Evaluation of left ventricular function in patients with mitral annular disjunction using speckle tracking echocardiography.

BACKGROUND: Mitral annular disjunction (MAD) is a structural abnormality characterized by the systolic detachment of the posterior mitral annulus and the ventricular myocardium. It is usually observed coexistent with mitral valve prolapse (MVP) and associated with a mechanical dysfunction despite preserved electrical isolation function of the mitral annulus. This study aimed to evaluate left ventricular (LV) function using speckle tracking echocardiography in MVP patients with MAD. METHODS: This study was designed as a prospective, single-center study including 103 patients with MVP and 40 age- and sex-matched control subjects. Transthoracic echocardiography and cardiac magnetic resonance imaging were performed to assess LV function and MAD presence. RESULTS: MAD (+) MVP (n = 34), MAD (-) MVP (n = 69), and control (n = 40) groups were enrolled in the study. Among the MVP patients, 34 (33%) had MAD. T-negativity in the inferior leads on electrocardiography was more frequent in the MAD (+) group than in the MAD (-) patients (4.3% vs. 20.6%, p = .014). Mitral regurgitation degree, Pickelhaube sign (17.6% vs. 1.4%, p = .005), and late gadolinium enhancement frequency (35.3% vs. 10.6%, p = .002) were significantly higher in MAD (+) patients. MAD (+) patients had significantly impaired global longitudinal strain (-23.1 ±  2.1 vs. -23.5 ± 2.3, p < .001), basal longitudinal strain (BLS) (-19.6 ±  1.5 vs. -20.5 ± 1.9, p < .001), Mid-Ventricular Longitudinal Strain (-22.2 ± 1.7 vs. -23.2 ± 2.2, p < .001) and LA strain (-24.5 ± 3.9 vs. -27.2 ± 3.6, p < .001) when compared to MAD (-) MVP patients, despite similar LV ejection fraction. All these values of MVP patients were also significantly lower than the control group. The mean MAD distance was 7.8 ± 3.2 mm in MAD (+) patients. Patients with two or more symptoms were higher in the MAD (+) group than in the MAD (-) group (4.3% vs. 44.1%, p < .001). CONCLUSION: This study demonstrated a significant decrease in longitudinal strain in MVP patients with MAD, indicating myocardial dysfunction. These findings suggest that MAD may contribute to LV dysfunction and highlight the importance of early detection in younger patients. Further research is needed to explore the functional implications and long-term outcomes of MAD.

Potential renoprotective effect of SGLT2 inhibitors against contrast-induced AKI in diabetic STEMI patients undergoing primary PCI.

BACKGROUND: It has been demonstrated that there is a significant reduction in the incidence of cardiovascular events, mortality rates, and worsening kidney disease in patients using sodium-glucose cotransporter 2 inhibitors (SGLT2i). However, there is limited information about the effect of SGLT2i on the incidence of contrast-induced acute kidney injury (CI-AKI) in patients undergoing primary percutaneous intervention (pPCI). AIMS: Our research was focused on examining how SGLT2i exposure impacts CI-AKI occurrence in patients with ST-segment elevation myocardial infarction (STEMI) and undergoing pPCI. RESULTS: This retrospective, single-center, case-control study included diabetic patients diagnosed with STEMI who underwent pPCI in a tertiary healthcare center between 2021 and 2022. The study population included SGLT2i users (n = 130) and non-SGLT2i users (n = 165). Inverse probability propensity score weighting and doubly robust estimation were performed to decrease bias and to balance covariate distribution for estimating average treatment for those treated. In a doubly robust inverse probability weighted regression model, in which covariates were balanced, CI-AKI risk was also found to be lower in the SGLT2i-user group (OR: 0.86 [0.76-0.98]; 95% CI; P = 0.028). In addition, ejection fraction, admission creatinine, albumin, and volume of contrast media were found to be independent predictors of CI-AKI in patients presenting with STEMI and undergoing pPCI. CONCLUSION: Our study provides evidence supporting the potential protective effect of SGLT2i against CI-AKI in diabetic patients presenting with STEMI and undergoing pPCI.

