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Rapid Progression (rapid + progression)
Selected AbstractsRapid Progression of a Basal Cell Carcinoma After Photodynamic TherapyDERMATOLOGIC SURGERY, Issue 8 2010JULIE K. KAREN MD The authors have indicated no significant interest with commercial supporters. [source] A Rare Case of Rapid Progression from Incurved Nail to Pincer NailDERMATOLOGIC SURGERY, Issue 9 2009MASAAKI KOSAKA MD No abstract is available for this article. [source] Rapid progression of small cell carcinoma in a renal transplant recipientINTERNATIONAL JOURNAL OF UROLOGY, Issue 6 2006ANTTI RANNIKKO Abstract, Immunosuppression is thought to be responsible for the increased incidence of tumor development after organ transplantation. Natural history of these tumors may be more aggressive than would be expected for a similar tumor in a patient without transplant. We describe the fulminant course of small cell carcinoma, likely of prostatic origin, in a kidney transplant patient. Small cell carcinoma (SCC) and especially SCC of the prostate is an aggressive and rare type of tumor. Due to its rarity, only case reports have been published. To our knowledge, this is the first case of SCC, likely of prostatic origin, in an organ transplant recipient (OTR). The case illustrates the aggressiveness of the disease as reflected by its fulminant progression, which may have been further accentuated by the immunosuppression. [source] Nipah virus RNA synthesis in cultured pig and human cellsJOURNAL OF MEDICAL VIROLOGY, Issue 8 2006Li-Yen Chang Abstract Nipah virus infection of porcine stable kidney cells (PS), human neuronal cells (SK-N-MC), human lung fibroblasts cells (MRC-5), and human monocytes (THP-1) were examined. Rapid progression of cytopathic effects (CPE) and cell death were noted in PS cell cultures treated with Nipah virus, followed by MRC-5, SK-N-MC, and THP-1 cell cultures, in descending order of rapidity. Significant increase in the intracellular Nipah virus RNA occurred beginning at 24 hr PI in all the infected cells. Whereas, the extracellular release of Nipah virus RNA increased significantly beginning at 48 and 72 hr PI for the infected MRC-5 cells and PS cells, respectively. No significant release of extracellular Nipah virus RNA was detected from the Nipah virus-infected SK-N-MC and THP-1 cells. At its peak, approximately 6.6 log PFU/µl of extracellular Nipah virus RNA was released from the Nipah virus-infected PS cells, with at least a 100-fold less virus RNA was recorded in the Nipah virus-infected SK-N-MC and THP-1. Approximately 15.2% (±0.1%) of the released virus from the infected PS cell cultures was infectious in contrast to approximately 5.5% (±0.7%) from the infected SK-N-MC cells. The findings suggest that there are no differences in the capacity to support Nipah virus replication between pigs and humans in fully susceptible PS and MRC-5 cells. However, there are differences between these cells and human neuronal cells and monocytes in the ability to support Nipah virus replication and virus release. J. Med. Virol. 78:1105,1112, 2006. © 2006 Wiley-Liss, Inc. [source] Rapid progression of subclinical age-related macular degeneration in the untreated fellow eye after intravitreal bevacizumabACTA OPHTHALMOLOGICA, Issue 6 2009Young Hee Yoon No abstract is available for this article. [source] Rapidly progressive internal root resorption: a case reportDENTAL TRAUMATOLOGY, Issue 5 2008David Keinan Usually the process is asymptomatic and diagnosed upon routine radiographic examination. This case report presents a rapid progression of internal resorption related directly to traumatic injury. A 16-year-old female arrived at the emergency room after a mild extrusion of the mandibular incisors. The initial treatment included repositioning and splinting of the teeth. Radiographs performed at repositioning and splinting demonstrated normal configuration of the incisor's roots. Ten months later progressive internal resorption of the left mandibular first incisor was diagnosed. While treating this tooth similar process was detected in the right mandibular second incisor and in the mandibular left second incisor. The lower right first incisor reacted inconsistently to vitality test. As a result