bluuebunny/crossref_metadata_2025 huggingface dataset:
Morphological and Immunophenotypic Features of Chronic Lymphocytic Leukemia
Authors: Matutes Estella , Polliack Aaron
Published: March 2003

In this review, we summarize the morphological features and immunophenotypic profile of chronic lymphocytic leukemia (CLL) cells, discuss the value of these investigations as front line diagnostic tests, and emphasize their correlation with the clinical features, disease progression, molecular genetics and pathogenesis of CLL.

In CLL, the morphology of the circulating cells is characteristic and typical in the majority of cases. However, 15% of patients, either at diagnosis or during the course of the disease, show atypical morphology reflected by either (1) an increased (>10%) number of circulating prolymphocytes, designated CLL/PL, or
(2) an increased (>15%) number of circulating lymphoplasmacytic and cleaved cells, designated ‘atypical’ CLL. There is strong evidence of a close association between atypical morphology (CLL/PL) and atypical (CLL) and clinical features, e.g. disease progression, advanced stage and survival, molecular genetics, particularly trisomy 12, but also the rare cases with t(11;14) or t(14;19), p53 abnormalities, unmutated immunoglobulin (Ig) VH genes and origin of the cell (naive, pregerminal center cell).

CLL cells have a distinct immunological repertoire different from that of other lymphopro‐liferative disorders. The typical CLL phenotype is CD5+, CD23+, FMC7–, weak expression of surface Ig (sIg) and weak or absent expression of membrane CD22 and CD79b. The latter marker identifies an extracellular epitope of the B‐cell receptor (BCR) β chain and its weak or absent expression in CLL may derive from the expression of a truncated form. This, together with the low expression of CD22, might explain the abnormal signal transduction of CLL cells similar to that of anergic B lymphocytes. Because no single marker is specific for CLL, a composite phenotype considering this set of 5 or 6 markers compounded into a scoring system helps to distinguish CLL from the other B‐cell malignancies. Immunophenotypic analysis has also been shown to be useful for minimal residual disease detection and adds valuable prognostic information because the expression of certain markers, such as FMC7 or CD38, seems to be associated with a poor outcome.

In addition, CLL cells express a variety of Bcl‐2 family proteins with a profile that favors inhibition of apoptosis which, together with the interaction with microenvironmental (e.g. stromal) cells and the release of cytokines, explains the long life span and subsequent accumulation of CLL cells in various organs. Despite controversies relating to the expression of adhesion molecules (selectins and integrins) in CLL cells, it appears that some of these molecules do play a role in the pathogenesis, biology and clinical patterns of the disease. In conclusion, morphology and immunophenotype are the two essential investigations, which must be carried out in all cases of CLL. Both provide relevant information in terms of diagnosis, course of the disease, prognosis and pathogenesis.
ACUTE MYELOID LEUKEMIA: TREATMENTOF ADULTS UNDER 60 YEARS
Authors: Burnett Alan K.
Published: March 2003

Current chemotherapy with advanced supportive care will enable 75–80% of acute myeloid leukemia patients aged 60 years or under to enter complete remission. Several regimens achieve broadly similar results. For patients who enter complete remission, the overall relapse risk is now 45–50%, but this is highly variable and is primarily determined by the biology of the disease. Cytogenetics are strongly influential in response to induction and consolidation with t(15:17), t(8:21) and inv(16) either occurring alone or with additional abnormalities having a relapse risk of about 30% and complex changes, abnormal 3q or abnormalities of chromosomes 5 and 7 resulting in a lower remission rate and a rapid relapse cumulating to 80%. FLT3 mutations occur in 25% of patients and are an independent predictor of relapse and, when combined with cytogenetics, adversely influence the prognosis in each cytogenetic risk group. Recent prospective collaborative group trials have endeavored to evaluate allogeneic and autologous bone marrow transplant against or in addition to consolidation chemotherapy. Suboptimal treatment delivery emerged as a problem. When the results were reported on an intention‐to‐treat basis, no overall survival advantage was consistently seen for either type of transplant. However, a significant reduction in risk of relapse was usually seen overall and within risk groups. Analysis within risk groups suggests that transplant is not indicated in good risk disease and continues to require evaluation in standard or poor risk patients. It is probable that traditional dose intensification has now reached its limits of tolerability, so new approaches will be required for further progress to be made. Modulation of chemoresistance mechanisms or immunologically directed chemotherapy represent immediate prospects for clinical study.
Differential metabolic response in AIDS‐related chronic protozoal diarrhoea
Authors: Sharpstone D. , Phelan M. , Gazzard B.
Published: March 2003

Objective As the pro‐inflammatory cytokine tumour necrosis factor‐alpha is greater in microsporidiosis than cryptosporidiosis, there may be a distinct metabolic response between the two organisms.

Design Male HIV seropositive subjects with an untreated AIDS‐defining diagnosis of microsporidiosis or cryptosporidiosis had measurement of oxygen consumption and carbon dioxide production by indirect calorimetry and body composition analysis to express resting energy expenditure (REE) and substrate oxidation per kilogram of metabolically active tissue.