The Definition of Sarcomeric and Non-Sarcomeric Gene Mutations in Hypertrophic Cardiomyopathy Patients: A Multicenter Diagnostic Study Across Türkiye.

BACKGROUND: Hypertrophic cardiomyopathy is a common genetic heart disease and up to 40%-60% of patients have mutations in cardiac sarcomere protein genes. This genetic diagnosis study aimed to detect pathogenic or likely pathogenic sarcomeric and non-sarcomeric gene mutations and to confirm a final molecular diagnosis in patients diagnosed with hypertrophic cardiomyopathy. METHODS: A total of 392 patients with hypertrophic cardiomyopathy were included in this nationwide multicenter study conducted at 23 centers across Türkiye. All samples were analyzed with a 17-gene hypertrophic cardiomyopathy panel using next-generation sequencing technology. The gene panel includes ACTC1, DES, FLNC, GLA, LAMP2, MYBPC3, MYH7, MYL2, MYL3, PLN, PRKAG2, PTPN11, TNNC1, TNNI3, TNNT2, TPM1, and TTR genes. RESULTS: The next-generation sequencing panel identified positive genetic variants (variants of unknown significance, likely pathogenic or pathogenic) in 12 genes for 121 of 392 samples, including sarcomeric gene mutations in 30.4% (119/392) of samples tested, galactosidase alpha variants in 0.5% (2/392) of samples and TTR variant in 0.025% (1/392). The likely pathogenic or pathogenic variants identified in 69 (57.0%) of 121 positive samples yielded a confirmed molecular diagnosis. The diagnostic yield was 17.1% (15.8% for hypertrophic cardiomyopathy variants) for hypertrophic cardiomyopathy and hypertrophic cardiomyopathy phenocopies and 0.5% for Fabry disease. CONCLUSIONS: Our study showed that the distribution of genetic mutations, the prevalence of Fabry disease, and TTR amyloidosis in the Turkish population diagnosed with hypertrophic cardiomyopathy were similar to the other populations, but the percentage of sarcomeric gene mutations was slightly lower.

Relationship between glycosylated hemoglobin and vitamin B12 deficiency anemia.

OBJECTIVE: Previous studies showed that vitamin B12 deficiency anemia causes a false increase in glycosylated hemoglobin (HbA1c) and that HbA1c decreases with B12 treatment. However, no study has been conducted on how much an increase in hemoglobin (Hgb) level causes a decrease in HbA1c level after treatment. METHODS: The study included 37 patients who were not diagnosed with diabetes, did not use anti-diabetic drugs, were pre-diabetic according to HbA1c level, and were diagnosed with vitamin B12 deficiency anemia in the patient group and 40 healthy volunteers of similar age and gender characteristics in the control group. The patient group was given 1 mg/day of cyanocobalamin (vitamin B12) orally for 3 months. Patients' Hgb, mean corpuscular volume, fasting plasma glucose, HbA1c, and vitamin B12 values were compared at the beginning and at the end of the 3rd month. RESULTS: In the patient group, it was determined that 0.94 mg/dL increase in Hgb after vitamin B12 treatment caused a 0.24 decrease in HbA1c (%). The initial HbA1c of the patient group was 6.01±0.20 and the 3rd-month HbA1c was 5.77±0.33; the initial and 3rd-month Hgb values were 11.31±0.28 and 12.26±0.33, respectively; the initial and 3rd-month vitamin B12 (ng/L) levels were 112.43±7.18 and 408.48±119.61, respectively; and there was a significant difference between the initial and 3rd-month values (p<0.001, p<0.001, p<0.001, respectively). Moreover, 35% of the patients in the patient group had no diagnosis of prediabetes according to the HbA1c level at the end of the 3rd month. CONCLUSION: Elimination of vitamin B12 deficiency anemia before making a diagnosis or treatment decision according to HbA1c level will prevent patients from misdiagnosis of diabetes and unnecessary treatment changes in diabetic patients.

