Sexual audience affects male's reproduction investment without consequences on reproductive outputs.

Males evolved plastic strategies to respond to male-male competition and exhibit adaptive traits and behaviors maximizing their access to the females and limiting sperm competition. Mating behaviors allow males to express quick responses to current sexual audience, that is, the number of nearby conspecifics prone to mate. In contrast, physiological responses are frequently delayed because they are constrained by the time and resources having to be mobilized to produce and export sperm and associated products. This is especially critical in species for which males produce spermatophores. Here we investigated in what extend moth males (the tortricid moth Lobesia botrana) producing spermatophores exhibit plastic behavioral and physiological responses to different sexual audiences before and during mating and the consequences for their reproductive output. We found that males adjusted their mating behaviors and spermatophore size to a potentially elevated risk of sperm competition perceived before mating. In addition, males responded to the closed presence of females during mating by reducing their mating duration. Surprisingly, the various behavioral and physiological responses we highlighted here were not fully reflected in their reproductive performance as we did not reveal any effect on fecundity and fertility of their mate. The selective pressure exerted on males experiencing male-male competition could thus be sufficient to trigger adjustment in male mating behaviors but constrains physiological responses according to the perception of competition.

Using Nurse Practitioners to Optimize Patient Flow in a Pediatric Emergency Department.

OBJECTIVES: Using nurse practitioners (NPs) in pediatric emergency departments (PEDs) is commonplace in the United States, yet little is known on the impact of NPs on patient flow measures in these environments. This study quantifies the impact of NPs on 2 common measures of patient flow. METHODS: We conducted a retrospective cohort study using administrative data from an academic tertiary care PED. Mean shift length of stay (LOS) and the daily proportion of patients leaving without being seen (LWBS) by a clinician were compared between shifts with and without NPs on duty, matched for external variables affecting the level of activity in the department. Multivariate regression analyses were also conducted to further adjust for covariates such as the total number of PED care providers, patient acuity distribution, and total volume seen in the ED. RESULTS: Despite a slightly reduced total number of providers present on shifts with NPs on duty, a modest but statistically significant reduction in mean shift LOS (-19.11 minutes [95% confidence interval (CI), -31.01 to -7.22]) and daily proportion of LWBS (-1.11% [95% CI, -1.97% to -0.26%]) was observed for shifts with NPs compared with shifts without NPs on duty. Regression analyses showed that incremental NPs on shift were associated with a decreased LOS (-18.76 minutes [95% CI, -24.51 to -13.02]) as well as a reduced odds of LWBS (odds ratio, 0.56; 95% CI, 0.37-0.87). CONCLUSIONS: Nurse practitioners have a modest impact on patient flow measures in a PED and are a valuable resource to optimize patient flow.

Aproximando Teatro e Naturologia: a formação do ator em Stanislavski e a formação do naturólogo / Approaching Theather and Naturology: the training of the actor in Stanislavski and the formation of the naturologist

Este artigo relata sobre a aproximação entre Teatro e Naturologia.Tem por objetivo identificar de que maneira a formação do ator poderá contribuir com a formação do naturólogo, a partir dos conceitos elaborados pelo ator e diretor Constantin Stanislavski. Trata--se de uma revisão bibliográfica qualitativa de caráter exploratório. O levantamento foi realizado nas bases de dados: BVS-Psicologia Brasil, LILACS e SciELO; com a delimitação temporal de 2000 até 2014 e selecionados artigos disponíveis com texto na Língua Portuguesa. Fontes primárias e secundárias referente à metodologia de Stanislavski também foram consultadas. Os aspectos discutidos foram no que diz respeito à preparação do corpo e expressão das emoções. Constatou-se que ocorre aproximação entre as duas formações visto que abordam conceitos semelhantes. Assim, foi possível apontar como a formação do ator poderá contribuir para a formação do naturólogo.(AU)

This article reports on the approximatin between Theater and Naturology. It aims to indentify how the formatin of the actor can contribute to the formation of the naturologist, from the concepts developed by the actor and director Constantin Stanilavski. This is a qualitative bibliographical review of an exploratory nature. The survey was carried out in the databases: VHL-Psychology Brazil, LILACS and SciELO; With the time delimitation from 2000 to 2014 and selected articles avaliable with text in the Portuguese language. Primary and secondary sources for Stanislavski´s methodology were also consulted. The aspects discussed were with regard to the preparation of the body and expression of emoctions. It was found that approximation occurs between the two formations since they approach similar concepts. Thus, it was possible to indicate how the formation of the actor could contribute to the formation of the naturologist.(AU)

Residential and occupational exposure to pesticides may increase risk for cutaneous melanoma: a case-control study conducted in the south of Brazil.

