An 11-year-old boy with consecutive development of systemic lupus erythematosus (SLE) and Wegener granulomatosis (WG) is presented. He was first admitted to the hospital with the findings of SLE, including crescentic glomerulonephritis, Coombs' test-positive hemolytic anemia, hypocomplementemia, antinuclear antibody (ANA) positivity, and elevated levels of anti-double-stranded (ds) DNA antibodies. He was treated successfully with steroids, cyclophosphamide, and peritoneal dialysis. One month after his discharge he developed an apparent viral infection. Three weeks afterwards he was readmitted with the findings of lower respiratory tract involvement, maxillary sinusitis, nasal septum perforation, p- and c-antineutrophil cytoplasmic antibody (ANCA) positivity, but normal complement, ANA, and anti-ds DNA levels, suggesting the diagnosis of WG. He did not respond to anti-infectious and immunosuppressive treatment, and he died of Pseudomonas sepsis.
Neurologic disorders can be seen in patients with end-stage renal failure owing to complications of hemodialysis or peritoneal dialysis. The disequilibrium syndrome can be seen, usually soon after or toward the end of dialysis. We report a patient with central pontine and extrapontine myelinolysis owing to disequilibrium syndrome. The patient had depressed consciousness, agitation, tremor, stupor and hyperactive deep tendon reflexes toward the end of the second peritoneal dialysis. A brain computed tomographic (CT) scan showed hypodense lesions in pontine and extrapontine locations without radiocontrast medium enhancement After 2 days, the patient had only minimal memory deficits. A control brain CT scan 1 week later showed a decrease of the lesions in central pontine and extrapontine locations. Central pontine and extrapontine myelinolysis should be suspected and investigated in the acute neurologic disorders of dialysis patients.
Departments of Pediatric Cardiology Radiology, Dr. Sami Ulus Pediatric Research and Training Hospital, Ankara, Turkey Department of Pediatric Cardiology, Medical Faculty, Fatih University, Istanbul, Turkey.
Unilateral pulmonary artery agenesis is a rare congenital anomaly caused by a backward displacement of the conical artery of the truncus arteriosus. It is commonly associated with additional cardiovascular abnormalities. A 7-year-old girl was admitted to our clinic with the complaint of shortness of breath upon exertion. Chest radiography revealed a hypoplastic right lung. Absence of the right pulmonary artery with atrial septal defect and pulmonary hypertension was demonstrated by echocardiography, computed tomography, and cardiac catheterization. Bosentan is effectively used to treat pulmonary arterial hypertension.
Idiopathic chronic eosinophilic pneumonia (ICEP) is a rare cause of chronic lung disease in children and adolescents. We described four-years old boy presenting with recurrent pneumonia and symptoms of bronchial asthma. Because of peripheral eosinophilia and bilateral pulmonary infiltrates patient investigated comprehensive and chronic eosinophilic pneumonia determined histopathologically. Other conditions causing eosinophilic pneumonia were ruled out. He showed a dramatic response to oral corticosteroid therapy. This report emphasizes that ICEP should be considered in pediatric age group on a cause for chronic hypoxemi or intractable symptoms of respiratory system.
Pulmonary tuberculosis (Tbc) continues to be an important cause of morbidity in children in our country and in the world. There are diagnostic difficulties in the evaluation of the patients with suspected Tbc. In our study of 118 cases, the signs or symptoms suggestive of pulmonary Tbc have a microbiologically confirmed ratio of 26% within the cases having a positive tuberculin test result or having a contact with an adult that had Tbc. Thirty-one (26%) patients were diagnosed with definite Tbc, 48 (41%) patients with probable Tbc and 28 (24%) patients with latent Tbc infection. The tuberculin test was positive in 22 (71%) patients with definite Tbc and in 29 (60%) patients with probable Tbc. A history of a contact with an adult having Tbc has been observed in 18 (58%) patients in definite Tbc case group, in 23 (48%) patients in probable Tbc case group and in 13 (46%) patients in latent Tbc infection case group. No significant difference has been observed between patients with definite or probable pulmonary Tbc according to the average age, sex, tuberculin test positivity and history of Tbc contact. The most common clinical symptoms that has been observed in both groups were cough, fever and weight loss and the most common radiological finding that has been found in both groups was persistent infiltration. Definite or probable Tbc cases were treated for a period of 6 months with 3 anti-Tbc drugs. There were no patients who had failed to response to the treatment. These results showed that, the diagnostic criteria used for probable pulmonary Tbc are also useful in detecting the patients who should be treated with anti-Tbc drugs.