A Hypothesis Regarding Neurosecretory Inhibition of Stress Mediators by Colchicine in Preventing Stress-Induced Familial Mediterranean Fever Attacks.

Familial Mediterranean fever (FMF) is a monogenic autoinflammatory disease characterized by recurrent episodes of fever and serositis. Colchicine (Col) has a crucial role in the prevention of amyloidosis and FMF attacks. The effect of Col on innate immune cells is based on the inhibition of the microtubule system. The microtubule system is also very important for neurosecretory functions. The inhibitory effect of Col on neurosecretory functions is an overlooked issue. Considering that the neuroimmune cross-talk process plays a role in the development of inflammatory diseases, the effect of Col on the neuronal system becomes important. FMF attacks are related to emotional stress. Therefore, the effect of Col on stress mediators is taken into consideration. In this hypothetical review, we discuss the possible effects of Col on the central nervous systems (CNS) and peripheral nervous systems (PNS) in light of mostly experimental study findings using animal models. Studies to be carried out on this subject will shed light on the pathogenesis of FMF attacks and the other possible mechanisms of action of Col apart from the anti-inflammatory features.

Hypercalcemia in IgG4-related disease: coincidental or associated? Case based review.

Immunoglobulin (Ig) G4-related disease (IgG4RD) is a chronic autoimmune disorder characterized by dense lymphoplasmacytic infiltrations and fibrosis of storiform pattern. The most typical manifestations include major salivary or lacrimal gland involvement, autoimmune pancreatitis, and retroperitoneal fibrosis. While the increase in IgG4 is the typical feature of the disease, hypercalcemia has been rarely reported in IgG4RD so far, only one of these cases has been shown parathyroid gland involvement (isolated involvement). In this study, we present a 43-year-old female patient with weight loss, pancreatic mass, lymphadenopathy, nodular lesion in the lung, hypercalcemia, and also increased level of serum IgG4. Histopathological investigation following parathyroidectomy revealed a dense lymphoplasmacytic infiltrate with an IgG4 to IgG ratio of > 50% in the fat tissue surrounding the parathyroid gland, particularly at the perivascular areas. This is the first systemic IgG4RD case in combination with hypercalcemia in the literature who was detected to have parathyroid adenoma. Our aim in this review is to emphasize that, although rarely, IgG4RD may be accompanied by hypercalcemia and parathyroid gland may be one of its target sites.

Endovascular treatment of aortic coarctation with a novel BeGraft aortic stent in children and young adults: a single-centre experience with short-term follow-up results.

OBJECTIVES: We present our experience and outcomes with the BeGraft in the treatment of aortic coarctation in a predominantly paediatric population. METHODS: This study includes a retrospective analysis of patients who had Begraft aortic stent implantation between 2018 and 2020 from a single centre. RESULTS: The BeGraft aortic stent was used in 11 patients (7 males, 4 females) with a median age of 14 (13-21) years and a median weight of 65 (46-103) kg. Coarctation was native in five patients and recurrent in six patients. Median stent diameter and length were 16 mm and 38 mm, respectively. The median peak-to-peak pressure was 30 (12-55) mmHg before the procedure and 5 (0-17) mmHg after the procedure. The stenting procedure was successful in 10 of the 11 patients. Stent migration to the abdominal aorta occurred on post-procedure day 1 in the 21-year-old patient, who had previously undergone surgical closure of the ventricular septal defect and balloon angioplasty for coarctation. After repositioning failed, the stent was safely fixed in the abdominal aorta. Strut distortion also occurred during balloon retrieval in one patient, but no aneurysm or in-stent restenosis was observed at 1-year follow-up. The patients were followed for a median of 14 (4-25) months and none required redilation. CONCLUSIONS: Our initial results demonstrated that the BeGraft aortic stent effectively reduced the pressure gradient in selected native and recurrent cases. Despite advantages such as a smaller sheath and low profile, more experience and medium- to long-term results are needed.

