A case of double-outlet left ventricle with atrioventricular discordance

The clinical, echocardiographic and angiographic aspects of a four-month-old boy with double-outlet left ventricle, atrioventricular discordance, L-malposition of the great arteries, ventricular septal defect and pulmonary stenosis are described. Additionally, in this patient, the right arcus aorta and the ligament of ductus arteriosus caused anterior compression of the trachea. The anterior position of the morphological left ventricle is the most interesting feature.     

Neonatal lupus erythematosus presenting with cholestatic hepatitis: a case report and review of the literature

Neonatal lupus erythematosus (NLE) is a disease primarily characterized by cardiac and/or cutaneous involvement. Hepatic, hematological, neurological and pulmonary involvement are rare manifestations and normally considered as mild and transient complications. But recent studies have shown more frequent hepatic involvement in NLE. We report a two month-old male infant, born to a clinically asymptomatic mother, presenting with significant hepatic involvement and annular, erythematous plaques with hyperkeratotic borders at the eyebrow region and anterior surface of trunk. Both the infant and his mother were positive for anti-Ro (SS-A) and anti-La (SS-B).     

Inflammatory bowel disease-like colitis in a young Turkish child with glycogen storage disease type 1b and elevated platelet count

Inflammatory bowel disease (IBD)-like colitis is a known entity in glycogen storage disease (GSD) type 1b patients. The mean age of the reported cases with IBD-like colitis was 12 +/- 5 years, and all had absolute neutrophil count (ANC) less than 1,000 cells/microl. We report a three-year-old girl with GSD type 1b that was dignosed by mutation analysis. The patient was hospitalized with fever, diarrhea, and perioral and anal ulcers. Colonoscopy was performed and IBD-like colitis was diagnosed. The patient had elevated platelet count beyond the age of three months, but IBD-like colitis was diagnosed at three years of age. An elevated platelet count may be a warning sign for the IBD-like colitis in young patients with GSD type 1b.     

Troponin-T levels in perinatally asphyxiated infants during the first 15 days of life

Aim: To measure serial cardiac troponin‐T, creatine kinase, creatine kinase‐MB, aspartate aminotransferase, alanine aminotransferase and lactate dehydrogenase levels in asphyxiated newborn infants during the first 15 d of life. Methods: Troponin‐T, creatine kinase, creatine kinase‐MB, aspartate aminotransferase, alanine aminotransferase and lactate dehydrogenase (LDH) concentrations were measured prospectively in blood samples obtained from 45 asphyxiated and 15 healthy term neonates within the first 2–4 h, third, seventh and 15th days. Results: Infants with severe asphyxia had significantly higher cardiac troponin‐T levels than grade I and II asphyxiated and healthy neonates within the first 2–4 h of life (0.34 ± 0.21 ag/ml vs 0.07 ± 0.03 ag/ml, 0.12 ± 0.07 ag/ml, 0.04 ± 0.02 ag/ml, respectively). Troponin‐T levels remained high on days 3 and 7 in severely asphyxiated neonates. The creatinine kinase‐MB levels were significantly higher in grade II and III asphyxiated neonates than grade I asphyxiated and healthy neonates within the first 2–4 h. No difference was found in creatinine kinase‐MB on day 3. There was cardiac involvement in 12 (80%) newborns of group III on B mode echocardiographic images on day 1. However, no echocardigraphic pathology was found in the seventh‐ and 15th‐day echocardiographic analysis in any groups. Conclusion: Our results suggest that asphyxia‐related cardiac changes were significant but reversible in severely asphyxiated neonates, and troponin T is a good determinant of the degree of injury to the heart within the first week of life. Cardiac troponin T also has a wider diagnostic frame than other diagnostic markers of myocardial damage.     

Evaluating cognitive functions with visual and auditory number assays and P300 in children with epilepsy

This study was planned to evaluate cognitive functions, especially attention and immediate recall, in children with epilepsy by using P300 and neuropsychological tests, which included visual and auditory number assays. Fifty five patients with partial seizures, 45 patients with generalized seizures and 20 patients with intractable seizures were enrolled in the study. Twenty five healthy children were taken as the control group. The results were as follows: 1. P300 latencies were significantly longer in the intractable and partial groups when compared with the control subjects. 2. The duration of epilepsy, seizure frequency, cerebral imaging pathologies were not significantly correlated with delayed P300 latencies. 3. The results of the visual and auditory number assays test showed significant abnormalities when each of the three groups were compared with the normal controls. 4. Though some subgroups of the neuropsychological tests were correlated with the P300 latencies, an overall significant correlation was not present between them. So, we suggest that neuropsychological tests are more convenient for the assessment of cognitive functions in children with epilepsy than the P300 recordings.     

Temporomandibular disorders in Turkish children with mixed and primary dentition: prevalence of signs and symptoms

The prevalence of signs and symptoms related to TM (temporomandibular) disorders was examined in 40 children with primary dentition and in 40 children with mixed dentition. The purpose of this study was to evaluate the relationship between dentition and TM disorders in the examined population. Maximum mouth opening was also evaluated. Clinical examination was done by one investigator. A questionnaire was used in combination with the clinical examination. The results showed that there was an increase in signs and symptoms from the primary to the mixed dentition group, but only the joint sounds were found significantly different between the two groups. There was no statistically significant difference in maximum mouth opening capacity between the groups.     

Menstrually related symptom changes in women with treatment-responsive bipolar disorder

OBJECTIVE: In the present study, we aimed to evaluate menstrually related symptom changes in euthymic women with treatment-responsive bipolar disorder (BD) compared with healthy control subjects and investigate the presence of premenstrual dysphoric disorder (PMDD). METHODS: Thirty-four euthymic women with treatment-responsive BD on mood-stabilizers (lithium and/or valproate) for at least 6 months and 35 control subjects with no history of medical/mental disorder between ages of 18 and 35 years with regular menstrual cycles were prospectively followed up for at least two consecutive menstrual cycles using the Daily Record of Severity of Problems-Short Form (DRSP). Each subject was administered the retrospective self-report questionnaire, Premenstrual Assessment Form (PAF), in the first postmenstrual phase of the menstrual cycle. Venous blood samples were collected between 19 and 22 days of menstrual cycle to evaluate ovulation by measuring the serum progesterone levels. RESULTS: The differences in mean age, age of onset of menses, cycle length and bleeding length did not appear to be significantly meaningful between groups. In the retrospective assessment of premenstrual symptom changes, controls complained more than women with BD. More controls showed a 30% change in DRSP and in depressive and physical sub-groups than the women with BD. Controls demonstrated a significant increase compared with treatment-responsive BD patients in total, depressive, anxiety and attention sub-group scores of DRSP from the postmenstrual to the premenstrual phase, whereas the scores of vegetative symptoms of controls and women with BD did not differ significantly during one cycle or both. Significant menstrual cycle effect was observed in both groups. CONCLUSION: Within the limitations of the study, the results suggest that ongoing mood-stabilizing treatment may have a prophylactic effect against premenstrual symptom changes in women with treatment-responsive BD.