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Results for “early progression”

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At least 19 recordsLinked to original sources

PubMed1990

Utility of short-latency evoked potentials in the classification of progressive, early onset cerebellar ataxias.

In recent years, several authors have proposed new classifications of inherited ataxias, some of them being based on systematic clinical studies of large groups of patients. This methodic approach has led to the identification of new types of ataxias and helped the development of molecular biology research in these diseases. Up to now, nerve conduction velocity and evoked potential studies have not been considered in the classification of hereditary ataxias. We have studied the results of short latency evoked potentials in 102 patients affected by a early onset, progressive cerebellar ataxia. Based on the results of this study and a review of the literature on this subject, we will evaluate the utility of nerve conduction velocity and evoked potential recordings in the classification of this group of diseases.

Adult↗

PubMed1995

A progressive early onset cataract gene maps to human chromosome 17q24.

Cerulean cataract is an autosomal dominant, early onset, progressive cataract characterized by blue or white opacifications in the nucleus and cortex of the lens. A large four-generation pedigree in which cerulean cataract segregates was studied for linkage analysis. A genome wide search was undertaken after the plausible candidate genes were excluded and the cerulean cataract phenotype was mapped to chromosome 17q24. The three markers closest to the disease gene are D17S802 (Z)(theta) = 9.46 at (theta) = 0.085), D17S836 (Z(theta) = 5.26 at (theta) = 0.031) and AFMa238yb5 (Z(theta) = 7.11 at (theta) = 0.032). Multipoint linkage analyses yielded a maximum lod score of Z(theta) = 13.71, placing the cerulean cataract gene between D17S802 and D17S836 at (theta) = 0.048 and (theta) = 0.013, respectively.

Cataract↗

PubMed1995

Early progression of ophthalmoplegia in patients with ischemic oculomotor nerve palsies.

OBJECTIVE: To describe the frequency and clinical correlates of early progression of ophthalmoplegia in patients with ischemic oculomotor nerve palsies. DESIGN: Cohort survey, case series. SETTING: Multispecialty clinic providing primary, secondary, and tertiary care in central and northern Wisconsin. PATIENTS: Sixteen patients evaluated within 1 week of the reported onset of ischemic oculomotor nerve palsy were identified and followed up prospectively using a standardized ophthalmoplegia grading scheme. All patients were followed up serially until their ophthalmoplegia resolved. MAIN OUTCOME MEASURES: Descriptive analysis of the temporal course of ophthalmoplegia and frequency of progression of deficits. Comparison between the group that had progression of ophthalmoplegia with the group that did not for age, hematocrit, cholesterol level, and adiposity; presence of diabetes, hypertension, hypercholesterolemia, and coronary artery disease; history of stroke; and tobacco use. RESULTS: Eleven (69%) of 16 patients had progression of ophthalmoplegia. The median time between reported onset and peak severity of ophthalmoplegia was 10 days. The only important difference between the progressive and nonprogressive groups was a shorter time to resolution of ophthalmoplegia for the nonprogressive group. CONCLUSIONS: Early progression of ophthalmoplegia occurs often in patients with ischemic oculomotor nerve palsies. The power to find differences between progressive and nonprogressive groups was limited by the small number of patients available for analysis.

Aged↗

PubMed1990

The operative treatment of progressive early onset scoliosis. A preliminary report.

Thirteen patients with progressive early onset scoliosis have been managed operatively in an attempt to achieve correction without bracing and to allow the spine to grow. All had posterior segmental spinal instrumentation (SSI) without fusion and 9 of 13 had anterior apical growth arrest as a separate additional procedure. At 2-year follow-up, curve correction averaged 46%. Patients who had anterior apical growth arrest and SSI without fusion had less curve deterioration than those who had SSI alone. New methods are described for 1) measuring growth of the instrumented segment of the spine and 2) calculating the predicted growth of the instrumented segment. Eight of the 13 had more than 50% of predicted growth, three had 30-50% of predicted growth, and two had less than 30% of predicted growth. Operative treatment has been successful in the short term in all but the most malignant form of infantile idiopathic scoliosis.

Bone Wires↗

PubMed1980

Collagen types synthesized in dermal fibroblast cultures from patients with early progressive systemic sclerosis.

