46,XX SRY-Positive Male Syndrome Presenting with Primary Hypogonadism in the Setting of Scleroderma

ObjectiveTo describe a case of SRY gene translocation in a man with scleroderma presenting with primary hypogonadism.MethodsWe present the clinical, physical, laboratory, and pathologic findings of the study patient and discuss the cytogenetic analysis and the cause of the sexual dysfunction. Relevant literature is reviewed.ResultsA 35-year-old man with a recent diagnosis of diffuse cutaneous sclerosis was referred by his rheumatologist because of a low testosterone level. His medical history was notable for right cryptorchidism corrected after birth. He had no history of sexual activity, but reported normal erectile function before his current presentation. Physical examination findings were remarkable for a height of 157.5 cm; weight of 72.7 kg; extensive, diffuse thickening of the skin; mild gynecomastia; little axillary and pubic hair; and soft testes (1-2 mL bilaterally). Initial laboratory testing revealed the following values: follicle-stimulating hormone, 22.1 mIU/mL (reference range, 1.4-18.1 mIU/mL); luteinizing hormone, 19.7 mIU/mL (reference range, 1.5-9.3 mIU/mL); total testosterone, 25 ng/dL (reference range, 241-827 ng/dL); and free direct testosterone, 0.8 pg/mL (reference range, 8.7-25.1 pg/mL). Laboratory test results were consistent with primary hypogonadism. A urologist performed testicular biopsy, which showed severe testicular atrophy with absent spermatogenesis. Primary hypogonadism due to Klinefelter syndrome or testicular fibrosis secondary to scleroderma was suspected. Karyotype analysis showed a 46,XX karyotype, and fluorescence in situ hybridization was consistent with a 46,XX,Xp22.3(SRY +) gene translocation. After a normal prostate-specific antigen level was documented, testosterone replacement therapy was initiated, and he was referred for genetic counseling.ConclusionsThe 46,XX SRY-positive male syndrome is rare. Adult diagnosis can be challenging because of normal sexual development. Scleroderma, which rarely can occur in Klinefelter-type syndromes, further complicated the diagnosis in this case. (Endocr Pract. 2011;17:95-98)     

A male patient from the West Indies with invasive ductal carcinoma in the right breast: A case report and literature review

Background: Men with breast carcinoma have a poor prognosis, especially in the younger age group (30–40 years of age), because most breast enlargements in young men are dismissed as gynecomastia, resulting in an unnecessary delay in treatment.Objective: We describe the case of a young male patient with invasive ductal breast carcinoma.Case summary: In November 2005, a 30-year-old Afro-Caribbean man presented at St. Clair Medical Centre in Port of Spain, Trinidad, with a painless lump in the right breast. Diagnostic findings revealed that the patient had stage II invasive ductal carcinoma, for which he underwent a mastectomy with total axillary clearance.Conclusion: Surgeons investigating unilateral breast swellings in young males should not dismiss them simply as gynecomastia, and should be wary of cancer.     

Invasive Breast Cancer After Initiation of Testosterone Replacement Therapy in A Man—A Warning To Endocrinologists

ObjectiveTo alert fellow endocrinologists of a rare side effect of testosterone therapy, for which men with hypogonadism must receive appropriate counseling and monitoring.MethodsWe present clinical features, laboratory data, and histopathologic findings in a man with hypogonadism who received testosterone replacement therapy.ResultsA 61-year-old man was referred to an endocrinologist after presenting to his general practitioner with erectile dysfunction and low libido. He had no history of hypothalamic, pituitary, or testicular disorders. There were no other illnesses or medications to account for low testosterone levels. Physical examination was unremarkable. There was no family history of malignant disease. Biochemical investigations confirmed the presence of primary hypogonadism, for which no cause (including Klinefelter syndrome) was identified. Testosterone therapy was initiated to improve sexual function and preserve bone density. Five weeks later, the patient returned to his general practitioner, complaining of a gradually enlarging lump in his right breast. When biopsy showed breast cancer, testosterone therapy was discontinued. Right mastectomy and axillary node clearance were performed. Further histologic examination revealed estrogen receptor-positive, invasive carcinoma, without nodal involvement. The patient remains on tamoxifen therapy and is undergoing follow-up in the breast clinic. After 6 months of treatment, estradiol levels were undetectable, and testosterone levels remained low.ConclusionAlthough breast cancer has been described in men with hypogonadism receiving long-term testosterone replacement therapy, to our knowledge this is the first report of breast cancer becoming clinically manifest after a short duration (5 weeks) of testosterone treatment. This case should remind clinicians that men receiving testosterone therapy should be warned of the risk of not only prostate cancer but also breast cancer. Patient self-monitoring and breast examinations by the attending physician are recommended. (Endocr Pract. 2008;14: 201-203)     

