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At least 721 records · Page 40Linked to original sources
PubMed1989

Characterization of stroma from Fuchs' endothelial dystrophy corneas.

Fuchs' endothelial dystrophy is commonly regarded as an endothelial cell disorder. In the present study we compared glycoconjugates of Fuchs' and normal corneas using FITC conjugated lectins [peanut agglutinin (PNA), castor bean agglutinin (RCA120), soybean agglutinin (SBA), and wheat germ agglutinin (WGA)]. Our results showed increased staining with RCA120 and PNA in the posterior region of the Fuchs' corneas, indicating an accumulation of terminal beta-galactose and B-D-galactose (1-3)-D-N-acetylgalactosamine residues. The stromal and epithelial regions of normal and Fuchs' corneas exhibited similar staining patterns with all lectins tested. Our collagen studies showed an increased extractability and abnormal amino acid analyses of collagen from Fuchs' corneas as compared with normals. The purified collagens did have similar banding patterns by sodium dodecyl sulfate gels. However, further characterization by 125(1) two-dimensional peptide mapping revealed that Fuchs' alpha 1-sized chains contained fingerprints that were distinctly different from normal cornea stromal collagen. These data suggest that in addition to abnormal accumulation of RCA120- and PNA-specific glycoconjugates in the posterior cornea, Fuchs' corneas contained stromal collagens with altered biochemical properties. We postulate that the characteristic deterioration of endothelial function in Fuchs' dystrophy may compromise the microenvironment of the stroma and its keratocytes, and thereby lead to an altered collagenous extracellular matrix.

Amino Acids↗
PubMed1999

Knowledge of consumers regarding the nature and extent of adulteration of Indian foods.

The present study was carried out on 60 women consumers selected from three different localities of Hisar city in India to study their knowledge regarding adulteration and detecting adulterants in commonly used food items. The post exposure correct responses of the respondents increased remarkably as 100.0 percent of the respondents gave correct responses regarding statements on adulteration of spices and condiments, the most common adulterated food item and to when and whom they could approach for making complaints about adulterated foods. Similarly, the respondents gained considerable knowledge regarding adulterants generally present in milk, legumes and definition of food adulteration. All the food samples except gene collected from the households of the respondents were found to be adulterated. The main adulterants in milk was water (70.0 percent); turmeric powder contained chalk powder (43.3 percent); chillies powder had artificial colour (100.0 percent); essential oils were removed from cardamom (36.6 percent); green gram and urd bean had water soluble colours; chick pea flour had artificial colours (70.0 percent) and chalk powder was present in sugar (36.6 percent). A kit was developed, tested and given to the housewives so that they could detect adulterants in commonly used food items at the household level.

Adult↗
PubMed2006

[Hereditary enzyme defects of erythrocytes: glucose-6-phosphate dehydrogenase deficiency and pyruvate kinase deficiency].

Possible causes for a normocytic hyperregeneratory anemia are beside an incomplete treatment of iron deficiency, vitamin B12 deficiency or folic acid deficiency notably a hemolysis. After exclusion of other causes of hemolysis like immune hemolytic anemias, microangiopathic hemolytic anemias and hemoglobinopathies, an enzyme deficiency of erythrocytes should be considered. By far the most common form worldwide is the Glucose-6-phosphate deficiency. In the most frequent variants of this disease hemolysis occurs only during stress, imposed for example by infection, "oxidative" drugs or after ingestion of fava beans. The most serious clinical complication of the Glucose-6-phosphate deficiency is the rarely observed neonatal icterus. Some enzyme variants can cause chronic hemolysis which is described as chronic nonsperocytic hemolytic anemia. This form of chronic anemia can also be caused by other enzyme deficiencies, most frequently by the Pyruvate kinase deficiency. All other deficiencies of glycolytic enzymes are even rarer. It should be noted that in some of these very rare forms neurological rather than hematological symptoms predominate the clinical syndrome. If there is suspicion, on the basis of clinical symptoms and/or familial history, diagnosis of an enzyme deficiency can be achieved relatively easy by measurement of the enzyme activity. Accurate diagnosis might be helpful in therapeutic decisions (e.g. splenectomy in certain forms) and it is essential for genetic counseling, since certain deficiencies are transmitted as autosomal recessive disorders (e.g. pyruvate kinase deficiency), while the most common form, the glucose-6-phosphate dehydrogenase deficiency is linked to the X-chromosome.

