Treatment of Pericardial Disease

The pericardium is composed of visceral and parietal components. In view of the pericardium's simple structure, pathologic processes involving it are understandably few. However, despite a limited number of clinical syndromes, the pericardium is affected by virtually every category of disease, including infectious, neoplastic, immune—inflammatory, metabolic, iatrogenic, and traumatic. Thus, the recognition of pericardial heart disease remains challenging. Treatment of pericardial disease is also problematic in that there is a paucity of randomized, placebo‐controlled trials from which appropriate therapy may be selected and important clinical decisions assisted. This article reviews pericarditis and its sequelae, pericardial effusions, cardiac tamponade and constrictive pericarditis,     

The Spectrum of β-Thalassemia Mutations in Hamadan Province,West Iran

β-Thalassemia (β-thal) is one of the most common hemoglobinopathies worldwide and is caused by mutations on the β-globin (HBB) gene. The aim of the present study was to determine the mutation spectrum of the β-globin gene in β-thal carriers who were originally from Hamadan Province, Western Iran. Two hundred and eighty-two β-thal carriers participated in the study. Amplification refractory mutation system-polymerase chain reaction (ARMS-PCR) and direct sequencing were used for detection of different mutations. A total of 25 different mutations, including 21 β-thal mutations and four other hemoglobin (Hb) variants, in 280 β-thal carriers (99.3%) were detected in the present study. Three types of mutations including IVS-II-1 (G>A) (HBB: c.315+1G>A) (26.24%), codons 8/9 (+G) (HBB: c.27_28insG) (14.54%) and codons 36/37 (–T) (HBB: c.112delT) (12.76%) accounted for more than 50.0% of the identified mutations. Moreover, IVS-I-110 (G>A) (HBB: c.93-21G>A), codon 44 (–C) (HBB: c.135delC) and IVS-I (25?bp deletion) (HBB: c.93-21_del), had frequencies of 7.09, 7.09 and 5.67%, respectively. Allele frequencies of the remaining 19 mutations were less than 5.0%. This study is the first comprehensive study on a large sample size in Hamadan Province, Iran. In conclusion, the present study significantly increased the spectrum of HBB gene mutations in Hamadan Province compared with previous studies. Therefore, these results can be helpful in identifying β-thal carriers and at-risk fetuses through prenatal diagnosis (PND).     

Detection of filaggrin gene mutation (2282del4) in Pakistani Ichthyosis vulgaris families

The aim of this study was to detect an 811 bp filaggrin (FLG) gene fragment known to carry a mutation 2282del4 which causes ichthyosis vulgaris. Seven clinically examined ichthyosis vulgaris families were included in this study. An 811 bp FLG gene fragment was targeted in the genomic DNA of all the members of the seven families by PCR amplification using known primers RPT1P7 and RPT2P1. Successful amplification of an 811 bp FLG gene fragment in all the families suggested the possible role of the 2282del4 mutation in causing ichthyosis vulgaris in Pakistani population.     

Oxidized low-density lipoprotein is negatively correlated with lecithin-cholesterol acyltransferase activity in type 2 diabetes mellitus

IntroductionIt is now believed that the oxidative modification of plasma lipoproteins enhance their atherogenicity in patients with type 2 diabetes. Because a variety of highly reactive lipid peroxidation products can transfer from oxidized low-density lipoprotein (ox-LDL) to high-density lipoprotein -cholesterol, the authors evaluated the association between ox-LDL and lecithin-cholesterol acyl-transferase (LCAT) activity, a key enzyme in reverse cholesterol transport and HDL remodeling.MethodsA total of 45 patients with diabetes and 45 age-, sex- and body mass index-matched healthy adult volunteers were enrolled. Fasting blood samples were obtained, and plasma glucose, lipid profile, creatinine, insulin, ox-LDL and LCAT activity were measured. Homeostasis model assessment of insulin resistance was also calculated.ResultsPatients with diabetes, compared with healthy participants, had a significantly higher ox-LDL (17.16 ± 3.75 U/L versus 7.93 ± 1.92 U/L, P < 0.001) and lower LCAT activity (73.7 ± 9.1 μmol/L/hr versus 88.7 ± 4.5 μmol/L/hr, P < 0.001). The higher level of LCAT activity completely disappeared after adjustment for ox-LDL. LCAT activity had a significant (P < 0.001) inverse correlation with ox-LDL (r = -0.77) in patients with diabetes and healthy participants (r =-0.75).ConclusionLCAT activity is significantly decreased in type 2 diabetes. The lower LCAT activity in type 2 diabetes might be through ox-LDL mechanism. ox-LDL may adversely affect high-density lipoprotein -cholesterol metabolism by reducing LCAT activity.     

Radioisotope choroid plexography. A preliminary report

Summary Radioisotope plexography seems as easy and innocuous as routine brain scanning. An introductory method is given which could be improved. The choroid plexuses of the four ventricles were well visualized in 15 patients. Consideration is given to the possible labeling process. Besides its morphological value, the test could give interesting clues in the dynamic study of hydrocephalus.     

Pneumographic signs of poorly compensated hydrocephalus]

A definition of "Hydrocephalus not completely arrested" is not easy to be given. Cerebral pneumography represents the most detailed neuroradiological examination. In spite of this, neither its analysis nor its measurements bring definite criteria for the favorable moment of locating a shunting system in that case. These criteria are reviewed systemetically. It is pointed out that only a careful synthesis of all signs can give a univoque answer, may be doubtful but never wrong. It is suggested from the personal experience that radioisotope cisternography is better suited for judging the state of compensation of an hydrocephalus. Radioisotopes do that without undue risks. It may be but one good reason for explaining the annual decrease in the number of pneumo-encephalographies (PEG) registered almost everywhere in the world. Other factors may be involved. No doubt that PEG has lost its first place in the study of patients who may have some kind of non tumoral hydrocephalus.     

Significance of bacteria in the mucilage phenomenon in the northern Adriatic Sea

Episodes of massive mucilage formation in the northern Adriatic Sea have been recorded for over a century but their cause is still a matter of conjecture and debate. It is generally thought that mucilage forms due to copious polysaccharide exudation by phosphorus limited algae. In this paper we develop the thesis that bacteria play major roles in mucilage formation. We argue that mucilage is largely produced as a consequence of bacteria-organic matter interactions and bacterial capsular polysaccharide synthesis. Ectohydrolytic enzymes of bacteria are critical in producing long-lived polysaccharides. Further, bacteria cause efficient P regeneration, particularly intensely in microscale features e.g. phycospheres, detritus and aggregates. Bacteria thus help sustain high rates of primary production despite vanishingly low levels of phosphorus in the bulk phase seawater. We integrate these roles of bacteria into a conceptual model which emphasizes microscale interactions of microbes within a seawater gel matrix as the basis for a mechanistic understanding of the accumulation of long-lived polysaccharide to form mucilage.