Research Study for Children With Salt Wasting Congenital Adrenal Hyperplasia
Condition: Adrenal Hyperplasia, Congenital
MeSH terms: Adrenal Hyperplasia, Congenital; Adrenogenital Syndrome; Adrenocortical Hyperfunction; Hyperplasia
Study type: Interventional

Summary: 
      The purpose of this study is to develop a more physiological approach to the management of
      children and adolescents with salt wasting Congenital Adrenal Hyperplasia.

      We will administer the glucocorticosteroid via insulin infusion pump to see whether this
      treatment will improve the serum hormone concentrations.
    

Detailed description: 
      The adrenal gland is a small organ of the body. It produces very important chemicals called
      hormones. One of these hormones, cortisol (the stress hormone) helps the body fight diseases.
      The other hormone is the aldosterone helps to maintain the normal amount of salt and water in
      the body. The third type of hormones are the androgens or male hormones, which cause some of
      the changes during puberty, like the growth of body hair and pimples.

      The salt wasting Congenital Adrenal Hyperplasia or CAH disease is a disease of the adrenal
      gland. Patients with this disease cannot make cortisol or the aldosterone. As a result, their
      body cannot fight diseases and cannot keep normal amounts of salt and water in the body. At
      the same time, the gland makes too much of the male hormones, which is bad for the body
      because too much male hormone slows down growth, increases the growth of body hair, and
      causes pimples and abnormal period in girls.

      Patients with this disease have to take medications every day. However, the treatment does
      not work very well, because usually the patients do not have the right amount of hormone in
      their body. Usually the body gets too much hormone right after taking the pills. A couple of
      hours later the body has too little of the hormones, because in the meantime the body gets
      rid of the medication.The healthy adrenal gland makes the hormones throughout the day in
      different amounts. The patients with this disease take the medication only a couple of times
      a day. They take the Florinef tablet once a day and the Cortisol tablet two or three times a
      day. The treatment that we use today by mouth cannot copy the natural hormone productions of
      the adrenal gland. Because of this it does not make much of a difference in the patient's
      life.

      We would like to improve the treatment and find out the effect of a new treatment. In this
      study we will try to imitate the body's normal hormone production and will give the
      medication via an insulin pump to see if this treatment method will decrease the male
      hormones in the blood. This study will help us to develop a new and better treatment for
      children and adolescents.
    

Intervention name(s): Hydrocortisone sodium acetate
Intervention type(s): Drug
Intervention description(s): Subcutaneous administration of medication via insulin pump
Other intervention name(s): Solu-Cortef injection
Intervention MeSH terms: Hydrocortisone; Hydrocortisone hemisuccinate
Intervention model: Single Group Assignment
Masking: None (Open Label)
Primary purpose: Treatment
Keywords: Salt Wasting Congenital Adrenal Hyperplasia; Subcutaneous Hydrocortisone