Rukiye Eker Omeroglu

Ultrasonic evaluation of early atherosclerosis in children and adolescents with type 1 diabetes mellitus.

OBJECTIVE To assess early atherosclerosis using B-mode imaging of the carotid artery in children and adolescents with type 1 diabetes mellitus (T1DM) and to evaluate the relationship between various risk factors and intimal plus medial thickness (IMT) in this population. METHODS Fifty-two children and adolescents (aged 3-18 years) with uncomplicated T1DM and 43 age- and gender-matched healthy controls were examined. B-mode imaging was used to determine the intimal plus medial thickness (IMT) of the carotid artery in all subjects. Patients with T1DM and control subjects were divided into two groups according to age and gender. Furthermore, duration of DM was considered for comparison. RESULTS Patients and control subjects showed no association between IMT and sex, systolic blood pressure (sBP), diastolic blood pressure (dBP), serum lipid levels or left ventricular ejection fraction (LVEF). However, statistical analysis indicated a good correlation between age and carotid arterial wall thickness in both diabetic and control groups. These findings were consistent with those in the literature. No correlation was found between IMT and the duration of DM. CONCLUSIONS This study indicates that there is no association between T1DM and IMT in children and adolescents with T1DM.

Congenital heart disease in children with Down's syndrome: Turkish experience of 13 years.

Background — Down’s syndrome (DS) is the most common chromosomal abnormality due to a trisomy of chromosome 21 commonly associated with congenital heart defects (CHDs). This study aimed to evaluate the frequency and types of CHD patterns in Turkish children with DS. Method — The data relate to paediatric patients with DS who underwent cardiologic screening between 1994 and 2007 and were reviewed in our Paediatric Cardiology unit. Results — Four hundred and twenty-one out of the 1042 paediatric patients with DS studied over a 13-year period had associated CHD. Of these, 320 (77.6%) had a single cardiac lesion, while the remaining 92 patients (22.4%) had multiple defects. The most common single defect was an atrioventricular septal defect (AVSD) found in 141 patients (34.2%), followed by 69 patients (16.7%) showing secundum type atrial septal defect, and ventricular septal defect in 68 patients (16.5%). AVSDs were the leading type, isolated or combined with other cardiac anomalies with an overall occurrence of 19.8% of paediatric patients with DS, and 49.2% of paediatric patients with both DS and CHD. Conclusion — This is the first study concerning the frequency and type of CHD observed in Turkish children with DS.The high frequency of AVSD in Turkish children with DS implied that early screening for CHDs by echocardiography is crucial. The correction of AVSDs in paediatric patients with DS should be performed in the first 6 months of life to avoid irreversible haemodynamic consequences of the defect.

Volatile solvent abuse caused glomerulopathy and tubulopathy in street children

Substance misuse among street children is a significant problem in developing countries. Volatile substances are the most abused agents. According to case reports, chronic renal diseases are common among substance-abusing street children. In this study, we examined the renal findings of 42 volatile substance–abusing street children and compared them with results from 49 healthy children (control). The street children’s weight, height, and blood pressure were lower than the controls’ (P < 0.05). However, their blood alkaline phosphatase and creatinine phosphokinase levels were higher (P < 0.05), and total blood protein, creatinine, and phosphorus levels were lower than the controls’ (P < 0.05). Furthermore, the street children’s glomerular filtration rates were within normal limits (P < 0.05), their urinary N-acetyl-beta-glucosaminidase (NAG), β2-microglobulin, microalbumin, protein, calcium, phosphorus, sodium, potassium, and chloride excretions were higher, and tubular phosphate reabsorption were lower than the controls’ (P < 0.05). Volatile substances have been charged with causing distal tubular disease, but increased urinary protein, NAG, β2-microglobulin, microalbumin, and electrolyte excretions also result from glomerular, proximal, and distal tubular influences. We believe that increased volatile substance products in the renal parenchyma are responsible for glomerular and tubular damage. Volatile substance–abusing street children should be examined for glomerular and proximal tubular function and distal tubular acidosis.

Investigation of cardiomyopathy in children with cirrhotic and noncirrhotic portal hypertension.

