Recurrent hemolytic uremic syndrome in the renal allograft.
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Adult↗
Biomedical subjects
N Haider
Publications and source records attributed to N Haider.
Post traumatic intra-abdominal pseudo-aneurysms in children.
Pseudoaneurysms are uncommon following blunt abdominal trauma. A review of the literature has shown that such cases are usually managed aggressively. We report our experience of two cases of post traumatic intra-abdominal pseudoaneurysms which were managed conservatively.
Abdominal Injuries↗
Abdominopelvic mesh compartmentalization reduces the complications of radiotherapy in children: a preliminary report.
AIM: The aim of this report was to assess the effects of radiotherapy (RT) in children with abdominopelvic tumours in whom a biodegradable (Polyglactin 910) mesh had been inserted prior to commencement of radiotherapy. METHODS: Retrospective analysis was carried out of four patients with abdominopelvic tumours who underwent radiotherapy between 2000 and 2002 as part of their management. RESULTS: All children tolerated radiotherapy well with no evidence of acute or chronic radiation enteritis. One child developed prolonged postoperative ileus and a second child developed infective diarrhoea and fever, not related to radiation. CONCLUSION: We have highlighted a good tolerance of radiotherapy in children following the insertion of a Polyglactin 910 mesh prior to starting radiation and would recommend further larger studies with longer follow-up to support this.
Abdominal Neoplasms↗
Synthesis and complete 1H, 13C and 15N NMR assignment of substituted isoxazolo[3,4-d]pyridazin-7(6H)-ones.
The synthesis and complete assignment of all hydrogen, carbon and nitrogen NMR signals of several new isoxazolo[3,4-d]pyridazin-7(6H)-ones is reported. The spectroscopic characterization is extended to previously described analogues.
Carbon Isotopes↗
Gastric stromal tumors in children.
Gastrointestinal stromal tumors (GIST) in children are rare and their behavior has been regarded as difficult to predict on pathological criteria. We report our experience with two gastric GISTs in children aged 10 and 11 years. Both remain alive and free of disease at 5 years and 2 years respectively. Comparison of the pathological features in the resected specimens with a recently proposed guidelines for predicting outcome in this group of tumors is reported.
Antigens, Neoplasm↗
Semicarbazide-sensitive amine oxidase: current status and perspectives.
Semicarbazide-sensitive amine-oxidase (SSAO) is present in various human tissues and in plasma. Oxidative deamination of short-chain aliphatic amines is catalyzed by this enzyme to afford the corresponding aldehydes, ammonia and hydrogen peroxide. Methylamine and aminoacetone have been recognized to be physiological substrates for SSAO. There are several pathological states where increased serum SSAO activity have been found, such as diabetes mellitus, congestive heart failure, multiple types of cerebral infarction, uraemia, and hepatic cirrhosis. The role of SSAO in pathophysiology of diabetes has been most extensively investigated. The elevated formation of the potentially cytotoxic products of the enzyme may contribute to the endothelial injury of blood vessels, resulting in the early development of severe atherosclerosis; it may also contribute to the pathogenesis of diabetic angiopathy. It is now suggested that SSAO inhibitors may prevent the development of atherosclerosis and diabetic complications as well. Inhibitors can be conveniently subdivided into the main groups of hydrazine derivatives, arylalkylamines, propenyl- and propargylamines, oxazolidinones, and haloalkylamines. Of them, aryl(alkyl)hydrazines, and 3-halo-2-phenylallylamines are generally very strong SSAO inhibitors. Most of these inhibitors of SSAO have been originally developed for other purposes, or they are simple chemical reagents with highly reactive structural element(s); these compounds have not been able to fulfil all criteria of high potency, selectivity, and acceptable toxicity. New potent compounds with selectivity and low toxicity are needed, which may prove useful tools for understanding the roles and function of SSAO, or they may even be valuable substances for treatment of various diseases.
Amine Oxidase (Copper-Containing)↗
Melanotic neuroectodermal tumour of infancy arising in skull.
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Biopsy, Needle↗
Apoptosis in atherosclerosis. Does it contribute to plaque instability?
Several reports have demonstrated apoptosis in the advanced human atheroma. Most clinical events however, are precipitated by plaque rupture, to a lesser extent erosion, and the development of occlusive thrombi. Whether the extent of apoptosis can influence lesion stability is not precisely known, however, there is emerging data supporting this role. Obvious difficulties arise when studying apoptosis in atherosclerotic plaques because of the complex nature of the disease and lack of an experimental model of plaque instability. This article applies a systematic approach to discuss the issue of apoptosis in context of early disease to complex symptomatic lesions that may become fatal.
