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Hereditary medullary thyroid carcinoma (hereditary MTC) is a rare malignancy, accounting for 25-30% of all MTC. It occurs as part of multiple endocrine neoplasia type 2 (MEN 2). Autosomal dominant gain-of-function mutations in the RET proto-oncogene is the cause of the disease, in which the common mutations are codons 609, 611, 618, 620, 630, 634 and 918. In recent years, the spectrum of RET gene mutations has changed. The classical mutations reduced, whereas the less aggressive mutations increased. Hereditary MTC is a time-dependent disease. Stages of the disorder at diagnosis can significantly influence survival rates. Based on the genotype-phenotype, RET mutations have been classified into four risk levels by American Thyroid Association (ATA) at 2009. The classification system guides the hereditary MTC management, including risk assessment, biochemical screenings and surgical intervention. Though the application of genetic testing and codon-specific phenotypes in hereditary MTC diagnosis is effective with high accuracy, there are some difficulties in implementing RET gene testing as a routine for MTC diagnosis. And most of carriers with RET mutations did not undergo thyroidectomy at the age recommended by the ATA guidelines. The aim of the study is to review the hereditary MTC and discuss the management dilemma.

The aim of this study is to identify clinical, etiologic, and laboratory factors that potentiate adverse outcome of hyperbilirubinemia among term and late preterm neonates in logistic regression analysis. A retrospective cohort of infants with total serum bilirubin (TSB) ≥ 20 mg/dL from 1995 to 2007 was surveyed. Eighteen infants had adverse outcome. Controls were 270 infants without sequelae. Risks were significantly higher in infants with six etiologies causing hyperbilirubinemia: sepsis (odds ratio [OR] = 161.7, 95% confidence interval [CI] = 11.7 to 2242.8), gastrointestinal obstruction (OR = 39.2, 95% CI = 2.7 to 567.3), Rh incompatibility (OR = 31.0, 95% CI = 5.1 to 188.9), hereditary spherocytosis (OR = 19.6, 95% CI = 1.6 to 235.5), ABO incompatibility (OR = 5.1, 95% CI = 1.3 to 19.7), and glucose-6-phosphate dehydrogenase deficiency (OR = 4.7, 95% CI = 1.3 to 16.7). Infants with acute bilirubin encephalopathy were more likely to have adverse outcome than subjects without acute bilirubin encephalopathy (OR = 281.7, 95% CI = 25.8 to 3076.7). Adverse outcome was more common in infants with a positive direct Coombs test (OR = 4.5, 95% CI = 1.3 to 15.4). Infants with hemoglobin < 10 g/dL tended to have adverse outcome more often than those with hemoglobin ≥ 13 g/dL (OR = 11.8, 95% CI = 3.3 to 42.9). Infants with TSB of 35 mg/dL or more (OR = 472.5, 95% CI = 47.8 to 4668.8) and of 30 to 34.9 mg/dL (OR = 9.5, 95% CI = 1.6 to 57.9) carry greater risks as compared with those with TSB of 20 to 24.9 mg/dL. In conclusion, this study quantitatively verified the potential risks for adverse outcome of neonatal hyperbilirubinemia.

Objective: To estimate the incidence and related risk factors of sepsis between road traffic injuries (RTIs) and non-RTIs. Methods: Clinical data of 339 patients with severe trauma who were admitted into ICU in both Third Affiliated Hospital of Army Military Medical University and ChongGang General Hospital from January 2012 to December 2015 were retrospectively analyzed. Twenty items of potential risk factors affecting sepsis were evaluated by univariate and multivariate Logistic Analysis with the purpose of drawing a comparison between RTI patients and non-RTI patients. Results: There were 154 cases of RTI and 185 cases of non-RTI entering the study period. The significant independent risk factor of sepsis in RTIs was SOFA 11 (OR=4.821; 95% CI=1.901-12.226; P=0.001). The significant independent risk factors of sepsis in non-RTIs were SOFA 11 (OR=12.410; 95% CI=2.559-60.185; P=0.002), tracheal intubation (OR=8.913; 95% CI=2.322-34.206; P=0.001), APACHE II 15 (OR=3.684; 95% CI=1.750-7.753; P=0.001). Conclusions: The clinical medical personnel should not give equal treatment to RTI patients and non-RTI patients admitted in ICU in that factors predicting sepsis within above two groups are different. The sample volume should be increased and validated in further prospective research.