Evaluation of hyperferritinemia causes in rheumatology practice: a retrospective, single-center experience.

Hyperferritinemia may develop due to various reasons such as inflammation, infection, or malignancy. The purpose of the study to explore the prevalence and to figure out the causes of general hyperferritinemia and extreme hyperferritinemia as detected through the ferritin measurements requested by the rheumatology department. Adult patients at the age of 18 years and older with at least one serum ferritin level measurement at or above 500 ng/mL as requested by the rheumatology department between January 2010 and December 2019 were evaluated retrospectively. Hyperferritinemia was detected in 4.7% of 11,498 serum ferritin tests. The mean age of 242 patients found to have hyperferritinemia was 53.7 ± 17.1 years; of the patients, 63.2% were female, and the mean serum ferritin value was 2820 ± 5080 ng/mL. The most common cause of hyperferritinemia was rheumatological diseases with a ratio of 59.1%, which was followed by infections, iron overload, and solid malignancy. Among the rheumatologic diseases, adult-onset Still's disease (AOSD), rheumatoid arthritis, and vasculitis were the cause accounting for hyperferritinemia. Ferritin levels were significantly higher in the AOSD group compared to the other rheumatologic disease groups (p < 0.0001). While extreme hyperferritinemia (ferritin ≥ 10,000 ng/mL) rate in our cohort was 0.2%, the most common cause was AOSD (15/17). In patients with hyperferritinemia, 3 month mortality was found to be 8.7%. CRP level was identified as the only independent predictor for the 3 month mortality in all patients [OR 1.088 (95% CI 1.004-1.178), p = 0.039]. Although rheumatologic disease activation and infections are the most common causes, the other causes should also be considered for the differential diagnosis.

Right ventricular regional deformation analysis in patients operated for tetralogy of Fallot.

PURPOSE: We aimed to compare the findings of a segmental speckle-tracking strain of right ventricle with those of cardiac magnetic resonance imaging in this setting. METHODS: In 26 patients with operated tetralogy of Fallot (mean age, 15.35 ± 2.3 years; range 11-18 years), right ventricular segmental speckle-straining (the basal, mid, apical segments of right ventricular septum and lateral free wall, and right ventricular apex) were determined using two-dimensional echocardiography. The echocardiographic findings were compared to right ventricular ejection fraction, right ventricular indexed end-diastolic volume, indexed end-systolic volume and pulmonary regurgitation fraction at cardiac magnetic resonance. RESULTS: Right ventricular global speckle strain was -18.6 ± 3.7and lateral free wall strain was -17.8 ± 4.9. Indexed right ventricular end diastolic volume was 171.7 ± 23.3 ml/m2, indexed right ventricular end systolic volume was 95.1 ± 35.0 ml/m2 and right ventricular ejection fraction was 44.76 ± 9.39%. Basal inferior septum is correlated with indexed right ventricular end-diastolic volume (RVEDV; r = -0.521, p = 0.015) and pulmonary regurgitation fraction (r = -0.584, p = 0.015). Cardiac magnetic resonance (CMR)-derived RVEDV is correlated with lateral free wall (r = -0.465, p = 0.034) and Global RV (r = 0.442, p = 0.045). CONCLUSION: Right ventricular basal inferior septal and apical longitudinal strain correlates with the measures of right ventricular performance on CMR. These parameters would be useful to monitor right ventricular performance in patients after tetralogy of Fallot repair.

