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Although Kawasaki disease is often self-limiting, significant cardiovascular sequelae may occur in the acute or late stage. The most common late complication is persistent coronary artery aneurysm, which can lead to myocardial ischaemia and even myocardial infarction. We report a case of coronary artery bypass grafting in a 16-year-old boy with a history of undiagnosed Kawasaki disease. Increased awareness of Kawasaki disease, especially among children between the ages of 6 months and 5 years, can increase early treatment and prevent serious complications that may occur in the future.
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Aneurisma Coronário , Doença da Artéria Coronariana , Síndrome de Linfonodos Mucocutâneos , Infarto do Miocárdio , Masculino , Criança , Humanos , Adolescente , Lactente , Síndrome de Linfonodos Mucocutâneos/complicações , Síndrome de Linfonodos Mucocutâneos/diagnóstico , Infarto do Miocárdio/complicações , Infarto do Miocárdio/diagnóstico , Ponte de Artéria Coronária , Doença da Artéria Coronariana/complicações , Aneurisma Coronário/diagnóstico por imagem , Aneurisma Coronário/etiologiaRESUMO
To compare the clinical and laboratory findings of multisystem inflammatory syndrome in children (MIS-C), patients with Kawasaki disease (KD) and with macrophage activating syndrome due to systemic juvenile idiopathic arthritis (sJIA-MAS) on real-life data. Patients diagnosed with MIS-C, KD, and sJIA-MAS from 12 different centers in Turkey who were followed for at least 6 months were included in the study. Demographic, clinical, and laboratory findings of all patients were analyzed. A total of 154 MIS-C, 59 KD, and 31 sJIA-MAS patients were included. The median age of patients with MIS-C were higher than those with KD while lower than those with sJIA-MAS (8.2, 3, 12 years, respectively). Myalgia (39.6%), cardiac (50.6%), gastrointestinal (72.7%), and neurological (22.1%) involvements were more common in patients with MIS-C compared to others. MIS-C patients had lower levels of lymphocyte (950 vs 1700 cells/µl) and thrombocyte (173,000 vs 355,000 cells/µl) counts and higher pro-BNP (1108 vs 55 pg/ml) levels than KD. Ferritin levels were higher in patients with MIS-C compared to patients with KD while they were lower than patients with sJIA-MAS (440, 170, 10,442 ng/ml, respectively). Patients with MIS-C had a shorter duration of hospitalization than sJIA-MAS (p = 0.02) while they required intensive care unit admission more frequently (55 vs 8 patients, p < 0.001). The median MAS/sJIA score of MIS-C patients was - 1.64 (- 5.23 to 9.68) and the median MAS/sJIA score of sJIA-MAS patients was -2.81 ([- 3.79] to [- 1.27]). MIS-C patients displayed certain differences in clinical and laboratory features when compared to KD and sJIA-MAS. Definition of the differences and similarities between MIS-C and the other intense inflammatory syndromes of childhood such as KD and MAS will help the clinicians while making timely diagnosis.
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Artrite Juvenil , Síndrome de Ativação Macrofágica , Síndrome de Linfonodos Mucocutâneos , Artrite Juvenil/complicações , Artrite Juvenil/diagnóstico , Biomarcadores , COVID-19/complicações , Criança , Ferritinas , Humanos , Síndrome de Ativação Macrofágica/diagnóstico , Síndrome de Ativação Macrofágica/etiologia , Macrófagos , Síndrome de Linfonodos Mucocutâneos/complicações , Síndrome de Linfonodos Mucocutâneos/diagnóstico , Síndrome de Resposta Inflamatória SistêmicaRESUMO
INTRODUCTION: A new entity, which occurs a few weeks after SARS-CoV-2 infection and resembling incomplete Kawasaki disease or toxic shock syndrome, has been defined and named multisystem inflammatory syndrome (MIS-C) associated with COVID-19 in children. The aim of our study was to describe histopathological characteristics of skin lesions of MIS-C patients to reveal whether there is a relationship between histopathological features and clinical manifestations. MATERIALS AND METHODS: Seventeen who had skin involvement of 57 patients who were diagnosed with MIS-C between December 2020 and February 2021 were included in this prospective study. Demographic information, laboratory findings, and patients' managements were recorded. Skin biopsies were taken simultaneously of each patient. Formalin-fixed, paraffin-embedded skin samples were examined microscopically. RESULTS: The rate of skin rash was 30% in patients with MIS-C and was predominantly the maculopapular type. The anatomical distribution of the rash was evaluated as localized in 10 and generalized in 7 patients. In patients with myocarditis, C-reactive protein and fibrinogen were found to be significantly higher, and lymphocyte and albumin values were found to be low. Herpes-like inclusions were found in the microscopic examination of 2 patients with a history of zona zoster in themselves or in their mother. There was a significant difference between keratinocyte necrosis and some clinical parameters. DISCUSSION: Localized skin lesions appear to be associated with a more severe inflammatory.
