Administration Of Intravenous Gamma Globulin Research Articles

Deficiência predominantemente de anticorpos: relato de caso

Abstract Objectives: present a case of Inborn errors of immunity (IEI) as a potential diagnosis in pediatric patients with recurrent infections. Description: male patient, 13 years old, since he was eight months old had recurrent diarrhea, sinusitis, otitis, abscesses and urinary tract infections. At the age of ten, he presented mastoiditis progressing to meningitis, he was admitted to a tertiary hospital, where an immunological evaluation was performed, which led to the diagnosis of Predominantly Antibody Deficiency (PAD), with suspected X-linked Agammaglobulinemia (XLA). Treatment was initiated with administration of intravenous gamma globulin 400 mg/kg every four weeks, with a significant improvement of the condition. Discussion: usually, the diagnosis of XLA tends to be made in the first three years of life. However, in this report, although the first manifestations started at eight months of age, there was a delay of ten years before starting the treatment. In fact, the diagnosis of children and adults with IEI can be delayed if healthcare professionals are unable to find the true cause of recurrent infections. Therefore, the relevance of considering such pathologies in the presence of risk signs is highlighted, as early diagnosis being essential in treating and preventing morbidities.

Neonatal Kawasaki disease with multiple arterial aneurysms: a case report

BackgroundKawasaki disease (KD) is a medium vessel vasculitis that typically occurs in children aged between 6 months and 5 years. It is extraordinarily rare in the neonatal period. KD-related systemic artery aneurysms (SAAs) have never been reported in neonates.Case presentationA male infant was transferred to our institution for persistent high-grade fever lasting 16 days. Symptoms started at day 14 of life, and he was admitted to a children’s hospital on the second day of fever. Physical examination at the time found no signs suggestive of KD. The only laboratory parameters which were of significance were values suggestive of systemic inflammation. However, his fever persisted and inflammatory markers continued to rise despite 2 weeks of antibiotic therapy. KD as a noninfectious cause of fever was considered when he came to our institution, and echocardiographic findings of left and right medium coronary artery aneurysms (CAAs) confirmed our suspicions. Full-body magnetic resonance angiography also revealed bilateral axillary artery aneurysms. Administration of intravenous gamma globulin resulted in rapid improvement. His fever resolved on the next day and CAAs and SAAs regressed to normal at 6 months and 3 months after diagnosis, respectively.ConclusionThis unique case of incomplete KD highlights the importance of considering KD in neonates with unexplained prolonged fever and reinforces the need to remain vigilant for SAAs in KD.

Clinical Features of Demyelinating Lesion in Medulla Oblongata and Cervical Cord Junction With the First Symptom of Neck Pain Misdiagnosed as Acute Stroke

This article aims to describe the clinical features of a demyelinating lesion in the medullary and cervical spinal cord junctions with a first symptom of neck pain misdiagnosed as acute stroke. The patient was initially misdiagnosed as ischemic stroke through stroke-like onset, clinical manifestations, physical examination, and brain computed tomography (CT); but the patient’s symptoms were not completely consistent with typical physical examination of stoke. Combined with brain magnetic resonance imaging (MRI) which showed demyelinating lesions and diagnostic treatment, the final diagnosis was the demyelinating lesion of the medulla oblongata and cervical cord junction. Acute hemiplegia in quiet state is not necessarily a stroke. Detailed physical examination and imaging data are helpful for differential diagnosis. Diagnostic treatment can help to confirm the diagnosis if necessary. Early use of methylprednisolone shock therapy combined with intravenous administration of gamma globulin for demyelinating disease can achieve good results, save limb function; and long-term medication is required to prevent rebound. J Med Cases. 2019;10(3):89-92 doi: https://doi.org/10.14740/jmc3270

Abstract O.14: Incidence rate of recurrent Kawasaki disease in Japan (2003-2012)

