Intravenous Gammaglobulin Treatment Research Articles

Pharmacogenomics of coronary artery response to intravenous gamma globulin in kawasaki disease

Kawasaki disease (KD) is a multisystem inflammatory illness of infants and young children that can result in acute vasculitis. The mechanism of coronary artery aneurysms (CAA) in KD despite intravenous gamma globulin (IVIG) treatment is not known. We performed a Whole Genome Sequencing (WGS) association analysis in a racially diverse cohort of KD patients treated with IVIG, both using AHA guidelines. We defined coronary aneurysm (CAA) (N = 234) as coronary z ≥ 2.5 and large coronary aneurysm (CAA/L) (N = 92) as z ≥ 5.0. We conducted logistic regression models to examine the association of genetic variants with CAA/L during acute KD and with persistence >6 weeks using an additive model between cases and 238 controls with no CAA. We adjusted for age, gender and three principal components of genetic ancestry. The top significant variants associated with CAA/L were in the intergenic regions (rs62154092 p < 6.32E–08 most significant). Variants in SMAT4, LOC100127, PTPRD, TCAF2 and KLRC2 were the most significant non-intergenic SNPs. Functional mapping and annotation (FUMA) analysis identified 12 genomic risk loci with eQTL or chromatin interactions mapped to 48 genes. Of these NDUFA5 has been implicated in KD CAA and MICU and ZMAT4 has potential functional implications. Genetic risk score using these 12 genomic risk loci yielded an area under the receiver operating characteristic curve (AUC) of 0.86. This pharmacogenomics study provides insights into the pathogenesis of CAA/L in IVIG-treated KD and shows that genomics can help define the cause of CAA/L to guide management and improve risk stratification of KD patients.

Multisystemic Inflammatory Syndrome Temporally Associated with COVID-19 in a Regional Pediatric Hospital from México.

Multisystemic inflammatory syndrome (MIS-C) is an inflammatory condition temporally associated with COVID-19 in children; nevertheless, the clinical and immunologic spectrum of MIS-C is heterogeneous, and its long-term effects are unknown. During the period of August 2020 to December 2021, a total of 52 MIS-C cases were confirmed in pediatric patients from the Hospital del Niño DIF Hidalgo, diagnosed using criteria from the World Health Organization. All patients had serologic IgG confirmation of SARS-CoV2, the mean age of the patients was 7 years, and 94% of the patients did not have a previous underlying disease. In addition to the presentation of lymphopenia, neutropenia, and thrombocytopenia, elevations in D-dimer and ferritin levels were observed in all patients. There was clinical improvement with intravenous gamma globulin and corticosteroid treatment.

Hyaline fibromatosis syndrome with a novel 4.41‐kb deletion in ANTXR2 gene: A case report and literature review

BackgroundHyaline fibromatosis syndrome is a rare autosomal recessive disorder with ANTXR2 mutations characterised by the accumulation of hyaline substances in tissues. We present a case with the severe form—infantile systemic hyalinosis (ISH)—with long survival and review the literature.Methods and resultsTrio‐exome sequencing revealed compound heterozygous mutations, including a novel 4.41 kb deletion on 4q21.21 and the previously reported c.1294C > T mutation, in the ANTXR2 gene. He was diagnosed with ISH and treated symptomatically. After follow‐ups until 4 years of age, his recurrent respiratory infections and diarrhoea improved after one severe diarrhoea attack treated with intravenous gamma globulin. He is now awaiting surgical excision of gingival hypertrophy and joint contractures.ConclusionThe novel gross deletion in ANTXR2 enriches the genetic mutation spectrum of hyaline fibromatosis syndrome. The manifestation of decreased foetal movement, acute‐infection attack or intravenous gamma globulin treatment may be associated with hyaline fibromatosis syndrome. A review of 116 reported cases reveals that missense mutations in the vWA domain are associated with joint symptoms, respiratory tract infection and diarrhoea, while frameshift mutations are associated with facial deformities and speech delays.We have enriched the current knowledge of the clinical manifestations and genetic mutation spectrum of HFS.

Legends of allergy and immunology: Donald Y. M. Leung.

