Acute Appendicitis: Sensitivity, Specificity and Diagnostic Accuracy of Thin-Section Contrast-Enhanced CT Findings

PURPOSE: To assess the sensitivity, specificity, and diagnostic accuracy of individual contrast-enhanced helical CT findings of acute appendicitis. MATERIALS AND METHODS: We retrospectively reviewed the appendiceal helical CT scans, obtained after intravenous contrast administration (abdomen; 7-mm collimation, abdominopelvic junction; 5-mm collimation), of 50 patients with surgically proven acute appendicitis and 112 with alternative diagnoses. The following parameters were analysed by three radiologists: enlarged appendix (> 6 mm in diameter), appendiceal wall thickening, appendiceal wall enhancement, no identification of the appendix, appendicolith(s), (appendiceal) intraluminal air, (appendiceal) intraluminal air extraluminal air, periappendiceal fat stranding, extraluminal fluid, phlegmon, abscess, lymphadenopathy, terminal ileal wall thickening, focal cecal apical thickening, focal colonic wall thickening, and segmental colonic wall thickening. RESULTS: The CT findings of acute appendicitis that statistically distinguished it from alternative diagnoses were an enlarged appendix (sensitivity; 92%, specificity; 93%, diagnostic accuracy; 93%), appendiceal wall thickening (for these three parameters: 68%, 96% and 88%, respectively), periappendiceal fat stranding (90%, 79%, 82%), appendiceal wall enhancement (72%, 86%, 82%), appendicolith (16%, 100%, 74%), and focal cecal apical thickening (14%, 100%, 74%) (for each, p < 0.05). CONCLUSION: On thin-section contrast-enhanced helical CT, an enlarged appendix and periappendiceal fat stranding were found in 90% or more patients with acute appendicitis. Appendiceal wall thickening and enhancement were clearly demonstrated and significant findings for diagnosis. Less common but specific findings include appendicolith, focal cecal apical thickening and intramural air, can also help us establish a diagnosis of acute appendicitis.

HFE gene mutation, C282Y causing hereditary hemochromatosis in Caucasian is extremely rare in Korean population

Hereditary hemochromatosis (HFE), which affects 1 in 400 and has an estimated carrier frequency of 1 in 10 individuals in Western population, results in multiple organ damage caused by iron deposition, and is treatable if detected early. C282Y mutation in HFE gene has been known to be responsible for the most hereditary hemochromatosis cases and 5-10% of white subjects are heterozygous for this mutation. However, the prevalence of hemochromatosis in the Asian population was reported to be very low and ethnic heterogeneity has been suspected. The aim of our study was to determine the prevalence of heterozygosity and homozygosity for the C282Y HFE gene mutations in 502 unrelated Koreans. Results revealed that none of them had the mutant gene, suggesting a significant ethnic difference when compared with Caucasians. Our study excluded underlying possibility of hereditary hemochromatosis in Korean which could mimic the findings of alcoholic liver disease with iron overload or liver cirrhosis with chronic hepatitis C.

Outcome of Cardiac Complication in Patients of Kawasaki Disease Treated with High-dose Gammaglobulin

PURPOSE: We performed the study to evaluate the risk factors associated with cardiac complication and the outcome of coronary lesions after high-dose (2g/kg, 1dose) gammaglobulin (IVIG) treatment in patients with Kawasaki disease (KD). METHODS: Retrospective studies were performed on 338 cases of KD treated with high-dose IVIG at this hospital from May 1994 to March 1999. RESULTS: Among 338 patients, fever was persistent for 8.09+/-3.45 days in all patients and after IVIG infusion, fever subsided in 26.5+/-26.4 hours. Forty-five patients (13.3%) with abnormal baseline echocardiogram remained febrile for 9.09+/-4.09 days, significantly longer than normal baseline echocardiogram group. There was no significant differences in age, sex and other clinical findings. In 7 patients (2.1%) usage of medications above 12 months were needed. Eleven cases(3.3%) with persistent fever after their first course of IVIG therapy became afebrile post-second course of IVIG in 5 of the 11 cases with cardiac complication and in 1 case with recurrence. Twelve (male 11, female 1) of the 338 cases recurred after 8.45+/-4.39 months. Three of those 12 cases had cardiac complication. CONCLUSION: The overall prevalence of persistent coronary abnormalities in KD patients treated with high-dose IVIG and aspirin remains low. Even if patients had cardiac complication, they improved later.

