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Objective The report was focused on polycystic ovary syndrome(PCOS) in female adolescents related to homosexual precocious puberty,and the new awareness of its influence on long-term health of homosexual precocious girls.Methods A girl with idiopathic central precocious puberty (ICPP) but diagnosed as PCOS in adolescence was reported and the data were reviewed.Results A girl was diagnosed as ICPP when she was near 8 years old with obesity,but without hyperinsulinemia,then she received the treatment of a 3.75 mg dose gonadotropin hormone analogues (GnRHa) every 28 days.Her gonads development was under control while her growth was arrested.Growth hormone(GH) injection started at 13 months.She stopped all medications when finished GH and GnRHa treatment for 12 months and 25 months.She went to see doctors again because of no menarche after discontinuing medication for nearly 2 years.She appeared obese and acne,hirsutism,athanens negricans and purple purple striae on the skin.Hyperinsulinemia and hypertestosterone were demonstrated.Pelvic B ultrasonography showed polycystic ovary,and she was diagnosed as PCOS.She was ordered to lose weight and to take metformin.And adrenocorticotropic hormone stimulating test was done,and B ultrasonography again ruled out atypical congenital adrenal hyperplasia and tumor of adrenal gland.She got her menarche 1 month later.Twelve months after the PCOS diagnosis and treatment,she had 4 menses,her insulin level decreased,glucose tolerance and her serum testosterone level turned normal.At the same time,the form of ovarian and follicular was significantly reduced.After taking mefformin for 20 months,her height and weight did not change,her menstrual was regular every 35-40 days,each time lasting about 7 days.She was followed up.We also reviewed literatures and learnt that precocious puberty might not be a benign situation and it might have an intrinsic relation to obesity,precocious puberty and PCOS.It might be the cofactors for causing PP and PCOS that accessed luteinizing hormone(LH) secretion and disorder of hypothalamus pituitary gonad(HPG) axis except obesity with hyperinsulinemia.Reported pubertas praecox in childhood developed to PCOS at 30％ with high prevalence of obesity.GnRHa suppressive therapy might relate to PCOS and had disputation for improving final height.There were fewer investigations on adolescent PCOS,and no consensus guideline on it in China.Conclusions Girls with ICPP may develop to PCOS some time later.The clinical features of the reported girl and the knowledge from literatures support the hypothesis that inner relationship between the CPP and the PCOS.The LH high secretion and disorder of HPG axis may be the causes of them; LH treatment on suppressing obesity should be cautious during treatment.The benefit and risk from GnRHa treatment should be evaluated thoroughly.Further clinical research should be conducted on adolescent PCOS.

A novel method based on cross sensitivity of cataluminescence (CTL) generated on the surface of a nanometer composite oxide was proposed for simultaneous determination of benzene and trimethylamine (TMA) in air. A variety of nanometer composite oxides based on Y2 O3 that showed catalytic activity to many gas molecules were synthesized. For the fabrication of the detector, nanometer composite oxide was directly coated on the ceramic rod to form a 0. 1-0. 15 mm thick layer. The ceramic rod with nanometer composite oxide was inserted into a quartz tube with an inner diameter of 10 mm. The temperature of nanometer composite oxide was controlled by the digital heater. When gas samples passed through the nanometer composite oxide in the quartz tube by the air flow, the CTL was generated during the catalytic oxidation on the surface of the nanometer composite oxide. The CTL signals were respectively recorded by two ultra weak chemiluminescence analyzers. The CTL intensity and selectivity for the determination of benzene and TMA on nano- Zr3 Y2 O9 which was characterized by TEM were bigger and better than those on other nanosized composite oxides. The optimum experimental conditions were tested. Selective determination was achieved at a wavelength of 440 nm for benzene and 540 nm for TMA. The surface temperature of the nanometer materials was about 313 ℃. The flow rate of air carrier was about 140 mL/ min. The limit of detection of this method was 0. 30 mg / m3 for benzene at 440 nm and 0. 70 mg / m3 for TMA at 540 nm. The linear range of CTL intensity versus concentration of benzene at 440 nm was 0. 8-105. 0 mg / m3 , benzene at 540 nm was 3. 0-130. 0 mg / m3 , TMA at 440 nm was 2. 5-232. 0 mg / m3 and TMA at 540 nm was 1. 2-156. 0 mg / m3 . The recovery of 5 testing standard samples by this method was 96. 8% -102. 3% for benzene and 97. 6% -103. 4%for TMA. Common coexistence matters, such as formaldehyde, ethanol, acetone, ammonia, sulfur dioxide and carbon dioxide, did not disturb the determination. The relative standard deviation (RSD) of CTL signals of a continuous 200 h detection of gas mixture of 50 mg / m3 benzene and 50 mg / m3 TMA was 2. 0% , which demonstrated the longevity and steady performance of nano-Zr3 Y2 O9 to benzene and TMA under this experimental conditions.

