27
Endocrine and metabolic disorders
Chapter map
Insulin-dependent diabetes mellitus is the most important endocrine condition in children. Other hormonal conditions, and inborn errors of metabolism, are relatively uncommon, and most are extremely rare. In general, they are characterized by their effects upon a child’s growth and development. It is important, therefore, to include endocrine and metabolic disorders in the differential diagnosis of a wide variety of clinical presentations. Early recognition and appropriate management may confer long-lasting benefit.
27.1.1 Type 1 diabetes (insulin dependent)
27.1.5 Type 2 diabetes (insulin-resistant diabetes)
27.3.1 Congenital hypothyroidism
27.3.2 Juvenile hypothyroidism
27.3.3 Hyperthyroidism and goitre
27.4.1 Congenital adrenal hyperplasia
27.5 Growth hormone deficiency
27.7 Inborn errors of metabolism
27.7.2 Medium Chain Acyl-CoA Dehydrogenase deficiency (MCADD)
27.1 Diabetes mellitus
27.1.1 Type 1 diabetes (insulin dependent)
Diabetes in children is nearly always Type 1. It has increased in frequency, now affecting around 1: 500 children. Most present after the age of 2 years. Often there is no family history, although other members of the family may have diabetes or other autoimmune disease. Children who are genetically predisposed develop diabetes following an unknown trigger. Possible triggers include viral infection.
27.1.2 Presentation
Presentation of diabetes- Polydipsia/thirst
- Polyuria/enuresis
- Weight loss
- Dehydration/vomiting
- Ketoacidosis
- Altered consciousness/coma.
At presentation, children are often ill and have a markedly elevated blood glucose level (>14 mmol/L), together with glycosuria and ketonuria. The glucose tolerance test is never needed to diagnose type 1 diabetes, but can be used to assess glucose control in other conditions. The HbA1C (percentage of glycosylated haemoglobin) is useful for diagnosis and monitoring, since it reflects recent blood sugar levels.
RESOURCE27.1.3 Management
TREATMENT Principles of acute management of ketoacidosis- Rehydration
- Control of blood sugar with insulin
- Care of electrolyte status, especially potassium.
Longer term management involves principally the introduction of dietary regulation and insulin therapy, and an intensive programme of support and education for the child and their family.
TREATMENT Management of diabetes mellitus- Regulated carbohydrate intake matching growth needs and activity
- Insulin regime designed around child and family’s needs and abilities
- Continuous monitoring of control – usually home blood glucose.
- Understanding of blood sugar control
- Good control reduces likelihood of complications
- Diabetes management can usually be adjusted around lifestyle
- How to adjust diet/insulin with activity/illness
- Recognition, importance and treatment of hypoglycaemia (‘hypos’).
PRACTICE POINTThere are a wide variety of different insulin preparations. The traditional regime of twice-daily, mixed short- and medium-acting insulin is being increasingly replaced by multi-dose regimes delivered by pen device (Figure 28.2), or continuous variable insulin delivered subcutaneously by body-worn pump. These bring greater flexibility, adaptation to lifestyle and better glucose control. However they require a high level of understanding on the part of parents or older children and teenagers. Insulin is injected subcutaneously, rotating round sites including the arms, thighs and abdomen.
PRACTICE POINTEach child will get different early symptoms of hypoglycaemia. It is essential that they understand the importance of recognizing a ‘hypo’. The child may wear a medical alert bracelet. The child, family, teachers and others caring for the child need to know what to do. If a child is unable to eat or drink, a glucose gel (Hypostop), sugar or jam can be smeared onto the buccal mucosa and is rapidly absorbed. The child’s parents should know how to administer subcutaneous or intramuscular glucagon in emergencies.
Diabetic control can be difficult in children. Loss of control during infection, and difficulty in maintaining tight control during periods of rapid growth are characteristic.
27.1.4 Family support
All children with diabetes, and their families, require intensive support. Children should be encouraged, as soon as they are old enough, to take responsibility for their diabetes. Membership of Diabetes UK is a great asset. Self-help groups and the outreach children’s nursing service can all help the child and the family to take diabetes in their stride, so that it is not a major interference with the normal way of life. Long-term complications, such as retinopathy and renal disease, are rare during childhood, but good glycaemic control is important to reduce their incidence in later life.
Chronic illness does not fit well into the teenage years. This is well known in diabetes, when depression and psychological disturbance become more common. The discipline of diabetes is particularly irksome at this age. Teenagers should be encouraged to become more independent of their parents, managing their own diabetes. Unfortunately they may frequently break dietary rules, cheat on tests or omit insulin.
27.1.5 Type 2 diabetes (insulin-resistant diabetes)
Previously very rare, this form of diabetes is increasingly recognized. The epidemic of childhood obesity largely explains the increase in type 2 diabetes. Family history is often positive. Unusual specific genetic forms are found. In the obese child with type 2 diabetes, weight loss and exercise are key. Oral drug treatments are used, and insulin depending upon control. Good control is important to reduce the risk of long-term complications.
27.2 Hypoglycaemia
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