Congenital complete atrioventricular block from fetal life to childhood : Diagnostic and therapeutic aspects

Roord, J.J. [Promotor]Sreeram, N. [Promotor

PENGEMBANGAN BUKU AJAR AKUNTANSI KAS BERBASIS SCIENTIFIC APPROACH PADA MATA PELAJARAN AKUNTANSI KEUANGAN KELAS XI DI SMK NEGERI SE SURABAYA

Penelitian pengembangan ini bertujuan untuk menghasilkan buku ajar akuntansi kas berbasis scientific approach pada mata pelajaran akuntansi keuangan kelas XI di SMK Negeri Se Surabaya agar dapat meningkatkan motivasi belajar, meningkatkan penguasaan konsep materi dan pemahaman siswa dalam proses pembelajaran materi yang bersangkutan. Selain itu, penelitian ini bertujuan untuk mengetahui kelayakan dan respon siswa terhadap buku ajar akuntansi kas berbasis scientific approach yang dikembangkan. Model pengembangan yang digunakan diadaptasi dari model pengembangan 4P yang terdiri dari empat tahapan yaitu tahap pendefinisisan, tahap perancangan, tahap pengembangan, dan tahap penyebaran sehingga menghasilkan buku ajar akuntansi kas berbasis scientific approach. Hasil pengembangan menunjukkan bahwa rata-rata kelayakan buku ajar sebesar 83,5%. Sedangkan respon siswa diperoleh persentase sebesar 88,7%. Dengan demikian, buku ajar akuntansi kas berbasis scientific approach dinyatakan sangat layak digunakan sebagai bahan ajar untuk meningkatkan penguasaan konsep materi dan pemahaman siswa kelas XI di SMK Negeri Se Surabaya pada mata pelajaran akuntansi keuangan materi akuntansi kas. Kata Kunci:  Buku ajar, Scientific Approach, Akuntansi Ka

Imaging the Lymphatic System in Fontan Patients

Contains fulltext : 204149.pdf (publisher's version ) (Closed access

Successful biventricular cardiac resynchronization therapy in a failing Fontan patient: Implications of ventriculo-ventricular interdependency in single ventricle physiology

Item does not contain fulltextWe describe a Fontan patient with severe heart failure who was successfully treated with biventricular cardiac resynchronization therapy (CRT). Our case shows that strain imaging might play a crucial role in guiding placement of pacing leads and in characterizing the electromechanical substrate associated with a favorable CRT response. Furthermore, we demonstrate for the first time that ventriculo-ventricular interdependency seems an important mechanical concept, which can be utilized to augment cardiac performance in failing Fontan patients with a functional hypoplastic ventricle

The added value of the electrocardiogram in Noonan syndrome

Noonan syndrome is a genetic disorder characteried by short stature, typical facial features, developmental delay, and CHD. In this single-centre retrospective study, we analysed typical Noonan syndrome-related electrocardiographic features in 95 patients with clinically and molecularly confirmed Noonan syndrome. Typical Noonan syndrome-related electrocardiographic features are left axis deviation, small left precordial R-waves, large right precordial S-waves, abnormal Q-wave, and abnormal wide QRS complex. In this representative cohort, CHD was found in 59 patients (62.1%) and typical Noonan syndrome-related electrographic features in 60 patients (63.2%). The typical Noonan syndrome-related electrographic features were also increased over baseline in patients without CHD (41.7%). Of all 95 patients, left axis deviation was seen in 46.3%, small left precordial R-waves in 30.5%, large right precordial S-waves in 5.3%, and abnormal Q-wave and wide QRS complex in 2.1%. There was no significant difference in the frequency of the individual-specific electrographic features between the group with CHD and the group without CHD. However, there were significantly more patients with a small left precordial R-wave in the subgroup with pulmonary stenosis compared to patients without pulmonary stenosis. Conclusion: Specific Noonan syndrome-related electrographic features are frequently present in patients with Noonan syndrome, also in the absence of CHD. These results suggest that there may be a continuum of cardiac anomalies from overt CHD to milder abnormalities that are only seen on electrocardiogram