of the severe and rapidly progressive nature of the process, root canal treatments were performed in all lower incisors. The follow-up radiographs demonstrate arrest of the internal resorption process. [source] The successful use of maggots in necrotizing fasciitis of the neck: A case reportHEAD & NECK: JOURNAL FOR THE SCIENCES & SPECIALTIES OF THE HEAD AND NECK, Issue 8 2004Simon F. Preuss MD Abstract Background. The use of maggots to digest necrotic tissue as a form of wound debridement has a long history in medicine. Necrotizing fasciitis of the neck has a high mortality rate despite aggressive surgical and medical intervention. The use of maggots in this disease has been reported only once before. Methods. We report the case of a 73-year-old woman, who underwent neck dissection and had necrotizing fasciitis of the neck develop shortly after. After initial surgical wound debridement, we used maggots as a biosurgical method for further debridement. A net containing 100 maggots (Biobag; BioMonde, Germany) was used. Results. Daily wound dressing showed rapid improvement of the wound; 4 days after beginning treatment, the wound was free of necroses. Conclusion. In this case, we could avoid repeated surgical wound debridement with the use of sterile maggots. The frequently rapid progression of necrotizing fasciitis could be well controlled. © 2004 Wiley Periodicals, Inc. Head Neck26: 747,750, 2004 [source] Development of autoimmune hepatitis in patients with typical primary biliary cirrhosis,HEPATOLOGY, Issue 1 2006Raoul Poupon Primary biliary cirrhosis (PBC),autoimmune hepatitis (AIH) overlap syndrome is a clinical entity characterized by the occurence of both conditions at the same time in the same patient. In addition to PBC-AIH overlap syndrome, transitions from one autoimmune disease to another have been reported, but no systematic series have been published. We report a series of 12 patients with consecutive occurrence of PBC and AIH (i.e., PBC followed by AIH). Among 282 PBC patients, 39 were identified who fulfilled criteria for probable or definitive AIH. AIH developed in 12 patients (4.3%). The baseline characteristics of the patients were similar to those of patients with classical PBC. Time elapsed between the diagnosis of PBC and the diagnosis of AIH varied from 6 months to 13 years. Patients with multiple flares of hepatitis at the time of diagnosis of AIH had cirrhosis on liver biopsy. Ten patients were given prednisone ± azathioprine; short-term as well as sustained remissions were obtained in 8 of these, while two had multiple relapses and eventually died 8 and 7 years after diagnosis of AIH. In conclusion, the development of superimposed AIH could not be predicted from baseline characteristics and initial response to UDCA therapy. If not detected early, superimposed AIH can result in rapid progression toward cirrhosis and liver failure in PBC patients. (HEPATOLOGY 2006;44:85,90.) [source] Frequent inactivation of SPARC by promoter hypermethylation in colon cancersINTERNATIONAL JOURNAL OF CANCER, Issue 3 2007Eungi Yang Abstract Epigenetic modification of gene expression plays an important role in the development of human cancers. The inactivation of SPARC through CpG island methylation was studied in colon cancers using oligonucleotide microarray analysis and methylation specific PCR (MSP). Gene expression of 7 colon cancer cell lines was evaluated before and after treatment with the demethylating agent 5-aza-2,-deoxycytidine (5Aza-dC) by oligonucleotide microarray analysis. Expression of SPARC was further examined in colon cancer cell lines and primary colorectal cancers, and the methylation status of the SPARC promoter was determined by MSP. SPARC expression was undetectable in 5 of 7 (71%) colorectal cancer cell lines. Induction of SPARC was demonstrated after treatment with the demethylating agent 5Aza-dC in 5 of the 7 cell lines. We examined the methylation status of the CpG island of SPARC in 7 colon cancer cell lines and in 20 test set of colon cancer tissues. MSP demonstrated hypermethylation of the CpG island of SPARC in 6 of 7 cell lines and in all 20 primary colon cancers, when compared with only 3 of 20 normal colon mucosa. Immunohistochemical analysis showed that SPARC expression