BACKGROUND: Incidences of primary cutaneous melanoma (CM) have risen over the last few decades, mainly among populations of White European extraction. Some risk factors for melanoma have been clearly established, but other potential risk factors, such as exposure to pesticides, are currently under study. METHODS: A case-control study on melanoma was conducted during 2012 and 2013 at three dermatological reference centers in Porto Alegre, Brazil. A total of 191 CM patients and sex- and age-matched control subjects were enrolled in the study. Data on domestic and occupational use of pesticides and the risk factors already established for CM were collected. Multivariate logistic regression was used to study the association between exposure to pesticides and melanoma risk. RESULTS: Subjects exposed to pesticides had twice the level of risk for melanoma (odds ratio [OR] 2.03, 95% confidence interval [CI] 1.03-6.89). When pesticides were used indoors for >10 years, the risk for CM increased further (OR 2.84, 95% CI 1.56-5.33). A high frequency of indoor use of pesticides (four or more times per year) was associated with a 44% increase in the risk for melanoma (OR 1.44, 95% CI 1.11-3.49). The domestic use of pesticides outdoors was not associated with increased risk. Subjects exposed to pesticides at an occupational level were at four times greater risk than subjects who were not occupationally exposed (OR 4.23, 95% CI 1.94-6.31). CONCLUSIONS: These findings indicate that the general use of pesticides, particularly indoor domestic use, frequently and over a long period, may be an independent environmental risk factor for CM.

Immune benefits from alternative host plants could maintain polyphagy in a phytophagous insect.

The tritrophic interactions hypothesis, integrating bottom-up (plant-herbivore) and top-down (herbivore-natural enemies) effects, predicts that specialist herbivores should outcompete generalists. However, some phytophagous insects have generalist diets, suggesting that maintenance of a diverse diet may confer certain fitness advantages that outweigh diet specialization. In field conditions, the European grapevine moth, Lobesia botrana, feeds on diverse locally rare alternative host plants (AHPs) although grapevines are a highly abundant and predictable food source. The laboratory studies presented here show that survival, growth, and constitutive levels of immune defences (concentration of haemocytes and phenoloxidase activity) of L. botrana larvae were significantly enhanced when they were fed AHPs rather than grape. These results indicated a strong positive effect of AHPs on life history traits and immune defences of L. botrana. Such positive effects of AHPs should be advantageous to the moth under heavy selective pressure by natural enemies and, as a consequence, favour the maintenance of a broad diet preference in this species. We therefore believe that our results account for the role of immunity in the maintenance of polyphagy in phytophagous insects.

Brazilian reference values for MPS II screening in dried blood spots--a fluorimetric assay.

OBJECTIVES: Mucopolysaccharidosis II (MPS II), or Hunter Syndrome, is a lysosomal storage disorder that is caused by the deficiency or absence of iduronate-2-sulfatase (IDS) enzyme; in this disease, early diagnosis is essential to provide higher life expectancy for patients. This study validates a fluorimetric assay that is used to assess IDS enzyme activity using dried blood spot (DBS) samples and presents the reference interval for the Brazilian population. DESIGN AND METHODS: Venous blood sample was collected in heparin tubes for leukocyte extraction and DBS preparation. IDS activity in the leukocytes was analyzed, and the results were considered the gold standard reference for the categorization of volunteers as positive or negative controls (PC and NC, respectively). IDS activity in the DBS was analyzed using an adapted version of the leukocyte assay. Statistical analyses were performed using a ROC curve to determine cutoff values and using a parametric Student's t test to compare values between genders. To verify that the assay yielded consistent results, a Bland-Altman plot was prepared. RESULTS: Leukocyte IDS activity values ranged between 2.71 and 17.36 nmol/mg protein/h in the NC group and between 0 and 0.11 nmol/mg protein/h in the PC group. Based on the DBS assay, activities ranged between 1.83 and 16.86 µmol/L blood/h in the NC group and between 0.58 and 4.32 µmol/L blood/h in the PC group. CONCLUSIONS: Reference values of IDS activity were determined in DBS with acceptable sensitivity and specificity. Therefore, the DBS assay described in this work may be a useful tool to screen MPS II patients in the Brazilian population.

