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                           Myxoid Tumours of Soft Tissue

 
December 2007

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Soft Tissue Pathology

Case Index

Infectious Disease Online; INDEX: A-D ; INDEX: E-L ; INDEX: M-P INDEX: Q-Z ; FUNGAL DISEASE ; VIRAL DISEASE.

Environmental Pathology- Smoking

 

Cigarette smoking and Cardio vascular Disease  

Cigarette smoking and Cancer

Non-Neoplastic Diseases in Smokers

Cigarette Smoking and diseases in Women

Environmental Pathology- Alcoholism (Mechanism of Tissue Injury)

Complication of Chronic Alcoholism

Environmental Pathology- Drug Abuse

Environmental Pathology - Iatrogenic Drug Injury

Iatrogenic Drug Injury - Oral contraceptives

Environmental Pathology - Effect of Chemicals

Toxic effect of volatile organic solvents and vapors

Toxic effect of agricultural chemicals

Environmental Pathology - Toxic effect of Metal

Lead Intoxication

Mercury Exposure

Arsenic

Cadmium

Nickel

Iron

Environmental Pathology - Physical Agents  

Environmental Pathology-Thermal Regulatory Dysfunction

Environmental Pathology - Hypothermia

Environmental Pathology - Hyperthermia

Environmental Pathology- Electrical Burns

Environmental Pathology- Altitude Related Illnesses

Environmental Pathology - Physical Injuries

Environmental Pathology - Radiation  

Whole-Body Irradiation

Localized Radiation Injury Associated with Radiotherapy

Radiation and Cancer

Cutaneous lesions after exposure to Radiation

Obesity

Protein Calorie Malnutrition

Marasmus

Vitamins:

Vitamin A Deficiency

Vitamin B Complex

Thiamine (B1)

Riboflavin(B2)

Niacin, niacinamide, nicotinic acid(B3)

Pyridoxine (B6)

Folic acid (B9)

Cyanocobalamin (B12)

Vitamin C Deficiency

Vitamin D Deficiency

Vitamin E Deficiency

Vitamin K  Deficiency

Minerals

Myxoid Tumours of Soft Tissue

Classification of Soft Tissue Tumour

Gross examination of soft tissue specimen          

A practical approach to histopathological reporting of soft tissue tumours

Grading of soft tissue tumours

Lipomatous tumours

Neural tumours

Vascular tumours

Myogenic tumours

Fibroblastic/Myofibroblastic tumours

Myofibroblastic tumours

Fibrohistiocytic tumours

ChondroOsseous tumours

Soft TissueTumours of Uncertain Differentiation               

Notochordal Tumour - Chordoma

Extra-adrenal Paraganglioma

Gastrointestinal Stromal Tumour

Anatomy and Histology of the Normal Lung and Airways

Examination of pulmonary and pleural biopsies

Useful chromatic and immunostains in pulmonary pathology

Percutaneous Needle and Trucut Biopsy Specimen

Bronchial Biopsy Specimen

Transbronchial Biopsy Specimen

Transbronchial biopsy in lung transplant recipients

Open lung biopsy

Lobectomy and pneumonectomy specimen

Histopathological reporting of pulmonary parenchymal biopsies

Histopathological reporting of pulmonary biopsies in cases of Idiopathic Pulmonary Fibrosis

Closed pleural biopsy  ;Open pleural biopsy 

Anatomical Distribution of Pulmonary Disease

Sarcoidosis

Extrinsic Allergic Alveolitis 

Pulmonary Eosinophilic Granuloma

Pathological Diagnosis of Granulomatous Lung Diseases

Infectious Granuloma of the Lung

Non-necrotising Granulomatous Inflammation of the lung

An approach to Histopathological Examination of Pulmonary Granulomatous Inflammation

Pneumoconiosis

Silicosis

Asbestosis

Coal Pneumoconiosis

Talcosis

Lymphangio leiomyomatosis

Pulmonary Mesenchymal Tumours

Primary Pulmonary Leiomyosarcoma

Primary Pulmonary Rhabdomyosarcoma

Primary Monophasic Synovial Sarcoma of the Lung

Neurogenic Tumours of the Lung

Pulmonary Malignant Fibrous Histiocytoma

Bone and Cartilage- forming Sarcoma of the Lung

Kaposi's Sarcoma and Angiosarcoma of the Lung

                           

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Vitamin A, a fat-soluble substance is important for the maintenance of a number of specialized epithelial linings, skeletal maturation, and the structure of the cell membranes. In addition, it is an important constituent of the photosensitive pigments in the retina. Nutritional Pathology Online : click

Vitamin A occurs naturally as retinoids or as a precursor, beta-carotene. The source of the precursor, carotene, is in plants, principally leafy, green vegetables. Fish livers are a particularly rich source of vitamin A itself. Vitamins : click

Both forms are absorbed from the intestinal mucosa.

