Long-term follow-up with stress echocardiograms of patients with Kawasaki's disease

Patients with a history of Kawasaki's disease (KD), particularly those not treated with intravenous gamma-globulin, are at risk of coronary artery aneurysms and later obstruction. Twenty-eight patients with a history of KD (4 had coronary artery aneurysms) were examined with stress echocardiograms. Fourteen patients received gamma-globulin < or =10 days of the onset, 8 patients received gamma-globulin >10 days and 6 received no gamma-globulin. The mean age at diagnosis was 7.2 +/- 4.1 years; the median follow-up was 8.0 +/- 7.4 years. All tests were negative. Using a binomial model, a power of 0.80, a sensitivity of each test of 80% and assuming uniform risk, the individual rate of failure to detect was <7%. At least 640 patients in each group would be needed to detect a difference of 3.5% vs. 7.0% and 184 in each group would be needed to detect a difference of 1.5% vs. 7.5%. We conclude that the probability of an abnormal stress echo in asymptomatic patients with a history of KD is at most 7% and that a more precise determination of the risk of an abnormal stress echo in KD requires a much larger study.     

Kawasaki disease: Medical therapies

Medical therapies in patients with Kawasaki disease (KD) are administered to reduce the prevalence of coronary aneurysms, reduce systemic inflammation, and prevent coronary thrombosis. All patients with acute KD should be treated with intravenous immunoglobulin (IVIG) 2 g/kg, generally administered over 10–12 hours. Aspirin has never been shown to prevent aneurysms, but is given for its anti‐inflammatory and antipyretic effects until the patient has been afebrile for ～2 days, then lowered to an antiplatelet dose. Adjunctive therapy with a longer course of corticosteroids, together with IVIG and aspirin, may be considered for primary treatment in patients at high risk for development of aneurysms. For patients who have persistent or recrudescent fever after IVIG treatment without other explanation, adjunctive therapies include retreatment with IVIG, a tapering course of corticosteroids, infliximab, cyclosporine, cyclophosphamide, and other immunomodulatory therapies. Antithrombotic therapies are tailored to the risk of thrombosis, and range from aspirin alone for 4–6 weeks in children without aneurysms to a combination of anticoagulation and antiplatelet therapy for those with giant aneurysms.     

Kawasaki disease in newborns and infants: refractory forms to immunoglobulin therapy

We studied 52 consecutive patients with Kawasaki disease hospitalized (1984 -2003) during the acute phase (mean age 2.5 + 2.4 years; range 0.3 to 16 years, 34 males, 18 cases with coronary aneurysms, median follow-up 6.7 years), and identified a subgroup presenting a refractory subtype to immunoglobulin therapy. RESULTS: forty-nine infants benefited from a first regimen of immunoglobulins, 8.4 + 6 days following the onset of symptoms. Eleven infants (1.4 + 1.2 years, range 0.3 - 4.3 years, median 1.7 years) were non-responders, with coronary aneurysms in 8 cases (giant aneurysms (>8 mm) in 4 cases). These 11 infants were treated a second time by immunoglobulins, but 6 cases (1.8 + 1.6 years, with two cases of severe ventricular dysfunction and 2 cases of fatal myocardial infarction) required an additive therapy with (oral or IV route) corticosteroids (2) and cyclophosphamide bolus (4) with or without repetitive plasmapheresis (4). Non-responder patients had their treatment onset later (p<0.0003) using higher dosages (p<0.005), a longer delay for fever or biological signs correction (p<0.02), a worsening of coronary lesions (p<0.05) with more coronary secondary aneurysms (p<.005). The aneurysms, more frequent at the second phase of the disease (p<0.0001) are associated with: a younger age (p<0.03), a lower weight (p<0.02), a later onset of treatment (p<0.03), prolonged fever or inflammatory syndrome (p<0.05), higher level of fibrinogene (p<0.02). The overall mortality (5.7%) is correlated with giant aneurysms (p<0.001), myocardial ischemia (p<0.0001), heart failure (p<0.0001), and lack of early response to treatment (p<0.003). CONCLUSION: immunoglobin therapy can be repeated. In case of severe forms, the use of corticosteroids, cyclophosphamide and plasmapheresis may be proposed.     