Humoral Hypercalcemia of Malignancy in a Patient with Large Cell Carcinoma of the Lung: Report of Case and Review of Literature

ObjectiveTo report a case of hypercalcemia associated with parathyroid hormone-related protein (PTHrP) in large cell carcinoma of the lung.MethodsWe present a case of PTHrP-mediated hypercalcemia in a patient with a large cell carcinoma of the lung and review the related literature.ResultsA 43-year-old African American man required medical attention because of lethargy, confusion, and poor oral intake. He had bullous emphysema attributable to a 50-pack-year smoking history. On physical examination, vital signs were normal, he was oriented to place and person but not time, and he had cachexia. Breath sounds were decreased in the left lower lung field. Findings on cardiac and abdominal examination were normal. Results of laboratory studies (and corresponding reference ranges) were as follows: calcium 12.1 mg/dL (8.5 to 10.5), albumin 2.0 g/dL (3.5 to 5.0), phosphorus 2 mg/dL (2.5 to 4.5), alkaline phosphatase 68 U/L (40 to 150), intact parathyroid hormone 5 pg/mL (10 to 60), PTHrP 7.0 pmol/L (0.0 to 1.5), 1,25-dihydroxyvitamin D 20.8 pg/mL (25.1 to 66.1), and 25-hydroxyvitamin D 3.7 ng/mL (10 to 60). Computed tomographic scans of the chest showed a large complex lesion in the left lower hemithorax, a small right pleural effusion, and extensive pulmonary emphysema bilaterally. Open lung biopsy revealed a large cell undifferentiated carcinoma. Abdominal and pelvic computed tomographic scans showed no evidence of metastatic involvement. A bone scan was negative for osseous metastatic lesions.ConclusionAlthough the finding is rare, patients with large cell carcinoma of the lung and hypercalcemia may have humoral hypercalcemia mediated by PTHrP. (Endocr Pract. 2007;13:389-395)     

Iatrogenic Osteoporosis,Bilateral Hip Osteonecrosis,and Secondary Adrenal Suppression in an Hiv-Infected Man Receiving Inhaled Corticosteroids and Ritonavir-Boosted Highly Active Antiretroviral Therapy

ObjectiveTo report the first case of severe osteoporosis associated with a vertebral pathologic fracture and osteonecrosis of femoral heads in an HIV-infected man receiving inhaled corticosteroids and ritonavir-boosted antiretroviral therapy.MethodsWe describe an HIV-infected man with severe osteoporosis, bilateral hip osteonecrosis, and secondary adrenal suppression, including detailed clinical, laboratory, and radiographic data, and review the related literature.ResultsA 60-year-old man with a 15-year history of HIV infection and a medical history of long-standing bronchiectasis treated with inhaled corticosteroids and hypogonadism treated with testosterone was referred to the endocrinology clinic after experiencing an osteoporotic vertebral fracture. He was taking ritonavir-boosted antiretroviral therapy. Osteonecrosis of both hips was also diagnosed, which required total hip replacement therapy.Laboratory evaluation revealed adrenal insufficiency due to increased effect of exogenous inhaled steroids and no other secondary causes of osteoporosis. A bone densitometry study showed osteoporosis of both hips and the lumbar spine. He was treated with intravenous pamidronate. During treatment, he developed bilateral femoral fractures after minor trauma.ConclusionsGiven the potential for increased serum levels of inhaled corticosteroids in patients taking ritonavirboosted highly active antiretroviral therapy, attention must be paid to the risk of bone loss in HIV-infected patients taking inhaled corticosteroids. Prescribing calcium and vitamin D supplementation and considering early osteoporosis screening are reasonable measures for this patient population. Interaction between inhaled corticosteroids and ritonavir may increase risk of hypothalamus-pituitary-adrenal axis suppression. (Endocr Pract. 2011;17:74-78)     

