[Clinical analysis of 10 patients with Fabry disease].

We aimed to analyze the clinical data of 10 patients (6 male and 4 female) with Fabry disease (FD). The mean age of the patients was (28.80±9.27) years. Seven patients had classical FD and three had delayed onset FD. Among the 10 patients, six had skin involvement and cutaneous angiokeratoma; five had hypohidrosis or anhidrosis; nine had intermittent neuralgia; and three had supraorbital ridge protrusion, forehead bulge, and lip thickening. Five patients had proteinuria, including one with chronic kidney disease stage 3 and one with chronic kidney disease stage 5. Cardiac involvement occurred in three patients, two had myocardial hypertrophy and one had valvular insufficiency. The activity of galactosidase decreased in seven patients (2.80-1.55 µmol·L-1·h-1). Plasma deacetyl-GL-3 was elevated in all 10 patients(3.12-120.00 ng/ml). Three patients underwent renal biopsy, wherein two cases of focal segmental glomerulosclerosis and one of mesangial proliferative glomerulonephritis was found. A large number of myeloid and zebra bodies were found in the podocytes in three patients, including a small number of myeloid and zebra bodies in the renal tubular epithelial cells in one patient with occasional zebra bodies in the renal interstitium. Nine patients had GLA gene mutations. One patient was c.102T>A, a de novo mutation. Four patients were treated with agalsidase α injection (0.2 mg/kg, intravenous infusion every 2 weeks), and their prognosis was good. FD has various clinical manifestations and multi-system involvement, which requires multidisciplinary cooperation. Detection of galactosidase activity, plasma globotriaosylsphingosine, and GLA gene mutation can help for accurate diagnosis.

[Efficacy and outcomes of shunt surgery for secondary hydrocephalus].

Records of secondary hydrocephalus patients undergoing shunt surgery in the Department of Neurosurgery of Peking Union Medical College Hospital from September 2012 to April 2022 and their clinical characteristics and outcomes were retrospectively reviewed and analyzed. Among 121 patients who received first time shunt placement, the most common causes of secondary hydrocephalus were brain hemorrhage (55, 45.5%) and trauma (35, 28.9%). Cognition decline (106, 87.6%), abnormal gait (50, 41.3%) and incontinence (40, 33.1%) were the most prevalent manifestations. Postoperative central nervous system infection (4, 3.3%), shunt obstruction (3, 2.5%) and subdural hematoma/effusion (4, 3.3%) were the most frequent neurological complications. Overall incidence of postoperative complications was 9% (11 cases) in the current cohort. And 50.5% (54/107) of the patients receiving shunting achieved a Glasgow outcome scale (GOS) score of at least 4. Shunt surgery is preferred for secondary hydrocephalus, especially for secondary normal pressure hydrocephalus. Moreover, it is recommended to complete cranioplasty in staged operation or one-stage operation for the patients with decompressive craniectomy.

[Critical care management and thinking of perihematomal edema after intracerebral hemorrhage].

The prognosis of patients with spontaneous intracerebral hemorrhage (sICH) is poor. It is of great significance to improve the neurological function of these patients and make them return to society. However, to date, no treatment has been proved to significantly improve the neurological prognosis of sICH patients. The perihematomal edema (PHE) is a quantifiable marker of secondary brain injury (SBI) after ICH. It is associated with dysfunction of ion channels of vascular endothelial cells, inflammatory response induced-blood brain barrier dysfunction, and iron deposition caused by red blood cell degradation after ICH. Given that the space-occupying effect of PHE, the direct relation with SBI, long growth course and variable growth of PHE among individuals, interrupting the expansion of PHE has become a therapeutic target to improve neurological outcomes in ICH patients. Conducting an integrated and individualized strategy of critical care management and performing the corresponding pre-clinical and translational clinical research targeting the pathophysiological mechanism, nature course, and risk factors of PHE deserves further exploration.

[Updated key points of Chinese Consensus for the Diagnosis and Treatment of Acromegaly (2021 edition)].

