Search

Background Cardiac affectation in amyloidosis is presented among 50% patients what implies worse prognostic. Early diagnosis and prognostic stratification is mandatory after the appearance of new therapies that could modify the course of the disease. Purpose The aim of the present study was to evaluate the main outcome predictors with multiparametric approach including clinical, laboratory, electrocardiographic and imaging parameters in patients with cardiac amyloidosis (CA). Methods We recruited patients (p) prospectively followed in our cardiomyopathy unit, for a mean period of 10 years (from 2010 to 2020) who were diagnosed with CA. Baseline clinical, laboratory and echocardiographic data were obtained. We collected cardiovascular risk factors and previous events. We performed a multimodality imaging study including echocardiogram (TTE) and cardiac magnetic resonance (CMR). TTE hallmarks of CA were recorded, as well as CMR parameters of cardiac function, myocardial mass and tissue characterization. Finally we evaluated the parameters related with mortality in the follow-up. Results A total of 98 p were included, with a mean age of 67.5±16.9 years old. Mean follow-up was 42,2±32 months. 22 p (24,4%) had light-chain amyloidosis (AL), 34 p (37,8%) wild type transthyretin amyloidosis (ATTRwt) and 34 p (37,8%) familiar transthyretin amyloidosis (ATTRm). 43p (47.78%) died in the follow-up. Factors related with mortality in the the follow-up where the age (HR 1.08, ppresence of hypertension (HR 2.81, p=0,002) diabetes (HR 3.12, p=0,001) and previous stroke (HR 2.69, p=0,01); NYHA class at diagnosis (HR 2.49, p0,001) and extension of late gadolinium enhancement (HR 1,06, p=0,03) at CMR. When evaluated within a multivariate analysis in a Cox regression model, the independent variables associated with mortality were NYHA class at diagnosis (p=0.002) and amyloidosis type (p=0.002) ATTRm had a better prognosis than ATTRwt and AL (Log-rank p Conclusion Mortality in cardiac amyloid patients is high. Clinical parameters like hypertension, diabetes, previous stroke and NYHA class at diagnosis; laboratory parameters like BNP and creatinine, main systolic and diastolic function at TTE, presence of auricular fibrosis and extension of late gadolinium enhancement at CRM are related with mortality in the follow-up. Among different types of CA, patients with ATTRm had a better prognosis, which may have therapeutic implications with the appearance of new therapies. Funding Acknowledgement Type of funding sources: None. Mortality

Background Cardiac affectation in amyloidosis is presented among 50% patients what implies worse prognostic. Early diagnosis and prognostic stratification is mandatory after the appearance of new therapies that could modify the course of the disease. Purpose The aim of our study was to assess the prognostic value of cardiac magnetic resonance parameters, including Tissue deformation, in cardiac amyloidosis patients. Methods We recruited patients (p) prospectively followed in our cardiomyopathy unit, for a mean period of 10 years (from 2010 to 2020) who were diagnosed with CA. Baseline clinical, laboratory and echocardiographic data were obtained. Contrast-enhanced CMR was performed on a 1.5 T clinical scanner. Routine analysis was performed with a semi-automatic software for volumetric analysis. When late gadolinium enhancement was present, percentage of area of enhancement (LGE) was calculated.All strain parameters were measured off-line using dedicated software. Left ventricular circumferential strain (CS) measurements were obtained using mid-ventricular level short-axis cine views. Longitudinal strain (LS) derived from cine SSFP of 2-, 3-, and 4-chamber long axis views.Finally we evaluated the CMR parameters related with the combined event of mortality and heart failure in the follow-up. Results A total of 98 p were included, with a mean age of 67.5±16.9 years old. Mean follow-up was 42,2±32 months. 22 p (24,4%) had light-chain amyloidosis (AL), 34 p (37,8%) wild type transthyretin amyloidosis (ATTRwt) and 34 p (37,8%) familiar transthyretin amyloidosis (ATTRm). 59p (60,8%) died or present heart failure during the follow-up. Patients who died or present heart failure in the follow-uo had lower left ventricular ejection fraction (48,25±12,2% vs 56,13±11,03%, p=0,003), higher myocardial mass (156,05±54,8g vs 120,84±56,1g, p=0,007), higher LGE (8,6±6,8% vs 4,4±2%), worst GLS (−14,76±6,1% vs −18,67±6,2%) and worst GCS (−23,3±9,7% vs −30,04±9,1%). Both, GLS and GCS were independently associated with the combined event of dead or heart failure when evaluated within a multivariate analysis in a Cox regression model, but GCS was the stronger predictor of events in the follow-up over other CMR parameters like LGE an myocardial mass (p Conclusion Mortality and appearance of heart failure in cardiac amyloid patients is high. The assessment of myocardial strain parameters by CMR tissue-tracking in this population is useful to predict adverse outcomes in the follow-up. Particularly, GCS, stratify patients in subgroups with different risk of events, with added value to classical CMR parameters. Funding Acknowledgement Type of funding sources: None. Death & Heart Failure by GCS

