A newborn infant with sternal malformation/vascular dysplasia association.

Sternal malformation/vascular dysplasia complex was described by Hersch et al. in 1985. The principle findings include cleft of the sternum covered by an atrophic skin, a midline abdominal raphe and hemangiomatosis. The inheritance pattern seems to be sporadic. We report a newborn baby with sternal defect, cleft lip and palate, supraumbilical raphe and hemangiomas.

Salbutamol intoxication: is salbutamol a drug-inducing fever? A case report and treatment strategy.

A four-year-old female with salbutamol intoxication was referred to our paediatric emergency medicine unit, due to agitation, tremulousness, sinus tachycardia, mild hypokalaemia and hyperglycaemia. On admission the child was agitated and had a noticeable tremor, an axillary temperature of 38 degrees C and a pulse rate of 185 beats/min. She had no identifiable focus of infection on physical examination to explain her fever. Gastric lavage, activated charcoal, intravenous hydration and electrocardiogram (ECG) monitoring were performed. Her plasma potassium level, blood sugar and QT interval were closely monitored during her hospital stay. Her fever, tachycardia and serum potassium and glucose levels returned to normal and she was discharged in good condition 24 h after admission. The difference of this case from prior cases of salbutamol intoxication was the observation of fever in the absence of evidence of infection. Since the cause of fever was not a reaction to the medication used in the treatment or related to environmental factors, it is assumed that salbutamol is a fever-inducing drug.

Osteomyelitis related to BCG vaccination: case report.

A nine-month-old girl with osteomyelitis related to BCG vaccination is presented. The patient did not have any clinical evidence of immunodeficiency. Her X-ray examination showed a lytic lesion of the right tibia and of the left ulna. Histopathological examination of biopsy material revealed a chronic granulomatous inflammatory reaction. She improved with intermittent short course antituberculosis therapy.

Yellow nail syndrome in a 10-year-old girl.

A 10-year-old girl with yellow dystrophic nails, bronchiectasis, chronic sinusitis and lower-limb lymphedema is presented. The underlying mechanism remains unknown although it has been postulated to be associated with lymphatic abnormalities. To date no causative treatment exists. Our patient was treated with conservative management, including a low-fat diet supplemented with medium-chain triglycerides. Moderate improvement in the lymphedema of the lower extremities was observed. To our knowledge this is the first case of yellow nail syndrome to be treated with diet.

Noninvasive cardiac evaluation of young patient with cystic fibrosis.

Cardiac involvement was evaluated by echocardiography in 26 young cystic fibrosis patients. The mean age was 48.4 months (range 3 months to 15 years). The findings were compared with 26 age- and sex-matched children without a history of cardiopulmonary complaints. All patients had normal values of left ventricular ejection fraction and fractional shortening. Interventricular septal and posterior left ventricular wall thicknesses were similar to control group but right ventricular free wall thickness was found greater than in the control group. Abnormal septal motion was documented in six patients. Right ventricular pre-ejection period to ventricular ejection time ratio was found over the upper limit of normal in two patients and there was a negative correlation with clinical Shwachman scores (r: -0.55). Left ventricular pre-ejection period to ventricular ejection time ratio was found over the upper limit of normal in five patients. For both mitral and tricuspid valves, the mean ratios of peak velocity during passive filling (E) phase of diastole to peak velocity during atrial contraction (A) phase were found significantly lower than in the control group (p < 0.05). Early diastolic peak velocity was similar to that in the control group but late atrial peak velocity was higher in the patient group (p < 0.05). Isovolumic relaxation time was found the same as in the control group. We conclude that cardiac changes in diastolic and systolic functions begin at very young ages in cystic fibrosis patients.

Evaluation of renal functions in children with congenital heart disease before and after cardiac angiography.

Radiocontrast nephrotoxicity, which has increased in incidence with widespread use of radiological methods in medicine, is a serious complication of radiocontrast materials. In this study, we have prospectively investigated whether children with cyanotic congenital heart disease are at risk for radiocontrast nephrotoxicity with the use of a nonionic low osmolar contrast agent. Thirty-five children (17 cyanotic and 18 acyanotic patients) who underwent diagnostic cardiac catheterization were subjects of the study. The age range was from five days to 13 years. The volume of contrast material was 3.11 +/- 1.37 ml/kg in cyanotic patients and 2.67 +/- 0.86 ml/kg in acyanotic patients. Blood samples and timed urine samples were taken from all patients 24 hours before and 48 hours after cardiac catheterization. Blood urea nitrogen, creatinine, sodium, and phosphorus in serum, and creatinine and N-acetyl-beta-D-glucosamine in urine were analyzed. There was not a statistically significant difference between the values before and after angiography. As a result, we could find no evidence of radiocontrast nephrotoxicity with the use of a nonionic contrast agent in cyanotic and acyanotic patients who underwent cardiac angiography.

Comparison of mid-term clinical experience with steroid-eluting active and passive fixation ventricular electrodes in children.

Although active fixation ventricular leads seem to have advantages over passive fixation leads, this study compares the follow-up results of active and passive fixation leads in children. We evaluated the implantation and follow-up data of 41 children with active (Accufix II DEC, group 1) (n = 20) or passive (Membrane E, group 2) (n = 21) fixation, steroid-eluting ventricular leads. All but one of the patients in group 1 completed the 12-month follow-up. The mean follow-up period in group 2 was 10.4 +/- 2.9 months (range 3-12 months, median 12 months). In both groups the mean pacing threshold was measured as 0.51 +/- 0.09 V versus 0.48 +/- 0.15 V (P > 0.05) at 0.5-ms pulse width, mean R wave amplitude as 9.9 +/- 2.5 mV versus 9.4 +/- 3.2 mV (P > 0.05), and mean impedance as 557 +/- 92 omega versus 664 +/- 160 omega (P < 0.05), respectively, at implantation. After the first week of pacing, mean threshold values in group 1 were significantly lower than those of group 2 (P < 0.01 and P < 0.05, respectively). During the follow-up period, lead impedance measurements did not show a significant difference between the two groups. In one patient from group 1, the lead (by unscrewing) was removed easily because of pacemaker pocket infection. No lead dislodgement or helix deformation occurred in group 1. Nevertheless, in one patient from group 2, the lead was extracted at 4-month postimplantation because of lead displacement. We conclude that the steroid-eluting active fixation lead (Accufix II DEC) have advantages of easier implantation and lower acute and chronic stimulation thresholds compared to the passive fixation lead (Membrane E). Therefore, Accufix II DEC is superior to Membrane E, and it is a better first choice in children with an implanted single chamber ventricular pacemaker.

Pneumomediastinum and pneumopericardium: unusual and rare complications of asthma in a 4 years old girl.

We describe a 4-year-old girl with asthma who presented with pneumomediastinum, pneumopericardium and subcutaneous emphysema. She was admitted to our hospital with dyspnea, chest pain, palpitation and cough of two days duration. She had attacks of cough, dyspnea and wheezing from two years of age, but she did not have a diagnosis of asthma previously. She was dyspneic and had subcutaneous emphysema in the neck, axilla and thorax. In the skin prick test (Center Lab. USA) she had positive reaction to Dermatophagoides pteronyssinus, Dermatophagoides farinae, mold mix, tree mix and grass mix. Pulmonary function tests could not be performed. In the chest X-ray air was seen in mediastinum and subcutaneous area and the epicardium was surrounded completely with air. She was treated successfully with inhaled salbutamol and budesonide. Radiological signs of pneumopericardium and pneumomediastinum disappeared completely in ten days period. In the light of this case we want to mention that early diagnosis and treatment of asthma should be done to prevent serious complication of asthma.