CORTICOSTEROID PRESCRIBING IN RHEUMATOID ARTHRITIS--THE FICTION AND THE FACT

The results of a postal questionnaire to consultant rheumatologists(80% response rate) suggest that most comply with current teachingregarding the indications for systemic corticosteroids in articularrheumatoid arthritis (RA) They are prescribed as a last resortand most frequently in the elderly. However, a review of 100consecutive RA out-patients revealed 24 patients currently takingcorticosteroids at a mean prednisolone dosage of 5.6 mg daily.Only two had been prescribed these drugs for extra-articularproblems. In 11 cases the treatment was not initiated by a rheumatologist.The discrepancies between the two surveys are discussed. KEY WORDS: Corticosteroids, Prescribing, Rheumatoid arthritis     

Infliximab treatment for refractory Kawasaki disease with coronary artery aneurysm.

Tumor necrosis factor-alpha (TNF-alpha) is considered to be 1 of the factors that induce vasculitis, including coronary artery aneurysm (CA), in Kawasaki disease (KD), because the blood concentration of TNF-alpha is higher in patients with CA compared with those without. Therefore, an anti-TNF-alphaagent (infliximab) was administered to a 1-month-old girl with refractory KD complicated by CA and subsequently, the CA improved and KD was controlled without complications 20 months after the onset.     

The effect of TNFalpha blockade in complicated, refractory Kawasaki disease

In Kawasaki disease (KD), a systemic vasculitis of childhood, serum levels of proinflammatory cytokines such as tumour necrosis factor alpha (TNFalpha) are elevated during the acute phase of the disease. Although the majority of children recover completely from a single dose of intravenous immunoglobulin (IVIG), the treatment is not always effective. In refractory cases of KD there are no documented treatment guidelines. A future role of biological agents directed against proinflammatory cytokines has recently been suggested by the American Heart Association (AHA). We describe two infants with severe KD, complicated by coronary as well as extracoronary aneurysms, who responded neither to repeated treatment with IVIG plus aspirin nor to corticosteroids. The children were subsequently treated with infliximab. In both cases, the effect was prompt and long-lasting. Clinical improvement was seen within a few days after the first dose, and regression of the aneurysms occurred within weeks.     

Percutaneous transcatheter arterial embolization of inferior pancreatico‐duodenal artery aneurysms associated with celiac artery stenosis or occlusion

Objectives: To report our experience with percutaneous TAE of true IPDA aneurysms. Background: Most IPDA aneurysms are ruptured at presentation causing a high mortality risk. Minimally invasive treatment approaches may improve overall outcomes in such patients. Methods: Between 1996 and 2007, seven patients (5 Males; mean age 55y) with symptomatic IPDA aneurysms and severe degree (>75%) celiac artery stenosis were treated with percutaneous TAE. The medical and imaging records were reviewed for demographics, clinical presentation, treatment, complications and follow‐up. Patients presented with epigastric pain (7/7), hemodynamic shock (2/7) and rectal bleeding (2/7). Selective catheter angiography was performed in all patients with the intent to embolize the aneurysms. Results: A total of nine aneurysms were seen in seven patients. Two patients had two aneurysms each. The aneurysms ranged in size from 0.5 to 4.0 cm (mean 1.9 cm). Trans‐catheter coil embolization was successful in 8/9 (89%) aneurysms in 6 patients. Following unsuccessful TAE of one aneurysm in one of the patient, the aneurysm was treated successfully with direct CT‐guided percutaneous transabdominal injection of N‐butyl‐2‐cyanoacrylate. There were no complications on follow up. Angioplasty and stenting of the celiac artery were performed in one patient for complete occlusion. None of the patients developed clinical or imaging evidence of visceral ischemia following embolization. None had recurrent symptoms during clinical follow‐up (median 3 years, range 0.5–13.5 years). Follow‐up CT (Median 6.6 months, range 4 days–11.5 years) in all patients showed no recurrence of the aneurysm. Conclusion: IPDA aneurysms associated with celiac axis stenosis can be successfully treated with percutaneous embolization with minimal recurrence. © 2009 Wiley‐Liss, Inc.     

Corticosteroid therapy for primary treatment of Kawasaki disease – weight of evidence: a meta-analysis and systematic review of the literature

Objective

Corticosteroids are the treatment of choice in most forms of vasculitis. However, their role in the primary treatment of Kawasaki disease (KD) is controversial. Our aim was to conduct a meta-analysis to assess the clinical course and coronary artery outcome of adding corticosteroids to standard therapy [intravenous immunoglobulin (IVIG) + aspirin] in patients with acute KD.