Lysosomal integral membrane protein 2 (LIMP-2) restricts the invasion of Trypanosoma cruzi extracellular amastigotes through the activity of the lysosomal enzyme ß-glucocerebrosidase.

Lysosomal integral membrane protein 2 (LIMP-2, SCARB2) is directly linked to ß-glucocerebrosidase enzyme (ßGC) and mediates the transport of this enzyme from the Golgi complex to lysosomes. Active ßGC cleaves the ß-glycosidic linkages of glucosylceramide, an intermediate in the metabolism of sphingoglycolipids, generating ceramide. In this study we used mouse embryonic fibroblasts (MEFs) deficient for LIMP-2 and observed that these cells were more susceptible to infection by extracellular amastigotes of the protozoan parasite Trypanosoma cruzi when compared to wild-type (WT) fibroblasts. The absence of LIMP-2 decreases the activity of ßGC measured in fibroblast extracts. Replacement of ßGC enzyme in LIMP-2 deficient fibroblasts restores the infectivity indices to those of WT cells in T. cruzi invasion assays. Considering the participation of ßGC in the production of host cell ceramide, we propose that T. cruzi extracellular amastigotes are more invasive to cells deficient in this membrane component. These results contribute to our understanding of the role of host cell lysosomal components in T. cruzi invasion.

Evaluation of oxidative stress markers and cardiovascular risk factors in Fabry Disease patients.

Fabry Disease, an X-linked inborn error of metabolism, is characterized by progressive renal insufficiency, with cardio and cerebrovascular involvement. Homocysteine (Hcy) is considered a risk factor for vascular diseases, but the mechanisms by which it produces cardiovascular damage are still poorly understood. Regarding the vascular involvement in FD patients, the analysis of factors related to thromboembolic events could be useful to improving our understanding of the disease. The aim of this study was to evaluate plasma Hcy and other parameters involved in the methionine cycle, as well as oxidative stress markers. The sample consisted of a group of 10 male FD patients and a control group of 8 healthy individuals, paired by age. Venous blood was collected for Hcy determination, molecular analysis, identification of thiobarbituric acid reactive substances, total glutathione and antioxidant enzymes activity, as well as vitamins quantification. Comparative analysis of FD patients versus the control group indicated hyperhomocysteinemia in 8 of the 10 FD patients, as well as a significant increase in overall glutathione levels and catalase activity. It is inferred that FD patients, apart from activation of the antioxidant system, present increased levels of plasma Hcy, although this is probably unrelated to common alterations in the methionine cycle.

New mutations in the GLA gene in Brazilian families with Fabry disease.

Fabry disease (FD) is an X-linked inborn error of glycosphingolipid catabolism that results from mutations in the alpha-galactosidase A (GLA) gene. Evaluating the enzymatic activity in male individuals usually performs the diagnosis of the disease, but in female carriers the diagnosis based only on enzyme assays is often inconclusive. In this work, we analyzed 568 individuals from 102 families with suspect of FD. Overall, 51 families presented 38 alterations in the GLA gene, among which 19 were not previously reported in literature. The alterations included 17 missense mutations, 7 nonsense mutations, 7 deletions, 6 insertions and 1 in the splice site. Six alterations (R112C, R118C, R220X, R227X, R342Q and R356W) occurred at CpG dinucleotides. Five mutations not previously described in the literature (A156D, K237X, A292V, I317S, c.1177_1178insG) were correlated with low GLA enzyme activity and with prediction of molecular damages. From the 13 deletions and insertions, 7 occurred in exons 6 or 7 (54%) and 11 led to the formation of a stop codon. The present study highlights the detection of new genomic alterations in the GLA gene in the Brazilian population, facilitating the selection of patients for recombinant enzyme-replacement trials and offering the possibility to perform prenatal diagnosis.

Evaluation of α-iduronidase in dried blood spots is an accurate tool for mucopolysaccharidosis I diagnosis.

