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At least 847 records · Page 47Linked to original sources
PubMed2002

Hemicrania continua: comparison between two different classification systems.

Hemicrania continua is an uncommon primary headache disorder. This study of nine patients compares two different classification systems, proposed by Pareja et al. and Goadsby and Lipton. Although it seems logical to position the nosologic status of hemicrania continua under group 3 of the International Headache Society Classification, as proposed by Pareja et al., the Goadsby and Lipton classification is more clinically useful and should be included in future International Headache Society reclassification.

Adult↗
PubMed1995

Modified neonatal growth assessment score: a multivariate approach to the detection of intrauterine growth retardation in the neonate.

The objective of this investigation was the development of a modified Neonatal Growth Assessment Score (mNGAS) for use in the evaluation of neonatal growth status. The growth of 74 fetuses at risk for intrauterine growth retardation (IUGR) was followed longitudinally, beginning in the first or early second trimester. Rossavik growth models derived from data obtained in the second trimester were used to predict the weight (WT), crown-heel length (CHL) and head (HC), abdominal (AC) and thigh circumferences (ThC) at birth, which were then actually measured within 24 h after delivery. These measurements were compared to age-specific size curves and used to calculate sets of five growth potential index (GPRIi) values, which in turn were used to calculate five-variable Neonatal Growth Assessment Scores (NGAS5). Neonates were initially classified as normal or IUGR on the basis of NGAS5, GPRIi, and anatomic measurements. A final classification based on principal component analysis and linear discriminant analysis was carried out. The score obtained using the first principal component function was defined to be mNGAS51. The effectiveness of mNGASij values, determined from 1-4 GPRIi values, in separating normal and IUGR neonates was also evaluated. Neonates initially considered to be normal had very few abnormal GPRI values or anatomic measurements, whereas the frequency of these abnormalities in IUGR neonates was significantly increased. However, no single anatomic variable was 100% normal in the normal neonates and 100% abnormal in IUGR neonates. Only 40% of IUGR neonates were small for gestational age. Classification of these neonates using principal component analysis and linear discriminant analysis was essentially the same (98.6%) as that made initially after reclassification of two IUGR neonates as normal. The characteristics of the initial and final normal and IUGR groups were very similar and the mNGAS51 was strongly correlated with the NGAS5 in the IUGR group. The effectiveness of mNGASij in separating normal and IUGR neonates increased with the number of GPRIi values included and the types used. GPRIThC and GPRIWT were the most important, followed by GPRIAC. GPRICHL and GPRIHC were much less important and in some cases detrimental. These findings support the concepts of a decrease in soft tissue mass as the initial step in the development of IUGR and the protection of head growth (brain-sparing). The characteristics of mNGAS51, particularly its comprehensiveness, its independence of differences in growth potential, its weighting of GPRIi values according to their importance in the detection of IUGR and its ability to detect different manifestations of IUGR in different individuals, indicate that this should be a most effective parameter for separating normal and IUGR neonates.

Embryonic and Fetal Development↗
PubMed2002

Influence of cytogenetic abnormalities on outcome after allogeneic bone marrow transplantation for acute myeloid leukemia in first complete remission.