To study the use of propofol sedation in patients undergoing continuous venous-venous haemodiafiltration (CVVHDF) and assess the curative effects.A retrospective study was conducted. Data from 74 patients with continuous sedation of propofol in emergency intensive care unit (ICU) from April 2009 to June 2011 were analyzed. There were 26 cases suffering from acute renal failure in CVVHDF group and 48 critical cases in non-CVVHDF group. Propofol administration duration, dose, the clearance rate and the average recovery time in both groups were analyzed, and plasma concentrations of propofol at inflow side and outflow side of CVVHDF circuit in 26 patients with CVVHDF were determined.Compared with non-CVVHDF group, it took a longer administration time (298.37±28.73 hours vs. 173.44±17.27 hours, P0.05) and higher dose (35.89±0.76 g vs. 21.82±0.62 g, P0.05) in CVVHDF group. There were no statistical significances on clearance rate at 2, 6, 24 hours after administration (2 hours: 13.85±1.15 ml/min vs. 14.41±1.21 ml/min, 6 hours: 5.92±0.52 ml/min vs. 6.32±0.59 ml/min, 24 hours: 4.75±0.41 ml/min vs. 5.33±0.45 ml/min) and recovery time (8.89±1.46 minutes vs. 8.47±1.37 minutes) between CVVHDF and non-CVVHDF groups (all P0.05). Plasma concentrations of propofol at inflow side and outflow side of CVVHDF circuit were not different after propofol administration in 26 CVVHDF patients.Compared with patients in non-CVVHDF group, the patients who received propofol in CVVHDF required higher total dosage and longer delivery time to maintain a good sedation. In both two groups clearance rate of propofol and maintenance of a stable blood concentration were the same. The use of a proper dose of propofol in CVVHDF did not affect wake up time of the patients, there were no complications.

To observe proliferation and differentiation of osteoblasts cultured in the plane on appropriate electrical stimulation, to specify whether it promote the proliferation, and observe expression of BMP-2 on electrical stimulation.Osteoblasts were extracted from the skull of rabbit offspring and cultured. Cells after the 2nd generations were cultured. In experimental group, cells had electrical stimulation, and same stimulation time and intensity were given. In control group cells had not electrical stimulation. The proliferation and differentiation were detected at different time, and BMP-2 protein expression was analyzed.The cell morphology of experimental group in 8 days under the light microscope was observed and showed a lot of proliferation of osteoblasts, pleomorphic changes, in 6 to 8 days a small amount of Calcified spots was also observed; while in the control group, proliferation was slower. Differentiation of the experimental group was significantly, alkaline phosphatase staining and calcium nodules were positive, quantitative analysis of alkaline phosphatase increaseed significantly. Experimental group showed that BMP-2 was gradually increased by immunohistochemistry analysis.Electrical stimulation can promote the proliferation and differentiation of osteoblasts and achieved the increasement the number of cells in short-term, intracellular staining by immunohistochemistry showed the increasement in expression of BMP-2.

To discuss surgical skills,precautions and complications of using titanium elastic intramedullary nails in the treatment of adult midshaft clavicular fractures and evaluate the therapeutic efficacy.From June 2006 to January 2009, 28 patients with completely displaced midshaft clavicular fractures (15 simple fractures, 8 wedge fractures and 5 comminuted fractures) were enrolled in the study,included 19 males and 9 females with a mean age of 39.0 years (range 19-67 years). The injury was on the left side in 14 cases and on the right side in 14 cases. The mean course of disease was 2.9 days. The shoulders of the patients were swollen, deformed and disabled before operation. X-rays revealed complete displacement of the clavicle. The mean clavicular shortening after injury was 6.76% vs. that measured after bone healing. The Constant-Murley Shoulder Scoring System was used to assess shoulder function, and the DASH Score was used to assess the disability degree of the upper arm.Closed operation with titanium elastic intramedullary nails was undertaken in 26 cases, and open reduction was performed in the remaining two cases. Satisfactory reduction was achieved in all patients, who were followed up for a mean of 10 months (range 6-15 months). The mean union time was 11.5 weeks. No severe complication occurred in any patient. The mean clavicular shortening after bone healing was 3.38%, which was significantly different as compared with the mean clavicular shortening after injury. Constant-Murley Shoulder Score was (97.0 +/- 4.2), and DASH score was (3.4 +/- 4.8). Anatomical reduction, functional recovery and appearance were satisfactory in all patients.Treatment of adult midshaft clavicular fractures with titanium elastic intramedullary nails is advantageous and may prove to be an alternative option for nonsurgical treatment and plate osteosynthesis of midshaft clavicular fractures in adults.