Beta-carotene is cleaved in the intestinal mucosa to the aldehyde and then reduced to retinoids.

The retinoids are bound to palmitic acid, absorbed to chylomicrons, and transported by the lymph to the general circulation.

Lipoprotein lipase releases the retinoids, after which it is stored in the liver, where 90% of the body's vitamin A is located.

Retinol is bound to a retinol-binding protein, transported with albumin, and extracted by the cell surfaces throughout the body.

Usually, rapid transit of food through the small intestine, or modification of available lipid by the addition of non-absorbable lipid carriers (for example, mineral oil) decreases the absorption of vitamin A.

Lack of vitamin A results principally in squamous metaplasia, especially in glandular epithelium.

One effect of this change is the formation of an epithelium whose structure is not adapted to functional needs.

Stratified squamous epithelium keratinizes and the keratin debris blocks sweat and tear glands.

Squamous metaplasia is common in the trachea and the bronchi, and broncho-pneumonia is a frequent cause of death.

The lining epithelium of the renal pelvis, pancreatic ducts, uterus, and salivary glands are also commonly affected.

Epithelial changes in the renal pelvis are occasionally associated with kidney stones.

With further diminution of vitamin A stores, squamous metaplasia of the epithelial cells of the conjunctiva and tear ducts occurs, which leads to xerophthalmia, a dryness and wrinkling of the cornea.

The cornea becomes softened (keratomalacia) and is vulnerable to ulceration and bacterial infection, complications which may lead to blindness.

Follicular hyperkeratosis, a skin disorder that results from occluded sebaceous glands, is also a feature of this disease.

The earliest sign of vitamin A deficiency often is diminished vision in dim light.

Vitamin A is a necessary component in the pigment of the retinal rods and is active in light transduction.

Since the aldehyde of vitamin A, retinal, is constantly being degraded during the generation of the light signal, a continuous supply of vitamin A is necessary of night vision.

Large doses of vitamin A, usually from excessive administration of vitamin supplements to children, lead to lesions of vitamin toxicity.

(Early Arctic explorer were said to have experienced vitamin A toxicity because they ate polar bear livers, which are particularly rich in the vitamin).

An enlarged liver and spleen are common, and microscopically these organs show lipid-laden macrophages. In the liver, vitamin A is also present in hepatocytes, and prolonged vitamin A toxicity has been incriminated in the production of cirrhosis.

Bone pain and neurologic symptoms such as hyperexcitability and headache may be the presenting symptoms.

Discontinuation of excess vitamin A consumption reverses all or most of the lesions.

Excessive carotene intake is benign and simply stains the skin yellow, an appearance that may be mistaken for jaundice.

Synthetic derivatives of retinoic acid are now increasingly used for their pharmacological effect in alleviating severe acne.

The doses of these compounds, which display potent vitamin A activity, is limited by vitamin A toxicity.

                         

Serum retinol and beta-carotene levels in subacute sclerosing panencephalitis.J Child Neurol. 2007 Mar;22(3):341-3.

Reduced serum levels of vitamin A affect morbidity and mortality in measles. The authors' newly diagnosed subacute sclerosing panencephalitis (n = 21) and age-matched control groups (n = 20) had mean serum beta-carotene levels of 1.12 +/- 0.56 and 1.50 +/- 0.52 microg/mL, respectively. Serum retinol <20 microg/dL was observed in 6 of 21 subacute sclerosing panencephalitis and 0 of 20 control cases (P < .05). Vitamin A deficiency can accompany subacute sclerosing panencephalitis: its contribution to the pathogenesis or course of the disease warrants further investigations.

Effectiveness of an early supplementation scheme of high-dose vitamin A versus standard WHO protocol in Gambian mothers and infants: a randomised controlled trial.Lancet. 2007 Jun 23;369(9579):2088-96.