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COVID-19/complicações , Exantema/etiologia , Pele/patologia , Síndrome de Resposta Inflamatória Sistêmica/complicações , Adolescente , Biópsia , COVID-19/imunologia , COVID-19/virologia , Criança , Pré-Escolar , Exantema/imunologia , Exantema/patologia , Feminino , Humanos , Masculino , Estudos Prospectivos , Pele/imunologia , Síndrome de Resposta Inflamatória Sistêmica/imunologia , Síndrome de Resposta Inflamatória Sistêmica/virologiaRESUMO
BACKGROUND: Acetylsalicylic acid (ASA) is a non-steroidal anti-inflammatory drug used in the treatment of acute rheumatic fever (ARF) and it can cause serious adverse effects. This study aimed to evaluate the clinical efficacy and side effects of ibuprofen in the treatment of ARF compared to the classic treatment, ASA. METHODS: Children who were hospitalized for the treatment of ARF with isolated arthritis and mild carditis between October 2015 and October 2018 and who received non-steroidal anti-inflammatory therapy were evaluated in the study. We compared the demographic data, clinical findings, efficacy, and side effects of the treatments of the children, who were divided into ASA and ibuprofen groups. RESULTS: Of the 38 patients included in the study, 21 were treated with ASA (ASA group), and 17 were given ibuprofen (IBU group). There was no difference between the groups regarding the length of hospital stay, total treatment time, time to resolution of clinical symptoms, and normalization of acute-phase reactants. Liver enzymes increased during treatment in 13 patients (62%) in the ASA group and three patients (18%) in the IBU group (P = 0.009). In the ASA group, 69% of patients with increased liver enzymes were under 11 years of age during treatment. Clinical side effects were observed in three patients in the ASA group while none were observed in the IBU group. CONCLUSION: The results of this study suggest that ibuprofen can be a safe alternative in the treatment of ARF, especially in young children. Although ibuprofen can be a safe and effective alternative to ASA, studies including larger series are needed on this subject.
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Ibuprofeno , Febre Reumática , Anti-Inflamatórios não Esteroides/efeitos adversos , Aspirina/efeitos adversos , Criança , Pré-Escolar , Humanos , Ibuprofeno/efeitos adversos , Resultado do TratamentoRESUMO
BACKGROUND: Rhabdomyolysis after spider bite has been reported in a small number of patients, and myocarditis in even fewer. However, arrhythmia associated with latrodectism in children has not been described in the literature to date. CASE SUMMARY: A girl presented approximately 4.5 h after being bitten on the left ankle by a black spider. Two unifocal premature ventricular contractions (PVCs) were observed on the electrocardiogram. In laboratory tests, creatine kinase was elevated. On day 2, levels of troponin, pro-brain and natriuretic peptide were elevated. Electrocardiogram revealed inverted and biphasic T waves. Echocardiography revealed mild left ventricular dilation, mitral and aortic valve regurgitation. Holter electrocardiogram showed PVCs. Her laboratory and echocardiography findings completely normalized after discharge, and no arrhythmia was observed on the Holter electrocardiogram during outpatient follow-up. CONCLUSION: Although spider bites are uncommon, they can cause serious systemic effects. These patients should be evaluated for arrhythmia, rhabdomyolysis and myocarditis.
Rarely, spider bites can cause serious systemic effects, severe morbidity and death. In a small number of patients, spider envenomation causes rhabdomyolysis and myocarditis. In the present case, the elevated troponin and pro-brain natriuretic peptide levels and electrocardiogram/echocardiography findings were consistent with myocarditis, and an increase in creatinine kinase level indicated rhabdomyolysis. In addition, the electrocardiogram and Holter electrocardiogram revealed unifocal premature ventricular contraction. To our knowledge, arrhythmia due to Latrodectus spider bite has not been described in children to date. In addition, this case demonstrates the coexistence of two serious systemic effects, rhabdomyolysis and myocarditis, with full recovery after appropriate treatment.
