bluuebunny/crossref_metadata_2025 huggingface dataset:
CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy): a neurovascular disease diagnosed by ultrastructural examination of the skin
Authors: Kanitakis Jean , Thobois Stéphane , Claudy Alain , Broussolle Emmanuel
Published: March 2003

Background: CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy) is a recently recognized neurovascular disease due to mutations of the Notch3 gene, manifesting with strokes or stroke‐like episodes, major psychiatric symptoms and dementia. The diagnosis can be confirmed either by molecular analysis or by ultrastructural examination of the brain or more simply the skin.

Methods:  The skin of a patient with a suspected diagnosis of CADASIL was studied by electron microscopy.

Results: Characteristic granular osmiophilic material within the basement membrane surrounding pericytes and smooth muscle cells of small and medium‐sized vessels of the skin were found, confirming the diagnosis of CADASIL.

Conclusions: CADASIL is an additional example of a neurologic disease that can be diagnosed thanks to electron microscopic examination of the skin. Dermatopathologists should be aware of these ultrastructural findings, all the more so since the disease could be more common than originally thought.
CD4+ T lymphocytopenia with disseminated HPV
Authors: Stetson Cloyce L. , Rapini Ronald P. , Tyring Stephen K. , Kimbrough Robert C.
Published: March 2003

Background:  There have been several reports of HIV‐negative patients with chronic idiopathic CD4+ T lymphocytopenia, the diagnostic criteria for which are: depressed numbers of circulating T lymphocytes (less than 300/µl or less than 20%) on more than one occasion; no laboratory evidence of HIV‐1 or HIV‐2 infection; and the absence of any defined immunodeficiency or therapy associated with depressed levels of CD4+ T lymphocytes.

Methods: We report a patient with disseminated human papillomavirus infection associated with idiopathic CD4+ T‐cell lymphocytopenia. A 50‐year‐old woman presented to the dermatology clinic with a 10‐year history of widespread verrucae involving the skin and the cervix.

Results: Biopsy from the arm revealed a common wart. PCR analysis performed from the paraffin‐embedded block was strongly positive for HPV type 2. Other HPV types (including EV‐associated HPV 5, 8, 14, 15, 17) were not found. Further laboratory work up revealed T‐cell lymphocytopenia, with an absolute CD4 count of 21. HIV tests were repeatedly negative. She was treated with interferon A 8 million units SQ three times per week with partial improvement. The patient underwent a hysterectomy for cervical dysplasia and a vulvectomy for vulvar intraepithelial neoplasia. She developed small‐cell lung carcinoma and died.

Conclusions: The diagnosis of idiopathic CD4+ T‐cell lymphocytopenia should be considered in any patient with widespread viral, fungal, or mycobacterial infection whose HIV test is negative, and appropriate evaluation of the absolute CD4+ counts should be performed.
Histomorphologic and immunophenotypic analysis of fibrofolliculomas and trichodiscomas in Birt–Hogg–Dube syndrome and sporadic disease
Authors: Collins Gary L. , Somach Stephen , Morgan Michael B.
Published: March 2003

Background:  Fibrofolliculomas and trichodiscomas are benign dermal neoplasms that likely derive from the mantle of the hair follicle and can occur sporadically or in association with Birt–Hogg–Dube syndrome (BHDS). Little is known about the pathogenesis and immunophenotypic properties of these entities.

Methods:  We investigated the histomorphologic and immnophenotypic properties of 15 fibrofolliculomas and trichodiscomas in two patients with BHDS and eight with sporadic disease.

Results:  We found the following: (1) fibrofolliculomas and trichodiscomas occurring in the setting of BHDS show microscopically contiguous histomorphologic features as compared to those occurring sporadically; (2) the immunophenotypic characteristics of syndromic‐associated and sporadic types are identical and consist of (3) perifollicular vimentin (+), CD34 (+) and Factor XIII (–) spindle cells.

Conclusions:  Despite subtle histomorphologic differences, trichodiscomas and fibrofolliculomas are immunophenotypically similar, and are thus likely derived from a similar histogenic precursor. Given the previously reported CD34 (+) immunophenotype of the hair mantle, our findings would support an origin of these lesions from the mantle of the hair follicle. The proliferation of CD34 (+) spindle cells seen in conjunction with these lesions should not be confused with other CD34 (+) dermal entities.
Keratinocyte dysplasia: an usual finding after transplantation or chemotherapy
Authors: Castaño Esther , Rodríguez‐Peralto Jose Luis , López‐Ríos Fernando , Gómez Carlos , Zimmermann Marta , Iglesias Díez Luis
Published: March 2003

Background:  Keratinocyte dysplasia is a histologic abnormality that has rarely been described in the first weeks after chemotherapy or transplantation. The purpose of this study was to determine the prevalence of early keratinocyte dysplasia after chemotherapy or transplant and to analyze the relationship between dysplasia and chemotherapeutic agents, transplantation or development of acute graft vs. host disease (GvHD).