Methods

We included randomised trials comparing the addition of corticosteroids to conventional primary therapy for Kawasaki disease.

Results

A total of four studies were identified, which included 447 patients. The meta-analysis revealed a significant reduction in re-treatments with IVIG in patients receiving corticosteroid plus standard therapy compared with standard therapy alone [odds ratio (OR) 0.48; 95% confidence interval (CI): 0.24– 0.95]. There was however no significant reduction in the incidence of coronary artery aneurysms among patients who received corticosteroid therapy plus standard therapy, compared with standard therapy alone for either up to a month (OR 0.74; 95% CI: 0.23–2.40) or over one month ([OR 0.74; 95% CI: 0.37–1.51). Similarly no significant differences between treatment groups were noted in incidence of adverse events (OR 0.81; 95% CI: 0.05–0.88).

Conclusion

The inclusion of corticosteroids in regimens for the initial treatment of Kawasaki disease decreased rates of re-treatment with intravenous immunoglobulin. However the addition of corticosteroids to standard therapy did not decrease the incidence of coronary aneurysms or adverse events.     

The older patient with syncope: practicalities and controversies

Introduction

Corticosteroid administration in Kawasaki disease (KD) is controversial but accepted as treatment for patients who do not respond to initial treatment. The impact of corticosteroids on evolving coronary artery aneurysms (CAA) and future vascular remodelling is unknown.

Methods and results

The clinical history of 80 patients (73% male; median age at diagnosis 2.2 years) seen from 1990 to 2008 with CAAs after KD were reviewed, 19 (24%) of whom received systemic corticosteroids in the acute phase (14 for ≤ 3 days, 5 for 4+ days). CAA z-scores were assessed at baseline, 2-3 months, and 1 year after the acute phase. Linear regression models adjusted for repeated measures were used to determine the association between change in CAA z-score over time and corticosteroid use, adjusting for patient age at diagnosis, gender, intravenous immunoglobulin use, total days of fever, albumin level, hemoglobin level and platelet count.

Results

The corticosteroid treated group had longer duration of fever in the acute phase (median 17 vs. 11 days, p = 0.04). Adjusted CAA z-scores at diagnosis, 2-3 months and 1 year follow-up for CAA in the left anterior descending decreased (from + 5.5 to + 3.5 to + 1.9) in those not treated with corticosteroids, but progressed for those treated with corticosteroids (from + 7.4 to + 17.5 to + 15.8), regardless of duration of corticosteroid treatment. Similar results were noted for CAA of the right coronary artery and the left main coronary artery.

Conclusions

The use of corticosteroids in the acute phase of KD for patients with evolving CAAs may be associated with worsening involvement and impaired vascular remodelling and warrants further study.     

Arterial lesions in Behçet's disease

BACKGROUND: Arterial involvement is a rare but serious condition in the course of Beh?et's disease. We aimed to assess the results of therapeutic approaches in our patients with arterial lesions caused by Beh?et's disease. PATIENTS AND METHODS: The records of 534 patients with Beh?et's disease between 1987 and 2002 were retrospectively evaluated for the presence of arterial lesions. All patients were followed up regularly at 3 to 6 months intervals. RESULTS: Arterial lesions were diagnosed in 21 (3.9%) patients. Eight of these patients had pulmonary artery aneurysms (PAA), and the other 13 patients had non-pulmonary arterial lesions. Urgent surgical intervention was performed in three patients with PAA leading to death in all three. In addition, three other patients died due to massive haemoptysis at home despite to immunosuppressive therapy. Only two out of eight patients with PAA are still alive who were treated with cyclophophamide and corticosteroids. Thirteen operations were performed in 7 out of 13 patients having non-pulmonary arterial lesions. Although ten of the operations were primary operations, three reoperations had to be performed. A stent-graft was applied for the management of an iliac artery aneurysm in one patient. Only one patient died 8 years after the first non-pulmonary arterial involvement following a type IV thoracoabdominal aortic aneurysm repair. Five patients with arterial occlusive lesions were successfully treated by corticosteroids. CONCLUSIONS: Pulmonary artery aneurysms in Beh?et's disease patients have a poor prognosis despite any form of therapy. High dose corticosteroids alone can be successfully used for isolated non-pulmonary arterial occlusive lesions, unless disabling symptoms occur. Surgery or stent-graft insertion is indicated for non-pulmonary arterial aneurysms because these aneurysms entail high risk of complications.     