Cytogenetic abnormalities detected at diagnosis are recognized as important in predicting response to chemotherapy in acute myeloid leukemia (AML). However, there is controversy concerning the prognostic significance of karyotype for outcome after allogeneic bone marrow transplantation (allo-BMT) performed in first complete remission (CR1). This single-institution report describes allo-BMT for AML in CR1 and the effect of diagnostic cytogenetic findings on the results of that treatment. Between August 1981 and December 1999, 93 patients underwent related donor (n = 82) or unrelated donor (n = 11) BMT. Conditioning and GVHD prophylaxis were achieved predominantly with busulfan and cyclophosphamide and with cyclosporine and methotrexate, respectively. Seventy-nine (85%) of 93 patients had successful marrow karyotyping at diagnosis, and the patients were categorized into 3 prognostic groups based on the British Medical Research Council AML 10 trial classification: 15 patients(19%) were classified as having favorable risk [inv(16), t(8;2 1), t(15;17)]; 55 (70%) as having intermediate risk [no abnormality, +8, +21, +22, del(7q), del(9q), 11q23 rearrangement, and other numerical or structural abnormalities]; and 9 (11%) as having adverse risk [-5, del(5q), -7, 3q rearrangements, > or = 5 abnormalities, t(6;9), t(9;22)]. The median follow-up was 93 months (range, 16-241 months). The overall survival (OS) rate, event-free survival (EFS) rate, relapse rate, and treatment-related mortality (TRM) were not statistically different between the groups. The 5-year actuarial EFS rates for favorable, intermediate, and adverse risk groups were 58% (95% confidence interval [CI], 29%-79%), 58% (95% CI, 43%-70%), and 67% (95% CI 28%-88%), respectively. Reclassification of patients into cytogenetic prognostic subgroups according to Southwest Oncology Group criteria did not change these results. In univariate analysis, the only variable found to have a prognostic influence on OS (P = .04) and TRM (P = .03) was the type of donor (unrelated donor was linked to a worse prognosis), which was confirmed in multivariate analysis. Our study suggests that presentation karyotype has less prognostic significance for outcome following allo-BMT than for outcome following conventional chemotherapy. In particular, AML patients with poor prognostic cytogenetic changes in CR1 who are unlikely to be cured with chemotherapy alone may benefit from allo-BMT.

Acute Disease↗
PubMed2003

The genetics of inflammatory bowel disease.

The inflammatory bowel diseases (IBD) comprise complex genetic disorders, with multiple contributing genes. Linkage studies have implicated several genomic regions as likely containing IBD susceptibility genes, with some observed uniquely in Crohn's disease (CD) or ulcerative colitis (UC), and others common to both disorders. The best replicated linkage region, IBD1, on chromosome 16q contains the CD susceptibility gene, NOD2/CARD15. NOD2/CARD15 is expressed in peripheral blood monocytes and is structurally related to the plant R proteins, which mediate host resistance to microbial pathogens. Three major coding region polymorphisms within NOD2/CARD15 have been highly associated with CD among patients of European descent. Having one copy of the risk alleles confers a 2-4-fold risk for developing CD, whereas double-dose carriage increases the risk 20-40-fold. All 3 major CD variants exhibit a deficit in NF-kappaB activation in response to bacterial components. Carriage of NOD2/CARD15 risk alleles is associated with ileal location, earlier disease onset, and stricturing phenotype. Other IBD genomic regions include IBD2 on chromosome 12q (observed more in UC), and IBD3, containing the major histocompatibility complex region. A short genomic region has been associated with CD on chromosome 5q, but the precise contributing gene is as yet unidentified. The characterization of additional IBD susceptibility genes could potentially lead to the identification of novel therapeutic agents for IBD, make possible a molecular reclassification of disease, and increase understanding of the contribution of environmental factors (notably, tobacco and the intestinal microbial milieu) to intestinal inflammation.

Animals↗
PubMed2007

The impact of reclassifying moderate CKD as a coronary heart disease risk equivalent on the number of US adults recommended lipid-lowering treatment.

BACKGROUND: The Third National Cholesterol Education Program Adult Treatment Panel (ATP-III) guidelines recommend consideration of lipid-lowering therapy at lower low-density lipoprotein cholesterol levels (>or=100 mg/dL [>or=2.59 mmol/L]) for adults with coronary heart disease risk equivalents. Chronic kidney disease is associated with increased coronary heart disease risk but is not included as a risk equivalent in these guidelines. METHODS: The impact of including moderate chronic kidney disease (estimated glomerular filtration rate, 30 to 59 mL/min/1.73 m(2) [0.50 to 0.98 mL/s]) as a coronary heart disease risk equivalent on the percentage and number of US adults with chronic kidney disease recommended lipid-lowering therapy was estimated by using data from the Third National Health and Nutrition Examination Survey. RESULTS: Of adults with moderate chronic kidney disease, 53.0% had a history of coronary heart disease or a risk equivalent, 24.7% reported a history of myocardial infarction or stroke, 17.7% had diabetes, 9.6% had angina, and 26.9% had a 10-year coronary heart disease risk greater than 20%. Using current ATP-III guidelines, lipid-lowering therapy is recommended for 61.4% of adults with moderate chronic kidney disease. If moderate chronic kidney disease was reclassified as a coronary heart disease risk equivalent, this percentage would increase to 87.7%, representing an increase in number of adults with moderate chronic kidney disease recommended lipid-lowering treatment from 4.5 to 6.5 million adults. CONCLUSION: This analysis shows that a majority of adults with moderate chronic kidney disease have coronary heart disease or risk equivalents. Nonetheless, a substantially greater proportion of US adults with moderate chronic kidney disease would be recommended lipid-lowering therapy through its reclassification as a coronary heart disease risk equivalent.