Methods:  A computer search for transplant patients was performed in the Departments of Hematology, Oncology and Surgery. Only patients with a skin biopsy taken within the first days of transplantation or chemotherapeutic treatment were included in the analysis.

Results:  Forty‐four patients were included, of these 19 were women and 25 were men. Thirty‐four (77.3%) of 44 biopsies showed keratinocyte dysplasia. This dysplasia was severe in 18 cases (40.9%). Cyclophosphamide was more likely to be associated with severe keratinocyte maturation disturbances (OR = 5.51) (p < 0.01) whereas cytarabine was associated with a lower risk (OR = 0.19) (p < 0.05).

Conclusions:  Severe keratinocyte dysplasia is a usual histologic finding in patients who have received chemotherapy and/or transplantation (40.9%). Cyclophosphamide is the main chemotherapeutic agent significantly associated with a higher risk of severe dysplasia (OR = 5.51). Causes other than GvHD or preconditioning treatment may be involved in the keratinocyte dysplasia, as it can be also found in transplanted patients who have not received chemotherapy and/or have not developed GvHD.
Mucocutaneous pigmented macule as a result of zinc deposition
Authors: Greenberg J. E. , Lynn M. , Kirsner R. S. , Elgart G. W. , Hanly A. J.
Published: March 2003

Background:  Mucocutaneous depositions of various metals such as silver, lead, gold, arsenic, mercury, iron, and bismuth have been previously published. Heavy metal deposition typically occurs in the setting of either prolonged topical application to intact skin, topical application to eroded or ulcerated skin, as a result of either parenteral administration, or due to penetrating traumatic exposure.

Method:  We report a unique case of mucocutaneous pigmentation occurring in a snow skier after topical application of a zinc‐containing sunblock. Formalin‐fixed paraffin‐embedded tissue was utilized for electron microscopy.

Result:  Backscatter electron imaging and energy dispersive spectroscopy revealed that the dominant metal present was zinc.

Conclusions:  Mucocutaneous deposition of metals is enhanced by damage to the surface epithelium. Metal‐containing topical agents, although commonly used, may rarely result in a permanent pigmentary alteration. We believe similar cases of mucocutaneous deposition of zinc exist; however, as these may be currently misdiagnosed as amalgam tattoos, the true incidence of this disorder is presently undefined.
Medicaid Enrollment among Elderly Medicare Beneficiaries: Individual Determinants, Effects of State Policy, and Impact on Service Use
Authors: Pezzin Liliana E. , Kasper Judith D.
Published: March 2003

Objective. To better understand factors associated with Medicaid enrollment among low‐income, community‐dwelling elderly persons and to examine the effect of Medicaid enrollment on the use of health care services by elderly persons, taking into account selection in program participation.

Data Sources. 1996 Medicare Current Beneficiary Survey (MCBS) Access to Care and Cost and Use files.

Methods. Individual‐level predictions of the probability of dual enrollment are obtained from equations that estimate jointly the residential status of Medicare beneficiaries (community versus institution) and the probability of Medicaid enrollment among community‐dwelling eligible beneficiaries. Predicted values are then substituted into the service use equations, which are estimated via two‐part models.

Principal Findings. Less than half of all community‐dwelling elderly persons with incomes at or below 100 percent of the Federal Poverty Level (FPL) were enrolled in Medicaid in 1996. Once selective enrollment was accounted for, there was limited evidence of a dual enrollment effect on service use. Although there were no effects of state Medicaid policy variables on the probability that beneficiaries lived in the community (as opposed to nursing homes), the effects of state's Medicaid generosity in home and community‐based services had a sizeable and statistically significant effect on influencing the likelihood that eligible elderly persons enrolled in Medicaid.

Conclusions. Our results provide compelling evidence that Medicaid participation can be influenced by state policy. The observation that „policy matters” provides new insights into how existing programs might reach a larger proportion of potentially eligible beneficiaries.
The Determinants of HMOs’ Contracting with Hospitals for Bypass Surgery
Authors: Gaskin Darrell J. , Escarce José J. , Schulman Kevin , Hadley Jack
Published: March 2003

Objective. Selective contracting with health care providers is one of the mechanisms HMOs (Health Maintenance Organizations) use to lower health care costs for their enrollees. However, are HMOs compromising quality to lower costs? To address this and other questions we identify factors that influence HMOs’ selective contracting for coronary artery bypass surgery (CABG).

Study Design. Using a logistic regression analysis, we estimated the effects of hospitals’ quality, costliness, and geographic convenience on HMOs’ decision to contract with a hospital for CABG services. We also estimated the impact of HMO characteristics and market characteristics on HMOs’ contracting decision.

Data Sources. A 1997 survey of a nationally representative sample of 50 HMOs that could have potentially contracted with 447 hospitals.

Principal Findings. About 44 percent of the HMO‐hospital pairs had a contract. We found that the probability of an HMO contracting with a hospital increased as hospital quality increased and decreased as distance increased. Hospital costliness had a negative but borderline significant (0.10<p<0.05) effect on the probability of a contract across all types of HMOs. However, this effect was much larger for IPA (Independent Practice Association)‐model HMOs than for either group/staff or network HMOs. An increase in HMO competition increased the probability of a contract while an increase in hospital competition decreased the probability of a contract. HMO penetration did not affect the probability of contracting. HMO characteristics also had significant effects on contracting decisions.