Anemia, Hemolytic, Congenital↗
PubMed2006

Cellulose synthesis is required for deposition of reticulate wall ingrowths in transfer cells.

Despite the recognized physiological importance of transfer cells, little is known about how these specialized cells achieve localized deposition of cell wall material, leading to amplification of plasma membrane surface area and enhanced membrane transport capacity. This study establishes that cellulose synthesis is a key early factor in the construction of 'reticulate' wall ingrowths, an elaborate but common form of localized wall deposition characteristic of most transfer cells. Using field emission scanning electron microscopy, wall ingrowths were first visible in epidermal transfer cells of Faba bean cotyledons as raised 'patches' of disorganized and tangled cellulosic material, and, from these structures, ingrowths emerged via further deposition of wall material. The cellulose biosynthesis inhibitors 2,6-dichlorobenzonitrile and isoxaben both caused dramatic reductions in the number of cells depositing wall ingrowths, altered wall ingrowth morphology and visibly disrupted microfibril structure. The restriction of cellulose deposition to discrete patches suggests a novel mechanism for cellulose synthesis in this circumstance. Overall, these results implicate a central role for cellulose synthesis in reticulate wall ingrowth morphology, especially at the initial stage of ingrowth formation, possibly by providing a template for the self-assembly of wall polymers.

Benzamides↗
PubMed1993

Collisional fragmentation of glycopeptides by electrospray ionization LC/MS and LC/MS/MS: methods for selective detection of glycopeptides in protein digests.

Mass spectrometric methods of glycopeptide-specific detection in liquid chromatography/electrospray mass spectrometry (LC/ESMS) of glycoprotein digests are explored using a variety of glycopeptide models and then applied to soluble complement receptor type I, a 240-kDa glycoprotein containing 25 potential sites of N-glycosylation. The most specific method, requiring a triple quadrupole, involves monitoring of sugar oxonium fragment ions during precursor-ion scan ESMS/MS. Signals derived from nonglycosylated peptides are virtually eliminated, resulting in a total-ion current chromatographic trace of only the glycopeptides present in the digest. The corresponding mass spectra yield molecular weight and glycopeptide microheterogeneity information. An alternative and complementary approach that we term collisional-excitation scanning also involves fragmentation of glycopeptides to sugar oxonium ion fragments but does not involve any mass-selection process, permitting the experiment to be performed on a single quadrupole instrument. The resulting total ion chromatogram is similar to the UV chromatogram (215 nm), but a selected-ion chromatogram for carbohydrate-specific ions such as the N-acetylhexosamine oxonium ion (m/z 204) produces a glycopeptide-specific trace. Although there can sometimes be peptide interferences in the spectra of the indicated glycopeptide-containing chromatographic peaks, this latter approach permits peptide mapping to be performed on the same data set that also indicates the location of glycopeptides in the chromatogram. Both methods are suitable for detection of glycopeptides with all common classes of oligosaccharides in either N- or O-linkage to the peptide.

Carbohydrate Sequence↗
PubMedDate not supplied

Life history parameters of the two-spotted spider mite (Tetranychus urticae Koch) feeding on bean leaves treated with pyrrolizidine alkaloids.

In this study we assess the effect of pyrrolizidine alkaloids (PAs) extracted from Lithospermum canescens on the biology of the two-spotted spider mite (Tetranychus urticae Koch). Lithospermum canenscens (Michaux) Lehm. (Boraginaceae) is a common prairie plant also known as Indian paint or hoary puccoon. A mixture of seven PAs with known chemical structures was used in this investigation. Mites treated with PAs showed a high mortality of juveniles, a decrease in female fecundity and a shortened longevity. The intrinsic rate of population increase (r(m)) was used as an indicator of T. urticae population performance after treatment with PAs. The r(m) value obtained with alkaloid-treated leaves was lower than that for mites developing on untreated leaves, which indicates that the mite population would develop much slower on treated plants. The results suggest that further studies should be performed to assess the possible use of PA extracts for spider mite control.

Animals↗
PubMed2026

Ultrastructural Insights Into the Reproductive Anatomy and Eggs of Cotton Pink Bollworm, Pectinophora gossypiella Saunders (Lepidoptera: Gelechiidae).

