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Root length, crown height, and root morphology in Turner syndrome.

Root length, crown height, and root morphology were studied on intraoral and panoramic radiographs in 33 Turner syndrome patients aged 7.0-16.7 years, subdivided on the basis of karyotype. Thirty-three normal girls aged 10.2-16.4 years served as controls. In the 45X patients and, with the exception of a few teeth, also in the isochromosome and mosaic karyotypes, root length and crown height of incisors, canines, and premolars were significantly reduced. Some teeth showed altered crown-root proportions. Maxillary first premolars showed a significantly increased number of two-rooted and three-rooted variants. Mandibular premolars and molars had a complex root morphology, and a classification system was established including four premolar and six molar root types. Premolars had a significantly increased number of root components. Some of the variants, such as a molar-like second premolar, are apparently specific for these patients. On several first molars a radix entomolaris was identified. Two separate mesial and one or two separate distal roots were also frequently seen. Our investigation demonstrates that X-chromosome deficiency influences root formation.

Adolescent↗

[Consensus 'diagnosis of short stature in children.' National Organization for Quality Assurance in Hospitals].

Each year about 2500 children in the Netherlands are referred to a specialist because of short stature. Aim of the consensus meeting: 'Diagnosis of short stature in childhood' by general practitioners, pediatricians and pediatric endocrinologists was to promote early diagnosis and treatment of aberrant growth but also to prevent unnecessary referral and interventions. For optimal treatment of growth disorders early diagnosis is of the utmost importance. Short stature is best detected by the use of standard deviation scores (SDS) instead of percentile lines. As cut-off point for referral SDS < -1.3 (< P10) was chosen in order to identify risk groups that need further evaluation. Based on international consensus a diagnostic classification of short stature is possible, distinguishing normal variants and (or) idiopathic short stature from primary and secondary growth disorders. Using target length, the growth curve in the SDS diagram, the actual age and puberty signs, the general practitioner can use a flow chart to determine whether there is a growth problem and whether this needs treatment in primary or secondary health care. Measuring skeletal age has no additional value. Attention should also be given to psychosocial problems as the cause or the effect of short stature.

Adolescent↗

[Comparative assessment of cognitive styles forming personal behavior in health and borderline personality disorder].

The author suggests a cognitive-analytical concept of personality focusing on cognitively mediated processes of cyclic interaction of different levels of consciousness and subconsciousness represented in the form of emotional-behavioral potential and functional-dynamic system. A cognitive type (cognitype)--a type of personality-centered cognitive operations that determines the pattern of behavioral cycle and distinctive features of social adaptation--is singled out as an integrative basic individual and psychological component of personality. Presented is classification of personality cognitypes, their adaptive and maladaptive variants, correlations between cognitypes and anomalies of personality. The principles of psychotherapy of maladaptive behavior, borderline and addictive disorders (positive reintegration of personality) are substantiated.

Adaptation, Psychological↗

Major retroperitoneal venous anomalies: surgical considerations.

Nineteen major anomalies of the vena cava or its branches were encountered in patients requiring abdominal vascular surgery and related procedures at Barnes Hospital during the past 5 years. The classification of these anomalies, their embryologic development, associated variants, and surgical considerations are discussed. Careful review of preoperative computed tomographic scans and familiarity with these anatomic variants may allow the surgeon effectively to avoid potentially disastrous intraoperative consequences.

Humans↗

[Nephroblastomas (Wilms' tumors) and special variations of nephroblastomas].