Coronary artery aneurysm induced by Kawasaki disease in children show features typical senescence.

BACKGROUND: Kawasaki disease (KD) causes coronary artery disease (CAD) in children. In addition, a history of KD is suspected to be a risk factor for the development of atherosclerotic heart disease in the future. Histological senescence changes are a common denominator in atherosclerotic lesions in adults, so the present study investigated whether histological senescence changes had already occurred in KD aneurysm. METHODS AND RESULTS: KD coronary aneurysms and internal mammary arteries retrieved from 5 children with KD (3, 4, 5, 6, and 11 years old, respectively) who underwent coronary artery bypass grafting, as well as giant coronary aneurysm size-reducing operations, were analyzed. Senescence-associated strong beta-galactosidase activity was observed in KD aneurysms, but not in the internal mammary arteries. An immunohistochemical analysis of the KD aneurysm using anti-CD31, anti-endothelial nitric oxide synthetase (eNOS), anti-vascular adhesion molecule-1 (VCAM-1), and anti-monocyte chemoattractant protein-1 (MCP-1) showed vascular endothelium CD31 staining, decreased staining of eNOS and strong staining of MCP-1 and VCAM-1. cDNA microarray gene expression profiling revealed increased MCP-1 expression in the KD aneurysm, a finding confirmed by quantitative polymerase chain reaction. CONCLUSIONS: Histological features of senescence and active remodeling gene expression show that the KD aneurysm is not a silent vasculitis terminal. The future fate of KD aneurysms, including atherosclerosis, should be monitored carefully.     

Difference Between Persistent Aneurysm,Regressed Aneurysm,and Coronary Dilation in Kawasaki Disease: An Optical Coherence Tomography Study

Background

Coronary artery (CA) aneurysms are a serious complication of Kawasaki disease (KD). Conventional imaging techniques often described segments with regressed aneurysms as normal, whereas studies have shown significant endothelial dysfunction.

Coronary Endothelial Dysfunction After Kawasaki Disease: Evaluation by Intracoronary Injection of Acetylcholine

Objectives. This study sought to assess the endothelial function of long-term coronary artery lesions in patients with Kawasaki disease (KD).Background. The vascular function of the coronary arteries in children with long-term KD remains uncertain. We report our findings of the vascular response of the coronary arteries to intracoronary injection of acetylcholine (ACh) in patients with KD.Methods. A total of 35 patients (25 patients with KD and 10 control subjects) were examined using coronary angiography. Individual arteries were divided into four groups according to the type of the coronary artery lesion: group 1 consisted of 25 sites with regressed aneurysms. These aneurysms had developed in the acute stage but had subsequently regressed and demonstrated normal findings on the follow-up coronary angiogram. Group 2 consisted of 24 sites with persistent aneurysms. Group 3 involved 60 angiographically normal sites in the same patients as those in group 1 or 2. Group 4 consisted of 30 sites in control subjects who had congenital heart disease with normal coronary arteries. During coronary angiography we infused 15 μg of ACh chloride into the coronary artery. The lumen diameters were measured using a cine videodensitometric analyzer to study the distensibility of the coronary artery wall.Results. The mean (±SD) change in diameter was an increase of 11.71 ± 12.34% in group 3 (coronary arteries without lesions in patients with KD) and 12.21 ± 9.71% in the control group, demonstrating marked vasodilation in both groups. In contrast, the changes in the regressed aneurysms of group 1 and in the persistent aneurysms of group 2 were −2.65 ± 12.12% and −0.08 ± 6.51%, respectively, demonstrating no change or mild vasoconstriction. The change in groups 1 and 2 was significantly less than that in group 3 or in the control group. Group 3 showed no significant difference from the control group.Conclusions. These findings suggest that long-term coronary artery lesions, even after aneurysm regression, may have impaired endothelial function. A long-term follow-up study for those patients is essential.     

Aortic aneurysm in patients with autoimmune diseases treated with corticosteroids.