Background: Cirrhotic cardiomyopathy (CCMP) is a functional disorder characterized by electrophysiological disturbances, and diastolic and/or systolic dysfunction in patients with liver disease. This disorder is a well-defined entity in adults, but pediatric data are limited. The aim of the study was to determine the incidence, features, and risk factors of CCMP in children with portal hypertension (PHT). Methods: This study included 50 children with cirrhotic PHT (40/50) and noncirrhotic PHT (10/50). Fifty healthy children were also selected for the control group. Electrocardiography and echocardiography were used to evaluate cardiac functions. Corrected QT (QTc) ≥ 0.45 was accepted as prolonged on electrocardiography. The study group was divided into 3 groups: cirrhotic, noncirrhotic, and control. Then, the CCMP group was created according to the diagnostic criteria. Latent CCMP was diagnosed in the presence of prolonged-QTc along with a minor criterion (tachycardia). Manifest CCMP was diagnosed in the presence of at least 2 major criteria (prolonged-QTc along with abnormal echocardiographic findings). Moreover, in this study, the risk factors for CCMP were investigated. Results: The CCMP group included 10 cases (20%). Nine of these cases had latent CCMP (18%), and the remaining one (2%) had manifest CCMP. All of the cases with CCMP had cirrhosis and ascites. None of the patients with CCMP had severe cardiac symptoms, but they were already using some cardioprotective drugs such as propanolol and spironolactone. As risk factors for CCMP, pediatric end-stage liver disease scores, Child-Pugh scores, and ascites grades were found to be significant for the determination of CCMP. The most important risk factor was ascites severity (P = 0.001, odds ratio 9.4). Conclusions: Approximately 20% of children with PHT have CCMP. A detailed cardiac examination should be carried out periodically in children with cirrhotic PHT, especially in the presence of ascites and high Child-Pugh score.

Prenatal Diagnosis of Persistent Left Superior Vena Cava and its Clinical Significance

BACKGROUND Persistent left superior vena cava (PLSVC) is a variant of systemic venous return which is observed in 0.3% of autopsies in the general population and in 4-8% of patients with congenital heart disease. AIMS To evaluate associated cardiac, extracardiac and chromosomal anomalies in prenatally diagnosed cases of PLSVC and to review their outcome. STUDY DESIGN Retrospective comparative study. METHODS The data of patients with a prenatal diagnosis of PLSVC between May 2008 and January 2013 were reviewed retrospectively. RESULTS Data of 31 cases were reviewed. Fifteen (48.4%) cases were associated with cardiac defects and 17 (54.8%) cases had associated extracardiac sonographic or postpartum findings. Two fetuses had karyotype anomalies. Outcome was significantly more favorable in cases not associated with cardiac defects in comparison to those associated with cardiac anomalies (84.6% vs. 33.3%, p=0.009). All cases with isolated PLSVC survived, while among the cases associated with extracardiac anomalies, with cardiac anomalies and with both extra-cardiac and cardiac anomalies, the survival rate was 75%, 50% and 22.2%, respectively. The most frequent group of cardiac anomalies associated with PLSVC was septal defects and VSD was the most common heart defect individually, being observed in nine fetuses. CONCLUSION Prenatally diagnosed PLSVC is associated with cardiac and extracardiac anomalies in the majority of cases. Outcome is significantly worse if PLSVC is associated with a cardiac defect, and the prognosis is excellent in isolated cases.

Does early hyperbaric oxygen therapy prevent extremity necrosis in Nicolau syndrome

Nicolau syndrome (embolia cutis medicamentosa or livedo-like dermatitis) was first defined in 1925 after the use of intramuscular bismuth salts for treatment of syphilis. It has been associated with administration of many other drugs, such as non-steroidal anti-inflammatory drugs, corticosteroids, local anesthetics, long acting penicillin and a-interferon. The most commonly identified triggers of Nicolau syndrome (NS) in children are antibiotics. NS is characterized by sudden onset of painful swelling, followed by livedoid erythema, circumscribed hemorrhagic patches and, ultimately, necrosis of the skin, subcutaneous fat and, occasionally, muscular tissue. In certain cases, the necrosis can be extensive; it may be accompanied by rectal hemorrhage, neurologic complications, compartment syndrome, acute renal failure and transient or permanent ischemia of the ipsilateral limb that may even require amputation. Furthermore, NS can be fatal in children. Treatment consists of medical (heparin, pentoxifylline) and surgical (excision, grafting, etc.) therapy, depending on the timing of the diagnosis. In this report, we present a case of NS developed after an injection of benzathine penicillin, in which we used early hyperbaric oxygen (HBO) therapy in addition to heparin and pentoxifylline.