The pink bollworm, Pectinophora gossypiella Saunders is a major pest of cotton, notorious for its high reproductive potential and rapid evolution of resistance to Bacillus thuringiensis (Bt) toxins. Despite its economic significance, detailed knowledge of its reproductive anatomy and egg ultrastructure has remained limited, constraining the development of advanced molecular control strategies such as CRISPR/Cas9-based genome editing. The present study provides the first comprehensive characterization of the reproductive system and egg surface morphology of P. gossypiella using stereomicroscopy and scanning electron microscopy (SEM) techniques. The male reproductive system consists of fused, bean-shaped testes, seminal vesicles, duplex and simplex ejaculatory ducts, and paired accessory glands. The female reproductive system comprises paired ovaries with four polytrophic ovarioles per ovary, lateral and common oviducts, accessory glands, corpus bursae, and spermathecal glands. Eggs are oval, dorsoventrally flattened, exhibit a reticulated chorion with distinct micropylar and aeropylar regions. SEM images revealed 6-9 rosette cells encircling a circular micropylar plate, 14-19 first order and 17-23 s order ribs, and 250-291 polygonal surface cells. The structural features of P. gossypiella eggs reveal key sites for sperm entry, aeropylar respiration, and candidate zones for microinjection in gene editing applications. These findings establish a morphological baseline critical for optimizing embryo manipulation and ribonucleoprotein (RNP) delivery in lepidopteran genome editing. This study represents a pioneering effort to integrate classical egg morphology with molecular entomology, thereby advancing precision genetic interventions aimed at resistance management and population suppression in P. gossypiella.

Animals↗
PubMed2000

Light weight concrete: 226Ra, 232Th, 40K contents and dose reduction assessment

Four types of light weight concrete (LWC) commonly used in Hong Kong, namely, autoclave aerated concrete (plus lime), autoclave aerated concrete (plus Pulverized Fuel Ash or PFA), concrete with synthetic aggregate 'Leca' and concrete with polystyrene bean as aggregate were measured for their 226Ra, 232Th and 40K contents using high-resolution gamma spectrometry. All the radionuclide contents except those for the PFA autoclave aerated concrete were below the world averages of building materials. The Ra-equivalents for these four LWC were 49.6, 249, 122 and 44.2 Bq kg(-1), respectively, and were much smaller than a recommended limit of 370 Bq kg(-1) for construction materials for dwellings. The gamma-dose rate for an indoor environment with partition walls built with LWC was estimated to be about 20 x 10(-8) Gy h(-1), which corresponded to a reduction in the effective dose of about 0.25 mSv y(-1) when compared to that obtained for an indoor environment built with normal concrete (NC) only. The Rn exhalation rates from the three lowest Ra-equivalent LWC were calculated as 0.509, 1.28, and 0.335 mBq m(-2) s(-1), respectively, which corresponded to a reduction in the indoor Rn concentration of around 14 Bq m(-3) and reduction in the tracheobronchial dose reaching 1 mSv y(-1) by using the James lung-dosimetry model, when compared to the case of NC.

Journal Article↗
PubMed1994

Synergism between CACGTG (G-box) and CACCTG cis-elements is required for activation of the bean seed storage protein beta-phaseolin gene.

Expression of bean seed storage protein phaseolin is under strict developmental control. Four distinct nuclear proteins recognize in vitro the proximal beta-phaseolin promoter (-295/+45) which confers spatial and temporal regulation of the native gene. Functional significance of these protein-binding sites was evaluated by substitution mutation of the motifs in the promoter, which was fused to GUS reporter gene, and subsequent transient gene expression assay using protoplasts from developing bean cotyledons. DNA-binding protein CAN binds three CANNTG motifs, CACGTG (-248/-243), CACCTG (-163/-158), and CATATG (-100/-95). Substitution mutation of the CACGTG motif, which is commonly known as G-box, reduced the -295 promoter activity by 75%, indicating that the G-box is a major positive cis-element. Mutation analyses also demonstrated that the CACCTG and CATATG motifs act as positive and negative cis-elements, respectively. Substitution mutation of all three CANNTG motifs essentially eliminated the -295 promoter activity. A construct containing the G-box and CACCTG motif resulted in a transcriptional level that is much greater than the sum of the transcriptional levels from the individual cis-elements, demonstrating that the G-box and CACCTG act synergistically. Substitution mutations of two AT-rich sequences, to which a nuclear protein AG-1 binds, showed that these sites function as major negative (-376/-367, -356/-347) or positive (-191/-182) cis-elements, and that the effect of the two AG-1 binding sites was counteractive in the -391 promoter. These results indicate that the three CANNTG motifs and two AG-1-binding sites play critical roles in transcription of the beta-phaseolin gene in cotyledons.