Apoptosis↗
Pitfalls in homozygosity mapping.
There is much interest in use of identity-by-descent (IBD) methods to map genes, both in Mendelian and in complex disorders. Homozygosity mapping provides a rapid means of mapping autosomal recessive genes in consanguineous families by identifying chromosomal regions that show homozygous IBD segments in pooled samples. In this report, we point out some potential pitfalls that arose during the course of homozygosity mapping of the enhanced S-cone syndrome gene, resulting from (1) unexpected allelic heterogeneity, so that the region containing the disease locus was missed as a result of pooling; (2) identification of a homozygous IBD region unrelated to the disease locus; and (3) the potential for inflation of LOD scores as a result of underestimation of the extent of inbreeding, which Broman and Weber suggest may be quite common.
Alleles↗
Localization of apoptotic macrophages at the site of plaque rupture in sudden coronary death.
Although apoptosis is a well-recognized phenomenon in chronic atherosclerotic disease, its role in sudden coronary death, in particular, acute plaque rupture is unknown. Culprit lesions from 40 cases of sudden coronary death were evaluated. Cases were divided into two mechanisms of death: ruptured plaques with acute thrombosis (n = 25) and stable plaques with and without healed myocardial infarction (n = 15). Apoptotic cells were identified by staining of fragmented DNA and confirmed in select cases by gold conjugate labeling combined with ultrastructural analysis. Additional studies were performed to examine the expression and activation of two inducers of apoptosis, caspases-1 and -3. Ruptured plaques showed extensive macrophage infiltration of the fibrous cap, in particular at rupture sites contrary to stable lesions, which contained fewer inflammatory cells. Among the culprit lesions, the overall incidence of apoptosis in fibrous caps was significantly greater in ruptured plaques (P < 0.001) and was predominantly localized to the CD68-positive macrophages. Furthermore, apoptosis at plaque rupture sites was more frequent than in areas of intact fibrous cap (P = 0. 028). Plaque rupture sites demonstrated a strong immunoreactivity to caspase-1 within the apoptotic macrophages; staining for caspase-3 was weak. Immunoblot analysis of ruptured plaques demonstrated caspase-1 up-regulation and the presence of its active p20 subunit whereas stable lesions showed only the precursor; nonatherosclerotic control segments were negative for both precursor and active enzyme. These findings demonstrate extensive apoptosis of macrophages limited to the site of plaque rupture. The proteolytic cleavage of caspase-1 in ruptured plaques suggests activation of this apoptotic precursor. Whether macrophage apoptosis is essential to acute plaque rupture or is a response to the rupture itself remains to be determined.
Apoptosis↗
Kinetics of migration of antioxidants from polyolefins in natural environments as a basis for bioconversion studies.
Plastics ending up in soil or landfill environment will eventually be degraded. At the same time a slow migration of additives from the plastics will occur. The additives and the degradation products of these and the polymer are potential compounds for bioconversion. To predict the rate of the migration of antioxidants from polyolefins, a medium-density polyethylene film MDPE containing two commercial antioxidants, Irganox 1010 and Irgafos 168, was exposed to simulated landfill environments, viz. aqueous media at pH 5 and 7, with temperatures of 25, 50, 80, and 105 degrees C and solid-to-liquid ratios of 1:5. The migration process of the antioxidants was studied by an ultrasonic extraction technique using chloroform as extraction solvent to recover the nonmigrated antioxidants from the polymeric matrix, followed by quantitative analysis of the extracted antioxidants by high performance liquid chromatography (HPLC) with acetonitrile as the mobile phase and a wavelength of 280 nm. It was found that the release of both Irganox 1010 and Irgafos 168 increases at higher temperature, longer time, and lower pH. Moreover, the migration of Irgafos 168 proceeds faster than that of Irganox 1010 at lower temperatures while at higher temperatures the opposite behavior was observed. No degradation of the polymeric matrix was detected under the testing time. Migration processes included diffusion of the antioxidants within the polymer and their desorption from the surface of the polymer into the boundary layers of leachate. In general, the kinetic of the migration process was well described by first-order equations while an Arrhenius-type relationship was applicable for the temperature dependence of the constants. The general constants calculated for the migration processes were applied to the prediction of the time required for complete release of the antioxidants under different landfill conditions.
Alkenes↗
Apoptosis in heart failure: release of cytochrome c from mitochondria and activation of caspase-3 in human cardiomyopathy.
