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Comparative results of percutaneous transluminal coronary angioplasty in patients with dynamic versus fixed coronary stenosis.

This study compares the results of percutaneous transluminal coronary angioplasty in a group of 132 patients (group A) with fixed atherosclerotic narrowing (no spontaneous or ergonovine-provoked spasm) and in a group of 97 patients (group B) with dynamic coronary stenosis (spasm superimposed on the stenosis). All these patients underwent complete follow-up angiography. The rate of restenosis (defined as a loss of 50% of the initial gain) was significantly higher in patients in group B (dynamic coronary stenosis) than in group A (fixed narrowing) (35 versus 22%, p less than 0.05). Despite treatment with a calcium antagonist, coronary artery spasm persisted in 44% of the patients in group B and was detected for the first time in 15% of the patients in group A. Thus, in patients with dynamic coronary stenosis, the results of coronary angioplasty were less satisfactory than in patients with fixed narrowing, and in both groups coronary artery spasm was frequently (64%) superimposed on the restenosis.

Adult↗

Salvage of an impacted canine associated with an adenomatoid odontogenic tumour: a case report.

The adenomatoid odontogenic tumour (AOT) has been known by a number of descriptive names (adenoameloblastoma, ameloblastic adenomatoid tumour, glandular ameloblastoma, and adenomatoid ameloblastoma) since it was first reported and later recognised as a distinct odontogenic lesion unrelated to ameloblastoma. Although it was considered to be a variant of ameloblastoma at one time leading surgeons to perform unduly aggressive surgery, the treatment outcome experience has borne out the benign, nonaggressive nature of this lesion. The AOT is now considered to be a hamartoma with completely benign behaviour. Recurrence seldom if ever occurs after surgical curettage. Thus, it appears needless to extract involved anterior teeth associated with the tumour, especially in children. We report a case in which surgical and orthodontic treatment helped to salvage an impacted mandibular canine associated with an AOT in an adolescent girl. We have not found such a procedure to have been done for this tumour in the literature.

Adolescent↗

Cor pulmonale: an unusual presentation of tropical eosinophilia.

Tropical pulmonary eosinophilia (TPE) is considered to be a variant of human filarial infection. The pulmonary manifestations of TPE have been well described. Extra-pulmonary features of the disease, although not commonly seen, have been reported previously. A 9-year-old Malay girl with a history of recurrent cough and wheezing was admitted because of cardiac failure. Physical examination revealed a very sick girl with tachypnoea, central cyanosis, finger clubbing, elevated jugular venous pulse, generalized crackles and rhonchi in the chest, a loud second heart sound and hepatosplenomegaly. A chest radiograph showed cardiomegaly and right pleural effusion. Laboratory investigations revealed hypochromic, microcytic anaemia with persistent blood eosinophilia (absolute eosinophil counts varied from 1.9 to 5.5 x 10(9)/1). The ELISA test for antifilarial IgG antibodies was strongly positive. She responded promptly to treatment with diethylcarbamazine. In summary, this is a patient with TPE who presented with cor pulmonale, probably due to late-stage interstitial pulmonary fibrosis. In order to prevent the long term morbidity of cardiorespiratory disability, the early signs of TPE should be recognized and the infection treated.

Animals↗

Autoimmune liver disease.

Autoimmune hepatitis can affect diverse ethnic groups, and its clinical expression and outcome can vary accordingly. An asymptomatic presentation may identify patients who respond more readily to medication. Celiac sprue is important to recognize and treat by gluten restriction. Centrilobular necrosis and coincidental destructive cholangitis do not preclude the diagnosis, and antibodies to soluble liver antigen/liver pancreas may identify patients susceptible to relapse. Women, who have non-DRB1*0401 DR4 alleles more commonly than do men, may respond to a diverse range of autoantigens. DRB1*1301 is associated with autoimmune hepatitis in Brazil, especially among children, and it may favor an indigenous triggering agent. Variant syndromes are heterogenous conditions that probably reflect referral biases, and molecular mimicry between foreign and self-antigens is the basis for most theories of pathogenesis. Immunosuppressive medications (eg, cyclosporine, mycophenolate mofetil) have been used empirically with success, and recurrent and de novo disease after liver transplantation must be considered in all patients with graft dysfunction.