Background COVID19 has been related to elevated CVB and biventricular dysfunction during hospitalization. However, it is unknown whether patients with biomarker elevation exhibit long-lasting abnormalities in cardiac function. Purpose To determine, using advanced echocardiography, the prevalence and type of cardiovascular sequelae after COVID19 infection with marked elevation of cardiovascular biomarkers (CVB), and their prognostic implications. Methods All patients admitted from March 1st to May 25th, 2020 to a tertiary referral hospital were included. Patients with cardiovascular disease antecedent, death during admission, or the first 30 days after discharge were excluded. Patients with hs-TnI >45 ng/L, NT-proBNP >300 pg/ml, and D-dimer >8000 ng/ml were separated based on each CVB elevation and matched with COVID controls (three biomarkers within the normal range) based on intensive care requirements and age. Results From a total of 2025 hospitalized COVID19 patients, 80 patients with significantly elevated CVB and 29 controls were finally included. No differences in baseline characteristics were observed among groups, but elevated CVB patients were sicker. Follow-up echocardiograms showed no differences among groups regarding LVEF or RV diameters, but TAPSE was lower if hs-TnI or D-dimer were elevated. Hs-TnI patients also had lower global myocardial work and global longitudinal strain. The presence of an abnormal echocardiogram was more frequent in the elevated CVB group compared to controls (23.8 vs 10.3%, P=0.123) but mainly associated with mild abnormalities in deformation parameters. Management did not change in any case and no major cardiovascular events except deep vein thrombosis occurred after a median follow-up of 7 months (Figure 1). Conclusions Minimal abnormalities in cardiac structure and function are observed in COVID19 survivors without previous cardiovascular diseases who presented a significant CVB rise at admission, with no impact on patient management or short-term prognosis. These results do not support a routine screening program after discharge in this population. Funding Acknowledgement Type of funding sources: None. Figure 1