was downregulated or absent in 17 of 20 colon cancers. A survival analysis of 292 validation set of colorectal carcinoma patients revealed a poorer prognosis for patients lacking SPARC expression than for patients with normal SPARC expression (56.79% vs. 75.83% 5-year survival rate, p = 0.0014). The results indicate that epigenetic gene silencing of SPARC is frequent in colon cancers, and that inactivation of SPARC is related to rapid progression of colon cancers. © 2007 Wiley-Liss, Inc. [source] Discontinuation of penicillamine in the absence of alternative orphan drugs (trientine,zinc): a case of decompensated liver cirrhosis in Wilson's diseaseJOURNAL OF CLINICAL PHARMACY & THERAPEUTICS, Issue 1 2007C. C. Ping MPharm Summary Objectives:, To report a case of early-decompensated liver cirrhosis secondary to discontinuation of penicillamine therapy in a patient with Wilson's disease. Case summary:, A 33-year-old Chinese female patient was diagnosed with Wilson's disease, for which penicillamine 250 mg p.o. once daily was prescribed. However, the patient developed intolerance and penicillamine was discontinued without alternative treatment. Five months later, she developed decompensated liver cirrhosis with hepatic encephalopathy. Eventually, the patient died because of the complications of sepsis and decompensated liver failure. Discussion:, Chelating agent is the mainstay of treatment in Wilson's disease, which is an inherited disorder of hepatic copper metabolism. Therapy must be instituted and continued for life once diagnosis is confirmed. Interruption of therapy can be fatal or cause irreversible relapse. Penicillamine given orally is the chelating agent of first choice. However, its unfavourable side-effects profile leads to discontinuation of therapy in 20,30% of patients. In most case reports, cessation of penicillamine without replacement treatment causes rapid progression to fulminant hepatitis, which is fatal unless liver transplantation is performed. Conclusion:, In this, we highlight a case of discontinuation of penicillamine in a patient with Wilson's disease without substitution with alternative regimen. This was caused by unavailability of the alternative agents such as trientine in our country. Consequently, the patient progressed to decompensated liver cirrhosis with encephalopathy and eventually passed-away within 5 months. One recent study supports a combination of trientine and zinc in treating patient with decompensated liver cirrhosis. This combination is capable of reversing liver failure and prevents the need of liver transplantation. Both trientine and zinc are not registered in Malaysia. Therefore, liver transplantation was probably the only treatment option for this patient. Hence, non-availability of orphan drugs in clinical practice is certainly a subject of serious concern. Systems for better management of patients with rare diseases need to be instituted by all the institutions concerned. [source] Nicotinic acetylcholine receptor activation mediates nicotine-induced enhancement of experimental periodontitisJOURNAL OF PERIODONTAL RESEARCH, Issue 3 2009T. Breivik Background and Objective:, Smokers have an increased risk of developing periodontitis as well as showing more rapid progression and resistance to treatment of the disease, but the biological mechanisms are poorly understood. This study was designed to investigate putative biological mechanisms by which nicotine may enhance the susceptibility and thus the course of periodontitis in an animal model. Material and Methods:, Ligature-induced periodontitis was applied in periodontitis-susceptible Fischer 344 rats. The animals were either given daily intraperitoneal injections of the nicotinic acetylcholine receptor antagonist mecamylamine (1 mg/kg) 45 min before subcutaneous injections in the neck skin of nicotine (0.8 mg/kg), or treated with the same amount of saline intraperitoneally and nicotine subcutaneously, or treated with mecamylamine and saline. Control animals received intraperitoneal and subcutaneous injections of saline only. Periodontal bone loss was assessed when the ligatures had been in place for 3 wk. Two hours before decapitation, all rats received lipopolysaccharide (LPS; 100 ,g/kg, intraperitoneally) to