Sexual Precocity in A 2-Year-Old Boy Caused by Indirect Exposure to Testosterone Cream

ObjectiveTo report a rare case of sexual precocity caused by inadvertent exposure to testosterone cream.MethodsWe report the clinical, laboratory, and radiologic findings of a boy presenting with sexual precocity; review short- and long-term consequences; and discuss preventative measures.ResultsA₂ and 7/12-year-old boy had onset of pubic hair without testicular enlargement and a period of rapid linear growth. History revealed possible topical testosterone exposure from close contact with the child’s father. On physical examination, the boy had Tanner stage II pubic hair distribution. Laboratory evaluation findings were normal for age except for the testosterone concentration, which was comparable to late-pubertal and adult male levels at 371 ng/dL (reference range, < 3-10 ng/dL for prepubertal male). Brain magnetic resonance imaging and testicular ultrasonography were normal. Skeletal age was advanced at age 4 and 6/12 years. Repeated laboratory evaluation, after the child’s father ceased testosterone use, revealed a normal testosterone concentration of 10 ng/dL. Thus, this boy’s sexual precocity was attributed to inadvertent exogenous androgen exposure.ConclusionsWhen examining a child with sexual precocity, asking about possible exposure to androgens and estrogens is important. Patients being treated with these products should be educated about the possible risks of testosterone exposure to others and ways to limit exposure. (Endocr Pract. 2008;14:1027-1030)     

Hypocalcemia and Parathyroid Function in Metastatic Prostate Cancer

ObjectiveTo report the occurrence of hypocalcemia in a patient with metastatic prostate cancer, discuss its pathogenesis, and review the related medical literature.MethodsAn 82-year-old man with a known history of prostate cancer was found to have a serum calcium level of 5.4 mg/dL during an admission to the hospital for small bowel obstruction. A thorough review of his medical history revealed a temporal relationship between the diagnosis of malignant disease and progressive hypocalcemia. A complete evaluation was performed, including laboratory and imaging studies, to ascertain the cause of the hypocalcemia.ResultsThe patient had no history of hypocalcemia before the diagnosis of, and initiation of antiandrogen therapy for, advanced prostate cancer. Serum magnesium and phosphorus levels were within normal limits. The serum calcium level responded to therapy in the hospital but remained between 5.8 and 7.1 mg/dL. The parathyroid hormone level was normal, and the 25-hydroxyvitamin D value was low. A 24-hour urine collection showed substantially reduced calcium excretion, and a whole-body bone scan revealed widespread metastatic deposits. These findings were compatible with hypocalcemia related to prostate cancer and bone metastatic lesions.ConclusionThis case serves as a reminder that hypocalcemia can be a manifestation of prostate cancer metastatic to bone. In contrast to the occurrence of secondary hyperparathyroidism in this setting, however, this patient had normal levels of parathyroid hormone. Review of similar previous reports and the causes and implications of a possible functional hypoparathyroid state are discussed. (Endocr Pract. 2005;11:254-258)     