Background Mitral annular disjunction (MAD) is a structural abnormality of the mitral annular fibrosus characterized by a separation between the atrial wall-mitral valve junction, and the left ventricular attachment (1). It has been associated with mitral valve prolapse (MVP) (2) but also, with arrhythmias and sudden cardiac death (SCD) (3). There is no evidence of its prevalence and clinical significance in patients with syndromic hereditary aortopathies. Purpose To evaluate the prevalence of MAD, PMV, and the combination of both in patients with syndromic hereditary thoracic aortic disease (HTAD) including Marfan (MFS), Loeys-Dietz (LDS) and vascular Ehlers-Danlos syndromes (vEDS), and its relationship with arrhythmias, SCD, mitral regurgitation (MR) severity and the need for mitral surgery at the follow-up. Methods Adult patients with syndromic HTAD seen at our specialized unit were retrospectively included. The presence of MAD, MVP, and significant MR at first echocardiogram were evaluated. Electronic medical records were reviewed to register the occurrence of arrhythmic events and the need of mitral surgery. Last echocardiogram available was also assessed to evaluate MR progression. Results A total of 295 patients were included (235 MFS, 42 LDS and 18 vEDS). Mean age at baseline was 39.0±14.4 and 52.9% were female. MAD was present in 87 (37.0%) of MFS, 6 (14.3%) of LDS and was not present in vEDS (p In MFS, the presence of MAD was significantly associated with MVP (p≤0.001) (Table 1). However, 14 (6.0%) of patients had isolated MAD (Table 2). At baseline, significant MR was observed in 18 (24.7%) of patients with concurrent MAD and MVP and was not present in patients with isolated MAD (Table 2). MVP (OR 16.85 IC 4.43 – 64.07) but not MAD (p=0.607), was associated with significant MR in the multivariate analysis. A second echocardiogram was available in 220 patients at ≥1 year (mean 4.1±1.4 years). Overall, 25 (11.4%) presented significant progression of MR, 0 in the isolated MAD group, 13 (19.4%) in the MAD/MVP group and 6 (20.0%) of the isolated MVP (p=0.007). After a mean clinical follow-up of 7.5±3.2 years, 10 patients required mitral surgery (6 prosthesis, 4 valvuloplasty), 22 (9.4%) presented atrial fibrillation, flutter or supraventricular tachycardia (SVT), and 2 (0.9%) SCD. After adjustment for the presence of MVP and time of follow-up, MAD was not associated with progression of MR (p=0.529) need for mitral surgery (p=0.096), atrial fibrillation-flutter or SVT (p=0.510) nor SCD. (p=0.997). Conclusions The prevalence of MAD in syndromic HTAD is high, especially in Marfan syndrome, and absent in vEDS. In this retrospective observational study, the presence of MAD in Marfan was not associated with mitral regurgitation evolution or arrhythmic events. Funding Acknowledgement Type of funding sources: None. Characteristics of MFS patientsPresence of significant MR by groups.

Background Myocardial work (MW) is a new imaging technique to assess left ventricular (LV) systolic function. It incorporates both deformation parameters (global longitudinal strain -GLS-) and loading conditions and gives information on global constructive work (GCW), global wasted work (GWW), global LV myocardial work index (GWI) and global LV myocardial work efficiency (GWE). Purpose The aim of this study was to describe the prognostic role of MW in predicting major adverse cardiovascular events (MACE) in patients with reduced LV ejection fraction (LVEF), and to compare it with GLS and LVEF. Methods We retrospectively included consecutive patients from 2012 to 2019 with dilated LV and LVEF Results 99 patients were included, 26 were women (26.3%), mean age at diagnosis was 57 years (SD 23). Mean LVEF was 32.5% (SD 10.3). Baseline patients characteristics are described in Table 1. During a median follow-up of 25 months (IQR 12), 24 MACE were recorded (24.4%). Patients with MACE had worse MW parameters: significantly lower MWI (805±360% vs 638±277%, p=0.04) and lower GCW (1116±535 mmHg vs 874±458 mmHg, p=0.05), and a tendency to lower GWE (83±11% vs 77±16%, p=0.084). Of note, both LVEF (33±10% vs 29±9%, p=0.123) and GLS (−9.99±3.7% vs −8.8±3.0, p=0.170) showed a trend but were not significantly associated with outcomes. This might suggest that MW variables are stronger prognostic predictors than traditional imaging parameters. Conclusions In patients with reduced LVEF, MW parameters including global MWI and GCW were associated with major adverse cardiovascular events. Of note, both EF and GLS seem to have less prognostic implications in this cohort when compared with MW. Our results are preliminary and larger studies are needed in order to fully understand the clinical utility of MW beyond traditional parameters. Results Funding Acknowledgement Type of funding source: Public hospital(s). Main funding source(s): Universitary Hospital Vall d'Hebron

Merkel cell carcinoma (MCC), first described by Cyril Toker in 1972,1 is a rare and aggressive skin cancer. Although it accounts for less than 1% of malignant skin tumors, it is the second leading cause of death from skin cancer behind melanoma.2 Despite its aggressive behavior, MCC may be curable in patients with local and node-positive disease.3 Even with a high rate of local and distant recurrence, treatment options exist that can improve overall survival and quality of life. Early diagnosis and timely intervention are key to improving health outcomes. The purpose of this work is to briefly review the features and treatment of MCC so that health care providers and policymakers are familiar with the disease and recognize the current limitations in Latin America that are barriers to improved outcomes.