Fibroblast cultures were established by explant culture from upper and lower dermis of 7 normal donors and 10 patients with early progressive systemic sclerosis (PSS). Antibodies against collagens containing the A and B chains, collagens which occur largely in vascular structures, were isolated and used for immunofluorescence studies. Cultures from lower PSS dermis accumulated significantly more of these collagens than fibroblast cultures from upper PSS dermis or cultures from upper or lower dermis of normal skin. These data indicate that the collagens containing the A and B chains in early PSS lesions are produced by cells of vascular origin and support the concept that alterations in vascular tissue play a prominent role in the pathogenesis of PSS.

Adult↗

PubMed1988

Evoked potential studies in Friedreich's ataxia and progressive early onset cerebellar ataxia.

We recorded somatosensory evoked potentials (SEP) in 15 patients affected by Friedreich's ataxia (FA) and in 9 patients with progressive early onset cerebellar ataxia (PEOCA). Brainstem auditory evoked potentials (BAEP) were also recorded in 14 FA patients and in five PEOCA patients. SEP results showed clear differences between groups of FA, evidence of peripheral involvement was seen in all patients, with absence of the N9 potential or a major reduction of its amplitude. In patients in whom central responses could be recorded, conduction velocity was normal or near normal up to the brainstem but was reduced from brainstem to cerebral cortex. Four patients with PEOCA had SEP abnormalities similar to those seen in FA. In the five other patients, the amplitude and latency of N9 were normal but conduction velocity was reduced from brainstem to cerebral cortex. In FA, BAEP were abnormal in all patients with a disease duration of four years or more but were normal in four of the five PEOCA patients. Systematic evoked potential recording is useful in the investigation of hereditary ataxias.

Adolescent↗

PubMed1993

Early progression stage of malignancy of human colon border-line adenoma as revealed by immunohistochemical demonstration of increased DNA-instability.

The degree of DNA-instability was used as the marker of malignancy and applied to adenoma (7 benign cases and 17 border-line cases) and cancer (8 carcinoma-in-adenoma cases and 17 invasive cancer cases) of human colon. Proliferative activity by PCNA index and the activity of protein synthesis by AgNORs were also estimated for all cases as the supporting markers of malignancy. In all border-line cases, the following findings were obtained: (1), the degree of DNA-instability as revealed by immunohistochemical staining with anti-single-stranded DNA antiserum after acid hydrolysis was increased in border-line adenoma to the level of invasive overt cancer, indicating its malignancy with marked DNA-instability; (2), reflecting the malignant character, abnormal mitosis and single cell necrosis were usually observed in all border-line adenomas by fluorescent Feulgen staining, indicating the DNA alterations; (3), not only the parenchymal but also the stromal PCNA indices were statistically larger in border-line adenoma than in benign adenoma, indicating the "stromal activation" in malignancy; (4), the volumes of AgNORs were much increased in border-line adenoma in comparison with those in benign adenoma, and these showed further increases with the progression of malignancy to the invasive overt cancer. These findings indicate that border-line adenoma of human colon has already malignant character at the early progression stage, although no apparent epithelial atypia, or destructive invasion, is taking place.

Adenoma↗

PubMed1983

Early progressive kyphosis following compression fractures. Two case reports from a series of "stable" thoracolumbar compression fractures.

In an 18-year-old man and an 18-year-old woman, significant kyphotic deformity progressively developed in the course of the healing of apparently stable thoracic compression fractures. The common features were: (1) multiple adjacent fractures; (2) location in the thoracic spine; (3) associated thoracic injury; and (4) youth. The possibility of early progressive kyphotic deformity should be carefully monitored so that appropriate treatment can be instituted before the deformity becomes significant. Although both isolated fractures and multiple-level fractures of the thoracolumbar spine occur predominantly between T12 and L2, there is a secondary area that is predisposed to compression fractures in sequence from T5 to T7.

Accidents↗

PubMed1992

Florid progressive transformation of germinal centers. A syndrome affecting young men, without early progression to nodular lymphocyte predominance Hodgkin's disease.