Partial deficiency in 17-ketosteroid reductase presenting as gynecomastia

We report a 14 year-old male with severe, long-lasting gynecomastia. Baseline serum androstenedione levels were elevated compared to testosterone levels (330 ng/dl vs 28 ng/dl). In order to evaluate testosterone biosynthesis by this patient in more detail, androstenedione, testosterone, dehydroepiandrosterone (DHEA) and estradiol responses to a single dose of hCG were measured. The responses observed were different from those reported in normal males in two respects: 1) there was no immediate rise in testosterone two to four hours after the injection of hCG, and 2) levels of androstenedione and estradiol at 24, 36 and 48 hours after injection were much higher than expected. We postulate that a partial defect in testicular 17-ketosteroid reductase activity was responsible for the abnormal androstenedione to testosterone ratio in our patient. This, in turn, lead to an increased peripheral synthesis of estrogens and marked gynecomastia.     

Chemotherapy-Induced Hypocalcemia

ObjectiveTo present a unique case of transient, asymptomatic chemotherapy-induced hypocalcemia not attributable to hypomagnesemia or tumor lysis syndrome and review causes of hypocalcemia related to cancer with and without use of chemotherapy.MethodsWe present a case detailing the clinical and laboratory findings of a patient who had severe hypocalcemia during chemotherapy and discuss causes of hypocalcemia with an extensive literature review of chemotherapeutic agents associated with this biochemical abnormality.ResultsIn a 90-year-old man, hypocalcemia developed during 2 courses of chemotherapy for Hodgkin lymphoma, with partial recovery between courses and normal serum calcium 10 months after completion of treatment. Magnesium, vitamin D, and parathyroid hormone levels were low normal. There was no evidence of tumor lysis syndrome. Of the various agents administered, vinca alkaloids seemed the most likely cause. Serial testing suggested that the underlying mechanism may have been acquired, reversible hypoparathyroidism. No other similar case was found in the published literature.ConclusionThe severe hypocalcemia in our patient could not be attributed to hypomagnesemia or tumor lysis syndrome, and it was clearly associated with the timing of his chemotherapeutic regimen. Possibilities include direct parathyroid hormone suppression or alteration of calcium sensing by the chemotherapeutic drugs. Serum calcium surveillance before and during chemotherapeutic management of cancer patients may reveal more instances and provide insight into the exact mechanism of this lesser known yet striking complication. (Endocr Pract. 2010;16:284-290)     

Tiratricol-Induced Periodic Paralysis: a review of nutraceuticals affecting thyroid function

ObjectiveTo review the potential adverse effects of thyroid hormone-based nutraceuticals and describe a case of Thyrotoxic Periodic Paralysis (TPP) after abuse of a dietary supplement containing 3,5,3′-triiodothyroacetic acid (tiratricol).MethodsWe review the literature on potential dangers and therapeutic misadventures of thyroid hormonebased nutraceuticals and present the clinical, laboratory, and radiologic data of a bodybuilder in whom hypokalemic TPP developed after use of “Triax Metabolic Accelerator.”ResultsA 23-year-old white man developed lower extremity paralysis, diaphoresis, and palpitations in the setting of low serum potassium levels. Laboratory results showed suppressed thyroid-stimulating hormone, low levels of free and total thyroxine, low total triiodothyronine level, and very low 24-hour radioiodine uptake. The patient ultimately admitted to taking a supplement containing tiratricol for approximately 2 months, and hypokalemic TPP was diagnosed. He was treated with potassium supplementation and a b-adrenergic blocking agent, which completely resolved his symptoms. Results of thyroid function tests normalized or approached normal 1 week after hospitalization, and future use of dietary supplements was strongly discouraged. Despite 2 warnings by the US Food and Drug Administration, products containing tiratricol are still available for sale on the Internet.ConclusionThis report illustrates both an unusual adverse effect of a nutraceutical containing tiratricol and the importance of educating our patients about the risks versus benefits of using these widely available but loosely regulated products. (Endocr Pract. 2011;17:610-615)     

Finding the right balance between resistance and sensitivity: a review of the cardiac manifestations of the syndrome of resistance to thyroid hormone and the implications for treatment

