Host defense deficiency in newborn nonhuman primate lungs

We have investigated two major aspects of the pulmonary host defense mechanism--alveolar macrophage function as a "first line of bacterial defense" and induced neutrophil migration into the lung as a "back-up defense." Chemotactic and phagocytic/killing assays revealed a functional deficiency in the alveolar macrophages of newborn primates. Serial bronchoalveolar lavage investigations revealed diminished neutrophil migration into the newborn primate lung. The overall pulmonary host defense capability in newborn primates was deficient. The results of this investigation may have direct clinical relevance to the susceptibility of newborns to infections and pneumonia.     

严重缺碘对体质及遗传性状影响的研究

对严重缺碘地区一个容貌特殊,身材较矮,智力低下的人群进行了体质特征及遗传性状的研究,并与国内有关本地区的调查资料进行了对照,提出人类体质特征和遗传性状除与人种、地理环境异同直接相关外,人体不可缺少的微量元素的摄入水平在一定程度上对其也产生影响。并且认为同一人种、民族居住同一地理位置所产生的体质差异应从水文、地质、生活方式、生活水平的不同进行综合分析。     

维生素D缺乏与儿童反复呼吸道感染的关系及机制

呼吸道感染是常见的儿科疾病,有着显著的发病率和死亡率,反复呼吸道感染会严重影响儿童的生活质量和身体健康。维生素D具有一系列与人体健康有关的作用,包括与呼吸道的感染有关。为了分析维生素D与儿童反复呼吸道感染的关系,探讨维生素D影响呼吸道感染的机制,本研究通过临床分析了维生素D缺乏与儿童反复呼吸道发生之间的相关性,通过构建炎症模型,观察了外源维生素D对人气道上皮细胞增殖分化的影响,运用ELISA检测了维生素D对人气道上皮细胞和中性粒细胞释放炎症因子(IL-1β,IL-6和TNF-α)的影响。本结果表明,维生素D缺乏与儿童反复呼吸道感染相关,并且维生素D能促进人气道上皮细胞的增殖分化,但对人气道上皮细胞和中性粒细胞炎症因子的产生无显著影响。     

Adenylosuccinate Lyase Deficiency—First British Case

A deficiency of adenylosuccinate lyase (ASDL) is characterised by the accumulation of SAICAriboside (SAICAr) and succinyladenosine (S‐Ado) in body fluids. The severity of the clinical presentation correlates with a low S‐Ado/SAICAr ratio in body fluids. We report the first British case of ADSL deficiency. The patient presented at 14 days with a progressive neonatal encephalopathy and seizures. There was marked axial and peripheral hypotonia. Brain MRI showed widespread white matter changes. She died at 4 weeks of age. Concentrations of SAICAr and SAdo were markedly elevated in urine, plasma and CSF and the SAdo/SAICAr ratio was low, consistent with the severe phenotype. The patient was compound heterozygous for 2 novel ADSL mutations; c.9 G > C (A3P) and c.572 C > T (R190X).     

The effect of reticulocyte hemoglobin content on the diagnosis of iron deficiency anemia: A meta-analysis study

BackgroundIron deficiency anemia (IDA) is the most common type of anemia worldwide and has many adverse effects on life quality. This meta-analysis study aims to show that reticulocyte hemoglobin content (CHr) is more effective than routinely used parameters in the diagnosis of IDA.MethodsComprehensive and systematic research was done using international databases including PubMed, Web of Science, Cochrane Library, Science Direct, and Google Scholar, which contain all articles published on IDA until December 29, 2020. Seventeen articles were included in the meta-analysis.ResultsThe analyses found the Cohen''s deffect size (Standardized Mean Difference) values of the parameters. Accordingly, CHr is 2.84 (95% CI 2.36 to 3.31), mean corpus volume (MCV) is 2.46 (95% CI 1.97 to 2.95), ferritin is 2.37 (95% CI 1.63 to 3.11), and transferrin saturation (TSAT) is 3.76 (95% CI 2.14 to 5.38). To diagnose IDA, the sensitivity value of the CHr concentration was found as 83.5% (95% CI 76.1 to 89.8), specificity value to be 91.8% (95% CI 85.5 to 96.4), and mean cut-off value as 28.2 pg.ConclusionsThe results of our study reveal the findings that CHr is a better biomarker than MCV and ferritin used in determining IDA, and its efficacy is lower than TSAT. It is very important to use it routinely for the pre-diagnosis of IDA, which is very important for public health. The groups in the study are heterogeneous but contain bias. Therefore, meta-analyses of studies with less heterogeneity of CHr are needed.     