Progressive transformation of germinal centers (PTGC) occurs focally in reactive lymph nodes, and has been reported with increased frequency in patients with nodular lymphocyte predominance Hodgkin's disease (NLPHD). It has been suggested that patients with lymph node biopsy samples showing PTGC are at increased risk for the development of NLPHD, and that Hodgkin's disease may evolve from PTGC. We report five young men (ages 14-24 years, mean 18) with prominent lymphadenopathy and florid PTGC, in whom careful examination and follow-up showed no progression to Hodgkin's disease. Three patients developed adenopathy that involved several node groups and two had localized adenopathy. Cervical (2), inguinal (2) and axillary (1) nodes ranging from 3 to 4 cm were excised. The number of progressively transformed germinal centers (PTGCs) ranged from 10 to 123 per specimen (mean 67); single sections contained nine to 29 PTGCs (mean 19). In three cases the nodal architecture was significantly distorted, suggestive of NLPHD, but Reed-Sternberg cells were absent. Follow-up is available for all patients (all untreated): three patients had persistent adenopathy 1 year 4 months to 10 years after diagnosis. Results of repeat biopsy in two patients (2 and 3 years after diagnosis) showed florid PTGC with no evidence of Hodgkin's disease. One of these patients had one subsequent biopsy 8 years after presentation; results showed only rare PTGCs. The fourth and fifth patients, who had presented with isolated adenopathy, were free of recurrent adenopathy at 2 and 5 years. These cases suggest a syndrome of lymphoid hyperplasia with florid PTGC in adolescent boys and young men. Although adenopathy can persist, there has been no progression to Hodgkin's disease. Recognition of this syndrome is important to avoid overdiagnosis of LPHD. Close follow-up of these patients will be necessary to evaluate the relationship of this disorder to NLPHD.

Adolescent↗

PubMed1994

Left ventricular function and coronary artery disease progression early after coronary bypass grafting.

To investigate the effects of coronary artery disease progression on left ventricular function in patients who suffer angina early after coronary artery bypass grafting, we studied the progression of coronary stenoses, the occurrence of graft occlusions, and measured left ventricular ejection fraction (regional and global) in 34 consecutive patients who underwent repeat angiography 25.2 +/- 3.5 (standard error of the mean) months postoperatively, from a total population of 550 patients who underwent bypass grafting. Resting left ventricular function and stenosis severity were assessed using a computerized, quantitative analysis system. Coronary stenosis progression was defined as an increase in the percentage of the stenotic occlusion by 30% or more, any increase in lesion severity that resulted in total coronary artery occlusion, or the occurrence of a new stenosis that occluded the artery by 50% or more. Group 1 comprised 21 patients with all grafts patent and group 2 comprised 13 patients with one or more grafts occluded (20 of 34 grafts). Coronary artery disease progressed in all patients in group 1, and this involved 22 of 54 (41%) grafted vessels and 3 of 15 (20%) nongrafted vessels (p < 0.05). Coronary artery disease progressed in 11 patients in group 2, involving 15 of 32 (47%) grafted vessels and 1 of 6 (17%) nongrafted vessels (p < 0.01). An increased collateral circulation was observed in both groups. The left ventricular ejection fraction remained unchanged in both groups (group 1, 0.60 +/- 0.03 versus 0.62 +/- 0.03; group 2, 0.62 +/- 0.05 versus 0.62 +/- 0.04 before and after bypass, respectively; p = not significant) and there was no difference between the groups.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

PubMed1985

Entamoeba histolytica: early progressive pathology in the cecum of the gerbil (Meriones unguiculatus).

The early formation of amebic cecal lesions in the gerbil (Meriones unguiculatus) was studied sequentially from 1 to 10 days post-inoculation. The earliest response of the cecal mucosa towards trophozoites of Entamoeba histolytica was local release of interglandular and goblet cell mucin. Trophozoites adhered to the surface epithelium or were trapped in the luminal mucus in the absence of epithelial cell damage. Focal mucosal erosion occurred later, and was preceded by crypt hyperplasia, edema and hemorrhaging of the lamina propria. The interglandular epithelial cells were cytolysed or were sloughed off into the lumen. Amebic mucosal erosion was accompanied by trophozoite entry in the crypts, but rarely into the lamina propria. The inflammatory cellular infiltrate was diffuse, and neutrophils were found in the lumen in contact with trophozoites. The findings of this study suggest that trophozoites of E. histolytica do not actively penetrate or invade the interglandular epithelium, but rather, that amebae are permitted to invade as a result of the destruction of the epithelium. Mucus depletion and the mucosal inflammation, probably in response to the adsorption of amebic toxins, preceded epithelial erosions.