Led by the China Pituitary Adenoma Specialist Council, the Chinese Consensus for the Diagnosis and Treatment of Acromegaly (2021 Edition) is developed combined with the research progress of pituitary growth hormone (GH)-secreting adenoma both at home and abroad, evidence-based evidence of the diagnosis and treatment of acromegaly, and China's national conditions. Based on the guideline for acromegaly (2013 Edition), the new version of consensus emphasizes the importance of multidisciplinary team (MDT) and individual therapy. The criteria for control of acromegaly is discussed. Pathological criteria for the diagnosis of pituitary GH-secreting adenoma is updated. New developments in surgery, drug and radiotherapy are introduced. Meanwhile, the diagnosis and treatment of acromegaly patients concurrent with other particular scenarios, including pregnancy and refractory pituitary GH-secreting adenoma is suggested. This article aims to describe the updated key points of the new version of the consensus, and thus facilitate the clinical implementation of standardized diagnosis and treatment for acromegaly patients.

[Application of apparent diffusion coefficient histogram in moderate to severe neonatal hypoxic-ischemic encephalopathy].

Objective: To investigate the diagnostic value of apparent diffusion coefficient (ADC) histogram in quantifying moderate to severe neonatal hypoxic-ischemic encephalopathy (HIE). Methods: A total of 49 children with moderate to severe HIE admitted to the Children's Hospital Affiliated to Nanjing Medical University from July 2015 to September 2018 were retrospectively analyzed. Cranial magnetic resonance imaging (MRI) data of 31 full-term neonates without neurological symptoms and signs who visited the hospital during the same period were recruited as the control group. ImgJ software was used to delineate the whole brain area at the thalamus/basal ganglia level as the area of interest, and MRIcron software was used to obtain ADC histogram parameters. The differences of ADC histogram parameters between HIE group and control group were compared. The correlation between ADC histogram parameters and neonatal neurobehavioral neurological assessment (NBNA) in HIE group was analyzed. Receiver operating characteristic (ROC) curve was used to evaluate the diagnostic efficiency of each parameter. Results: The mean value, minimum value, maximum value, mode-value, variance, heterogeneity and 10%-90% ADC values of HIE group were all greater than those of the control group(634±65,310±36,1 683±18,711±87,66 928±921,5 907±204,477±50,513±53,542±56,568±59,591±61,625±92,661±97,709±105,811±119),the differences were statistically significant(all P<0.05), while mode-count of HIE group was smaller than that of the control group(272±22 to 395±83), and the difference was statistically significant (t=2.996,P<0.05). All the above parameters with statistical differences were correlated with NBNA score, among which mode-count was negatively correlated with NBNA score(r=-0.369,P<0.05), and the rest were positively correlated(all P<0.05). The area under the ROC curve (AUC) of variance had the highest diagnostic efficiency (0.881), followed by 90% ADC value and ADC maximum value. Conclusion: ADC histogram is helpful for the diagnosis of HIE, objectively and quantitatively reflecting the diffusion information of brain, and assessing the severity of HIE.

[The necessity and importance of standardized management of acromegaly: from the perspective of development of expert consensuses and clinical guidelines].

Acromegaly is a rare disease, and multidisciplinary collaboration is essential for its diagnosis, treatment, and follow-up.In recent years, a series of novel findings have been echieved in clinical studies on acromegaly.Therefore, the China Pituitary Adenoma Specialist council has convened Chinese specialists in neurosurgery, endocrinology, radiology, and radiotherapy to release the Chinese Consensus for the Diagnosis and Treatment of Acromegaly (2021), which aims to promote the standardized and individualized management of acromegaly.Looking back to the past, the consensuses and guidelines have played vital roles in establishing the widely recognized biochemical remission criteria, promoting new drugs and novel therapeutic strategies which are of significance for standardized treatment, and emphasizing the need to focus on the systemic complications of acromegaly and the long-term quality of life. In this editorial, we briefly reviewed the expert consensuses and clinical guidelines on acromegaly at home and abroad, and discussed their important roles in promoting standardized disease management from three aspects including biochemical remission standards, medical treatment, and the diagnosis and treatment of systemic complications.

[Performance of Knosp grade for cavernous sinus invasion of Cushing's disease].

