Successful Treatment of Kasabach-Merritt Syndrome with Prednisone and Epsilon-Aminocaproic Acid

The Kasabach-Merritt syndrome is characterized by thrombocytopenia and localized coagulopathy associated with a hemangioma. Most techniques applied to eradicate the tumor or accelerate its involution (surgery, radiation therapy, embolization) are invasive and require transfusion of large amounts of blood products. In some cases, medical treatment is the only alternative. Efficacy of steroids and antifibronolytic agents has already been described, but even this approach is associated with the administration of blood products. We report two cases of infants with Kasabach-Merritt syndrome associated with cardiac and hepatic hemangiomas. At admission, both had signs of cardiac failure. They were successfully treated with prednisone and epsilon-aminocaproic acid (EACA). Blood products were not required once the diagnosis was made. These observations have important implications for the management of patients with Kasabach-Merritt syndrome because they show that even in severe cases blood transfusions can be avoided by the use of prednisone and EACA.     

Clinical features of Poncet’s disease. From the description of 198 cases found in the literature

Poncet’s disease (PD) is an entity described as a reactive arthritis due to tuberculous infection elsewhere from the joints. PD existence has been questioned; however, more cases have been reported over the years. Due to its rare nature, little is known about the clinical picture of this disease and no prospective studies had been made to address this issue. We performed a systematic review of the written literature on PD in different databases using the key words “Poncet’s disease,” “tuberculous rheumatism,” and “tuberculous reactive arthritis.” Out of 78 articles, 198 patients were included in the analysis, plus our patient. Several characteristic patterns were found. Also, a review of the pathogenesis and some hypotheses are made. PD is a well-defined entity, which should be taken as a reactive arthritis for future studies given the increase in TB incidence and prevalence around the world, especially in high-burden countries.     

Primary Duodenal Tuberculosis: a Case Report

Gastroduodenal tuberculosis (TB) is a rare condition and is often associated with pulmonary TB. We report the case of a primary duodenal TB in an immunocompetent patient without evidence of pulmonary TB. Diagnosis of this disease is difficult and is often confused with chronic peptic ulcer disease. Literature data concerning this challenging diagnosis are analysed.     

Bcl-2 protein expression is the strongest independent prognostic factor of survival in primary cutaneous large B-cell lymphomas

Bcl-2 protein expression has been associated with poor prognosis in patients with noncutaneous diffuse large B-cell lymphomas. In primary cutaneous large B-cell lymphomas, the location on the leg, the round-cell morphology defined as the predominance of centroblasts and immunoblasts over large centrocytes, and multiple skin lesions were identified as adverse prognostic factors. The prognostic value of bcl-2 protein expression has not been studied in large series of patients. We evaluated 80 primary cutaneous large B-cell lymphomas collected by the French Study Group on Cutaneous Lymphomas. The prognostic value of age, sex, number of lesions, cutaneous extent, location, serum lactate dehydrogenase (LDH) level, B symptoms, morphology, and bcl-2 protein expression was studied. The overall 5-year specific survival rate was 65%. In univariate analysis, advanced age, multiple skin lesions (n = 48), location on the leg (n = 25), round-cell morphology (n = 32), and bcl-2 expression (n = 39) were significantly related to death from lymphoma. In multivariate analysis, bcl-2 expression (P =.0003), multiple skin lesions (P =.004), and age remained independent prognostic factors. The 5-year specific survival rates in bcl-2-positive and bcl-2-negative patients were 41% and 89%, respectively (P <.0001). A new prognostic classification of primary cutaneous B-cell lymphoma should be based primarily on bcl-2 protein expression rather than the location of skin lesions.     

Managing children with chronic myeloid leukaemia (CML)

Chronic myeloid leukaemia in children and young people is a relatively rare form of leukaemia that shows increased incidence with age and some evidence suggests that the molecular basis differs from that in adults. Significant advances in targeted therapy with the development and use in children of tyrosine kinase inhibitors and the ability to monitor and understand the prognostic significance of minimal residual disease by standardized molecular techniques has shifted the management of this condition from bone marrow transplantation as the main therapeutic modality to individualized treatment for each patient based on achieving specific milestones. The physiological changes occurring during childhood, particularly those affecting growth and development and the long‐term use of treatment, pose specific challenges in this age group, which we are only beginning to understand.     

