Comparative examinations of gastric juice in healthy infants and in infants suffering from enteritis (author's transl)]

There are significant differences between the shapes of curves of gastric juice volume, acid quantity, pH levels, and peptic activity, in the three age groups of healthy infants under examination. Significant differences are also seen in all three age groups with regard to the quantity of acid calculated in relation to 1 ml gastric juice, as well as the relative peptic activity (volume activity). The amount of gastric juice is proportional to the bodyweight and body surface of the infants, whereas acid concentration and peptic volume activity show a relatively steeper increase.     

Safety and pharmacokinetics of multiple doses of recombinant human CuZn superoxide dismutase administered intratracheally to premature neonates with respiratory distress syndrome

OBJECTIVES: To examine the safety and pharmacokinetics of multiple intratracheal (IT) doses of recombinant human CuZn superoxide dismutase (rhSOD) in premature infants with respiratory distress syndrome who are at risk for developing bronchopulmonary dysplasia (BPD). Methods. Thirty-three infants (700 to 1300 g) were randomized and blindly received saline, 2.5 mg/kg or 5 mg/kg rhSOD IT within 2 hours of surfactant administration. Infants were treated every 48 hours (as long as endotracheal intubation was required) up to 7 doses. Serial blood and urine studies, chest radiographs, neurosonograms, SOD concentration and activity measurements, and tracheal aspirate (TA) inflammatory markers were assessed throughout the 28-day study. RESULTS: SOD concentrations in serum (0.1 [0.05/0.15] microg/mL-geometric mean with lower/upper confidence intervals), tracheal aspirates (TA) (0.2 [0.1/0.3] microg/mL) and urine (0.3 [0.2/0.4] microg/mL) were similar at baseline in all 3 groups and did not change significantly in the placebo group. In the rhSOD treatment groups, SOD concentrations were increased on day 3 and did not change significantly thereafter over the 14-day dosing period (also measured on days 5, 7, and 13). SOD concentrations averaged 0.4 [0.3/0.5] microg/mL in serum, 0.8 [0.6/1.2] microg/mL in TA and 1.1 [1.0/1.3] microg/mL in urine for the low-dose group and 0.6 [0.5/0.7] microg/mL in serum, 1.1 [0.9/1.5] microg/mL in TA, and 2.2 [1.6/2.9] microg/mL in urine for the high-dose group over the 14-day dosing period. Enzyme activity directly correlated with SOD concentration and rhSOD was active even when excreted in urine. TA markers of acute lung injury (neutrophil chemotactic activity, albumin concentration) were lower in the rhSOD agroups compared with placebo. No significant differences in any clinical outcome variable were noted between groups. CONCLUSIONS: These data indicate that multiple IT doses of rhSOD increase the concentration and activity of the enzyme in serum, TA and urine, reduce TA lung injury markers and are well-tolerated. Further clinical trials examining the efficacy of rhSOD in the prevention of BPD are warranted.     

Trends in practice characteristics: analyses of 19 periodic surveys (1987-1992) of Fellows of the American Academy of Pediatrics

OBJECTIVE: To examine 6 years of practice characteristics data of Fellows of the American Academy of Pediatrics (AAP), focusing on sex differences for specialty area, primary activity, practice setting, and practice location. METHODS: We analyzed data from 19 Periodic Surveys that were fielded between 1987 and 1992. The Periodic Survey is used to survey AAP members regularly about current issues in pediatric practice. There are no duplicate respondents in these analyses of the first 19 Periodic Surveys. We collapsed the 19 surveys into the years in which they were fielded, and analyzed sex differences for each of the 6 years. In addition, we ran logistic regressions on several questions, including all 16 868 respondents, to examine how the characteristics of the specialty have been affected by the increase in the number of female pediatricians, controlling for survey year, age of respondents, and specialty area practiced. RESULTS: The proportion of nonresident AAP members who are female has grown throughout the 6 years; in 1987, 26.9% were female, and in 1992, 36.4% were female. For 5 of the 6 years there were sex differences in specialty area, usually concerning pediatric subspecialties. Substantial sex differences occurred in primary activity, in which each year women were more likely than men to be salaried. Men were more often in group practices, whereas women were generally more likely to practice in hospitals or clinics. Logistic regression demonstrated that there are sex differences in practice characteristics across time, but there is also a substantial change in practice characteristics accountable to survey year, eg, a pediatrician of either sex was 75% more likely to be salaried in 1992 than in 1987. CONCLUSIONS: Throughout the 6-year period, AAP members became increasingly more likely to practice general pediatrics, to be salaried, and to be younger-all effects independent of sex, all effects stronger for females. Rapid transformations in the health care system will likely reduce current sex differences in practice characteristics of the future.     