induce a robust immune and stress response. Results:, Compared with saline/saline-treated control animals, saline/nicotine-treated rats developed significantly more periodontal bone loss, and LPS provoked a significantly smaller increase in circulating levels of the cytokines tumour necrosis factor-,, transforming growth factor-1, and interleukin-10. Mecamylamine pretreatment of nicotine-treated rats abrogated the increased periodontal bone loss and the LPS-induced decrease in tumour necrosis factor-,, but had no significant effects on the levels of transforming growth factor-1, and interleukin-10, or the stress hormone corticosterone. Conclusion:, The results indicate that nicotine enhances the susceptibility to periodontitis via nicotinic acetylcholine receptors, which may act by suppressing protective immune responses through the cholinergic anti-inflammatory pathway. [source] Long-term benefit to pallidal deep brain stimulation in a case of dystonia secondary to pantothenate kinase-associated neurodegenerationMOVEMENT DISORDERS, Issue 12 2006Martin Krause MD Abstract Pantothenate kinase-associated neurodegeneration (PKAN) is a rare autosomal recessive disorder with onset in childhood and rapid progression. There is no causative and insufficient symptomatic drug therapy. Deep brain stimulation (DBS) of the internal pallidum (GPi) has been reported to improve motor function. Most case reports, however, are limited to short observational periods. The impact of DBS on the progression and life expectancy in PKAN is unknown. We present a 5-year outcome and video documentation of bilateral GPi-DBS of an adolescent patient suffering from genetically defined PKAN. © 2006 Movement Disorder Society [source] Cervico-facial necrotizing fasciitisORAL DISEASES, Issue 2 2009R Ord Necrotizing fasciitis of the cervical facial region is a rare entity that has seen an increasing prevalence in the last 20 years. It is most common in patients with an underlying systemic disease leading to immunosuppression, but can be seen in healthy adults and children. It is characterized by soft tissue destruction which is disproportionate to its clinical symptoms and signs, with rapid progression and fatal outcome, if not treated rapidly and radically. We present a review of the etio-pathogenesis and management of this challenging disease. [source] Short-term follow-up of patients with sickle cell disease and albuminuriaPEDIATRIC BLOOD & CANCER, Issue 6 2008Ofelia Alvarez MD Abstract Background Albuminuria with normal serum creatinine occurs frequently in patients with sickle cell disease (SCD), but the rate of progression to more advanced chronic renal disease is unknown. The purpose of this study was to investigate the rate of progression of children and young adults with SCD and albuminuria over time. Procedure Urine albumin/creatinine (A/C) ratios and serum creatinine were obtained serially. Serum cystatin C levels were determined in a subgroup of 20 patients. Results Of 38 patients with SCD who had albuminuria (30 with microalbuminuria and 8 with proteinuria), 10.5% had progressive disease during follow-up of 20,±,12 months. Progressive disease was observed in 2 of 30 patients with MA because MA worsened to either intermittent proteinuria (1 patient), or persistent proteinuria after 7 months follow-up (1 patient). Two of eight patients with proteinuria worsened to nephrotic-range after 8 and 17 months with elevations of serum creatinine. All eight patients with proteinuria were treated with angiotensin blockade and/or hydroxyurea. Of those, six patients responded to treatment with decreased albuminuria and no changes in serum creatinine. Serum cystatin C level trended to increase before serum creatinine in patients with proteinuria. Conclusions Patients with rapid progression to nephrotic-range proteinuria showed decreased kidney function. Therefore, patients with albuminuria should be monitored closely for progression, and therapy with hydroxyurea and/or angiotensin blockade should be considered for patients who develop proteinuria. Serum cystatin C appears more sensitive than serum creatinine to detect early decrease in kidney function. Pediatr Blood Cancer 2008;50:1236,1239. © 2008 Wiley-Liss, Inc. [source] Severe Course of Primary