Amebiasis↗

PubMed1992

Effects of dietary protein restriction and oil type on the early progression of murine polycystic kidney disease.

A paucity of research data exists on the potential for early dietary modification to directly retard cystic growth and proliferation in polycystic kidney disease (PKD). We have therefore examined the relative effects of dietary protein levels and oil type on the progression of disease in a murine model of PKD. In the first study, weanling DBA/2FG-pcy (pcy) mice were fed either a normal (NP), 25%, or low (LP), 6%, casein diet with 10% of either sunflower seed oil (SO) (containing n-6 fatty acids), or fish oil (FO) (containing n-3 fatty acids), in a 2 x 2 design. At the end of the dietary treatment, kidney weight relative to body weight was higher in mice on the NP diets. In addition, kidney phospholipid to kidney weight (mumol/g) was lower in pcy mice on NP diets, indicating that the increased kidney size was largely due to increased cyst development. Replacement of dietary SO with FO resulted in alterations in renal phospholipid fatty acid compositions: 18:2 n-6, 20:4 n-6, and 22:5 n-6 were lower, and 20:5 n-3, 22:5 n-3, and 22:6 n-3 were higher in FO-fed animals. No effect of dietary lipid type on disease progression was noted, however. In a second study, morphometric analysis revealed an 11% lower percentage cyst area and a 46% lower total cyst area (mm2) in kidney sections derived from mice on LP diets compared to NP diets. These results indicate that early dietary protein restriction in PKD prior to clinical manifestation of symptoms of the disease may have a significant impact on the pathogenesis of PKD.

Animals↗

PubMed1994

Early progression stage of malignancy as revealed by immunohistochemical demonstration of DNA instability; I, Human gastric adenomas.

The degree of DNA-instability as revealed by the immunohistochemical staining with monoclonal anti-single-stranded DNA antibody after acid hydrolysis (DNA-instability test) was used as the marker of malignancy. This was applied to human gastric regenerative epithelium in chronic peptic ulcer (5 cases), adenoma (35 cases), and well differentiated tubular adenocarcinoma (5 cases). Proliferative activity was evaluated by proliferating cell nuclear antigen (PCNA) immunohistochemistry, and the quantitative analyses of the mean number and mean area of silver-stained nucleolar organizer regions (AgNORs) per one nucleus were performed for all these cases. All cancers and adenomas were positively stained by the DNA-instability test diffusely, indicating the malignant character of the latter from the view point of DNA-instability, in contrast to the negative stainability of all regenerative epithelium. The percent number of PCNA-positive cells and mean number and mean area of AgNORs tended to be larger in adenoma and cancer than in regenerative epithelium, although the differences were not usually statistically significant. Supporting the malignant character of adenoma, single cell necroses and abnormal mitoses were almost always present in the lesion. In conclusion, all adenoma lesions were regarded as malignant in nature, namely, in-situ carcinoma, existing at an early stage of progression of malignancy.

3,3'-Diaminobenzidine↗

PubMed1991

Assessment of protein intake in early progressive renal disease.

Mean protein intake in a 4-day dietary survey of 34 patients with early renal disease was 1.2 +/- 0.27 g/kg ideal body weight daily. Nine subjects were taking over twice the recommended minimum intake. An increased protein intake was associated with a greater phosphate and sodium intake and an increased excretion of urea, phosphate, and sodium. Daily intake fluctuated markedly during the study (coefficient of variance 22.5 +/- 10.9%). Protein catabolic rate calculated from urea excretion correlated with protein intake, r = 0.60. Mean difference between the two estimates was 0.04 +/- 0.28 g/kg per day. It is proposed that if urea excretion exceeds 4.5 mmol/kg per day in a single collection (protein catabolic rate 0.98 g/kg per day), serial measurements should be made to determine protein intake more accurately and give dietary advice.