Background Left ventricular noncompaction (LVNC) is associated with an increased risk of systemic embolisms (SE). However, incidence and risk factors are not well established. Purpose To evaluate the rate of SE in LVNC and describe risk factors. Methods LNVC patients were included in a multicentric registry. Those with SE were considered for the analysis. Results 514 patients with LVNC from 10 Spanish centres were recruited from 2000 to 2018. During a median follow-up of 4.2 years (IQR 1.9–7.1), 23 patients (4.5%) had a SE. Patients with SE (Table 1) were older at diagnosis, with no differences in gender and had similar cardiovascular risk factors. They were more frequently under oral anticoagulation (OAC). Besides, they had a more reduced LVEF, and more dilated LV and left atrium (LA). Late gadolinium enhancement (LGE) was more frequent, altogether suggesting a more severe phenotype. Patients with SE had non-significantly higher rates of hospitalization for heart failure (33% vs 24%, p=0.31) and atrial fibrillation (35% vs 19%, p=0.10). In multivariate analysis, only LA diameter was an independent predictor of SE (OR 1.04, p=0.04). A LA diameter>45 mm had an independent 3 fold increased risk of SE (OR 3.04, p=0.02) (Image 1). Table 1 Systemic embolisms (n=23) No systemic embolisms (n=491) p Men, n (%) 15 (65) 289 (56) 0.52 Median age at diagnosis (IQR), yr 60 (48–76) 48 (30–64) 0.02 Median follow up (IQR), yr 5.9 (3.1–7.8) 4.2 (1.8–7.1) 0.18 Hypertension, % 8 (33) 118 (24) 0.31 Diabetes mellitus, % 3 (14) 39 (8) 0.41 OAC, % 19 (83) 118 (24) 0.01 LVEF (SD), % 37 (15) 48 (17) 0.01 LVEDD (SD), mm 58 (11) 54 (10) 0.04 LVESD (SD), mm 45 (13) 38 (11) 0.01 LA diameter (SD), mm 46 (9) 39 (9) 0.01 LVEDV CMR (SD), mL 193 (75) 163 (70) 0.12 LVESV CMR (SD), mL 121 (64) 85 (64) 0.04 LGE, % 9 (40) 88 (18) 0.04 Conclusions LVNC carries a moderate mid-term risk of SE, which appears to be irrespective of atrial fibrillation and associated with age, LV dilatation and systolic dysfunction and mainly LA dilatation. This subgroup of patients should be considered for oral anticoagulation in primary prevention.

Background Late gadolinium enhancement (LGE) is the clinical reference standard for estimation of infarct extension and prediction of functional recovery following reperfused acute ST-segment elevation myocardial infarction (STEMI). Nevertheless, myocardial edema, microvascular obstruction and intramyocardial hemorrhage as well as the timing of image acquisition after contrast administration may influence the extent of LGE and underestimate the potential for recovery. Dobutamine stress testing has been recommended to more accurately predict functional recovery when infarct transmurality is between 25 to 75%. We hypothesized that cardiac magnetic resonance (CMR) tissue tracking strain analysis may provide additional value to LGE for the prediction of functional recovery. Methods In 370 patients with STEMI who underwent successful primary percutaneous revascularization and were studied with CMR within 3–5 days of the event, peak systolic longitudinal (LS), circumferential (CS) and radial (RS) strain were analyzed with routine SSFP images of 3 long-axis and a stack of short-axis slices (Tissue Tracking, CVI42®, Figure panel A and B). Inversion-recovery echogradient sequences were analyzed 20 minutes after contrast administration for LGE transmurality (Panel C). All per-segment analysis was performed according to the AHA 16-segment model. CMR was repeated at 6 months and functional recovery was defined as persistent normokinesia or improvement of wall motion score from baseline to 6-month CMR. Results At baseline CMR, of a total of 5920 segments 70.4% were normokinetic, 7.2% were hypokinetic, 21.9% were akinetic and 0.6% were dyskinetic. All strain parameters decreased significantly with worsening wall motion. At follow-up, 81.5% of the segments showed functional recovery. All strain parameters were significantly associated with functional recovery (p CS analysis in an inferior STEMI Conclusions Acute CMR tissue tracking strain analysis complements LGE assessment for prediction of functional recovery following an STEMI. The combination of LGE infarct transmurality under 50% and a CS strain higher than −10.7% showed the highest accuracy for prediction of recovery of function.