Objective: To review the outcomes in patients with Cushing's disease (CD) after transsphenoidal surgery (TSS) and to assess the performance of Knosp grade for cavernous sinus invasion (CSI). Methods: Medical records were reviewed for patients with CD undergoing TSS from 1978 to 2017 at Peking Union Medical College Hospital. Based on whether CSI was observed during surgery, the sensitivity and specificity of Knosp grade for CSI were evaluated. Results: There were 1 061 records of CD reviewed, 83 (7.82%) of which were invasive tumors evaluated by surgery, and 44 (53.0%) of these remained in endocrinological remission at the last follow-up. The sensitivity of Knosp grade for CSI was 47.0%, and the specificity was 91.1%; besides, the positive predictive value was 31.0%, and the negative predictive value was 95.3%. Conclusions: Initial TSS for CD with invasive adenomas is of relatively low remission rate. Knosp grade 0-2 for non-invasive tumors is reliable, while it is doubtful whether or not Knosp grade 3-4 indicates invasive tumor.

[The surgical treatment in adults with idiopathic normal pressure hydrocephalus].

Objective: To investigate the effect of Ventriculoperitoneal shunt (VPS)in adult patients with idiopathic normal pressure hydrocephalus (iNPH). Methods: The patients with idiopathic normal pressure hydrocephalus were reviewed, who were admitted into the Peking union medical college hospital from October 2010 to October 2016.The patients treated via VPS were retrospectively analyzed by collecting the data including clinic features, iNPH score and imaging data. Results: 33 patients were included in the group.3 months after operation, the iNPH scores were significantly decreased (3.0±1.0 vs 6.0±2.5, P<0.01), and there were no difference among 3 month, 6 month and 12 month post operation.The Evans index and the width of the three ventricles were also significantly decreased.1 patient suffered from subdural hematoma and no other complications such as infection, obstruction or death occurred. Conclusions: VPS is an effective treatment approach of iNPH and multidisciplinary team is the key in diagnosis.

Analysis of bromocriptine treatment in pregnant pituitary prolactinoma patients.

OBJECTIVE: To investigate the therapeutic effects and duration of bromocriptine treatment during pregnancy in patients with pituitary prolactinoma. MATERIALS AND METHODS: A retrospective analysis of the clinical data of 230 female pituitary prolactinoma patients at the Beijing Union Medical College Hospital neurosurgery clinic from January 2001 to May 2014 was conducted. When confirmed pregnant, patients in the control group immediately stopped taking bromocriptine, but patients in the treatment group continued to take the same dose of bromocriptine. RESULTS: The embryos stop rate in the control group was 16.7%, significantly higher than the rate in the natural population (p < 0.05), while the rate in the treatment group (0.9%) not statistically different from that of the natural population (p > 0.05). There was no significant difference in the embryonic malformation rate between the two study groups compared to the normal pregnancy group (p > 0.05). CONCLUSION: Pregnant pituitary prolactinoma patients should not stop bromocriptine treatment, but should instead continue with the same dose for four months. For patients with macroadenoma, bromocriptine should be taken during the entire pregnancy. Blood prolactin, progesterone, human chorionic gonadotropin (hCG), and visual dysfunction should be monitored every two weeks during treatment. Patients should be treated with progesterone and hCG if the blood levels become too low. If regular monitoring shows that prolactin has increased too fast and/or visual dysfunction worsened, the dose of bromocriptine should be in- creased. The authors have found that bromocriptine treatment during pregnancy significantly reduces the embryo stop rate without in- creasing the embryo deformity rate; therefore, bromocriptine treatment is safe and necessary during pregnancy of pituitary prolactinoma patients.

[Cost-effectiveness analysis of octreotide long acting release and lanreotide slow release for the treatment of postoperative patients with active acromegaly in China].