Base Sequence↗
PubMedDate not supplied

Frequency of glucose 6 phosphate dehydrogenase deficiency and related hemolytic anemia in Riyadh, Saudi Arabia.

BACKGROUND: Glucose 6 Phosphate dehydrogenase deficiency is present in over 400 million people world wide. It is more common iin tropical and subtropical countries and is one of the important causes of hemolytic anemia and neonatal jaundice. We studied the frequency of glucose-6-phosphate dehydrogenase deficiency and associated complications in Central Region (Riyadh) of Saudi Arabia. METHODS: A total of 1740 subjects referred by Ministry of Interior and different hospitals in Riyadh were investigated for glucose-6-phosphate dehydrogenase deficiency. Glucose 6 phosphate dehydrogenase activity was determined by a screening test described by Beutler. RESULTS: Out of these, 106 (6.09%) subjects were deficient. The subjects were divided into marriage and hospital groups. In marriage group deficiency was 4.1% while in hospital group it was 13.3%. In 54 glucose-6-phosphate dehydrogenase deficient patients red blood cell count and haemoglobin levels were determined to see the degree of anaemia. Sixty one percent (61%) had anaemia. In hospital patients 8% patients had severe anaemia while in marriage group no patients had severe anaemia. However mild anaemia was seen in 25% subjects in marriage group. CONCLUSIONS: In conclusion the study indicates that glucose-6-phosphate dehydrogenase deficiency is common in the central region of Saudi Arabia and a lot of patients present with haemolytic episodes. The haemolytic crisis however is not related to the intake of fava beans. The type of variant causing anaemia and suggestions for prevention in marriage group are outlined.

Adolescent↗
PubMed1985

Effects of commonly consumed vegetables on hepatic xenobiotic-metabolizing enzymes in the mouse.

To investigate the effects of consuming a variety of common vegetables on the activities of xenobiotic-metabolizing enzymes in the mouse liver, male C57BL/6 mice were fed purified diets supplemented isocalorically with 20% freeze-dried powdered kidney bean, soya bean, alfalfa, cauliflower, mustard greens, carrot, kale, Brussels sprouts, beet, egg plant or onion or with 40% sweet potato. Hepatic ethoxycoumarin O-deethylase (ECD) activity was increased significantly (P less than or equal to 0.05) over the controls in the mice fed diets containing kidney bean (1.5-fold), alfalfa (1.6-fold), cauliflower (2.2-fold), mustard greens (1.2-fold), carrots (1.2-fold) and kale (1.3-fold). No significant increases in ECD activity were seen in the other groups. Aryl hydrocarbon hydroxylase (AHH) activity was assayed in the cauliflower and Brussels sprouts groups and was found to be unaffected by these diets. Glutathione S-transferase (GST) and epoxide hydratase (EH) activities were increased (P less than or equal to 0.05) by soya bean (1.2- and 1.6-fold respectively), Brussels sprouts (2.0- and 1.6-fold), cauliflower (1.2- and 1.6-fold), alfalfa (1.3- and 2.0-fold) and onion (1.8- and 2.3-fold). No significant increases in GST or EH activities were seen in the other groups. Of the twelve vegetables investigated, only three (sweet potato, beet and egg plant) had no statistically significant effects on any of the xenobiotic-metabolizing parameters tested. However, the beet diet caused an apparent inhibition of ECD activity (74% of control) and the sweet potato diet caused an apparent increase (1.3-fold) in GST activity, although statistical significance could not be established at P less than or equal to 0.05.

7-Alkoxycoumarin O-Dealkylase↗
PubMed1992

Characterization of a new kallikrein-like enzyme (KLP-S3) of the rat submandibular gland.