Apoptosis has been shown to contribute to loss of cardiomyocytes in cardiomyopathy, progressive decline in left ventricular function, and congestive heart failure. Because the molecular mechanisms involved in apoptosis of cardiocytes are not completely understood, we studied the biochemical and ultrastructural characteristics of upstream regulators of apoptosis in hearts explanted from patients undergoing transplantation. Sixteen explanted hearts from patients undergoing heart transplantation were studied by electron microscopy or immunoblotting to detect release of mitochondrial cytochrome c and activation of caspase-3. The hearts explanted from five victims of motor vehicle accidents or myocardial ventricular tissues from three donor hearts were used as controls. Evidence of apoptosis was observed only in endstage cardiomyopathy. There was significant accumulation of cytochrome c in the cytosol, over myofibrils, and near intercalated discs of cardiomyocytes in failing hearts. The release of mitochondrial cytochrome c was associated with activation of caspase-3 and cleavage of its substrate protein kinase C delta but not poly(ADP-ribose) polymerase. By contrast, there was no apparent accumulation of cytosolic cytochrome c or caspase-3 activation in the hearts used as controls. The present study provides in vivo evidence of cytochrome c-dependent activation of cysteine proteases in human cardiomyopathy. Activation of proteases supports the phenomenon of apoptosis in myopathic process. Because loss of myocytes contributes to myocardial dysfunction and is a predictor of adverse outcomes in the patients with congestive heart failure, the present demonstration of an activated apoptotic cascade in cardiomyopathy could provide the basis for novel interventional strategies.
Animals↗
Cutaneous leishmaniasis in Yemen.
BACKGROUND: Cutaneous leishmainiasis is widespread in the Yemen, but has not been fully documented. METHODS: We have studied 42 cases from the Hajjah and Amran Governorates of the Yemen Republic. The clinical profile of these cases was recorded in a special protocol. The diagnosis was based on positive slit skin smear and histopathologic studies when needed. RESULTS: The age of the patients ranged between 1 and 65 years (median age, 19 years) and the duration of the disease ranged between 2 months and 12 years (median duration, 6 months). Most of the patients had a single lesion. Slit skin smear was positive for parasites in 35 cases (highly positive in 15). From the analysis of features, the disease could be classified into dry type (31 patients), wet type (seven patients), and chronic cutaneous leishmaniasis (four patients). Most of the patients required prolonged (4-6 weeks) treatment with sodium stibogluconate; the smear was still positive in eight patients, seven of whom were further treated successfully with a combination of rifampicin and isoniazid for 2-3 months. One who was treated successfully with intralesional injections of sodium stibogluconate. CONCLUSIONS: Cutaneous leishmaniasis in this region seems to be endemic, has a long chronic course, shows a highly positive smear even 1-6 years after onset, and gives an inadequate response to prolonged systemic therapy. Further epidemiologic studies and taxonomic differentiation of the species are required.
Adolescent↗
Complement factor H gene mutation associated with autosomal recessive atypical hemolytic uremic syndrome.
Atypical hemolytic uremic syndrome (HUS) presents with the clinical features of hypertension, microangiopathic hemolytic anemia, and acute renal failure. Both dominant and recessive modes of inheritance have been reported. This study describes the genetic and functional analysis of a large Bedouin kindred with autosomal recessive HUS. The kindred consists of several related nuclear families in which all parent unions of affected children are consanguineous. A previous report demonstrated that a dominant form of HUS maps to chromosome 1q and that complement factor H (CFH), a regulatory component of the complement system, lies within the region and is involved in the dominant disorder. Early-onset and persistent hypocomplementemia in this Bedouin kindred prompted us to evaluate the CFH gene. Linkage analysis was performed, demonstrating linkage between the disorder and the markers near the CFH gene. Mutation analysis of the CFH coding region revealed a single missense mutation. Functional analyses demonstrate that the mutant CFH is properly expressed and synthesized but that it is not transported normally from the cell. This is the first study reporting that a recessive, atypical, early-onset, and relapsing HUS is associated with the CFH protein and that a CFH mutation affects intracellular trafficking and secretion.
Age of Onset↗
Iraqi-Jewish kindreds with optic atrophy plus (3-methylglutaconic aciduria type 3) demonstrate linkage disequilibrium with the CTG repeat in the 3' untranslated region of the myotonic dystrophy protein kinase gene.