Adult↗

PubMedDate not supplied

The start of an autoimmune disease: idiotypic network during early progression of myasthenia gravis.

Anti-acetylcholine-receptor antibodies of IgG and IgM classes and antiidiotypic antibodies were determined in patients with myasthenia gravis at various times after the start of the disease. Patients with a disease duration of less than one year had a higher prevalence of antiidiotypic antibodies (31/32) than patients who had had the disease for more than 5 years (49/79), and the concentration of antiidiotypic antibodies was also higher in patients with early disease (p less than 0.005). The concentrations of antiidiotypic antibodies decreased during progression of the disease concomittant with an increase in IgG anti-receptor antibodies. A change from IgM to IgG anti-receptor antibody production was also found. In two patients, who developed myasthenia after bone-marrow grafting and who were followed before start of disease, antiidiotypic antibodies appeared before anti-receptor antibodies and before symptoms of myasthenia were present. The high prevalence and concentration of antiidiotypic antibodies in early disease indicate that development and expression of antiidiotypic antibodies are important in early myasthenia gravis.

Age Factors↗

PubMed1984

Small cell lung cancer 1973-1983: early progress and recent obstacles.

The recognition that the vast majority of patients with small cell lung cancer have distant metastatic disease at the time of diagnosis led to the use of systemic chemotherapy and consequent major improvements in survival in the early to mid-1970's. In the past five years, however, the pace of therapeutic advances has slowed. Recently evaluated treatment strategies, including more intensive induction chemotherapy, "late intensive" therapy of responding patients, alternation of chemotherapeutic regimens, integration of chest irradiation with drug therapy, large field irradiation, and reappraisal of the value of surgical resection, are discussed in this review. Advances in understanding of the cell biology of small cell lung cancer which may eventually lead to new forms of treatment are summarized.

Antineoplastic Combined Chemotherapy Protocols↗

PubMedDate not supplied

Early progressive supranuclear palsy: pathology and clinical presentation.

Progressive supranuclear palsy (PSP) is a syndrome of supranuclear ophthalmoplegic palsy, pseudobulbar palsy, rigidity of the limbs, nuchal dystonia, and dementia in which the gaze palsy is the hallmark of the disease. Most neurologists are reluctant to consider the diagnosis unless visual problems exist. Since the earliest complaints of PSP are said to be variable and subtle, accurate diagnosis is often delayed and initial pathologic changes of the disease not well studied. Two patients came to autopsy with dementia, gait disturbances, and/or dysarthria but no eye findings by history or physical exams. Symptoms had been attributed to metastatic cancers. At autopsy prominent globose neurofibrillary tangles with variable cell loss, microglial nodules, and neuronophagia were found in the locus ceruleus, third cranial nerve complex, nucleus supratrochlearis, nucleus centralis superior, and nucleus basalis of Meynert with mild pallor of the globus pallidus, mild cell loss in the dentate nucleus of the cerebellum, and sparing of the superior colliculus. The diagnosis of early PSP was made. These cases serve to 1) detail the more limited neuropathologic changes in early PSP, 2) reemphasize that the earliest clinical symptoms of PSP are not gaze palsies, and 3) remind clinicians to consider PSP in their differential diagnosis in patients with gait disturbances, dementia, and/or dysarthria, and 4) document PSP in association with carcinoma in two cases.

Aged↗

PubMed1981

Abnormalities of pulmonary vascular dynamics and inflammation in early progressive systemic sclerosis.

Abnormalities of pulmonary function were studied in 10 patients with progressive systemic sclerosis (PSS) and 3 control subjects. All underwent 81M krypton lung scanning and total body gallium scanning. Immune complexes were measured by Raji cell radioimmunoassay and polyethylene glycol (PEG) assay. Perfusion scans were abnormal in 7 of 9 patients, and 5 of 9 showed a decrease in pulmonary perfusion after cold challenge. Increased gallium uptake was noted in the lungs of 6 of 9 patients. Krypton scans were normal in the control group. Elevated immune complexes were noted in 8 of 10 patients by the Raji assay and in 5 of 10 with the PEG assay. Efforts to separate patients with PSS into subgroups may lead to a better understanding of and advances in therapy for PSS.

Adult↗