Disorders of sex development(DSD) include anomalies of sex chromosomes,gonads,reproductive ducts,and genitalia.Here,the term "intersex" is avoided because of its imprecision.The purpose of this review is to assist health care professionals in the provision of treatment,education,and support to children born with DSD and to their families.The goal of DSD treatment is to achieve the long-term physical,psychological,and sexual well-being of the patients.In the case of DSD it involves several principles.Providing medical and surgical care to deal with a complication that threatens to the patient's physical well-being;minimizing the potential for the patient and family to feel ashamed,stigmatized,or overly obsessed with genital appearance;delaying elective surgical and hormonal treatments until the patient can actively participate in decision-making;telling the truth to the family and the child and addressing psychosocial distress of the children and their parents.This approach is termed as "patient-centered".

Objective To explore the effect of the thyroid morphologic change,the initiating treatment time and the thyroid function(serum FT_4,TSH)on mental development in children with congenital hypothyroidism (CH).Methods After diagnosed,52 cases of CH were orally administrated with L-T_4,then their mental and physical development were monitored and thyroid morphologic changes were observed by thyroid imaging(~(99m)TcO_4) and type B ultrasonography.The Bayley test was performed at the age of 10 to 30 months and expressed as mental developmental index(MDI)and psychomotor developmental index(PDI).Analysis was conducted upon treatment timing and thyroid morphologic changes.Results The MDI in those cases with morphologic abnormalities treated within 90 days was significantly lower than that with normal morphology(91?20 vs 105?15,P

Objective To explore the curative effect of endourological treatment of pediatric urethral hemangioma with holmium laser.Methods Two children with urethral hemangioma were enrolled in this study. One urethral hemangioma in the bulbous urethra,another in the posterior urethra. Modalities of diagnosis before operation included B ultrasound,intravenous urogram(IVU) and endoscopy. Two children with urethral hemangioma underwent holmium laser therapy.Results After endourological trearment with holmium laser, two children had been cured.Six months to 4 years follow-up did not find recurrent urethral hemangioma.Conclusions The diagnosis of pediatric urethral hemangioma mainly rely on endoscopy. Endourological treatment with holmium laser is a minimally invasive,safe and effective methods for pediatric urethral hemangiomas.

Hair Cells, Auditory, Inner ; cytology ; Synapses ; physiology ; ultrastructure

Acute Disease ; Adult ; Humans ; Male ; Occupational Diseases ; diagnosis ; therapy ; Phosphines ; poisoning ; Poisoning ; diagnosis ; therapy

Objective To analyze the characteristics of TCM syndromes of children abnormal sweating;To investigate the correlation between diseases and primary and secondary syndromes. Methods Through a multi-center epidemiology investigation, 400 cases were collected to statistically analyze the five TCM syndromes of children abnormal sweating, and characteristics of each syndrome were analyzed. Results In the 400 cases of children abnormal sweating, there were 129 cases (32.25%) of dysfunction of lung-wei pattern, 50 cases (12.50%) of nutrient qi and defensive qi discordance, 84 cases (21.00%) of qi and yin deficiency pattern, 57 cases (14.25%) of dampness heat forced steamed pattern, and 80 cases (20.00%) of qi and yang deficiency pattern. Spontaneous sweating and night sweating were found in the four patterns besides qi and yin deficiency pattern, and night sweating can be mainly found in qi and yin deficiency. Each type is consistent in the syndrome differentiation and cluster analysis. Conclusion Spontaneous sweating and night sweating, each has the certification of yin and yang. Spontaneous sweating can be caused by yin deficiency while night sweating can be caused by yang deficiency. Each pattern of children abnormal sweating has its own syndrome characteristic, which can provide the basis for clinical diagnosis and treatment.

To ensure the quality and safety of Panax notoginseng, a method for the simultaneous determination of 10 mycotoxins in Panax notoginseng was developed using ultra performance liquid chromatography tandem mass spectrometry (UPLC-MS/MS). The sample was extracted with acetonitrile and purified by HLB multifunction cleanup column. The separation was performed on a Phenomenex Kinetex XB-C18 column by gradient elution using methanol and 5 mmol·L(-1) ammonium acetate as mobile phase. The targeted compounds were detected in MRM mode by mass spectrometry with electrospray ionization (ESI) source operated in both positive and negative ionization modes. The linear relationships of the 10 mycotoxins were good in their respective linear ranges. The correlation coefficients (r) ranged from 0.9981 to 1.0000. The LOQs of the 10 mycotoxins were between 0.15 and 8.6 μg·kg(-1). The average recoveries ranged from 73.8% to 107.0% with relative standard deviations (RSDs) of 0.10%-10.9%. The results demonstrated that the proposed method was sensitive and accurate, and suitable for the mycotoxins quantification in Panax notoginseng.
Chromatography, High Pressure Liquid ; Chromatography, Liquid ; Drug Contamination ; Mycotoxins ; analysis ; Panax notoginseng ; chemistry ; Tandem Mass Spectrometry