Levoatrial Cardinal Vein: Occluder Embolization and Complication Management

In rare cases, levoatrial cardinal vein may occur as an isolated condition without additional congenital anomalies. Depending on the direction and flow of the shunt, this pathology may produce symptoms; alternatively, it may be asymptomatic, as in the case presented in this study. In asymptomatic cases, complications, such as paradoxical embolism and brain abscess, can arise later. In the 11-year-old patient whose case is presented here, the levoatrial cardinal vein was asymptomatic and incidentally detected. The percutaneous closure method was applied first. However, by 16 hours after the procedure, the occluder device had embolized to the iliac artery. Emergency surgery was performed; first, the occluder device was removed, and levoatrial cardinal vein ligation was then performed via a mini-thoracotomy. The symptoms, diagnosis, and treatment modalities of isolated levoatrial cardinal vein are discussed in the context of this case described herein.

Cytopathology-histopathology correlation and the effect of nodule diameter on diagnostic performance in patients undergoing thyroid fine-needle aspiration biopsy.

INTRODUCTION: Although thyroid fine-needle aspiration biopsy (FNAB) is established to have a good overall sensitivity and specificity, various outcomes have been reported on its performance in large nodules. The aim of the study was to evaluate the diagnostic performance of FNAB and the effect of the nodule diameter on its diagnostic performance. MATERIALS AND METHODS: The outcomes of a total of 7319 patients who underwent FNAB over the course of 5 years were analyzed retrospectively and 648 patients who had undergone post-FNAB thyroidectomy or lobectomy were included in the study. FNAB results were classified according to the Bethesda system. After evaluating the compatibility between cytology and pathology results, all-nodules and diameter-based (<4 cm and ≥4 cm) sensitivity, specificity, false positivity, false negativity, and accuracy rates of FNAB were calculated. RESULTS: Sensitivity of FNAB was 85.4% for all nodules, 88.3% for nodules <4 cm, and 75.8% for nodules >4 cm (P < 0.001). Specificity was 58.4% for all nodules, 49.3% for nodules <4 cm, and 75.1% for nodules >4cm (P < 0.001). While false positivity was 41.6% for all nodules, it was 50.7% for nodules smaller than 4 cm and was 24.9% for nodules larger than 4 cm (P < 0.001). False negativity was 14.6% for all nodules and was 11.7% for nodules smaller than 4 cm and 24.2% for nodules larger than 4 cm (P < 0.001). Finally, among the entire set of nodules, the accuracy was 64.4%, which was 59.2% in nodules smaller than 4 cm, and 75.2% in nodules larger than 4 cm (P < 0.001). CONCLUSION: Despite a higher rate of false negativity, FNAB has higher specificity and accuracy in large nodules than those in the small nodules. Nodule diameter should not be used alone as a criterion to recommend thyroidectomy to the patient.

Familial Mediterranean fever: the molecular pathways from stress exposure to attacks.

FMF is an autoinflammatory disease characterized by recurrent attacks and increased IL-1 synthesis owing to activation of the pyrin inflammasome. Although knowledge of the mechanisms leading to the activation of pyrin inflammasome is increasing, it is still unknown why the disease is characterized by attack. The emergence of FMF attacks after emotional stress and the induction of attacks with metaraminol in previous decades suggested that stress-induced sympathoadrenal system activation might play a role in inflammasome activation and triggering attacks. In this review, we will review the possible molecular mechanism of stress mediators on the inflammation pathway and inflammasome activation. Studies on stress mediators and their impact on inflammation pathways will provide a better understanding of stress-related exacerbation mechanisms in both autoinflammatory and autoimmune diseases. This review provides a new perspective on this subject and will contribute to new studies.

The acromegaly registry of ten different centers in Turkey.