BACKGROUND: Mucopolysaccharidosis type I (MPS I) is caused by a deficiency of the α-L-iduronidase (IDUA), which leads to the accumulation of glycosaminoglycans in lysosomes. MPS I patients present a spectrum ranging from a severe to an attenuated phenotype. Once clinical suspicion is present, diagnosis of MPS I can be performed by enzyme activity determination and/or molecular analysis. The aim of this study was to establish a reference interval value to IDUA activity using a dried blood spots (DBS) assay and to evaluate whether this assay could be a secure tool to diagnose MPS I patients. RESULTS: IDUA activity range on HV DBS samples were 1.40-7.78 µmol/l blood/hr. Regarding the validation group, 11 of the 36 individuals clinically suspected of MPS I had the diagnosis confirmed by DBS and reference assay (leukocytes). When we considered the new proposed cutoff value of 1.5 µmol/l blood/hr, the sensitivity, specificity, and predictive values were 100%. CONCLUSIONS: Our results strongly suggest that the determination of IDUA activity using a DBS assay is a secure tool for MPS I diagnosis. However, it is extremely important to assure that all recommendations for collection, transport, and storage are correctly followed to guarantee the quality of the samples.

Reference values for lysosomal enzymes activities using dried blood spots samples - a Brazilian experience.

BACKGROUND: Lysosomal storage diseases (LSD) are inherited disorders caused by deficiency of lysosomal enzymes in which early diagnosis is essential to provide timely treatment. This study reports interval values for the activity of lysosomal enzymes that are deficient in Mucopolysaccharidosis type I, Fabry, Gaucher and Pompe disease, using dried blood spots on filter paper (DBS) samples in a Brazilian population. RESULTS: Reference activity values were obtained from healthy volunteers samples for alpha-galactosidase A (4.57 ± 1.37 umol/L/h), beta-glucosidase (3.06 ± 0.99 umol/L/h), alpha-glucosidase (ratio: 13.19 ± 4.26; % inhibition: 70.66 ± 7.60), alpha-iduronidase (3.45 ± 1.21 umol/L/h) and beta-galactosidase (14.09 ± 4.36 umol/L/h). CONCLUSION: Reference values of five lysosomal enzymes were determined for a Brazilian population sample. However, as our results differ from other laboratories, it highlights the importance of establishing specific reference values for each center.

Avaliação do aprendizado dos pacientes sobre a regra do ABCD: um estudo randomizado no sul do Brasil / Evaluation of patients' learning about the ABCD rule: a randomized study in southern Brazil

Fundamentos: A regra do ABCD é utilizada para orientar médicos, profissionais da saúde e pacientes quanto ao reconhecimento das principais características de lesões cutâneas suspeitas de melanoma. Não há, no Brasil,estudos que validem a utilização da regra do ABCD pelos pacientes após orientações realizadas por dermatologistas. Objetivos: Avaliar o aprendizado da regra do ABCD por pacientes atendidos em centro dermatológico de referência no sul do Brasil. Métodos: Estudo randomizado com 80 pacientes ambulatoriais de ambos os sexos, com 12 anos ou mais. Foram avaliados: grau de escolaridade, renda mensal e acesso aos meios de comunicação. O grupo de intervenção recebeu orientações sobre o emprego da regra do ABCD, ao passo que o grupo-controle não recebeu. Ambos os grupos foram avaliados quanto às suas respostas em três momentos (basal, fora do consultório e no consultório, 15 dias após) em painel de fotografias. O nível de significância utilizado foi o p < 0,05 e o poder de 0,80. Resultados: O grupo que recebeu as informações respondeu de forma correta, com mais frequência, quanto ao diagnóstico dos melanomas, quando comparado ao grupo-controle (p < 0,01). Excetuando-se o acesso ao rádio, que influenciou positivamente os resultados (p < 0,05), as outras variáveis avaliadas não os afetaram. Conclusões: A regra do ABCD pode ser usada para capacitar pacientes acima de 17 anos a identificar alterações sugestivas de melanoma. Esse aprendizado independe de sexo, nível de escolaridade, renda mensal e acesso aos meios de comunicação, exceto rádio.

Background: the ABCD rule is used to guide physicians, health care professionals and patients to recognize the main characteristics of suspicious skin lesions for melanoma. In Brazil there are no studies to validate the use of the ABCD rule by patients after instructions given by dermatologists. Objectives: to evaluate the learning of the ABCD rule by patients at a dermatology center of reference in the Southern region of Brazil. Methods: a randomized study, with 80 outpatients. The following were evaluated: sex, age, level of schooling, monthly income and access to means of communication. The intervention group received instructions regarding the use of the ABCD rule and the control group did not. Both groups were evaluated at three points in time (baseline; outside the office; and in the doctor's office, 15 days later) regarding their answers about a panel of photographs. The level of significance used was p < 0.05 and a power of 0.80. Results: the group that received information answered correctly regarding the diagnosis of melanomas more often compared to the control group (p < 0.01).Except access to radio broadcasting, which had a positive influence of the results (p < 0.05), the other variables evaluated did not influence the results. Conclusions: the ABCD rule can be used to train patients, above the age of 17 years, to identify changes that suggest melanoma. This learning is independent of sex, level of schooling, monthly income and access to means of communication, except the radio.