This issue is honoring Donald Leung, who is best known for his work in atopic dermatitis (AD), along with many other contributions in allergy and immunology. Donald was born October 1, 1949 in New York City. He received his PhD in biochemistry and MD at the University of Chicago. He subsequently went on to residency and fellowship at Children’s Hospital Boston where he continued as a faculty member at Harvard Medical School until 1989. With Dr. Raif Geha, they studied the role of Th2 cells in allergic responses and he began work in Kawasaki’s disease and AD. He made many relevant discoveries regarding Kawasaki Disease including its treatment with intravenous gamma globulin treatment for which he received a ten year NHLBI MERIT award.

Analysis of clinical characteristics of the 12 cases of neonatal dengue fever in Guangzhou in 2014 and literatures review

To explore the clinical manifestations and laboratory examination characteristics of neonatal dengue fever. A retrospective analysis of 12 cases of neonatal dengue fever treated in the Guangdong Women and Children's Hospital and Guangzhou Women and Children's Medical Center was conducted, and related literature was reviewed. Twelve cases of neonatal dengue fever included 9 males and 3 females; their age was 30 min after birth to 29 d, the age of onset was 30 min-24 d. (1) CLINICAL FEATURES: fever was present in 11 cases, rash in 6 cases, bleeding in 1 case, jaundice in 5 cases, cough in 1 case, coagulopathy in 2 cases. (2) Mothers' perinatal conditions: 7 mothers were confirmed to have dengue fever, 2 mothers had suspected dengue fever, and in 3 mothers the dengue fever was excluded. Eight mothers prenatally had fever. (3) LABORATORY TESTS: Thrombocytopenia was found in 11 cases (19×10(9)-156×10(9)/L), activated partial thromboplastin time prolonged in 11 cases (44.0-89.8 s), fibrinogen decreased in 5 cases (1.17-3.02 g/L), aspartate aminotransferase (AST) increased in 5 cases (28-78 U/L), creatine kinase (CK-MB) increased in 4 cases (13-86 U/L), hypokalemia in 1 case (2.8-5.1 mmol/L ), C- reactive protein (CRP) increased in 6 cases (0.04-46.05 mg/L). (4) Treatment and prognosis: platelet transfusion was used in 2 cases, anti-infective therapy was given to 6 cases, intravenous gamma globulin treatment was used in 5 cases, hospitalization was 4-17 d, 10 cases were cured, 2 cases were discharged after condition was improved. Literature search was performed with "neonatal dengue" as keywords at Wanfang, Weipu, and CNKI, no relevant reports were found. Pubmed search was done with "neonatal dengue" and "case report" as keywords, 15 reports were retrieved during 1990 and 2014, which reported 30 cases, all acquired the disease via vertical transmission; the main clinical manifestations were fever, rash, petechiae, anemia, jaundice, tachycardia, and hepatomegaly; laboratory test: all had visible purpura due to thrombocytopenia; anti-infective treatment, platelet transfusion and symptomatic treatment were given, and all the 30 cases of infants were cured. The clinical manifestations of neonatal dengue fever are characterized by fever and the disorder of blood coagulation system. The course of disease is mild, lack of specific clinical symptoms, and the prognosis is good. Mother to infant vertical transmission of dengue virus is one of the ways of transmission.

Abstract 83: Usefulness of Serum Immunoglobulin G Levels Calculated in the Acute Phase of Kawasaki disease

Background: Intravenous gamma-globulin treatment (IVGG) is an established therapy for acute phase Kawasaki disease (KD). Since a lot of studies have already testified that the efficacy of IVGG is dose-dependent, a single large dose of IVGG has been accepted as a standard treatment of KD. Some reports described the increasing rate of serum Immunoglobulin G (IgG) after IVGG would have a relationship with efficacy of IVGG. It should, therefore, be very important to compass trends of serum IgG levels in each in KD patient. However, it is unable to frequently measure values of serum IgG in KD patients from the standpoint of medical expenses. If we can estimate IgG levels in KD patients from routine biochemical data without additional costs, that is very helpful to assess inflammatory status in acute phase of KD. Aim to this study: We hypothesized that serum levels of IgG in acute phase of KD patients could be extrapolated by serum values of total protein (TP) and albumin (Alb), and, we tried to establish a simple formula to estimate serum levels of IgG. Materials and methods: We enrolled 42 KD cases (27 ± 21 month, Male 24/Female 18) that were treated in Saga University Hospital, Japan from April of 2010 to August of 2014. All cases met the diagnostic criteria of KD. They were initially treated with IVGG and aspirin or flurbiprofen. 12 patients (28.6%) needed additional treatments such as steroid and cyclosporine A. In this study, we examined relations before and after treatments of serum TP and Alb and IgG in serum of them and tried to establish a simple formula to estimate serum levels of IgG in acute phase of KD. For statistical analysis, we used paired t-test. Results: We found that values obtained by subtracting serum values of IgG and Alb from TP were almost constant through acute phase in each KD patient, regardless of differences in clinical course and therapies. Therefore, we could estimate serum IgG values, once we checked levels of TP, Alb and IgG before treatment. Calculated IgG values were lineally correlated with values of directly measured IgG (P&lt;0.0001, r=0.90). From this finding, we would assess the course of IgG levels during acute phase of KD patients correctly. Conclusion: By estimation of IgG levels, we can grasp inflammatory status of KD and make appropriate therapeutic plans.

