Gürkan Altun

Capillary leak syndrome in a 5-month-old infant associated with intractable diarrhoea

Abstract Systemic capillary leak syndrome (SCLS) is a rare disorder of unknown pathophysiology, characterised by episodic life-threatening hypotension, haemoconcentration and hypo-albuminaemia. A 5-month-old child presented with episodes of relapsing diarrhoea, vomiting and hyponatraemia and developed generalised oedema during treatment. Clinical and laboratory findings were consistent with acute SCLS. Treatment with careful fluid replacement, fresh frozen plasma and aminophylline in the acute phase and teophylline and gingko biloba in the chronic phase led to sustained remission during follow-up for over 1 year. To our knowledge, this is the youngest case of SCLS in the literature and is unusual in that it presented with diarrhoea.

Outcome for patients with isolated atrial septal defects in the oval fossa diagnosed in infancy

OBJECTIVE Our aim was to investigate the change in diameter of holes within the oval fossa, and the role of aneurismal formation in reducing the size of the hole, in patients diagnosed during infancy with isolated defects in the floor of the fossa. PATIENTS In a retrospective study, we included 100 patients diagnosed during the first year of life with an isolated defect in the floor of the oval fossa who had subsequently been observed for at least 5 years. There were 56 females and 44 males. They had been admitted to hospital because of a murmur in 65, heart failure in 9, and other reasons in 17. The remaining 9 patients were referred from other institutions with an established diagnosis of defects within the oval fossa. Patients were grouped according to the size of the deficiency in the floor of the fossa. Defects of diameter less than 5 mm were considered to be small, and 20 patients had such defects. Medium sized defects were judged to be between 5 and 8 mm, with 26 patients fulfilling this criterion, with the other 54 patients having large defects with diameters greater than 8 mm. RESULTS The overall spontaneous rate of closure was 27%. Of those with medium defects, half closed spontaneously, but only 7.5% of those with large defects showed such closure. Of the patients who were diagnosed with heart failure, 9 had defects measuring 7 mm, and of these, 6 required surgical closure, 1 patient had spontaneous closure, while the defect became smaller in the remaining 2. On the other hand, in 9 out of 10 patients who had aneurysms, the diameter of the defect was between 7 and 15 mm. Amongst these patients, the defect closed spontaneously in 3, and reduced in size in the others. CONCLUSION When holes within the oval fossa measure 8 mm or below, the majority of patients with experience either spontaneous closure or show decrease in size of the defect. In those with larger defects, the size usually increases, and surgery is needed for closure. If there is aneurismal formation, however, even when the defect measures more than 8 mm, the defect usually closes on its own or gets smaller.

Diffusion-weighted magnetic resonance imaging findings of a patient with neonatal citrullinemia during acute episode

Reports on citrullinemia usually describe the imaging findings in the adult form of the disease. However, neuroimaging findings of neonatal cases are seldom reported. We report on the diffusion-weighted magnetic resonance imaging (MRI) findings of a one-year old infant with neonatal citrullinemia during an acute episode and discuss possible underlying mechanisms for abnormal diffusion findings.

Predictors of vasovagal syncope recurrence in children and adolescents and value of head-up tilt table test

OBJECTIVE The aim of the study was to define predictors of syncope recurrence in children and adolescents with vasovagal syncope and to determine the value of tilt test. METHODS A retrospective observational study performed of prospective cohort of 150 patients aged between 8-18 years who were referred to our clinic because of fainting or who underwent tilt test with the pre-diagnosis of vasovagal syncope. The progress updated by telephone or face-to-face interview. Unpaired t-test, Mann-Whitney U test used for normal and non-normal distributed variables. Logistic regression analysis was used to determine the predictors of recurrence. RESULTS Tilt test was positive in 97 and negative in 53 patients. Forty-eight patients had mixed, 34 had vasodepressor and 15 had cardioinhibitory type syncope. Recurrence found significantly higher in patients who had syncope in the first 20 minutes of the test (p=0.012). The number of the episodes decreased after the test; 3.86±4.75 vs 0.73±0.44, p<0.001). The recurrence was higher in patients who had more than 4 episodes. The recurrence was similar between positive and negative tilt groups. Age of syncope (OR 1.01, 95% CI 1.002, p=0.027) positive family history (OR 4.47, 95% CI 1.071-1.389, p=0.001) and the number of previous syncopal episodes (OR 1.22, 95% CI 1.882-10.623, p=0.003) were identified as risk factors for recurrence of vasovagal syncope. CONCLUSION Age of syncope, positive family history and the number of previous syncopes are the predictors of recurrence of vasovagal syncope in children and adolescents. The number of recurrent episodes decreased after the test independently from Head-up tilt test results.

Acquired von Willebrand syndrome in children with aortic and pulmonary stenosis

Summary Introduction: This prospective study was planned to investigate the frequency and relationship of acquired von Willebrand syndrome (AVWS) with aortic and pulmonary stenosis in patients. Methods: A total of 84 children, ranging from two to 18 years of age, were enrolled in this study. Of these, 28 had isolated aortic stenosis, 32 had isolated pulmonary stenosis and 24 were healthy. Children with aortic and pulmonary stenosis associated with other congenital heart diseases were excluded. Children with hypothyroidism, renal or liver disease, malignancy or autoimmune disease were also excluded. Wholeblood count, blood group, factor VIII level, prothrombin time (PT), activated partial thromboplastin time (aPTT), von Willebrand factor antigen (VWF:Ag), ristocetin co-factor (VWF:RCo), and bleeding time using a platelet-function analyser (PFA-100) were performed in all patients. All of the children in the study underwent a detailed physical examination and echocardiographic evaluation. Results: A history of bleeding was positive in 18% of the aortic stenosis group, 9% of the pulmonary stenosis group, and 4% of the control group. Seven of 60 (12%) patients had laboratory findings that implied a diagnosis of AVWS, and two of these (28%) had a history of bleeding. The frequency of AVWS was 14% in patients with aortic stenosis and 9% in those with pulmonary stenosis. Conclusion: AVWS is not rare in stenotic obstructive cardiac diseases. A detailed history of bleeding should be taken from patients with valvular disease. Even if the history is negative, whole blood count, PT and aPTT should be performed. If necessary, PFA-100 closure time and further tests should be planned for the diagnosis of AVWS.

Intrauterine idiopathic severe ductal constriction diagnosed by fetal echocardiography: a cause of hydrops fetalis.

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A 13-year-old boy with a short QT interval.

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Left ventricular mechanics are affected in children with celiac disease: A study based on two‐dimensional speckle tracking echocardiography

An association between cardiomyopathy and celiac disease has been reported, but not frequently. We examined the effects on cardiac mechanics in children with celiac disease (CD) by two‐dimensional speckle tracking echocardiography (2DSTE).