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.

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.

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.

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 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.

Objective To analyze the clinical characteristics and prognosis of patients with insulin autoimmune syndrome (IAS) and to get better understanding of IAS by literatures reviewing.Methods Nine cases of IAS who were diagnosed in the General Hospital of the People's Liberation Army from 2001 to 2011 were analyzed.Results All patients had hypoglycemic syndrome and episodes of hypoglycemia during both postprandial and fasting states.Most cases (8/9) were accompanied with autoimmune diseases,including 6 cases of Graves' disease treated with methimazole.The biochemical data showed extremely elevated serum insulin level (9/9) during hypoglycemic episode.For most patients,the tests of insulin autoantibodies were positive (7/9) while results of imaging examinations were negative(8/9).After removal of possible offending medications and with diet treatment,hypoglycemic episodes were ameliorated in 5 of 9 cases.For severe patients,acarbose (1/9) and prednisone (3/9)therapy were useful.During the period of follow-up,four cases experienced no episode of hypoglycemia and 3 cases with markedly reduced episodes.Conclusions IAS is characterized by hypoglycemic episodes,elevated blood insulin levels,and positive insulin autoantibodies.It is strongly related with autoimmune disease and is able to be induced by methimazole.Most patients undergo remission after diet treatment,drug withdrawal,and oral prednisone.

Objective To study gender-related differences of clinical characteristics and vascular complications in patients with aldosterone-producing adenoma(APA).Method Consecutive 315 patients with APA confirmed by pathological diagnosis were included and the clinical features and vascular complications were compared based on gender.Results (1) Of the 315 patients with APA,female accounted for 52.7％ (166/315).Male patients with APA presented a higher BMI,compared with females [(25.4± 2.9) vs (24.1 ± 3.2) kg/m2,P＜0.01],and the history of smoking and drinking was more common in male patients.No significant difference was found in regard to the age,duration,hypokalemia,tumor size,and family history of hypertension between two groups (P＞0.05).(2) No significant difference was found in the prevalence of hypertension,duration of hypertension,blood pressure,and antihypertensive medieation between two groups.However,the patients with grade 3 hypertension tended to be more prevalent in males(P =0.08).(3) Serum aldosterone concentrations were similar in two groups,but 24 h urinary aldosterone showed an increased trend in male group (P =0.07).(4) The overall prevalences of cardiovascular (51.0％ vs 36.1％) and cerebrovascular (9.4％ vs 3.0％) complications were significantly higher in male group (P ＜ 0.05).Further analysis of cardiac events revealed significantly higher rates of left ventricular hypertrophy (23.5％ vs 13.9％) and arrhythmia (21.5％ vs 10.8％) in males (P＜0.05).However,no significant difference in the prevalence between two groups was found in regard to cerebral hemorrhage,infarction,and chronic renal insufficiency.(5) The fall of blood pressure and recovery from hypokalemia were comparable between males and females in two weeks after sugery.However,in the patients with persistent hypertension,the number of antihypertensive drugs used in males was greater than that in females and more male patients needed ≥ 2 types of antihypertensive drugs.Conclusion There were significant gender-related differences with regard to clinical features and vascular complications in patients with APA.

A 31-year-old male normotensive patient with aldosterone-producmg adenoma complained of thirst,polydipsia,polyuria,and periodical paraplegia.The diagnosis is raised by signs of hypokalemia.Despite the lack of hypertension,primary aldosteronism was confirmed by persistent hypokalemia,increased urinary potassium,increased urinary and plasma aldosterone levels and suppressed plasma rennin activity (PRA).The blood pressure profile was studied by ambulatory monitoring,and the mean blood pressure of 24h was normal and the circadian rhythm remained normal. Surgical removal of the histologically typical aldosterone-producing adenomas normalized the kalemia.The patient had a marked fall in blood pressure with mean values of 21/17 mm Hg ( diurnal and nocturnal blood pressure were 19/17 and 22/17 mm Hg respectively)and recovery of normal urinary and plasma aldosterone levels and PRA 6 weeks after surgery.This suggests that excess serum aldosterone induced relative hypertension in those patients whose blood pressure was spontaneously very low.Our observations call for primary hyperaldosteronism assay in patients with hypokalemia and renal potassium leakage.

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.