1/12. Monitoring disease activity in pemphigus with enzyme-linked immunosorbent assay using recombinant desmogleins 1 and 3.BACKGROUND: pemphigus is an antidesmoglein (Dsg) autoimmune disease that is divided into two major subtypes: pemphigus foliaceus (PF) and pemphigus vulgaris (PV). We previously developed enzyme-linked immunosorbent assays (ELISAs) using recombinant Dsg1 and Dsg3 to detect IgG autoantibodies in patients with pemphigus. The protocol for the ELISAs was optimized for serological diagnosis, but under the conditions used, these assays were not particularly useful for monitoring disease activity in certain patients. That is, the sera from some patients with high-titre antibodies continued to show high index values in the ELISA after clinical improvement. OBJECTIVES: In the study reported here, we modified the ELISA protocol to obtain 'true' index values that exhibit a better correlation with disease activity. methods: We tested two cases of pemphigus foliaceus (PF) and four cases of pemphigus vulgaris (PV), each with ELISA index values greater than 150 for Dsg1 or Dsg3. We ran an ELISA with sera from these patients serially diluted from 1 : 100 to 1 : 12,800. We then performed ELISA with a series of PV No. 1 sera diluted to 1 : 800 and PV No. 2-4 and PF No. 1-2 sera diluted to 1 : 1600, after which we plotted the ELISA index values against the time course of disease activity. RESULTS: In each of these cases, there was no apparent decline, over the course of the disease activity, in the ELISA index values at a serum dilution of 1 : 100, probably because the antigen-antibody reaction was saturated at that dilution. After running an ELISA with sera serially diluted from 1 : 100 to 1 : 12,800 we found that a linear dose-dependency between the dilution value and the index value was only observed when sera were diluted to 1 : 800 or more in one case (PV No.1) and to 1 : 1600 or more in the other five cases (PV No. 2-4, PF No. 1-2). After performing ELISA with these series as outlined above we plotted the ELISA index values against the time course of disease activity and found that the index values obtained from these appropriately diluted sera fluctuated in parallel with disease activity, and declined with clinical improvement. CONCLUSIONS: These findings indicate that when appropriate dilutions are used in Dsg1 and Dsg3 ELISA, these assays can provide useful serological information for assessing disease activity in PF and PV.- - - - - - - - - - ranking = 1keywords = outline (Clic here for more details about this article) |
2/12. Karyomegalic nephropathy: an uncommon cause of progressive renal failure.BACKGROUND: Karyomegalic nephropathy, first identified in 1974, represents an increasingly recognized, but perhaps underdiagnosed condition associated with interstitial nephritis. It undoubtedly leads to end-stage renal disease requiring renal support. methods AND RESULTS: We present a series of six cases of karyomegalic nephropathy. The age at diagnosis was 9-51 years, median 33 years. Impaired renal function, proteinuria, and haematuria were present in the majority of cases. Non-specific elevated liver enzymes were present in three cases. Two patients died soon after transplantation from overwhelming respiratory sepsis. The classical histological features of large, abnormal hyperchromatic nuclei with irregular outlines within epithelial cells were present in renal epithelial cells. Abnormality of dna ploidy distributions compared with age- and sex-matched controls, and characterized by the presence of significant numbers of cells with high ploidy values was present in cases but not in controls. Mitotic figures were absent. Proliferation markers, Ki-67 and proliferating cell nuclear antigen/cyclin were not significantly elevated in those cases examined. Human leukocyte antigen analysis did not support the clustering of A9 or B35, in the cases or their families. CONCLUSIONS: The presence of significant renal impairment, positive urine sediment, abnormal liver enzymes, and early age of onset should alert one to the presence of karyomegalic nephropathy. It represents an underdiagnosed disorder with a high degree of ploidy indicative of karyotypic abnormality.- - - - - - - - - - ranking = 1keywords = outline (Clic here for more details about this article) |
3/12. Neurofibromatosis type 1: report of two contrasting cases.We present two cases of neurofibromatosis type 1 (NF-1), one a 35 year old male who first recognised his problem at the age of fifteen years and at the time of assessment, satisfied the National Institute of health (NIH) diagnostic criteria for NF-1 and had a nodular plexiform neurofibroma involving the left fifth dorsal nerve root and a diffuse plexiform neurofibroma involving the left lower limb. The second patient, a 45 year old female recognised her problem at the age of 39 years, did not quite satisfy the NIH diagnostic criteria for NF 1 and had diffuse plexiform neurofibroma involving both lower limbs and buttocks almost symmetrically, a finding which has not previously been described to the best of our knowledge. The scarcity of management options are briefly outlined.- - - - - - - - - - ranking = 1keywords = outline (Clic here for more details about this article) |