Acute Cerebellar Infarction Associated with Intravenous Gammaglobulin Treatment in Idiopathic Thrombocytopenic Purpura

Intravenous immunoglobulins (IVIGs) are used for a variety of immunologic and hematologic disorders. Hemorheologic alteration or the rapid increase of platelet counts by IVIG administration can cause thrombotic adverse events. We present a 58-year-old woman with a previous diagnosis of idiopathic thrombocytopenic purpura who developed cerebellar infarction immediately after IVIG treatment. We discuss a possible role of IVIG in cerebral ischemia and management strategies.

Augmented TLR2 expression on monocytes in both human Kawasaki disease and a mouse model of coronary arteritis.

BackgroundKawasaki disease (KD) of unknown immunopathogenesis is an acute febrile systemic vasculitis and the leading cause of acquired heart diseases in childhood. To search for a better strategy for the prevention and treatment of KD, this study compared and validated human KD immunopathogenesis in a mouse model of Lactobacillus casei cell wall extract (LCWE)-induced coronary arteritis.MethodsRecruited subjects fulfilled the criteria of KD and were admitted for intravenous gamma globulin (IVIG) treatment at the Kaohsiung Chang Gung Memorial Hospital from 2001 to 2009. Blood samples from KD patients were collected before and after IVIG treatment, and cardiovascular abnormalities were examined by transthoracic echocardiography. Wild-type male BALB/c mice (4-week-old) were intraperitoneally injected with LCWE (1 mg/mL) to induce coronary arteritis. The induced immune response in mice was examined on days 1, 3, 7, and 14 post injections, and histopathology studies were performed on days 7 and 14.ResultsBoth human KD patients and LCWE-treated mice developed coronary arteritis, myocarditis, valvulitis, and pericarditis, as well as elevated plasma levels of interleukin (IL)-2, IL-6, IL-10, monocyte chemoattractant protein (MCP)-1, and tumor necrosis factor (TNF)-α in acute phase. Most of these proinflammatory cytokines declined to normal levels in mice, whereas normal levels were achieved in patients only after IVIG treatment, with a few exceptions. Toll-like receptor (TLR)-2, but not TLR4 surface enhancement on circulating CD14+ monocytes, was augmented in KD patients before IVIG treatment and in LCWE-treated mice, which declined in patients after IVIG treatment.ConclusionThis result suggests that that not only TLR2 augmentation on CD14+ monocytes might be an inflammatory marker for both human KD patients and LCWE-induced CAL mouse model but also this model is feasible for studying therapeutic strategies of coronary arteritis in human KD by modulating TLR2-mediated immune activation on CD14+ monocytes.

The clinical response of West Nile virus neuroinvasive disease to intravenous immunoglobulin therapy.

The aim of the study was to determine whether intravenous gamma globulin (IVIG) treatment is effective in patients with West Nile Virus (WNV) neuroinvasive disease.We contacted hospital based infectious disease experts in Israeli hospitals to identify patients with WNV neuroinvasive disease who were treated with IVIG. The main outcome measure was neurological response after treatment. There were 12 patients who received IVIG and four improved within 48 h. Three patients died, 6 had partial recovery, and 3 recovered completely. Eleven of the 12 patients were infected with Israeli genotypes that are highly homologous to Europe/Africa viruses. The rapid response in some patients suggests that IVIG is effective, and might be used to treat patients with WNV neuroinvasive disease with IVIG.