The submandibular gland of the rat contains several enzymes belonging to the kallikrein family. These include tissue kallikrein, antigen gamma (T-kininogenase), esterase B and tonin. In the present study, a new member of this family, which we have named KLP-S3, was identified and purified from the submandibular gland. KLP-S3 was classified as a kallikrein-like enzyme on the basis of its immunological similarity to other kallikrein-like enzymes and its showing 70% and 73% identity in partial amino acid sequence with tissue kallikrein and tonin respectively. Furthermore, the 44 sequenced amino acid residues showed complete correspondence to the mRNA S3 of the kallikrein gene family, which was the rationale for the name kallikrein-like protein (KLP) S3. KLP-S3 consisted of three isoenzymes with pI 6.75, 6.90 and 6.95, which significantly differed from those of other kallikrein-like enzymes. In conjunction with its immunological relationship to kallikrein, this parameter (pI) was considered robust enough to identify the enzyme during purification, since a specific physiological substrate for KLP-S3 has yet to be identified. In SDS/PAGE the three isoenzymes ran as one band with a molecular mass of 25,800 Da, which after reduction with 2-mercaptoethanol was split into two chains with molecular masses of 16,500 and 13,300 Da. In common with other kallikrein-like enzymes, KLP-S3 was inhibited by phenylmethanesulphonyl fluoride, and was thus classified as a serine protease. It was also inhibited by soya-bean trypsin inhibitor but not by aprotinin. It showed weak reactivity against the chromogenic substrates S2288, S2266, S2366 and S2302 (D-Ile-Pro-Arg 4-nitroanilide, D-Val-Leu-Arg 4-nitroanilide, Glu-Pro-Arg 4-nitroanilide and D-Pro-Phe-Arg 4-nitroanilide respectively) and did not cleave rat T-kininogen or dog high-molecular-mass/low-molecular-mass kininogen. Its specific angiotensin II-generating activity (angiotensin I as substrate) was 0.04% of that of rat tonin. KLP-S3 (1-100 nM) induced a statistically significant angiotensin-independent contraction of isolated rat aorta rings. The maximum contraction was 15% of the response to the alpha-adrenoceptor agonist phenylephrine (1 microM). The concentration of KLP-S3 in the rat submandibular gland was by single radial immunodiffusion estimated to be 47 +/- 3 micrograms/mg of protein.

Amino Acid Sequence↗
PubMed1987

Total nucleotide sequences of the infectious cloned DNAs of bean golden mosaic virus.

Complete nucleotide sequences of the infectious cloned DNA components (DNA A and B) of bean bolden mosaic virus were determined. The DNA A (2585 nucleotides) and DNA B (2647 nucleotides) have little sequence homology with each other, but both A and B contain a common region of 205 nucleotides. A possible large hairpin structure is detected in the common region. Nucleotide sequences of DNAs A and B revealed the presence of 8 potential coding regions for proteins (m.w. greater than 10,000). Among them, four open reading frames (ORFs 1-4) encode proteins of m.w. 30,000 or greater, and are individually coded in virion DNA A and B senses (+) and their complementary senses (-), respectively. The other four ORFs 5-8 are in virion DNA B(+) and its complementary sense (-). All of the ORFs 1-4 have regulatory signals for RNA synthesis (TATAA/T) in the region 5' upstream from a potential start codon ATG.

Base Sequence↗
PubMed2000

[Granulosa cell tumour: review of the literature].

Granulosa cell tumours account for only 5% of ovarian malignancies and there is currently no standard treatment. It is considered as a low-grade malignancy with a favorable prognosis but relapse and extraovarian spread may occur as late as 20 years after diagnosis. It is thus important to better define the characteristics and prognostic factors of this histological subtype in order to recommend appropriate therapy. We performed a review of the literature. This disease is most commonly diagnosed in patients around 50 years old or at puberty. In most cases patients present identical symptoms associated with endometrial hyperplasia or neoplasia. Call-Exner bodies and "coffee bean" cells are characteristic of the histopathology of these tumors. Various prognostic factors are currently being evaluated. Surgery is the treatment for local disease. Patients with locally advanced, recurrent or metastatic tumors require chemotherapy, although the optimal regimen remains to be determined.

Age Factors↗
PubMed2002

Reduction of Escherichia coli O157:H7 and Salmonella spp. on laboratory-inoculated mung bean seed by chlorine treatmentt.

