Ovotestis is a rare disorder of sexual differentiation in which the gonads have both ovarian and testicular elements. The patients always present with ambiguous external genitalia, and there are usually with serious disorder between chromosomal sex, gonadal sex, social sex, and psychological sex. The definite diagnosis and gender confirmation, appropriate surgery in internal genitalia and orthomorphia in external genitalia, as well as psychological support are essential for a multidisciplinary medical group in managing this disease. The medical data of two children with ovotestis who were reared as boy or girl respectively were analyzed.

Objective To investigate the clinical characteristics of multiple endocrine neoplasia type 1(MEN1)patients presenting with hypoglycemic coma as chief manifestation and the related clinical experience in diagnosis and therapy.Methods We analyzed the clinical data of 4 patients who were hospitMized because of recurrent hypoglycemic coma and diagnosed as having MEN1 by endocrinolgical,radiological and pathological examinations.Results In the 4 cases of Whipple trilogy,radiological examination showed occupying lesion in the pancreas and pathological examination confirmed the diagnosis of insulinoma.In 2 cases the insulinomas were multiple.In this series,one cage was complicated with pituitary adenoma,parathyroidoma(recurrent after operation)and adrenocortical adenoma,one case with pituitary adenoma,parathyroidoma(2 tumors)and adrenal nodular hyperplasia,one case with pituitary adenoma and parathyroidoma,and the remaining one with pituitary adenoma and suspectable parathyroidoma.Conclusions For patients with insulinoma,MEN1 should be considered.In patients with MEN1,the presence of multiple or ectopic Darathymid adenomas(or hyperplasia)and insulinomas should be inspected during operation.After operation,examinations should be regularly performed to identify whether the diseases relapse or new endocrine neoplasias occur.

HepG2 ceils were treated with various concentrations of tumor necrosis factor-or (TNF-α) for 24 hours. RT-PCR and Western blot were used to measure protein tyrosine phosphatase-1 B(PTP-1B)expression, and luciferase reporter assay was used to detect NF-kB activity. The results showed that treatment of HepG2 cells with TNF-α for 24 hours led to upregulation of PTP-1B and NF-kB activity in a dose-dependent manner. Inhibition of NF-kB by PI)TC significantly attenuated TNF-α-induced PTP-IB expression in HepG2 cells. Thus, the transactivation of NF-kB seems to play an important role in the expression of PTP-1B in HepG2 cells induced by TNF-α.

Objective To study the cerebrospinal fluid(CSF) and serum level of human chorionic gonadotropin (HCG) in patients with intracranial germinoma and to evaluate its diagnostic and therapeutic value. Methods Thirty-one patients with intracranial germinoma receiving estimation of HCG in CSF and serum in our hospital were retrospectively analyzed in terms of HCG level, its influencing factors and the relationship of HCG with clinical features. Results HCG levels in CSF of the 31 cases ranged from 0. 17 IU/L to 5316. 98 IU/L with a median value of 3.44 IU/L. The sensitivity of diagnosis increased from 80. 6% to 90. 3%, when the cut point of HCG in CSF changed from 0. 60 IU/L to 0. 50 IU/L. The sensitivity increased from 83.9% to 93.5% when the cut point of the ratio of CSF/serum HCG decreased from 1.8 to 1.7. HCG level of germinoma located in pineal region was higher than that in basal ganglia region, while it is lowest in sellar region. The ratio of CSF/serum HCG in different parts showed no difference. Multiple risk factors analysis revealed that serum HCG (r =0. 886,P =0. 0001 ) and tumor size ( r=0.748, P=0.0211 ) were positively correlated with the HCG level in CSF, while course of the disease,age and gender were not correlated. After radiation therapy, HCG in CSF and serum decreased dramatically as compared with those before radiation. Conclusions The HCG level and its dynamic change were sensitive marker of intracranial germinomas. Based on our analysis, HCG in CSF over 0. 50 IU/L and the its ratio in CSF/serum over 1.7 were highly indicative of the possibility of intracranial germinomas.

All three cases of recurrent lymphocytic hypophysitis were female, one of them being 70-yearold postmenopausal woman.Adenohypophysis, cavernous sinus, and optic chiasm were involved in case 1,hypothalamus and neurohypophysis were invaded in case 2, and adenohypophysis and hypophyseal stalk were involved in case 3.Relapse occured 4 months after operation in case 1, then high dosage methylprednisolone pulse therapy (HDMPT) brought about a remission lasting for 14 months before second relapse set in.Relapse occurred in case 2 at 16 months after prednisone treatment was discontinued, and case 3 recurred during the period of dose reduction.All three patients were then treated with glucocorticoid plus azathioprine for 16 weeks, and good response was seen in 3 cases.During follow-up, the symptoms were relieved and significant reduction of lesion was revealed by MRI in all thee patients, and the pituitary function was resumed in case1and 3.