High-frequency sonography and color Doppler in the management of pigmented skin lesions

We aimed to evaluate high-frequency sonography (HFS) coupled with color Doppler in the management of pigmented skin lesions (PSL). HFS examination was performed in 111 patients with 130 PSL. A color Doppler study was conducted in 107 lesions, to assess intralesional vascularization. Imaging findings were compared with histologic diagnosis. In the case of melanoma, sonographic and histologic maximum thickness measurements were compared. HFS showed 114 of the 130 lesions. Among the detected lesions, HFS alone provided 100% sensitivity and 100% specificity in the distinction of melanoma/nevi from other lesions, and 100% sensitivity and 32% specificity in the distinction of melanomas from nonmelanoma lesions. Sonographic and histologic measurement of melanoma thickness strongly correlated (r > 0.96, p < 0.001). Color Doppler detection of intralesional vessels had a 100% specificity and 34% sensitivity in the distinction of melanomas from other PSL. HFS coupled with color Doppler is a simple, reliable tool for PSL management.     

The economic impact of dementia in Belgium: results of the National Dementia Economic Study (NADES)

OBJECTIVES: To estimate costs associated with dementia and its severity and to identify main cost determinants. DESIGN: One-year prospective cohort study. SETTING: 231 general practitioners (GPs) and 15 specialist clinics throughout Belgium. SUBJECTS: 605 patients aged > or = 65 years (219 referent patients, 218 demented patients at home and 168 demented patients in institution). OUTCOME MEASURES: Medical costs (visits to GPs/specialists, physiotherapy, hospitalisation, nursing, incontinence, medication) and non-medical costs (special equipment, services, professional help and caregiving). RESULTS: Total monthly costs amounted to 368.50 Euro dollar for referent patients, 445.56 Euro dollar for demented patients at home and 2301.7 Euro dollar for demented patients in institutions. Highest costs were measured in patients with severe dementia (556.88 Euro dollar at home, 2465.28 Euro dollar in institutions). In demented patients at home, 60% of costs were accounted for by the health system, with hospitalisation and medication being the main cost components (36% and 20%). In demented patients in institution, 46% of the costs were accounted for by the health system, with residential costs and nursing being the main cost components (42% and 32%). In multivariate covariance analysis, the main determinants of costs for demented patients at home were physical dependence and co-morbidity (neoplasm, cardiovascular disease). The adjusted difference between demented and referent patients was 25 Euro dollar per month. CONCLUSIONS: A large fraction of the costs observed in dementia is explained by the association of dementia with physical dependence, co-morbidity and need for caregiving. From an economic point of view, the results support the caring for patients at home.     

The measurement of sense of competence in caregivers of patients with dementia

BACKGROUND: In the context of a cohort study on the socio-economic consequences of dementia in Belgium, we evaluated the validity, reliability and feasibility of the French version of the Dutch Sense of Competence questionnaire (SCQ), and of its short version (the SSCQ), in caregivers of demented patients The questionnaire was based on Zarit's burden interview and on a theoretical family-crisis model. METHODS: Construct validity was evaluated by factor analysis and by comparison of the results with those of the original SCQ study. Reliability was evaluated by Cronbach's alpha and by item-total correlations. Feasibility was assessed by a standardized index of missing values. RESULTS: The three domains found in the original SCQ were identified by factor analysis: consequences of involvement in care for the personal life of the caregiver, satisfaction with one's own performance as a caregiver and satisfaction with the elderly person as a recipient of care. The mean scores were similar to those in the original study, except for the consequences for personal life. Cronbach's alpha for both the SCQ and the SSCQ exceeded the 0.70 criterion. Two of the 27 items did not meet the item-total correlation criterion; the SSCQ items all met the criterion. The standardised index of missing values was deemed acceptable. CONCLUSIONS: The French versions of the SCQ and the SSCQ are valid and reliable. Like the Dutch version, the French version of the SCQ can be a useful outcome measure for the evaluation of intervention studies and the SSCQ is suitable for the daily practice.