ObjectiveTo review cardiac manifestations in the syndrome of resistance to thyroid hormone (RTH) and to question the general recommendation that the thyroidstimulating hormone (TSH) value be the guide to thyroid hormone replacement.MethodsThe syndrome of RTH is caused by mutations in the carboxyterminal portion of the β isoform of the thyroid hormone receptor, resulting in variable clinical manifestations. It is generally recommended that the replacement of thyroid hormone in patients with RTH be guided by the serum TSH concentration. The variable responsiveness of tissues to thyroid hormone, however, makes it difficult to balance the correct replacement dose. We present a case that brings into question the conventional wisdom about the replacement dose of thyroid hormone in this scenario, and we review the pertinent literature.ResultsA 54- year-old man with RTH was treated with levothyroxine and increasing doses of liothyronine sodium as part of an evaluation of RTH. On day 10 of theprotocol, he developed atrial fibrillation despite a normal level of TSH (1.1 mIU/L). Administration of liothyronine was discontinued, and cardioversion was planned; however, the patient’s heart rhythm converted spontaneously to normal sinus rhythm.ConclusionReplacement of thyroid hormone in patients with RTH should include careful monitoring of thyrotoxic cardiac side effects in addition to consideration of normalization of the TSH level. (Endocr Pract. 2012;18:252- 255)     

Estrogen Levels Do Not Rise With Testosterone Treatment For Transgender Men

Objective: Existing transgender treatment guidelines suggest that for transmasculine treatment, there is a possible need for estrogen-lowering strategies adjunct to testosterone therapy. Further, guidelines advocate consideration of prophylactic female reproductive tissue surgeries for transgender men to avoid the possibility of estrogen-related health risks. Despite the paucity of objective data, some transgender men seek conversion inhibitors. We sought to determine estradiol levels in transgender men treated with testosterone therapy and the change in those levels with treatment, if any.Methods: Estradiol levels were extracted from the electronic medical records of 34 anonymized transgender men treated with testosterone therapy at the Endocrinology Clinic at Boston Medical Center. Data were sufficient to observe 6 years of follow-up.Results: With increased testosterone levels in trans-gender men, a significant decrease in estradiol levels was noted. There was a significant negative correlation between testosterone levels and body mass index, which may serve to explain part of the mechanism for the fall in estradiol levels. Even though the fall in estradiol levels was significant statistically, the actual levels remained within the normal male range, even with 6 years of follow-up.Conclusion: These data suggest that when exogenous testosterone is used to achieve normal serum male testosterone levels for transgender men, it is converted to normal male levels of estradiol, with some decline in those estradiol levels that might be attributable to a fall in fat mass. There appears to be no role for aromatase conversion inhibitors or other estrogen-reducing strategies in trans-gender men.Abbreviation: BMI = body mass index     

Enumeration of micro-organisms in processed soy products with an automated most probable number method compared with standard plate method

Aim: The automated TEMPO system (bioMerieux) is based on the most probable number (MPN) method for the enumeration of micro‐organisms in foods. In this study, we evaluated the performance of the TEMPO system as a diagnostic tool in comparison with the standard method in processed soy products. Methods and Results: A verification study was conducted using artificially contaminated soy product samples such as soy protein isolate, water‐soluble soy polysaccharides, soy milk and processed soy food. Five types of micro‐organisms were analysed using the automated MPN method (total aerobic bacteria, total coliforms, Enterobacteriaceae, yeast and mould and Staphylococcus aureus) vs the standard plate method. The results from each of the methods were highly correlated (r > 0·95). Naturally contaminated processed soy products on the market were also studied. There were no discrepancies observed between the respective methods. Conclusions: TEMPO methods were equivalent to the corresponding standard plate methods with very good rates of agreement. Significance and Impact of the Study: The automated MPN method is more practical and reliable for in‐house microbiological testing in processed soy products.     