Erythrosis pigmentosa peribuccalis is an infrequent condition, with fewer than 35 cases reported in the literature, that affects mainly women. It presents as small papules that form a hyperpigmented plaque around the mouth and nose. Little is known about the etiology of this condition, its dermoscopic characteristics have not been described, and no effective treatment has been reported. We report a 15-year-old girl with this rare dermatosis who was successfully managed with daily calcipotriol, weekly topical ivermectin, and strict photoprotection. We also describe for the first time the dermoscopic findings of this entity.

Inflammatory breast cancer is an aggressive and infiltrative malignancy that is often misdiagnosed as an infection because of its symptoms and signs of inflammation, delaying proper diagnosis and treatment. We report a case of inflammatory breast cancer showing correlation between dermoscopic and histopathological diagnoses. We highlight the utility of dermoscopy for skin biopsy site selection.

Background/Objective Papular epidermal nevus with skyline basal cell layer (PENS) is a recently described type of epidermal nevus with characteristic histopathologic findings, mainly regular, rectangular acanthosis and a well-demarcated basal cell layer with clear palisading and separation between basal cell nuclei and the first row of Malpighian cell nuclei. Although the first reports described randomly distributed lesions appearing sporadically in otherwise healthy patients, cases of Blaschkoid distribution, lesions associated with extracutaneous manifestations, and familial cases have been reported. Methods We performed a review of the clinical charts of all patients with histologic diagnosis of PENS in our hospital. We evaluated epidemiologic, clinical, and histologic features. We then reviewed the literature with a particular emphasis on the presence or absence of extra-cutaneous associations. Results Three patients with PENS are described. One had a single lesion, one had three lesions, and one, a patient with mild developmental delay, a curved penis, and hypospadias, had multiple lesions. Conclusion The probability of having extracutaneous manifestations is 6.3 times as great in individuals with more than four lesions. Therefore these patients may need closer follow-up.

Polyomavirus has been used extensively to study tumour induction in mice. Although most neoplasms are well characterized, those arising from hair follicles have been referred to by different names during the last four decades. The purpose of this research was to contribute to a more accurate histological characterization of these tumours as well as to study the viral progression from the onset of infection to the development of neoplasms. Polyomavirus A2 was inoculated into newborn C3H/BiDa mice, and at different time-points (from 5 to 70 days post-inoculation) the mice were sacrificed and studied using histological, immunocytochemical, ultrastructural and virological methods. The fully developed hair follicle tumours consisted of a proliferation of matrix cells that evolved into 'shadow' cells with empty nuclei and finally into amorphous keratin; the tumours were therefore diagnosed as pilomatricomas. Viral VP-1 was observed only in fully differentiated cells and not in proliferating-cell-nuclear-antigen (PCNA)-positive cells in the same tumour. In conclusion, Polyomavirus first replicated in the skin, and then disseminated through the blood and reached the outer sheath of the hair follicles and finally infected matrix cells, leading to the development of pilomatricomas from which infectious virus was isolated.

Intravascular lymphoma is a rare subtype of extranodal diffuse large B-cell lymphoma characterized by clonal proliferation of lymphocytes inside of small and medium caliber vessels. Its incidence is estimated at one case per million. The clinical picture is very variable, but frequently has skin and central nervous system involvement. It is diagnosed by demonstrating pathological blood vessel infiltration by lymphoma cells. We report a 44 years old male presenting with fever, malaise and erythematous lesions in the abdominal wall. An abdominal wall biopsy showed dilated vascular vessels with atypical cells in their lumen, compatible with large B-cell intravascular lymphoma. He was treated with rituximab, cyclophosphamide, adriamycin, vincristine and prednisone and an autologous hematopoietic stem cell transplantation, achieving a complete remission that has lasted two years.