Methods Resting energy expenditure (REE), non‐protein respiratory quotient (NPRQ), fat and carbohydrate oxidation were calculated from respiratory gas analysis. Fat, fat‐free and appendicular muscle masses were measured by dual‐energy X‐ray absorptiometry. Subjects with protozoal diarrhoea were compared to other newly diagnosed, active opportunistic infections. Controls were asymptomatic

HIV‐seropositive men matched by peripheral CD4 count.

Results Seven subjects with microsporidiosis and six with cryptosporidiosis were compared with 24 subjects with other AIDS‐defining diagnosis (Pneumocystis carinii pneumonia, cytomegalovirus enteritis and Mycobacterium avium‐intracellulare) and 10 controls free from secondary infection. Subjects with cryptosporidiosis had a decreased REE, a significantly increased NPRQ (P< 0.05), decreased fat oxidation (P < 0.05) and increased carbohydrate oxidation compared to microsporidiosis. Subjects with other AIDS diagnoses had an increased REE (P < 0.01) and fat oxidation and decreased carbohydrate oxidation compared to cryptosporidiosis, and a similar metabolic response to microsporidiosis.

Conclusions The metabolic response to cryptosporidiosis differs from microsporidiosis and associated weight loss may be mediated by different mechanisms. Metabolism in other AIDS diagnoses, including microsporidiosis, is compatible with a cachectic response.
Detection of Mycoplasma fermentans in broncho‐alveolar lavage fluid specimens from AIDS patients with lower respiratory tract infection
Authors: Ainsworth Jg , Clarke J , Lipman M , Mitchell D , Taylor‐Robinson D
Published: March 2003

Objective  To investigate the role of Mycoplasma fermentans in lower respiratory tract disease of HIV‐positive patients.

Methods  A polymerase chain reaction (PCR) assay was used to detect M. fermentans in broncho‐alveolar lavage (BAL) fluid and peripheral blood mononuclear cells (PBMCs) collected from 78 hospitalized HIV‐positive patients with lower respiratory tract disease (the index group). BAL specimens were also assessed from two control groups; 25 HIV‐positive patients without respiratory symptoms and 46 patients with a respiratory illness who did not have risk factors for HIV.

Results  Seventy‐one (91%) of the 78 patients who comprised the index group had AIDS. Sixty‐one patients provided BAL and PBMC specimens; 16 provided a BAL specimen and one only a PBMC specimen; 15 (25%) of the 61 patients had M. fermentans positive BAL fluid, of whom 10 (67%) also had a positive PBMC specimen. In contrast, of the 46 patients who had a negative BAL fluid, only three (6.4%) had a positive PBMC sample (P < 0.00001 Fisher's exact test). Overall, 21 (27%) of 77 patients had M. fermentans positive BAL fluid. Of these positive patients, 16 also had another microorganism in the BAL fluid and for five patients M. fermentans was detected alone. Compared with patients in the index group, none of the 46 BAL fluid specimens from patients at low risk of HIV infection was M. fermentans positive (P < 0.001, χ2 15.13). Two (8%) of the 25 HIV‐positive patients without respiratory symptoms had a M. fermentans positive BAL fluid, positivity that was also statistically different from the index group (P = 0.045, χ2 4.01).

Conclusions  M. fermentans frequently invades the lower respiratory tract of AIDS patients as an opportunist and may cause disease in some.
Candidate vaccines for immunotherapy in HIV
Authors: Gotch FM , Imami N , Hardy G
Published: March 2003

In this short article we review the immunopathology of HIV infection and delineate the kinds of immune responses seen in acute infection and in long‐term nonprogression that are understood to be necessary if the host is to maintain long‐term control of the virus. In this context we discuss the potential promise of therapeutic vaccination in HIV infection.
The impact of the M184V substitution in HIV‐1 reverse transcriptase on treatment response
Authors: Miller V , Stark T , Loeliger AE , Lange JMA
Published: March 2003

The M184V mutation in the HIV‐1 reverse transcriptase gene is primarily associated with rapid, high‐level lamivudine (3TC) resistance. It has also been observed to arise under selective pressure by abacavir, to which it confers low‐level resistance.

Although the development of viral drug resistance remains a major concern in antiretroviral therapy, it is known that some immunological and clinical benefit can still be derived from highly active antiretroviral therapy (HAART) regimens despite resistance‐associated virological failure. This residual benefit on a failing regimen is commonly attributed to the preservation of fitness‐reducing protease inhibitor (PI) resistance mutations under continued drug pressure. However, fitness‐reducing nucleoside reverse transcriptase inhibitor (NRTI) mutations may also contribute to the effect. M184V is both common in the treated population and fitness‐reducing. A number of studies, both of dual nucleoside therapy and HAART, have noted a residual treatment effect for 3TC despite the assumed or observed presence of M184V and high‐level phenotypic resistance. The speed and consistency with which this mutation is selected by 3TC under suboptimal viral suppression therefore makes M184V a particularly interesting model for further clinical studies on the association of drug resistance with ongoing treatment benefit.

While fitness considerations are likely to be a major contributor to the clinical observations noted, there are a number of other potential mechanisms that may contribute to a continuing response to 3TC in the presence of M184V. These include the delay and reversal of zidovudine (ZDV) resistance, hypersensitization to other NRTIs, reduced reverse transcriptase (RT) processivity and a possible reduction in RT pyrophosphorolysis.

