Treatment And Follow-up Of A Type A Insulin Resistance Syndrome

Insulin resistance is a condition that the functions of insulin decrease because of some disorders in its process. It has differential manifestations in different kinds of disorders and diseases. Type A insulin resistance syndrome is a rare disorder characterized by severe insulin resistance due to defects in signaling through the insulin receptor. It normally affects adolescent females with the phenotype of hyperinsulinism, acanthosis nigricans, virilisation, and ovary dysfunction, such as menstrual disorder, amenorrhea.In this work, an 15-year-old female patient with severe insulin resistance and hyperandrogenism was diagnosed type A insulin resistance after a thorough physical examination and checkup, then a treatment of oral drugs was offeredand a follow-up of clinical manifestations and physical symptoms was executed, in which metabolism, insulin resistance and gonadal hormones were also monitored. It is aimed to increase the recognitions in diagnosis and therapy of Type A insulin resistance syndrome.1.examination and diagnosisA general physical examination and medical history were executed, to acquaint a comprehensive recognition of the circumstance of metabolism and endocrinology, followed by a series of checkups and chemical examinations: routine ECG, measurement of bone age, gynaecological ultrasonic examination, thyroid function determination, autoantibody series and immunoglobulin series determination, indirect immune histochemistry of gastrocnemius, biochemistry measurement, determination of gonadal hormones(FSH, LH, PRL, T, P,E2), OGTT and insulin/C-peptide release test.2.treatment and follow-upMetformin and spironolactone were administrated in the beginning, then pioglitazone was added after the second reexamination because of insulin sensitivity declined. In every reexamination, clinical manifestations and symptoms were observed, some chemical examinations and checks were reviewed, such as gynaecological ultrasonic examination, biochemistry measurement, determination of gonadal hormones, OGTT and insulin/C-peptide release test.Results: Type A insulin resistance syndrome was diagnosed after a thorough checkup. In the first phase when metformin (0.25g tid) and spironolactone(20mg tid) was administrated, blood glucose, insulin and serum androgen level were all decreased, with insulin sensitivity increased. While in the second phase that administrative treatment was continued, the level of blood sugar, serum insulin and androgen was raised again, and insulin sensitivity was descended. Then pioglitazone(15mg/dag) was added into the treatment plan in the third phase, the level of blood sugar, serum insulin and androgen was declined again, and insulin sensitivity was improved. In the third month after the treatment, menstruation was reoccurred at a irregular intervals of 22～35 days, but in the 11th month, the intervals recover to 28～30 days, still with a low volume. Hypertrichosis were clearly improved in the observation after the treatment. Conclusion: 1.Type A insulin resistance syndrome should be considered when a adolescent female is found with hyperinsulinism, hyperandrogenism and acanthosis nigricans. Additional examinations must be executed to exclude other conditions of severe insulin resistance, function test of insulin receptor and gene test are strongly recommended if a good experimental condition obtained. 2. Metformin was useful in treatment of type A insulin resistance syndrome in a short period, but, a prolonged therapy did not bring a further benefit in improving the circumstance of severe insulin resistance. The metformin therapy has its limits. 3. Pioglitazone actually attenuated insulin resistance and ameliorated masculinization of type A insulin resistance syndrome, a further research is needed to observe the long-period effect.