Chitotriosidase determination in plasma and in dried blood spots: a comparison using two different substrates in a microplate assay.

BACKGROUND: Chitotriosidase (CT) is a macrophage glycosylhydrolase referred as a biochemical marker on diagnosis and prognosis for Gaucher Disease (GD). The aim of the study was to validate a microplate assay for plasma and dried blood spots on filter paper (DBS) for CT activity determination and to compare CT activity using 4-methylumbelliferyl-b-D-N,N',N''-triacetyl-chitotrioside (4MU-C3) and 4-methylumbelliferyl-deoxychitobiose (4MU-dC2) as substrates. METHODS: Heparinized blood was collected from 12 healthy volunteers (HV) and 14 treated GD patients. Total blood was used to prepare DBS and plasma was obtained by centrifugation. CT activity measurements were done on tube and microplate by a fluorimetric assay using plasma and punches of the DBS samples. RESULTS: The use of 4MU-C3 presented a good correlation among plasma and DBS when analyzing the groups altogether (Pearson=0.76), but the correlation was weaker when we isolated GD patients (Pearson=0.67). The use of 4MU-dC2 increased the correlation among the samples for this group of individuals (Pearson=0.89). CONCLUSION: The possibility of DBS assay facilitates sample transport and storage and CT activity determination on a microplate assay improves laboratory routine. The use of 4MU-dC2 may be more appropriate for GD patients diagnosis on DBS.

[Evaluation of patients' learning about the ABCD rule: A randomized study in southern Brazil]. / Avaliação do aprendizado dos pacientes sobre a regra do ABCD: um estudo randomizado no sul do Brasil.

BACKGROUND: the ABCD rule is used to guide physicians, health care professionals and patients to recognize the main characteristics of suspicious skin lesions for melanoma. In Brazil there are no studies to validate the use of the ABCD rule by patients after instructions given by dermatologists. OBJECTIVES: to evaluate the learning of the ABCD rule by patients at a dermatology center of reference in the Southern region of Brazil. METHODS: a randomized study, with 80 outpatients. The following were evaluated: sex, age, level of schooling, monthly income and access to means of communication. The intervention group received instructions regarding the use of the ABCD rule and the control group did not. Both groups were evaluated at three points in time (baseline; outside the office; and in the doctor's office, 15 days later) regarding their answers about a panel of photographs. The level of significance used was p < 0.05 and a power of 0.80. RESULTS: the group that received information answered correctly regarding the diagnosis of melanomas more often compared to the control group (p < 0.01). Except access to radio broadcasting, which had a positive influence of the results (p < 0.05), the other variables evaluated did not influence the results. CONCLUSIONS: the ABCD rule can be used to train patients, above the age of 17 years, to identify changes that suggest melanoma. This learning is independent of sex, level of schooling, monthly income and access to means of communication, except the radio.

Sputum examination in the clinical management of community-acquired pneumonia.

OBJECTIVE: To evaluate the frequency of the use of sputum examination in the clinical management of community-acquired pneumonia (CAP) in a general hospital and to determine whether its use has an impact on mortality. METHODS: The medical records of CAP patients treated as inpatients between May and November of 2004 at the Nossa Senhora da Conceição Hospital, located in Porto Alegre, Brazil, were reviewed regarding the following aspects: age; gender; severity of pneumonia (Fine score); presence of sputum; sputum bacteriology; treatment history; change in treatment; and mortality. RESULTS: A total of 274 CAP patients (134 males and 140 females) were evaluated. Using the Fine score to quantify severity, we classified 79 (28.8%) of those 274 patients as class II, 45 (16.4%) as class III, 97 (35.4%) as class IV, and 53 (19.3%) as class V. Sputum examination was carried out in 92 patients (33.6%). A valid sample was obtained in 37 cases (13.5%), and an etiological diagnosis was obtained in 26 (9.5%), resulting in a change of treatment in only 9 cases (3.3%). Overall mortality was 18.6%. Advanced age (above 65), CAP severity, and dry cough were associated with an increase in the mortality rate. Sputum examination did not alter any clinical outcome or have any influence on mortality. CONCLUSION: Sputum examination was used in a minority of patients and was not associated with any noticeable benefit in the clinical management of patients with CAP treated in a hospital setting.