Objective: To evaluate the cost-effectiveness of octreotide long acting release (LAR) vs lanreotide slow release (SR) for the treatment of postoperative acromegalic patients with elevated levels of growth hormone (GH) and insulin-like growth factor 1 (IGF-1) in China. Methods: A decision tree model was constructed and the treatment impact was projected for one year in Chinese setting. The clinical efficacy measure used was the percentage of patients achieving normalization (control) of either IGF-1 or GH levels. Efficacy of octreotide LAR and lanreotide SR, incidence of comorbidities, impact of acromegaly on health-related quality of life, and drug-related side effects data were obtained from literature. The cost of medication was collected through a chart review from five hospitals in five cities of China. Clinical experts from these hospitals were requested to complete a questionnaire to document the utilization of medical resources, costs of comorbidities, side effects as well as cost of administration. One-way sensitivity analysis was performed to evaluate the robustness of the results. Results: Compared to lanreotied SR group, the percentage of patients achieving normalization of IGF-1 and GH levels of octreotide LAR group were 10% and 9% higher, respectively. When either IGF-1 or GH control were used as the efficacy measure, patients in the octreotide LAR group exhibit less comorbidities and need less continued treatment with a second operation and radiotherapy than those in lanreotide SR group. When IGF-1 was used as efficacy measure, octreotide LAR not only achieved better efficacy but resulted in overall cost-saving, with a total cost savings of ï¿¥ 3 792 per patient for one year, which demonstrated that octreotide LAR was a dominant cost-saving strategy. When GH control was used as the efficacy measure, octreotide LAR achieved a better overall clinical efficacy with a slightly higher total costs (ï¿¥ 4 121 higher per patient per year). Sensitivity analysis didn't change the conclusion that octreotide LAR remains dominant over lanreotide SR, indicating the robustness of this model. Conclusion: Octreotide LAR achieved better overall biochemical control compared with lanreotide SR which result in less comorbidity rate, second operation and radiotherapy as well as related costs.

[The localization diagnosis of patients with adrenocorticotropic hormone-dependent Cushing's syndrome in adolescence].

Objective: To elucidate the clinical characteristics and localization diagnosis of patients with adrenocorticotropic hormone (ACTH)-dependent Cushing's syndrome (CS) in adolescence. Methods: The clinical data, laboratory examination and localization diagnosis from 35 patients aged less than 18 years old with adolescent CS who were treated at Peking Union Medical College Hospital between January 1990 and March 2012 were analyzed. Results: There were 29 cases of Cushing's disease (CD) and 6 cases of ectopic ACTH syndrome (EAS). Compared to patients with EAS, those with CD were older at diagnosis[(15.2±2.7) vs (12.8±4.4) years], and had longer disease course[(1.9±1.5) vs (0.7±0.3) years]and higher serum potassium[(3.8±0.6) vs (2.5±0.7) mmol/L], however the plasma ACTH level[(15.4±14.9) vs (42.5±22.7) pmol/L]was lower (all P<0.05). If the cut-off of the ratio of 24-hour urine free cortisol (24 h UFC) after low-dose dexamethasone suppression test (LDDST) to before LDDST was 0.65, the sensibility to diagnose CD was 70.8%, and the specificity was 100%. If the cut-off of the 24 h UFC ratio after high-dose dexamethasone suppression test (HDDST) to before HDDST was 0.54, the sensibility to diagnose CD was 91.7%, and the specificity was 100%. If the cut-off of the plasma ACTH ratio of inferior petrosal vein[bilateral inferior petrosal sinus sampling (BIPSS)]to peripheral vein was 2, only 6 CD patients (6/8) met it. Conclusion: The study suggested that HDDST was more meaningful in the localization diagnosis of patients with ACTH-dependent CS in adolescence.

[The diagnosis and treatment of systemic lupus erythematosus complicated with hydrocephalus].

Objective: To investigate the pathogenesis and management of the hydrocephalus in patients with systemic lupus erythematosus (SLE). Methods: Eight cases of hospitalized lupus patients with hydrocephalus in Peking Union Medical College Hospital from Jan 1990 to Mar 2017 were analyzed retrospectively.We collected the patients' medical records including medical history, CT and/or MRI images and analysis of cerebrospinal fluid via lumbar puncture in order to investigate the pathogenesis of the hydrocephalus in patients with SLE and summarize treatment experience. Results: All the 8 patients with SLE complicated with hydrocephalus were treated with steroids or immunosuppressive agents, and 5 cases were diagnosed with the central nervous system infection. Three cases received ventriculoperitoneal shunt, 5 cases received treatment of medicine.They all were followed up for 2-12 months, of which 3 cases were markedly effective, 1 case effective and 4 cases dead. Conclusion: The pathogenesis of lupus combined with hydrocephalus may have a certain relationship with the central infection, should take effective anti-infection treatment.We recommend cerebrospinal fluid shunt surgery intervention.

[The surgery of thyrotropin-secreting pituitary adenomas and the significance of thyroid stimulating hormone level in follow-up].