We determined the influence of oral contraceptives (OC) on the capacity of the endothelium to release tissue-type plasminogen activator (t-PA). Twenty-three healthy premenopausal women were studied: 12 nonusers and 11 users of OC. Net endothelial release rates of t-PA were calculated as the product of the arteriovenous concentration gradient and forearm plasma flow in response to intra-arterial bradykinin (BK: 12.5–50 ng · 100 ml tissue−1· min−1) and sodium nitroprusside (SNP: 1.0–4.0 μg · 100 ml tissue−1· min−1). Net release of t-PA antigen and increment in t-PA activity across the forearm to BK increased ( P < 0.01) in a dose-dependent fashion and to similar extents in the nonusers and users of OC. At the highest BK dose, net release of t-PA antigen was 64.5 ± 8.2 and 66.2 ± 15.4 ng · 100 ml tissue−1· min−1in the nonusers and users of OC, whereas the net increment in t-PA activity was 18.6 ± 3.0 and 16.0 ± 2.0 IU · 100 ml tissue−1· min−1, respectively. There was no effect of SNP on t-PA release in either group. These results indicate that endothelial t-PA release is not altered in premenopausal women who use oral contraception.

Background: Indirect measures of body composition suggest that Hispanic women have an excess prevalence of overweight and obesity compared with white women. Socioeconomic status (SES) is a potentially confounding factor in such studies. Objective: Our aim was to determine whether Hispanic ethnicity is associated with higher total and regional adiposity and lower fat-free mass (FFM) in healthy women across the adult age range. Design: We used a prospective cross-sectional design to examine total and regional body composition in 54 Hispanic women (primarily of Mexican descent) and 56 white women of similar SES. Results: The groups were not significantly different in mean age, body mass, or SES, although the Hispanic women were shorter (P < 0.05). Body mass index (in kg/m 2 ; 25.2 ± 0.5 compared with 23.9 ± 0.5; P < 0.05), percentage body fat (38.4 ± 0.8% compared with 34.9 ± 1.3%: P < 0.05), and total fat mass (25.0 ± I.0 compared with 23.0 ± 1.2 kg; P = 0.10) were or tended to be higher in the Hispanic women. The greater total adiposity of the Hispanic women was primarily the result of higher percentage fat and fat mass in the trunk (P < 0.05); within the trunk region, abdominal and subscapular skinfold thicknesses were 30-40% greater in the Hispanic women (P

Hispanic women may undergo greater age-related reductions in physical functional capacity compared with Caucasian women. If so, a greater rate of decline in muscle strength with age could contribute. We tested this hypothesis in 82 healthy sedentary Caucasian (n = 37) and Hispanic (n = 45) adult women aged 21-78 years of similar socioeconomic status. Absolute one-repetition maximum (1-RM) strength of the knee extensors (KE) declined with advancing age in the Caucasian (r = -.55, p < .01) and Hispanic (r = -.45, p < .01) women; the rates of decline were similar in the two groups (-7% to 8% x decade(-1), p = .60). KE strength normalized for thigh fat-free mass (FFM) also declined with age in the Caucasian (r = .52, p < .01) and Hispanic (r = -.41, p < .01) women, the rates of decline being similar (-6% to 7% x decade(-1), p = .66). For all functional performance tasks (10-m walk, stair ascent, stair descent, and chair stand), performance time increased with advancing age (mean of four tasks vs age: Caucasian, r = .64, p < .01; Hispanic, r = .56, p < .01). Absolute and normalized KE 1-RM were inversely related to the mean time for the four performance tasks (r = -.34 to -.58, all p < .01). Normalized KE 1-RM was the best independent predictor of the age-related decline in task performance in both groups. These cross-sectional findings do not support the hypothesis that Hispanic ethnicity per se is associated with a greater decline in KE strength and performance tasks associated with KE strength in healthy women.

