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Neurological syndromes in factitious disorder.

Factitious disorder is characterized by the intentional feigning of physical or psychological signs and symptoms. The best known type of factitious disorder, Munchausen syndrome, is marked by a chronic unremitting course with repeated hospitalizations. The purpose of this study was to assess the frequency, psychopathological phenomenology, and diagnostic classification according to DSM-III-R in patients with factitious disorder presenting as neurological syndromes. We prospectively included all patients who were hospitalized at our Department of Neurology, Freie Universität Berlin, during a 1-year period. Five of 1538 (.3%) patients were diagnosed as having factitious disorder with feigning of neurological syndromes. Four presented with the classic variant, Munchausen syndrome. All patients had similar, characteristic psychopathological features including self-discharge, aggressive behavior, pseudologia phantastica, and hospital wandering. In these cases the additional diagnosis of personality disorder was made according to DSM-III-R criteria. We concluded that factitious disorder presenting with neurological syndromes may be more prevalent than generally assumed. Our findings confirm the idea of frequent coincidence of factitious and personality disorders.

Adult↗

Benign epithelial odontogenic tumors.

Teeth are formed from a complex interaction of primitive ectoderm and ectomesenchymal tissues. Because humans develop 2 sets of teeth (deciduous and permanent), odontogenesis is a prolonged biologic process. Residues of odontogenic tissues are present in most humans- both during and after odontogenesis. These elements may be found in either bone or soft tissue of the jaws and may contribute to the formation of odontogenic tumors in these sites. Further, the mixture of epithelium and mesenchyme necessary for tooth formation allows for the development of tumors composed of either element or for mixed neoplasms. This article discusses 4 of the 5 benign odontogenic neoplasms that are of epithelial origin and offers an agreed on classification scheme, which includes important clinicopathological subtypes. Specifically discussed are ameloblastoma, calcifying epithelial odontogenic tumor (Pindborg tumor), adenomatoid odontogenic tumor, and squamous odontogenic tumor. A brief history of each tumor is given along with a discussion of demographic data, clinical findings, radiographic features, and gross features where useful. A thorough discussion is presented of diagnostic histopathology including histologic variants. Generally accepted modes of therapy and follow-up recommendations are discussed.

Ameloblastoma↗

[Is the new WHO's histological typing clinically useful for the diagnosis of follicular cancer of the thyroid?].

We tried to re-diagnose our 57 cases of thyroid follicular cancer between 1965 and 1988 in accordance with Histological Typing of Thyroid Tumours published by WHO in 1988. 1) The incidence of follicular cancer in differentiated cancers was decreased from 17.9% to 8.3% (26 cases). The number of widely invasive type and minimally invasive type was 13 cases equally. 2) Twenty four cases were diagnosed as papillary cancer. The reason was that 20 cases had a small focus of papillary structure, and other 4 cases had features of follicular variant type of papillary cancer. These 24 cases had good prognosis as our 262 cases of papillary cancer, in contrast to 26 cases of follicular cancer having worse prognosis. 3) The incidence of distant metastasis in follicular carcinoma was increased from 28.1% to 42.3%; 23.9% in minimally invasive type and 61.5% in widely invasive type, respectively. New WHO classification is acceptable according to this clinical study of our cases. We would have to treat more aggressively the patient diagnosed follicular cancer by WHO classification because of high incidence of distant metastasis.

Adenocarcinoma↗

Variant maple syrup urine disease in mother and daughter.

Intermittent MSUD in a mother and her daughter is reported. Fibroblast cultures were studied for branched-chain keto acid decarboxylase and results show that the mother has approximately 12% while the daughter has 5% of the normal enzyme activity. Other key members in the family were also studied for enzyme activity. It appears that the child has inherited an abnormal gene from her homozygous mother and another abnormal gene from her heterozygous father.A classification based on the degree of residual enzyme activity and protein tolerance places the mother in grade III and the daughter in grade II category. Classical MSUD, where the enzyme activity is less than 2% of normal, belongs to grade I.

Adult↗

[Variants in the growth of meningiomas of the base of the skull spreading into the orbit and paranasal sinuses].

Meningiomas of the skull base involving the orbit and paranasal sinuses were diagnosed in 254 patients. The patients were operated on in 1982-1991. All craniocephalic meningiomas involve the anterior skull base bones. The involvement presents either as destruction (nodular tumor) or as hyperostosis (infiltrative growth of the tumor). All patients were divided into 8 groups with different location of skull bones involvement. A classification of meningiomas of skull base disseminating into the orbit and paranasal sinuses is proposed, proceeding from the anatomical principle. This classification helps plan a surgical access, scope of intervention, probable size of skull defect and method of its closure, possible postoperative functional and cosmetic defects.