The full impact of M184V on therapeutic prospects will require further elucidation; ideally, the risk/benefit of preserving this substitution would be investigated in randomized trials. However, existing data suggest that the presence of this mutation may preserve some benefit in spite of the loss of 3TC susceptibility which, with further study, may prove valuable.
Cellulite: a review
Authors: Rossi Ana Beatris R , Vergnanini André Luiz
Published: March 2003

Gynoid lipodystrophy (cellulite) is an extremely controversial topic. A lack of knowledge regarding specific aetiopathogenic factors, as well as the opportunism of some professionals and the media, has fuelled debate regarding the scientific basis of this condition. This article reviews the clinical, epidemiological, histopathological and therapeutic aspects of cellulite.
Purpuric contact dermatitis in patients with allergic reaction to textile dyes and resins
Authors: Lazarov A , Cordoba M
Published: March 2003

Background Purpuric lesions have been described as an uncommon manifestation of allergic contact dermatitis in individual case reports.

Objective We describe a series of patients who developed purpuric allergic contact dermatitis to textile dyes and resins in their personal clothing. Our purpose was to study the patients clinically and histopathologically and to define the most frequent allergens, which cause purpuric allergic contact dermatitis.

Methods One hundred and three patients were clinically evaluated and tested with the Textile Color & Finish Series (TCFS) (Chemotechnique Diagnostics) and Standard Series (TRUE Tests) because of suspected allergic contact dermatitis (ACD) to clothing. The patients with clinical features of purpura as presenting sign of ACD were studied. Biopsies from the purpuric lesions were performed in three patients.

Results Thirty of the 103 patients (29.1%) had positive reaction to an allergen from the TCFS. Clinically purpuric ACD was observed in 8.7% of all the cases studied (n = 9 of 103). Nine of the 30 patch‐positive patients to the TCFS (30%) demonstrated purpuric macules, papules and patches. Patch testing of the nine patients with purpuric contact dermatitis, with the TCFS, resulted in 26 positive patch test results. The major causative allergens were the following: Disperse Blue 106 and Disperse Blue 124 in 26.9% each, Disperse Blue 85 in 11.5%, and ethyleneurea melamine formaldehyde in 7.7%. Positive patch tests were observed to dimethylol dihydroxyethyleneurea, dimethylol propyleneurea, tetramethylol acetylenediurea, urea formaldehyde, melamine formaldehyde, Disperse Red 17, and Basic Red 46 3.8% in each. Purpuric patch test reaction was observed in five cases. The patch test results had present relevance in all the cases. Lesional biopsies demonstrated acanthosis, spongiosis and parakeratosis. The blood vessels were dilated, without signs of vasculitis. The inflammatory infiltrate was composed of lymphocytes and erythrocytes. The extravasated erythrocytes had a perivascular and interstitial distribution in the superficial and deep plexus and were observed at the dermo–epidermal junction as well as in the epidermis.

Conclusion Purpuric contact dermatitis is not an uncommon clinical form of ACD to textile dyes and resins. New allergens, which can evoke the development of purpuric allergic contact dermatitis and have not been described in the literature until now include: ethyleneurea melamineformaldehyde, dimethylol dihydroxyethyleneurea, tetramethylol acetylenediurea, urea formaldehyde, melamine formaldehyde and Disperse Red 17.
Nail changes in Langerhans cell histiocytosis
Authors: Jain Sanjiv , Sehgal Virendra N , Bajaj Pramila
Published: March 2003

Nail changes in Langerhans cell histiocytosis are distinctly uncommon. Paronychial erythema, swelling and subungual pustules of the fingernails and toenails were cardinal, and were supported by diffuse as well as dense collections of mononuclear Langerhans cells evidenced by microscopic investigation. Oral administration of co‐trimoxazole (800 mg sulphamethoxazole + 160 mg trimethoprim) every 12 h, 50 mg/d cyclophosphamide and 80 mg/d predinisolone were the mainstay of treatment, supported by scalp tar shampoo and local betamethasone lotion application.
Aetiological factors in vulvar dermatitis
Authors: Crone Am , Stewart Ejc , Wojnarowska F , Powell Sm
Published: March 2003

Aim To determine aetiological factors in vulvar dermatitis.

Background Dermatitis of the vulva is a common clinical diagnosis in the specialized vulva clinic, although the exact prevalence is unknown. Aetiological factors are poorly understood in particular the incidence of atopy and seborrhoeic dermatitis. Other factors implicated are iron deficiency and exogenous causes such as contact irritants and allergens.

Methods During a 1‐year period, 38 patients with an initial clinical diagnosis of vulvar dermatitis were questioned, examined and investigated with full blood count, serum ferritin, bacterial swab where indicated and punch biopsy. After three patients had been excluded with other diagnoses, prick testing and patch testing were carried out.

Results Of the 35 patients with vulvar dermatitis, 34 (97%) had either an atopic background and/or seborrhoeic dermatitis. Iron deficiency occurred in seven patients (20%) and relevant positive patch tests in nine patients (26%). Twenty patients had biopsies and all had confirmatory histology.