To investigate the incidence of recurrent Kawasaki disease, data of nationwide surveys of Kawasaki disease in Japan from January 2003 (18th survey) to December 2012 (22th survey) are analyzed. Our group previously reported the incidence rate of recurrent Kawasaki disease (Nakamura et al., 1994 and Hirata et al., 2001). In the current study, 104,751 patients with 498,388.9 person-years are observed to calculate the rate of recurrence. The incidence rate of recurrence is 4.14 per 1,000 person-years, with a high incidence within the 12 months from the first episode. The observed person-year categorized by some variables is calculated, such as sex, age at first episode, period of time after the initial episode, presence of cardiac sequelae in the acute (within 2 months) and late (after 2 months) stage, exposure to intravenous gamma globulin (IVGG) therapy, presence of additional IVGG administration, and exposure to steroid therapy. Using those data, incidence rate of recurrent Kawasaki disease and incidence rate ratio are calculated according to such variables, and potential risk factors for recurrence of Kawasaki disease are discussed.

Three Cases of Passively Acquired anti-GAD Antibody Following Administration of Intravenous Gamma Globulin

Three Cases of Passively Acquired anti-GAD Antibody Following Administration of Intravenous Gamma Globulin

Immediate Relief of Mycoplasma pneumoniae Encephalitis Symptoms After Intravenous Immunoglobulin

Mycoplasma pneumoniae may cause acute encephalitis, resulting in severe neurologic complications despite antibiotic therapy. We report the case of a 12-year-old patient who presented with acute onset of orofacial tics, motor restlessness, compulsive behavior, and cerebellar symptoms. Cerebrospinal fluid examination demonstrated lymphocytic meningitis. Polymerase chain reaction for M. pneumoniae was strongly positive in the cerebrospinal fluid. Blood and cerebrospinal fluid were negative for M. pneumoniae antibodies (immunoglobulin M and immunoglobulin G). The child was administered intravenous gamma-globulin, which led to a dramatic improvement of her clinical condition and disappearance of the symptoms within 72 hours. This novel case points to the potential value of gamma-globulin in M. pneumoniae encephalitis confirmed with polymerase chain reaction and suggests that immediate administration of intravenous gamma-globulin in suspected mycoplasma encephalitis should be investigated in a larger patient cohort.

Hemorheological changes after intravenous gammaglobulin administration in patients with neurological disorders

Hemorheological changes due to intravenous gammaglobulin (IVIg) administration have been considered to influence blood viscosity and this way the blood flow in microcirculation. The study was carried out in the group of 10 patients with various neurological disorders (seven with polyradiculoneuropathy, two suffering from myasthenia and one with multiple sclerosis). Patients were treated routinely with intravenous gammaglobulin infusion (Sandoglobulin, Sandoz, 24 g a day in the course of 5 days therapy). The following hemorheological factors were estimated: relative blood viscosity, plasma viscosity, red cell deformability and erythrocytes aggregation. For rheological examination the microviscosimeter Low Shear 40 (Contraves) was used. Each patient was examined two times: before treatment initiation and at the end of therapy after five days. At the comparison of first and last measurements a significant increase of plasma viscosity (p<0.04) was found, whereas erythrocyte deformability was significantly improved (p<0.05). The value of relative blood viscosity at shear rate of 0.1 s(-1) was significantly decreased (p<0.05) and statistically unchanged at other studied shear rates. The results suggest an existence of a protective feedback mechanism in the studied group of patients which has been exemplified by the red cell elasticity improvement.