Aged↗
PubMed2002

Southeast Asian patients with chronic hepatitis C: the impact of novel genotypes and race on treatment outcome.

Hepatitis C virus (HCV) genotype and other host and viral factors influence treatment outcome in chronic HCV infection. We evaluated the effect of race and genotype on interferon and ribavirin treatment outcome in 70 Southeast Asian (SEA) and 50 white patients. Genotype was based on the 5' untranslated region (5'UTR) with a commonly used line probe assay (INNO-LiPA HCV II) that may mistype genotype 7, 8, or 9 as 1b. HCV core region sequencing resulted in reclassification of 8 genotype 1 and 25 genotype 1b SEA subjects as genotype 7, 8, or 9. Twenty-six SEA genotype 7, 8, and 9 (79%) and 10 SEA true genotype 1b (59%) patients achieved a sustained virologic response (SVR) compared with 15 (34%) white genotype 1b patients. Logistic regression analysis showed that SEA patients with genotype 7, 8, or 9 were more likely to achieve a SVR than white genotype 1b patients (OR 16.56; 95%CI 4.16, 65.91) as were SEA true genotype 1b patients compared with white genotype 1b patients (OR 4.63; 95%CI 1.19, 18.04). In conclusion, a proportion of SEA patients classified by INNO-LiPA as genotype 1b were in reality genotype 7, 8, or 9. In comparison with white genotype 1b patients, both SEA genotype 1b and SEA genotype 7, 8, and 9 patients showed a significantly greater SVR. HCV core sequencing was necessary to determine genotype accurately in persons potentially exposed to HCV genotypes 7, 8, or 9. This study also supports the concept that race and ethnicity are important determinants of treatment outcome in HCV infected patients.

Adult↗
PubMed2002

Protein surplus myopathies and other rare congenital myopathies.

The protein surplus myopathies have emerged as a newly recognized subgroup of morphologically defined myopathies within the spectrum of congenital myopathies because of the accumulation of protein aggregates, some of them mutant proteins. Currently, nosologic, including molecular criteria include desmin-related myopathies, actinopathies, and hereditary inclusion body myopathies, whereas hyaline body myopathy is still a putative form of protein surplus myopathy because of lack of any molecular data. The congenital myopathies (CM), foremost including nemaline and myotubular myopathies, have given evidence that, despite their epidemiologic rarity, the molecular age has dawned in CM and has even revealed surprising new nosologic features requiring reassessment and reclassification of certain CM. It is to be expected that a recently updated ENMC Consortium on "Protein surplus and other congenital myopathies" may procure important new information.

Actin Cytoskeleton↗
PubMed2000

Maternal serum alpha-fetoprotein and altitude.

Maternal serum alpha-fetoprotein (MS-alphaFP) testing is widely used to screen for fetal defects. MS-alphaFP concentrations are affected by a number of variables such as gestational age, maternal weight, number of fetuses, race, and insulin-dependent diabetes. Undefined geographic factors may also influence MS-alphaFP. We have examined the effect of altitude in a sample of 1063 MS-alphaFP results selected to span a range of altitudes. The study sample was subjected to linear regression with and without a term for altitude, and multiple-of-the-median (MoM) values were calculated before and after adjusting for altitude. The median MS-alphaFP was found to decrease an average of 1 ng/mL for every 1100 ft increase in altitude, a change approximately equivalent to that seen with an increase in maternal weight of 6 lb. Adjusting for altitude resulted in the reclassification of 36 of 1063 patient results (3.4%), although the clinical utility of this adjustment remains unexamined.

Altitude↗
PubMed1979

Development of a classification rule for four clinical therapeutic psychotropic drug classes with EEG power-spectrum variables of human volunteers.