BACKGROUND: Aortic aneurysm is a rare but life-threatening cardiovascular complication in patients with autoimmune disorders. The purpose of this study was to clarify the characteristic clinical features and the pathological mechanism of aneurysmal formation in those patients treated with corticosteroids. METHODS: Among 429 patients operated on for abdominal aortic aneurysm during the past 10 years, six patients with autoimmune diseases treated with corticosteroids (one with progressive systemic sclerosis, one with rheumatoid arthritis and four with systemic lupus erythematosus) were reviewed retrospectively. Their data were compared with those of 391 patients with atherosclerotic aneurysms with no autoimmune disorders. The resected aneurysmal walls of the six patients were also compared histopathologically with those of the last six consecutive patients in the control group. RESULTS: The average age of the patients with autoimmune disease was younger than that of the control group (53.8+/-16.6 vs 71.8+/-7.8 years; p<0.05). Patients with autoimmune disease had received long-term corticosteroid therapy for 15-32 years; mean 22.2+/-6.5 years. Pathological examination showed that the destructive change of the medial elastic lamina in the autoimmune disease group was wider than that in the controls. Most patients had no complications in the postoperative follow-up period (5.1+/-3.2 years), while one patient died of rupture of a dissecting aneurysm two years after operation. CONCLUSIONS: Prolonged corticosteroid treatment probably plays a major role in the disintegration of connective tissue of the media, possibly together with primary aortic wall involvement and/or vasculitic damage in patients with autoimmune disorders, which can result in aortic aneurysmal enlargement.     

Peripheral gangrene associated with Kawasaki disease.

Three American infants with Kawasaki disease (KD) complicated by peripheral extremity gangrene are reported. Eight such patients (only 1 from Japan) have been reported previously. These 11 patients, infants less than 7 months old at onset of KD, are predominantly non-Asian. At least nine had associated giant coronary aneurysms, and eight had associated peripheral arterial aneurysms. In eight infants the diagnosis of KD was not established and therapy was not instituted until greater than or equal to 14 days after onset. Peripheral ischemia initially was noted 15-31 days after onset. Although the pathogenesis of this complication is not well understood, it likely includes some combination of local peripheral arteritis, arteriospasm, thrombosis peripherally and/or more proximally (e.g., in an axillary artery aneurysm), and cardiogenic shock. Treatment may include use of antiinflammatory agents such as salicylates and intravenous gamma globulin, vasodilative agents and/or methods, and thrombolytic and/or anticoagulant agents in an attempt to prevent the potentially devastating consequences of progressive gangrene.     

Stent-graft treatment of infected aortic and arterial aneurysms.

PURPOSE: To evaluate the feasibility and effectiveness of endovascular stent-graft repair of infected aortic and arterial aneurysms. METHODS: Eight patients (5 men; mean age 56.6 years, range 30-85) with infected saccular aneurysms in the brachiocephalic artery (n=1), proximal descending thoracic aorta (n=1), infrarenal abdominal aorta (n=3), common iliac artery (n=1), and common femoral artery (n=2) were treated with stent-graft placement and intravenous antibiotic treatment for at least 6 weeks followed by case-specific administration of oral suppressive antibiotics. All patients were considered to be in the high-surgical-risk group. RESULTS: Exclusion of the infected aneurysm was successful in all patients. However, 2 patients died within 30 days of uncontrolled sepsis, and 1 patient died at 6 months after rupture of a persistently infected aneurysm (37% mortality rate). Over a follow-up that ranged to 8 years, the 5 survivors showed complete resolution of the infected aneurysms; no stent-graft infection was observed during follow-up. CONCLUSION: The acceptable technical and clinical success of endovascular aneurysm repair makes this a promising treatment for infected aortic and arterial aneurysms. However, it is crucial that the infection is treated adequately prior to stent-graft placement.     

Budesonide in the treatment of refractory celiac disease

OBJECTIVE: Corticosteroids are used in patients with refractory celiac disease. In order to minimize their systemic side effects, we assessed the role of a locally active sustained release corticosteroid with minimal systemic bioavailability in patients with refractory celiac disease in an open labeled noncontrolled study. METHODS: Patients who received budesonide for refractory celiac disease were classified according to whether they were primarily or secondarily unresponsive to the diet, and whether they had a polyclonal (type I) or clonal (type II) expansion of intraepithelial lymphocytes. The response to budesonide was assessed globally and by reduction in bowel movements. RESULTS: Patients (N = 29, 72% female) received budesonide for a mean of 6.7 +/- 8.5 months, 5 patients (18%) had type II disease (clonal T-cell population); 76% responded to the medication, 55% completely. Response occurred when budesonide was used alone or with oral corticosteroids and/or azathioprine. There was an objective improvement in the number of bowel movements in those that responded. Response occurred in those with either primary or secondary refractory disease and in those with type II disease, irrespective of the presence of microscopic colitis (N = 7). There was no improvement in the duodenal biopsy over the study period and there were no side effects of budesonide. CONCLUSIONS: Budesonide may be of value in the management of refractory celiac disease.     