Objective Adipsic hypernatremia is very rare in clinical practice and only a few cases have been reported so far. Since the pathogenesis of adipsic hypernatremia is unclear, therapeutic method is very limited and uncertain. We previously found the existence of partial central diabetes insipidus in the patient with adipsic hypernatremia according to a series of clinical studies. In this clinical study, we observed the response of vasopressin in the treatment of adipsic hypernatremia as a routinely used agent in treating central diabetes insipidus.Methods Five patients with chronic sustained hypernatremia and hypodipsia were diagnosed as adipsic hypernatremia according to the criteria by Halter. After testing plasma electrolytes, urinary osmolality, plasma osmolality, and free water clearance (CH2O);with evaluating thirsty grade and anterior pituitary function, patients were forced to drink water 2 000-2 500 ml each day combined with prednisone and/or levethyroxin supplementation therapy if hypopituitarsm was revealed. One week later, since the hypernatremia was unrelieved, desmopressin acetate (0.05-0.2 mg/d)was administered to those 5 patients. About 5-7 days later, plasma electrolytes, urinary and plasma osmolality, and free water clearance ( CH2O ) were reevaluated. Osmolality was detected by the method of freezing point depression and thirst grade by the method of visual analogue scales. Results The plasma sodium was 160-190 mmol/L and plasma osmolality was 330-370 mmoL/L without thirsty perception during the hospitalization. Four of the five patients were revealed to be suffering from hypopituitarism, but the hypernatremia could not be corrected by intentional water intake combined with prednisone treatment. After administering vasopressin 0.05-0.2 mg/d to these patients, that hypernatremia was relieved, and hyperosmolality and hypodypsia were improved effectively. During this period of time, no side effects were detected in these patients. Conclusion We testified the existence of central partial diabetes insipidus in the patients with adipsic hypernatremia. Desmopressin acetate in the treatment of adipsic hypernatremia is proved to be effective, simple, and safe.

Objective To explore risk factors of recurrence in papillary thyroid microcarcinomas ( PTMC ) and papillary thyroid carcinomas with 1-2 cm diameter. Methods From January, 2008 to December, 2010 in PLA General Hospital, 323 eligible patients received first surgery and diagnosed pathologically with papillary thyroid cancer≤2 cm were analyzed retrospectively. According to rumor size, patients were divided into PTMC and PTC of 1-2 cm, which were investigated recurrence factors. Results Finally we indentified 320 PTC≤2 cm, including 218 (68.1%)PTMCand102(31.9%)PTCof1-2cmwithamedianfollow-uptimeof72.5(55-90)months.32cases (10%)of patients relapse, includig 22 cases(10%)in PTMC and 10 cases(9. 8%)in PTC of 1-2 cm. In the clinical characteristics analyses of PTC≤2 cm, the PTC of 1-2 cm was different from PTMC in age, lymph node metastasis and TNM stage. The univariate analysis showed that tumor location and lymph node metastasis influenced recurrence of PTMC and PTC of 1-2 cm,while tumor foci and extrathytoidal extension were risk factors of recurrence in PTMC but not in PTC of 1-2 cm. Lymph node metastasis was independent factor which influenced the recurrence of PTMC and PT C of 1-2 cm according to COX multivariate analysis. Conclusion Disease recurrence did not differ significantly between the PTMC and PTC of 1-2 cm and lymph node metastasis was an independent recurrence factor.