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Viúva Negra , Miocardite , Rabdomiólise , Picada de Aranha , Venenos de Aranha , Animais , Arritmias Cardíacas/diagnóstico , Arritmias Cardíacas/etiologia , Feminino , Humanos , Miocardite/diagnóstico , Miocardite/etiologia , Picada de Aranha/induzido quimicamente , Picada de Aranha/complicações , Picada de Aranha/diagnóstico , Venenos de Aranha/efeitos adversosRESUMO
Swyer-James-MacLeod syndrome is characterized radiologically by hyperlucency in a single lung lobe, accompanied by reduced vascularity, alveolar hyperdistention, and air trapping, without bronchial airway obstruction. The most common congenital heart defect in childhood, ventricular septal defect, leads to irreversible pulmonary hypertension and Eisenmenger syndrome if not treated promptly. This case report presents a 25-year-old patient with Swyer-James-MacLeod syndrome and Eisenmenger syndrome. It is crucial to include Swyer-James-MacLeod syndrome in the differential diagnosis of patients with atypically distributed pulmonary emphysema and unilateral hyperlucency for early diagnosis and timely intervention.
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Complexo de Eisenmenger , Pulmão Hipertransparente , Humanos , Complexo de Eisenmenger/complicações , Complexo de Eisenmenger/diagnóstico por imagem , Adulto , Pulmão Hipertransparente/diagnóstico por imagem , Pulmão Hipertransparente/complicações , Pulmão Hipertransparente/diagnóstico , Diagnóstico Diferencial , Masculino , Tomografia Computadorizada por Raios XRESUMO
Introduction It has been shown that cardiac functions begin to deteriorate in growth hormone (GH) deficiency even in childhood. However, little is known about how GH deficiency affects arrhythmogenesis. The aim of this study was to evaluate the parameters of P wave dispersion (Pd), QT dispersion (QTd), corrected QT (QTc) dispersion (QTcd), T wave peak-to-end (Tp-e) interval, Tp-e/QT ratio, and Tp-e/QTc ratio in children with GH deficiency. This study also aimed to evaluate the relationship of these parameters with insulin-like growth factor 1 (IGF-1) and insulin-like growth factor binding protein 3 (IGFBP-3). Method In the study, records of children diagnosed with GH deficiency in Adana City Training and Research Hospital Pediatric Endocrine Outpatient Clinic between September 2021 and December 2022 were retrospectively reviewed. The control group consisted of children in the same age group who applied to the Emergency Outpatient Clinic with a complaint of chest pain and no pathological finding was detected. The electrocardiograms (ECGs) of all patients were retrospectively evaluated. Results There were a total of 82 children in the study, 41 of whom were diagnosed with GH deficiency and 41 in the healthy control group. The age and male/female ratio of children with GH deficiency were similar to those in the control group (p>0.05). There were 27 (66%) children with complete GH deficiency and 14 (34%) children with partial GH deficiency. P wave dispersion was similar in both GH-deficient children and control group children. It was also similar in children with complete and partial GH deficiency (p>0.05). QT and QTc dispersions were found to be increased in children with GH deficiency, although not statistically significant, compared to the control group (p>0.05). Tp-e interval, Tp-e/QTmax (longest QT interval), and Tp-e/QTcmax (longest QTc interval) ratios were increased in children with GH deficiency compared to the control group (p=0.001, p=0.003, and p=0.001, respectively). QT and QTc dispersion, Tp-e interval, Tp-e/QTmax, and Tp-e/QTcmax ratios were found to be increased in children with complete GH deficiency compared to children with partial GH deficiency, but the difference was not significant (p>0.05). No correlation was found between these ECG parameters and IGF-1, IGFBP-3, and peak GH levels after stimulation tests (p>0.05). Conclusion We found in our study that the Tp-e interval was longer and Tp-e/QT and Tp-e/QTc ratios were increased in children with GH deficiency. These results suggest that the risk of ventricular arrhythmias in children with GH deficiency may start to increase from childhood. However, further prospective studies are needed to confirm our results.