Requirements for Physician Competencies in Allergy: Key Clinical Competencies Appropriate for the Care of Patients With Allergic or Immunologic Diseases: A Position Statement of the World Allergy Organization

Allergic diseases are quite prevalent worldwide, and the incidence of allergy is increasing everywhere [1-7]. Because allergic and immunologic processes overlap all organ systems, allergy is not always taught in medical schools as a separate subject. Indeed, lack of recognition of the specialty and of the need to teach about allergic and immunologic diseases results in allergy not being included at all in some medical school curricula [8]. With an estimated 22% of the global population experiencing allergic and immunologic diseases, it is time to recognize and strengthen education in allergy and immunology [8]. The World Allergy Organization (WAO), an alliance of 74 national and regional allergy societies, created this consensus document to establish educational guidelines for worldwide application to help identify and correct allergy education and training deficiencies and to define appropriate competencies. In creating this consensus, it is recognized that each country has its own principles and goals in medical education at the undergraduate and postgraduate levels. This document defines what WAO considers medical practitioners should know to care appropriately for allergic patients.

Update on the treatment of primary immunodeficiencies

A general review of advances in the treatment of Primary Immunodeficiencies (PID) has been performed. Treatment with immunoglobulins is indicated in cases of humoral immunodeficiencies and in selected cases of combined immunodeficiencies. The use of intramuscular immunoglobulins in the treatment of PID was abandoned after obtaining the intravenous immunoglobulins, since these are much more effective and have fewer adverse effects. Now subcutaneous immunoglobulins are also available. Immunoglobulins help to keep the patients free of symptoms and infections as these substances are able to neutralise infectious agents, modulate and promote the immune response and favour phagocytosis. Adverse effects have been reported in 5-15% of patients receiving IVIg, and patients with deficiencies of subclasses of IgG with IgA deficiency and/or anti-IgA antibodies are at risk of severe reactions. No severe adverse effects of subcutaneous immuneglobulins have been reported and the medication can be self-administered. The efficacy and safety of IVIg and SCIg are similar and SCIg administered at home is associated with better quality of life. Stem Cell Transplantation (SCT) in Primary Immunodeficiencies is aimed at restoring the number and/or function of lymphocytes or phagocytes. Matched, related or unrelated donors, or related haploidentical donors are selected. HLA class II mismatched unrelated donors are avoided owing to the risk of severe graft versus host disease (GVHD). Stem cells are obtained from bone marrow, cord blood or peripheral blood. Prophylactic immunossupression (as well as donor T lymphocyte depletion in haploidentical and unrelated donors) is performed to avoid or minimize GVHD. Less toxic “reduced intensity” protocols now exist for pre-transplantation conditioning, indicated to avoid graft rejection if there is residual T-lymphocyte immunity in the host. In the majority of Severe Combined Immunodeficiencies (SCID), SCT results in T lymphocytes graft and the antibody immunodeficiency persists in many cases. The results are better the earlier it is performed, with the absence of previous infections, and with the degree of matching. The patient must be maintained in a laminar flow room with broad anti-infectious prophylaxis and with the intravenous administration of gammaglobulin for a variable period. Many other complications can be expected. Gene therapy. Patients with PID are ideal candidates, as they are monogenic, the haematopoietic cells are easily obtained and virus replication is easy within them. Vectors (viruses) “infect” the stem cells of the patient's bone marrow, producing the transfection of the wild (healthy) gene in these cells. Encouraging results have been achieved in X-linked SCID as there are a number of patients who are considered “cured”, although neoplastic processes have occurred due to the activation of proto-oncogenes close to the point of insertion of the external gene, using retroviruses as vectors; there are now trials with adenovirus, physical methods (direct injection…) and chemical methods (viral modification, artificial viruses…). Gene therapy has also been performed in patients with Chronic Granulomatous Disease and trials will improve in the future with changes in protocols used in oncology and infectious diseases.