The results of the National Wilms' Tumor Study (NWTS) enabled the subdivision of nephroblastomas into subtypes with "favorable and unfavorable histology". Nephroblastomas with "unfavorable histology" could be discriminated by identifying those tumors not responding to therapeutic regimes proven successful for most cases with "favorable histology". A major disadvantage of the NWTS classification has been the exclusion of cytodifferentiated nephroblastoma variants, which, in contrast to typical nephroblastomas, can be cured by complete nephrectomy with wide excision of perinephric soft tissue. In the current study all types of nephroblastoma and nephroblastoma variants were included to encompass the whole morphological spectrum which these tumors may assume. This unselected material is necessary to define the relation between morphology and prognosis and to compare the treatment results of various clinical trials. Three hundred and four cases of nephroblastoma and related neoplasms on file at the Pediatric Tumor Registry, Kiel, were investigated by conventional light microscopy, electron microscopy, immunohistochemistry and DNA-flow cytometry. Of the "typical" nephroblastomas 50% occurred in the left kidney, 45% in the right kidney, and 5% were bilateral. Five cases were located in extrarenal sites. There were 121 males and 114 females. The peak incidence was noted in the third year of life. Of 135 patients 111 are alive and well, nine are living with disease, and 10 patients have died of disease. The blastemal predominant and stromal predominant types in our study were more frequent than in the NWTS. By contrast, the mixed and epithelial predominant types were more frequent in the NWTS. Patients with nephroblastomas of mixed or blastemal predominant type were older than those with epithelial predominant or stromal predominant type. Electron microscopy showed that nephroblastoma is derived from metanephric blastema. Blastemal cells are capable of differentiating into tubular epithelial cells and stromal cells. Undifferentiated blastemal cells contain exclusively vimentin intermediate filaments, better differentiated blastemal cells vimentin and cytokeratin, and stromal cells exclusively vimentin. Preoperative radio- and/or chemotherapy led to a marked reduction of undifferentiated blastema and poorly differentiated tubules, whereas better differentiated tubules, striated muscle, hyaline cartilage, cells with anaplastic and sarcomatous elements were not affected. Thus, identification of highly malignant nephroblastomas with anaplasia and sarcomatous renal tumors was even possible after preoperative treatment. Congenital mesoblastic nephroma (CMN; n = 17) is a low-grade malignant, cytodifferentiated nephroblastoma which very rarely occurs beyond the fourth month of life and has an excellent prognosis, provided it has been completely resected.(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent↗

Prognostic implications of blast cell morphology in childhood acute lymphoblastic leukemia: a report from the Childrens Cancer Study Group.

The Childrens Cancer Study Group (CCSG) has evaluated French-American-British (FAB) morphology in newly diagnosed children with acute lymphoblastic leukemia (ALL) since 1975. A modification of the FAB system was used in which individual lymphoblast cells were scored and designated as either L1 or L2 on the basis of distinctive morphologic characteristics. L1 ALL was defined as less than 10% L2 cells and greater than 90% L1 cells; L2 ALL was defined as greater than or equal to 10% L2 cells and less than 90% L1 cells. FAB morphology was an independent predictor of overall survival (P = 0.02) in CCSG-141 and a highly significant predictor of successful induction of complete remission and event-free survival in the CCSG-160 series (P = 0.00001). These studies involved nearly 3900 patients. Two concordance studies have been performed. In the first (1981) study, overall concordance between the FAB reference laboratory and member institutions was 76% using a two-category system (L1, non-L1), 86% for L1 cases, and 47% for non-L1 cases. In the second (1984) concordance study, the use of more stringent, semiquantitative definitions of L1 and L2 lymphoblasts did not improve overall (75%), L1 (89%), or non-L1 (46%) concordance. The results of reference laboratory classification more powerfully predicted event-free survival than did member institutions (P = 0.016 vs P = 0.125). Quality control factors (slide preparation, cellularity, staining quality, and discipline of the reviewer) did not influence concordance. These results justify the continued assignment of patients to protocols of the CCSG-100 series on the basis of the modified FAB classification. The biological significance of the FAB morphologic variants remains to be determined.

Child↗

[Clinico-morphological characteristics and working classification of kidney diseases in patients with systemic scleroderma].

Based on a study of 110 patients with systemic scleroderma (SSD) (74 had involved and 36 uninvolved kidneys) the authors provide the clinico-functional and morphological characteristics of renal lesions and the working classification of sclerodermic nephropathy (SN). The two basic variants of SN were recognized: acute and chronic. The latter one was subdivided into clinical, moderate and pronounced SN. A correlation was revealed between the functional (glomerular filtration lowering) and ultrastructural (reticulation of endotheliocytes and thickening of the basal membrane of the glomerular capillaries) signs evidencing derangement of the microcirculatory bed. Morphological examination of the kidneys disclosed alterations that mirror the complex pathogenesis of SSD: derangement of the microcirculatory bed, immunopathological disorders, activation of connective tissue components.

Adolescent↗

[Carcinoma of the major duodenal papilla].

The analysis of 134 postmortem cases and 65 clinical observations of carcinoma of the major duodenal papilla (MDP) has revealed different variants of development of carcinoma of MDP. The classification by the TNM system is made. In 34% of cases carcinoma was found to spread beyond the duodenum limit. Papillectomy should not be considered radical intervention. Average survival time after papillectomy was 17 months, after pancreatoduodenal resection it was 34 months. Fibroduodenoscopy, laparoscopic cholecystocholangiography in combination with relaxation duodenography was used for early diagnosis of carcinoma of MDP. Laparoscopic investigation was always accomplished by laparoscopic cholecystostomy which permitted avoiding hepatic insufficiency in the postoperative period.

Adult↗

[The dependence of the progression of chronic glomerulonephritis on the clinical and morphological types of glomerulonephritis and on the tubulointerstitial changes].