Hyperoxaluria and Renal Failure After Domino Hepatic TransplantationAMERICAN JOURNAL OF TRANSPLANTATION, Issue 9 2005Alessandro Franchello We report herein a domino orthotopic liver transplantation (LT), from a 38-year-old woman undergoing liver-kidney transplantation (LKT) for primary hyperoxaluria type I (PH1) to a recipient with cirrhosis and hepatocellular carcinoma. Delayed onset of PH1 and renal failure and 10% residual alanine-glyoxylate aminotransferase (AGT) activity in domino liver justified its use for domino procedure. The clinical course after LKT was similar to that described in other series, including ours. Renal function started promptly and maintained despite sustained hyperoxaluria from dissolution of oxalotic deposits. Conversely, the domino recipient manifested severe hyperoxaluria and developed nephrolithiasis and renal insufficiency with rapid progression over 2 months. A new LT resulted in slow decrease of oxaluria and improvement of renal function. Therefore, PH1 behaved quite differently in these two patients, leading us to conclude that domino LT using livers from PH1 patients should be considered very carefully, only as a bridge to definitive LT in recipients with critical clinical conditions. [source] Phasic muscle activity in sleep and clinical features of Parkinson diseaseANNALS OF NEUROLOGY, Issue 3 2010Donald L. Bliwise PhD Objective: The absence of atonia during rapid eye movement (REM) sleep and dream-enactment behavior (REM sleep behavior disorder [RBD]) are common features of sleep in the alpha-synucleinopathies. This study examined this phenomenon quantitatively, using the phasic electromyographic metric (PEM), in relation to clinical features of idiopathic Parkinson disease (PD). Based on previous studies suggesting that RBD may be prognostic for the development of later parkinsonism, we hypothesized that clinical indicators of disease severity and more rapid progression would be related to PEM. Methods: A cross-sectional convenience sample of 55 idiopathic PD patients from a movement disorders clinic in a tertiary care medical center underwent overnight polysomnography. PEM, the percentage of 2.5-second intervals containing phasic muscle activity, was quantified separately for REM and non-REM (NREM) sleep from 5 different electrode sites. Results: Higher PEM rates were seen in patients with symmetric disease, as well as in akinetic-rigid versus tremor-predominant patients. Men had higher PEM relative to women. Results occurred in all muscle groups in both REM and NREM sleep. Interpretation: Although our data were cross-sectional, phasic muscle activity during sleep suggests disinhibition of descending motor projections in PD broadly reflective of more advanced and/or progressive disease. Elevated PEM during sleep may represent a functional window into brainstem modulation of spinal cord activity and is broadly consistent with the early pathologic involvement of non-nigral brainstem regions in PD, as described by Braak. ANN NEUROL 2010 [source] Oxidized/misfolded superoxide dismutase-1: the cause of all amyotrophic lateral sclerosis?ANNALS OF NEUROLOGY, Issue 6 2007Edor Kabashi PhD The identification in 1993 of superoxide dismutase-1 (SOD1) mutations as the cause of 10 to 20% of familial amyotrophic lateral sclerosis cases, which represents 1 to 2% of all amyotrophic lateral sclerosis (ALS) cases, prompted a substantial amount of research into the mechanisms of SOD1-mediated toxicity. Recent experiments have demonstrated that oxidation of wild-type SOD1 leads to its misfolding, causing it to gain many of the same toxic properties as mutant SOD1. In vitro studies of oxidized/misfolded SOD1 and in vivo studies of misfolded SOD1 have indicated that these protein species are selectively toxic to motor neurons, suggesting that oxidized/misfolded SOD1 could lead to ALS even in individuals who do not carry an SOD1 mutation. It has also been reported that glial cells secrete oxidized/misfolded mutant SOD1 to the extracellular environment, where it can trigger the selective death of motor neurons, offering a possible explanation for the noncell autonomous nature of mutant SOD1 toxicity and the rapid progression of disease once the first symptoms develop. Therefore, considering