Low-dose cyclosporine A in a patient with X-linked immune dysregulation, polyendocrinopathy and enteropathy

Successful treatment with oral low-dose cyclosporine A given as a single daily dose is reported in a Japanese patient with immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome. Immune dysregulation, polyendocrinopathy and enteropathy is a rare and potentially fatal X-linked disease (IPEX, OMIM 304790). It is characterised by multiple autoimmune disorders in infancy, that warrants long-term aggressive immunosuppressive therapy [2, 3,6]; however, prolonged use of immunosuppressants may lead to direct toxicity [3]. We previously reported a Japanese infant with IPEX who presented with a novel mutation of the FOXP3gene [5]. The patient was successfully treated by early initiation of oral low-dose cyclosporine A (CsA) given as a single daily dose combined with low-dose prednisolone; for over 3 years he remained free of the both IPEX signs and therapyrelated toxicity. The patient reported here presented with severe secretory diarrhoea at the age of 2 months, and was diagnosed as having IPEX, based on the duodenal biopsy findings (total villous atrophy with crypt hyperplasia), presence of circulating anti-enterocyte antibody (by the immunohistochemical method using the patient’s serum and normal human ileal tissue: when the patient’s serum or control sera were applied to normal mucosa, only the patient’s serum stained the brush border of ileum epithelial cells) and results of sequence analysis of the FOXP3gene: a novel mutation of T1117G substitution in exon 10 [5]. Complete remission was achieved within a short time following prompt initiation of low-dose CsA (2 mg/kg, single daily dose, initially administered intravenously and later switched to oral administration of Neoral before breakfast), combined with prednisolone (0.5 mg/kg, daily). The peak blood levels of oral CsA (1 h post dosing) were maintained at around 700–800 ng/ml, while the trough blood levels of the drug were less than 25 ng/ml. No extra-intestinal signs were encountered, such as type 1 diabetes mellitus, pancreatitis, thyroiditis, haemolytic anaemia, glomerulonephritis or atopic dermatitis. The patient remained reasonably healthy thereafter and developed well under treatment with low-dose oral CsA given as a single daily dose and prophylactic intravenous gamma-globulin administration (200 mg/kg, administered monthly). The prednisolone dose could eventually be tapered to 0.25 mg/kg, daily.

A systematic review of intravenous gamma globulin for therapy of acute myocarditis

BackgroundIntravenous gamma globulin (IVGG) is commonly used in the management of acute myocarditis. The objective of this study was to systematically review the literature evaluating this practice.MethodsWe conducted a comprehensive search (electronic databases, trials registries, conference proceedings, reference lists, contact with authors) to identify studies evaluating the use of IVGG in adults and children with a clinical or histologically proven diagnosis of myocarditis of possible viral etiology and symptoms of less than six months duration. Two reviewers independently screened the searches, applied inclusion criteria, and graded the evidence.ResultsResults were described qualitatively; data were not pooled because only one randomized controlled trial (RCT) with 62 patients was identified. The RCT showed no benefit with respect to cardiac function, functional outcome, or event-free survival. A small, uncontrolled trial (n = 10) showed significant improvement in LVEF from a mean of 24% to 41% 12 months after IVGG in nine survivors. A retrospective cohort study of pediatric patients showed improvement in cardiac function and a trend towards improved survival in patients receiving IVGG (n = 21) versus historic controls (n = 25). Ten case reports and two case series (total n = 21) described improvement in cardiac function after administration of IVGG; two case reports showed no benefit of IVGG. One case of hemolytic anemia was attributed to IVGG.ConclusionThere is insufficient data from methodologically strong studies to recommend routine use of IVGG for acute myocarditis. Future randomized studies that take into account the etiology of acute myocarditis will be required to determine the efficacy of IVGG.