The Need to Define Treatment Goals for Protein-Losing Enteropathy in Fontan Care and Research

Item does not contain fulltex

Transient neonatal myelosuppression after fetal exposure to maternal chemotherapy. Case report and review of the literature

Transient neonatal myelosuppression (TNM) is a rare but potentially life-threatening adverse effect of fetal exposure to maternal chemotherapy during pregnancy. We report a case of TNM in a preterm infant born to a mother diagnosed with acute lymphoblastic leukemia during pregnancy. The mother received chemotherapy during the second and third trimester. The neonate was successfully treated with supportive care. In addition, we also conducted a medical literature review and identified another 14 cases of TNM. Although the long-term outcome of these children is not known, short-term survival is relatively good. Prompt recognition and aggressive treatment of infants at risk for TNM is mandatory. Copyright © 2008 S. Karger AG

Comparability of different Z-score equations for aortic root dimensions in children with Marfan syndrome

BACKGROUND: Aortic root dilation is a major complication of Marfan syndrome and is one of the most important criteria in establishing the diagnosis. Currently, different echocardiographic nomograms are used to calculate aortic root Z-scores. The aim of the present study was to assess the potential differences in aortic root measurements when aortic root Z-scores were obtained in a cohort of paediatric Marfan patients using several published nomograms. METHODS: In a cohort of 100 children with Marfan syndrome, Z-scores for aortic root dimensions were calculated according to the nomograms of Pettersen et al, Gautier et al, Colan et al, and Lopez et al. Bland-Altman plots were used to estimate mean differences in Z-scores and to establish limits of agreement. RESULTS: The mean Z-score of the sinus of Valsalva for Lopez et al was significantly higher compared to Gautier et al (p < 0.01) and Pettersen et al (p = 0.03). The nomogram of Lopez et al resulted in substantially higher Z-scores in patients with a large sinus of Valsalva diameter. Thirty-five percentage of the studied patients would have a Z-score ≥ 2 using Lopez et al compared to 20% for Pettersen et al, 21% for Gautier et al, and 33% for Colan et al. CONCLUSION: The currently available nomograms for calculating Z-scores of aortic dilation in children with Marfan syndrome lead to clinically relevant differences in Z-scores, especially in children with a relative large aortic root diameter. This could have impact on both the diagnosis and treatment of patients with Marfan syndrome

Cardiac involvement in two rare neuromuscular diseases: LAMA2-related muscular dystrophy and SELENON-related myopathy

LAMA2-related muscular dystrophy (LAMA2-MD) and SELENON(SEPN1)-related myopathy (SELENON-RM) are rare neuromuscular diseases caused by mutations in the LAMA2 and SELENON (SEPN1) gene, respectively. Systematic reviews on cardiac features in both neuromuscular diseases are lacking. This scoping review aims to elucidate the cardiac involvement in LAMA2-MD or SELENON-RM. Three electronic databases (PubMed, Embase and Cochrane) were searched. All studies, case reports and case series with information on cardiac features in LAMA2-MD or SELENON-RM patients were included. Study selection and data extraction were performed by two independent reviewers. 31 Articles on LAMA2-MD and 17 articles on SELENON-RM met the inclusion criteria, resulting in the inclusion of 131 LAMA2-MD and 192 SELENON-RM cases. In 41% of LAMA2-RM cases, a cardiac abnormality was present. Left ventricular systolic dysfunction and arrhythmia were most frequently described. In 15% of SELENON-RM cases, a cardiac abnormality was reported, of which pulmonary hypertension, including right ventricular dysfunction secondary to pulmonary failure, was most prevalent. We conclude that in LAMA2-MD primary left ventricular dysfunction and in SELENON-RM secondary right ventricular dysfunction are frequently reported. Optimal cardiorespiratory surveillance by screening of asymptomatic patients every two years with ECG, Holter and echocardiography is necessary for early detection and/or treatment of cardiac manifestations