PP44 HYDROPS OF THE GALLBLADDER ASSOCIATED WITH STENOSIS OF THE CYSTIC DUCT OF INDEFINITE NATURE: WHEN IS A MEDICAL OR A SURGICAL APPROACH TO BE CONSIDERED?

Moreover, as in our case, typical signs and symptoms may appear sequentially and not simultaneously. The management of KD is divided according to the clinical stage: in the acute phase the intravenous gamma globulin treatment (IVIG) is the first choice therapy and it must be carry out in the first 10 days of illness. Aspirin: high doses in the acute phase; lower doses in the convalescence. In conclusion, paediatricians must be aware that cholangitis, in absence of any reasonable explanation of the illness, may be an initial presentation of KD, in order to treat the disease and prevent the coronary complications.

Clinical analysis of systemic lupus erythematosus in 13 children with the first symptom of thrombocytopenic purpura

Objective To review the diagnosis and treatment of children with systemic lupus erythematosus. Methods 13 children of systemic lupus erythematosus with acute immune thrombocytopenic purpura as the first symptom were analyzed retrospectively, who were admitted from Jan 2004 to Jun 2009 in our hospital. Results Thirteen cases were diagnosed as acute immune thrombocytopenie purpura according to clinical manifestations and bone marrow examination results. Hormones and intravenous gamma globulin treatment were ineffective. The immune parameters and other clinical manifestations were monitored. According to the characteristics of blood system change and the response to hormone, other immunosuppressive agents such as cyclosporine A ,mycophenolate mofetil and cyclophosphamide were adminitrated. Thirteen children were diagnosed finally as systemic lupus erythematosus at 2 to 24 months from onset. Other immunosuppressive treatment was effective. Conclusion For children with thrombocytopenia, especially adolescent girls, we should pay attention to dynamic monitoring of anti-nuclear antibodies and other immunological parameters,for fear of the possibility of systemic lupus erythematosus. Key words: Systemic lupus enythematosus; Thrombocytopenic purpura; Children

H1N1 Encephalitis with Malignant Edema and Review of Neurologic Complications from Influenza

BackgroundInfluenza virus infection of the respiratory tract is associated with a range of neurologic complications. The emergence of 2009 pandemic influenza A (H1N1) virus has been linked to neurological complications, including encephalopathy and encephalitis.MethodsCase report and literature review.ResultsWe reviewed case management of a 20-year old Hispanic male who developed febrile upper respiratory tract signs and symptoms followed by a confusional state. He had rapid neurologic decline and his clinical course was complicated by refractory seizures and malignant brain edema. He was managed with oseltamavir and peramavir, corticosteroids, intravenous gamma globulin treatment, anticonvulsants, intracranial pressure management with external ventricular drain placement, hyperosmolar therapy, sedation, and mechanical ventilation. Reverse transcriptase polymerase chain reaction analysis of nasal secretions confirmed 2009 H1N1 virus infection; cerebrospinal fluid (CSF) was negative for 2009 H1N1 viral RNA. Follow-up imaging demonstrated improvement in brain edema but restricted diffusion in the basal ganglia. We provide a review of the clinical spectrum of neurologic complications of seasonal influenza and 2009 H1N1, and current approaches towards managing these complications.Conclusions2009 H1N1-associated acute encephalitis and encephalopathy appear to be variable in severity, including a subset of patients with a malignant clinical course complicated by high morbidity and mortality. Since the H1N1 influenza virus has not been detected in the CSF or brain tissue in patients with this diagnosis, the emerging view is that the host immune response plays a key role in pathogenesis.

Necrotizing fasciitis in the head and neck region: an analysis of standard treatment effectiveness

A standard treatment procedure for necrotizing fasciitis in the head and neck region was introduced in 1999 at Rigshospitalet (National Hospital of Denmark) Copenhagen. The new procedure introduced more drastic surgical debridement than before, combined with a set antibiotic regime and intravenous gamma globulin and adjunctive hyperbaric oxygen treatment (HBO). To evaluate the effect of this, a retrospective study was undertaken, involving 19 patients treated for NF at the ENT department from 1996-2004. Between 1996 and 1999 eight patients were treated (non-HBO) from 1999-2004 eleven patients were treated (HBO group). Length of antibiotic treatment was very similar in the two groups (mean 22.5 days) as was bacteriology. Aetiological focus differed marginally with the HBO group showing a clear tendency towards odontogen focus. The HBO group was found to undergo significantly more debridement procedures (3.36). The most drastic difference in the two groups however, was the reduction in mortality. The non-HBO group had a mortality of 75% and in the HBO group they all survived. This obviously resulted in a prolonged hospital stay for the HBO group (mean 30.8 days). The study concluded that the reduction in mortality was due to the combined effects of the different entities in the new treatment guidelines. It was not possible to isolate a specific factor responsible for the change.