Adolescent↗

The classification of patients with chronic pain: age and sex differences.

OBJECTIVE: To further develop an empirically based classification system for chronic pain patients through the examination of age and sex differences, and incorporation of pain duration in the grouping algorithm. SUBJECTS: Three hundred seventy-four chronic pain patients (300 aged 13 to 59 years; 74 aged 60 to 89 years) assessed at an outpatient, multidisciplinary pain management centre. METHODS: Patients completed measures of demographic and descriptive information, pain intensity (box rating scale), perceived disability (modified Pain Disability Index) and affective distress (Symptom Checklist-90 Revised) before multidisciplinary treatment. Standardized scores from the assessment measures were entered into a series of hierarchical, multivariate cluster analyses to identify underlying patient subgroups. RESULTS: Age-based patient groupings from prior research were partially replicated. Significant differences in clinical presentations were observed across age and sex groups. Pain duration was found to make an important contribution to the patient groupings. 'Good control' (low pain, disability, distress) and variants of 'chronic pain syndrome' (elevated pain, disability, distress) groupings were identified across all analyses. Two variants of a 'stoic' profile were identified among older patients, with low levels of distress relative to pain and perceived disability. One of these profiles was associated with long pain duration and was found only among males. Several unique clinical profiles were identified for female patients. CONCLUSIONS: There are important age and sex differences in the clinical presentations of chronic pain patients. Some older patients present with unique clinical profiles that may reflect cohort differences, and/or physiological or psychological adjustment processes. There appears to be a greater number of distinct chronic pain presentations among females. Research on the classification of chronic pain patients within homogeneous diagnostic subgroups is needed.

Adolescent↗

The role of frontopolar cortex in subgoal processing during working memory.

Neuroimaging studies have implicated the anterior-most or frontopolar regions of prefrontal cortex (FP-PFC, e.g., Brodmann's Area 10) as playing a central role in higher cognitive functions such as planning, problem solving, reasoning, and episodic memory retrieval. The current functional magnetic resonance imaging (fMRI) study tested the hypothesis that FP-PFC subserves processes related to the monitoring and management of subgoals, while maintaining information in working memory (WM). Subjects were scanned while performing two variants of a simple delayed response WM task. In the control WM condition, subjects monitored for the presence of a specific concrete probe word (LIME) occurring following a specific abstract cue word (FATE). In the subgoal WM condition, subjects monitored for the presence of any concrete probe word immediately following any abstract cue word. Thus, the task required semantic classification of the probe word (the subgoal task), while the cue was simultaneously maintained in WM, so that both pieces of information could be integrated into a target determination. In a second control condition, subjects performed abstract/concrete semantic classification without WM demands. A region within right FP-PFC was identified which showed significant activation during the subgoal WM condition, but no activity in either of the two control conditions. However, this FP-PFC region was not modulated by direct manipulation of active maintenance demands. In contrast, left dorsolateral PFC was affected by active maintenance demands, but the effect did not interact with the presence of a subgoal task. Finally, left ventral PFC regions showed activation in response to semantic classification, but were not affected by WM demands. These results suggest a triple dissociation of function within PFC regions, and further indicate that FP-PFC is selectively engaged by the requirement to monitor and integrate subgoals during WM tasks.

Adolescent↗

A variant of Kaplan's accessory branch of the dorsal cutaneous branch of the ulnar nerve: a case report and review of the literature.

Kaplan's accessory branch is an aberrant branch of the dorsal cutaneous branch of the ulnar nerve that arises proximal to the styloid process of the ulna and courses ulnar to the pisiform. Variations of this anomaly have been described as having an end point of connection to the sensory branch of the ulnar nerve, to the motor branch of the ulnar nerve (rare), to the digital nerve at the level of the midhypothenar eminence, or to the proximal interphalangeal joint of the small finger or as running as an independent branch to the volar aspect of the small finger. We report a variant of Kaplan's accessory branch that coursed through the insertion of the flexor carpi ulnaris, a groove on the ulnar aspect of the pisiform, and connected to the ulnar nerve trunk proximal to its bifurcation into its motor and sensory branch. Based on the findings of the case presented and a review of the literature we offer a classification system for this anomaly.

Female↗

Genomic and molecular evolutionary analysis of a newly identified infectious agent (SEN virus) and its relationship to the TT virus family.