BACKGROUND: Most developing countries have adopted a standard WHO dosing schedule for vitamin A supplementation. However, in 2002 the International Vitamin A Consultative Group (IVACG) Annecy Accord recommended a new high-dose regimen for mothers and infants. Our aim was to test whether the new high-dose regimen of vitamin A supplementation would increase maternal and infant plasma vitamin A, reduce infant Helicobacter pylori infection and nasopharyngeal pneumococcal carriage, and improve infant gut epithelial integrity. METHODS: In an area of moderate vitamin A deficiency in rural Gambia, 220 mother-infant pairs were enrolled in a randomised double-blind trial between September, 2001, and October, 2004, that compared the IVACG high dose with the WHO dose. The primary endpoints were levels of maternal and infant plasma vitamin A, H pylori infection, pneumococcal carriage, and gut epithelial integrity. The trial is registered as ISRCTN 98554309. FINDINGS: 197 infants completed follow-up to 12 months (99 high dose and 98 WHO dose). There were no adverse events at dosing. No differences were found in the primary outcomes for high-dose versus WHO schedule: maternal vitamin A concentration at 2 months +0.02 micromol/L (95% CI -0.10 to 0.15); infant vitamin A at 5 months +0.01 micromol/L (-0.06 to 0.08); H pylori infection at 12 months -0.3% (-14.7 to 14.2); maternal pneumococcal carriage at 12 months -2.0% (-13.7 to 9.7); infant pneumococcal carriage at 12 months -4.1% (-15.8 to 7.6); infant gut mucosal damage at 12 months 5.2% (-8.7 to 19.2). There were more clinic attendances by the high-dose group in the first 6 months of life (p=0.018). INTERPRETATION: Our results do not lend support to the proposal to increase the existing WHO standard dosing schedule for vitamin A in areas of moderate vitamin A deficiency. Caution is urged for future studies because trials have shown possible adverse effects of higher doses of vitamin A, and potential negative interactions with the expanded programme on immunisation (EPI) vaccines.

Retinoid signaling during spermatogenesis as revealed by genetic and metabolic manipulations of retinoic acid receptor alpha.Soc Reprod Fertil Suppl. 2007;63:11-23.

The importance of dietary retinol (vitamin A) and retinoid signaling for normal development and differentiation has been recognised for many years. Vitamin A deficiency results in a variety of abnormalities, most of which can be corrected by supplementing the diet with all-trans-retinoic acid (ATRA), with the exception of blindness and male sterility. ATRA, an active metabolite of vitamin A, functions primarily by binding to nuclear receptors of the steroid hormone superfamily, the retinoic acid receptors (RARs). Gene targeting studies revealed the importance of ATRA signaling through the RARs for spermatogenesis. Mice that are homozygous for a null mutation in the gene encoding RARalpha, Rara-/-, exhibit defects in spermatogenesis and male sterility. The abnormalities in these RARalpha-deficient testes have been examined in detail in a series of recent studies from our laboratory and will be summarised in this paper. We also review how dietary, pharmacologic and genetic strategies, alone or in combination, can be used to gain further insight into retinoid function in mammalian spermatogenesis.

Impact of mass supplementation of vitamin A.  Indian J Pediatr. 2007 May;74(5):443-7.

OBJECTIVE: To study the impact of mass supplementation of Vitamin A solution on morbidity due to diarrhea, Acute respiratory infection (ARI) and xerophthalmia. METHODS: The two rounds of age specific mass distribution of Vitamin A solution were undertaken during January 2000 and December 2000 respectively covering 27,642 (98.7%) and 31,762 (88.0%) children respectively out of total beneficiaries in two round of PPI in Chandigarh. A random sample of 276 children from intervention area and 252 children from control area in the age group of 1-5 yr were followed up on monthly basis for morbidity pattern for a period of nine mth. The morbidity pattern for intervention and control area children was compared to see the impact of mass supplementation of Vitamin A solution. RESULTS: The average annual episodes of diarrhea in intervention children were lower (3.9 per yr) as compared to control children (5.2 per yr) although difference was not statistically significant (P>0.05) except in initial month. The average annual episodes of ARI in intervention children were lower (5.1 per yr) as compared to Control children (6.0 per yr) although difference was not significant (P>0.05) except in initial first mth. There was significant decline in vitamin A deficiency (VAD) as no case of Bitot's spot was found in intervention children as compared to control children where the prevalence of Bitot's spot ranged from 4.3-5.08% during different visits. The mortality rate was found to be higher in control children with a death rate of 8 per 1000 children during the study period as compared to intervention children where no death was recorded. CONCLUSION: It is concluded that mass supplementation of vitamin A led to significant reduction in xerophthalmia and decline in mortality in the intervention area as compared to control area.