Exame do escarro no manejo clínico dos pacientes com pneumonia adquirida na comunidade / Sputum examination in the clinical management of community-acquired pneumonia

OBJETIVO: Este estudo retrospectivo avaliou a freqüência do uso da bacteriologia do escarro no manejo clínico de pacientes com pneumonia adquirida na comunidade (PAC) em um hospital geral, e se a utilização deste método modificou a mortalidade. MÉTODOS: Os prontuários de pacientes internados no Hospital Nossa Senhora da Conceição, em Porto Alegre (RS) Brasil, para tratamento de PAC entre maio e novembro de 2004 foram revisados quanto aos seguintes aspectos: idade; sexo; gravidade da pneumonia (escore de Fine); presença de expectoração; bacteriologia do escarro; história de tratamento; resposta clínica; troca de tratamento; e mortalidade. RESULTADOS: Foram avaliados 274 pacientes com PAC, sendo 134 do sexo masculino. Dentre os 274 pacientes, 79 (28,8 por cento) apresentavam, de acordo com o escore de Fine, classe II; 45 (16,4 por cento), classe III; 97 (35,4 por cento), classe IV; e 53 (19,3 por cento), classe V. Em 92 pacientes (33,6 por cento), uma amostra de escarro foi colhida para exame bacteriológico. Obtivemos amostra válida em 37 casos (13,5 por cento) e diagnóstico etiológico em 26 (9,5 por cento), o que resultou em modificação do tratamento em apenas 9 casos (3,3 por cento). A mortalidade geral foi 18,6 por cento. Idade acima de 65 anos, a gravidade da PAC e a ausência de escarro associaram-se à maior mortalidade. A bacteriologia do escarro não influenciou o desfecho clínico, nem a taxa de mortalidade. CONCLUSÃO: O exame do escarro foi uma ferramenta diagnóstica utilizada na minoria dos pacientes, e não trouxe benefício detectável no manejo clínico dos pacientes com PAC tratados em ambiente hospitalar.

OBJECTIVE: To evaluate the frequency of the use of sputum examination in the clinical management of community-acquired pneumonia (CAP) in a general hospital and to determine whether its use has an impact on mortality. METHODS: The medical records of CAP patients treated as inpatients between May and November of 2004 at the Nossa Senhora da Conceição Hospital, located in Porto Alegre, Brazil, were reviewed regarding the following aspects: age; gender; severity of pneumonia (Fine score); presence of sputum; sputum bacteriology; treatment history; change in treatment; and mortality. RESULTS: A total of 274 CAP patients (134 males and 140 females) were evaluated. Using the Fine score to quantify severity, we classified 79 (28.8 percent) of those 274 patients as class II, 45 (16.4 percent) as class III, 97 (35.4 percent) as class IV, and 53 (19.3 percent) as class V. Sputum examination was carried out in 92 patients (33.6 percent). A valid sample was obtained in 37 cases (13.5 percent), and an etiological diagnosis was obtained in 26 (9.5 percent), resulting in a change of treatment in only 9 cases (3.3 percent). Overall mortality was 18.6 percent. Advanced age (above 65), CAP severity, and dry cough were associated with an increase in the mortality rate. Sputum examination did not alter any clinical outcome or have any influence on mortality. CONCLUSION: Sputum examination was used in a minority of patients and was not associated with any noticeable benefit in the clinical management of patients with CAP treated in a hospital setting.

Noncompaction of the left ventricle: a new cardiomyopathy is presented to the clinician.

Noncompaction of the left ventricular myocardium is a morphogenetic abnormality involving loss of compaction of the myocardial fiber meshwork during intrauterine life. It is an extremely rare condition, accounting for only 0.05% of the cases evaluated in databanks. It has been described in both genders, in many ethnic groups and at different ages. Recently published studies of case series have shown a high mortality rate among these patients during follow-up of up to 48 months. Many cases have so far been misdiagnosed due to poor knowledge of the findings relating to this syndrome. There needs to be an attempt at early and accurate diagnosis, because of the need to investigate the patients family upon such diagnosis, and today this can be achieved using echocardiographic criteria.