Iraqi-Jewish optic atrophy plus is an autosomal recessive condition characterized by infantile optic atrophy, an early onset movement disorder, and 3-methylglutaconic aciduria. Other features include spastic paraplegia, mild ataxia, mild cognitive deficiency and dysarthria. This disorder was identified in inbred Iraqi-Jewish kindreds in which relationships between most of the affected individuals were unknown. In this study we identify linkage to chromosome 19q13.2-q13.3 by using a DNA pooling strategy to perform a genome wide screen followed by a high density search for shared segments among affected individuals in candidate regions identified in the initial genome wide screen. A significantly high positive lod score of 6.14 at zero recombination was obtained for the CTG repeat in the 3' untranslated region of the myotonic dystrophy protein kinase gene. The existence of multiple recombinant individuals indicates the disease interval can be further narrowed with additional markers. Linkage disequilibrium was seen in six polymorphic markers across a 1 Mb interval. This region is well characterized and contains several candidate genes.
Chromosome Mapping↗
Apoptosis in myocytes in end-stage heart failure.
BACKGROUND: Heart failure can result from a variety of causes, including ischemic, hypertensive, toxic, and inflammatory heart disease. However, the cellular mechanisms responsible for the progressive deterioration of myocardial function observed in heart failure remain unclear and may result from apoptosis (programmed cell death). METHODS: We examined seven explanted hearts obtained during cardiac transplantation for evidence of apoptosis. All seven patients had severe chronic heart failure: four had idiopathic dilated cardiomyopathy, and three had ischemic cardiomyopathy. DNA fragmentation (an indicator of apoptosis) was identified histochemically by in situ end-labeling as well as by agarose-gel electrophoresis of end-labeled DNA. Myocardial tissues obtained from four patients who had had a myocardial infarction one to two days previously were used as positive controls, and heart tissues obtained from four persons who died in motor vehicle accidents were used as negative controls for the end-labeling studies. RESULTS: Hearts from all four patients with idiopathic dilated cardiomyopathy and from one of the three patients with ischemic cardiomyopathy had histochemical evidence of DNA fragmentation. All four myocardial samples from patients with dilated cardiomyopathy also demonstrated DNA laddering, a characteristic of apoptosis, whereas this was not seen in any of the samples from patients with ischemic cardiomyopathy. Histological evidence of apoptosis was also observed in the central necrotic zone of acute myocardial infarcts, but not in myocardium remote from the infarcted zone. Rare isolated apoptotic myocytes were seen in the myocardium from the four persons who died in motor vehicle accidents. CONCLUSIONS: Loss of myocytes due to apoptosis occurs in patients with end-stage cardiomyopathy and may contribute to progressive myocardial dysfunction.
Accidents, Traffic↗
Ocular cicatricial pemphigoid. A case report of monozygotic twins discordant for the disease.
OBJECTIVE: To identify the major histocompatibility complex markers and the autoantibody associated with ocular cicatricial pemphigoid (OCP) in a proband, her unaffected cotwin, and the children of the cotwin. Ocular cicatricial pemphigoid is a chronic autoimmune disorder that affects the conjunctiva and other squamous epithelium. It is associated with the major histocompatibility complex class II alleles that are presumed to provide enhanced susceptibility to the disease. We encountered a pair of monozygotic female twins, one of whom has OCP. In addition to totally identical physical appearances since birth, the two sisters have identical blood groups. METHODS: The following studies were performed on the patient, her unaffected cotwin sister, and her children: (1) polymorphism of major histocompatibility complex class II genes by DNA typing, (2) sequence analysis of DQ beta gene second and third exons, and (3) serologic evaluation for the presence of anti-basement membrane zone autoantibodies specific for OCP by Western immunoblot with the use of skin and conjunctiva lysates as substrates. RESULT: Both monozygotic twins had the same HLA haplotypes. The sequence analysis of the second and third exons of DQ beta genes revealed no significant differences between the proband and her unaffected cotwin. Autoantibodies specific to OCP were detected only in the patient's serum. The serum of the unaffected cotwin and the other relatives did not demonstrate the presence of the OCP autoantibody. CONCLUSION: This isolated family study does not support a single-gene theory for the development of OCP. It is most likely due to a multigene effect and associated with environmental factors.
Aged↗
Pyridazine analogs of piperidinylbenzophenone immunomodulators. 70th communication: syntheses and reactions of pyridazines.
Starting from conveniently accessible halogen-substituted phenyl pyridazinyl ketones, a series of piperidinyl-substituted diazabenzophenones was prepared. The new compounds were screened for immunomodulatory activity.
Adjuvants, Immunologic↗