A novel method based on cross sensitivity of cataluminescence (CTL) on nano-Ti3CeY2O11 was proposed for simultaneous determination of formaldehyde, benzene and sulfur dioxide in air.The relations between the concentrations of formaldehyde, benzene and sulfur dioxide and their CTL intensities were respectively ascertained at three wavelengths.The accurate concentrations of formaldehyde, benzene and sulfur dioxide can be calculated by superimposed total CTL intensities.The three analysis wavelengths are 420 nm, 535 nm and 680 nm.The surface temperature of the sensitive materials is 280℃.The carrier gas flow rate is 130 mL/min.The detection limits (3σ) are 0.04 mg/m3 for formaldehyde, 0.05 mg/m3 for benzene and 0.10 mg/m3 for sulfur dioxide, respectively.The linear ranges of CTL intensity versus analyte concentration are 0.08-75.60 mg/m3 for formaldehyde, 0.1-101.40 mg/m3 for benzene and 0.3 to 115.00 mg/m3 for sulfur dioxide.The recoveries of 12 testing standard samples by this method are 96.4%-103.7% for formaldehyde, 97.8%-102.5% for benzene and 97.2%-103.3% for sulfur dioxide.Common coexisting substances, such as acetaldehyde, toluene, hydrogen sulfide, ammonia, methanol, ethanol and carbon dioxide, do not disturb the determination.The relative deviation of CTL signals of continuous 200 h detection for gaseous mixture containing formaldehyde, benzene and sulfur dioxide is less than 2%, which shows the longevity of the nanometer composite oxide to formaldehyde, benzene and sulfur dioxide.This method makes full use of the cross sensitive phenomenon, and can realize the on-line analysis of formaldehyde, benzene and sulfur dioxide in air.

Objective · To explore the relationship between baseline lipid profiles and long-term cardiovascular outcomes after intervention with hypoglycemic drugs metformin and glipizide and to detect lipid components that can predict the long-term cardiovascular effect of metformin and glipizide.Methods· Liquid chromatography-quadrupole time of flight-mass spectrometry (LC-QTOF/MS) was used to measure 119 lipid components in baseline serum for 116 patients with type 2 diabetes (T2DM) and atherosclerotic heart disease (CHD) who were treated with glipizide (56 cases,the glipizide group)or metformin (60 cases,the metformin group).Cardiovascular complex end points (including cardiovascular death,all-cause death,nonfatal myocardial infarction,nonfatal stroke,and arterial revascularization) of all patients were followed up.The relationship between lipid components and cardiovascular complex end points was analyzed with Logistic regression analysis.The category-free net reclassification index (cfNRI) and the integrated discrimination improvement (IDI) were used to evaluate whether lipid components are helpful for predicting the recurrent cardiovascular events.Results· The differences in baseline drug distribution,clinical characteristics,and biochemical indexes between two groups were not statistically significant,except for diuretics use,serum PC (O-34:2) level,and SM (d18:0-24:0) level.Logistic regression analysis showed that baseline ChE (20:4) was a protective factor for recurrent cardiovascular events in the glipizide group (OR=0.87,P=0.039).ChE (20:4) significantly increased the cfNRI and IDI of cardiovascular complex end points by 69％ and 0.07,respectively (P=0.011,P=0.028).Baseline SM (dl 8:1-22:0) was a risk factor for recurrent cardiovascular events in the metformin group and all participants (OR=1.65,P=0.039;OR=1.64,P=0.014).SM (d18:1-22:0) significantly increased the cfNRI of cardiovascular complex end points in the metformin group and all participants by 74％ and 55％,respectively (P=0.012,P=0.005).Conclusion· Of 119 lipid components measured with LC-QTOF/MS,baseline ChE (20:4) is a protective factor and SM (d18:1-22:0) is a risk factor for cardiovascular complex end points in with T2DM and CHD patients after long-term treatment with metformin and glipizide.Both lipid components are helpful for improving the prediction of recurrent cardiovascular events.

