Introduction Cushing's Disease (CD) denotes a pathologic endogenous hypercortisolism secondary to excessive adrenocorticotropic hormone (ACTH) production. Cushing Disease is a rare condition with an estimated incidence of approximately 1.2-1.4 new cases / 1. 000. 000 per year. Although surgical intervention is the gold standard in the management of CD, many patients may present persistence or recurrence of disease despite surgical intervention, resulting in significant deterioration of their quality of life (QoL). For those in whom pituitary surgery is not an option or has not been curative, medical therapies have been developed. One of these newer treatments is the drug Osilodrostat, an inhibitor of the enzyme 11-beta-hydroxylase, responsible for the final step of cortisol biosynthesis in the adrenal glands. Case Discussion Case of a 21 year-old female patient with history of Cushing's Disease status post-transsphenoidal surgery (TSS) in 2017, obesity and oligomenorrhea who presented to our clinics showing signs and symptoms of hypercortisolism despite previously documented post-surgical remission in 2017. Patient reported recent development of acne, weight gain, round face, elevated blood pressure and mood changes. At the moment of evaluation, her most recent MRI showed post-surgical changes without a discrete lesion. Work-up was compatible with recurrence of Cushing's with an ACTH at 75pg/mL (nl, 10-60pg/mL), urinary free cortisol (UFC) at 1,122mcg/24h (nl, 4-50mcg/24h) and serum morning cortisol at 24. 0mcg/dL (nl, 5-20mcg/dL). In view of a negative MRI for a pituitary lesion, a Bilateral Inferior Petrosal Sinus Sampling (BIPSS) was performed. The BIPSS procedure revealed a pituitary source with a right pituitary lateralization suggestive of a right ACTH-producing tumor. The patient was consulted to Neurosurgery Service, but while waiting for repeat surgery, control of hypercortisolism was needed. The patient was started on Osilodrostat 2mg twice a day and was able to achieve normalization of UFC to 4mcg/24h, serum morning cortisol 5mcg/dL and late-night salivary cortisol (LNSC) of 30ng/dl (<90ng/dL) with significant improvement in her QoL, depression and clinical manifestations. The patient tolerated therapy well. Conclusion Cushing's Disease is a complex condition with potential serious complications if untreated. Even though TSS is the first line therapy, approximately 25% of patients show persistence of disease, and a similar proportion may experience recurrence. When surgery fails, medical treatment such as Osilodrostat, can temporarily suppress excessive cortisol production and ameliorate its clinical manifestations improving patients’ QoL while more definitive therapy is established. Presentation: No date and time listed
Introduction Primary hyperparathyroidism (HPT) is characterized by an overproduction of parathyroid hormone (PTH) by a parathyroid adenoma, parathyroid hyperplasia, or parathyroid carcinoma classically causing hypercalcemia. Incidence of this pathology during pregnancy is very low (1%), as the great majority of cases are diagnosed in adults >45 years old. When present,it can be associated with detrimental maternal and fetal outcomes. Complications may include maternal nephrolithiasis, hyperemesis, and pancreatitis, along with miscarriage, fetal demise, low birth weight, neonatal hypocalcemia, and tetany. Considering the incidence of HPT in pregnancy is uncommon, there is limited data regarding diagnostic and treatment options for these patients. Clinical Case This is the case of a 28-year-old pregnant female at 12 weeks gestational age (WGA) who had a history of Type 1 Diabetes Mellitus. She was evaluated regarding metabolic control during pregnancy, however, was incidentally found with hypercalcemia. She denied any history of fractures, abdominal discomfort, bone pains or nephrolithiasis; she also had no pertinent family history associated with hypercalcemia. Laboratories showed a calcium level of 14.9 md/dL (nl, 8.6-10.2) with a phosphorus level of 1.9 mg/dL (nl, 2.5-4.5), which raised the suspicion of HPT. Further workup demonstrated high parathyroid hormone (PTH) 245 pg/mL (nl, 18.4-80.1), vitamin D-25-OH 24.7 ng/mL (nl, 30-100) and 24hr urine calcium collection of 402.5 mg/24hr (nl, 100-300); the latter excluding the possibility of familial hypocalciuric hypercalcemia. Patient was managed conservatively with IV hydration resulting in mild improvement of calcium to 12.4 mg/dL. Thyroid/Neck ultrasound revealed a thyroid gland with normal homogeneous texture and no distinct focal vascularized structure to suggest parathyroid origin. Other diagnostic imaging for localization of parathyroid disease with technetium-99m methoxyisobutylisonitrile parathyroid scintigraphy or single-photon emission Computed Tomography are not recommended in pregnancy due to insufficient safety data and radiation risk. After discussion with a multidisciplinary team including endocrine surgeons, endocrinologists, and obstetricians, an exploratory neck surgery for possible parathyroidectomy was recommended. She underwent surgery at 13 WGA where a left superior adenoma was identified and completely removed without complications. Intraoperative PTH levels revealed a pre-excision PTH of 342 pg/mL (18.4-80.1), post-excision PTH at 5 minutes of 76 pg/mL, and at 10 minutes of 50 ng/mL, suggestive of successful surgical removal. Pathology report was consistent with left superior parathyroid adenoma. Both calcium and phosphorus levels normalized post-surgery [Ca 9.6 mg/dL (nl, 8.6-10.2) and 2.7 mg/dL (2.5-4.5)]. Conclusion This is an unusual case of a patient with asymptomatic severe hypercalcemia associated to HPT diagnosed during pregnancy. In this patient conservative medical treatment proved to be unsuccessful. Parathyroidectomy early on the 2 nd trimester was advantageous to avoid both maternal and fetal complications during pregnancy. Presentation: No date and time listed
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