Conclusions. The results suggest that HMOs value quality, geographic convenience, and costliness, and that the importance of quality and costliness vary with HMO. Greater HMO competition encourages broader hospital networks whereas greater hospital competition leads to more restrictive networks.
Understanding Coordination of Care from the Consumer's Perspective in a Regional Health System
Authors: Harrison Alexandra , Verhoef Marja
Published: March 2003

Objective To understand and develop a model about the meaning of coordination to consumers who experienced a transition from acute care to home care.

Study Design. A qualitative, exploratory study using Grounded Theory.

Data Sources/Analysis. Thirty‐three consumers in the Calgary Regional Health Authority who had experienced the transition from an acute care hospital back into the community with home care support were interviewed. They were asked to describe their transition experience and what aspects of coordination were important to them. Interviews were recorded, transcribed, and analyzed using constant comparison. The coding and retrieval of information was facilitated by the computer software program Nud*ist.

Principal Findings. The resulting model has four components: (1) the meaning of coordination to consumers; (2) aspects of health care system support that are important for coordination; (3) elements that prepared consumers to return home; and (4) the components of a successful transition experience. Consumers appeared to play a crucial role in spanning organizational boundaries by participating in the coordination of their own care.

Conclusions. Consumers must be included in health care decisions as recipients of services and major players in the transition processes related to their care. Health care providers need to ensure that consumers are prepared to carry out their coordination role and managers need to foster a culture that values the consumer “voice” in organizational processes.
Managed Behavioral Health Care: An Instrument to Characterize Critical Elements of Public Sector Programs
Authors: Susan Ridgely M. , Giard Julienne , Shern David , Mulkern Virginia , Audrey Burnam M.
Published: March 2003

Objective. To develop an instrument to characterize public sector managed behavioral health care arrangements to capture key differences between managed and „unmanaged” care and among managed care arrangements.

Study Design. The instrument was developed by a multi‐institutional group of collaborators with participation of an expert panel. Included are six domains predicted to have an impact on access, service utilization, costs, and quality. The domains are: characteristics of the managed care plan, enrolled population, benefit design, payment and risk arrangements, composition of provider networks, and accountability. Data are collected at three levels: managed care organization, subcontractor, and network of service providers.

Data Collection Methods. Data are collected through contract abstraction and key informant interviews. A multilevel coding scheme is used to organize the data into a matrix along key domains, which is then reviewed and verified by the key informants.

Principal Findings This instrument can usefully differentiate between and among Medicaid fee‐for‐service programs and Medicaid managed care plans along key domains of interest. Beyond documenting basic features of the plans and providing contextual information, these data will support the refinement and testing of hypotheses about the impact of public sector managed care on access, quality, costs, and outcomes of care.

Conclusions. If managed behavioral health care research is to advance beyond simple case study comparisons, a well‐conceptualized set of instruments is necessary.
Development and evaluation of a stand‐alone web‐based CAL program
Authors: Perryer G. , Walmsley A. D. , Barclay C. W. , Shaw L. , Smith A. J.
Published: March 2003

The use of web browser technology allows the construction of computer‐aided learning programs which will have a familiar interface to dentists. This paper reports on the evaluation of a web‐based CAL program which covered the topic of tooth wear with an emphasis on the rôle of erosion in its aetiology. Evaluation of the software was made by 50 dentists contacted by an electronic mailing list. They completed a questionnaire which evaluated aspects of the functionality of the program educational objectives. The average age of the dentists was 39 years (range 24–70) and 82% were male. The average time of use was 1 h 10 min (range 15 min to 3 h). A significant change in their knowledge of tooth wear before and after using the program was recorded. Both the use of patient cases and leaflets scored highly with other aspects such as treatment planning and patient advice receiving high marks. All dentists commented that they had gained greater knowledge with particular reference to treatment and advice of tooth wear problems. 34 dentists (68%) found the web interface easy to use. Negative comments were mainly attributed to the quality of the pictures (4/50) which were corrected in the final release version. Respondents were asked to compare the program to other educational media on the same subject. 80% rated this CAL program as better than video, and 14% claimed it was as good as video. 84% rated it better than books, and 14% said it was as good as books. 98% preferred it to audio tapes and 86% preferred it to journals. The average amount that they would pay for a CAL package such as “tooth wear” would be 42 ECU. It is concluded that web browser software is a suitable medium for the use of a CAL program and this is reflected in its ease of use by dentists.
Educating overseas students: just another responsibility or a chance to grow for faculty?
Authors: Morio Ikuko , Kawaguchi Yoko , Suda Hideaki , Eto Kazuhiro
Published: March 2003