Successful Fertility Restoration after Allogenic Hematopoietic Stem Cell Transplantation

ObjectiveMyeloablative conditioning regimens given prior to hematopoietic stem cell transplantation (HSCT) frequently cause permanent sterility in men. In patients with sickle cell disease (SCD) we use a nonmyeloablative regimen with sirolimus, alemtuzumab, and low-dose total-body irradiation (300 centigrays) with gonadal shielding preceding allogeneic HSCT. We report here the restoration of azoospermia in a patient with SCD after allogeneic HSCT. We discuss the impact of our patient’s underlying chronic medical conditions and the therapies he had received (frequent blood transfusions, iron chelating drugs, ribavirin, hydroxyurea, opioids), as well as the impact of the nonmyeloablative conditioning regimen on male gonadal function, and we review the literature on this topic.MethodsWe determined the patient’s reproductive hormonal values and his semen parameters before, during, and after HSCT and infertility treatment. In addition, we routinely measured his serum laboratory parameters pertinent to SCD and infertility, such as iron and ferritin levels. A karyotype analysis was performed to assess the potential presence of Klinefelter syndrome. Finally, imaging studies of the patient’s brain and testes were done to rule out further underlying pathology.ResultsA 42-year-old man with SCD, transfusional iron overload, and hepatitis C underwent a nonmyeloablative allogeneic HSCT. One year later he desired to father a child but was found to be azoospermic in the context of hypogonadotropic hypogonadism. Restoration of fertility was attempted with human chorionic gonadotropin (2,000 IU) plus human menopausal gonadotropin (75 IU follicle-stimulating hormone) injected subcutaneously 3 times weekly. Within 6 months of treatment, the patient’s serum calculated free testosterone value normalized, and his sperm count and sperm motility improved. After 10 months, he successfully initiated a pregnancy through intercourse. The pregnancy was uncomplicated, and a healthy daughter was delivered naturally at term.ConclusionDespite exposure to several gonadotoxins, transfusional iron overload and nonmyeloablative conditioning with radiation causing severe testicular atrophy suggesting extensive damage to seminiferous tubules and possibly Leydig cells, gonadotropins were efficacious in restoring our patient’s reproductive capability. (Endocr Pract. 2014;20:e157-e161)     

Gynecomasty as a first sign of adrenal carcinoma--case report

We present a case of 50 year-old man with feminizing adrenal carcinoma. He was admitted to the hospital because of weakness and one year history of gynecomastia and high blood pressure. Examinations revealed a large left adrenal mass and increased levels of estradiol. Patient underwent adrenalectomy and followed by mitotan therapy as the result of histopathological examination was adrenocortical carcinoma. One year after operation patient stays free from the recurrence of the disease and his estradiol, androstendion and DHEA levels are below the detection limits. We report this case because feminizing adrenal carcinoma is a very rare but serious disease and gynecomastia that could be its manifestation is quite frequent symptom in men's population and thus it could easily be missed. In every case of gynecomastia related to estradiol excess feminizing tumors of testis and adrenal gland should be ruled out.     

Pheochromocytoma-Induced Aggression?

ObjectiveTo document a case of pheochromocytoma with an unusually high plasma ratio of norepinephrine to epinephrine concentrations (NE:E), and a history of violent and aggressive behavior (which has been reported to be associated with increased NE:E ratios).MethodsWe present the history of present illness, history of aggressive behavior, and the clinical course of a man who was found to have pheochromocytoma with a remarkable catecholamine profile. We also review the literature on the relationship of catecholamine ratios to behavior.ResultsA 33-year-old man presented to the emergency department with the chief complaint of palpitations and chest pain. A physical exam revealed markedly elevated blood pressure. On admission, a computed tomographic scan of the abdomen revealed a 10 by 10-cm heterogeneous mass of 20 Hounsfield units superior to the right kidney. His plasma NE:E ratio was 35, and his 24-hour urine ratio of normetanephrine to metanephrine concentrations was greater than 26. The tumor was successfully removed with laparoscopic adrenalectomy, and the histologic findings revealed benign pheochromocytoma. There was no immediate change in the patient’s behavior. He was incarcerated the week after surgery, and lost to follow-up.ConclusionPrimarily norepinephrine-producing pheochromocytoma may have contributed to this patient’s violent and aggressive behavior. Catecholamine levels may remain elevated for 1 week following surgery. Even if this patient’s norepinephrine level had dropped rapidly after removal of the pheochromocytoma, and was not elevated a week later when he was arrested, it is possible that his aggressive behavior may have been conditioned by long exposure to elevated levels of norepinephrine. (Endocr Pract. 2011;17:e126-e129)     

