Novel electrocardiographic criteria may render possible the more accurate recognition of cardiac amyloidosis.

AIMS: The early diagnosis of cardiac amyloidosis (CA) is paramount, since there are effective therapies that improve patient survival. The diagnostic accuracy of classical electrocardiographic (ECG) signs, such as low voltage, pseudoinfarct pattern, and conduction disturbances in the diagnosis of CA, is inferior to that of the echocardiographic myocardial deformation criteria; therefore, our aim was to find more accurate novel ECG criteria for this purpose. METHODS: We tested the diagnostic value of five novel ECG criteria, two of them devised by us, in 34 patients with confirmed CA (20 transthyretin amyloidosis and 14 AL amyloidosis) and 45 control patients with left ventricular hypertrophy on echocardiography due to hypertension, valvular aortic stenosis and hypertrophic cardiomyopathy. The following novel ECG criteria, that suggested CA, were tested: QRS amplitude in lead I < 0.55 mV (I < 0.55); QRS amplitude in lead aVR < 0.5 mV (aVR < 0.5); average QRS amplitude of leads I + aVR < 0.575 mV [(I + aVR) < 0.575]; average QRS amplitude of leads I + aVR/average QRS amplitude of leads V1-4 < 0.375 [(I + aVR)/(V1-4) < 0.375]; average QRS amplitude of leads I + aVR/longest intrinsicoid deflection in leads I,aVL,V1-6 < 0.0115 [(I + aVR)/I,aVL,V1-6ID < 0.0115]. RESULTS: The I < 0.55, aVR < 0.5, (I + aVR) < 0.575, (I + aVR)/(V1-4) < 0.375, (I + aVR)/I,aVL,V1-6ID < 0.0115 test accuracy (TA) were 81%, 84.8%, 82.3%, 84.8%, and 83.3%, respectively; the sensitivity (SE): 76.5%, 82.4%, 85.3%, 82.4%, and 76.9%; specificity (SP): 84.4%, 86.7%, 80%, 86.7%, and 87.5%; positive predictive values (PPV): 78.8%, 82.4%, 76.3%, 82.4%, and 80%; negative predictive values (NPV): 82.6%, 86.7%, 87.8%, 86.7%, and 85.4%; area under curve (AUC) values: 0.8922, 0.8794, 09016, 0.8824, and 0.8462 were respectively. These parameters of the novel ECG criteria were at least as good as those reported by other authors in the literature of the qualitative (TA: 67%, SE: 80%, SP: 34%, PPV: 75%, NPV: 42%, AUC: 0.57) and quantitative apical sparing (TA: 64-80%, SE: 66-81.3%, SP: 55-78.3%, PPV: 33-83.9%, NPV: 41-75%, AUC: 0.62-0.68) and left ventricular ejection fraction/global longitudinal strain >4.1 (TA: 77%, SE: 93%, SP: 38%, PPV: 79%, NPV: 69%, AUC: 0.65) echocardiographic criteria. Among the classical criteria, the low voltage in limb leads criterion was present most frequently (in 73.5%) in patients with CA, with slightly worse diagnostic value than the novel ECG criteria (TA: 78.5%, SE: 73.5%, SP: 82.2%, PPV: 75.8%, NPV: 80.4%). CONCLUSIONS: The novel ECG criteria [mostly the aVR < 0.5, (I + aVR)/(V1-4) < 0.375] seem at least as reliable in the diagnosis of CA as the best echocardiographic myocardial deformation criteria and might be used either together with the echocardiographic criteria or as stand-alone criteria to diagnose CA in the future.

Maternal factors in the origin of isolated anorectal malformations - a population-based case-control study.

OBJECTIVE: In most patients affected by isolated anorectal malformation (IARM), the etiology is unknown. Thus, the aim of this study was to analyze the possible role of maternal risk factors in the origin of IARM. METHODS: The study samples included 231 cases with IARM, 361 matched and 38 151 population controls without any defect in the population-based large dataset of the Hungarian Case-Control Surveillance of Congenital Abnormalities, 1980-1996. Cases with IARM were evaluated in the function of maternal diseases and related drug use. RESULTS: The findings of this case-control study suggested that cases with IARM have an obvious male excess. The mothers of cases with IARM had a lower incidence of severe nausea and vomiting in pregnancy and a higher incidence of acute infectious diseases in the urinary tract. CONCLUSIONS: Lack of nausea and vomiting in pregnancy and the higher incidence of urinary tract infections may have a role in the development of IARM.