Objective: To explore the clinical characteristics of thyrotropin-secreting pituitary adenomas and the effect of the transsphenoidal approach of procedure with/without drug treatment for thyrotropin-secreting pituitary adenomas, and to investigate the correlation between the prognosis of thyrotropin-secreting pituitary adenomas and the postoperative level of thyroid stimulating hormone (TSH). Methods: A total of 45 patients with thyrotropin-secreting pituitary adenoma who hospitalized in Peking Union Medical College Hospital from 2000 to 2015 were enrolled in the study.We collected the clinical features, hormone levels, imaging findings, treatment and follow-up data of these patients. Results: The average age of the patients was 40.26 years old and the average disease duration was 5.83 years.Among them, 8 cases were microadenomas and 37 cases were macroadenoma.Thirty patients were treated with somatostatin analogues before surgery.TSH level was significantly decreased after operation (P=0.012). All of them had done the pituitary surgery, including 44 transsphenoidal surgery and 1 subfrontal surgery that had recurrence one year after the transsphenoidal surgery.The TSH level of 34 cases went back to normal on the third day after surgery.The remission rates on three months and one year after surgery in patients with TSH level<0.1 mIU/L on postoperative day (POD) 3 were significantly higher than those in patients with TSH level>0.1 mIU/L on POD3.As well as the remission rates on three months and one year after surgery in patients with TSH level normal on postoperative one month were significantly higher than those in patients with TSH level abnormal on postoperative one month. Conclusions: TSH level of postoperative one month could be used to predict prognosis and monitor recurrence.

[Surgical treatment of pituitary adenomas in childhood and adolescence].

Objective: To investigate the clinical characteristics and treatment of pituitary adenomas of childhood and adolescence. Methods: Thisarticle was a retrospective study of 140 pituitary adenomas of childhood and adolescence that was admitted into Peking Union Medical College Hospital from December 1987 to December 2014, whose clinical manifestations, hormone secretions, images, pathological types, surgical complications and follow-ups were studied. Results: A total of 58 (41.4%) males and 82 females (58.6%) were included, whose mean age was 12.5 years old (age range from 7 to 18). As for the type of tumors, ACTH adenoma, PRL adenoma, GH adenoma, non-functional adenoma and multiple-secreting adenoma accounted for 35.7%, 25.7%, 12.2%, 25.7% and 0.7%, respectively. Microadenoma accounted for 33.6% of total patients, while macroadenoma and giant adenoma accounted for 60.0% and 6.4% respectively. 19.3% of adenomas in our study wereinvasive. Transsphenoidal approach surgery was commonly used and accounted for 97.9% of cases in our study, 2 of which used intraoperative navigation assistance. Total resection was achieved in 93.6% of patients, and the rest achieved subtotal resection. 108 (77.1%) patients got full term follow-up until 1 year after surgery, and tumor recurred in 14 patients (13.0%). Conclusions: ACTH-secreting adenoma is the most common pituitary adenoma in childhood and adolescence which need surgical treatment. Transsphenoidal approach surgery is the most commonly used surgical procedure. The recurrence rate is about 13%, and the patients need regular examination of pituitary hormones and image study to confirm the recurrence.

[Analysis of curative effect of surgical therapy for male prolactinoma].

OBJECTIVE: To summarize the treatment outcome of surgery for male prolactinoma. METHODS: One hundred and eighty-four cases of male prolactinoma who underwent surgery were analyzed retrospectively.The average age of patients was 36.3 years, and the mean course was 41.9 months.The main clinical manifestation included sexual dysfunction (47.4%), headache (55.9%) and visual disturbance (46.7%). The serum prolactin levels ranged from 6.83 to 9 325.32 nmol/L.The size of tumors varied from 6 mm to 70 mm.Suprasellar adenoma with visual deficits accounted for 40.7%. 98.9% patients underwent pituitary adenoma resection via single nasal transsphenoidal approach. RESULTS: Postoperative pathological Ki-67 index of most patients (45.1%) were less than 1%.After surgical therapy, 163 patients (88.6%) got relief of symptoms, 57 patients (31.0%) achieved initial remission, and 26 patients (45.6%) unfortunately recurred. The possibility of gross resection decreased as tumor size increased (P<0.05). Preoperative PRL had no significant relation with both tumor size and extent of resection (P>0.05). The favorable prognosis predictive factors included microadenoma, intrasellar adenoma and Ki-67 index≤3 (P<0.01). CONCLUSION: Male prolactinomas is an invasive tumor with higher serum prolactin level.Tumor size could predict degree of gross resection while predictors for recurrence include tumor size, growth pattern and Ki-67 index.

