No abstract available.
Peer Review*

BACKGROUND AND OBJECTIVES: Idiopathic restrictive cardiomyopathy is a very rare and poorly recognized disease in children. This study is performed to describe the clinical course and to define potential predictors of outcome. MATERIAL AND METHOD: We reviewed the medical records and diagnostic studies of 11 consecutive patients during the period from Jan.1991 to Aug. 2000. RESULTS: The age at diagnosis was 1.2-13 years (median 7 years) and the duration of follow up was 3-90 months (median 3.6 years). All except one were symptomatic (dyspnea in ten, chest pain in four). The chest pain was associated with significant ST depression on both resting and exercise ECG, suggesting myocardial ischemia. Two had complete heart block as either initial or terminal event. Cardiac catheterization was done in nine ( mean pulmonary arterial wedge pressure 23+/-6mmHg, systolic pulmonary arterial pressure 47+/-14mmHg, mean right atrial pressure 11+/-9mmHg). Echocardiographic dimensional ratio of left atrium and aorta (LA/Ao) was 2.41+/-0.58. Mitral E/A inflow ratio was 2.72+/-1.42, E wave deceleration time was 93.6+/-44.2ms. During follow up, six died. The 2 year and 5 year cumulative survival rates were 54.5% and 18.8% respectively. The predictor for nonsurvivor were pulmonary venous congestion and LA/Ao >2.5(p<0.05). Verapamil was tried in 6 cases without favorable effect in all. CONCLUSION: Considerable numbers of restrictive cardiomyopathy have myocardial ischemia associated with ST depression and chest pain. The patients with pulmonary venous congestion and severe left atrial enlargement (LA/Ao>2.5) were at risk for death, requiring prompt definitive treatment such as cardiac transplantation.
Aorta ; Arterial Pressure ; Atrial Pressure ; Cardiac Catheterization ; Cardiac Catheters ; Cardiomyopathy, Restrictive* ; Chest Pain ; Child* ; Deceleration ; Depression ; Diagnosis ; Echocardiography ; Electrocardiography ; Follow-Up Studies ; Heart Atria ; Heart Block ; Heart Transplantation ; Humans ; Hyperemia ; Medical Records ; Myocardial Ischemia ; Pulmonary Wedge Pressure ; Survival Rate ; Verapamil

Kawasaki disease (KD) is an immune-mediated disease which is a leading cause of acquired cardiovascular disease in developed country. Recently, tumor necrosis factor-alpha (TNF-alpha) blocker, infliximab has been considered a promising option for patients with refractory KD. Although chronic use of a TNF-alpha blocker could increase risk of opportunistic infections, a few studies have documented that use of infliximab was safe without serious adverse effects in patients with KD. We observed serious bacterial infection after infliximab treatment in an infant with refractory KD. Our patient was a 5-month-old male infant diagnosed with KD who did not respond to repeated doses of intravenous immunoglobulin. We effectively treated him with a single infusion of infliximab (5 mg/kg), but gram-negative (Acinetobacter lwoffii) septicemia developed after infliximab infusion. Therefore, we report a case of serious septicemia after treatment with infliximab, and suggest considering the risk of severe infection when deciding whether to prescribe infliximab to an infant with refractory KD.
Bacterial Infections ; Cardiovascular Diseases ; Developed Countries ; Humans ; Immunoglobulins ; Infant* ; Male ; Mucocutaneous Lymph Node Syndrome* ; Opportunistic Infections ; Sepsis* ; Tumor Necrosis Factor-alpha ; Infliximab

The fungus aspergillus can cause a variety of pulmonary disorders. Aspergilloma is a noninvasive aspergillus colonization of virtually any type of preexisting pulmonary cavity or Cystic space. Invasive pulmonary aspergillosis is serious, usually fatal infection in patients being treated with immunosuppressants or who have chronic debilitating disease. Allergic bronchopulmonary aspergillosis is charaterized clinically by asthma, blood and sputum eosinophilia and positive immunologic reaction to aspergillus antigen. Awareness of the radio-graphic and CT findings of pulmonary aspergillosis is important in making the diagnosis of aspergillus-caused pulmonary disorders. In this pictorial essay, we illustrated various radiological findings of pulmonary aspergillosis focused on CT findings correlated with gross pathologic specimens.
Aspergillosis, Allergic Bronchopulmonary ; Aspergillus ; Asthma ; Colon ; Diagnosis ; Eosinophilia ; Fungi ; Humans ; Immunosuppressive Agents ; Invasive Pulmonary Aspergillosis ; Pulmonary Aspergillosis* ; Sputum