Assessment of Ventricular Function Using Myocardial Performance Index in Patients with Atrial Septal Defect

PURPOSE: This study was designed to define normal values for a nongeometric MPI in children and evaluate the utility of m%cardial performance index(MPI) in congenital heart disease with distorted ventricular geometry. METHODS: The study population consisted of 44 normal patients and 28 patients with atrial septal defect(ASD) with dilated right ventricle and paradoxical septal motion. Right ventricular(RV) and left ventricular(LV) isovolumic contraction time, isovolumic relaxation time, ejection time, ejection fraction and pre-ejection period/ejection time(PEP/ET) were measured using conventional echo- Doppler methods. The MPI measures the ratio of total time spent in isovolumic activity(isovolumic contraction time and isovolumic relaxation time) to ejection time. RESULTS: In normal children, the RV MPI was 0.33+/-0.09 and the LV MPI was 0.36+/-0.04. In the ASD group, LV function seemed grossly normal but LV EF was significantly lower than normal children(57+/-3% vs 67+/-4%, P<0.05), but both LV and RV MPI had no statistically significant difference. After correction of ASD, RV MPI was increased and LV EF(57+/-3% vs 64+/-3%, P<0.05) as wll as the RV PEP/ET(0.27+/-0.05 vs 0.38+/-0.06, P<0.05) were significantly greater than the normal or the preoperation group. CONCLUSION: Components of the MPI are easily measured with conventional Doppler technique using standard echocardiographic examination. These results suggest that the MPI is useful as a means of quantitative assessment of ventricular performance in patients with complex ventricular geometry, particularly RV.

A Case Of Cerebellar Hemorrhage Associated with Cavernous Hemangioma and Developmental Venous Anomaly

Developmental venous anomalies are congenital anomalies of the intracranial venous drainage and clinically asymptomatic. Cavernous hemangiomas are vascular malformations composed of dilated vascular channels lined with a single layer of endothelial cells without any intervening normal neural tissue. Although, cavernous hemangioma may be clinically silent, but frequently cause variable neurologic manifestations; intracranial hemorrhage, seizures, and focal neurologic deficits. Frequent association of these two diseases has already been reported and it has been suggested that cavernous hemangiomas not the developmental venous anomalies that cause the acute clinical symptoms. A 4 year old girl with sudden onset of headache, vomiting, gait disturbance and signs of unilateral cerebellar dysfunction 2 days before admission showed a 2.5x2.5cm sized hematoma with heterogenous signal intensity both in CT and MRI in the left cerebellar hemisphere. Four-vessel cerebral angiography revealed characteristic findings of developmental venous anomalies. Pathologic findings of the surgically removed hematoma disclosed typical feature of cavernous hemangioma. Based on the findings in this case and review of the literatures, we concluded that the possibility of other diseases such as cavernous hemangiomas must be considered in patients who has intracranial hemorrhage due to the developmental venous anomalies.

A Case of Plasma Cell Type Giant Lymph Node Hyperplasia (Castleman's Disease) / 대한소아혈액종양학회지

Castleman's disease was first described in 1956 in a group of patients with localized mediastinal lymph node enlargement characterized by hyperplasia of lymphoid follicles and marked capillary proliferation with endothelial hyperplasia. They have been divided into 2 histologic types: the hyaline-vascular type, which was more common and usually asymptomatic, was characterized by small hyaline-vascular follicles and interfollicular capillary proliferation; the plasma-cell type was characterized by large follicles with intervening sheets of plasma cells. Systemic manifestations, such as fever, anemia and hyperglobulinemia, were frequently associated with the plasma cell type. Localized lesions have behaved in a benign fashion, and complete surgical excision has been curative. But recent years, reports have described a multicentric variety with severe systemic manifestations, exorable clinical course and poor outcome. Although Castleman's disease has been described at all ages, the disease is rare in childhood. This paper describes a case of plasma cell type Castleman's disease in a 12-year-old boy and review of the literature. We conclude that the Castleman's disease must be considered in childhood lymphadenopathy and the clinicians should be mindful of the malignant potential of the disease and their possible multicentricity. Appropriate treatment plan, close follow-up and periodic surveillance are necessary.