OBJECTIVES: To describe biochemical and clinical features, and therapeutic outcomes of acromegaly patients in Turkey. METHODS: Retrospective multicenter epidemiological study of 547 patients followed in 10 centers of the Turkish Acromegaly registry. RESULTS: A total of 547 acromegaly patients (55% female) with a median age of 41 was included in this study. Majority of patients had a macroadenoma (78%). Transsphenoidal surgery was performed as primary treatment in 92% of the patients (n = 503). Surgical remission rate was 39% (197/503) in all operated patients. Overall disease control was achieved in 70% of patients. Remission group were significantly older than non-remission group (p = .002). Patients with microadenomas had significantly higher remission rates than patients with macroadenomas (p < .001). Patients with microadenomas were significantly older at the time of diagnosis when compared to patients with macroadenomas (p < .001). Preoperative growth hormone (GH) and insulin-like growth factor 1 (IGF-1) levels were significantly lower in the remission group (p < .001). Initial IGF-1 and GH levels were significantly higher in macroadenomas compared to microadenomas (p < .001). Medical treatment was administered as a second-line treatment (97%) in almost all patients without remission. Radiotherapy was preferred in 21% of the patients mostly as a third line treatment. CONCLUSIONS: This is one of the largest real life studies evaluating the epidemiological characteristics and treatment outcomes of patients with acromegaly who were followed in different centers in Turkey. Transsphenoidal surgery in the treatment of acromegaly still remains the most valid method. Medical treatment options may improve long-term disease outcomes in patients who cannot be controlled with surgical treatment (up to 70%).

Hyperkalemia in type 4 renal tubular acidosis associated with systemic lupus erythematosus.

Renal tubular acidosis (RTA) is a normal anion gap metabolic acidosis that manifests with insufficiency of hydrogen ion excretion or bicarbonate (HCO3) reuptake as a result of renal tubular dysfunction independent of glomerular filtration rate. Hypokalemic RTA subtypes co-existing with autoimmune diseases particularly appear in Sjogren's syndrome, but rarely in systemic lupus erythematosus (SLE). Type 4 RTA associated with hyperkalemia is very rare during the course of SLE and hence has been scarcely reported in the literature. Here, we report a 42-year-old patient for whom regular follow-up was ongoing due to class IV lupus nephritis when she developed hyperkalemia. The patient had normal anion gap hyperkalemic metabolic acidosis and her urine pH was 5.5. Type 4 RTA was considered and, therefore, tests for renin and aldosterone levels were requested, which revealed that renin was suppressed and aldosterone was decreased. Upon diagnosis of SLE-associated type 4 RTA, short-term oral HCO3 and fludrocortisone were initiated. Potassium (K) and HCO3 levels improved at day 15 of therapy. In this review, we analyzed our case along with five other reports (a total of seven cases) of SLE-associated type 4 RTA we identified through a literature search. We wanted to highlight RTA for differential diagnosis of hyperkalemia emerging during SLE/lupus nephritis and we also discussed possible underlying mechanisms.

Adolescents with unexplained chest pain reported depression and impaired emotional and social functioning.

AIM: Chest pain is common in adolescence, but there are no established criteria for managing this problem, which is rarely associated with cardiac disease. This study addressed the gaps in the literature by evaluating psychosocial factors that could be associated with medically unexplained chest pain. METHODS: We consecutively selected 100 patients (68% girls) aged 13-18 who were diagnosed with unexplained chest pain when they presented to the cardiology outpatient clinics of Tepecik Research Hospital, Izmir, Turkey, between 30 September 2015 and 30 June 2018. The controls were 76 age- and sex-matched adolescents (69% girls) aged 13-18 who were undergoing routine cardiology assessments before joining sports clubs. We assessed their health-related quality of life and any depression and physical symptoms. RESULTS: Regression analysis showed some adolescents were a number of times more likely to report chest pain. These included those who reported boredom (4.1 times), felt stressed or anxious (2.2) and those who experienced sleep disturbance (2.6), co-morbid headaches (2.0), back pain (3.1) and impaired social functioning (1.2). CONCLUSION: The results indicated a significant association between unexplained chest pain and physical symptoms, depression and impaired emotional and social functioning. These factors warrant further evaluation.

What is the optimal time for measuring glucose concentration to detect steroid-induced hyperglycemia in patients with rheumatic diseases?