Corticosteroid alteration of carboplatin-induced hematopoietic toxicity in a murine model

Corticosteroids exhibit extensive hematopoietic effects both in vitro and in vivo. Some of the previously studied effects suggested that corticosteroids may alter hematopoietic toxicity of chemotherapeutic agents. In this study, we examined (1) the optimum dose and schedule of cortisone acetate (CA) to reduce hematopoietic toxicity of carboplatin (CB) and (2) possible mechanisms involved in this protective effect. CA given subcutaneously at 0.5 mg/d per mouse for 7 days before CB reduced CB-induced mortality due to neutropenia from 88% in controls to 14% in CA-treated mice (P < .05). Lower CA doses were not effective. Three days of pretreatment (but not 1 day) was as effective as 7 days. CA given after CB had no effect on mortality. Pharmacokinetic studies of CA at 0.5 mg per mouse demonstrated blood levels of cortisol achievable in patients (peak level, 82 micrograms/dL). CA treatment markedly reduced spleen cell number and colony-forming units- granulocyte/macrophage (CFU-GM) as well as bone marrow CFU-GM. Bone marrow CFU-GM removed from CA-treated mice demonstrated increased resistance to platinum and increased resistance to high specific activity 3H-thymidine. These findings suggest that treatment of mice with CA induces cellular resistance of hematopoietic precursors to platinum and, thus, reduces CB hematotoxicity. CA or other corticosteroids may be useful in reducing clinical toxicity of CB.     

Efficacy of iodine-123-15-(p-iodophenyl)-3-R, S-methylpentadecanoic acid single photon emission computed tomography imaging in detecting myocardial ischemia in children with Kawasaki disease.

To evaluate its efficacy in detecting myocardial ischemia in children, iodine-123-labeled 15-(p-iodophenyl)-3-R, S-methylpentadecanoic acid (BMIPP) myocardial single photon emission computed tomography (SPECT) imaging was performed in 16 pediatric patients with Kawasaki disease (KD, 11 male, 5 female; mean age and range: 13 years 8 months and 8 years 11 months to 17 years 7 months). Five children with chest pain and no cardiac disease were studied as controls (2 male, 3 female; mean age and range: 13 years 4 months and 9 years 4 months to 17 years 11 months). Selective coronary angiography was also performed in the 16 patients to evaluate the location of coronary stenosis and coronary aneurysms. The SPECT images were expressed as polar maps (Bull's eye maps) and the 'defect' area was defined as where the uptake of BMIPP was less than the standardized BMIPP images of the 5 control children. In the 16 patients, 33 segments had coronary aneurysms and 10 (10/33: 30.3%) had significant coronary stenosis on selective coronary angiography. Nine of the 10 (90%) segments with significant coronary stenosis showed a defect on the BMIPP image whereas only 6 of the 23 (26.1%) segments without coronary stenosis showed a defect on BMIPP imaging. The sensitivity of BMIPP SPECT imaging for detection of coronary stenosis was 90% (9/10) and its specificity was 73.9% (17/23), whereas the sensitivity of (201)Tl SPECT imaging was 80% (8/10) and its specificity was 60% (14/23). There was no significant difference between the BMIPP and 201Tl SPECT images in either the sensitivity or specificity for the detection of coronary stenosis. In the present series, only one case had discordant BMIPP uptake (BMIPP uptake < (201)Tl uptake) in which there was a large coronary aneurysm and re-canalization after complete obstruction at segment 1 of the right coronary artery. This discordant BMIPP uptake reflects the possibility of ischemic but viable myocardium after re-canalization of a large aneurysm in KD. In conclusion, BMIPP SPECT imaging is useful for detecting the areas of ischemic myocardium caused by coronary artery stenosis in children with KD.     

Corticosteroid therapy for liver abscess in chronic granulomatous disease

Liver abscesses in chronic granulomatous disease (CGD) are typically difficult to treat and often require surgery. We describe 9 X-linked CGD patients with staphylococcal liver abscesses refractory to conventional therapy successfully treated with corticosteroids and antibiotics. Corticosteroids may have a role in treatment of Staphylococcus aureus liver abscesses in CGD.