Conclusion This study suggests that the main aetiological factor in vulvar dermatitis is an endogenous predisposition. Iron deficiency may also contribute, while contact with irritants and allergens may explain the localization and frequently complicates and exacerbates the picture. Secondary sensitization to multiple substances is common.
Erythromelalgia in a patient with AIDS
Authors: Mørk Cato , Kalgaard Ole M , Myrvang Bjørn , Kvernebo Knut
Published: March 2003

Erythromelalgia is a clinical syndrome characterized by burning pain in the extremities together with erythema and increased skin temperature. Typically, the patients experience relief from cold, and aggravation from warmth. Symptoms are hypothesized to be caused by arteriovenous shunting and reduced nutritive skin capillary perfusion with corresponding tissue hypoxia. Erythromelalgia is most often primary, but may be secondary to a wide variety of diseases. We report erythromelalgia in a patient with acquired immune deficiency syndrome (AIDS). At peak pain intensity he actively cooled hands and feet for more than 12 h/day. Many doctors handling human immunodeficiency virus/AIDS patients are unfamiliar with erythromelalgia, and the condition can easily be overlooked, especially the more common milder cases.
Second malignancies in melanoma patients in Thuringia
Authors: Wolff J , Wollina U
Published: March 2003

Objective

The incidence of melanoma is increasing. Melanoma patients are at risk for the development of second neoplasias. Data for the new German Bundesländer are not available, but would be suitable to define frequency, site and type of secondary malignancies and conclusions for follow‐up of melanoma patients.

Design

Retrospective study at the Melanoma Outpatient Clinic of the Department of Dermatology and Allergology at the University of Jena (Germany) for patients seen between June 1966 and June 1999. To investigate the impact of second malignancies on survival a case‐comparison study of mortality was performed. The log‐rank test and χ2 ‐test were used to investigate statistical significance. There were 554 patients with malignant melanoma, 237 male and 317 female, with an age at time of diagnosis between 17.0 and 90.1 years (mean 53.7 years). The mean follow‐up was 5.6 years.

Results

Sixty‐one patients (11.0%) developed a second tumour. The total number of tumours was 83. Forty‐five patients developed one, 16 developed ≥ 2 second tumours. Basal cell carcinoma (BBC) was the most frequent neoplasia (17 patients, 22 tumours; mean age 64.9 years). A second melanoma was found in 15 patients, while two developed a third melanoma. The mean tumour thickness was 0.81 mm (in second or third melanomas) compared with 1.92 mm of primary melanomas. Seven female patients developed breast cancer (eight cancers; mean age 57.3 years). The other second tumours included skin cancer (eight), gastrointestinal tract tumours (four), genital cancers (19), brain tumours (two), lung cancer (two) and other tumours (six). The difference in survival of patients with second tumours was not statistically significant from age‐, sex‐ and melanoma thickness‐matched controls.

Conclusions

Second malignancies were seen in 11.0% of melanoma patients. Most important are second skin tumours such as second melanomas and BCC, recommending follow‐up by the dermatologist. In a group of patients with regular follow‐up examinations, no negative impact of second tumours (BCC, melanoma, breast cancer) on overall survival could be detected.
Photosensitivity induced by oral itraconazole
Authors: Alvarez‐Fernández Jg , Castaño‐Suárez E , Cornejo‐Navarro P , Gómez de la Fuente E , Ortiz de Frutos Fj , Iglesias‐Diez L
Published: March 2003

A case of photosensitivity induced by itraconazole is reported. A 70‐year‐old woman had erythema, oedema and vesicles on sun‐exposed areas after 5 days of itraconazole treatment for oral candidiasis. Oral photochallenge using itraconazole and sun irradiation was positive, but photopatch test was negative. Photosensitivity from azoles is an uncommon adverse effect. Only three other cases have been described, two induced by ketoconazole and one by itraconazole.
Topical cidofovir for severe cutaneous human papillomavirus and molluscum contagiosum infections in patients with HIV/AIDS. A pilot study
Authors: Calista D
Published: March 2003

Background

Cidofovir is a nucleoside analogue of deoxycytidine with a strong activity against several DNA viruses, including herpes, pox and human papilloma virus (HPV).

Material and methods

Fourteen acquired immunodeficiency syndrome patients, 10 with extensive HPV lesions and four with molluscum contagiosum (MC) infections, unresponsive to conventional therapies, were treated with a cream containing cidofovir 1%. All the subjects had been on treatment with highly active antiretroviral therapy for almost 1 year before starting the cream. Measured end‐points of therapy were efficacy, tolerability, side‐effects and freedom from recurrence.

Results

Thirteen of the 14 patients (92.8%) completed the therapy, one dropped out. These 13 eventually cleared their MC or warts, over varying periods of time. In nine, the lesions regressed 2 weeks from the end of the first cycle of therapy. Three patients needed two cycles and the last three consecutive courses of topical therapy before the cutaneous lesions healed. No recurrence was observed in nine patients over an average follow‐up period of 24.1 months (range 12–30 months). Four patients had isolated relapses, which were successfully treated with simple curettage.

Side‐effects

All the patients experienced side‐effects where they applied the cream. Inflammation, erosion and a burning sensation were the most frequent. Postinflammatory hyperpigmentation was observed in six cases, while two developed a transient alopecia on the beard area. No systemic side‐effects or alteration of laboratory data were noted.