Objective To study clinical and immunological features of renal tubular acidosis (RTA) patients complicated with autoimmunity disease. Methods Data of 60 patients of RTA complicated with autoimmune disease and 40 without it during 1999 and 2009 were reviewed, including clinical features,immunological examinations and renal tubular function. Results Among 60 patients of RTA, 59 were type Ⅰ, one type Ⅱ, and 55 complicated with Sjogen's syndrome (92%), one with systemic lupus erythematosus, nine with autoimmune thyroid disease, and one with rheumatoid arthritis. Flaccid paralysis was manifested in 50 (83%) RTA patients complicated with autoimmune disease, polyuria in 28 (47%),polydipsia in 28 (47%) , bone disorder in 24 (40%) and arthralgia in 28 (47%) cases, but of those without autoimmune disease, bone disease only in eight (20%) and arthralgia in nine (22%) cases, with statistically significant difference ( P ＜0. 01 ). Serum level of parathyroid hormone increased noticeably, with prevalence of positive antinuclear antibody, anti-SSA antibody and anti-SSB antibody of 88 % (49/56),84% (47/56) and 43% (24/56), respectively in RTA patients with autoimmune disease, all significantly higher than those in the patients without it ( P ＜ 0. 05 ). Conclusions Sjogen' s syndrome is the most common and prevalence of bone disorder and arthralgia are significantly higher in patients of RTA complicated with autoimmune disease, which should be examined as early as possible for timely diagnosis and treatment.

Objective To better understand the clinical management of tumor-induced osteomalacia (TIO) by analyzing the clinical features, diagnosis, treatment, postoperative biochemical changes, and clinical status in 12 cases of TIO. Methods Twelve cases of TIO hospitalized from 2004 to April 2010 were reviewed retrospectively. All cases were diagnosed based on their clinical manifestation, hypophosphatemia, and image study including technetium-99m octreotide scintigraphy (99mTc-Oct). Resuits There were 7 males and 5 females with mean age of (41.8±9.6) years (20 to 56 years). The course of disease was from 2 to 14 years ( median course 4.0 years). They all presented with bone pain, gait disturbance, muscle pain, and muscle weakness. Serum phosphate( Pi)levels were low in 12 cases with a range from 0.30 to 0.56 mmol/L. 99mTc-Oct was performed in 9 cases and it showed that the lesions were located in head of femur, fibula, retrocalcaneal area, foot, humerus,metacarpal, posterior chest wall or near nasal bone (apex partis petrosae ossis temporalis). Subcutaneous soft tissue mass was found in another 3 cases at loin, thigh, and foot by physical examination. The tumors were confirmed by CT, MRI or ultrasonography. Twelve patients underwent operation to remove the tumors and histopathology showed hemangioendothelioma or fibrous angioma (6 cases), giant cell tumor or fibroma of tendon sheath(4 cases), liposarcoma(1case), and phosphaturic mesenchymal tumor(1case). Serum Pi levels returned to normal in 10 patients after resection of tumor. During 2 to 64 months follow up, symptoms of bone pain and muscle weakness were improved obviously. Conclusions Patients with hypophosphatemic osteomalacia should be thoroughly investigated for TIO. 99mTc-Oct and other imaging examinations can effectively locate the tumors. Once the hidden tumor is found and excised, the patient will recover and enjoy normal life with normalized Pi concentrations and marked improvement of symptoms.

Objective To improve the positivity for insulinoma localization by imaging before surgery.Methods The clinical data of 119 patients with insulinoma who had been admitted to Chinese PLA General Hospital from January 1985 to April 2008 were retrospectively reviewed. Results One hundred patients with hypoglycemia who had been operated and most of them (98/100) diagnosed as insulinoma pathologically were analyzed. They had undergone various methods of localization by imaging before surgery. The positive rates for tumor localization were 22.9% (17/74) by uhrasonography (US), 55.2% (48/87) by CT, 58.8% (10/17) by MRI,76.6% (49/64) by digital subtraction angiography (DSA) ,93.3% (42/45) by endtoscopic ultrasonography (EUS)and 94.7% (18/19) by uhrasonic contrast (UC). While the rates of accuracy for tumor localization were 88.2% (15/17) by US ,87.5% (42/48) by CT, 90.0% (9/10) by MR1,100.0% (49/49) by DSA, 85.7% (36/42) by EUS and 83.3% (15/18) by UC. The rates of accuracy in patients with one,two and three positive images were 88.9% (24/27), 96. 9% (32/33) and 94. 7% (18/19), respectively. The positive rates in patients with combination of two images were 47.6% (30/63) by CT and US ,75.5% (40/53) by CT and DSA ,89.2% (33/37)by CT and EUS,90.0% (27/30) by EUS and DSA ,78.6% (11/14) by DSA and UC ,92.3% (12/13) by CT and UC, and 90.9% (10/1 i) hy EUS and UC, respectively. Conclusion The positive rates and accuracy of localization for insulinoma were increased by combining at least two images, and the combinations of any two of four images including CT, DSA, EUS and UC are suggested. While considering both the cost and effect, we recommend CT combined either with EUS or UC.