Intravenous gamma-globulin therapy improves hypercytokinemia in the acute phase of Kawasaki disease

Proinflammatory cytokinemia and subsequent endothelial cell activation are the major pathological features of Kawasaki disease (KD), which progresses to systemic vasculitis and results in coronary artery lesions (CALs). We studied the serum levels of proinflammatory cytokines and of the soluble receptors before and after intravenous gamma-globulin (IVGG) treatment to investigate whether the anti-inflammatory effect of IVGG was due to the reduction of increased serum levels of their cytokines and soluble receptors. In the acute phase of KD, the serum levels of interleukin (IL)-2 and IL-5, as well as those of IL-6, IL-10, and interferon-γ, were markedly higher than those in controls. The level of tumor necrosis factor-α was higher than that in the control, but the difference was slight and not significant. The soluble IL-6 receptor levels were lower than those of the controls. After IVGG administration, the increased levels of these cytokines and soluble receptors were abruptly down-regulated to within their normal ranges. The patients enrolled in the present study were all effectively treated with IVGG, without a steroid, and improved without any residual CALs. Overall, IVGG administration to patients with KD was found to be effective in reducing inflammatory processes and in preventing CALs, and was followed by reduction of the serum levels of the proinflammatory cytokines and their receptors.

Meta-analysis of the results of intravenous gamma globulin treatment of coronary artery lesions in Kawasaki disease

The objective of this study was to evaluate the efficacy of intravenous gamma globulin (IVGG) therapy on the prevention of coronary artery lesions (CALs) in patients with Kawasaki disease (KD), with reference to the literature on meta-analyses in randomized controlled studies. Studies from 1984 to the end of 2000 obtained from the National Library of Medicine or from the bibliographies of these articles were used in the analysis. The total number of patients with KD covered in 17 articles was 4020. All the articles were examined for the number of doses per day, the duration of administration, and the total number of IVGG doses. The number of patients in each group was counted, and the incidence of CALs was evaluated at 30 or 60 days after onset. The results of these searches were further analyzed by meta-analytical methods. The administration of IVGG significantly decreased the incidence of CALs in a dose-dependent manner: At 30 days after onset the incidence of CALs was 29.4% without IVGG but 21.6% with a total dosage of IVGG < 1000 mg/kg, 10.8% with a total dosage of 1000–2000 mg/kg, and 10.2% with a total dosage of ≥2000 mg/kg. Compared with the incidence of CALs without IVGG, the odds ratio (OR) was 0.662 [95% confidence interval (CI) 0.519–0.815)] at <1000 mg/kg, 0.292 (95% CI 0.222–0.371) with 1000–2000 mg/kg, and 0.274 (95% CI 0.207–0.349) with ≥2000 mg/kg. At 60 days, the values had decreased to 17.3%, 13.8% [OR 0.767 (95% CI 0.585–1.005)], 5.8% [OR 0.296 (95% CI 0.200–0.436)], and 4.9% [OR 0.244 (95% CI 0.170–0.349)], respectively. The meta-analyses also indicated that high doses of IVGG (≥2000 mg/kg per day) given in a single dose prevented CALs more effectively than the same dosages divided into five daily doses in the patients with KD: The incidence of CALs at 30 days after disease onset was 2.4% with a single dose versus 12.9% with divided doses. Compared with divided doses, the OR with a single dose was 0.164 (95% CI 0.064–0.393) and 2.8% versus 6.1% at 60 days [OR 0.450 (95% CI 0.206–0.956)]. We clearly confirmed that higher doses of IVGG (≥2000 mg/kg per day) administered in a single infusion were more effective for preventing CALs, as evaluated during both the subacute and convalescent phases of KD.

Giant coronary aneurysms due to Kawasaki disease: A case−control study

Epidemiologic features of giant coronary aneurysm due to Kawasaki disease and its risk factors are still not clear. Sixty-six patients with giant coronary aneurysms were reported to a 15th nationwide survey of Kawasaki disease in Japan. With all other patients treated in the same hospital as a control group, odds ratios were calculated for certain potential risk factors. Infant males aged less than 1 year,neutrophil concentration among leukocyte, late administration of intravenous gammaglobulin (IVGG) therapy and additional administration of IVGGwere considered as risk factors of giant coronary aneurysms due to Kawasaki disease. In univariate analysis, use of IVGG therapy and a large amount of IVGG (2500+ mg/kg)elevated the risk, whereas the relationship disappeared after the adjustment. The observation of 66 cases with giant coronary aneurysms due to Kawasaki disease reported to the nationwide survey provides some risk factors and consideration about the aneurysms.