An objective rule for the classification of psychotropic substances has been developed. Classification is based on data from five basic studies simultaneously designed and performed and involving 75 healthy volunteers who ingested 20 different psychotropic drugs and 5 placebos in single oral dosages. Each volunteer took one psychostimulant, one antidepressant, one neuroleptic, one minor tranquilizer and one placebo in a double-blind Latin square cross-over design. The variables were 6 frequency bands, based on power spectrum estimates and determined by factor analysis, plus total power in the 1.5-30.0 Hz range. An objective classification rule was established by multi-group (5 groups) linear discriminant analysis. Reclassification of the substances by the new rule yielded correct results for 17 out of 20 psychotropic drugs and 4 out of 5 placebos. Of placebos from various studies not used for the establishment of the classification rule, 7/9 were classified correctly. The validity of the rule for other classes of substances will have to be verified in independent studies.

Adult↗
PubMed2002

[Personality and personality disorders I. Universality and sensitivity of dimensional personality models as diagnostic systems for personality disorders].

OBJECTIVE: A dimensional diagnostic system for personality disorders (PD) postulates continuous transitions from normal to disordered personalities (continuity hypothesis) and universal validity of basic personality dimensions (universal hypothesis). In the present study three dimensional personality models that claim to provide a systematic representation of the overall domain of personality disorders were compared: the Big-Five model proposed by Costa and McCrae, the psychobiological model proposed by Cloninger and colleagues, and the "Dimensional Assessment of Personal Pathology (DAPP)" model proposed by Livesley and colleagues. METHOD: The "Six Factor Test" (SFT) measuring the Big-Five factors of personality, the "Temperament and Character Inventory (TCI)" measuring 4 temperament and 3 character dimensions, and the DAPP measuring 18 basic traits and 4 second ordered factors were administered to general population subjects (n = 156), and a clinical sample (n = 220) including a subsample of 69 patients with at least one diagnosis of DSM-IV PD. Group comparisons, regression analyses, and facet theoretical analyses were conducted. RESULTS: The nonmetric similarity analyses of the three personality models show a nearly identical radex-representation of the second ordered factors in the non-clinical and clinical sample reflecting an universal validity of 4 basic personality dimensions and confirming the universal hypothesis. In comparison with the BIG-Five concept and the psychobiological model the DAPP model seems to be more sensitive to differentiate PD patients from controls with a reclassification rate of 94.5 %. CONCLUSIONS: The Big-Five model, the DAPP and the TCI represent a substantially similar domain despite their different conceptualization. However, the DAPP was more sensitive to differences between PD patients and controls, offered a more comprehensive account of PD, and could differentiate the two groups more effectively.

Character↗
PubMed1995

[Histopathologic correlates of false-positive cytologic findings in the uterine cervix].

Between 1984 and 1992, we reinvestigated 170 cases of cervical smears obtained in conization or hysterectomy which had produced false positive cytologic findings. We looked for morphological changes that could explain the cytologic findings. Such changes were found in half of the cases. These cases included 54 (63.6%) specimens with marked cellular polymorphism as a result of inflammation, tissue regeneration, or atrophy. In 51.9% of the cases showed histologic signs of possible HPV infection. The initial findings required reclassification in 12.9% of the cases, and in fifteen cases (17.6%) the biopsy matched the cytologic findings, without being validated by the final findings. We observed histologic phenomena corresponding to the cytologic findings in 30.8% of the cases in group IIID (n = 26) using Papanicolaou's classification system. In groups IV a (n = 72), IV b (n = 44), and V (n = 28), we found a histologic explanation in 54.2%, 47.7%, and 60.7% of the cases, respectively. In most cases we observed polymorphic cellular changes primarily associated with HPV.

Biopsy↗
PubMed1997

[Retinitis pigmentosa--clinical, genetic and pathophysiologic aspects].

Retinitis pigmentosa defines a genetically heterogenous group of disorders characterized by degenerations of photoreceptors and pigment epithelium. This article reviews our current knowledge of the genetical, clinical and pathophysiological aspects of this disease complex. Therapeutic concepts under current investigation are discussed as well. In recent years tremendous new insights have been made using molecular techniques for the investigation of retinal dystrophies. Ophthalmoscopically very similar patterns of photoreceptor dystrophies have been related to different gene mutations. In contrast, mutations in a single gene may cause different clinical patterns of photoreceptor dystrophies. Therefore, these recent results suggest that a reclassification of retinal dystrophies on the basis of their genetic origin may be favourable. In the future molecular genetics and the recent developments may play an increasing role for clinical classification and evaluation of photoreceptor dystrophies. The continued clinical and experimental research on hereditary disorders may help to elucidate further the wide disease spectrum and thereby developing new classifications and efficient therapeutic concepts.