Role of vitamin A supplementation in the treatment of tuberculosis.Natl Med J India. 2007 Jan-Feb;20(1):16-21.

Vitamin A deficiency has been commonly observed in patients with tuberculosis. Low serum retinol levels return to normal after antituberculosis treatment even when no supplements are provided. The deficiency of vitamin A observed in patients with tuberculosis might have contributed to the development of tuberculous disease in them. Alternatively, deficiency could be the result of loss of appetite, poor intestinal absorption, increased urinary loss of vitamin A or acute phase reaction in TB. Vitamin A deficiency lowers immunity while vitamin A supplementation reduces morbidity and mortality, particularly from measles and diarrhoea. Vitamin A supplementation also decreases the mortality rate in HIV-infected children and delays the progression of HIV disease in infected subjects. A higher incidence of lung cancer and increased mortality have been observed in smokers after beta-carotene supplementation. Zinc deficiency is also common in tuberculosis, which may impose a secondary vitamin A deficiency. Clinical trials have shown conflicting results regarding the effect of supplementation of vitamin A, alone or with other micronutrients, on time taken to sputum conversion in patients with pulmonary tuberculosis. Supplementation with multiple micronutrients (including zinc) rather than vitamin A alone may be more beneficial in patients with tuberculosis, but clinical trials on such a combination are lacking.

Benefit of vitamin A supplementation on ascaris reinfection is less evident in stunted children.J Nutr. 2007 Jun;137(6):1455-9.

Despite the common coexistence of vitamin A deficiency and Ascaris infection in preschool children in developing countries, and despite the widespread use of vitamin A supplements, remarkably little is understood about the impact of vitamin A supplementation on this gastrointestinal nematode. The Ministry of Health of Panama recently initiated a vitamin A supplementation program in rural indigenous populations. We took advantage of this initiative to assess the benefit of 200,000 IU (60 mg retinol) vitamin A on reinfection with Ascaris following deworming. Baseline stool exams, anthropometry, and socio-economic data were collected for 328 preschool children from 12-60 mo of age (106 supplemented within previous 3 mo and 222 unsupplemented within previous 6 mo). All children were dewormed with albendazole, and reinfection levels were monitored 3 and 5 mo later. Baseline prevalence of Ascaris was 79.5%. Stepwise regression showed that Ascaris intensity was lower in Vit A-supplemented children at baseline and 3 mo after deworming, but not after 5 mo. As 61% of the children were stunted, the impact of supplementation on Ascaris reinfection was examined separately for stunted and children of normal height. Prevalence and intensity of Ascaris at baseline and 3 mo after deworming were lower in children of normal height, but in stunted children the benefit was restricted to those who were dewormed within 6 wk of supplementation. Our study provides evidence that combined vitamin A supplementation and deworming reduces Ascaris reinfection in children living in areas of chronic parasitosis, but that the duration of the benefit is less in stunted children.

Assessment of vitamin A nutritional status in newborn preterm infants.Nutrition. 2007 Jun;23(6):454-60. Epub 2007 May 17.

OBJECTIVE: This study assessed the vitamin A nutritional status of preterm infants determined by the vitamin A relative dose-response test (RDR) compared with serum levels of vitamin A, retinol-binding protein (RBP), transthyretin (TTR), and retinol relations with carrier proteins. METHODS: Serum levels of retinol, RBP, and TTR and retinol/RBP, retinol/TTR, and RBP/TTR molar ratios were determined in 120 infants at 7 d and in 92 at 28 d. For the determination of the performance of the tests, the RDR was considered the reference method. The sensitivity and specificity for all possible cutoff values were determined by constructing receiver operator characteristic curves. The areas under the curves were used to estimate the overall accuracy of the tests. The best cutoff values to be used for the calculation of sensitivity and specificity were determined with 95% confidence intervals. RESULTS: RDR indicated vitamin A deficiency in 60% of the infants at 7 d and in 51.1% at 28 d. In the receiver operator characteristic curves, the best area under the curve was 0.710 obtained for serum retinol at 28 d of postnatal age and considered moderately accurate. The least inadequate cutoff level was set at 25 mg/dL, but no value was considered adequate due to low sensitivity and/or low specificity. CONCLUSION: Compared with RDR, the determination of serum levels of retinol, RBP, and TTR and their molar ratios are not adequate to assess nutritional vitamin A status in preterm infants.