Journal Article↗

Primitive myxoid mesenchymal tumor of infancy: a clinicopathologic report of 6 cases.

Soft tissue sarcomas in the first year of life are rare, and the most common sarcomas in infancy are embryonal rhabdomyosarcoma, Ewing sarcoma/primitive neuroectodermal tumor, congenital infantile fibrosarcoma, and primitive sarcomas such as undifferentiated sarcoma. In this study, we report 6 cases of a primitive myxoid mesenchymal tumor of infancy (PMMTI), which previously may have been included under the diagnostic categories of congenital-infantile fibrosarcoma or infantile fibromatosis. PMMTI occurred in 6 infants, 3 of whom had a congenital presentation of a soft tissue mass. All patients were otherwise healthy. The tumors occurred on the trunk, extremities, and head and neck. Grossly, the tumors were nonencapsulated and had a multinodular appearance with focal infiltrative growth, a white fleshy cut surface, and a tumor diameter ranging from 2 to 15 cm. Histologically, a diffuse growth of primitive spindle, polygonal, and round cells occurred in a myxoid background. The tumor cells were arranged in a vaguely nodular pattern with peripheral collagenized stroma, higher cellularity at the periphery, and a delicate vascular network in the background. Immunohistochemically, the tumors displayed diffuse reactivity for vimentin and no reactivity for smooth muscle actin, muscle specific actin, desmin, S-100 protein, or myogenin. Electron microscopy documented a poorly differentiated fibroblastic proliferation. Four cases tested negative for the ETV6-NTRK3 gene fusion by RT-PCR. One tumor had a complex karyotypic abnormality with rearrangements involving chromosomes Y, 9, and 3. Three patients had recurrences or metastasis treated with a combination of surgery and chemotherapy. One patient is alive with persistent locally aggressive disease, 2 are alive with no evidence of recurrence, 1 had a recurrence treated surgically without further follow-up information, 1 patient died with persistent tumor and sepsis 6 weeks after diagnosis, and 1 patient was lost to follow-up. The morphologic appearance combined with the ultrastructural features and absence of the typical gene rearrangement of congenital-infantile fibrosarcoma are unique, and we propose that PMMTI represents a new category of pediatric fibroblastic-myofibroblastic tumor.

Dermatofibrosarcoma↗

Uterine tumor resembling ovarian sex-cord tumor with osteoid metaplasia.

A rare uterine tumor showing sex-cord-like differentiation and marked osteoid metaplasia was found in a 33-year-old female. The tumor, occupying the uterine fundus, was multilobular, fleshy, and yellowish white, with punctations and poorly defined tumor margins into the subserosa. The tumor nodules were mostly composed of small, round, rather regular neoplastic cells with scanty cytoplasm and prominent nuclei. Epithelioid neoplastic cells with striking ovarian sex-cord-like arrangement, surrounded by fibrous tissue, were noticed. Polygonal neoplastic cells with marked osteoid metaplasia were also detected. The authors introduce an infrequent variant of uterine tumor resembling ovarian sex-cord tumor with osteoid metaplasia, probably of endometrial stromal cell origin. Because of deep myometrial infiltration without vascular invasion, malignant potential is considered. The patient is now alive without evidence of local recurrence or distant metastasis after abdominal total hysterectomy.

Adult↗

Treatment of encephalomyocarditis virus-induced central nervous system demyelination with monoclonal anti-T-cell antibodies.

Infection of BALB/c mice with the M variant of encephalomyocarditis virus resulted in the development of a paralytic syndrome in 7 to 10 days. The paralysis was maximal during the period of viral clearance; most of the animals recovered from the initial deficit and showed no delayed recurrences. Pathologically, the white matter of brain and spinal cord showed well-demarcated areas of perivascular cuffing, demyelination, and, during recovery, remyelination by oligodendrocytes--all suggestive of postinfectious encephalomyelitis. Depletion of either the CD4 or CD8 subset of T cells in vivo with the appropriate monoclonal antibody, GK1.5 or 2.43, respectively, administered one day (24 h) prior to infection was sufficient to limit the development of the paralytic syndrome by 79% (GK1.5) and 82% (2.43).

