Testosterone Replacement and Bone Mineral Density in Male Pituitary Tumor Patients

BACKGROUND: Hypopituitarism is associated with osteoporosis and osteopenia especially when hypogonadotropic hypogonadism is present. Despite hypopituitarism being an important cause of secondary osteoporosis, osteoporosis in patients receiving surgery for pituitary tumors in Korea has not been studied. In this study, we evaluated the effects of testosterone replacement therapy (TRT) on bone mineral density (BMD) in postoperative hypogonadal patients with pituitary tumors. METHODS: To examine the effect of TRT on BMD, we performed a retrospective observational study in 21 postoperative male patients who underwent pituitary tumor surgery between 2003 and 2012 at the Ajou University Hospital. Testosterone was replaced in postoperative hypogonadal patients by regular intramuscular injection, daily oral medication, or application of transdermal gel. BMD (g/cm2) measurements of central skeletal sites (lumbar spine, femoral neck, and total femur) were obtained using dual-energy X-ray absorptiometry (GE Lunar). For lumbar spine BMD, L1 to L4 values were chosen for analysis. Femur neck and total femur were also analyzed. RESULTS: During the follow-up period (mean, 56 months; range, 12 to 99 months) serum testosterone levels increased with the administration of TRT (P=0.007). There was significant improvement (4.56%+/-9.81%) in the lumbar spine BMD compared to baseline BMD. There were no significant changes in the femur neck BMD or total femur BMD. We did not find any statistically significant relationships between changes in testosterone levels and BMD using Spearman correlation analysis. CONCLUSION: Our results indicated that TRT used in the postoperative period for hypogonadal pituitary tumor surgery patients may have beneficial effects on the BMD of the spine.

The Ability of beta-Cells to Compensate for Insulin Resistance is Restored with a Reduction in Excess Growth Hormone in Korean Acromegalic Patients

The aim of this study was to assess the prevalence of diabetes and to study the effects of excess growth hormone (GH) on insulin sensitivity and beta-cell function in Korean acromegalic patients. One hundred and eighty-four acromegalic patients were analyzed to assess the prevalence of diabetes, and 52 naive acromegalic patients were enrolled in order to analyze insulin sensitivity and insulin secretion. Patients underwent a 75 g oral glucose tolerance test with measurements of GH, glucose, insulin, and C-peptide levels. The insulin sensitivity index and beta-cell function index were calculated and compared according to glucose status. Changes in the insulin sensitivity index and beta-cell function index were evaluated one to two months after surgery. Of the 184 patients, 17.4% were in the normal glucose tolerance (NGT) group, 45.1% were in the pre-diabetic group and 37.5% were in the diabetic group. The insulin sensitivity index (ISI0,120) was significantly higher and the HOMA-IR was lower in the NGT compared to the diabetic group (P = 0.001 and P = 0.037, respectively). The ISI0,120 and disposition index were significantly improved after tumor resection. Our findings suggest that both insulin sensitivity and beta-cell function are improved by tumor resection in acromegalic patients.

Diagnostic Value of a Chimeric TSH Receptor (Mc4)-Based Bioassay for Graves' Disease

BACKGROUND/AIMS: Graves' disease (GD) is caused by thyroid-stimulating hormone receptor (TSHR) and thyroid-stimulating immunoglobulin (TSI). We used a recently introduced, technically enhanced TSI bioassay to assess its diagnostic value and determine the cut-off in patients in high iodine intake area. METHODS: In a cross-sectional setting, we collected serum from 67 patients with untreated GD, 130 with GD under treatment, 22 with GD in remission, 42 with Hashimoto's thyroiditis, 12 with subacute thyroiditis, 20 with postpartum thyroiditis, and 93 euthyroid controls. TSI was measured using the Thyretaintrade mark bioassay, which is based on Chinese hamster ovary cells transfected with chimeric TSHR (Mc4). TSI levels are reported as a specimen-to-reference ratio percentage (SRR%). RESULTS: The TSI levels in patients with GD (either treated or not) were significantly higher than those of the remaining patients (p < 0.05). The new bioassay showed a sensitivity of 97.0% and a specificity of 95.9% with a cut-off value of 123.0 SRR% for GD. A weak correlation was found between TSI and thyrotropin-binding inhibiting immunoglobulin (TBII) (rs = 0.259, p = 0.03), but no correlation was found between TSI and tri-iodothyronine or free thyroxine. CONCLUSIONS: The Mc4-CHO bioassay showed comparable diagnostic value for GD with the conventional TBII assay. We propose a cut-off of 123.0 SRR% in areas where iodine intake is high.

Clinicopathological Characteristics and Prognostic Factors of Medullary Thyroid Carcinoma

BACKGROUND: Studies on the clinicopathological characteristics and prognostic factors of medullary thyroid carcinoma (MTC) in Korea are very rare. METHODS: We enrolled 56 MTC patients who underwent surgery at Samsung Medical Center from 1995 to 2006. We analyzed their gender, age at diagnosis, the pathologic findings, the TNM stage, the association with multiple endocrine neoplasia (MEN), RET protooncogene mutation and the, serum basal calcitonin levels before and after the surgery. We investigated the overall survival and the prognostic factors. RESULTS: The mean age at diagnosis was 46 years and the male/female ratio was 1:2.7. Fine needle aspiration cytology detected 61% of the MTC. The mean tumor size was 2.6 cm (range: 0.2-9.0 cm). Fifty-two percent of patients had the TNM stage more than III at the time of diagnosis. Distant metastasis was found in 5.3% (3/56) of the patients, either at the time of diagnosis or during the follow-up period. Hereditary MTC comprised of 23% of the patients and the disease developed at a younger age (38 years vs. 48 years, respectively, P < 0.05) with more bilaterality. RET protooncogene mutations were found in 27% (9/33) of the patients and most of them were in codon 634. After the primary surgery, the serum basal calcitonin levels were persistently elevated over 13 ng/L in 49% of the patients. The overall 5-year survival rate was 95.5%. Tumor size and distant metastasis were the significant prognostic factors for survival by univariate analysis (P < 0.05). CONCLUSION: There were no significant differences in the clinicopathological characteristics of MTC and survival in Korea compared to those of the Western countries.

