnosis of congenital adrenal hypoplasia associated with hypospadias was made. A molecular genetics study confirmed the diagnosis of X-linked congenital adrenal hypoplasia due to <it>DAX-1</it> mutations and hypospadias due to <it>MAMLD1</it> mutation. He was started on hydrocortisone and fludrocortisone treatment. After three weeks of treatment, his symptoms improved and his blood sugar, sodium, potassium and cortisol levels normalized.</p> <p>Conclusions</p> <p>We report the case of an Egyptian baby with an association of congenital adrenal hypoplasia due to <it>DAX-1</it> mutation and hypospadias due to <it>MAMLD1</it> mutation. Early diagnosis of this association and determining its optimal treatment are vital in helping to avoid its fatal course.</p