[Analysis of outcomes and learning curve of endoscopic transsphenoidal surgeries for 124 patients with pituitary adenomas].

OBJECTIVE: To investigate the outcomes of the Endoscopic transsphenoidal surgery for patients with pituitary adenomas, analyze the learning curve and provide reference for future surgeries. METHODS: Retrospective analysis was carried out on 124 patients by endoscopic transsphenoidal surgery with a single neurosurgeon over a period spanning from January 2010 to January 2014 at Peking Union Medical College Hospital.The changes of endocrine and tumor imaging before and after surgery were analysed. Operative time and complication rates of one surgeon in the early period of learning curve were compared with that in later period. RESULTS: There were significant differences in Gross total resection (GTR) rate of pituitary adenomas with different sizes and different Knosp classifications (P<0.01, P<0.01). GTR rate of huge adenomas was significantly lower than that of macroadenoma and adenomas (P<0.05). GTR rate of Knosp 4 grade adenoma was significantly lower than that of Knosp 0-3 level (P<0.05). No significant difference in GTR among all types of functional pituitary adenomas and hormone levels after surgery was observed (P>0.05). In addition, no significant difference (P>0.05) in complications among different sizes, Knosp grade and type of pituitary adenomas was observed.GTR of Knosp 4 adenoma in later period of the learning curve was significantly higher than that in early period (P<0.05). Meanwhile the operative time was significantly lower than early period (P<0.05). CONCLUSIONS: Endoscopic transsphenoidal pituitary adenoma resection has the advantages of wider surgical field, higher GTR rate, less trauma, fewer complications and better life quality of patients.Through standardized learning, the GTR rate of the invasive pituitary adenomas can be improved.

[The value and comparison of plasma adrenocorticotropic hormone and urinary free cortisol in predicting long-term outcome after operation of Cushing's disease].

Objective: To study and the value of morning plasma adrenocorticotropic hormone (ACTH) and urinary free cortisol (24 h UFC) within 3 days after operation in patients with Cushing's disease in predicting the long-term outcome. Methods: The clinical data of 140 patients with Cushing's disease who were treated in Department of Neurosurgery of PUMCH from 2012 to 2014 were analyzed retrospectively.The univariate analysis, multivariate Logistic analysis, ROC curve analysis and other statistical methods were used to study the predicting value of morning plasma ACTH and 24 h UFC in 3 days post operation. Results: Univariate analysis showed that in the two groups of the early remission and no remission, there was significant statistical difference between the preoperative ACTH, preoperative 24 h UFC, postoperative ACTH and postoperative 24 h UFC (P<0.05, <0.01, <0.01). Logistic analysis showed that ACTH and 24 h UFC after operation of two groups had significant difference (P<0.01, <0.05). ROC curve analysis showed that postoperative cutoff values of ACTH and 24 h UFC were 4.11 pmol/L (18.7 pg/ml) and 281.42 nmol (102 µg)/24 h. ROC analysis was performed to evaluate the predicting performance of postoperative ACTH, resulting in an area under the curve (AUC) of 0.917 (95%CI: 0.858-0.957, P<0.01). In comparison, 24 h UFC had an AUC of 0.814 (95%CI: 0.739-0.875, P<0.01). The predicting value of ACTH is significantly better than that of 24 h UFC (P=0.005). Conclusion: Early morning 24 h UFC and ACTH within 3 days after operation both showed considerable accuracy in predicting the long-term outcome of Cushing's disease, and the significance of ACTH was even greater than that of 24 h UFC.

[Comparison of efficacy between the serum cortisol and 24 hour urine free cortisol in combined dexamethasone suppression test in the diagnosis of Cushing syndrome].

