新生儿红斑狼疮八例分析

Objective Neonatal lupus erythematosus (NLE) is an uncommon" passive autoimmune disease, which is associated with transplacental passage of maternal antibodies. It is often misdiagnosed as intrauterine infection or sepsis. The main purpose of this retrospective study was to summarize its clinical manifestations related with pathogenesis.Methods Data of all the NLE neonates, including clinical manifestations, immunochemical evidence of serum antinuclear antibodies (ANA), antibody to Ro/Sjogren's syndrome A ( anti-Ro/SSA), antibody to La/Sjogren' s syndrome B (anti-La/SSB) and anti-dsDNA antibodies in both infants and mothers, and images from head ultrasound and CT scans were analyzed. Follow-up was performed until one and half years of age or when all the clinical abnormalities had been resolved.Results Totally 8 cases (3 males and 5 females ) seen between September 2003 and February 2006 met the diagnostic criteria of NLE, in whom 4 were small for gestational age and one was born prematurely. Mean gestational age was (38.1 ± 1.9 ) weeks, mean birth weight (2605 ± 420) g, mean admission age (22.4 ± 27.7 ) days (2 hours-72 days) and mean age of onset (9.4 ± 12. 1)days (0-28 days). The common clinical manifestations included cutaneous lupus lesions (8 infants ), neural system abnormalities (2 infants ) and congenital heart block (2 infants). Annular, erythematous or desquamative lesions were seen in skin and all disappeared before 6 months of age. One patient presented with third degree atrio-ventricular block and was delivered by cesarean section because of " fetal distress" He did not recover by the end of one and half years follow-up. One infant was hypotonic with delayed neuro-motor development initially and during follow-up with both abnormal neonatal behavioral neurological assessment (NBNA) and imaging findings. Brain CT scan showed generalized low density involving periventricular and deep white matter at one week of age. At the age of one and a half years, he presented with normal mental development index determined by Child Development Center of China (CDCC) infant intelligence mensuration. Other abnormal clinical findings such as hepatosplenomegaly, anemia, thrombocytopenia, cholestasis and elevated liver enzyme activities were all resolved before 6 months of age. Only 3 mothers of the NLE infants were diagnosed as systemic lupus erythematosus (SLE) before parturition and only one received partial therapy. At least anti-Ro/SSA antibody or anti-La/SSB antibody or ANA was found in the affected patients. Seven cases had circulating anti-Ro and/or anti-La antibodies in the mothers and in the newborns, while ANA was positive in seven newborns and in all mothers. All the clinical symptoms disappeared before 18 months ot age except for congenital heart block. No special intervention was applied.Conclusions Serum auto-antibodies should be investigated to rule out NLE when a newborn infant has congenital heart block or rashes or thrombocytopenia, although there is no maternal history of SLE. Central nervous system abnormalities in NLE are likely to be a transient phenomenon and whether it will cause long-term sequelae is uncertain.

Length and thickness of 152 corpus cal osa were measured in neonates within 24 hours of birth. Using ultrasonic diagnostic equipment with a neonatal brain-specific probe, corpus cal osum length and thickness of the genu, body, and splenium were measured on the standard mid-sagittal plane, and the anteroposterior diameter of the genu was measured in the coronal plane. Results showed that corpus cal osum length as wel as thickness of the genu and splenium increased with gesta-tional age and birth weight, while other measures did not. These three factors on the standard mid-sagittal plane are therefore likely to be suitable for real-time evaluation of corpus cal osum de-velopment in premature infants using cranial ultrasound. Further analysis revealed that thickness of the body and splenium and the anteroposterior diameter of the genu were greater in male infants than in female infants, suggesting that there are sex differences in corpus cal osum size during the neonatal period. A second set of measurements were taken from 40 premature infants whose ges-tational age was 34 weeks or less. Corpus cal osum measurements were corrected to a gestational age of 40 weeks, and infants were grouped for analysis depending on the outcome of a neonatal behavioral neurological assessment. Compared with infants with a normal neurological assessment, corpus cal osum length and genu and splenium thicknesses were less in those with abnormalities, indicating that corpus cal osum growth in premature infants is associated with neurobehavioral de-velopment during the early extrauterine stage.

应用振幅整合脑电图监测体外膜肺治疗过程中的新生儿癫癎1例

体外膜肺(ECMO)通常用于挽救对呼吸机治疗无效的新生儿的生命,但经体外膜肺治疗的患儿易发生缺氧缺血性损伤、脑梗塞、颅内出血和癫癇发作,因此对神经发育造成不良影响.