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A previously healthy 12-year-old girl presented to the emergency department with severe chest pain and dyspnea that woke her from sleep. She had short-term syncope just before the emergency admission. On physical examination, respiratory rate was 26 breaths per minute while resting, and blood pressure was 92/56 mmHg. Other physical examination findings were insignificant. She had no past medical history, and her family history was unremarkable. Laboratory test results showed elevated C-reactive protein (27 mg/L; reference range: <5 mg/L) and white blood cell count (13.7 K/µL; reference range: 4-12 K/µL). Other laboratory test results were within normal limits, including troponin T value (3 ng/L; reference range: 3-14 ng/L). An electrocardiogram showed 1 mm ST-segment elevation in bipolar (D1 and D2) limb leads, and augmented vector foot leads, and echocardiography revealed a complicated pericardial effusion and a suspicious mass adjacent to the left ventricle.
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Neoplasias do Mediastino , Teratoma , Humanos , Feminino , Criança , Pericárdio/diagnóstico por imagem , Ruptura/complicações , Neoplasias do Mediastino/complicações , Neoplasias do Mediastino/diagnóstico por imagem , Dor no Peito/etiologia , Teratoma/complicações , Teratoma/diagnóstico , Teratoma/cirurgiaRESUMO
BACKGROUND: Duchenne muscular dystrophy (DMD) is an X-linked muscular disease which is caused by the absence of dystrophin. Troponin elevation with acute chest pain may indicate acute myocardial injury in these patients. We report a case of DMD that presented with ACP and troponin elevation, who was diagnosed with acute myocardial injury, and successfully treated with corticosteroids. CASE PRESENTATION: A 9-year-old with DMD was admitted to the emergency department with the complaint of acute chest pain. His electrocardiogram (ECG) revealed inferior ST elevation and serum troponin T was elevated. The transthoracic echocardiography (TTE) demonstrated inferolateral and anterolateral hypokinesia with depressed left ventricular function. An ECG-gated coronary computed tomography angiography ruled out acute coronary syndrome. Cardiac magnetic resonance imaging revealed mid-wall to sub-epicardial late gadolinium enhancement at the basal to the mid inferior lateral wall of the left ventricle and corresponding hyperintensity on T2-weighted imaging, consistent with acute myocarditis. A diagnosis of acute myocardial injury associated with DMD was made. He was treated with anticongestive therapy and 2 mg/kg/day of oral methylprednisolone. Chest pain resolved the next day, and ST-segment elevation returned to normal on the third day. Troponin T decreased in the sixth hour of oral methylprednisolone treatment. TTE on the fifth day revealed improved left ventricular function. CONCLUSION: Despite advances in contemporary cardiopulmonary therapies, cardiomyopathy remains the leading cause of death in patients with DMD. Acute chest pain attacks with elevated troponin in patients with DMD without coronary artery disease may indicate acute myocardial injury. Recognition and appropriate treatment of acute myocardial injury episodes in DMD patients may delay the development of cardiomyopathy.
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Traumatismos Cardíacos , Distrofia Muscular de Duchenne , Masculino , Humanos , Criança , Troponina T , Meios de Contraste , Gadolínio , Esteroides , Dor no Peito , TroponinaRESUMO
INTRODUCTION: Infective endocarditis (IE) is a rare disease with high mortality and morbidity. In recent years, an increase in the frequency of infective endocarditis has been observed due to the increase in the survival of cases with congenital heart diseases (CHDs) and the use of central catheters. In addition to revealing the incidence of IE in our clinic, this study aimed to evaluate the demographic characteristics, predisposing factors, clinical, laboratory, microbiological, and echocardiographic findings, and the complications of follow-up of our patients diagnosed with IE in light of the literature. MATERIAL AND METHODS: Thirteen patients with IE who were hospitalized in Pediatric Cardiology Clinic, Pamukkale University Medical Faculty Hospital between January 2016 and August 2021 were retrospectively reviewed. The patients included in the study