Conclusion

Cidofovir appears to offer an effective therapeutic alternative option for lesions that are unresponsive to conventional methods. Appropriate clinical trials are required, however, to confirm the true efficacy and safety of topical cidofovir.
Systemic ketoconazole for yeast allergic patients with atopic dermatitis
Authors: Bäck O , Bartosik J
Published: March 2003

Background

Adult patients with atopic dermatitis (AD), especially with the head–neck distribution, are often sensitized to the lipophilic yeast Malassezia furfur/Pityrosporum orbiculare, which is considered to contribute to the pathogenesis of the dermatitis.

Objective

Evaluation of the efficacy of oral ketoconazole on immunological and clinical parameters in yeast allergic adult patients with AD.

Study design

Randomized double‐blind placebo‐controlled study.

Subjects

Twenty‐nine patients with specific IgE antibodies to M. furfur/P. orbiculare and with elevated serum IgE participated in the investigation. Fifteen subjects were treated with 200 mg ketoconazole daily and 14 received placebo for 3 months. Betamethasone cream was allowed as supplementary therapy and the consumption was registered. The clinical score (SCORAD), total serum IgE and specific IgE antibodies to M. furfur/P. orbiculare, Candida albicans and Dermatophagoides pteronyssinus were monitored at the starting point and by the end of the first and third month.

Results

In the actively treated group the levels of specific IgE to M. furfur/P. orbiculare and C. albicans as well as total serum IgE decreased significantly. Sensitization to D. pteronyssinus was not influenced. The clinical score decreased in both groups, and the improvement was correlated to the consumption of topical steroids in the control group but not in the ketoconazole group.
The effect of hair loss on quality of life
Authors: Williamson D , Gonzalez M , Finlay AY
Published: March 2003

Background

The aim of this study was to quantify the effect of hair loss on quality of life. Patients were recruited from an alopecia support group, and were assessed using the Dermatology Life Quality Index (DLQI) and an adapted version of the DLQI. Financial utility questions, an abbreviated version of the Center for Epidemiologic Studies Depression Scale and open‐ended questions were also used.

Observations

Seventy (90% response rate) questionnaires were returned. DLQI scores in responders with hair loss (mean score = 8.3, SD = 5.6, range 0–23, n = 70) were similar to those recorded in severe psoriasis. The hair loss continued to have a significant impact on life quality well after the initial event (median duration of hair loss = 138 months ± 114; range 7–588, n = 70). Forty per cent of patients also felt dissatisfied with the way in which their doctor dealt with them.

Conclusions

This study specifically identifies the feelings of loss of self‐confidence, low self‐esteem and heightened self‐consciousness in people affected by hair loss.
Potential human papillomavirus reactivation following topical corticosteroid therapy of genital lichen sclerosus and erosive lichen planus
Authors: Von Krogh G , Dahlman‐Ghozlan K , Syrjänen S
Published: March 2003

Using a highly sensitive polymerase chain reaction (PCR) technique, based on general GP5+/GP6+ PCR primers covering 34 different human papillomavirus (HPV) types, the presence of HPV DNA was studied in paraffin‐embedded penile biopsies from 20 men treated topically with corticosteroids. Clobetasol propionate was applied for 2–16 (mean 7) weeks by 19 men (age 18–73; mean 40) with lichen sclerosus. High‐risk HPV was detected prior to therapy in three patients (16%) who lacked clinical or histopathological signs of HPV infection. Following therapy high‐risk HPV was detected in biopsies from four men (21%), of whom three also exhibited clinical and/or light microscopic signs of HPV infection. Low‐risk HPV DNA was not detected in any of these samples. Four biopsies were collected during a 5‐year period from a 51‐year‐old man who was treated repeatedly with topical mild–moderate potent corticosteroids at intervals of up to 10 weeks for penile erosive lichen planus, followed by nine clinical outbreaks of typical condylomas that consistently showed the presence of low‐risk HPV DNA only. These observations indicate that long‐lasting topical corticosteroid therapy occasionally may be associated with opportunistic reactivation of a latent high‐ and low‐risk mucosotrophic HPV type infection. The importance of clinical follow‐up is underlined.
Dermatofibrosarcoma protuberans: a clinicopathological study of 20 cases
Authors: Oliveira‐Soares R , Viana I , Vale E , Soares‐Almeida LM , Picoto A
Published: March 2003

Aim To review the clinical and histological data of 20 cases of dermatofibrosarcoma protuberans presenting at two dermatology centres in Lisbon from 1978 to 1998.

Patients and methods The 20 subjects comprised nine males and 11 females ranging in age from 25 to 79 years, with highest frequency of subjects in the 30–50 year olds. We reviewed the clinical features, histopathological aspects, including morphologic variants and immunohistochemical studies.

Results Median age at diagnosis was 51 years and the trunk was the most frequent location. The characteristic histologic storiform pattern was seen in all cases. Three subjects presented fibrosarcomatous areas, one with myoid differentiation and another with multinucleated giant cells. Immunohistochemical stains revealed CD34 expression in the 18 specimens tested, FXIIIa was negative, and these two antigens proved important for the differential diagnosis of this neoplasm. Local wide excision was performed in 13 cases and seven patients underwent Moh's micrographic surgery. Follow‐up ranged from 2 months to 17 years and three recurrences were recorded, two following classical surgery and one after Moh's surgery; there was no difference in the rate of local recurrence (15%) for the two kinds of treatment in our series.
The ‘common mole’ from the point of view of digital dermoscopy analysis: subjective vs. objective evaluation of easy pigmented skin lesions
Authors: Burroni M , Rubegni P , Dell’eva G , Santini S , Perotti R , Biagioli M , Taddeucci P , Andreassi L
Published: March 2003

Background The term ‘common mole’, often used to describe a subset of benign pigmented skin lesions, is traditionally defined on the basis of morpho‐chromatic features. In recent years, certain research groups have developed equipment and methods, such as digital dermoscopy analysis, that enable objective evaluation of pigmented skin lesions.