With a gradual decrease of the university‐entering young population, Japanese universities face more and more competition for survival. It is up to each university’s efforts to be distinctive and competitive to flourish toward the 21st century. At Tokyo Medical and Dental University, the Faculty of Medicine and the Faculty of Dentistry will be reorganised in April 2000 as a graduate‐school‐centred university, where faculty will face increased demands in research activities while maintaining or even improving undergraduate education. On the other hand, our university has a 20‐year history of accepting overseas students mainly from Asia, and ranks first among Japanese national universities in terms of the number of overseas students studying health sciences. Do our faculty regard teaching overseas students as just another responsibility to be fulfilled or as a unique opportunity to grow as a university faculty? In October 1998, a questionnaire was conducted to discover the faculty attitude towards education of overseas students. Out of 211 dental faculty members, 164 responded to the questionnaire. The results indicated that about 90% of the respondents were interested in teaching overseas students and that those who had already served as a supervisor or a tutor found the experience very meaningful. The results also indicated that they encountered problems deriving from language and culture. It was suggested that our university should redefine overseas student education as an important part of our mission by establishing a more comprehensive system for accepting and teaching overseas students.
Assessment of a preclinical training system with indirect vision for dental education
Authors: Díaz María J. , Sánchez Esperanza , Hidalgo Juan J. , Vega José M. , Yanguas Manuel
Published: March 2003

The aim of the present study is the evaluation of a pre‐clinical training system using indirect vision for dental education purposes. The study population was undergraduate dental students between the ages of 18–20, at the beginning of their first dental course. From a total of 105 students, those presenting some condition that would have had influence on their manual skills and those who for some circumstance left the study after beginning it were excluded, resulting in 69 students (51 female and 18 male) taking part in the study. The study design involved two sessions separated by a 7‐day interval in which the students used a reflection box to develop psychomotor skills in mirror use. In every session they performed an initial evaluation test, some training exercises and a final evaluation test. The evaluation tests and the training exercises consisted in following a curved, straight or convoluted shape in the reflection box. Four groups of students were used who trained in different ways using curved or straight lines. Evaluation of the tests was based on the number of errors made and the time to completion. All students showed an improvement in their performance of following curved and straight lines with indirect vision after this form of training. The results after the first training session were statistically significant, while the further improvement was not significant after the 2nd training session. Those students who trained first with curved lines and later with straight lines produced more errors than the other groups. Female students performed better than male students in this assessment.
Undergraduate education in endodontology at two European dental schools
Authors: Petersson Kerstin , Olsson Helena , Söderström Christina , Fouilloux Isabelle , Jegat Nadège , Lévy Gérard
Published: March 2003

The aim of this study was to compare the courses in endodontics and to assess the treatment quality in the student clinics in two dental schools, in Malmö, Sweden and Paris, France. A further aim was to improve the curriculum development in Paris 5 and Malmö by testing student exchange programmes. The comparison was based on the guidelines for undergraduate education set up by the European Society of Endodontology (ESE) [Int. Endod. J. 25 (1992) 169] and on the criteria formulated by Qualtrough and Dummer [Int. Endod. J. 30 (1997) 234]. The latter criteria covered the following aspects: educational methods, the timing of endodontic teaching, pre‐clinical practical exercises, student assessment, recommended literature, clinical/practical procedures, the education of the staff and number of students per teacher. The quality guidelines for endodontic treatment set up by the ESE [Int. Endod. J. 27 (1994) 115] were used for the assessment of the quality of the treatment. The following aspects were covered: history, diagnosis and treatment planning, records, infection control, root‐canal treatment, assessment of endodontic treatment. The undergraduate education in endodontics was fundamentally similar in Paris 5 and Malmö. The main differences observed were related to:

Educational methods: In Malmö, problem‐based learning and in Paris 5, traditional.

Assessment of student performance. In Malmö, self‐assessment and in Paris 5, credits for clinical/practical procedures.

Clinical/practical procedures relating to infection control. Aseptic treatment regimens were more meticulously performed in Malmö than in Paris 5.

Assessment (follow‐up) of all endodontic treatments was a routine only in Malmö.
Habitat size and number in multi‐habitat landscapes: a model approach based on species‐area curves
Authors: Tjørve Even
Published: March 2003

This paper discusses species diversity in simple multi‐habitat environments. Its main purpose is to present simple mathematical and graphical models on how landscape patterns affect species numbers. The idea is to build models of species diversity in multi‐habitat landscapes by combining species‐area curves for different habitats. Predictions are made about how variables such as species richness and species overlap between habitats influence the proportion of the total landscape each habitat should constitute, and how many habitats it should be divided into in order to be able to sustain the maximal number of species. Habitat size and numbers are the only factors discussed here, not habitat spatial patterns. Among the predictions are: 1) where there are differences in species diversity between habitats, optimal landscape patterns contain larger proportions of species rich habitats. 2) Species overlap between habitats shifts the optimum further towards larger proportions of species rich habitat types. 3) Species overlap also shifts the optimum towards fewer habitat types. 4) Species diversity in landscapes with large species overlap is more resistant to changes in landscape (or reserve) size. This type of model approach can produce theories useful to nature and landscape management in general, and the design of nature reserves and national parks in particular.
Is the heart of Fennoscandian rodent cycle still beating? A 14‐year study of small mammals and Tengmalm's owls in northern Norway
Authors: Strann Karl‐Birger , Yoccoz Nigel G. , Ims Rolf A.
Published: March 2003