Animals↗

Genome-wide profiling of papillary thyroid cancer identifies MUC1 as an independent prognostic marker.

Clinicopathological variables used at present for prognostication and treatment selection for papillary thyroid carcinomas (PTCs) do not uniformly predict tumor behavior, necessitating identification of novel prognostic markers. Complicating the assessment is the long natural history of PTC and our rudimentary knowledge of its genetic composition. In this study we took advantage of differences in clinical behavior of two distinct variants of PTC, the aggressive tall-cell variant (TCV) and indolent conventional PTC (cPTC), to identify molecular prognosticators of outcome using complementary genome wide analyses. Comparative genome hybridization (CGH) and cDNA microarray (17,840 genes) analyses were used to detect changes in DNA copy number and gene expression in pathological cPTC and TCV. The findings from CGH and cDNA microarray analyses were correlated and validated by real-time PCR and immunohistochemical analyses on a series of 100 cases of cPTC and TCV. Genes identified by this approach were evaluated as prognostic markers in cPTC by immunohistochemistry on tissue arrays. CGH identified significant differences in the presence (76 versus 27%; P = 0.001) and type of DNA copy number aberrations in TCV compared with cPTC. Recurrent gains of 1p34-36, 1q21, 6p21-22, 9q34, 11q13, 17q25, 19, and 22 and losses of 2q21-31, 4, 5p14-q21, 6q11-22, 8q11-22, 9q11-32, and 13q21-31 were unique to TCV. Hierarchical clustering of gene expression profiles revealed significant overlap between TCV and cPTC, but further analysis identified 82 dysregulated genes differentially expressed among the PTC variants. Of these, MUC1 was of particular interest because amplification of 1q by CGH correlated with MUC1 amplification by real-time PCR analysis and protein overexpression by immunohistochemistry in TCV (P = 0.005). Multivariate analysis revealed a significant association between MUC1 overexpression and treatment outcome, independent of histopathological categorization (P = 0.03). Analysis of a validation series containing a matched group of aggressive and indolent cPTCs confirmed the association between MUC1 overexpression and survival (relative risk, 2.3; 95% confidence interval, 1.1-5.5; P = 0.03). Our data suggest that MUC1 dysregulation is associated with aggressive behavior of PTC and may serve as a prognostic marker and potential therapeutic target in this disease.

Carcinoma, Papillary↗

Targeted inhibition of the KLF6 splice variant, KLF6 SV1, suppresses prostate cancer cell growth and spread.

Prostate cancer is a leading cause of cancer death in men. Risk prognostication, treatment stratification, and the development of rational therapeutic strategies lag because the molecular mechanisms underlying the initiation and progression from primary to metastatic disease are unknown. Multiple lines of evidence now suggest that KLF6 is a key prostate cancer tumor suppressor gene including loss and/or mutation in prostate cancer tumors and cell lines and decreased KLF6 expression levels in recurrent prostate cancer samples. Most recently, we identified a common KLF6 germ line single nucleotide polymorphism that is associated with an increased relative risk of prostate cancer and the increased production of three alternatively spliced, dominant-negative KLF6 isoforms. Here we show that although wild-type KLF6 (wtKLF6) acts as a classic tumor suppressor, the single nucleotide polymorphism-increased splice isoform, KLF6 SV1, displays a markedly opposite effect on cell proliferation, colony formation, and invasion. In addition, whereas wtKLF6 knockdown increases tumor growth in nude mice >2-fold, short interfering RNA-mediated KLF6 SV1 inhibition reduces growth by approximately 50% and decreases the expression of a number of growth- and angiogenesis-related proteins. Together, these findings begin to highlight a dynamic and functional antagonism between wtKLF6 and its splice variant KLF6 SV1 in tumor growth and dissemination.

Alternative Splicing↗

Recurrent conjunctival melanoma with neuroidal spindle cell features.