The effects of surgical repair on P-wave dispersion in children with secundum atrial septal defect

IntroductionAtrial septal defect (ASD) is one of the most common congenital heart diseases in children. P-wave dispersion has been reported to be associated with non-homogeneous propagation of sinus impulses. The heterogeneity of atrial conduction time may predispose the atria to arrhythmias. The aim of this study was to determine the impact of surgical repair on P-wave indices in children with isolated secundum ASD.MethodsChildren with isolated secundum ASD undergoing surgical repair (n=50; mean age, 7.0±3.0 years) and healthy controls (n=51; mean age, 7.6±2.7 years) were compared. Maximum P-wave duration (Pmax), shortest duration (Pmin) and P-wave dispersion (Pd) were measured using 12-lead surface electrocardiography.ResultsMean Pmax was found to be significantly higher in children with ASD compared with controls (95.2±10.8 vs 84.1±9.2 msec; P<0.001), and Pd before surgery was significantly higher compared with controls (47.4±12.0 vs 38.8±9.7 msec; P<0.001). Both P-wave indices were significantly decreased within the first year after surgical closure — the values decreased to those comparable to healthy controls (Pmax, 86.2±9.7 msec; Pd, 39.8±10.7 msec; P>0.05).ConclusionSurgical closure of ASD in children decreases Pmax and P-wave conduction time. We speculate that earlier closure of the defect may play an important role in avoiding permanent changes in the atrial myocardium and atrial fibrillation in adulthood.

[Dilated cardiomyopathy associated with dystrophic epidermolysis bullosa: role of micronutrient deficiency?].

Autosomal recessive dystrophic epidermolysis bullosa (DEB) is a chronic skin disorder characterized by widespread bullous formation, erosions, and scar formation. There have been reports of dilated cardiomyopathy and death in patients with DEB. The pathogenesis of cardiomyopathy in DEB remains uncertain, but some drugs, viral infections, iron loading, micronutrient deficiencies such as selenium and carnitine have been implicated. A 16-year-old boy who was followed-up from birth with the diagnosis of DEB presented with respiratory distress and heart failure symptoms of two-week history and early fatigue within the past year. Etiological evaluation showed a low plasma selenium level. Echocardiographic examination yielded the diagnosis of dilated cardiomyopathy. Findings of viral serology tests and metabolic screening were normal. Selenium replacement and anticongestive treatment were initiated, which led to partial improvement in cardiac functions. The authors draw attention to the possible role of micronutrient deficiency in the development of cardiomyopathy in patients with DEB.

Can cystinosis cause coronary artery dilatation

In children, dilated coronary arteries are usually caused by Kawasaki’s disease. We report four children with dilated coronary arteries and nephropathic cystinosis.

Electrocardiographic and echocardiographic findings in street children known to be substance abusers

Substance abuse is prevalent in adolescent street children, and death is reported as secondary to aspiration, accidental trauma, asphyxia, cardiac arrhythmia, anoxia, vagal inhibition and respiratory depression. In this study, we examined electrocardiographic and echocardiographic findings from 53 street male adolescents, comparing our findings to those obtained from 61 controls in the same age group. The street children smoked cigarettes (98.1%) and had used, or were using, thinner (73.6%), glue (75.5%), hashish (79.2%), morphine or its products (24.5%), ecstasy (37.7%), anti-emetics (13.2%) and alcohol (60.4%). On examination, their blood pressures were lower than the control group. Electrocardiographically, PR, QRS, QT were found to be longer (p less than 0.05) than the values for healthy controls. Although it was not statistically significant, QTc duration was also longer than the control group. Echocardiography revealed increased diameters of the left ventricle and atrium, the aorta, and the coronary arteries as compared to the healthy children (p less than 0.05).