Analyses of spatial and temporal patterns of the small mammal cycle in Fennoscandia have led to two main conclusions: a south‐north geographical gradient in the strength of density dependence and period length, and a change in temporal dynamics with less clear periodicity and lower amplitude of fluctuations in recent years. Fourteen years (1985–1998) of small mammals trapping and Tengmalm's owls monitoring data provided clear evidence for 3‐yr cycles of two vole species in inner Troms, north Norway, at the same latitude and only 80 km from Kilpisjärvi, north Finland, where 4–5‐yr cycles have been the norm until the end of 1980s. The response of Tengmalm's owls to variation in small rodents abundance was not delayed, as was observed in central Finland. Common shrews appeared to have more stable dynamics. The features of the cycle, and a reassessment of the previously described patterns, show that the dynamics may be different over short distances. Furthermore, snow cover and habitat fragmentation should explicitly be taken into account in any understanding of the Fennoscandian gradient, and surrogate variables such as latitude should be avoided. We stress the need for a more extensive coverage of spatial and temporal patterns in cyclicity with respect to assumed ecological causes.
Host population density and body mass as determinants of species richness in parasite communities: comparative analyses of directly transmitted nematodes of mammals
Authors: Arneberg Per
Published: March 2003

Epidemiological theory predicts positive correlations between host population density or body mass and species richness among parasite communities. Here I test these predictions by a comparative study of communities of directly transmitted mammalian parasites, gastrointestinal strongylid nematodes. I use data from 45 species of mammals, representing examination of 17 200 individual hosts. The variable studied was the average number of gastrointestinal strongylid nematode species per host population, and three different methods were used to obtain estimates of parasite species richness that are unbiased by number of host individuals examined. Analyses were done using the phylogenetically independent contrast method. Host population density and parasite species richness were strongly positively correlated when the effects of host body weight had been controlled for. Controlling for other variables did not change this, and the relationship was found regardless of method used to correct for uneven sampling effort among host species. A positive relationship between parasite species richness and host body weight was also found, but the effect of host densities had to be controlled for to see this. These relationships between host traits and species richness of directly transmitted parasites are stronger than patterns found using data on indirectly transmitted mammalian parasites, and suggests that links between host traits and parasite species richness are stronger than previously suggested. The results are consistent with parasite species richness being positively linked to pathogen transmission rates and reductions in transmission rates possibly increasing extinction probabilities in parasite populations. The results also suggest that parasites may exert a cost of increases in rate of population energy usage, and thus show that pathogens may be important in generating independence between body mass and rate of population energy usage among host species.
Gap colonization in the Patagonian semidesert: seed bank and diaspore morphology
Authors: Fernández Roberto J. , Golluscio Rodolfo A. , Bisigato Alejandro J. , Soriano Alberto
Published: March 2003

We report work on a cold, windy South American steppe dominated by tussock grasses and shrubs of small stature that, together, cover only half of the soil surface. Our objective was to find out why seedlings and juveniles of these dominant species are generally absent from the bare or poorly‐populated spots (gaps) that exist between established individuals. We hypothesized that matrix‐forming species fail to colonize gaps because of a lack of properly‐placed seeds, contained in diaspores which because of their morphology are blown away from gaps that otherwise would constitute safe sites for recruitment. We evaluated diaspore size and wing loading (weight:area ratio) for all common species in the community, collected seed‐bank samples in different occasions and microsites, and performed detailed field observations for one gap‐dwelling species during several years. We found that: 1) Seeds of the dominant, matrix‐forming species are uncommon in the soil bank of the center of the gaps between established vegetation. 2) Seeds of the dominant species are more abundant towards the edges of the gaps than at their center. 3) Diaspores of those species present in the seed bank of the gaps are smaller than diaspores of absent species; contrary to expectations, not all gap‐dwelling species had larger diaspore wing loading than non‐gap species. 4) Seeds and adult densities of the most common gap species (the annual Camissonia dentata), were correlated between them and across subsequent years. We conclude that it is not an overall shortage of seeds that precludes the dominant species from becoming established in the gaps, but rather the seeds’ uneven spatial distribution. We further argue that gaps would be suitable sites for recruitment, but large diaspore size makes seeds of the dominant species to be blown away by the strong westerly winds.
Different responses to cold weather in two pied flycatcher populations
Authors: Eeva T. , Lehikoinen E. , Rönkä M. , Lummaa V. , Currie D.
Published: March 2003