Oxidative DNA base damage, antioxidant enzyme activities and selenium status in highly iodine-deficient goitrous children

The objective of this study was to investigate oxidative DNA damage, and the levels of antioxidant enzymes (AOE) and selenium (Se) in relation to iodine deficiency and/or goiter in children. The study was performed in a group of goitrous high school children (15-18 years of age) ( n =14) with severe or moderate iodine deficiency. Thyroid hormones (TSH, FT 4 , TT 4 , FT 3 , TT 3 ), urinary iodine (UI) and plasma Se levels, and erythrocyte glutathione peroxidase (GSHPx), superoxide dismutase (SOD) and catalase (CAT) activities were determined and compared with those of a control group consisting of non-goitrous high school children ( n =14) with normal UI levels or mild iodine deficiency. In the goitrous group, concentrations of FT 4 , TT 4 , plasma Se and UI, and activities of GSHPx and SOD were found to be significantly lower. Six typical hydroxyl radical-induced base lesions in genomic DNA of peripheral blood were identified and quantified by gas chromatography/isotope-dilution mass spectrometry (GC/IDMS), and higher levels of DNA base lesions were observed in the goitrous group. The results suggest that highly iodine-deficient goitrous children may be under oxidative stress, which may lead to greater level of oxidative damage to DNA. This study supports the evidence for the reported relationship between iodine deficiency and the increased incidence of thyroid malignancies.     

Leaf anatomy and chloroplast ultrastructure of Mn-deficient orange plants

Leaf samples of Mn-deficient and Mn-sufficient (control) ‘Navelate’ orange plants grown in a greenhouse were taken to investigate the effects of Mn deficiency in leaf structure and chloroplast ultrastructure. Total leaf chlorophyll concentration was significantly lower in Mn-deficient plants than in control ones. Entire lamina thickness was not altered due to Mn deficiency. However, Mn deficiency resulted in disorganization of mesophyll cells, mainly of palisade parenchyma cells. The number of mesophyll chloroplasts per cellular area and their length were both affected negatively. The membranous system of chloroplasts was also disorganized. The percentages of starch grains and plastoglobuli per chloroplast of Mn-deficient leaves were significantly greater than those of control leaves.     

Strategies for Correcting Very Long Chain Acyl-CoA Dehydrogenase Deficiency

Very long acyl-CoA dehydrogenase (VLCAD) deficiency is a genetic pediatric disorder presenting with a spectrum of phenotypes that remains for the most part untreatable. Here, we present a novel strategy for the correction of VLCAD deficiency by increasing mutant VLCAD enzymatic activity. Treatment of VLCAD-deficient fibroblasts, which express distinct mutant VLCAD protein and exhibit deficient fatty acid β-oxidation, with S-nitroso-N-acetylcysteine induced site-specific S-nitrosylation of VLCAD mutants at cysteine residue 237. Cysteine 237 S-nitrosylation was associated with an 8–17-fold increase in VLCAD-specific activity and concomitant correction of acylcarnitine profile and β-oxidation capacity, two hallmarks of the disorder. Overall, this study provides biochemical evidence for a potential therapeutic modality to correct β-oxidation deficiencies.     

Investigation of the Effects of Some Drugs and Phenolic Compounds on Human Dihydrofolate Reductase Activity

Dihydrofolate reductase (DHFR) plays a fundamental role in cellular metabolism and cell growth. Inhibition of this enzyme will cause a decrease in the amount of folate that occurs in many metabolic processes, and the deficiency of which may cause various diseases. This study investigated the effects of some drugs and phenolic compounds on DHFR activity in vitro. To determine the inhibitory effect of compounds, enzyme activity was measured with a final concentration of an inhibitor ranging from 10 μM to 51 mM. DHFR was inhibited effectively by naringin, ferulic acid, and levofloxacin with IC₅₀ values under 660 μM. Syringic acid, cefepime, ceftizoxime, cefazolin, ceftriaxone, and ceftazidime exhibited inhibitory effects on the enzyme activity with IC₅₀ values in the range of 3.840–30.224 mM. K_i constants were calculated using the Cheng–Prusoff equation. K_i constants calculated in the range of 0.009–2.024 mM with respect to nicotinamide adenine dinucleotide phosphate oxidase (NADPH) and in the range of 0.060–5.830 mM about FH₂.