Large pleural effusion necessitates chest tube drainage in a patient with Kawasaki disease

An 11-month-old Turkish boy was hospitalised with clinical and roentgen graphic evidence of large pleural effusion on the third day of fever and misdiagnosed as parapneumonic effusion. Due to worsening respiratory distress chest tube drainage was performed. Four days later the classic signs of Kawasaki disease appeared. His clinical condition improved gradually and fever subsided after intravenous gammaglobulin and aspirin treatment. A mild transient dilatation of the right coronary artery was seen and returned to the normal diameter within a few weeks. To our knowledge, large pleural effusion in a case of Kawasaki disease, in which chest tube drainage was needed, has not been reported. We describe here a patient with complete Kawasaki disease whose initial presentation mimicked a parapneumonic effusion.

Intravenous Immunoglobulin for Guillain-Barré Syndrome: How Effective?

Guillain-Barré syndrome is an acute inflammatory demyelinating neuropathy characterized by progressive symmetric polyradiculoneuritis, predominantly manifested by weakness and areflexia. In this article, we report our findings in 25 children treated with intravenous immunoglobulin and compare them with the remaining 30 children who received supportive care only. Only supportive care was given to 30 children who were not able to receive intravenous gammaglobulin because of shortcomings in intravenous gammaglobulin availability owing to a poor import during those years. Twenty-five patients were treated with intravenous gammaglobulin; they received intravenous gammaglobulin 0.4 g/kg/day for 5 consecutive days. Seventeen of the intravenous gammaglobulin group had received intravenous gammaglobulin within 10 days after the first symptoms, and eight of them had received intravenous gammaglobulin after the first 10 days. The average time elapsed for the symptoms to reach the maximum level was 6.9 (range 4-12) days in patients receiving intravenous gammaglobulin in the first 10 days, and it was significantly shorter than the time elapsed for the supportive care group (6.9 versus 8.8 days, respectively) (P < .05). Admission to the hospital after the first symptom, disability grade, time to improve in disability grade, the period of hospitalization, and mortality were not different in the intravenous gammaglobulin and supportive care groups (P > .05). Our suggestion for intravenous gammaglobulin treatment in Guillain-Barré syndrome is that if the patient has risk factors for respiratory insufficiency, then the treatment should be started. We more confidently carry out the follow-up of these patients after the results of this study. In conclusion, although it has been reported that intravenous gammaglobulin facilitates improvement in the disease and the decrease in mortality in children with Guillain-Barré syndrome, it has been mentioned in some studies that the intravenous gammaglobulin treatment was not better than supportive care, as in our study. However, further studies are essential to determine when intravenous gammaglobulin should be given to patients having which clinical and laboratory findings.

Prediction of non-responsiveness to standard high-dose gamma-globulin therapy in patients with acute Kawasaki disease before starting initial treatment

Clinical, laboratory, and echocardiographic data were retrospectively analyzed in 112 patients with acute Kawasaki disease who received high-dose (2 g/kg) intravenous gamma-globulin (IVIG) treatment within 2 days and were compared for those who were responsive and non-responsive to initial IVIG treatment. Coronary arteries adjusted for body surface area (BSA) were evaluated quantitatively by comparison with the mean dimensions for 85 normal control subjects. The incidence of coronary abnormalities was higher in IVIG-non-responsive patients as compared to IVIG-responsive patients (71% versus 5%, p<0.0001). Univariate analysis of pre-IVIG data showed that the neutrophil count and serum levels of C-reactive protein (CRP), total bilirubin (TB), aspartate aminotransferase (AST), alanine aminotransferase, and lactate dehydrogenase (LDH) were significantly higher in IVIG-non-responsive versus responsive patients. Multivariate analysis selected CRP (p=0.009), TB (p<0.001), and AST (p=0.002) as independent predictors of non-responsiveness to initial IVIG treatment. By defining predictive values, patients with at least two of three predictors (CRP>or=7.0 mg, TB>or=0.9 mg, or AST>or=200 IU/L) are considered to be non-responsive to IVIG for acute Kawasaki disease. Alternatively, more intense initial therapy may be a promising therapeutic strategy for patients who are predicted to be IVIG-non-responsive.