We compared how breeding parameters differ according to prevailing weather conditions between a marginal, subarctic (69°N) and temperate (61°N) population of the pied flycatcherFicedula hypoleuca,a small migratory insectivorous passerine. We predicted that the effects of weather on breeding performance (clutch size, hatching success, nestling growth, fledging success) would be greater at northern latitudes, where the weather conditions are more extreme and unpredictable. We found that the breeding parameters, except clutch size, were not, however, inferior in the north. Northern birds, unlike the southern ones, responded to colder conditions by laying smaller clutches and maintaining a larger energy reserve (indicated by higher female body mass and higher levels of subcutaneous fat). If a cold spell occurred during the nestling period, southern flycatchers had 5–10% lower fledging success than the northern ones. Our results indicate that in the north, the breeding individuals coped with cold and variable weather better than the individuals in the southern population. This could be adaptive, because at high latitudes there is a higher probability of cold weather at the time of breeding.
Splenectomy in patients with malignant non‐Hodgkin's lymphoma
Authors: Xiros Nikolaos , Economopoulos Theofanis , Christodoulidis Charalambos , Dervenoulas John , Papageorgiou Efstathios , Mellou Sophia , Styloyiannis Sotirios , Tsirigotis Panagiotis , Raptis Sotirios A.
Published: March 2003

Abstract: Splenectomy in patients with non‐Hodgkin's lymphoma (NHL) is performed for either diagnostic or therapeutic reasons. We report on a series of 29 patients with NHL and splenomegaly who underwent splenectomy during the years 1979–1998 in our hospital. According to the indication for splenectomy our patients were categorized in three groups. Group A: In 20 patients splenectomy was performed for diagnostic reasons. Group B: Three patients were splenectomized for autoimmune haemolytic anaemia (AIHA). Group C: Six patients underwent splenectomy because of hypersplenism. A definitive histopathological diagnosis of NHL was obtained in all patients of group A. Hypersplenism and AIHA were resolved in all patients after splenectomy. One (3.5%) patient died postoperatively because of septicemia complicated by disseminated intravascular coagulation. Six postoperative complications were observed in 4 (14%) patients. Splenectomy, with an acceptable surgical risk, has the potential to establish the diagnosis of NHL in patients with splenomegaly without lymphadenopathy and negative bone marrow findings. Moreover, splenectomy has the capacity to modify the disease course in patients with NHL complicated by AIHA or hypersplenism.
β‐Thalassaemia intermedia in Lebanon
Authors: Qatanani M. , Taher A. , Koussa S. , Naaman R. , Fisher C. , Rugless M. , Old J. , Zahed L.
Published: March 2003

Abstract: Approximately one third of thalassaemia patients onrecord in Lebanon have thalassaemia intermedia. We haveanalysed three factors in a panel of 73 patients with this less severeform of the disease in our population: mild β‐globin gene mutations, deletions in the α‐globin gene and the presence of a polymorphismfor the enzyme XmnI in the Gγ‐promoter region. The results show that the most important contributing factor is the β‐genotype:68% of patients have a mild β+ mutation (IVSI‐6, cd29,−88 or −87), while 26% of patients are positive for the XmnI polymorphism associated with increased production ofHbF, which showed strong linkage to particular mutations(IVSII‐1, cd8 and cd30). However, the genotype–phenotypecorrelation is difficult, because many patients were initially misdiagnosed as thalassaemia major and were started early on regular blood transfusions, which was stopped later on. This illustrates well the importance of an early accurate diagnosis of thalassaemia intermedia for appropriate clinical management.
A whole‐blood flow cytometric assay for leukocyte CD11b expression using fluorescence signal triggering
Authors: Li Nailin , Halldén Gunilla , Hjemdah Paul
Published: March 2003

Abstract: A flow cytometric assay for measurements of leukocyte CD11b expression in whole blood has been developed and evaluated. The method is based on triggering of the flow cytometer by a fluorescent pan leukocyte marker, RPE‐CD45. This enabled flow cytometric analysis in whole blood, and avoidance of in vitro artefacts related to cell purification and hemolysis. Our methodological evaluation suggested the following routine procedure: sampling with sodium citrate as the anticoagulant, sample incubation at 22°C, and mild sample fixation with 0.5% formaldehyde saline. The latter provided good sample stability during 24 h. Moreover, the assay provided good assay reproducibility, low labelling antibody consumption, and minimal sample manipulation (<30 min) and acquisition time demands. The assay seems to reflect the CD11b expression of circulating leukocytes, and is also suitable for studies of agonist stimulated CD11b expression in leukocyte subpopulations in vitro. When full CD11b responsiveness to agonist stimulation is desired, samples should be incubated at 37°C, but this also elevated CD11b expression in unstimulated samples. The present whole‐blood technique is thus suitable for analyses of CD11b expression for both research and clinical routine laboratory use. The assay can easily be modified for measurements of other leukocyte antigens by use of other specific fluorescent antibodies.
Megakaryocyte c‐Mpl expression inchronic myeloproliferative disorders and the myelodysplastic syndrome: immunoperoxidase staining patterns and clinical correlates
Authors: Yoon Soo‐Young , Li Chin‐Yang , Tefferi Ayalew
Published: March 2003