OBJECTIVE: Corticosteroids may cause hyperglycemia and diabetes mellitus (DM). Development of DM during long-term steroid use has been well studied; however, data regarding the short-term effects of steroid therapy are scarce. In this study, we aimed to detect the actual time of short-term steroid-induced hyperglycemia in patients without previous impaired glucose metabolism, and the ideal time (which day and in relation to meals) of glucose measurement. METHODS: The 7-point blood glucose (BG) measurements of patients who were commenced moderate to high-dose steroids (≥15 mg/day prednisolone or its equivalent) due to rheumatological diseases during the first 5 days of steroid therapy were recorded. Fasting BG ≥ 7 mmol/L (126 mg/dL) or random BG ≥ 11.1 mmol/L (200 mg/dL) were considered as overt DM in accordance with the 2016 American Diabetes Association guideline, and post-meal BG ≥10 mmol/L (180 mg/dL) was considered as steroid-induced hyperglycemia. RESULTS: Fifteen males (mean age: 44 ±â€¯16 years) and 35 females (mean age: 41 ±â€¯12 years) were recruited to the study. One thousand seven hundred fifty fasting, pre-meal, and 2-hours post-meal BG concentrations were analyzed. Twenty-one (42%) patients developed steroid-induced DM and 39 (78%) developed steroid-induced hyperglycemia. The highest glucose concentrations were detected on the 3rd day of steroid therapy and 2-h after meals (p < .0001). CONCLUSION: Intermediate to high-dose steroid therapy causes hyperglycemia after lunch and dinner on the 3rd day of treatment. This time period should be taken into consideration in the detection and treatment of steroid-induced hyperglycemia.

Kawasaki disease in Turkish children: a single center experience with emphasis on intravenous immunoglobulin resistance and giant coronary aneurysms.

Yilmazer MM, Özdemir R, Mese T, Küçük M, Öner T, Devrim I, Bayram N, Güven B, Tavli V. Kawasaki disease in Turkish children: a single center experience with emphasis on intravenous immunoglobulin resistance and giant coronary aneurysms. Turk J Pediatr 2019; 61: 648-656. Prompt diagnosis and the administration of intravenous immunoglobulin (IVIG) has reduced the incidence of coronary artery abnormalities (CAA) in Kawasaki Disease (KD). The resistance to treatment and development of the coronary sequelae remain the most important problems in KD. We aimed to determine the predicting factors of nonresponse to initial IVIG therapy and to analyze the cases who had giant coronary aneurysms. A total of 120 KD cases, including 61 children fulfilling the criteria for KD and 59 with incomplete KD were enrolled into this study. Demographic, laboratory, clinical, echocardiographic characteristics, and treatment regimens were reviewed, retrospectively. The median age of the patients was 33.5 months (range: 3-168 months). Coronary artery aneurysms were detected in 35 patients (29%) at the time of diagnosis. Twenty-eight patients had coronary aneurysms small or medium in size, one had a large, and seven had giant coronary aneurysms. CAA persisted in 8 cases in the follow-up, all of which were large or giant aneurysms. A ten month-old girl with a giant coronary aneurysm was referred to coronary bypass surgery in the subacute phase of follow-up, due to myocardial ischemia. Eighteen patients were unresponsive to the initial IVIG therapy (%15), of whom 10 were diagnosed as cKD and 8 were iKD. Patients who did not respond to initial IVIG therapy, had higher white blood cell (WBC) count, higher C-reactive protein (CRP) and lower albumin levels than those who did (P < 0.05). In univarite analysis; CRP, WBC and albumin were found to be significant predictors of nonresponse to initial IVIG therapy, while a stepwise multiple linear regression analysis showed that WBC count and albumin levels were significantly correlated with nonresponse to initial treatment with IVIG. Our study showed that WBC count and albumin levels might be used as predictors of nonresponse to the IVIG therapy in Turkish children with KD.