ABSTRACT
Takayasu arteritis is a a rare, chronic large-vessel vasculitis that predominantly affects aorta, its major branches and the pulmonary arteries; it is the most common, granulomatous inflammation of large arteries in children.It induces a variety of nonspecific inflammatory symptoms and ischemic symptoms due to stenotic lesions.Recent advances in imaging modalities including magnetic resonance angiography, computed tomography(CT), sonography, and fluorodeoxy glucose positron emission tomography/CT(FDG-PET/CT)allow accurate diagnosis of Takayasu arteritis and shorter duration between onset of the disease and diagnosis.Medical treatment for Takayasu arteritis is also changing.In addition to the traditional glucocorticoids and immunosuppressants, many new biological agents such as TNF-α antagonists and tocilizumab are being applied to patients with Takayasu arteritis refractory to conventional treatment with favorable results.This review critically discusses recent advances in medical management of Takayasu arteritis, with a special focus on the rationale and evidence to support the use of biologic agents in this disease.
ABSTRACT
Objective:To study the respiratory morbidity and the risk factors of respiratory complications in late-preterm infants.Methods:The data of 959 late-preterm infants in 21 hospitals in Beijing from October 2015 to April 2016 were collected.These infants were divided into the respiratory morbidity group (237 cases) and the control group (722 cases) according to whether they had short-term respiratory morbidity after birth.Clinical data of the two groups were compared.Results:Among the 959 late-preterm babies, 530 were male and 429 were female.Two hundred and thirty-seven cases (24.7%) developed short-term respiratory morbidity after birth.Infectious pneumonia developed in the most cases (81 cases, 8.4%), followed by transient tachypnea (65 cases, 6.8%), amniotic fluid aspiration (51 cases, 5.3%), and respiratory distress syndrome (24 cases, 2.5%) successively.All the infants recovered and discharged.There were no differences between gender and maternal age between 2 groups (all P>0.05). Compared with the control group, more late-preterm infants were delivered by cesarean section (73.4% vs.59.7%, χ2=14.43, P<0.001) and the 1-minute Apgar score was lower [(9.41±1.66) scores vs.(9.83±0.53) scores, t=5.40, P<0.001] in the respiratory morbidity group.The differences were statistically significant.There were more cases with maternal complications in the respiratory morbidity group that in the control group (66.7% vs.58.6%, χ2=4.877, P=0.027), but no difference in various complications between 2 groups was observed ( P>0.05). In the respiratory morbidity group, the most frequent complications were maternal hypertension and preeclampsia (27.8% vs.22.6%, χ2=2.728, P=0.099). There were no differences between 2 groups in gestational age, birth weight and birth length (all P>0.05). There were more infants small for gestational age and large for gestational age in the respiratory morbidity group than in the control group (18.8% vs.14.1%, 6.3% vs.2.4%, χ2=8.960, P=0.011). The duration of hospitalization of the respiratory morbidity group was significantly longer than that of the control group [(9.00±4.42) d vs.(6.82±4.19) d, t=6.676, P<0.001] since the infants with respiratory morbidity needed to be hospita-lized. Conclusions:Respiratory diseases occur in about 1/4 of late-preterm infants.Infants who are delivered by cesarean section and whose mothers are complicated with the maternal hypertension and preeclampsia should be monitored closely.Respiratory support should be provided for infants not appropriate for gestational age who are more likely to suffer from respiratory diseases, so that they can successfully pass through the transition period.
ABSTRACT
Objective To investigate the nutritional status of vitamin D (Vit D) in newborns in Xi'an, and to provide a theoretical basis for timely and targeted formulation of regional and individualized Vit D nutrition intervention strategies. Methods A cross sectional survey was performed in newborns within 24 hours life age at the neonatal department of China Xidian Group Hospital. Serum 25-hydroxyvitamin D (25(OH)D) levels were detected and compared for the mean among groups in accordance with gestational age, birth weight and gender. Multiple linear regression analysis was used to analyze the effect of each factor on serum 25 (OH) D levels. A P-value of P 0.05). Conclusion Vit D nutritional deficiency existed in some newborns in Xi'an. Measures need to be taken to improve the status of newborn Vit D deficiency.