A new group of transmissible single-stranded (ss) DNA viruses (SENV) distantly related to the large TT virus (TTV) family was recently identified. Eight different SENV isolates have been found, some with an association with posttransfusion hepatitis. A phylogenetic analysis of near-complete open-reading frame 1, including conserved motifs and excluding recombinant regions, was performed. The analysis used TTV-like minivirus as an outgroup, to determine a root of the phylogenetic tree, and compared 8 SENV isolates, 6 prototype TTV isolates, and 7 TTV variants (including SANBAN, TUS01, PMV, and YONBAN). Four distinct clusters separated by a bootstrap value of 100% were observed. YONBAN isolates formed a distinct outer group, representing the earliest recognized phylogenetic divergence (group 1). Prototype TTV formed group 2, PMV formed group 3, and SENV, SANBAN, and TUS01 isolates formed group 4, the most recently evolved group. This taxonomic classification suggests that these circular ssDNA viruses probably evolved from a common ancestor virus.

Amino Acid Sequence↗

A method of constant stimuli for examining heartbeat detection: comparison with the Brener-Kluvitse and Whitehead methods.

A method of constant stimuli (MCS) for measuring heartbeat detection was examined by comparing performance on this method with the Brener and Kluvitse (BK) method and a variant of the Whitehead (WH') method. All methods required subjects to judge the simultaneity of heartbeat sensations and tones, and each yielded measures of judgment precision and of the temporal location of heartbeat sensations relative to the electrocardiogram R-wave. Both measures were found to be significantly correlated across tasks. A greater proportion of subjects met criteria for classification as heartbeat detectors on the MCS (54%) and BK (50%) tasks than on the WH' task (33%). In the MCS and BK tasks, subjects judged tones presented 100-300 ms after the R-wave to be most simultaneous with heartbeat sensations. Intratask correlations showed that only the MCS procedure yielded stable measurements of both judgment precision and temporal location. The MCS procedure possesses commendable psychometric properties and provides a simpler means than the BK procedure of examining heartbeat detection.

Adult↗

The value of morphological prognostic criteria in the assessment of renal cell carcinoma.

In this study, we examined some of the morphological values from the large number of prognostic parameters indicated in the literature by establishing survival statistics. Primary distant metastasis formation was confirmed to be a prognostically unfavorable factor. Regional metastasis formation takes an intermediate position between the primarily distantly metastasized and the primary non-metastasized renal cell carcinoma. In the primarily non-metastasized renal cell carcinoma, tumor invasion into the renal vein is associated with prognostic deterioration, obviously due to a linkage to other unfavorable tumor characteristics. The histological degree of malignancy as a single prognostic parameter seems to be of little informational value if no other tumor data are taken into consideration. The classification of renal tumors according to Robson proves to be of great clinical relevance, especially when it is combined with the histological degree of malignancy. The prognostic scheme by Hermanek 'combined staging and grading' must be recommended as the prognostic scheme of choice. Apart from special histological types of the renal cell carcinoma, such as the papillary or sarcomatous variant, and with reservation as to the tendency to unpredictable secondary metastasis formation that is peculiar to the renal cell carcinoma, a prediction of the prognosis according to the morphological criteria mentioned above is possible to a satisfactory degree within certain limits.

Adult↗

Isolation and characterization of factor IX Chapel Hill: comparison to normal human factor IX.

A classification scheme for patients with hemophilia B has been devised. Assessment of factor IX antigen and factor IX coagulant activity has confirmed the genetic heterogeneity of hemophilia B and has shown that some affected patients have normal amounts of factor IX-like protein. An abnormal factor IX, factor IX Chapel Hill, has been isolated from one of these variants and compared structurally and functionally to the purified normal human factor IX molecule. The major difference between factor IX Chapel Hill and normal human factor IX appears to be defective activation of the abnormal molecule by factor XIa and calcium.

Factor IX↗

[Clinical and pathological aspects of some odontogenic tumors and related lesions].

The jaw bones are the most common sites for epithelial cysts and tumors in the human skeleton due to their close relationship to the tooth and odontogenesis. Most of these cysts and tumors have in common an origin from the tissues of tooth formation and constitute a very diverse group of lesions that reflects the complex development of the dental structures. This group of odontogenic lesions occurs predominantly in the jaws, with a predilection for young people. Some aggressive lesions have attracted much attention as they often produce marked facial deformity and tend to recur after insufficient surgery. The most widely quoted classification of odontogenic tumors is that proposed in the WHO booklet published in 1992. There are, however, a number of controversial issues that need to be addressed with respect to clinicopathological subtypings, terminology, and diagnosis which all have direct bearings on therapeutic and/or prognostic implications. In the light of recent publication of the WHO new classification and a series of related studies undertaken by the author's group, this paper is to put together an overview on some of these important aspects related to unicystic ameloblastoma, clear cell odontogenic tumor, calcifying odontogenic cyst, odontogenic keratocyst, ameloblastic fibroma and its related lesions. Consequent upon a better understanding of the nature of these tumors and their variants, the various treatment protocols currently recommended by different surgeons will be better rationalized.