were evaluated in terms of demographic characteristics, predisposing factors, clinical, laboratory and microbiological findings, echocardiography data, surgical intervention needs, and complications. The incidence of IE in our clinic was defined as the rate of IE among patients admitted to the hospital and reported as the number of patients with IE per 100,000 hospital admissions. RESULTS: The median age of these 13 patients was 11 (7-14) years, and the male/female ratio was 6/7. The five-year IE incidence in Pediatric Cardiology Clinic, Pamukkale University Medical Faculty Hospital, was found to be 2.5 in approximately 100,000 hospital admissions. A predisposing factor was detected in all patients. Six patients (46%) had CHDs, and four patients (31%) had acquired heart disease. The other three (23%) patients were receiving immunosuppressive therapy, and these patients had long-term port catheters. Eight (62%) patients had positive blood cultures. Streptococcus viridans, Streptococcus gordonii, Staphylococcus aureus, coagulase-negativeStaphylococcus, Brucella spp, and Candida albicans were isolated in the blood cultures of these patients. IE-related complications developed in seven (54%) patients. Five (38%) had heart failure, three patients (23%) had thromboembolic events, two (15%) had glomerulonephritis, and one (8%) had thrombophlebitis. Four patients were referred to early surgery. Two patients with recurrent IE attacks were referred to surgery after their treatment was completed. CONCLUSION: The incidence of IE has shown an increase recently with increased rates of survival attributable to corrective surgeries performed for congenital heart diseases, increased prosthetic materials used in cardiac surgeries, and increased use of permanent catheters. In our study, the incidence of IE was found to be 2.5 in 100,000 hospital admissions. Our results have shown that rheumatic heart diseases, besides CHDs, are still an important risk factor for Turkey. Due to the low number of cases in IE studies in the pediatric population, there is a need for further studies to be conducted in large series in this field.
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BACKGROUND: Kidney involvement related to infective endocarditis (IE) may present with different clinical findings. The most common histopathological finding of renal involvement is a combination of proliferative and exudative glomerulonephritis. However, severe acute kidney injury (AKI) induced by crescentic glomerulonephritis (CGN) is extremely rare in children with IE. To date, only 4 pediatric cases with IE-induced CGN had been reported. We present a 14-year old girl with IE-induced CGN. CASE: A 14-year old girl with fever, macroscopic hematuria, oliguria, and acute kidney injury (AKI) was admitted to our clinic. The medical history revealed that the patient had undergone several cardiac interventions due to truncus arteriosus type 1, and she recovered from IE-induced glomerulonephritis following antibiotherapy six months ago. During admission, the patient was diagnosed with IE according to one major (positive imaging finding) and three minor (fever, predisposing cardiac disease, and immunological criterion) criteria. Immediate antibiotic treatment was initiated. A kidney biopsy was performed, which showed crescentic glomerulonephritis (CGN with crescents, > 50%). Daily pulse steroid (3 days), monthly pulse cyclophosphamide (6 doses), and oral steroid (2 mg/kg/day) therapy were initiated with gradual dose tapering. The patient underwent 12 hemodialysis sessions until the 38 < sup > th < /sup > day of the treatment. She was discharged on the 45th day of treatment with normal kidney function tests and negative acute phase reactants. Treatment was maintained with mycophenolate mofetil (MMF) after a 6-month course of cyclophosphamide. MMF was discontinued in the 12th month. At the 18thmonth follow-up visit the patient had mild proteinuria, and was on ramipril therapy. CONCLUSIONS: The occurrence of CGN should be considered in children with predisposing cardiac disease, who develop hematuria, proteinuria, and severe AKI. Although antibiotic therapy alone is often sufficient in this immune complex GN induced by infection, early initiation of additional immunosuppressive therapy in the presence of CGN may be beneficial for long term preservation of kidney functions.
