الفهرس 
introductionOnly 14 pages are availabe for public view
 |  | from | 169 |  |  |
| | | from | 169 | | |
Abstract
Hypoglycemia is a common problem in infants and children. It can cause seizures, coma, permanent brain damage and death. Several metabolic systems are activated during fasting to prevent hypoglycemia. Thus, hypoglycemia can be viewed as a failure of one these fasting systems or an abnormality in one of the hormones that controls these systems.
Diagnosis of causes of hypoglycemia requires a good knowledge of the complex mechanisms which maintain blood glucose concentration between the narrow range of 2.5 and 6.6 mmol/l (45 – 118 mg/dl) at the fasting or fed state. Hypoglycemic disorders can be categorized -based on the fasting system or hormone involved- into defects in glycogenolysis, defects in gluconeogenesis, fatty acid oxidation disorders, defects in ketogenesis, counterregulatory hormone defects, and hyperinsulinism.
In our series, hyperinsulinism is the most frequent cause of persistent hypoglycemia in infancy and childhood. In hyperinsulinism, excess insulin inhibits all the fasting systems, including glycogenolysis, proteinolysis, gluconeogenesis, lipolysis, and ketogenesis. In addition, insulin continues to promote glucose uptake by the liver and muscle. As a result, children with hyperinsulinism can become hypoglycemic fairly rapidly and may need large amounts of carbohydrates to maintain a normal blood glucose level. Early medical intervention is necessary to avoid neurological damage.
Because the lack of epidemiological data of diseases causing hypoglycemia in developing countries, this work aimed to identify clinical characters, laboratory parameters and treatment outcomes of hypoglycemic disorders especially hyperinsulinism in Egyptian children.
This is a descriptive study included 40 non diabetic patients attended the Diabetes Endocrine Metabolic Pediatric Unit at Cairo University Children’s Hospital being a referral center for all Egyptian governorates.
Demographic data, clinical examinations, laboratory and radiological investigations and treatment options are collected from the patients and analyzed.
The results of this study showed that:
Hormonal causes of hypoglycemia in infants and children account for 67.5% of our cases. However, non hormonal causes of hypoglycemia represent 32.5%.
Hyperinsulinism is by far the most common cause of hypoglycemia in infants and children, corresponding to more than 80% of hormonal patients. However, other hormonal causes of hypoglycemia represent 18.5% of hormonal group.
Persistent hyperinsulinemic patients are more than those of transient hyperinsulinism representing 64% and 36% of hyperinsulinemic cases respectively.
Patients with hormonal causes of hypoglycemia showed significant younger age of onset and at diagnosis than that in non hormonal group.
Persistent hyperinsulinemic cases had significant younger age of onset than that in transient hyperinsulinemic cases.
First and second degree cousin marriages were found in parents of 67% of hormonal group and in parents of 57% of persistent hyperinsulinemic patients.
Hypoglycemic convulsions were the most common presentation followed by different degrees of disturbed conscious level then vomiting frequency.
The mean fasting tolerance time of hormonal cases (3 hours) was significantly lower than that in non hormonal group (12 hours). However, there is no significant difference between transient and persistent hyperinsulinemic patients regarding fasting tolerance time.
Delayed motor and mental development were more frequent in non hormonal than hormonal cases and in persistent than transient hyperinsulinemic cases but this difference did not reach a significant value.
About 57% of persistent hyperinsulinemic patients had delayed mental development while 86% showed poor response to treatment and 7% died (causes of death mentioned before in chapter of case reports).
Responding to treatment in transient hyperinsulinemic patients was significantly better than that in persistent hyperinsulinemic patients.
Persistent hyperinsulinaemic hypoglycaemia remains a major diagnostic and management challenge. Early suspicion and recognition is critical with definitive investigation and medical therapy to avoid hypoglycaemia.
Although medical management is very laborious for the family and physician, it should be applied until euglycemia is accomplished. Moreover, the early diagnosis of persistent hyperinsulinaemic hypoglycaemia and the successful hypoglycemic control are very necessary to prevent permanent neurological sequelae.