False positive serologic tests after administration of intravenous gamma globulin: case report and institutional quality management resolution

A quality management program was designed to eliminate false positive serological testing after administration of intravenous gamma globulin (IVIgG). The intervention and follow‐up chart review was performed in a tertiary care teaching hospital. After the index false positive test was reported, a multidisciplinary team sent memos to all affiliated physicians and devised a warning label to use in the patient charts after IVIgG administration. After the intervention, follow‐up of 28 patients who received a total of 162 doses of IVIgG revealed that five had at least one serologic test ordered which might have produced a false positive. However, chart review suggested that these serologic results did not influence patient treatment. Serological testing after IVIgG administration can generate erroneous results that can lead to costly delays in diagnosis and treatment. Process control actions can proactively foster proper laboratory ordering practices and interpretation.

Respiratory disorders in common variable immunodeficiency

Common variable immunodeficiency (CVID) is a heterogeneous immunodeficiency syndrome characterized by hypogammaglobulinemia, recurrent bacterial infections, and various immunologic abnormalities. The clinical presentation is generally that of recurrent pyogenic sinopulmonary infections.Our objectives were to study the prevalence of lung involvement and the response to intravenous immunoglobulin replacement therapy in 19 patients with CVID. Nineteen patients (12 men) with a mean age (SD) of 33¶1 (17¶1) years had a previous diagnosis of CVID and were treated with intravenous immunoglobulin replacement. All patients underwent complete pulmonary function tests and high-resolution computed tomography (HRCT) examination. Bronchiectasis was diagnosed in 11 (58%) patients and eight (42%) were multi-lobar bronchiectasis. Chronic airflow limitation (CAL) was present in 10 (53%) patients and a restrictive pattern was seen in one case. Eleven patients (58%) presented a decrease in single-breath carbon monoxide diffusing capacity of the lung (DLco). Before intravenous immunoglobulin replacement therapy (INIRT), 84% of patients had suffered from at least one episode of pneumonia. Episodes of lower respiratory tract infection decreased significantly from 0¶28 per patient and year before replacement therapy to 0¶16 per patient and year after treatment. The mean duration of replacement therapy was 7¶5 years.In conclusion lung involvement was frequent in patients with CVID. Long-term administration of intravenous gammaglobulin resulted in a substantial reduction of pneumonic episodes.

Cerebrospinal fluid profile in patients with acute Kawasaki disease.

Kawasaki disease (KD) is an acute vasculitis of infancy and early childhood for which there is currently no diagnostic test. The clinical presentation of KD may initially resemble other infectious diseases, including bacterial or viral meningitis. For this reason lumbar puncture (LP) is sometimes performed during the evaluation of these patients. To understand the range of cerebrospinal fluid (CSF) changes that may be associated with acute KD, a retrospective review of unselected KD patients from three pediatric centers was performed. Retrospective chart review was performed on KD patients evaluated during the first 10 days of illness who had an LP performed before the administration of intravenous gamma-globulin. During the 6.5-year study period, 46 KD patients underwent LP as part of their clinical evaluation. Of these patients 18 (39.1%) had CSF pleocytosis, 1 (2.2%) had a CSF glucose <45 mg/dl and 8 (17.4%) had an elevated CSF protein. Of the patients with CSF pleocytosis, the median white blood cell count was 22.5 cells (range, 7 to 320 cells), with a median of 6.0% neutrophils (range, 0 to 79%) and 91.5% mononuclear cells (range, 11 to 100%). In the present series approximately one-third of KD patients who underwent an LP had CSF pleocytosis with a mononuclear cell predominance. No patient had significant hypoglycorrhachia, and elevation of the CSF protein was uncommon. CSF abnormalities were similar between US and Japanese KD patients. The basis for the CSF pleocytosis in acute KD patients remains unknown.