4/12. Nontraumatic acute complete paraplegia resulting from cervical disc herniation: a case report.STUDY DESIGN: A case report of nontraumatic acute complete paraplegia resulting from cervical disc herniation. OBJECTIVES: To describe a rare case of nontraumatic paraplegia resulting from enlargement of a herniated disc in the cervical spine and to outline appropriate management of a patient with severe spinal cord compression secondary to disc herniation with developmental spinal canal stenosis. SUMMARY OF BACKGROUND DATA: Acute progression of myelopathy into complete paraplegia resulting from disc herniation is rare. There are only four reported cases of nontraumatic acute myelopathy secondary to cervical disc herniation. No other report has described magnetic resonance imaging findings noted before and after the onset of acute myelopathy. methods: A cervical disc herniation at C6-C7 is reported in a 29-year-old man who had nontraumatic acute complete paraplegia. Neurologic and magnetic resonance imaging findings are evaluated and discussed.RESULTS: Disc herniation at C6-C7 enlarged nontraumatically, resulting in complete paraplegia. Emergent anterior decompression followed by secondary posterior multilevel decompression was performed. magnetic resonance imaging studies revealed localized high signal intensity change in the spinal cord. No neurologic recovery was achieved 3 years post-surgery. CONCLUSION: We emphasize that there is a possibility of acute, irreversible progression of paralysis secondary to nontraumatic enlargement of cervical disc herniation with canal stenosis. In these cases, immediate early decompressive surgery is crucial to the prevention of severe myelopathy.- - - - - - - - - - ranking = 1keywords = outline (Clic here for more details about this article) |
5/12. Longitudinal analysis of hearing loss in a case of hemosiderosis of the central nervous system.OBJECTIVE: To describe cochleovestibular aspects of superficial hemosiderosis of the central nervous system. BACKGROUND: Superficial hemosiderosis of the central nervous system is a rare disease in which cochleovestibular impairment, cerebellar ataxia, and myelopathy are the most frequent signs. Chronic recurrent subarachnoidal hemorrhage with bleeding into the cerebrospinal fluid is the cause of deposition of hemosiderin in leptomeningeal and subpial tissue, cranial nerves, and spinal cord. Removing the cause of bleeding can prevent irreversible damage to these structures. Because this is the only effective treatment, an early diagnosis is crucial. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENT: A 72-year-old woman with superficial hemosiderosis of the central nervous system that developed when she was age 39. methods: Neurologic and imaging diagnostic examinations and longitudinal evaluation of cochleovestibular features were performed. neurosurgery was not performed. RESULTS: Progressive bilateral sensorineural hearing loss and severe vestibular hyporeflexia developed within 15 years, which can be attributed to lesions in the cochleovestibular system. Additional pathology of the central nervous system developed later. CONCLUSION: The patient demonstrated cochlear and vestibular findings that are typical of this pathologic condition. It is the first documented case with extensive serial audiometry used to precisely outline the degree of hearing deterioration during the course of the disease.- - - - - - - - - - ranking = 1keywords = outline (Clic here for more details about this article) |
6/12. Imprint cytology of metastatic sialoblastoma. A case report.BACKGROUND: Sialoblastoma is a rare, aggressive and potentially malignant perinatal/congenital tumor that recapitulates the developing salivary gland. There is only 1 brief description of the cytologic findings of metastatic sialoblastoma and 1 poorly documented case of lung metastasis in the literature. CASE: A 75-month-old girl with a history of recurrent sialoblastoma initially diagnosed at 21 months and treated with multiple incomplete surgical excisions, chemotherapy and radiation presented with a solitary lung nodule. Imprint smears and frozen section of the mass were diagnostic of metastatic sialoblastoma. CONCLUSION: Cytologic features of sialoblastoma showed complete concordance with histology and included the presence of variably arranged, tight, solid clusters of atypical-appearing, basaloidlike cells in a background of dispersed epithelial and myoepithelial cells. The clusters contained admixed benign ductal cells and dense, metachromatic, magenta hyaline globular material with smooth, rounded outlines. The differential diagnoses include neoplasms composed of either basaloid cells and/or admixed hyaline matrix material and included pleomorphic adenoma, basal cell adenoma and adenoid cystic carcinoma. All these neoplasms affect patients in the first 2 years of life, whereas sialoblastoma usually occurs in the first 2 decades of life.- - - - - - - - - - ranking = 1keywords = outline (Clic here for more details about this article) |