Abstract: The objectives of this study were to expand on recent observations that have suggested decreased thrombopoietin receptor (c‐Mpl) expression in megakaryocytes of patients with polycythemia vera (PV) and agnogenic myeloid metaplasia (AMM). We applied an immunoperoxidase method with anti‐c‐Mpl antibody to 55 bone marrow sections from previously untreated patients with chronic myeloproliferative disorder (CMPD) or myelodysplastic syndrome (MDS). These included 8 patients with PV, 15 with AMM, 9 with essential thrombocythemia, 5 with chronic myelocytic leukemia, 9 with the 5q− syndrome and 9 with MDS with fibrosis. The findings were compared with those in four patients with reactive erythrocytosis (RE), six with immune thrombocytopenic purpura (ITP) and five normal controls. Staining intensity (SI) was moderate to strong both in normal controls and in patients with RE or ITP. In contrast, SI was weak in variable proportions of the megakaryocytes in every one of the aforementioned clonal myeloid disorders. The staining pattern (SP) was relatively uniform in MDS and heterogeneous in CMPD. Neither SI nor SP was significantly correlated with certain clinical or laboratory parameters. We concluded that altered megakaryocyte c‐Mpl expression may be a nonspecific phenomenon in various subtypes of both CMPD and MDS. However, the characteristic staining patterns may complementthe morphological distinction between clonal and reactivemyeloproliferation.
Acute promyelocytic leukemia and pregnancy
Authors: Giagounidis A. A. N. , Beckmann M. W. , Giagounidis A. S. , Aivado M. , Emde T. , Germing U. , Riehs T. , Heyll A. , Aul C.
Published: March 2003

Abstract: In acute promyelocytic leukemia (APL), the use of all‐trans‐retinoic acid (ATRA) as a differentiating agent induces complete remission in a high percentage of patients. In pregnancy, however, this drug bears the risk of severe teratogenicity to the child. We report the case of a 23‐yr‐old woman at 21 weeks' gestation suffering from APL. She was treated with ATRA (45 mg/m2) for 40 d and two courses of standard chemotherapy. The mother achieved complete remission within 22 d of treatment. Fetal development was normal, and a healthy premature girl was born in the 35th week of pregnancy. In a review of the literature we have identified 14 cases of APL in pregnancy treated with ATRA alone or in combination with chemotherapy. ATRA has been used as early as in the 3rd week of gestation and in no case have malformations or other teratogenic effects occurred. Side‐effects, however, ranged from fetal cardiac arrythmias to induction of labour. Although known to exhibit severe teratogenic effects during the first trimester of pregnancy, ATRA seems to be reasonably safe during the second and third trimesters in the treatment of APL. However, careful obstetric follow‐up is mandatory regarding fetal cardiac complications.
A single institutional experience with 43 pregnancies in essential thrombocythemia
Authors: Wright Curtis A. , Tefferi Ayalew
Published: March 2003

Abstract: Objectives: We describe the periconception circumstances and outcome of 43 consecutive pregnancies in an unselected group of young women with essential thrombocythemia (ET). Patients and methods: We retrospectively studied 74 consecutive cases of young women with ET seen at our institution, among whom 43 pregnancies occurred in 20 patients. Results: Of the 43 pregnancies, 22 (51%) were successful (21 term and 1 preterm live births) and 21 (49%) ended in miscarriages (1 ectopic pregnancy, 2 elective abortions, 16 first‐trimester spontaneous abortions, 1 stillbirth at 22 wk, and 1 abruptio placentae at 33 wk). Management of ET at the time of conception included either no specific therapy (16 cases) or the use of aspirin alone (24 cases), a cytoreductive agent (2 cases), or heparin (1 case). There were no significant differences with respect to platelet count or the effect of treatment with aspirin, either at the time of conception or during the first trimester, among cases of successful pregnancies (22), all miscarriages (21), or first‐trimester spontaneous abortions (16). The findings were similar when the analysis was restricted to only first‐time pregnancies. In patients with multiple pregnancies, the outcome of a subsequent pregnancy was not predicted by the outcome of the first. In general, in successful cases the last two trimesters were mostly uneventful, with healthy offspring being reported in all cases. Conclusions: Pregnant patients with ET have an increased risk of first‐trimester abortion which is not predictable by preconception platelet count or aspirin therapy. In addition, our experience does not support the use of prophylactic platelet apheresis during delivery.
Benign thymic hyperplasia after chemotherapy for acute myeloid leukemia
Authors: Mishra S. K , Melinkeri S. R , Dabadghao S
Published: March 2003

Abstract: Thymic hyperplasia can occur after cytotoxic therapy for various malignancies. The possible cause could be rebound enlargement after initial atrophy caused by these drugs. During the treatment of hematological malignancies this could be a cause of great concern. We report here a case of thymic hyperplasia after chemotherapy for acute myeloid leukemia. Awareness of this unusual side‐effect may prevent needless investigation and therapy.
Atypical hemoglobin H disease in a Thai patient resulting from a combination of α‐thalassemia 1 and hemoglobin Constant Spring with hemoglobin J Bangkok heterozygosity
Authors: Fucharoen Supan , Ayukarn Kasama , Sanchaisuriya Kanokwan , Fucharoen Goonnapa
Published: March 2003