The fungus aspergillus can cause a variety of pulmonary disorders. Aspergilloma is a noninvasive aspergillus colonization of virtually any type of preexisting pulmonary cavity or Cystic space. Invasive pulmonary aspergillosis is serious, usually fatal infection in patients being treated with immunosuppressants or who have chronic debilitating disease. Allergic bronchopulmonary aspergillosis is charaterized clinically by asthma, blood and sputum eosinophilia and positive immunologic reaction to aspergillus antigen. Awareness of the radio-graphic and CT findings of pulmonary aspergillosis is important in making the diagnosis of aspergillus-caused pulmonary disorders. In this pictorial essay, we illustrated various radiological findings of pulmonary aspergillosis focused on CT findings correlated with gross pathologic specimens.
Aspergillosis, Allergic Bronchopulmonary ; Aspergillus ; Asthma ; Colon ; Diagnosis ; Eosinophilia ; Fungi ; Humans ; Immunosuppressive Agents ; Invasive Pulmonary Aspergillosis ; Pulmonary Aspergillosis* ; Sputum

BACKGROUND: This study aimed to investigate bradycardia as an adverse effect after administration of dexmedetomidine during 33degrees C target temperature management. METHODS: A retrospective study was conducted on patients who underwent 33degrees C target temperature management in the emergency department during a 49-month study period. We collected data including age, sex, weight, diagnosis, bradycardia occurrence, target temperature management duration, sedative drug, and several clinical and laboratory results. We conducted logistic regression for an analysis of factors associated with bradycardia. RESULTS: A total of 68 patients were selected. Among them, 39 (57.4%) showed bradycardia, and 56 (82.4%) were treated with dexmedetomidine. The odds ratio for bradycardia in the carbon monoxide poisoning group compared to the cardiac arrest group and in patients with higher body weight were 7.448 (95% confidence interval [CI] 1.834-30.244, p = 0.005) and 1.058 (95% CI 1.002-1.123, p = 0.044), respectively. In the bradycardia with dexmedetomidine group, the infusion rate of dexmedetomidine was 0.41 +/- 0.15 microg/kg/h. Decisions of charged doctor's were 1) slowing infusion rate and 2) stopping infusion or administering atropine for bradycardia. No cases required cardiac pacing or worsened to asystole. CONCLUSIONS: Despite the frequent occurrence of bradycardia after administration of dexmedetomidine during 33degrees C target temperature management, bradycardia was completely recovered after reducing infusion rate or stopping infusion. However, reducing the infusion rate of dexmedetomidine lower than the standard maintenance dose could be necessary to prevent bradycardia from developing in patients with higher body weight or carbon monoxide poisoning during 33degrees C targeted temperature management.
Atropine ; Body Weight ; Bradycardia* ; Carbon Monoxide Poisoning ; Dexmedetomidine* ; Diagnosis ; Emergency Service, Hospital ; Heart Arrest ; Humans ; Hypothermia, Induced ; Logistic Models ; Odds Ratio ; Retrospective Studies

No abstract available.

Prompt medical attention is crucial for congenital intracranial vascular malformations in children and newborns due to potential severe outcomes. Imaging is pivotal for accurate identification, given the diverse risks and treatment strategies. This article aims to enhance the identification and understanding of congenital intracranial vascular abnormalities including arteriovenous malformation, arteriovenous fistula, cavernous malformation, capillary telangiectasia, developmental venous anomaly, and sinus pericranii in pediatric patients.

A 43-year-old female presented with persistent headache and dizziness which had first occurred two years earlier. The physical and neurological findings at admission were unremarkable, though plain radiography revealed the presence of a dense calcified mass in the left frontal area, and CT showed that a homogeneous high-density nodule was attached to the inner surface of the left frontal skull. The hard bony mass found and excised during surgery was shown at histopathologic examination to be a subdural osteoma. We describe the clinicopathologic findings of this entity and discuss the radiological features which suggest its subdural location.
Adult ; Case Report ; Female ; Frontal Bone/*pathology/*radiography/surgery ; Human ; Osteoma/*pathology/*radiography/surgery ; Skull Neoplasms/*pathology/*radiography/surgery ; Subdural Space/pathology/radiography/surgery ; Tomography, X-Ray Computed