Biochemical assessment of vitamin A in schoolchildren from a rural community.J Pediatr (Rio J). 2007 May/June;83(3):247-252.

OBJECTIVE: To investigate the prevalence of vitamin A deficiency among schoolchildren from a rural area in the Distrito Federal, Brazil, and to correlate this with rates of anemia and malnutrition. METHODS: From a total of 179 students, the study recruited 155 schoolchildren (5 to 18 years), whose parents gave permission for blood tests. Plasma retinol concentration was assayed by high resolution liquid chromatography, and levels of plasma vitamin A lower than 20 microg/dL were defined as abnormal or deficient in vitamin A. Hemoglobin was measured by an automated cell counter, and anemia was defined as serum concentrations of less than 11.5 and 12.0 g/dL for children and adolescents, respectively. Nutritional status was assessed using z scores for weight/height, height/age and body mass index percentiles. RESULTS: The results indicated that 33.55% of the schoolchildren tested had a vitamin A deficiency, with a prevalence of 35.44% among children (5-9 years) and 31.58% among adolescents (10-18 years). No correlation was observed between the prevalence of vitamin A deficiency and prevalence rates of anemia or malnutrition. Both sexes and all ages were homogeneous for vitamin A deficiency. CONCLUSIONS: The elevated prevalence of vitamin A deficiency among the children and adolescents attending this rural school identify a public health problem in the region. These results indicate that age groups from 5 years onwards should be included in those at risk of hypovitaminosis A and that they should be included in public policies aimed at combating hypovitaminosis A.

Vitamin A deficiency (VAD), teratogenic, and surgical models of congenital diaphragmatic hernia (CDH).Am J Med Genet C Semin Med Genet. 2007 May 15;145(2):139-57.

Congenital diaphragmatic hernia (CDH) is a congenital malformation that occurs with a frequency of 0.08 to 0.45 per 1,000 births. Children with CDH are born with the abdominal contents herniated through the diaphragm and exhibit an associated pulmonary hypoplasia which is frequently accompanied by severe morbidity and mortality. Although the etiology of CDH is largely unknown, considerable progress has been made in understanding its molecular mechanisms through the usage of genetic, teratogenic, and surgical models. The following review focuses on the teratogenic and surgical models of CDH and the possible molecular mechanisms of nitrofen (a diphenyl ether, formerly used as an herbicide) in both induction of CDH and pulmonary hypoplasia. In addition, the mechanisms of other compounds including several anti-inflammatory agents that have been linked to CDH will be discussed. Furthermore, this review will also explore the importance of vitamin A in lung and diaphragm development and the possible mechanisms of teratogen interference in vitamin A homeostasis. Continued exploration of these models will bring forth a clearer understanding of CDH and its molecular underpinnings, which will ultimately facilitate development of therapeutic strategies.

Vitamin A stability in salt triple fortified with iodine, iron, and vitamin A.Food Nutr Bull. 2006 Sep;27(3):252-9.

BACKGROUND: Dietary micronutrient deficiencies, which lead to diseases such as iodine deficiency disorders, iron-deficiency anemia, and vitamin A deficiency, are serious public health problems in the developing world. Fortifying salt with iodine, iron, and vitamin A is an attractive approach to simultaneously reduce the deficiencies of these three micronutrients in the diet. OBJECTIVE: To explore the technical feasibility of producing triple-fortified salt fortified with iodine, iron, and vitamin A that would be stable under the climatic conditions of developing countries (i.e., high temperature and high humidity). METHODS: Triple-fortified salt was obtained by granulation and encapsulation of commercially produced vitamin A products, iodine, and iron compounds. Vitamin A retention was determined in the presence of five iron and two iodine compounds, in different combinations, under three different storage conditions. The influence of commercial stabilization techniques for the vitamin A palmitate source used (spray-dried or dissolved in oil), and the type of binder used for granulation on vitamin A retention in triple-fortified salt was studied. The influence of temperature, humidity, and chemical interactions on vitamin A stability in triple-fortified salt was also investigated. RESULTS: The most stable formulation retained 77.73% of vitamin A after 2 months of storage at 40 degrees C, 60% relative humidity, and 95% under ambient conditions. CONCLUSIONS: The results indicate that the production of a stable triple-fortified salt is technically feasible.