Objective In this study we use a digital dermoscopy analyser trained for the recognition of pigmented skin lesions to compare the subjective definition of ‘common’ and the mathematical concept of ‘close to the mean of measurements’.

Methods A subset (100) of digital images of flat pigmented lesions, obtained in daily practice, were classified by trained and non‐expert clinicians as common moles (60) or clear‐cut melanoma (40), and processed with a DB‐Mips analyser. The resulting parameters, validated by a classifier, were used to evaluate Hotelling's T2 multivariate distances from the mean.

Results Common’ moles could not be clearly defined in terms of closeness to the means of objectively evaluated parameters. Their diagnosis includes many other evaluations and clusters of variables.

Conclusion The clinical semantics of the term ‘common’ does not conform to any unambiguous mathematical definition.
Effects of Four Therapy Procedures on Communication in People with Profound Intellectual Disabilities
Authors: Lindsay William R. , Black Esther , Broxholme Sarah , Pitcaithly Deborah , Hornsby Nicola
Published: March 2003

A number of alternative therapies have recently been employed with people who have intellectual disabilities (IDs). The present study examines the effects of four frequently used therapies on the communication of people with profound ID. Communication was assessed using five measures of positive communication and five measures of negative communication. The therapies assessed were Snoezelen, active therapy, relaxation and aromatherapy/hand massage. There were eight participants in the present study and each received all four of the therapeutic procedures in a counterbalanced design. Treatment procedures were videotaped at sessions 5, 10, 15 and 20, and later scored for defined measures of communication. Both Snoezelen and relaxation increased the level of positive communication and had some effect on decreasing negative communication. However, active therapy and aromatherapy/hand massage had little or no effect on communication. The lack of a no‐treatment control is noted, especially in the light of trends seen at baseline. Considering the lack of generalization of therapeutic effects, the present results should be treated with caution.
Family Views of the Quality of Residential Supports
Authors: Walsh Patricia Noonan , Linehan Christine , Hillery John , Durkan Joe , Emerson Eric , Hatton Chris , Robertson Janet , Gregory Nicky , Kessissoglou Sophia , Hallam Angela , Knapp Martin , Jaerbrink Krister , Netten Ann
Published: March 2003

The quality and costs of residential supports provided to adults with intellectual disabilities (IDs) living in the UK and Ireland were investigated. The present paper describes findings related to relatives' level of contact with their family members and their appraisal of current residential supports. The relatives (n = 210) of adults living in campus settings and dispersed housing completed a questionnaire devised to determine their satisfaction with their family members' current residential supports and were invited to name both the features which they like and features which they wish to see improved. Relatives whose family members with IDs lived closer to the family home, who were younger and who took part in more daily activities in their current residence reported higher levels of contact. The respondents believed that current residential supports were better than those previously received. While relatives of residents in campus settings reported higher levels of family contact, satisfaction ratings were similar for relatives of residents in both campus settings and dispersed housing. Staff support, physical aspects of the residences and the standard of care provided were the aspects of residential supports which were most liked; preferred improvements included higher staff ratios and better day activities. Levels of family contact may be predicted by distance as well as by residents' characteristics and type of residence. A considerable overlap among groups of relatives as to ratings of satisfaction and the aspects of residences which they liked was apparent. Research strategies targeting informed appraisals of service quality on the part of both residents and their relatives are proposed.

Happy families are all alike; every unhappy family is unhappy in its own way.

– Leo Tolstoy, Anna Karenina, Part 1, Chapter 1
Home‐based Programmes for Parents with Intellectual Disabilities: Lessons from Practice
Authors: Llewellyn Gwynnyth , McConnell David , Russo Domenica , Mayes Rachel , Honey Anne
Published: March 2003

Background Increasing emphasis is being given to carrying out parent education programmes at home with widely reported benefits. The purpose of this paper is to draw lessons for practitioners from implementing a randomized trial of a home‐based parent education programme for parents with intellectual disabilities that addressed child health and safety issues.

Methods A thematic analysis of case notes and parent feedback from the 45 parent participants in this programme was conducted prior to two team sessions to discuss the findings.

Results Programme‐specific issues included format, content and presentation style. Themes relevant to home‐based programmes for parents with intellectual disabilities more generally included the suitability of the home as a learning environment; identifying parent priorities; parent readiness to learn; and parent control (or lack of) over the home environment. In addition, practice points for parent educators were identified.

Conclusions The home environment benefits both teaching and learning, however, as this paper highlights careful consideration needs to be given to each parent‐learner's home situation. We recommend that in future the processes involved in home‐based programmes be reported alongside programme outcomes.
Attitudes towards Sexuality, Sterilization and Parenting Rights of Persons with Intellectual Disabilities
Authors: Aunos M. , Feldman M. A.
Published: March 2003

Background and Methods  We review articles pertaining to attitudes towards sexuality, sterilization, procreation and parenting by people with intellectual disabilities. Most empirical studies were conducted after the appearance of the principles of normalization and role valorization in the 1970s.