Ameloblastoma↗

Personality disorder diagnosis.

Every person has a characteristic manner of thinking, feeling, and relating to others. Some of these personality traits can be so dysfunctional as to warrant a diagnosis of personality disorder. The World Health Organization's International Classification of Diseases (ICD- 10) includes ten personality disorder diagnoses. Three issues of particular importance for the diagnosis of personality disorders are their differentiation from other mental disorders, from general personality functioning, and from each other. Each of these issues is discussed in turn, and it is suggested that personality disorders are more accurately and effectively diagnosed as maladaptive variants of common personality traits.

Journal Article↗

Endobronchial adenocarcinoma with endometrioid features and prominent neuroendocrine differentiation. A variant of fetal adenocarcinoma.

An endobronchial tumor, resected from a 77-year-old man, had an endometrioid histologic pattern consistent with fetal adenocarcinoma. A distinctive feature of the neoplasm was prominent neuroendocrine differentiation, including single, discrete neuroendocrine cells; aggregates of neuroendocrine cells resembling miniature carcinoid tumors; and a single focus of undifferentiated small cell carcinoma. Immunohistochemical staining of neuroendocrine cells revealed the presence of neuron-specific enolase, chromogranin, somatostatin, insulin, and serotonin. The heterogeneous cell populations caused problems in differential diagnosis and histologic classification. This case demonstrates that fetal adenocarcinoma may occur as a central endobronchial mass and express a variable degree of neuroendocrine differentiation.

Adenocarcinoma↗

[A contribution to the classification of seborrheic keratoses (author's transl)].

A clinico-statistical study is reported, basing on a material of 639 excised tumours which were defined histologically as seborrheic keratoses. All statements concerning clinical diagnoses and differential diagnoses, localization, development, age and sex of patients were transferred to punch cards. By means of a suitable EDA program, basic counts and a multidimensional analysis of conditioned frequencies [14] were performed. The results indicate, that the different clinical forms of seborrheic warts, often discriminated in the literature, are variants of the same entity.

Adolescent↗

French American British (FAB) morphological classification of childhood lymphoblastic leukaemia and its clinical importance.

As part of the Medical Research Council Leukaemia Trial UKALL VIII, 738 unselected children with acute lymphoblastic leukaemia (ALL) had the morphology of their marrow blast cells reviewed by a panel of three haematologists. Ninety four (13%) showed appearances classifiable as type L2 by the French American and British (FAB) cooperative group's criteria, five (0.7%) were typed L3, and the remaining 639 (86%) as L1. Disregarding the patients classified as L3, those with the L2 variant showed an inferior disease free survival to that of the remainder (p less than 0.01), and more of them failed to remit after receiving "standard" remission induction treatment (p less than 0.01). They included an excess of older children (p less than 0.01) with less profound marrow failure at diagnosis, and fewer of them expressed the common ALL antigen (p = 0.05). There was no association between L2 morphology and the diagnostic white cell count, sex, or the presence of a mediastinal mass. These findings confirm earlier reports that FAB L2 ALL is associated with a poor prognosis and that it occurs more commonly in older children. The high remission failure rate is a recent observation and indicates that alternative early treatment may be appropriate for such patients.

Actuarial Analysis↗

Mutator transposon in maize and MULEs in the plant genome.

Mutator (Mu) is by far the most mutagenic plant transposon. The high frequency of transposition and the tendency to insert into low copy sequences for such transposon have made it the primary means by which genes are mutagenized in maize (Zea mays L.). Mus like elements (MULEs) are widespread among angiosperms and multiple-diverged functional variants can be present in a single genome. MULEs often capture genetic sequences. These Pack-MuLEs can mobilize thousands of gene fragments, which may have had a significant impact on host genome evolution. There is also evidence that MULEs can move between reproductively isolated species. Here we present an overview of the discovery, features and utility of Mu transposon. Classification of Mu elements and future directions of related research are also discussed. Understanding Mu will help us elucidate the dynamic genome.

DNA Transposable Elements↗