Gonadotropin-Releasing Hormone Agonist-Induced Pituitary Apoplexy in Treatment of Prostate Cancer: Case Report and Review of Literature

ObjectiveTo describe a case and review the literature on the rare complication of pituitary apoplexy after administration of a gonadotropin-releasing hormone agonist (GnRHa) for treatment of patients with prostate cancer.MethodsWe present a detailed case report of a patient with immediate signs of pituitary apoplexy after receiving a GnRHa and review the 6 previously reported cases in the literature.ResultsA 60-year-old man presented to a local hospital with severe headache, nausea, vomiting, and diplopia. Prostate cancer had recently been diagnosed, and he had received his first dose of a GnRHa 4 hours before this presentation. On physical examination, he was confused and had ptosis of the left eye. A head computed tomographic scan without contrast enhancement showed soft tissue filling the sella, without intracranial hemorrhage or mass effect. He was discharged with the diagnosis of viral meningitis. Three weeks later, he presented again with severe headache and diplopia. He had confusion, lethargy, disorientation, a blood pressure of 88/64 mm Hg, and left cranial nerve III, IV, and VI paralysis. Magnetic resonance imaging of the brain revealed an enhancing pituitary mass with hemorrhage extending to the optic chiasm, consistent with pituitary apoplexy. Laboratory results were consistent with panhypopituitarism. Surgical excision revealed a necrotic pituitary macroadenoma with hemorrhage. Tumor immunohistochemical staining was positive only for luteinizing hormone.ConclusionWe describe a rare adverse effect of GnRHa therapy, which unmasked a gonadotropin-secreting pituitary macroadenoma. This case adds to the 6 previously reported cases of GnRHa administration inducing pituitary apoplexy in men with prostate cancer. (Endocr Pract. 2007;13:642-646)     

Residual dysphasia after severe hypoglycemia in a patient with immune-mediated primary Adrenal insufficiency and type 1 diabetes mellitus: Case report and systematic review of the literature

ObjectiveTo describe a 24-year-old patient with immune-mediated primary adrenal insufficiency and type 1 diabetes mellitus (T1DM) receiving intensive diabetes management who was comatose at presentation attributable to severe hypoglycemia and had residual dysphasia after recovery and to summarize the related literature.MethodsWe present a case report and the findings on systematic review of the pertinent literature to identify the cumulative incidence of severe hypoglycemia with use of intensive insulin therapy in patients with primary adrenal insufficiency and T1DM and to determine the incidence of dysphasia after severe hypoglycemia.ResultsAfter 5 days of mechanical ventilation, our patient was revived. He had severe dysphasia after recovery of consciousness. Magnetic resonance imaging of the brain revealed encephalomalacia in the left temporal, frontal, and parietal lobes. After 6 years of follow-up, he continues to have residual deficits of expressive dysphasia and difficult-to-control seizures but no other neurologic disorders. Systematic review of the literature revealed that studies from the 1950s reported mortality due to hypoglycemia in such a cohort, but no recent studies have described the cumulative incidence of severe hypoglycemia in a cohort of patients with primary adrenal insufficiency and T1DM. To the best of our knowledge, we report the first findings on magnetic resonance imaging of the head in such a patient.ConclusionFortunately, residual dysphasia is an infrequent outcome after severe hypoglycemia. (Endocr Pract. 2007;13:384-388)