ABSTRACT
Objective To investigate the risk factors of hyperbilirubinemia in late preterm infants. Methods The clinical data of 815 late preterm infants (449 males and 366 females) from 25 hospitals in Beijing were collected from October 2015 to April 2016, including 340 cases(41.7％) with hyperbilirubinemia (hyperbilirubinemia group), and 475 cases without hyperbilirubinemia (control group). The clinical data of two groups were compared, and the maternal factors influencing hyperbilirubinemia in late preterm infants were analyzed with logistic regression. Results There were no significant differences in gender ratio (M:F 1.39 vs. 1.12, t=1.811,P=0.172)and birth weight[(2502.6±439.6)g vs. (2470.2±402.9)g,χ2=2.330,P=0.127)]between two groups. The incidence rates of hyperbilirubinemia in infants of 34 wks, 35 wks and 36 wks of gestational age were 22.9％(87/174), 35％(119/300) and 42.1％(143/341) respectively (χ2=1.218,P=0.544). The multivariate logistic regression analysis indicated that the maternal age(OR=1.044,95％ CI:1.010-1.080,P=0.011)was independent risk factor and multiple births(OR=1.365,95％CI:0.989-1.883,P=0.048), premature rupture of membranes(OR=2.350,95％ CI:1.440-3.833,P=0.001), cesarean section(OR=1.540,95％CI:0.588-4.031,P=0.014)were risk factors for hyperbilirubinemia in late preterm infants. Conclusions The incidence of hyperbilirubinemia in late preterm infants is relatively high. Maternal age, multiple births, premature rupture of membranes and cesarean section are risk maternal factors related to hyperbilirubinemia in late preterm infants.
ABSTRACT
Objective To evaluate the nutritional status of extremely low birth weight (ELBW) infants and the effects of nutritional support strategy alterations on their growth during hospitalization.Method From 2005 to 2014,clinical data of ELBW infants admitted to the neonatal intensive care unit (NICU) in our hospital were retrospectively analyzed.The clinical data included their general status,enteral and parental nutritional support strategy and complications during hospitalization The patients were assigned into pre5 group and late5 group.Those who survived and discharged from 2005 to 2009 were the pre5 group,and those who survived and discharged fromn 2010 to 2014 were the late5 group.The independent t test and chi square test were used for statistical analysis.Result A total of 58 ELBW infants were enrolled in the study,including 18 patients in the pre5 group and 40 in the late5 group.No statistically significant differences existed between the two groups on gestational age,birth weight,Z score (weight for length and gender),birth length,head circumference and main complications during hospitalization (P ＞0.05).Pre5 group had higher incidence of small for gestational age (SGA) than late5 group (16/18 vs.25/40,P =0.037),while the EUGR ratio at discharge (14/18 vs.21/40,P =0.061) was similar.When compared with pre5 group,late5 group had larger amount of initial enteral feeding volume [4.4 ml/(kg · d) vs.2.4 ml/(kg · d),P =0.014] and feeding volume at the end of the first week [(19.8 ± 16.0) ml/(kg · d) vs.(12.2 ±9.5) ml/(kg · d),P =0.036].Similarly,the starting dose of amino acids in parenteral nutrition [2.0g/(kg· d) vs.1.0 g/(kg· d),P＜0.001],maximum dose of amino acids [4.0g/(kg.d) vs.3.5 g/(kg · d),P ＜ 0.001],total calories at the end of the first week [(82.6 ± 12.6) kcal/(kg · d) vs.(71.1±15.2) kcal/(kg· d),P=0.004] and the second week [(103.7 ±19.8) kcal/(kg· d) vs.(92.3 ± 17.9) kcal/(kg · d),P =0.041],the weight gain velocity from birth to discharge [(18.7 ± 2.9) g/(kg.d) vs.(16.9±2.8) g/(kg· d),P=0.031] and change of Z scores (AZ) [-0.6 (-1.0,-0.4) vs.-1.2 (-1.6,-0.8),P =0.004] showed significantl differences between the two groups,with better outcomes in late5 group.However,the duration of parenteral nutrition,the total amount of amino acids,the time reaching total enteral feeding,the length of hospital stay were similar between the two groups.Ten cases(61.1％)of infants in pre5 group were breastfed,and four of them used human milk fortifier (HMF) (fortified rate was 22％).32 cases (80％) in late5 group were breastfed and 23 cases used HMF (fortified rate was 57.5％).The time to initiate HMF in the late5 group was at (30.2 ± 13.2) days,and human milk amount was (89.9 ± 34.5) ml/kg,fortified duration was (32.8 ± 15.7) days.Conclusion The enteral feeding strategy were more vigorous in the last 5 years than before,the initial feeding volume,the increasing rate,the initial dosage of amino acid,and maximum dosage of amino acid had been increased.Human milk and HMF of preterm infants were preferred.The vigorous nutritional support strategy were effective for the weight gain of ELBW infants without obvious side effects during hospitalization.