7/12. Peritoneal mesothelioma after environmental asbestos exposure.mesothelioma are primary malignant neoplasms of the serous membranes. They usually involve the pleura and rarely the pericardium, the peritoneum and the tunica vaginalis testis. About 90% are associated with exposure to asbestos. The exposure is generally occupational, an environmental inhalation of asbestos and asbestiform fibers in areas in turkey has been observed and presents a major health problem. This report of a patient from Anatolia with peritoneal mesothelioma after environmental exposure outlines the importance of considering this pathology in the differential diagnosis of a Turkish patient presenting with ascites.- - - - - - - - - - ranking = 1keywords = outline (Clic here for more details about this article) |
8/12. cardiac resynchronization therapy and automatic implantable cardiac defibrillators in the treatment of heart failure: a review article.PURPOSE: To review the use of cardiac resynchronization therapy (CRT) and automatic implantable cardiac defibrillators (AICDs) in heart failure (HF) patients. DATA SOURCES: Selected scientific literature. CONCLUSIONS: New developments in device therapy for HF patients are helping to decrease morbidity and mortality in this challenging patient population. CRT improves left ventricular (LV) ejection fraction, quality of life, 6-min walk distances, and new york Heart association scores in select patients. AICDs can prevent sudden cardiac death in those who have LV dysfunction and are at risk for ventricular arrhythmias. Cardiac devices are now becoming a standard of care for those with HF who meet certain criteria. IMPLICATIONS FOR PRACTICE: Despite advances in medical therapy for treating LV dysfunction, newly diagnosed patients face a 50% mortality rate in 5 years. The natural history of HF leads to continual deterioration of function unless adverse cardiac remodeling is reversed. Until recently, the only means for improving symptoms and cardiac function has been through the optimization of standard medicines that are indicated for LV dysfunction, such as angiotensin-converting enzyme inhibitors and beta-blockers. However, not all patients benefit from medical management alone. Cardiac devices may now be considered when significant symptoms persist after standard medicines are optimized. When practitioners use a multiple-modality approach, careful patient selection based on the inclusion criteria used in the trials outlined in this article will likely lead to improved management of those with LV dysfunction.- - - - - - - - - - ranking = 1keywords = outline (Clic here for more details about this article) |
9/12. kidney disease associated with primary antiphospholipid syndrome: clinical signs and histopathological features in an case experience of five cases.BACKGROUND: The primary antiphospholipid syndrome (PAPS) is characterized by the presence of circulating antiphospholipid antibodies, clinically associated with blood hypercoagulability. Renal involvement in course of PAPS is very frequent, although the true prevalence of PAPS-correlated kidney disease is difficult to estimate. MATERIALS AND methods: We reviewed 270 consecutive renal biopsies examined in our nephrology Division of Bari University Hospital between 1998 and 2004 to identify those performed in patients with PAPS. RESULTS: We identified five biopsies performed in patients with PAPS. In three patients the diagnosis of PAPS was made at onset of the kidney disorder, while in the other two cases the initial diagnosis was primary focal segmental glomerulosclerosis (FSGS). In these cases the subsequent finding of positive antiphospholipid antibodies reoriented the diagnosis toward PAPS-correlated nephropathy. The clinical onset of kidney disease consisted of acute renal failure in three patients and urinary abnormalities in the other two. Histological examination of renal biopsies showed vascular lesions (intimal fibrous hyperplasia, arteriolar hyalinosis, double outline of the capillary walls) in four patients. Focal segmental glomerulosclerosis was present in four patients, two of whom showed double outline of the capillary walls. All patients had tubulo-interstitial lesions, while immunofluorescence was positive in only two patients. All patients preserved stable renal function throughout follow-up (mean value: 10.6 years, range 4 months-24 years). The prevalence of PAPS-correlated nephropathy in our population was 1.85% CONCLUSION: Our data confirm that PAPS-associated nephropathy has slow progression and rarely leads to end-stage renal failure. The prevalence of PAPS-correlated nephropathy is likely underestimated because some patients with a diagnosis of primary focal sclerosis may actually be affected by PAPS.- - - - - - - - - - ranking = 2keywords = outline (Clic here for more details about this article) |
10/12. Advancing prostate cancer: treatment options for the urologist.Today's urologists often face the dilemma of how to treat patients who have advancing prostate cancer. The diversity of this patient population makes treatment decisions challenging. For over 60 years the mainstay of treatment for patients who have advancing prostate cancer has been androgen deprivation therapy. Now there are new chemotherapeutic options, novel hormone manipulations, and other adjunctive therapies available. Based on a case presentation, the authors have attempted to outline for the practicing urologist, a logical progression of treatment options for advancing prostate cancer.- - - - - - - - - - ranking = 1keywords = outline (Clic here for more details about this article) |
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