فصلنامه آزمایشگاه و تشخیص
پیاپی 33 (پاییز 1395)

Niemann-Pick disease (NPD) refers to a group of lysosomal storage diseases with autosomal recessive inheritance pattern that cause abnormal metabolism of lipids. NPD clinically is placed into four different types, A, B, C and D. Types A and B occur due to defects in SMPD1 gene involved in metabolism of sphingomyelin. Types C and D are caused by mutations in NPC1 and NPC2 genes involved in metabolism of cholesterol. Diagnosis of the disease is based on clinical assessment and then biochemical and finally molecular genetic testing. Molecular genetic testing, include sequencing, use of multigenic panel as well as indirect methods using polymorphic markers located in the proximity of the genes associated with the disease. In this article the information regarding the Niemann-Pick disease and its diagnosis with emphasis on types A and B was reviewed.

Fungal sinusitis cases can be classified into three main categories: Allergic Fungal Sinusitis, Fungus ball, and Invasive form. Invasive Fungal Sinusitis is further subdivided into three categories: Acute/fulminant, Chronic, and Granulomatous. Dematiaceous fungi are common causes of both Chronic Invasive Sinusitis and Allergic Fungal Sinusitis(AFS). They are also may cause sinus fungus balls.
Allergic Fungal Sinusitis :It is estimated to cause approximately 7% of chronic sinusitis cases for which surgery is required. Diagnosis requires operative specimens to be processed appropriately for histologic examination and fungal culture. Historical and laboratory evidence of type I hypersensitivity is suggestive of this diagnosis. Histopathologic examination is particularly important, because it allows for the fungal cause to be established and is the basis for distinguishing AFS from Chronic Fungal Sinusitis. AFS is characterized by the presence of Allergic mucin and by the absence of tissue invasion. The most commonly implicated Dematiaceous organisms include: Bipolaris, Curvularia, Exserohilum, Alternaria, and Cladosporium species.
Chronic Fungal Sinusitis:The same organisms that have been implicated in AFS can also cause a slowly progressive but invasive form of sinusitis. The diagnostic approach is similar to that for AFS. The presence of fungal elements in the mucosal tissue or bone by definition indicate invasive chronic sinusitis, and rules out AFS.
Sinus Fungus Balls:Histopathologic findings reveal densely packed hyphae adjacent to the mucosa, but there is no invasion of mucosa, vessels, or bone. Allergic mucin is not seen.

Adipose tissue is recognized as an active endocrine organ that secretes a number of substances referred to as adipokines including: leptin, adiponectin, visfatin, resistin, omentin and …which act at both autocrine/paracrine and endocrine level. Adipokines participate in the regulation of glucose and lipid metabolism, energy homeostasis, feeding behavior, insulin sensitivity, inflammation, adipogenesis, immunity, vascular function, coagulation and ....for example said leptin acts as the sensor for fat mass and its concentration in the blood circulation is correlated with the body fat storage, as result its production increases in obesity. Conversely, adiponectin is only adipokines that has negative relationship with body mass And is involved in glucose and lipid homeostasis. Also expressed that resistin Can be considered a link between obesity, insulin resistance and diabetes. Visfatin has inflammatory and anti-apoptotic ability which can play important role in infectious and inflammatory diseases. Another adipose tissue hormones is omentin which significantly contributes to the reduction of acute phase protein in endothelial cells and therefore can be considered as an anti-inflammatory adipokine. As a resalt understanding the underlying machanisms of adipokines in obesity, insulin resistance, inflammation and other diseases has great importance to reduce the risk factors as well as therapeutic measures. So our understanding of the pathogenesis of obesity, its metabolic side effects as well as different effects of adipokines in this tissue is necessary. Therefore the aim of this study is a brief review on adipose tissue hormones and their function in the body.
The study is done by searching the English and Persian databases like: Pubmed, Google scholar, SID, Iranmedex with terms such as: leptin, adiponectin, visfatin, resistin, omentin and adipose tissue hormones between the years 1996 untill 2015, and finally 54 references were studied.

Multidrug efflux systems are one of the most important antibiotic resistance mechanisms among microorganisms. Efflux pump systems can be specific substrate or can confer resistance to multidrug antimicrobial by facilitating the extrusion of broad rang of compounds including antibiotics, biocides, heavy metals, and organic pollutants, plant-produced compounds, quorum sensing signals or bacterial metabolites. There are few efflux pump inhibitors are available in market and research in progress raises the hope that they may be found in the future.

The requirement of cholesterol for internalization of eukaryotic pathogens like protozoa (Leishmaniasis, Malaria and Toxoplasmosis) and the exchange of cholesterol along with othermetabolites during reproduction in Schistosomes (helminths) under variable circumstances are poorly understood. In patients infected with some other helminthes, alterations in the lipid profilehave been observed. Also, the mechanisms involved in lipid changes especially in membraneproteins related to parasite infections remain uncertain. Present review of literature shows that parasites induce significant changes in lipid parameters, as has been shown in the in vitro study where substitution of serum by lipid/cholesterol in medium and in experimental models (in vivo).Thus changes in lipid profile occur in patients having active infections with most of the parasites.Membrane proteins are probably involved in such reactions. All parasites may be metabolizing cholesterol, but the exact relationship with pathogenic mechanism is not clear. So far, studies suggest that there may be some factors or enzymes, which allow the parasite to breakup and consume lipid/cholesterol. Further studies are needed for better understanding of the mechanisms involved in vivo. The present review analysis the various studies till date and the role of cholesterol in pathogenesis of different parasitic infections.

Platelet function testing is both complex and labor intensive. A stepwise approach to the evaluation of patients with suspected platelet disorders will optimize the use of laboratory resources, beginning with an appropriate clinical evaluation to determine whether the bleeding is consistent with a defect of primary hemostasis. Bleeding assessment tools, evaluation of platelet counts, and review of peripheral blood cell morphology can aid the initial assessment. For patients requiring further laboratory testing, platelet aggregometry, secretion assays, and von Willebrand factor assays are the most useful next steps and will direct further specialized testing including flow cytometry, electron microscopy, and molecular diagnostics. Guidelines and recommendations for standardizing platelet function testing, with a particular focus on light transmission aggregometry, are available and can provide a template for clinical laboratories in establishing procedures that will optimize diagnosis and assure quality results. This review outlines an approach to platelet function testing and reviews testing methods available to clinical laboratories.