A 66-year-old white woman had had a 20-year history of flat primary acquired melanosis involving the left inferior forniceal and palpebral conjunctiva. Over the ensuring 12 years, the patient experienced multiple recurrences of invasive malignant melanoma that emerged from the progressive primary acquired melanosis. Two of these recurrences were composed of nonpigmented spindle cells, and in the most florid invasive malignant melanoma that developed, the spindle cells formed a nodule 7.5 mm thick. The spindle cells were organized into fascicles and small bundles, the latter separated by a loose stroma that was devoid of mucopolysaccharides. The fascicular and neuroidal features in this case were sufficiently well developed to suggest the incorrect diagnosis of a neural tumor or a neurofibroma. However, the presence of intraepithelial atypical melanocytes at the edge of the spindle cell lesion, the absence of mucopolysaccharides in the stroma, the mitotic activity, and the absence of intercellular reticulin fibers favored the diagnosis of a spindle cell invasive malignant melanoma. This morphologic variant of conjunctival melanoma is compared with related cutaneous lesions of melanoma featuring a spindle cell population.

Aged↗

Coexistence of (partial) immune defects and risk of recurrent respiratory infections.

BACKGROUND: Respiratory infections are major causes of morbidity and mortality, but determinants of susceptibility are poorly defined. We studied whether and to what extent immunologic and genetic factors are associated with increased susceptibility to respiratory infections. METHODS: We evaluated the prevalence of IgA, IgM, IgG, and IgG subclass deficiencies, impairment in the antibody response against pneumococcal polysaccharides, G2m(n) allotypes, Fc gamma RIIa polymorphisms, partial C2 and partial C4 deficiency, promoter polymorphisms in MBL2, and lymphocyte subset deficiencies in a control population and in consecutive children with recurrent respiratory infections. RESULTS: IgA and/or IgG subclass deficiency was found in 27 of 55 patients (49%) and 6 of 43 controls (14%) (P = 0.0006). An impaired antibody response to polysaccharides was found in 7 patients (19%) and in 0 of 37 controls (P = 0.002). The Gm(n)marker was absent in 25 of 55 patients (45%) and 6 of 42 controls (14%) (P = 0.009). The MBL2 variants O/O, A/O, and A/A occurred in 9, 14, and 32 of the 55 patients, respectively, and in 1, 19, and 23 of the 43 controls, respectively (P = 0.05). There was no increase in the prevalence of partial C4 deficiency, C2 deficiency, lymphocyte subset deficiency, or Fc gamma RIIa polymorphism in the patients compared to the controls. A combination of at least 2 immune defects was found in 31 of 55 patients (56%) and in 4 of 42 controls (11.6%) (P <0.0001). CONCLUSION: Specific antipolysaccharide antibody deficiency, IgA and/or IgG subclass deficiency, Gm(n) allotype, and MBL2 genotype are susceptibility factors for recurrent respiratory infections, and coexistence of several immune defects is the strongest risk factor in this study.

Adolescent↗

[Prognostic parameters in peptic ulcer disease].

Tendency of peptic ulcer to frequent recurrence and spontaneous healing as to life-threatening complications are apart of the natural history of ulcer disease. In a retrospective study of 7 years, we examined 27 simple factors for their prognostic value by 218 patients suffering from ulcer disease which were diagnosed by fibroscopy. The interpretation with help multi analysis, the variant and discriminant analysis showed by patients with stomach ulcer that, the prognosis of the combination between renunciation of nicotine the begin of the disease until 30th years of the people, more than three foods daily, stomach cancer in the family, blood group A and left finger position would be suitable. The prognosis was better for patients with duodenal ulcer who have nicotine abstinence, childlessness negative history of the family with view to ulcer disease, blood group 0, positive Rh-factor and the working time.

Analysis of Variance↗

[Erosive pustular dermatosis of the scalp. A new entity or a variant of pyoderma vegetans].

We report on a 69-year-old woman suffering from an erosive pustular and crusted lesion of the scalp having persisted over years. There could not be found any external cause, and a large number of different physical and medicamental therapies failed. Prompt healing was achieved, however, after a low serum zinc level was corrected by a zinc preparation administered orally.-The dermatosis described closely resembles the so-called "erosive pustular dermatosis of the scalp". A search of the earlier dermatological literature supported our view that this is not a new disease, but rather a variant of pyoderma vegetans, a disease showing extremely variable clinical and histopathological features.

Aged↗

[Atraumatic procedures in common abdominal operations].