Resistance to the thyroid hormone is a rare and hereditary thyroid disease,the signs and symptoms are diverse and the onset is hidden.It is often misdiagnosed as a common thyroid disease and given inappropriate diagnosis and treatment.Improper diagnosis and treatment would cause adverse effect on growth and development of sick children.It is necessary to improve awareness,understanding and mastery of this disease,so as to improve the level of clinical diagnosis and treatment.

OBJECTIVETo summarize the clinical features of idiopathic hypogonadotropic hypogonadism (IHH) diagnosed during childhood, and detect mutations in KAL1 and FGFR1, acting as key clues for diagnoses.

METHODWe collected and analyzed clinical data of 21 cases (including demographic data, chief complaint, history of present illness, family history, physical examination, laboratory tests and imaging studies, etc.) diagnosed with IHH from December 2008 to February 2013. Polymerase chain reaction and gene sequencing was applied to detect mutations on KAL1 and FGFR1. Fifty healthy unrelated individuals were choosen as controls.

RESULTOf 21 patients with IHH, 19 were males and 2 females, they visited us initially from 8-17 years old, with an average of (13.58 ± 2.38) years old. Sixteen cases were KS patients (76%). One boy reported abnormal sense of smelling but having olfactory perfect picture on MRI; 2/19 male cases had no puberty when they were over 13-14 years old without abnormal external genitalia. 8/19 cases only had small penis, 8/19 had both of cryptorchidism and small penis, and the Case 2 also had hypospadias. One boy had cryptorchidism combined with a normal penis. Only 2 girls diagnosed as IHH who visited us because of no puberty signs when they were 13 and 16 years old, respectively. Other clinical manifestations included: one with gynecomastia, 2 had mental retardation, and one was deaf; one with high palatal arch; one with mirror-movement and one with left renal agenesis but normal renal function respectively. Laboratory tests showed that the basic testosterone (T) is low and with inappropriately low or normal gonadotropin hormones. The results of cases of standard human chorionic gonadotropin (HCG) test of 7 cases out of 19 male children's were normal (testosterone>1 100 ng/L), and another nine cases continued to complete the extended HCG test, and the testosterone levels of two of them (cases 6, 8) were still lower than 1 000 ng/L. Family history: the parents in 9/21 family had delayed puberty, involving only one parent in 6 families, involving both in 2 families and the other one was an uncle having micropenis with a child. Among these 21 cases, only one boy's father had hyposmia and his first emission age was 14-15 years. Eleven patients accompanied abnormal sense of smelling and the olfactory organ abnormalities on MRI, 4 had olfactory organ abnormalities on MRI while they had good smelling function self-reportedly. We got 15 samples (12 KS and 3 nIHH cases) to screen the mutation of KAL1 (14 exons) and FGFR1 (18 exons). A splicing mutation c.1062+1G>A in KAL1 is identified in case 17 with IHH. One novel heterozygous FGFR1 mutation, a single base deletion mutation on the exon 1 c.27delC is identified in case 14. This mutation causes the premature termination codons.

CONCLUSIONThis pilot research showed that IHH/KS diagnosis in children depends on clinical manifestation rather than gene analysis. Small penis or cryptorchidism, smelling abnormality and positive familial history may contribute to the KS/HH diagnosis. MRI of olfactory bulb acts as important proof for diagnosis of KS. Mutations in KAL1 and FGFR1 gene are not main causes of Kallmann syndrome.