Genetic analysison the association between polymorphisms of UGT1A1and GST and neonatal hyperbilirubinemia

Background: Congenital duodenal obstruction (CDO) is one of the most common anomalies in newborns, and accounting for nearly half of all cases of neonatal intestinal obstruction. This study aimed to review our single-center experience in managing congenital duodenal obstruction while evaluate the outcomes.
Methods: We conducted a retrospective analysis of the records of all neonates dianogsed with congenital duodenal obstruction admitted to our center between January 2003 and December 2012. We analyzed demographic criteria, clinical manifestations, associated anomalies, radiologic findings, surgical methods, postoperative complications, and fi nal outcomes.
Results: The study comprised 287 newborns (193 boys and 94 girls). Birth weight ranged from 950 g to 4850 g. Fifty-three patients were born prematurely between 28 and 36 weeks' gestation. Malrotation was diagnosed in 174 patients, annular pancreas in 66, duodenal web in 55, duodenal atresia or stenosis in 9, preduodenal portal vein in 2, and congenital band compression in 1. Twenty patients had various combinations of these conditions. Presenting symptoms included bilious vomiting, dehydration, and weight loss. X-rays of the upper abdomen demonstrated the presence of a typical doublebubble sign or air-fl uid levels in 68.64% of patients, and confi rmatory upper and/or lower gastrointestinal contrast studies were obtained in 64.11%. Multiple associated abnormalities were observed in 50.52% of the patients. Various surgical approaches were used, including Ladd's procedure, duodenoplasty, duodenoduodenostomy, duodenojejunostomy, or a combination of these. Seventeen patients died postoperatively and 14 required re-operation.
Conclusions: Congenital duodenal obstruction is a complex entity with various etiologies and often includes multiple concomitant disorders. Timely diagnosis and aggressive surgery are key to improving prognosis. Care should be taken to address all of the causes of duodenal obstruction and/or associated alimentary tract anomalies during surgery.

Background: Hyperbilirubinemia is a frequently seen condition in neonates. This study was undertaken to determine the role of urinary tract infections (UTIs) in the etiology of indirect hyperbilirubinemia in neonates with jaundice in their fi rst two weeks of life.
Methods: The study was conducted prospectively. The subjects were neonates aged 4-14 days with hyperbilirubinemia which could not be detected by routine tests and was suffi ciently severe to necessitate phototherapy.
Results: The study was performed in 104 neonates, of whom 18% (n=19) had UTI. The most frequently identified micro-organism was Escherichia coli (43%). Phototherapy duration and rebound bilirubin level were higher in neonates with UTI (P<0.05).
Conclusion: UTI should be investigated in neonates with hyperbilirubinemia of unknown etiology in the fi rst two weeks of life.

Background: Neonates with ABO hemolytic disease are at greater risk for developing significant hyperbilirubinemia. We aimed to determine whether sixth hour transcutaneous bilirubin (TcB) could predict such a risk.
Methods: TcB measurements were obtained at the 6th hour of life in blood group A or B neonates born to blood group O, rhesus factor compatible mothers. Subsequent hyperbilirubinemia was monitored and considered significant if a neonate required phototherapy/exchange transfusion. The predictive role of sixth hour TcB was estimated.
Results: Of 144 ABO incompatible neonates, 41(OA, 24; O-B, 17) had significant hyperbilirubinemia. Mean sixth hour TcB was significantly higher among neonates who developed significant hyperbilirubinemia than those who did not (5.83±1.35 mg/dL vs. 3.65±0.96 mg/dL, P<0.001). Sixth hour TcB value >4 mg/dL had the highest sensitivity of 93.5% and >6 mg/dL had the highest specifi city of 99%. Area under receiver operating characteristic curve was 0.898.
Conclusion: Sixth hour TcB predicts subsequent significant hyperbilirubinemia in ABO incompatible neonates.

Microbiological safety of expressed breast milk for preterm admitted to neonatal unit in china

Objective To determine the degrees and types of bacterial contamination in home-expressed mother's milk for preterm admitted to neonatal unit in China.Methods The present paper was a descriptive study enrolled a total of 75 human breast milk samples from February 2nd to March 1 st 2015.The primary outcome was microbiological features of breast milk samples collected at home.A sample of more than 104 colony-forming units/mL is considered as the significantly bacterial contaminated breast milk.Results Among the milk samples obtained from the mothers of 75 neonatal babies,69.3 ％ had substantial bacterial growth.This high contamination rate could be due to the Chinese tradition of avoiding bathing for one month after childbirth.Conclusion Un-processed breast milk expressed by mothers in home was not safe for high risk preterm babies.Health care needs to pay attention to the risk of that in China.Good hygienic practice and strict process control for breast expression,including collection,transportation and storage should be developed for Chinese mothers of hospitalized preterm infants.