Abstract
J.R. is a 17-year-old Hispanic male with gigantism, large pituitary macroadenoma, left maxillary mass, and features of acromegaly. Referred by PCP (Primary Care Provider) for rapid growth at age 14 8/12. Preclinic magnetic resonance imaging (MRI) revealed macroadenoma; labs: GH, 81.6 ng/ml; IGF-1, 637 ng/ml; IGF (Insulin Like Growth Factor)-BP3, 6.6 mg/L; prolactin, 10.3 ng/ml. The thyroid function tests, testosterone, and LH/FSH (Luteinizing Hormone/Follicle stimulating hormone)were normal. Birth weight was 6 lb 7 oz; C-section for fetal distress. Mother reports normal growth until age 9; she then noticed accelerated growth. Orthodontist noted dental/jaw abnormalities. No family history of abnormal stature, pubertal or thyroid problems. PCP referred patient for neurology and endocrine evaluations. Coarse, asymmetrical facies; prominent left maxilla; prognathia; and noticeable overbite. Height 186.5 cm, >97 percentile (11 cm > predicted mid-parental height range 167.9–177.9 cm); weight 66.8 kg, 84 percentile; arm span 194 cm. Thyroid gland unremarkable. Normal external genitalia—Tanner 2 pubic hair; Tanner 3 descended testes. Café au lait spots noted on trunk. Patient underwent partial tumor resection at age 14 9/12; postsurgery IGF-1 remained elevated 746 ng/ml with normal IGF-BP3 4.9 mg/L. Genetic testing for McCune-Albright Syndrome was normal. Subsequent visits included laboratory monitoring, height/weight screening, bone age films, MRIs, and oral glucose tolerance test (OGTT) to monitor progression. Upon diagnosing adrenal insufficiency and low testosterone, daily oral hydrocortisone and monthly Intramuscular (IM) testosterone were prescribed at age 16 7/12. OGTT at age 16 7/12 revealed lack of GH suppression; monthly IM octreotide was initiated. Despite octreotide dose increases during the treatment course, GH levels remained elevated during OGTT. Remaining tumor's proximity to the cavernous sinus posed risks for brain surgery. J.R. was evaluated by NIH at age 17 1/12; no other metabolic cause for excessive GH secretion was found; repeat tumor resection with biopsy of left maxilla was recommended. Patient underwent recommended resection/biopsy at age 17 4/12. Random postsurgery GH levels were suppressed, but rose to 4.6 ng/ml. Radiation treatment began at age 17 8/12 to further control GH levels. The literature suggests that GH levels may be successfully suppressed by administering octreotide. Surgical intervention often follows failed medical management. Nurses play a key role in coordinating appointments, facilitating prescription refills, administering medications, reassuring caregivers, educating patients, and communicating with families.
Published Version
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