Mucocutaneous manifestations of the hyper-IgM immunodeficiency syndrome

Background: The recurrent pyogenic infections of patients with hyper-IgM syndrome are controlled by intravenous gamma globulin administration, but patients may suffer from early-onset oral ulcerations and warts. Objective: We have characterized the mucocutaneous manifestations associated with this condition to allow physicians to more readily identify it. Methods: Three male patients with the mucocutaneous manifestations of the hyper-IgM syndrome are described. In one, histopathologic examination of the oral mucosal lesion was performed. Results: Recurrent large, painful oral ulcerations can occur that are not necessarily associated with neutropenia nor do they respond to granulocyte colony-stimulating factor administration. Histopathologic examination of an ulcer showed a heavy infiltrate of mixed inflammatory cells. Warts tend to be widespread and resistant to traditional therapy. Conclusion: Physicians should consider this uncommon condition when examining a male patient with severe oral ulcers or recalcitrant widespread warts. (J Am Acad Dermatol 1998;38:191-6.)

The absence of evidence of staphylococcal toxin involvement in the pathogenesis of Kawasaki disease.

To detect a causative superantigen and to clarify a possible role for staphylococci in Kawasaki disease (KD), culture supernatants of individual bacterial isolates from 11 acute-stage patients were studied. Toxic shock syndrome toxin-1 (TSST-1) and antibody to TSST-1 and enterotoxins A (SEA), B (SEB), and C (SEC) in acute (mean, day 7) and late convalescent (mean, month 15) sera from 26 patients (12 with coronary artery aneurysms) and 22 age-matched controls were measured. Only 1 of 60 supernatants was mitogenic for human lymphocytes; it was 1 of the 4 Staphylococcus aureus isolates. Mitogenicity was neutralized by sera obtained after administration of intravenous gamma globulin (mean, week 4) but not by late convalescent sera. TSST-1 was detectable in 2 of 26 acute sera and 1 of 22 control sera. No KD but 1 control serum had IgM to TSST-1. IgG seroconversion rates to TSST-1, SEA, SEB, and SEC were 10%, 15%, 21% and 16%, respectively. These data do not support the involvement of toxin-producing staphylococci in KD.

1002-91 Plasma Endothelin-1 in Acute Phase of Kawasaki Disease Complicated with Coronary Aneurysm

There are some cases of Kawasaki disease (KD) complicated with coronary aneurysm (CA) in spite of intravenous administration of gammaglobulin (IVGG). We prospectively tested whether plasma endothelin-1 (ET) could be a predictive factor of CA. This study consisted of 60 patients in acute phase of KD. There were 34 male subjects and 26 female subjects aged 4 months-9years. All patients were proved normal coronary arteries by echocardiography when hospitalized before treated with oral aspirin and IVGG therapy. 9 patients were complicated with CA revealed by echocardiography every 2nd or 3rd day. Blood samples for ET were obtained from veins during febrile period before 5th day in hospital and in afebrile period. ET was measured by radioimmunoassay by using ET antibody. Normal range of ET measured by this method is 4.0 pg/ml (0–1 year) or 2.0 pg/ml (2years or older). We evaluated corrected ET (ratio of measured ET to normal upper limit for age). In patients with intact coronary artery, corrected ET were 0.96 ± 0.47 (febrile period) and 0.59 ± 0.22 (afebrile period). In patients complicated with CA, corrected ET were 1.63 ± 0.50 (febrile; p &lt; 0.05) and 1.45 ± 0.38 (afebrile; p &lt; 0.01) and all of them with CA sustained higher ET level than normal range until afebrile period. Specificity of prediction for CA was 66.7%, sensitivity of it was 100% as result. We supposed that plasma ET is a good predictive factor of CA in KD.