Congestive heart failure caused by vitamin D deficiency?

We describe a child, 3.5 months old, with severe vitamin D deficiency, profound hypocalcaemia, hyperphosphataemia, dilated left ventricle, severely reduced myocardial contractility and congestive heart failure. She also had depressed thyroid function with subnormal thyroxine and non-detectable serum thyrotropin (TSH) levels. The child promptly responded to calcium infusions, conventional anticongestive therapy and calcitriol. She is now 3 years old and received no medication. Myocardial function is normal but she has motor delay. We believe that her transitory congestive heart failure was caused by severe vitamin D deficiency with profound hypocalcaemia.     

Collagen type VI expression during cardiac development and in human fetuses with trisomy 21

The role played by specific extracellular matrix molecules in normal endocardial cushion differentiation into valves and septa remains to be established. In this respect, type collagen VI is of particular interest because genes encoding the alpha1 and alpha2 chains are located on chromosome 21, and defects involving the atrioventricular (AV) cushions are frequent in trisomy 21. Collagen VI expression was studied in normal human embryonic and fetal hearts (5-18 weeks of development) and compared by immunohistochemistry with results from fetuses (10-16 weeks of development) with trisomy 21. During normal endocardial cushion differentiation (5-8 weeks) there was marked collagen VI expression in the AV cushions, whereas only minor expression was seen in the outflow tract cushions. In the normal fetuses (10-18 weeks), collagen VI in the AV cushions had condensed into a marked zone on the atrial side of the leaflets, as well as subendocardially in other regions of high shear stress. Morphological defects involving the endocardial cushion-derived structures were present in all trisomy 21 cases. An abnormally large membranous septum was observed in three cases. An AV septal defect (AVSD) was present in two, while one had a ventricular septal defect (VSD). Two cases presented with a secondary atrial septal defect (ASDII), and one had an AVSD. Mild to moderate valve dysmorphia was found in all cases. Collagen VI staining in trisomy 21 was more intense than in the normal subjects; however, there were no differences in the spatial expression patterns. We conclude that collagen VI is expressed in the AV cushions and persists during valve differentiation. Collagen VI is more prominent in fetal trisomy 21 hearts than in normal hearts. We hypothesise that collagen VI has a role in the development of heart defects involving endocardial cushion differentiation-specifically in the AV canal, the most common site of malformations affecting children with trisomy 21.     

Applicability of LDLR flanking microsatellite polymorphisms for prenatal diagnosis of homozygous state for familial hypercholesterolemia

Analysis of newly identified microsatellite polymorphisms flanking the low density lipoprotein receptor (LDLR) gene was undertaken in the kindred of a child with apparent homozygous LDLR deficiency. The applicability of these approaches to prenatal diagnosis is considered and compared with previous approaches applying functional studies of the LDLR in amniotic fibroblasts.     

The survival motor neuron protein in spinal muscular atrophy

The 38 kDa survival motor neuron (SMN) protein is encoded by two ubiquitously expressed genes: telomeric SMN (SMN(T)) and centromeric SMN (SMN(C)). Mutations in SMN(T), but not SMN(C), cause proximal spinal muscular atrophy (SMA), an autosomal recessive disorder that results in loss of motor neurons. SMN is found in the cytoplasm and nucleus. The nuclear form is located in structures termed gems. Using a panel of anti-SMN antibodies, we demonstrate that the SMN protein is expressed from both the SMN(T) and SMN(C) genes. Western blot analysis of fibroblasts from SMA patients with various clinical severities of SMA showed a moderate reduction in the amount of SMN protein, particularly in type I (most severe) patients. Immunocytochemical analysis of SMA patient fibroblasts indicates a significant reduction in the number of gems in type I SMA patients and a correlation of the number of gems with clinical severity. This correlation to phenotype using primary fibroblasts may serve as a useful diagnostic tool in an easily accessible tissue. SMN is expressed at high levels in brain, kidney and liver, moderate levels in skeletal and cardiac muscle, and low levels in fibroblasts and lymphocytes. In SMA patients, the SMN level was moderately reduced in muscle and lymphoblasts. In contrast, SMN was expressed at high levels in spinal cord from normals and non- SMA disease controls, but was reduced 100-fold in spinal cord from type I patients. The marked reduction of SMN in type I SMA spinal cords is consistent with the features of this motor neuron disease. We suggest that disruption of SMN(T) in type I patients results in loss of SMN from motor neurons, resulting in the degeneration of these neurons.      