Sustained Maintenance of Normal Insulin-like Growth Factor-I during Pregnancy and Successful Delivery in an Acromegalic Patient with Octreotide-LAR(R) Treatment

We report here on a 34-year-old Korean woman with active acromegaly and who received Octreotide-LAR(R) for 12 months following transsphenoidal pituitary surgery. During Octreotide-LAR(R) treatment, the clinical improvement was paralleled with the decrease of the growth hormone levels to 1.1 ng/mL and the insulin-like growth factor-I (IGF-I) levels to 345.5 ng/mL. Octreotide-LAR(R) was discontinued when the patient was found to be at the 12th week of pregnancy. During pregnancy, the patient experienced clinical well-being and she maintained her IGF-I levels within the normal range for her age-matched despite discontinuation of Octreotide-LAR(R) treatment at early gestation. She delivered a full-term healthy male infant. The serum IGF-I levels of the patient increased progressively increased after delivery. This report describes a successful pregnancy in an acromegalic woman who was exposed to Octreotide-LAR(R) during the early gestational period. She and who showed an unexpected pattern of persistently normal IGF-I levels through the pregnancy despite discontinuation of Octreotide-LAR(R) therapy.

Autosomal Dominant Hypocalcemia Caused by an Activating Mutation of the Calcium-Sensing Receptor Gene: The First Case Report in Korea

Hypoparathyroidism is an abnormality of calcium metabolism characterized by low serum levels of parathyroid hormone in spite of hypocalcemia. The causes of hypoparathyroidism are numerous. Activating mutations in the calcium-sensing receptor (CaSR) gene are well-known causes of familial isolated hypoparathyroidism, also known as autosomal dominant hypocalcemia (ADH). Here we describe members of a Korean family with a heterozygous Pro221Leu mutation causing ADH. This case is the first report in Korea.

A Case of Resistance to Thyroid Hormone with Thyroid Cancer

Resistance to thyroid hormone (RTH) is an autosomal dominant hereditary disorder that is difficult to diagnose because of its rarity and variable clinical features. The magnitude of RTH is caused by mutations in the thyroid hormone receptor beta (TRbeta) gene. We recently treated a 38-yr-old woman with RTH who had incidental papillary thyroid carcinoma. She presented with goiter and displayed elevated thyroid hormone levels with an unsuppressed TSH. She was determined to harbor a missense mutation of M310T in exon 9 of the TRbeta gene, and diagnosed with generalized RTH. This mutation has not yet been reported in Korea. RTH is very rare and easily overlooked, but should be considered in patients who present with goiter and elevated thyroid hormone levels with an unsuppressed TSH. The association between thyroid cancer and RTH needs further study.

Drug-induced hepatitis complicated by pure red cell aplasia and autoimmune hemolytic anemia: a case report / 대한내과학회지

Pure red cell aplasia is a rare hematopoietic complication of drug-induced hepatitis, with only five cases reported worldwide. Moreover, pure red cell aplasia associated with autoimmune hemolytic anemia in the setting of drug-induced hepatitis is exceedingly rare. We recently experienced a case of drug-induced hepatitis complicated by pure red cell aplasia and autoimmune hemolytic anemia without parvovirus B19 infection. Here, we report the case and review the literature.

A Case of a Jejunal Dieulafoy's Lesion Mimicking a Submucosal Tumor / 대한소화기내시경학회지

Dieulafoy's lesion is a rare cause of severe gastrointestinal bleeding. In most cases, Dieulafoy's lesion is typically found in the stomach within 6 cm from the gastroesophageal junction, but the presence of lesions has also been described throughout the gastrointestinal tract. A jejunal Dieulafoy's lesion is extremely rare, and only a few cases have been reported in the Korean population. We experienced a case of a jejunal dieulafoy's lesion mimicking a submucosal tumor that presented with massive gastrointestinal bleeding. The lesion was diagnosed by the use of double balloon enteroscopy and was then confirmed by a pathological examination of the surgical specimen.

A case of well-differentiated squamous cell carcinoma of the duodenum / 대한내과학회지

Squamous cell carcinoma of the duodenum is an extremely rare neoplasm worldwide. A 44-year-old female patient was admitted to our institution for abdominal pain, and she was diagnosed with primary squamous cell carcinoma of the duodenum. The duodenal cancer was inoperable due to vascular invasion and regional lymph node metastasis. The patient was treated with palliative chemotherapy, but her disease progressed, and she was eventually referred to a local hospital for supportive care. The prognosis of squamous cell carcinoma of the duodenum is poor compared with that of usual duodenal adenocarcinoma. We report the details of this case of squamous cell carcinoma of the duodenum along with a review of the literature.

A Case of Primary Amyloidosis with Nephrotic Syndrome and Factor X Deficiency / 대한신장학회지

We report a case of nephrotic syndrome and factor X deficiency secondary to primary amyloidosis. A 58-year-old man was referred to our hospital for evaluation of nephrotic syndrome and bleeding tendency. He was confirmed to have primary amyloidosis by renal biopsy, immunofixation electrophoresis and bone marrow findings. His bleeding tendency was due to prothrombin time prolongation caused by isolated factor X deficiency. If any patient with nephrotic syndrome has bleeding tendency due to coagulation abnormalities, that patient should be considered to have factor X deficiency secondary to primary amyloidosis.