Maternal factors in the origin of infantile hypertrophic pyloric stenosis: A population-based case-control study.

In most patients affected by isolated infantile hypertrophic pyloric stenosis (IHPS) the etiology is largely unknown. Thus, the aim of this study was to estimate possible maternal risk factors in the origin of IHPS. The study samples included 241 cases with IHPS, 357 matched controls and 38,151 population controls without any defect in the population-based large dataset of the Hungarian Case-Control Surveillance of Congenital Abnormalities, 1980-1996. Exposures that had been medically recorded in prenatal maternity logbooks during the critical period of IHPS were evaluated separately. The findings of this case-control study suggested that--beyond the well-known robust male excess (85.5%)--maternal hyperthyroidism (OR with 95% CI: 4.17, 1.53-11.38) and oral nalidixic acid treatment (OR with 95% CI: 6.53, 3.03-14.06) associated with a higher risk for IHPS in their children. In conclusion, our findings suggest that cases with IHPS had mothers with a higher proportion of hyperthyroidism and nalidixic acid treatment during pregnancy.

Birth outcomes of patients with isolated anorectal malformations: A population-based case-control study.

In most patients affected by isolated anorectal malformation (IARM) the etiology is largely unknown. Thus, the aim of our project was to analyze possible risk factors for IARM. In the first step, birth outcomes of cases with IARM were analyzed on the basis of maternal socio-demographic variables, and these data are presented in this paper. Gestational age at delivery, birthweight, preterm birth, low birthweight and small for gestational age of cases with IARM were evaluated in the function of maternal age, birth/pregnancy order, marital and employment status of mothers in the population-based large dataset of the Hungarian Case-Control Surveillance of Congenital Abnormalities, 1980-1996. The study samples included 231 live-born cases with IARM, 361 matched and 38 151 population controls without any defect. IARMs are more frequent in males, twins and newborn infants with low birthweight and small-for-gestational-age, the latter being the consequence of intrauterine growth restriction. In addition, mothers of cases were younger but with higher birth order, and had lower socio-economic status. These maternal variables are characteristic for the gypsy population in Hungary. The higher proportion of gypsy women among the mothers of cases with IARM was confirmed during the home visits of the study. Male sex and intrauterine growth restriction of cases, in addition to low socioeconomic status and gypsy origin of mothers may have a role in the risk of IARMs.

Modeling cost-effectiveness of cervical cancer screening in Hungary.

OBJECTIVES: Our aim was to compare the cost-effectiveness of two national cervical cancer screening programs aiming to involve those who do not regularly participate in the screening program in Hungary with no screening, using a public health-care payer's perspective and a 20-year time horizon. METHODS: We built a Markov model based on disease progression. The health-care costs of screening and treatment were received from real-word data. Other input data were obtained from the literature. The cost-effectiveness of the current screening program (a screening test that combines cytology and colposcopy in gynecological outpatient services) and of a planned new screening program (only cytology, and Pap smear is taken locally by public health nurses), both supported with a more active communication campaign, were compared with no screening. RESULTS: The incremental cost-effectiveness ratio of the intensified current screening practice was $33,100 per quality-adjusted life-year compared with no screening, whereas the incremental cost-effectiveness ratio of the renewed program was $18,990 per quality-adjusted life-year compared with no screening. The most influential parameters in the deterministic analysis were the quality-of-life weights of undetected stage I or IIA cancer. In the probabilistic sensitivity analysis, 99.9% of the simulations were below the incremental cost-effectiveness ratio of $30,000 per quality-adjusted life-year in the case of the renewed strategy. CONCLUSIONS: Providing services closer to the population is a rational economic option for the reform of the Hungarian cervical cancer screening program. The other policy aspects of this development, human resource need, stakeholders' interests, organizational aspects, and attitude of the target population need to be carefully considered.