Three U.S. outbreaks of foodborne illness due to consumption of contaminated raw mung bean sprouts occurred in the past 2 years and were caused by Salmonella Enteritidis. The original source of the pathogens is thought to have been the seed. The aim of this study was to determine whether treatment with aqueous chlorine would eliminate the pathogens from mung bean seed inoculated in the laboratory with four-strain cocktails of Escherichia coli O157:H7 and Salmonella spp. Treatments (for 5, 10, or 15 min) with buffered (500 mM potassium phosphate, pH 6.8) or unbuffered solutions containing 0.3 or 3.0% (wt/vol) Ca(OCl)2 were tested. In order to mimic common commercial practice, seed was rinsed before and after treatment with sterile tap water. Treatment for 15 min with buffer (500 mM potassium phosphate, pH 6.8) or sterile water in combination with the seed rinses resulted in maximum reductions of approximately 3 log10 CFU/g. The largest reductions (4 to 5 log10 CFU/g) for the chlorine treatments in combination with the rinses were obtained after treatment with buffered 3.0% (wt/vol) Ca(OCl)2 for 15 min. Treatment of mung bean seed for 15 min with unbuffered or buffered 3.0% (wt/vol) Ca(OCl)2 did not adversely affect germination. Even though treatments with 3% (wt/vol) Ca(OCl)2 in combination with the water rinses were effective in greatly reducing the populations of both bacterial pathogens, these treatments did not result in the elimination of the pathogens from laboratory-inoculated seed.

Anti-Bacterial Agents↗
PubMed1999

Dietary lectins can induce in vitro release of IL-4 and IL-13 from human basophils.

Dietary lectins, present in beans and other edible plant products, pose a potential threat to consumers due to their capacity to induce histamine release from basophils. In this study, we analyzed the capacity of 16 common, in particular dietary, lectins to induce human basophils to secrete IL-4 and IL-13, the key promoters of Th2 responses and IgE synthesis. Several of the lectins, especially concanavalin A, lentil lectin, phytohemagglutinin, Pisum sativum agglutinin and Sambucus nigra agglutinin, triggered basophils to release IL-4 at concentrations of up to 1 ng/10(6) basophils. Lectins with high IL-4-inducing capacity also stimulated the release of IL-13 and histamine. Lectin-induced IL-4 and IL-13 release reached a maximum after 4-6 h and more than 18 h, respectively. Affinoblotting revealed that lectins with the capacity to induce mediator release bind to IgE, suggesting IgE binding as initial step of signal generation. In conclusion, several dietary lectins can trigger human basophils to release IL-4 and IL-13. Since lectins can enter the circulation after oral uptake, they might play a role in inducing the so-called early IL-4 required to switch the immune response towards a Th2 response and type I allergy.

Basophils↗
PubMed1998

Mechanical model testing of rebreathing potential in infant bedding materials.

Rebreathing of expired air may be a lethal hazard for prone sleeping infants. This paper describes a mechanical model to simulate infant breathing, and examines the effects of bedding on exhaled air retention. Under simulated rebreathing conditions, the model allows the monitoring of raised carbon dioxide (CO2) inside an artificial lung-trachea system. Resulting levels of CO2 (although probably exaggerated in the mechanical model compared with an infant, due to the model's fixed breathing rate and volume) suggest that common bedding materials vary widely in inherent rebreathing potential. In face down tests, maximum airway CO2 ranged from less than 5% on sheets and waterproof mattresses to over 25% on sheepskins, bean bag cushions, and some pillows and comforters. Concentrations of CO2 decreased with increasing head angle of the doll, away from the face down position. Recreations of 29 infant death scenes also showed large CO2 increases on some bedding materials, suggesting these infants could have died while rebreathing.

Bedding and Linens↗
PubMed2004

Blue rubber bleb nevus syndrome: case report and literature review.

Blue Rubber Bleb Nevus Syndrome ( BRBNS) is a rare disorder characterized by multiple cutaneous venous malformations in the skin and gastrointestinal tract associated with intestinal hemorrhage and iron deficiency anemia. Other organs may also be involved. BRBNS has a potential for serious or fatal bleeding. The causes of this syndrome are unknown. Its most common presentation is in the form of sporadic cases, but dominant autosomal inheritance has been described. Although it was first recognized by Gascoyen in 1860, only one hundred years later did Bean further describe these lesions and coined the term BRBNS. A MEDLINE search yielded about 200 case reports published till 2003. We present a case of this syndrome diagnosed in a 16- year - old patient with both upper and lower gastrointestinal bleeding. He had severe anemia and venous swellings on the trunk. Similar lesions were found in the stomach, bowel and on his foot. In addition, we review the available literature on the epidemiology, clinical features, associated conditions, diagnosis and treatment.

Adolescent↗