To examine the effects of various concentration of nicotine on bone marrow stromal cells (BMSCs) proliferation and differentiation of cartilaginous in vitro.BMSCs was obtained from femoral bone and tibia of New-Zealand albino rabbit. The cells of the 3rd generation were used in study. Different concentration of nicotine (0, 1 x 10(-7), 1 x 10(-6), 1 x 10(-5) M) were added into BMSCs. BMSCs proliferation was analyzed by MTT assay at the 1, 4, 7, 14 days. The expression of collagen type II and aggrecan as the marker genes of cartilaginous differentiation from BMSCs were detected by reverse transcriptase-polymerase chain reaction (RT-PCR).Microscope showed that BMSCs transformed from round to fusiform shape. The concentration of nicotine in 1 x 10(-7), 1 x 10(-6) M had a significant positive effect on cell proliferation and the expression of type II collagen in a time-dependent manner when supplemented in commonly used induction media (P0.05). Concentrations of nicotine in 1 x 10(-7) can promote the expression of aggrecan at the 7th day after induction,and in 1 x 10(-5) M may inhibit the expression of type II collagen and aggrecan.It was implied that local application of nicotine at an appropriate concentration may be a promising approach for enhancing cartilaginous differentiation capacity of BMSCs in cartilage tissue engineering.

Our objective was to identify the association between maternal diet with Chinese herbal medicines and prolonged jaundice of breast-fed infants. Healthy infants at 25 to 45 days of age were eligible for enrollment into this prospective study. Jaundice was defined as a transcutaneous bilirubin (TcB) value ≥ 5 mg/dL. A questionnaire survey asking feeding type, stool pattern, and maternal diet was conducted at the time of TcB measurement. A total of 1148 infants were enrolled, including 151 formula-fed, 436 combination-fed, and 561 breast-fed infants. The incidences of jaundice were 4.0% in formula-fed infants, 15.1% in combination-fed infants, and 39.8% in breast-fed infants (P<0.001). In addition, jaundice was noted in 37.1% of preterm infants and 25.0% of term infants (P<0.001). Furthermore, jaundice was more common in breast-fed infants whose mothers did not consume the traditional Chinese herbal medicines than in breast-fed infants whose mothers did consume such medicines (P<0.001). In conclusion, this cohort study has identified late-preterm birth and breast feeding as the contributory factors for prolonged jaundice of apparently well infants. The data indicate that postpartum diet with Chinese herbal medicines is associated with breast milk jaundice.

To evaluate the preliminary clinical outcomes of coccygectomy in patients with coccydynia after a failure of conservative treatment.From May 2002 to January 2010, 31 patients with coccydynia were treated by coccygectomy in our department after conservative measures had failed to produce significant relief. A questionnaire, which included the extent of relief in the painful area, improvement in quality of life, intensity of pain in the sitting position, and pain score during daily activities, was used to evaluate the results.All patients were followed up for 1 to 6 years (mean 3.3 years). The results were excellent in 20 patients (64.5%), good in 7 patients (22.6%), moderate in 3 patients (9.7%) and poor in 1 patient (3.2%). The excellent and good rates amounted to 87.1%. All patients except one had complete resolution of their symptoms and were subjectively highly satisfied with the outcomes of the surgery. Only 2 cases of superficial infection were observed postoperatively.Coccygectomy is a feasible management option for patients with coccygodynia that has no response to conservative treatments.