Eosinophilic annular erythema is a rare benign recurrent disease, originally described in children, characterized by the recurrent appearance of persistent non-pruritic, urticarial annular lesions. Histologically a perivascular infiltrate composed of lymphocytes and abundant eosinophils in the dermis is exhibited. We report the case of a 15-year-old boy who presented with a 4-year history of recurrent flares of erythematous annular plaques on the trunk and extremities. The lesions resolved spontaneously after 3-5 weeks with no accompanying signs. A biopsy showed a mainly perivascular lymphocytic infiltrate with numerous eosinophils in the dermis.

Heart disease is a frequent complication of chronic kidney disease and the major cause of death in patients on renal replacement therapy. The purpose of this study was to evaluate the impact of successful kidney transplantation on systolic and diastolic ventricular dysfunction and pulmonary arterial hypertension in patients with chronic kidney disease (CKD).The study included 35 patients18 years of age with CKD who had successful kidney transplantations. Ventricular function and pulmonary arterial pressure were evaluated by echocardiography before and 1 year after transplant.The mean age of subjects was 40 ± 14 years, and 63% were men. Mean left ventricular ejection fraction (LVEF) was 52 ± 16%. Before transplant, 28 (80%) of the patients had ventricular dysfunction (34.3% diastolic and 45.7% systolic). Pulmonary arterial hypertension was found in 48.6%. Ventricular dysfunction was associated with dialysis of2 years duration before transplant. The LVEF of the entire group increased from 52% to 64% (P.001) by 12 months after kidney transplant. Left ventricular diameters, wall thickness, and pulmonary arterial systolic pressure decreased significantly after transplantation Echocardiograms became normal 1 year after transplant in 8 (66.7%) of the patients with diastolic dysfunction and 9 (56.2%) with systolic dysfunction, and diastolic dysfunction persisted in 5 (31.2%).Because kidney transplantation led to considerable improvement in left ventricular systolic and diastolic function as well as pulmonary arterial pressure of patients with CKD, optimal treatment for dysfunction and transplant as soon as possible is recommended.

A normolipemic patient with diffuse plane xanthomas, IgG monoclonal gammopathy of unknown significance, low levels of C4, and systemic inflammatory symptoms is presented. Delay from disease onset to diagnosis is discussed.

To the Editors: We read with great interest the letter by Tenholder and colleagues (1), who reported the association of bronchiolitis obliterans organizing pneumonia with the myelodysplastic syndro...

The last 100 patients with malignant melanoma were studied following actual methodology, with these results: mean age at diagnosis was 55.3 years; mean age at the beginning of disease was 51.8 years. Lesions were more frequent in patients with pale skin; there was no predominance of any color of eyes. Forty-nine percent of the patients were referred with a previous nevus which was confirmed only in 7%. One tumor developed on a giant pigmentary congenital nevus. Lesions showed no preponderance in one sex. Four percent of patients had multiple lesions. Familial background was found in 5%. The localization was most often to the head and neck, trunk, and legs. The most frequent types were SSMM and NMM; the first one on trunk and the second one on legs. The SSMM was observed in younger people than NMM. Level IV was seen in the greatest number of cases, with an average thickness of 3.07 mm. In SSMM, level III predominated, while in NMM, level IV predominated. The average thickness of SSMM was 1.82 mm; that of NMM was 6.25 mm. Our data are representative of malignant melanoma in Argentina. Therapy and evolution of lesions will be analyzed with an appropriate long-term follow-up.

Tn 1971, Dabska 1 reported 12 cases of giant hair matrix tumors. We report here a case in which the tumor reached an unusually large size. Report of a Case A 58-year-old woman had a large, progressively growing tumor. It had been present for the past seven years and covered the whole scalp like a turban. On examination, the lesion was seen to be lobulated, pink-yellow, and foul smelling (Fig 1). It was 25 × 20 × 5 cm. There were no palpable regional lymph nodes. No notable abnormalities were found on roentgenographic examination of the skull and chest. Histopathologic Findings A biopsy specimen obtained at another medical institution was interpreted as being consistent with squamous cell carcinoma. The slides were sent to us for examination. The specimen showed a proliferative epithelial process containing large eosinophilic cells with central oval nuclei and prominent nucleoli. Some pleomorphism was observed, and there