that sporadic (SALS) and familial ALS (FALS) cases are clinically indistinguishable, the toxic properties of mutated SOD1 are similar to that of oxidized/misfolded wild-type SOD1 (wtSOD1), and secreted/extracellular misfolded SOD1 is selectively toxic to motor neurons, we propose that oxidized/misfolded SOD1 is the cause of most forms of classic ALS and should be a prime target for the design of ALS treatments. Ann Neurol 2007 [source] Sézary syndrome associated with granulomatous lesions during treatment with bexaroteneBRITISH JOURNAL OF DERMATOLOGY, Issue 2 2006A. Ruiz-de-Casas Summary Sézary syndrome (SS) is a leukaemic variant of cutaneous T-cell lymphoma (CTCL). We report a patient with SS who developed granulomatous lesions. These lesions broke out during treatment with bexarotene when the disease had appeared to stabilize. After a partial clinical remission the disease showed rapid progression and finally led to the patient's death. This contradicts the initial assessment, which considered the granulomatous inflammation as a good prognostic factor in CTCL. [source] Diagnostic value and prognostic significance of protein S-100,, melanoma-inhibitory activity, and tyrosinase/MART-1 reverse transcription-polymerase chain reaction in the follow-up of high-risk melanoma patientsCANCER, Issue 7 2003Claus Garbe M.D. Abstract BACKGROUND Cutaneous melanoma is the most aggressive form of skin carcinoma in humans, frequently with a rapid progression of disease. To detect early developing metastasis, laboratory tests to determine levels of lactate dehydrogenase (LDH) and alkaline phosphatase (AP) form part of the regular follow-up, but often cannot discover recurrent disease at a sufficiently early stage. METHODS To evaluate the diagnostic accuracy of protein S-100, (S-100,), melanoma-inhibitory activity (MIA), LDH, AP, and tyrosinase/MART-1 reverse transcription-polymerase chain reaction (RT-PCR), the authors included 296 consecutive AJCC Stage II or III clinically disease-free melanoma patients. Follow-up examinations were performed every 3 months and blood samples were drawn to determine the levels of these tumor markers. RESULTS Metastasis occurred in 41 of the 296 patients during a median follow-up period of 19 months (range, 1,33 months). The sensitivity to detect new metastases was 29% for protein S-100,, 22% for MIA, 2% for LDH, 17% for AP, and 24% for RT-PCR. The diagnostic accuracy was best for MIA (86%) and S-100, (84%), whereas AP (79%), LDH (77%), and RT-PCR (72%) demonstrated lower values. Elevated values of S-100, and MIA during follow-up examinations were associated with decreased survival rates in the further course of the disease, but pathologic findings of the other tumor markers showed no prognostic impact. CONCLUSIONS To the authors' knowledge, the current study is the first comparison of the diagnostic accuracy of currently available tumor markers in the follow-up of high-risk melanoma patients. Protein S-100, and MIA demonstrated a higher sensitivity, specificity, and diagnostic accuracy in the diagnosis of newly occurring metastasis compared with to the tumor markers AP, LDH, and RT-PCR diagnostics. Therefore, the tumor markers S-100, and MIA may be useful in the follow-up of disease-free Stage II and III melanoma patients. Cancer 2003;97:1737,45. © 2003 American Cancer Society. DOI 10.1002/cncr.11250 [source] Gene expression profiling of advanced-stage serous ovarian cancers distinguishes novel subclasses and implicates ZEB2 in tumor progression and prognosisCANCER SCIENCE, Issue 8 2009Kosuke Yoshihara To elucidate the mechanisms of rapid progression of serous ovarian cancer, gene expression profiles from 43 ovarian cancer tissues comprising eight early stage and 35 advanced stage tissues were carried out using oligonucleotide microarrays of 18 716 genes. By non-negative matrix factorization analysis using 178 genes, which were extracted as stage-specific genes, 35 advanced stage cases were classified into two subclasses with superior (n = 17) and poor (n = 18) outcome evaluated by progression-free survival (log rank test, P = 0.03). Of the 178 stage-specific genes, 112 genes were identified as showing different expression between the two subclasses. Of the 48 genes selected for biological