Animals↗
PubMed1989

[Microinvasive stage Ia cancer of the uterine cervix--results of a multicenter clinic based analysis].

A retrospective multicentre study to investigate diagnosis, treatment and end results of treatment of cervical cancer stage Ia, was carried out in 6 departments of gynaecological oncology. After reclassification by a reference pathologist, among the 936 cervical cancer cases primarily diagnosed and treated as stage Ia between 1970 and 1980, only 530 (56.6%) met the criteria of microinvasive cancer stage Ia. Misclassifications concerned all participating centres with statistically significant differences amongst them. Overdiagnosis (reference diagnosis only CIN I-III, 42.5%) was more frequent than underdiagnosis (reference diagnosis stage Ib--0.9%). In comparison to 1970-74, in the period 1975-80 a significant increase of cases detected asymptomatically (86.5%) was observed. The percentage of cases primarily diagnosed by cone biopsy, also increased significantly and amounted to 71.2%. Patients with cervical cancer stage Ia were most frequently treated by surgery alone (93.2%). Radiotherapy alone did not play any important role (5.7%). There were only a few cases treated by combined surgery and radiotherapy (5.7%) with a decreasing trend over time. Women under the age of 45 years were significantly more frequently treated by the conservative method (cone biopsy, simple hysterectomy) than older ones, without any significant relation between depth of invasion and radicality of treatment. A total of 19 (14 local, 5 lymph node) recurrences were diagnosed between 9 and 110 months after primary treatment. Local recurrences could be observed more frequently after limited than extended treatment. There was no significant relation between depth of invasion and frequency of recurrences, but the latter were significantly increased in cases with histologically proven invasion of blood or lymph vessels.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1984

[Ossifying fibroma of the bone].

In the spectrum of the fibroosseous bone tumours the ossifying fibroma of the bone (OF) is situated as a not yet clearly defined entity with many names. The present paper deals with the question wether a term innovation brings elucidation to clinical management. Therefore 811 previously published and 16 additional cases have been analysed with respect to their clinical and radiological behavior. In addition a simplificated histological reclassification was performed on 284 well documented literature cases. It was found that there are no great differences between these tumours according to their localisation, their clinical behavior, and their prognosis. All tumours of this group are suitable to different categories of histological maturation of the same tumour best called "ossifying fibroma".

Adolescent↗
PubMed2026

A Quantitative Lung Mucin Score to Identify Chronic Bronchitis.

BACKGROUND: We previously demonstrated that sputum total mucin concentration is an objective marker for chronic bronchitis (CB). This current study introduces a novel Mucin Quantitative Score (MUCQ) that combines total mucin concentration and mucin composition to improve the assessment of risk, onset of clinically diagnosed disease, and progression of muco-obstructive lung diseases. METHODS: Patients from the SPIROMICS (SubPopulations and InteRmediate Outcome Measures in COPD Study) cohort were classified as having CB, or not, based on clinical questionnaires. Using the measured total mucin, MUC5AC, and MUC5B concentrations in sputum samples, we calculated MUCQ as [Total mucin]×([MUC5AC]÷[MUC5B])÷100 μg/ml, which is a unitless, weighted concentration score. Our primary outcome was the net reclassification of patients with a diagnosis of CB, or not, based on total mucin concentrations in their sputum compared with using the MUCQ score. Participants were first classified as CB- positive or -negative using a total mucin concentration threshold of 2306 μg/ml, then reclassified using the MUCQ threshold of 4.30. Associated z statistics and a P value for the primary outcome are reported. RESULTS: Among 164 patients in the SPIROMICS cohort with clinically defined CB, using the MUCQ score up-classified 18 patients who were currently smoking to a diagnosis of CB and down-classified 5 patients who were currently smoking and 3 control participants who had never smoked, compared with the classification of CB was based on total mucin concentrations alone (P=0.001). In addition, MUCQ correlated with other clinical and pathological indices of chronic airway disease and airway obstruction. CONCLUSIONS: The MUCQ metric was superior in distinguishing patients with CB compared to a total mucin concentration. Trials are needed to ascertain the prospective use of MUCQ metrics in research and clinical settings for assessment, management, and tracking therapeutic responses in CB and potentially other muco-obstructive conditions. (Funded by the National Institutes of Health and others.).