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Injúria Renal Aguda , Endocardite , Glomerulonefrite Membranoproliferativa , Glomerulonefrite , Feminino , Criança , Humanos , Adolescente , Hematúria , Glomerulonefrite/complicações , Endocardite/tratamento farmacológico , Injúria Renal Aguda/terapia , Injúria Renal Aguda/tratamento farmacológico , Proteinúria , Ciclofosfamida/uso terapêutico , Antibacterianos/uso terapêutico , Rim/patologiaRESUMO
OBJECTIVES: This study aims to investigate serum pentraxin 3 (PTX3) levels during acute episode of acute rheumatic fever (ARF) and their relationship with disease severity. PATIENTS AND METHODS: The prospective study was conducted between January 2015 and December 2018 and included 52 ARF patients (22 girls, 30 boys, mean age 10.7±2.1 years; range, 5 to 16 years) experiencing an acute episode and 22 healthy children (13 girls, 9 boys, mean age 10.3±3.8 years; range, 5 to 16 years). ARF patients were classified into three groups based on the clinical course: isolated arthritis (n=17), mild carditis (n=19), and moderate/severe carditis (n=16). Blood samples were collected from all patients before treatment and from the healthy children in the control group to measure PTX3 levels. PTX3 was measured using sandwich enzyme-linked immunosorbent assay method. RESULTS: Plasma PTX3 levels were significantly higher in ARF group compared to the control group (4.7±5.2 and 1.2±1.7 ng/mL, p<0.001). Subgroup analysis of serum PTX3 levels in ARF patients with isolated arthritis, mild carditis, and moderate/severe carditis (3.2±3.1 ng/mL, 4.3±5 ng/mL, and 6.7±6.6 ng/mL, respectively) showed that serum PTX3 was significantly higher in the moderate/severe carditis group compared to the other groups (p<0.05). Analysis of echocardiographic data showed that serum PTX3 was positively correlated with left ventricular end-diastolic diameter, left atrial diameters, and mitral A velocity and negatively correlated with E/A ratio (p<0.05; r=0.231, 0.402, 0.562, -0.586, respectively). CONCLUSION: High PTX3 level during an acute episode of ARF may help predict the clinical course and the severity of accompanying carditis. However, prospective studies with larger sample sizes are needed.
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Cardiac involvement is very rare in patients with Henoch-Schönlein purpura (HSP). In this case study, we present an 8-year-old girl presenting with HSP-induced myocarditis and thrombus in the right atrium and HSP nephritis. To date, 15 cases of HSP-related cardiac involvement have been reported in the PubMed/MEDLINE, Scopus, and Google Scholar databases. These cases, together with our case, are included in this review. We excluded those patients with other rheumatologic diseases (acute rheumatic fever, acute post-streptococcal glomerulonephritis, Kawasaki disease) accompanied by HSP. Three were children and 13 were adults and all were male except our case. This review revealed tachyarrhythmia, chest pain, dyspnea, murmur, and heart failure as the major signs. Cardiac tests, electrocardiogram (ECG), and imaging methods (echocardiography in all patients, cardiac magnetic resonance imaging (MRI) in three, cardiac biopsy in one, and post-mortem necropsy in three) showed that the cardiac involvements were pericardial effusion, intra-atrial thrombus, myocarditis, coronary artery changes, myocardial ischemia, infarction and necrosis, subendocardial hemorrhage, and left ventricular dilatation. Kidney involvement was not observed in three patients. As the treatment, high-dose prednisolone and cyclophosphamide, oral corticosteroid, azathioprine, nadroparin calcium, ACE inhibitors, calcium antagonists, beta-blockers, and diuretics were used. Eleven patients (all three children and eight of the adults) had a complete cardiac recovery. Cardiac involvement in adults was more likely to be fatal. Death (three patients), ischemia, and infarct have been reported only in adults. We suggested that early and aggressive treatment can be life-saving. MRI examination is effective at identifying cardiac involvement.
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Glomerulonefrite , Vasculite por IgA , Miocardite , Febre Reumática , Trombose , Adulto , Criança , Feminino , Humanos , Vasculite por IgA/complicações , Vasculite por IgA/tratamento farmacológico , Masculino , Miocardite/complicações , Miocardite/diagnóstico por imagem , Trombose/complicações , Trombose/diagnóstico por imagemRESUMO
PURPOSE: Uterine sarcoma accounts for 3-9% of uterine malignant tumors and has poor prognosis. Pazopanib is an oral multi-kinase inhibitor and the only tyrosine kinase inhibitor which has been approved for metastatic soft tissue sarcoma. In the present study we aimed to evaluate the efficacy of pazopanib in metastatic uterine sarcoma. METHODS: The data of 28 metastatic uterine sarcoma patients receiving pazopanib therapy, who were followed in four oncology centers in Ankara, Turkey between May 2013 and June 2018, were retrospectively analyzed. Patients over 18 years, ECOG performance status ≤ 2, receiving at least one line of chemotherapy for metastatic disease, measurable disease at diagnosis, and histologically proven uterine high grade sarcoma were the inclusion criteria. Progression-free survival (PFS), overall survival (OS), and response rates to pazopanib were retrospectively evaluated. RESULTS: The median age was 53 years (range, 26-76). The majority of the patients had uterine leiomyosarcoma (LMS) (n=25, 89.3%), 2 (7.1%) had undifferentiated uterine sarcoma (UUS), and 1(3.6%) had high grade endometrial stromal sarcoma (ESS). The most common site of metastasis was lung (n: 21, 75%). The median time for pazopanib therapy was 5 months (0.6-28.3). In 22 patients (78.5%), pazopanib was discontinued due to disease progression, while 2 patients (7.1%) quitted therapy owing to toxicity. Partial response was achieved in 4 patients (14.3%), while 17 (60.7%) had stable disease. Median PFS was 5.2 months (95% CI 2.8-7.5) and median OS was 11.4 months (95% CI 3.4-19.5). CONCLUSION: In the present study aiming to assess the real-life outcome of pazopanib-treated patients, we found that pazopanib is efficient in metastatic uterine sarcoma, and our results correspond to the literature.