Peripheral neuropathy in two patients with rheumatoid arthritis receiving infliximab treatment

Antitumor necrosis alpha agents have been successfully used for the treatment of rheumatoid and seronegative arthritis, Crohn's disease, psoriasis, and severe cases of vasculitis. Several side effects have been observed in patients receiving these agents including hypersensitivity reactions, infections, drug-induced lupus, or demyelinating syndromes. The presence of peripheral neuropathy has been reported only in isolated cases. We describe two cases of peripheral neuropathy which occurred in patients with rheumatoid arthritis receiving infliximab treatment, one with multifocal motor neuropathy with conduction block and another with axonal sensory polyneuropathy, reversed upon discontinuation of infliximab and intravenous gammaglobulin treatment.

Serum homocysteine and tumor necrosis factor-alpha levels after intravenous gammaglobulin treatment in patients with Kawasaki disease

Purpose:Homocysteine is a strong and independent risk factor for cardiovascular disease. The deleterious effects of homocysteine included endothelial dysfunction, arterial intimal-medial thickening, wall stiffness and procoagulant activity. However, the precise mechanism responsible for homocysteine release in children with coronary artery disease is still unknown. The purpose of this study was to investigate serum homocysteine and tumor necrosis factor(TNF)-α levels and identify whether these levels had any association with the development of coronary artery lesions in Kawasaki disease(KD). Methods:Serum homocysteine and TNF-α levels were measured in 24 KD patients(group 1, eight patients with normal coronary artery; group 2, 16 patients with coronary artery lesions) and 21 controls(group 3, 10 afebrile controls; group 4, 11 febrile controls). Blood samples were drawn from each study group before and after intravenous immunoglobulin(IVIG) therapy and in the convalescent stage. Results:The homocysteine levels before IVIG therapy were significantly higher in group 1 than in group 3, and in group 2 than in group 3 and 4. The TNF-α levels before IVIG therapy were significantly higher in group 2 than group 3 and 4. Serum homocysteine and TNF-α levels were highest in group 2 before IVIG therapy. In the acute KD patients, serum homocysteine levels correlated significantly with TNF-α levels. Conclusion:The increased serum homocysteine levels in the acute stage increase the susceptibility to coronary arterial lesions in KD. TNF-α may also play an important role in the formation of coronary arterial lesions in KD

Intravenous gamma globulin is effective as an urgent treatment in Brucella‐induced severe thrombocytopenic purpura

Severe thrombocytopenia is a rare hematologic manifestation of brucellosis, which can occasionally be associated with bleeding into the skin and from mucosal sites. Prompt recognition of this brucellosis complication and aggressive therapy is vital because the mortality rate associated with bleeding into the central nervous system is high. We report a case of a patient infected with Brucella melitensis who was admitted with a severe case of thrombocytopenic purpura. The patient responded well to intravenous gamma globulin (IVIg) treatment with platelet recovery within 2-3 days. For cases of Brucella-induced thrombocytopenic purpura, IVIg may be administered as an urgent therapy until the microbial therapy takes effect.

Initial Intravenous γ-Globulin Treatment Failure in Iranian Children with Kawasaki Disease

The purpose of this study was to determine the initial rates of intravenous gamma-globulin treatment (IVIG) failure in Kawasaki disease (KD) and their predisposing factors. This study was a retrospective analysis of the initial response to IVIG (2 g/kg), assessed from the medical reports of all patients admitted to Namazee Hospital pediatric ward, from March 1998 to March 2002, and who fulfilled the criteria for KD. Data were available for 64 patients, 58 of whom (90.6%) became afebrile 48 hours after completion of the initial dose of IVIG (Group I) and six (9.4%) who remained febrile (Group II). Two patients had a prompt response to a second dose of IVIG. In Group I, five patients (8.6%) developed coronary artery disease, seen on echocardiography. In Group II, two patients (33.3%) developed coronary artery disease. No significant difference was found in the prevalence of coronary artery disease between the two groups (p = 0.12), or in age or gender. The rate of initial treatment failure was 9.4% in this cohort of patients, which is comparable with previous reports. No predictive factors such as coronary artery disease, age or gender were found for initial treatment failure in KD.