function by gene ontology analysis or Ingenuity Pathway Analysis, five genes (ZEB2, CDH1, LTBP2, COL16A1, and ACTA2) were extracted as candidates for prognostic factors associated with progression-free survival. The relationship between high ZEB2 or low CDH1 expression and shorter progression-free survival was validated by real-time RT-PCR experiments of 37 independent advanced stage cancer samples. ZEB2 expression was negatively correlated with CDH1 expression in advanced stage samples, whereas ZEB2 knockdown in ovarian adenocarcinoma SKOV3 cells resulted in an increase in CDH1 expression. Multivariate analysis showed that high ZEB2 expression was independently associated with poor prognosis. Furthermore, the prognostic effect of E-cadherin encoded by CDH1 was verified using immunohistochemical analysis of an independent advanced stage cancer samples set (n = 74). These findings suggest that the expression of epithelial,mesenchymal transition-related genes such as ZEB2 and CDH1 may play important roles in the invasion process of advanced stage serous ovarian cancer. (Cancer Sci 2009) [source] Axonal and demyelinating forms of the MPZ Thr124Met mutationACTA NEUROLOGICA SCANDINAVICA, Issue 3 2003S. Kurihara Objective , We report on a Japanese family with Charcot,Marie,Tooth disease (CMT) with the Thr124Met mutation in the peripheral myelin protein zero (MPZ) gene. Material and methods , Based on the clinical study, we investigated MPZ gene by direct sequence analysis and polymerase chain reaction,restriction fragment length polymorphism analysis. Results , Genotyping of four symptomatic family members showed that one family member with severe disease symptoms was homozygous, while the other three were heterozygous. The heterozygous cases were clinicopathologically determined to be the axonal type, which is characterized by late-onset and slow progression associated with Adie's pupil and deafness. The homozygous case was the demyelinating type, which showed earlier onset, rapid progression, sural nerve demyelination, and cranial nerve demyelination at autopsy. Conclusions , We suggest that axonal and demyelinating forms of CMT are not two distinct classes, but rather parts of a spectrum of genotypically related conditions, particularly with some MPZ mutations. [source] Non-atopic intrinsic asthma and the ,family tree' of chronic respiratory disease syndromesCLINICAL & EXPERIMENTAL ALLERGY, Issue 6 2009P. G. Holt Summary We present a scheme below in which the most common forms of inflammatory diseases of the respiratory tract, notably atopic and non-atopic asthma and COPD, are depicted as separate offshoots from a common ,at-risk' pathway underpinned by genotypes related to aberrations in control of host defence and tissue repair mechanisms. We propose that entrance into this pathway is initially programmed by environmental experience during infancy and early childhood, in particular by severe lower respiratory tract infection, and that further progression towards expression of specific disease phenotype(s) is determined by the nature, timing and frequency of additional environmental insults subsequently encountered. At the one extreme, early sensitization of at-risk subjects to aeroallergens can potentially drive rapid progression towards expression of the atopic asthmatic phenotype under the dual onslaught of inflammatory responses to allergens/pathogens. At the opposite end of the spectrum the drip-feed effects of occasional infections on respiratory function(s) are amplified over a longer time frame by inflammation resulting from exposure to tobacco smoke and/or related chemical pollutants. Non-atopic asthma is envisaged to fit between these two extremes, being driven essentially by the downstream effects of respiratory infections alone in at-risk subjects. An important common factor in all three disease phenotypes is that acute exacerbations are typically driven by infections, the host responses to which display a characteristic T-helper type 2-like footprint, which in our view points to underlying genotype(s) which result in unbalanced host responses to respiratory pathogens. [source] Haemangioendothelioma on the conjunctiva of the upper eyelidCLINICAL & EXPERIMENTAL OPHTHALMOLOGY, Issue 8 2006Sang Hyoung