Results  Across studies, special education teachers and university students appear to hold more positive attitudes towards sexuality and sexuality education programs than parents and service workers. People with intellectual disabilities have conservative attitudes towards sexual intercourse and homosexuality, but may be accepting intimate contact by familiar persons. Despite the ban on involuntary sterilization, it appears that many parents and teachers of persons with intellectual disabilities still support it as a form of contraception, especially for persons with severe intellectual disabilities. Likewise, attitudes towards parenting by persons with intellectual disabilities remain negative, and these attitudes may adversely affect the provision of competency‐enhancing supports and services for parents with intellectual disabilities and their children.

Conclusions  It is recommended that new studies should be undertaken, comparing attitudes across different groups involved with persons with intellectual disabilities and examining the impact of prejudicial attitudes on sexual expression and parenting by persons with intellectual disabilities.
Children of Mothers with Intellectual Disability: Stigma, Mother–Child Relationship and Self‐esteem
Authors: Perkins Tiffany S. , Holburn Steve , Deaux Kay , Flory Michael J. , Vietze Peter M.
Published: March 2003

Background  We investigated mother–child relationships and self‐esteem of typical children of mothers with intellectual disability.

Methods  Eighteen girls and 18 boys from various ethnic groups were administered questionnaires to assess: (a) attachment style; (b) caregiver style; (c) perception of maternal stigma; and (d) self‐esteem. The children were also asked to list the identities or roles that they play in life.

Results  Results suggested that: (a) the relationship between the child's perception of stigma and attachment to the mother is mediated by the warmth of the mother's caregiving style; and (b) if the child has an avoidant or anxious/ambivalent attachment to the mother, self‐esteem tends to be lower. Furthermore, multiple identities contribute to positive self‐esteem among these children.

Conclusions  Results are discussed in relation to the model presented and the consistency of the findings with attachment theory.
Umbilical artery velocimetry and non‐stress test in monitoring high‐risk pregnancies. A comparative longitudinal study
Authors: Malcus P. , van Beek E. , Maršál K.
Published: March 2003

In a longitudinal comparative study, umbilical artery velocimetry and the non‐stress test were used in parallel for surveillance of 153 hospitalized high‐risk pregnancies. The occurrence of abnormal flow velocity waveforms of the umbilical artery, with increased pulsatility index but maintained diastolic flow velocity, was an inconsistent finding and often related to pregnancy complications other than intrauterine growth retardation. Among patients with small‐for‐gestational age fetuses or pre‐eclampsia with a small‐for‐gestational age fetus, there was a significantly higher frequency of abnormal umbilical flow velocity waveforms. This was a consistent finding and was often associated with an absence of diastolic flow velocity and operative delivery for fetal distress. An abnormal non‐stress test had a significant relationship with intrauterine growth retardation, but a lower predictive capacity for this condition than the abnormal umbilical artery flow velocity waveform. In patients with small‐for‐gestational age fetuses and no other complication of pregnancy, umbilical artery velocimetry can be used as a reliable tool for identifying those fetuses that need intensified surveillance. In cases with additional complications, a combined use of blood velocimetry and the non‐stress test is advocated. Copyright © 1991 International Society of Ultrasound in Obstetrics and Gynecology
Fetal tachyarrhythmias: transplacental and direct treatment of the fetus—a report of 60 cases
Authors: Hansmann M. , Gembruch U. , Bald R. , Manz M. , Redel D. A.
Published: March 2003

From 1981 to 1990, 60 fetuses with tachyarrhythmia (21–39 weeks of gestation) were treated in utero. Of these, 54 were cases of supraventricular tachycardia, and six of atrial flutter. Non‐immune fetal hydrops was present in 21 cases with supraventricular tachycardia and in five cases with atrial flutter, a total of 26 cases.

Transplacental treatment by maternally administered antiarrhythmic drugs (digoxin only or in combination with verapamil) produced good results in non‐hydropic fetuses. In this group, all 34 fetuses survived. In fetuses with hydrops, 20 out of 26 survived.

In 13 fetuses of the 26 with hydrops, direct fetal therapy was performed in addition to the transplacental therapy when the tachyarrhythmia was refractory to transplacental treatment. During the 9 years of this study, a variety of direct treatment regimes have been used consisting of intraperitoneal and/or umbilical intravenous administrations of different drugs. Since 1988, umbilical vein punctures have shown that the transplacental passage of digoxin (and amiodarone) is hampered in the presence of hydrops, and direct treatment may he necessary in these cases. Amiodarone seems to he the drug of choice for direct therapy. It is highly effective in supraventricular tachycardia and atria1 flutter. The long elimination half‐time of amiodarone reduces the number of umbilical cord punctures needed to maintain the therapeutic drug level in the fetus. Copyright © 1991 International Society of Ultrasound in Obstetrics and Gynecology
Foramen ovale size in the normal and abnormal human fetal heart: an indicator of transatrial flow physiology
Authors: Feit L. R. , Copel J. A. , Kleinman C. S.
Published: March 2003