ABSTRACT
Objective To summarize the clinical features and possible impacts of Goodpasture's syndrome in pregnancy on the pulmonary and kidney of the newborn and the mothers. Methods One patient diagnosed Goodpasture's syndrome in pregnancy hospitalized in Peking Union Medical College Hospital on August 23 in 2011 delivered a neonate with bullae of lung. And literatures including 8 cases of pregnancy complicated by Goodpasture's syndrome worldwide through Medline were reviewed. Results (1) Case report:one 31-year-old women presented with acute renal failure at 30+6 weeks of gestation and delivered a male infant with birth weight 1 900 g by caesarean section at 31+1 weeks of gestation. Diagnosis was confirmed as Goodpasture's syndrome with anti-glomerular basement membrane(GBM) antibodies in serum and renal biopsy after delivery. Then she was treated with methylprednisolone, cyclophosphamide, plasmapheresis and dialysis. The neonate showed the lung bullae in the right middle lobe and bilateral intraventricular hemorrhage but renal function was transient normal with anti-GBM as 113.1 EU/ml. The baby was treated by glucocorticoid for two months and clinical symptoms were improved. Anti-GBM antibodies and chest CT showed normal. After been followed up for two years, the baby was normal. (2) Literatures review:the main manifestations of Goodpasture's syndrome in pregnancy were malignant hypertension and renal failure but respiratory symptoms were not obvious. Treated with plasmapheresis, hematodialysis and glucocorticoid maybe have good effects. Most cases had premature delivery. Neonatal anti-GBM antibodies coming from mothers could result to cerebral, renal and pulmonary injury which could be treated by glucocorticoid. Conclusions The Clinical features of pregnancy complicating the Goodpasture's syndrome are malignant hypertension and renal failure. Diagnosis was depended on positive anti-GBM antibodies and renal pathological changes and treatment were depended on plasmapheresis, hematodialysis and glucocorticoid. Neonatal cerebral, renal and pulmonary injury resulting from anti-GBM antibodies coming from mothers should be followed up, and glucocorticoid should be taken if necessary.
ABSTRACT
<p><b>OBJECTIVE</b>To investigate the clinical and genetic features of 2 patients with Rubinstein-Taybi syndrome.</p><p><b>METHOD</b>Using next generation sequencing (NGS) the CREBBP and EP300 genes of 2 children who were diagnosed as Rubinstein-Taybi syndrome at Peking Union Medical College Hospital. The mutations identified by NGS were verified by PCR were analyzed.</p><p><b>RESULT</b>The 2 patients at the age of 5 months and 4.5 years manifested short stature (the height were 60 cm and 99 cm respectively), low hairline, thick and dense hair and eyebrows, long lash, epicanthus of both eyes, protruded supercilliary arch, broad and flat thumbs and halluces, and particular facial abnormalities. Patient 2 had language retardation besides. One missense mutation of c.3535A>G, p.Ser1179Gly was found in CREBBP gene in patient 1 and one microdeletion mutation of c.4995_4999delCGCCT, p. Ala1666Pro fs66x was found inpatient 2. Both mutations were reported for the first time.</p><p><b>CONCLUSION</b>Rubinstein-Taybi syndrome is characterized by mental and growth retardation, wide and flat thumbs and first toes, and dysmorphic facial features. CREBBP is one of the causative genes. Mutation detection on CREBBP gene can confirm the diagnosis of Rubinstein-Taybi syndrome.</p>
Subject(s)
Child , Humans , Male , CREB-Binding Protein , Genetics , High-Throughput Nucleotide Sequencing , Mutation , Genetics , Mutation, Missense , Rubinstein-Taybi Syndrome , Diagnosis , GeneticsABSTRACT
Objective To observe the clinical efficacy of octreotide for treatment of bowel obstruction. Methods Ninety-five cases of bowel obstruction treated from January 2007 to July 2010 were divided into study group (48 cases) and control group (47 cases) by random digits table. Patients in control group received the conventional therapy including fasting, gastrointestinal decompression, total parenteral nutrition, sufficient fluid infusion, water-electrolyte and acetic-alkali equilibrium maintenance and infection prevention, while patients in study group received octreotide 0.1 mg by subcutaneous injection on the base of above conventional therapy, the clinical symptomatic relief time, gastrointestinal decompression volume,anus exhaust time, hospitalization time and effective power of both groups were observed and compared.Results The clinical symptomatic relief time, gastrointestinal decompression volume, anus exhaust time and hospitalization time in study group were all significantly lower than those in control group, the differences was statistically significant (P ＜ 0.05); the effective power in study group was 97.9%(47/48),which was significantly higher than that in control group(87.2% ,41/47), the difference was statistically significant(P ＜0.05). There was no obvious adverse reactions occurred, the patients of treatment futility were therapied by surgery later. Conclusion Octreotide used to treat bowel obstruction has a reliable efficacy with low reverse reaction and deserves general application.