To evaluate the feasibility and therapeutic effect of treating sternoclavicular joint dislocation by K-wire and tension band wire fixation, and to improve the safety and stability of this technique.This study consisted of 9 cases, 6 males and 3 females with the mean age of 25 years (range, 9-62 years). The causes were traffic accident in 7 cases, falling in 1 case and fight in 1 case. The duration from injury to operation was 2 hours to 7 days. There were 5 left dislocations and 4 right dislocations; 8 anterior dislocations and 1 posterior dislocation, including one combined with left scapular fracture and one with left olecranon fracture. Open reduction and internal fixation using K-wires and tension band wires were performed to treat dislocations.All patients were followed up for 6 to 24 months, 10 months on average. According to Rockwood's rating scale on postoperative sternoclavicular joint, 8 cases achieved excellent outcomes with an average score of 13.88, and the rest case achieved a good outcome with the score of 12. Anatomical reduction was obtained in all cases. There were no such postoperative complications as severe infection, injury to blood vessel and nerve, failure of fixation, etc. Patients were all satisfied with the anatomical reduction and functional recovery.The technique of K-wire and tension band wire fixation is safe, simple, effective, less invasive and has been successfully used in orthopedic surgery. It is effective in treating sternoclavicular joint dislocation though it has some disadvantages.

Background and objective : In the literature, there are conflicting data on the neonatal outcome in preterm infants who were delivered for maternal pre-eclampsia. The purpose of this study is to investigate the effect of maternal pre-eclampsia on neonatal morbidity and 2-year developmental outcome in a population of preterm infants delivered before 32 weeks of gestation. Methods : The hospital records of all 89 surviving VLBW infants with GA below 32 weeks born from January 1997 to December 1999 were reviewed retrospectively. Data on respiratory outcome, sepsis and intraventricular hemorrhage (IVH) were compiled and analyzed for their association to maternal pre-eclampsia. Seventy-eight infants were assessed employing the Bayley Scales of Infant Development for developmental outcome at 2 years of corrected age. Results : There was no difference in neonatal morbidity between groups. More infants born to pre-eclamptic mothers had lower MDI scores at 24 months of age ( P =0.04) as compared to infants without maternal pre-eclampsia. After multiple logistic regression analysis, pre-eclampsia ( P =0.007, OR=10.8) remained a significant risk factor of mildly delayed MDI at 24 months of age. Conclusion : Delivery before 32 weeks because of pre-eclampsia was associated with an increased risk of poor cognitive outcome. There was no significant difference in the postnatal course in comparison with infants born after pregnancies not complicated by pre-eclampsia.

Background Hyperbilirubinemia is a common disorder during neonatal period in Taiwan. Gene variants may play an important role in the development of neonatal hyperbilirubinemia. The current study investigated the association between neonatal hyperbilirubinemia and common gene variants involving the production and metabolism of bilirubin. Methods This prospective study enrolled 444 healthy infants born in the Chang Gung Memorial Hospital at Taipei from 2013–2015. Hyperbilirubinemia was defined as a total bilirubin ≥ 15 mg/dL. A log-binomial model was used to assess the risk of gene variants. Results The most common genetic variant was short heme oxygenase (HO)-1 promoter GT-allele (

Background Formula-fed (FF) infants often have harder stools and higher stool concentrations of fatty acid soaps compared to breastfed infants. Feeding high sn-2 palmitate or the prebiotic oligofructose (OF) may soften stools, reduce stool soaps, and decrease fecal calcium loss. Methods We investigated the effect of high sn-2 palmitate alone and in combination with OF on stool palmitate soap, total soap and calcium concentrations, stool consistency, gastrointestinal (GI) tolerance, anthropometrics, and hydration in FF infants. This double-blind trial randomized 165 healthy term infants 25–45 days old to receive Control formula (n = 54), formula containing high sn-2 palmitate (sn-2; n = 56), or formula containing high sn-2 palmitate plus 3 g/L OF (sn-2+OF; n = 55). A non-randomized human milk (HM)-fed group was also included (n = 55). The primary endpoint, stool composition, was determined after 28 days of feeding, and was assessed using ANOVA accompanied by pairwise comparisons. Stool consistency, GI tolerance and hydration were assessed at baseline, day 14 (GI tolerance only) and day 28. Results Infants fed sn-2 had lower stool palmitate soaps compared to Control (P =0.0028); while those fed sn-2+OF had reduced stool palmitate soaps compared to both Control and sn-2 (both P