Humans↗
PubMed1978

Porphyria cutanea tarda in three generations of a single family.

We examined conjugal and blood relatives of one kindred for evidence of porphyria cutanea tarda. The disease was identified in eight family members in three generations. Four were classified as having overt porphyria cutanea tarda because of four criteria: photo-enhanced dermatosis; excessive urinary excretion of uroporphyrins; characteristic thin-layer chromatographic pattern of urinary porphyrins; and decreased activity of erythrocyte uroporphyrinogen decarboxylase. Two patients with excessive excretion of uroporphyrins or characteristic chromatograms, or both, and decreased uroporphyrinogen decarboxylase activity were classified as having subclinical porphyria cutanea tarda, and two with decreased uroporphyrinogen decarboxylase activity only were classified as having latent porphyria cutanea tarda. This study provides further evidence that prophyria cutanea tarda can be a familial disease inherited in an autosomal dominant fashion. We propose a reclassification of porphria cutanea tarda as an overt, subclinical or latent disorder.

Adult↗
PubMed1985

The autopsy as a measure of accuracy of the death certificate.

To determine the extent of agreement on underlying cause of death between death certificates and autopsy reports, we analyzed 272 randomly selected autopsy reports and corresponding death certificates from among all such data on autopsies performed in Connecticut in 1980. In 29 per cent of the deaths, a major disagreement on the underlying cause of death led to reclassification of the death in a different International Classification of Diseases major disease category. In an additional 26 per cent, the death certificate and autopsy report agreed on the major disease category but attributed the death to a different specific disease. Deaths due to neoplasms were most accurately diagnosed, with a sensitivity of 87 per cent and a positive predictive value of 85 per cent. Deaths resulting from diseases of the respiratory or digestive system were associated with the highest rates of disagreement. Diseases most commonly overdiagnosed were circulatory disorders, ill-defined conditions, and respiratory diseases. Diseases most commonly underdiagnosed as the cause of death on the death certificate were specific traumatic conditions and gastrointestinal disorders. The autopsy remains an important method for ensuring the quality of mortality statistics.

Autopsy↗
PubMed2000

Clinical information determines the impact of transesophageal echocardiography on the diagnosis of infective endocarditis by the duke criteria.

BACKGROUND: Although transesophageal echocardiography (TEE) is more sensitive than transthoracic echocardiography (TTE) in detecting echocardiographic evidence of infective endocarditis (IE), the impact of TEE on the clinical diagnosis of IE has not been clearly delineated. METHODS AND RESULTS: We studied 112 patients with 114 suspected episodes of IE over a 6-year period who underwent both TTE and TEE during their diagnostic evaluation. Using the results of these studies along with clinical and microbiologic data, we attempted to determine the incremental value of TEE to the Duke Endocarditis Diagnostic Criteria. Patients were initially classified into a diagnostic category of the Duke criteria with TTE data, and then the diagnostic classification was reconsidered with TEE data. A diagnostic category reassignment occurred in 25 of 114 episodes of IE evaluated when TEE results were incorporated into the evaluation with the Duke criteria (22 patients were reclassified from possible IE to definite IE whereas 3 patients were reclassified from rejected to possible IE). Diagnostic reclassification occurred in 9 (11%) of the 80 episodes of suspected IE with native cardiac valves and 13 (34%) of 34 episodes with prosthetic cardiac valves. Most patients reclassified from possible IE to definite IE with TEE data (19 of 22) had an intermediate clinical likelihood of IE, whereas 92% of patients had negative TTE results. Pathologic examination of valvular tissue in 22 of the 114 episodes of suspected IE revealed that the positive predictive value of the Duke criteria with TEE data for diagnosis of IE was 85% in patients with native valves and 89% in patients with prosthetic valves. CONCLUSIONS: When clinical evidence of IE is present, TEE improves the sensitivity of the Duke criteria to diagnose definite IE. TEE data appears to be especially useful for the diagnostic evaluation of patients with suspected IE who have prosthetic valves.

Diagnosis, Differential↗