Progression of chronic glomerulonephritis (CGN) is strongly associated with morphologic type of the disease, tubulointerstitial changes, some clinical syndromes. The aim of the study was to trace relations between the onset of chronic renal failure within 7 years since the diagnosis (fast progression of CGN--FP CGN), CGN clinical variant according to M. Ia. Ratner et al. classification (1987) and histomorphological changes in the renal biopsy. Unfavorable clinical types (active nephritic types and nephrotic-hypertensive type) proved dominating predictor of FP CGN not only because of close relationship between these type and FP CGN but also due to FP CGN occurrence in morphologically unfavorable morphological types and tubulointerstitial changes in line with concomitant unfavorable clinical types.

Adolescent↗

[Systemic vascular purpura: clinical etiological variants].

Systemic vascular purpura (SVP) represents angiites, heterogeneous by etiology and pathogenesis. Their pathogenetic classification does not fully reflect the clinical picture, course, and prognosis. The authors suggest a clinical etiological approach to differential diagnosis of SVP. With this aim in view they examined 82 patients with SVP hospitalized at Clinic of Therapy and Occupational Diseases of I. M. Setchenov Moscow Medical Academy in 1993-1996. Etiologically-associated differences in clinical manifestations of SVP were detected and clinical etiological variants of disease were distinguished. Endotoxemia was found to be often associated with purpura of different etiology, which can be regarded as a stage in the pathogenesis. The data allow creation of a clinical etiological classification of SVP and development of new therapeutic approaches.

Adolescent↗

[Classification of the types of blood supply of the muscles from the standpoint of plastic surgery].

Based on an experience with 120 composite flaps including the muscle and with special account of literature data the author describes a new classification of types of blood supply of muscles. The author proposes 6 types of blood supply of muscles and substantiates a direct relationship between the anatomy of muscle vessels and variants of its use for plasty. A comparison of the new classification and other classifications was made.

Blood Vessels↗

[The importance of the clinical classification of chronic glomerulonephritis for the prognosis of its progression and of the efficacy of therapy].

A significant correlation exists between clinical types of chronic glomerulonephritis (CGN) in a new clinical classification and morphological CGN types according to classification of WHO experts. Rapid progression is significantly correlated with clinicomorphological variants of CGN represented by unfavorable clinical types and unfavorable morphological types. No rapid progression occurred in combination of clinically favourable types and morphologically favourable and unfavorable types. The above classification is recommended for practice.

Adolescent↗

Adenocarcinoma of the lung mimicking inflammatory lung disease with honeycombing.

Pulmonary adenocarcinoma of the lung and its variants are well-defined entities, since the recent WHO classification of lung tumours. However, scant descriptions have been allocated to associated stromal changes, such as prominent inflammation and fibrosis, which can overshadow a tumoral proliferation and masquerade as a benign reactive process and this has not been recognised as a histopathological variant. The case of a 72-yr-old farmer who presented a multifocal well-differentiated adenocarcinoma that mimicked honeycomb lung with bronchiolectasis radiologically, on computed tomography scan and histologically at open lung biopsy, is reported. Histological pitfalls in the biopsy were represented by mild atypical cuboidal or columnar epithelial cells lining bronchiolar structures resembling florid bronchiolar metaplasia in a background of extensive fibrosis and inflammation, features that mimicked inflammatory honeycombing. However, histological analysis of the surgical resection of the main lesion, performed because of a clinical alteration of the patient, confirmed the diagnosis of multifocal adenocarcinoma of mixed subtype. A monomorphic proliferation of clear cells, lack of associated ciliated or squamous cells and presence of significant cytologic atypia gave a diagnosis of malignancy. This case illustrates how inflammatory and fibrotic changes may conceal a correct diagnosis of carcinoma and emphasises the importance of adequate sampling in such cases.

Adenocarcinoma↗

[Genetic and biological features define two types of follicular non-Hodgkin grade 3 lymphoma].