Cho MD Abstract A 62-year-old man visited the authors' clinic complaining of a mass on the palpebral conjunctiva of the right upper eyelid. The 2.0 cm × 1.2 cm sized, red and painless mass underwent incision and biopsy for histopathological examination. The mass was confirmed to be Kaposiform haemangioendothelioma characterized by densely packed spindle cells. These cells were positive to vimentin, CD31 and factor VIII-related antigen by immunohistochemical stain. The mass was completely resolved by oral steroid therapy and has not recurred through the presentation. Kaposiform haemangioendothelioma generally occurs in infant and adolescent periods and is characterized by rapid progression and invasion to adjacent tissue. Herein, an unusual case of Kaposiform haemangioendothelioma affecting the conjunctiva of the upper eyelid on a middle-aged man is reported. [source] Severe loss of vision after removal of cataract caused by intravitreal triamcinolone in combination with photodynamic therapy for exudative age-related macular degenerationCLINICAL & EXPERIMENTAL OPHTHALMOLOGY, Issue 5 2005Jörgen Larsson MD Abstract Intravitreal triamcinolone has been suggested as an adjunctive to photodynamic therapy in the treatment of exudative macular degeneration. One of the side-effects of intravitreal triamcinolone is the development of cataract, and it is known that cataract extraction can exacerbate macular degeneration. A case is presented where combined intravitreal triamcinolone and photodynamic therapy stopped the progression of the exudative macular degeneration. Subsequent progression of cataract required cataract extraction, which resulted in a very rapid progression of the exudative macular degeneration and loss of vision. [source] Recurrent hepatitis C virus disease after liver transplantation and concurrent biliary tract complications: poor outcomeCLINICAL TRANSPLANTATION, Issue 4 2006Lior H. Katz Abstract:, Recurrent hepatitis C virus (HCV) infection is particularly aggressive in the post-liver transplantation setting, with rapid progression of liver fibrosis. Biliary complications remain a significant cause of morbidity following liver transplantation. Post-cholecystectomy biliary strictures are associated with advanced hepatic fibrosis. The aim of this retrospective study was to determine whether the presence of biliary complications affects survival in liver transplant recipients with recurrent HCV disease. The files of liver transplant recipients (53.7% male; mean age 52.7 ± 10.3 yr) were reviewed for incidence, type and treatment of biliary complications, and findings were compared between those who developed recurrent HCV disease (n = 47, 83.9%) and those who did not (n = 9). Twenty-one biliary complications developed in 12 patients with recurrent HCV (25.5%). Treatment with endoscopic retrograde cholangiopancreatography or percutaneous transhepatic cholangiography with balloon dilatation and stent placement or surgical revision was successful in nine (75%). Three biliary complications developed in three patients with no recurrence (p = NS). There was no statistically significant association between recurrent HCV disease and biliary complications. However, among those with recurrent disease, the recurrence was severe in nine of 12 recipients with biliary complications (75%) but in only nine of 35 without biliary complications (26%) (p = 0.001). Death was documented in eight patients with severe recurrence (44.4%), including three (37.5%) with biliary complications and two (7%) with non-severe recurrence, neither of whom had biliary complications (p = 0.003). Antiviral treatment was successful in nine of 25 patients (36%) who received it. On multivariate analysis, biliary complications were a significant predictor of severe recurrence (OR 27.0, 95% confidence interval 2.07,351.4) (p = 0.012). Fibrosis stage in the second biopsy was significantly correlated with serum alanine aminotransferase (p = 0.01) and with duration of biliary obstruction (p = 0.07). In conclusion, biliary complications of liver transplantation strongly affect outcome in patients with recurrent HCV disease despite attempts to relieve the biliary obstruction and to treat the recurrent HCV disease. [source] | ||||||||||||||||||||||||||||