Objective:To investigate the epidemiological and clinical features of patients with hemorrhagic fever with renal syndrome (HFRS) from 2017 to 2019.Methods:Seventy-five patients with HFRS from the Department of Infectious Diseases, The First Affiliated Hospital of Anhui Medical University during January 1, 2017 to December 31, 2019 were included. The data of epidemiology, clinical symptoms, blood routine, urine routine, serum creatinine, liver function and other laboratory examination indexes were retrospectively analyzed. The measurement data with skewness distribution were expressed by M( QR) and compared by nonparametric test. Multivariate logistic regression analysis was used to analyze disease-related risk factors. Results:The 75 patients were mainly located in the western and northern regions of Anhui Province. A total of 37 cases (49.3%) were infected during November, December and January next year. Fifty-four (72.0%) patients were farmers and 10(13.3%) patients had a clear history of rodent contact. Only 19(25.3%) patients had typical clinical manifestations of "three red and three pain" . Fifty-eight (77.3%) patients had elevated white blood cell count, 67(89.3%) patients had decreased platelet count, 55(73.3%) patients had urinary protein + + + , 65(86.7%) patients had abnormal urinary occult blood, and 67(89.3%) patients had elevated serum creatinine. The serum creatinine and potassium levels in 31 severe and critical patients were 495(301) μmol/L and 4.14(0.77) mmol/L, respectively, which were both higher than those in 44 mild and moderate patients (235(289) μmol/L and 3.65(1.02) mmol/L, respectively). The differences were both statistically significant ( Z=-3.187 and -2.796, respectively, both P<0.01). Multivariate logistic regression analysis showed that serum creatinine (odds ratio ( OR)=1.005, 95% confidence interval ( CI)1.002-1.008) and serum potassium ( OR=2.632, 95% CI 1.098-6.313) were independent risk factors for disease severity. All patients received comprehensive medical treatment, and 27 patients received renal replacement therapy. Sixty-eight patients had good prognosis and four patients died. Conclusions:HFRS is still common in the rural area in winter and spring. Patients with atypical clinical manifestations and severe and critical patients should be intensively monitored.

Objective To explore the guidance significance of the functional MRI and DTI (fMRI ,DTI) ,intraoperative ultra-sound(IOUS) ,neuronavigation ,electrocorticography(EcoG) monitoring used in surgical treatment of low-grade gliomas with epi-lepsy as main symptom located near the eloquent brain regions .Methods 23 neurosurgical patients in the First Affiliated Hospital of Chongqing Medical University during 2009-2010 were performed the retrospective analysis .The preoperative fMRI ,DTI deter-mined the positional relation between the lesions with the conduction bundle and the eloquent brain regions ,the electrophysiological and imageological examinations positioned the epileptogenic focus and lesions ,the MRI-mediated neuronavigation system was adopt-ed to formulate the surgical plan and choose the best surgical approach ,IOUS was used to perform the realtime monitoring for pre-cisely positioning the lesion range and determining the extent of resection ,and the intraoperative EcoG was adopted to determine the epileptogenic focus localization ,the lesions and the epileptogenic focus was dealed by the operating microscope for avoiding the func-tional region ,and the patient′s prognosis was evaluated and recored in detail after operation .Results By the precisely positioning the lesions and epileptogenic focus by fMRI ,DTI ,neuronavigation and ultrasound ,the lesion resection degrees by the operative mi-croscope and intraopertaive pathological guidance were 17 cases of Ⅰ-Ⅱ grade ,4 cases of Ⅲ grade and 2 case of Ⅳ-Ⅴ grade .1 case of motor aphasia ,4 cases of hemiplegia and monoplegia and 1 case of disturbance of consciousness after operation were improved by the treatment of neurotrophy ,dehydration and hyperbaric oxygen and discharged from hospital with rehabilitation .No death case occurred .The evaluation of the life quality :20 cases ofⅠ-Ⅱ grade ,3 cases of Ⅲ grade and no vegetable survival case of Ⅳ grade . The evaluation of resection clinical effect :20 cases of cure ,3 cases of improvement ,no case of as before and exacerbation .After fol-lowed up for 6-24 months ,according to Engel classification of seizure efficacy assessment :Ⅰ-Ⅱ grade in 21 cases ,Ⅲ grade in 2 case ,no case of Ⅳgrade .Conclusion fMRI ,DTI ,neuronavigation ,IOUS and EcoG for guiding the operation of low grade gliomas located near the eloquent brain regions can resect the lesion to the largest extent and simultaneously deal with epileptogenic focus , effectively protect the neurological function of the functional region ,greatly reduce the side-injury of the normal brain tissues in the functional region ,at the same time increase the curative effect of symptomatic epilepsy .

Objective To evaluate the effect on the reversal of left ventricular aneurysm (LVA) and on heart function of percutaneous coronary intervention (PCI) therapy at different time of acute myocardial infarction (AMI). Methods A total of 326 patients with primary anterior AMI-accompanied LVA diagnosed by left ventriculography (LVG) from January 2001 to July 2004 were enrolled in this study. They were randomized into 4 groups according to the time of accepting PCI:≤3 h group, 4-6 h group, 7-12 h group and one week group. At the baseline and 6 months after AMI, the parameters of left ventricular diastolic volume index (LVEDVI), left ventricular systolic volume index (LVESVI), left ventricular ejection fraction (LVEF), left ventricular wall motion score (LVWMS) and left ventricular end diastolic pressure (LVEDP) were measured by LVG. The paradox volume index (PVI) was measured by equilibrium radionuclide at one week and 6 months after AMI.At 3 year following up to, the major adverse cardiac events (MACE) were recorded. Results At 6 months after PCI, the LVESVI, LVEDVI, WMS and LVEDP were all decreased while LVEF was increased in the four groups as compared with pre-PCl (P<0.05, respectively). Those parameters changed most obviously in ≤3 h group. At the 6th month after PCI, the PVI was lower in ≤3 h group (12.1±2.1)% than in 4-6 h, 7-12 h and one week group [(15.4±2.4)%, (16.5±2.5)% and (20.4±3.7)%, all P<0.05]. Within the 3 years follow-up, the MACE was significantly lower in 3 h, 4-6 h and 7-12 h groups than in one week group, and the mortality was lower too [(2.8%, 3.0% and 3.1% vs. 17.9%, all P<0.05]. Conclusions The early, fully and permanent open of the infraction-related artery can effectively inhibit the left ventricular remodeling process, prevent LVA formation, improve LV function and prognosis.