In the REAL classification system, follicular lymphomas (FL) were subdivided into three grades depending on the number of blasts (6). In this study, we were interested in defining biological parameters possibly being important in the delineation of subgroups. Between 1990 and 1998, biological and cytogenetic investigations were performed on 91 FL. Clonal aberrations were found in all cases. The tumours were subclassified according to the blast content and the morphology of the centrocytes into 29 FL 1, 33 FL 2, 15 FL 3, and 14 FL 3 with a diffuse large B-cell lymphoma component (FL 3 + DLBL). They were characterised by classical cytogenetics, for their mitotic (MI) and proliferative (PI) indices, and CD10, bcl-2, and p53-expression. In contrast to FL 1 and FL 2, which showed a common genetic background with t(14;18), and only differed by their blast content and MI/PI, FL 3 (with or without associated DLBL) turned out to be an inhomogeneous group. 11 follicular lymphomas (with > 150 blasts/10HPF) still showed maturation to centrocytes. They were positive for CD10 and harboured the t(14;18) in 73%. These cases correspond to a "high grade" variant of centroblastic-centrocytic lymphoma according to the Kiel classification (FL 3a). In 18 cases with a follicular or follicular and diffuse growth pattern, the infiltrate consisted of centroblasts exclusively. These tumours were CD10+ in only 50% and were t(14;18)+ in only 22%. Secretory differentiation (clg+) was found in 44%. They were--with respect to primary and secondary chromosome aberrations--more comparable to a follicular variant of DLBL and hence, correspond to centroblastic lymphoma, follicular or centroblastic lymphoma, follicular and diffuse according to the Kiel classification (FL 3b). By histomorphological, biological and cytogenetic investigations, therefore, FL 3 can be delineated into two different biological subgroups with obviously different transformation pathways.

Antigens, CD↗

[Effects of immunologic markers on prognosis in acute lymphoblastic leukemia in young children].

Altogether 77 children aged up to 3 years with acute lymphoblastic leukemia (ALL) were examined for the immunological phenotype of blast cells. L1, L1/L2, L2/L1, L2 variants of ALL and the undifferentiated one were established in accordance with criteria of the FAB classification. T1, zero and Ia immunosubvariants were recorded most frequently. Mature cell T2 and pre-B variants (3 and 2 cases, respectively) were rare; B-cellular acute lymphoblastic leukemia was lacking; the "common" subvariant was revealed in 28 patients. The patients' age produced the highest effect on the prognosis: the significantly least disease standing and remissions were noted in a group of children under 2 years as compared to those aged 2 to 3 years. As for immunological markers, expression of Thy1-antigen exerted an unfavourable effect on the prognosis and duration of the first remission.

Antibodies, Monoclonal↗

Erythroleukemia: a comparison between the previous FAB approach and the WHO classification.

Erythroleukemia is, within FAB classification, a proliferation of erythroblasts superior to 50% and of myeloblasts superior to 30%. The new WHO classification abolishes the frontier between RAEB-t with 20% and leukemia with 30% of blasts. AML6 variant is a new entity characterized by the proliferation of immature erythroblasts and the absence of non-erythroid blast cells. We analyzed 16 erythroleukemia, 5 RAEB-t and 2 AML6 variants to clarify their relationship. We suggest on survival, karyotype and cytologic characteristics that secondary erythroleukemia are the same entity as RAEB-t, confirming the WHO classification and that amongst de novo erythroleukemia, there is 'AML6 variant' with pure erythroid lineage proliferation.

Adult↗

Treatment results of oral verrucous carcinoma and its biological behavior.

The biologic behavior of and optimal treatment for oral verrucous carcinoma (VC) remain controversial. We analyzed the clinicopathological characteristics of 12 patients with oral VC. Immunohistochemical techniques were used to evaluate p53 protein, CD44 variant 9, and proliferating cell nucleus antigen. The TNM classification (UICC, 1997) was T1 in 1 patient, T2 in 3, T3 in 4, and T4 in 4. All patients were classified as N0M0. Four patients were treated by surgery alone and 8 by surgery after chemotherapy, radiotherapy, or both. After surgery, two patients had primary recurrence of disease. Immunohistochemically, the proliferative activity of tumor cells as evaluated by proliferating cell nuclear antigen labeling index and p53 protein expression was similar in VC and well-differentiated squamous cell carcinoma. However, CD44 varient 9 expression was positive in 8 of 10 VC, suggesting that oral VC is associated with a low risk of lymph node metastasis. Positive CD44 variant 9 expression by most oral VCs, indicating a low risk of cervical lymph node metastasis, suggests that most cases can be controlled by surgical intervention.

Adult↗

Classification and survival rate of patients with serous cystadenocarcinoma of the ovaries.

The comparative evaluation of two stage classifications including USSR Ministry of Health stage classification and TNM system was performed on the base of studies of the end results of 419 patients with serous cystadenocarcinoma of the ovaries. It has been established that TNM system is of a greater importance in establishing more accurate prognosis than the stage classification of the Ministry of Health. In TNM system four variants of the degree of extension correspond to each stage that allows to evaluate not only the extent of the primary tumor that also of the metastatic spread. The achieved results point to the superiority of the TNM system and serve as a base for transition to the TNM system in the classification of ovarian carcinoma.

Cystadenocarcinoma↗