Abstract: A case of hemoglobin H disease in combination with hemoglobin Constant Spring and a β‐globin chain variant is reported in a 3‐yr‐old Thai girl. On routine cellulose acetate electrophoresis, one abnormal band in addition to the hemoglobins A, A2, H, Bart's and Constant Spring was detected. The amount of this abnormal band movement towards more anodic to the hemoglobin A was 35.7%. DNA analysis of the α‐globin gene cluster by polymerase chain reaction (PCR) revealed a combination of defects caused by the SEA‐type α‐thalassemia 1 and the α‐Constant Spring gene. Analysis of β‐globin gene by PCR and DNA sequencing also detected the heterozygosity for the GGC–GAC mutation at codon 56, leading to a substitution of aspartic acid for glycine resulting in the hemoglobin J Bangkok. The hematologic data of this unusual case of hemoglobin H disease are presented and compared with two compound heterozygotes for hemoglobin J Bangkok and α‐thalassemia 1 found in the patient's father and grandfather. A simple DNA assay based on an allele‐specific PCR for rapid diagnosis of the hemoglobin J Bangkok is also described.
In multiple myeloma increased thrombopoietin (Tpo) production may be involved in the maintenance of platelet production
Authors: Folman C. C. , De Jong C. M. , De Haas M. , Von Dem Borne A. E. G. KR.
Published: March 2003

Abstract: In multiple myeloma (MM), suppression of haematopoiesis occurs as a result of expansion of malignant cells in the bone marrow. Thrombopoietin (Tpo) levels in patients with impaired platelet production are generally found to be highly elevated. To examine the circulating Tpo levels in patients with MM, Tpo levels were measured in 50 serum samples from 34 patients. Tpo levels were subsequently related to disease stage, and cell numbers and markers, i.e. platelet count, leukocyte count and haemoglobin (Hb) concentration. Elevated Tpo levels were found in association with decreased platelet counts (n=8), but also in patients with normal platelet counts (n=14). The latter group included patients without and with signs of impaired haematopoiesis, i.e. with decreased Hb concentration and decreased leukocyte count. These results show that neither platelet counts nor Tpo levels are reliable parameters to judge bone‐marrow failure in patients with MM. Furthermore, in patients with MM, increased Tpo levels may play a role in the maintenance of thrombocytopoiesis. The origin of the increased Tpo levels remains to be determined.
Leukemic transformation with trisomy 8 in essential thrombocythemia: a report of four cases
Authors: Hirose Yuko , Masaki Yasufumi , Sugai Susumu
Published: March 2003

Abstract: Karyotype analysis of bone marrow samples was performed in 20 cases of essential thrombocythemia (ET) at the time of diagnosis. Three patients had karyotype abnormalities at the time of diagnosis; trisomy 8, deletion of Y, and del(9)(q?) in each. The patient who had trisomy 8 at the time of diagnosis underwent myeloid blastic transformation in 35 months. Three patients whose karyotypes were normal at the time of diagnosis developed a chromosome abnormality with trisomy 8 when they developed myeloid blastic transformation 38, 79 and 86 months after initial diagnosis of ET. Two patients with blastic transformation had been treated with busulfan, one with hydroxyurea and one with methyl‐6‐[3‐(2‐chloroethyl)‐3‐nitorosoureido]‐6‐deoxy‐α‐d‐glucopyranoside (MCNU). It is suggested that progression of the disease may have increased the incidence of trisomy 8 and the development of leukemic transformation.
Comparison of reticulated platelet count and mean platelet volume determination in the evaluation of bone marrow recovery after aplastic chemotherapy
Authors: Macchi I. , Chamlian V. , Sadoun A. , Le Dirach A. , Guilhot J. , Guilhot F. , Brizard A.
Published: March 2003

Abstract: Reticulated platelet count provides an estimate of thrombopoiesis in the same way as reticulocyte count is a measure of erythropoiesis. We applied thiazole orange (TO) staining, followed by fluorescence‐activated flow‐cytometric analysis, to platelets in whole‐blood samples from normal subjects and 18 aplastic patients after chemotherapy for haematologic malignancies. The percentage of TO‐positive platelets in 30 control subjects was 5.7 ± 2.4% (mean ± 1 SD), determining the threshold of reticulated platelet positivity as up to 10.5% (mean + 2 SD). In the 18 patients studied, the mean percentage of TO‐positive platelets was 4.3 ± 1.89% during aplasia and 23.3 ± 9.43% during bone marrow recovery, respectively (P < 0.05). All patients had a percentage of TO‐positive platelets of up to 10.5%. In comparison, mean platelet volume during bone marrow recovery increased in 12 cases of the 18 patients studied. We conclude that flow cytometric analysis of reticulated platelets is a sensitive and specific test for evaluating thrombopoiesis recovery during aplastic chemotherapy, and platelet transfusion should be reconsidered in these patients.