OBJECTIVE: To compare the sensitivity and specificity between the 24 hour urine free cortisol (24 h UFC) and serum cortisol in dexamethasone suppression test (DST) in the diagnosis of Cushing syndrome (CS). METHODS: Combined low dose DST (LDDST) and high dose DST (HDDST) were carried out in 67 cases of CS with surgically confirmed cases in recent 3 years(from January 2011 to November 2015). The serum cortisol and 24 h UFC were collected simultaneously for each subject and the sensitivity and specificity of serum cortisol and 24 h UFC were compared. RESULTS: There were Cushing disease (CD) group (n=53), ectopic adrenocorticotropic hormone (ACTH) syndrome group (n=7) and ACTH-independent Cushing syndrome group (n=7) according to the etiology of hypercorticordism.There were no significant differences among 3 groups in gender and age.The sensitivity of serum cortisol of different cut off points(50, 110, 140 nmol/L and 50% of control)after LDDST was 97.01%, 86.57%, 83.58% and 70.15% respectively.Meanwhile, the sensitivity of cutoff point of 24 h UFC <32 nmol in combined LDDST was 92.54% in the diagnosis of Cushing syndrome.There was no significant differences in two groups between serum cortisol <110 nmol/L and 24 h UFC <32 nmol.However, the sensitivity of serum cortisol <50 nmol/L was significantly higher than 24 h UFC<32 nmol (P<0.05). Furthermore, in combined HDDST, if the suppression rate was ≥50%, the sensitivity of serum cortisol and 24 h UFC in differentiating the etiology of Cushing disease was 60.38% and 90.57%, and the specificity was 91.43% and 96.00% respectively.There were significant differences between serum cortisol and 24 h UFC in both of sensitivity and specificity (both P<0.05). In addition, if the suppression rate of 24 h UFC in HDDST was adjusted to 60.85% according to receiver operating characteristic (ROC) curve, it could have the best levels of sensitivity (92.6%) with the specificity of 85.7%. If the suppression rate of serum cortisol was adjusted to 61.53% in HDDST according to ROC curve, it could have the best sensitivity (64.8%) with the specificity of 78.6% accordingly. CONCLUSION: In combined LDDST, the serum cortisol <50 nmol/L had a higher sensitivity than the 24 h UFC<32 nmol when they were used as the criteria in determining the diagnosis of CS.In HDDST, the sensitivity and specificity of suppression rate of 24 h UFC ≥50% were better than serum cortisol to differentiate the etiology of CS.

[Use early morning serum cortisollevel to evaluate the effect of Cushing's disease after transsphenoidal surgery].

OBJECTIVE: To predict the therapeutic effect of Cushing's disease after transsphenoidal surgery by using morning serum cortisol level. METHODS: The clinical data of 275 cases that had transsphenoidal surgery in Peking Union Medical College Hospital from 2010 to 2014 were analyzed retrospectively.Early morning serum cortisol level less than 140 nmol/L 3 days postoperation was usedto predict endocrinological remission. And long-term efficacy was evaluated by follow-up. RESULTS: Of the 275 patients, there were 49 males and 226 females; average age was 36.5 years old.Remission wasconfirmed in 201 cases, the remission rate was 73.1%, and 8 cases recurrent duringfollow-up.There were 17 macroadenomas, theremission rate was 47.1%; 258 microadenomas and MRI negative adenomas, the remission rate was 74.8%.And 43 recurrent cases had reoperations; the remission rate was 46.5%. CONCLUSION: Early morningserum cortisol 3 days post operation can evaluate the effectof transsphenoidal surgery, but even if the level of cortisol is less than 140 nmol/L, there is still tumor recurrence.Patients should be follow-up for a lifetime.

[Perioperative management and surgical treatment of neurosurgical critical patients with thrombocytopenia].

OBJECTIVE: To study the perioperative management and surgical treatment of patients with neurosurgical critical diseases and thrombocytopenia. METHODS: Eleven patients with the diagnosis of neurosurgical critical diseases and thrombocytopenia who received surgical treatment in Department of Neurosurgery, Peking Union Medical College Hospital from 2010 to 2015 were reviewed retrospectively. All eleven patients received platelet transfusions preoperatively. The safety and efficacy of surgical treatment were analyzed by GOS score at 6 months after operations. RESULTS: Platelet counts of six patients who received minimally invasive surgery reached 80×10(9) /L by platelet transfusions preoperatively. Platelet counts of five patients who received Craniotomy and ventriculoperitoneal shunt reached 100×10(9) /L by platelet transfusions preoperatively. Eight patients received platelet transfusions postoperatively according to a low platelet count 24 hours after operations. Two patients died after surgery and one died after discharge. All other eight survival patients got well recovery with an average GOS score of 4.5 at six months after operations. CONCLUSIONS: Patients with neurosurgical critical diseases and thrombocytopenia could receive surgical treatment in collaboration with relative departments.