We used fetal echocardiography to measure the sizes of the foramen ovale and atrial septum in 80 human fetuses from 17.5 to 38 weeks gestation. Forty‐six fetuses had normal cardiac anatomy, 19 had left heart obstructive lesions, and 15 had right heart obstructive lesions. Pulsed and color flow Doppler studies were incorporated when available. We found that normal fetuses had a foramen ovale/atrial septum size ratio of 0.33 ± 0.04 (mean ± SD). In all (52%) fetuses in which it was available, pulsed Doppler study revealed bidirectional, but predominantly right‐to‐left, flow through the foramen ovale. Fetuses with left heart obstructive lesions had a foramen ovale/atrial septum size ratio smaller than normal (0.28 ± 0.05; p < 0.001). Seven of nine fetuses with left heart obstructive lesions who had color Doppler studies demonstrated reversal of the normal flow pattern, exhibiting unidirectional left‐to‐right transforamenal flow. Those with right heart obstructive lesions had a larger than normal foramen ovale/atrial septum size ratio (0.47 ± 0.04; p < 0.001). Nine fetuses with right heart obstructive lesions had color Doppler studies which demonstrated almost exclusive right‐to‐left transforamenal flow. We conclude that foramen ovale/atrial septum size ratio and Doppler interrogation of transatrial flow are helpful adjuncts in determining the presence of congenital heart disease in utero. In addition, early detection of abnormal foramen ovale/atrial septum size ratio may predict whether left or right ventricular development will be impaired throughout gestation, even before gross disparity of ventricular size is apparent. Copyright © 1991 International Society of Ultrasound in Obstetrics and Gynecology
Continuous maternal hyperoxygenation in the treatment of early fetal growth retardation
Authors: Ribbert L. S. M. , van Lingen R. A. , Visser G. H. A.
Published: March 2003

Continuous maternal hyperoxygenation treatment (2.5 l/min by means of a nasopharyngeal cannula) was given in four patients presenting with intrauterine growth‐retarded (IUGR) fetuses and decelerative fetal heart rate (FHR) patterns at 27–28 weeks of gestation. The effect of maternal hyperoxia was studied longitudinally. Neither the incidence of generalized fetal movements (FGM%) nor the pulsatility index of the internal carotid artery increased under hyperoxia. In fact, both variables decreased progressively. FHR variation was abnormal prior to the start of hyperoxygenation and showed a slight but transient increase. On average, maternal hyperoxygenation ‘resulted’ in a prolongation of the duration of pregnancy of 9 days. The neonatal mortality was similar in the study group as compared to a control group of IUGR infants. However, hypoglycemia, thrombocytopenia and disseminated intravascular coagulation at birth were found more frequently in the study group. Conversely, blood gas abnormalities were less frequent in the fetuses of mothers that were treated with oxygen. We conclude that positive effects of oxygen therapy in IUGR fetuses remain uncertain and that detrimental effects due to prolongation of intrauterine malnutrition have not as yet been sufficiently excluded. Copyright © 1991 International Society of Ultrasound in Obstetrics and Gynecology
Early sonographic diagnosis of intrapericardial teratoma
Authors: Catanzarite V. , Mehalek K. , Maida C. , Mendoza A.
Published: March 2003

An intrapericardial teratoma associated with a large pleural effusion and elevated aortic outflow velocity was diagnosed at 23.6 weeks' gestation. Less than 4 weeks earlier, an obstetric scan, including a four‐chamber heart view, had been normal. In less than a week following diagnosis, fetal hydrops and bradycardia developed. ‘Heroic’ pericardiocentesis was declined by the patient, and the fetus died. This case demonstrates the potential for rapid growth in pericardial teratomas, and illustrates the possibility of anticipating fetal compromise from tumor mass effect, when intracardiac Doppler studies are abnormal. Implications for management of these rare tumors, and specifically for the hypothetical role of fetal surgery, are discussed. Copyright © 1994 International Society of Ultrasound in Obstetrics and Gynecology
Routine prenatal ultrasound screening for fetal abnormalities: 22 years' experience
Authors: Carrera J. M. , Torrents M. , Mortera C. , Cusí V. , Muñoz A.
Published: March 2003

We describe the results of routine obstetric ultrasound present, several trials (e.g. the Euro Fetus–European examination over a period of 22 years (1970–91) at the Institut Universitari Dexeus, Barcelona, Spain.

A total of 1006 malformed fetuses or neonates were identified at abortion or delivery. The prevalence of fetal abnormalities was 3.03%. Fetal anomalies were diagnosed antenatally in 788 (78.33%) cases. Early prenatal diagnosis (before 22 weeks of gestation) was made in 598 (59.44%) cases. The detection of malformed fetuses increased from 19.75% in the first phase of the study (1970–74) to 96.33% in the last phase (1990–91). The specificity of the method was over 99%, throughout the study period. The most frequently diagnosed anomalies were urinary tract anomalies (22.86%) of cases), followed by head and neck anomalies (18.68%), musculoskeletal anomalies (8.64%), heart anomalies (7.35%) and gastrointestinal anomalies (7.35%). The earliest diagnosed malformations were those of the thoracoabdominal wall (81.08%), followed by defects of the urinary tract (70.86%) and of the diaphragm (70.83%). Copyright © 1995 International Society of Ultrasound in Obstetrics and Gynecology