Cervista和MALDI-TOF-MS HPV法用于子宫颈癌筛查效果评价检测

目的：中美合作深圳万例子宫颈癌筛查项目（SHENCCASTⅡ）,主要研究目的就是评价人类乳头状瘤病毒（HPV）作为子宫颈癌初筛方法的可行性。笔者报道研究项目中期评估数据,以HybridCaptureII（HC-II）法HPV检测为对照,评价新的CervistaHPV检测方法和MALDI-TOF-MSHPV基因分型检测技术（MALDI-TOF-MS）在宫颈癌筛查中的效果。方法：本研究拟募集深圳市区、郊区及周边农村地区1万名年龄25岁至59岁、至少3年未参加过子宫颈癌筛查、未接受过子宫切除术及放疗、有过性生活的非孕期女性为筛查对象。宫颈细胞由医生取样,收集在PreservCyt细胞保存液中,首先液基细胞学制片,然后进行HC-Ⅱ、Cervista和MALDI-TOF-MS3种方法的HPV检测。细胞学≥ASCUS及任一方法HPV检测结果阳性者均进一步行阴道镜下四象限多点活检及ECC病理诊断。结果：5316例参加筛查女性中,5043例数据齐全。HC-Ⅱ、Cervist、MALDI-TOF-MS3种HPV检测方法HPV阳性率分别是：14.6%,12.2%,14%。发现CIN2以上病变150例,CIN3以上病变97例,宫颈癌5例。对于发现CIN2及以上病变,HC-Ⅱ、Cervista、MALDI-TOF-MS3种HPV检测的敏感性、特异性、阳性预测值、阴性预测值分别是：94.7%、87.9%、19.3%、99.8%;90.7%、90.2%、22%、99.7%和92.7%、88.4%、19.7%、99.8%。对于检出CIN3及以上病变,HC-Ⅱ、Cervista、MALDI-TOF-MS的敏感性、特异性、阳性预测值、阴性预测值分别是：97%、87%、12.8%、99.9%;91.8%、89.3%、14.4%、99.8%和94.9%、87.6%、13.1%、99.9%。Cervista和HC-Ⅱ、MALDI-TOF-MS的敏感性、特异性比较有统计学差异（P〈0.01）,但MALDI-TOF-MS和HC-Ⅱ比较没有显著差异。结论：HPV检测作为人群为基础的子宫颈癌筛查的初筛方法是可行的,MALDI-TOF-MSHPV检测的敏感性、特异性可与目前公认的金标准HC-Ⅱ法HPV检测相媲美,同时又因其检测的高通量、相对较低的价格,以及可行基因分型而更具应用前景。     

Perceived prevalence of peanut allergy in Great Britain and its association with other atopic conditions and with peanut allergy in other household members

BACKGROUND: Despite increasing awareness of peanut allergy, little is known of its prevalence. We report on a two-stage interview survey conducted in Great Britain. METHODS: A total of 16434 adults (aged 15+ years) reported their own allergies and atopies and named cohabitants with peanut allergy (stage 1). Follow-up interviews were conducted with identified sufferers from peanut allergy (stage 2). RESULTS: At stage 1, peanut allergy was reported in 58 respondents and 205 other household members. When we accounted for cases where peanut allergy was unconfirmed or newly reported at stage 2, the prevalence, based on 124 confirmed sufferers, was estimated as 0.48% (95% confidence interval 0.40%-0.55%). The prevalence in children (0.61%, 0.41%-0.82%) was slightly higher than in adults; age-of-onset was strikingly earlier. Prevalence was strongly associated with other atopies, particularly tree-nut allergy. Cases tended significantly to cluster in households. Half of cases had never consulted a doctor. Exactly 7.4% reported being hospitalized after a reaction. CONCLUSIONS: Peanut allergy is reported by 1 in 200 of the population and is commoner in those reporting other atopies. The fact of similar rates in children and adults argues against a recent marked rise in prevalence. The frequency and potential lethality of this disorder emphasize the need for sufferers to demographic factors, other food allergies, atopic conditions, and allergy in family/household members. Our study comprised a screening survey and detailed interviews with sufferers identified. The frequency and potential lethality of this disorder emphasize the need for sufferers to receive correct medical advice on management [corrected].