Adolescent ; Child ; DNA Mutational Analysis ; Exons ; genetics ; Extracellular Matrix Proteins ; genetics ; Female ; Heterozygote ; Humans ; Hypogonadism ; diagnosis ; genetics ; Kallmann Syndrome ; genetics ; Male ; Mutation ; genetics ; Nerve Tissue Proteins ; genetics ; Olfaction Disorders ; Receptor, Fibroblast Growth Factor, Type 1 ; genetics ; Sexual Maturation

Objective To investigate the prevalence and risk factors of H-type hypertension among middle-aged and elderly people in Beijing community. Methods A cross-sectional study by recruiting 1 458 middle-aged and elderly people from a cohort of the "Beijing Longitudinal Study of Aging" in 2009 was conducted. All participants were asked to complete a standardized questionnaire, physical examination and laboratory examinations. Hyperhomocysteinemia was defined as homocysteine (Hcy) > 15 μmol/L, and H- type hypertension was defined as having hypertension and hyperhomocysteinemia simultaneously. The prevalence of H-type hypertension was estimated by using the results of 2000 Beijing population census to weight the data. Multivariate Logistic regression analysis was preformed to estimate the associated factors of H-type hypertension. Results The age was (69.48 ± 8.09) years, and the distribution of Hcy was skewed with the median of 16.56 μmol/L. After weight, the prevalence of hyperhomocysteinemia was 57.20% (834/1 458), and the prevalence of H-type hypertension was 35.32% (515/1 458), accounting for 59.47% (515/866) in patients with hypertension. The ageing, male, hyperuricemia, estimated glomerular filtration rate (eGFR) and insufficiency of fishes/ shrimps were independent risk factors of H-type hypertension ( OR = 2.30, 1.04, 1.02, 0.95 and 0.67; 95% CI 1.54-3.44, 1.02-1.06, 1.04-1.06, 0.94-0.97 and 0.46-0.97; P＜0.01 or＜0.05). Conclusions The proportion of H-type hypertension is high in the middle-aged and elderly people in Beijing community. Lowering uric acid, protection of renal function and intake sufficient fishes and shrimps might prevent and control H-type hypertension.

Calcitonin ; therapeutic use ; Humans ; Hypercalcemia ; drug therapy ; etiology ; In Situ Hybridization, Fluorescence ; Williams Syndrome ; complications ; drug therapy

BACKGROUNDIt has been indicated that autologous hematopoietic stem cell transplantation (AHST) is a promising treatment to adults with type 1 diabetes, however, the application of AHST therapy to children with type 1 diabetes still needs more data. The aim of this study was to assess the clinical effect of immune intervention combined with AHST and conventional insulin therapy in the treatment of children with newly diagnosed type 1 diabetes.

METHODSThis 1:2 matched case-control study was comprised of 42 children who were newly diagnosed with type 1 diabetes in the Department of Endocrinology, Beijing Children's Hospital from 2009-2010. The case group included 14 patients, who were treated with AHST within the first 3 months after being diagnosed with diabetes at request of their parents during 2009-2010. The control group included 28 patients with newly diagnosed type 1 diabetes at the same period of hospitalization. We compared the baseline and follow-up data of them, including ketoacidosis onset, clinical variables (glycosylated hemoglobin (HbA1c), insulin dosage and serum C-peptide).

RESULTSThe clinical characteristics of the patients was comparable between the case group and the control group. At 6-12 months ((10.7±4.2) months) after AHST treatment, we found 11 patients in the case group did not stop the insulin therapy, three cases stopped insulin treatment for 2, 3 and 11 months, respectively. No diabetic ketoacidosis (DKA) occurred after transplantation in all the patients in the case group. HbA1c in the control group was significant lower than that in the case group (P < 0.01), while the insulin dosage and serum C-peptide were not significant different between the two groups (P > 0.05). In order to eliminate the honeymoon effect, we performed final follow-up at the 3-5 years ((4.2±1.8) years) after AHST treatment, and found that HbA1c in the control group was still lower than that in the case group (P < 0.01); however, the insulin dosage and serum C-peptide were not significantly different between the two groups (P > 0.05). Moreover, the insulin dosage was not significant different from baseline to follow-up period in the case group.

CONCLUSIONAHST treatment showed no advantage in effectiveness in children with newly diagnosed type 1 diabetes, both in insulin dose and long term blood glucose control.

Adolescent ; Case-Control Studies ; Child ; Child, Preschool ; Diabetes Mellitus, Type 1 ; drug therapy ; therapy ; Female ; Hematopoietic Stem Cell Transplantation ; Humans ; Hypoglycemic Agents ; therapeutic use ; Infant ; Insulin ; therapeutic use ; Male ; Transplantation, Autologous ; Treatment Outcome