The surgical tactics and technical improvements are discussed against the background of personal experience with some common abdominal operations, among which the appendectomy variant leaving the cecum intra-abdominally and using chain retrograde ligature in some cases, with choice of access according to the requirements of the individual case. Special attention is called to retrocecal extraperitoneal complications and Meckel's diverticulum--clinical picture and operative tactics. Emphasis is laid on the advantages of intestinal anastomoses--latero-lateral variant, termino-terminal technique in colo-colostomy and possibilities of latero-terminal sigmoidorectoanastomosis.

Abdomen↗

Cotyledonoid leiomyoma: a benign uterine tumor with alarming gross appearance.

Cotyledonoid leiomyoma or "grapelike" leiomyoma is a very rare tumor among the ever-expanding repertoire of growth variants described in benign uterine leiomyoma. We report a case of cotyledonoid leiomyoma in a 55-year-old woman who presented with menorrhagia and uterine prolapse. A large multinodular fungating tumor adhering to the right posterolateral wall of the uterus and extending to the broad ligament was discovered at vaginal hysterectomy. With a provisional diagnosis of sarcoma, total hysterectomy and bilateral salpingo-oophorectomy were performed. Postoperatively, the patient was well with no evidence of recurrence at 14 months. Pathologic examination revealed a 10-cm, red-brown tumor that comprised multiple bulbous processes protruding over the uterine surface, in continuity with a dissecting intramyometrial component. It was composed of fascicles and nodules of bland-looking smooth muscle cells with prominent perinodular hydropic degeneration. Coagulative necrosis, mitoses, and nuclear atypia were absent. Cotyledonoid leiomyoma apparently results from a combination of several uncommon growth patterns operating together, including subserosal growth, dissecting growth, and perinodular hydropic degeneration. Increased awareness of this grossly alarming variant of benign uterine leiomyoma can help avoid overtreatment.

Female↗

Jaundice in pregnancy--1976.

An integrative survey is given of three disease processes, in which recent progress of a fundamental nature has been made, primarily affecting the liver, either coincident with or caused by the gravid state. The three conditions considered include (1) recurrent cholestasis of pregnancy (RCP), (2) viral hepatitis coincident with pregnancy, and (3) acute fatty liver of pregnancy (AFLP). In addition to an assessment of our present knowledge with respect to RCP and AFLP, new genetic hypotheses are proposed. In the latter, the proposal of an ornithine transcarbamylase deficiency, similar to that seen in Reye's syndrome, has potential therapeutic implications that are explored. In light of the currently available information on the interaction between maternal viral hepatitis and the variant forms of vertical maternal-fetal transmission, tentative recommendations regarding management of the newborn are suggested.

Anabolic Agents↗

Anxiety and major depression comorbidity in a family study of obsessive-compulsive disorder.

To understand the familial relationship between obsessive-compulsive disorder (OCD), other anxiety disorders, and major depressive disorder (MDD), we examined the rates of anxiety disorders and MDD in first-degree relatives of OCD probands and controls, the association between age at onset of OCD and the occurrence of other anxiety disorders and major depressive disorder in relatives of probands, and the co-transmission of specific anxiety disorders, MDD, and OCD within families of probands. Recurrence risks were estimated from 466 first-degree relatives of 100 probands with OCD and 113 first-degree relatives of 33 non-psychiatric controls. Rates of non-OCD anxiety disorders and MDD were comparable in relatives of OCD probands and controls. Rates of anxiety disorders and MDD were higher among case relatives with OCD than among case relatives without OCD and control relatives. Fifty percent of case relatives with OCD had at least one comorbid anxiety disorder. Early age at onset (<10 years) in probands was associated with higher rates of anxiety and depression comorbidity among case relatives with OCD but not among case relatives without OCD. The occurrence of specific anxiety disorders and MDD in case relatives was independent of the same comorbid diagnosis in the OCD probands. OCD, panic disorder, generalized anxiety disorder, and MDD occurred together more often than expected by chance among individuals with OCD. Furthermore, age at onset in probands is associated with specific anxiety and affective comorbidity among case relatives. These findings support the hypothesis that early- and late-onset OCD represent different etiologic variants.

Age of Onset↗