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Pirimidinas/uso terapêutico , Sarcoma/tratamento farmacológico , Sulfonamidas/uso terapêutico , Neoplasias Uterinas/tratamento farmacológico , Adulto , Idoso , Feminino , Humanos , Indazóis , Pessoa de Meia-Idade , Metástase Neoplásica , Pirimidinas/farmacologia , Sarcoma/patologia , Sulfonamidas/farmacologia , Neoplasias Uterinas/patologiaRESUMO
Complex clinical presentation with diverse timing of particular symptoms may cause diagnostic difficulties, especially in children and adolescents. This paper presents diagnostic difficulties and pitfalls in 3 children with acquired primary hypothyroidism due to Hashimoto's thyroiditis (HT) presenting with unusual manifestations. We described 3 children with acquired primary hypothyroidism due to HT. One of our patients had musculoskeletal pain and was diagnosed and treated as having connective tissue disease. Another patient presented with chest pain, dyspnea, and swelling in the abdomen. She had a massive pericardial effusion (PE). Two patients had severe growth failure. A third patient with Down syndrome had a small PE. Her complaint was dyspnea during sleep. All patients improved with thyroxin therapy. Patients with hypothyroidism due to HT who have complicated clinical manifestations were misdiagnosed and mismanaged at childhood and adolescence. Growth failure is an important sign in children and adolescents. In the presence of complicated manifestations in children and adolescents, thyroid dysfunction must be considered in differential diagnosis.
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[This corrects the article on p. 164 in vol. 21, PMID: 27777910.].
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OBJECTIVE: Our aim was comparison of preoperative and postoperative right ventricular functions of children with adenotonsillar hypertrophy (ATH) who have findings of upper airway obstruction, using new echocardiographic parameters. METHODS: Forty-one children who have admitted to our hospital with symptoms suggestive of upper airway obstruction, whose history and physical examination findings suggest upper airway obstruction and who have undergone adenoidectomy/adenotonsillectomy and 40 healthy children, all of whom between 2 and 12 years of age, were included in the study. Patient group was evaluated by pulsed wave tissue Doppler echocardiography as well as with conventional echocardiography before the operation and 6 months after the operation. RESULTS: Of 41 children in study group, 26 (63.4%) had adenotonsillectomy and 15 (36.6%) had adenoidectomy. Tricuspid annular plane systolic excursion (TAPSE) was significantly lower in preoperative group compared to control group (18.46±1.67, 19.77±1.62; p=0.000, respectively). Myocardial performance index (MPI) was significantly higher in preoperative group than postoperative and control group (0.40±0.07, 0.36±0.06, 0.35±0.07; p=0.032, respectively). Tricuspid isovolumic acceleration (TIVA) was significantly lower in preoperative group than preoperative and control group (2.97±0.8, 3.43±0.7, 3.43±0.9; p=0.020, respectively). Disappearance of this difference was found between postoperative and control groups (p=0.984). Pulmonary acceleration time (PAcT) was found to be significantly lower in preoperative group compared to postoperative and control group (109.68±18.03, 118.93±17.46, 120.0±14.07; p=0.010, respectively). Mean pulmonary artery pressure (mPAP) was significantly higher in preoperative group than control group (29.64±8.11, 24.95±6.33; p=0.010, respectively). In postoperative group mPAP was found to be similar to control group (25.48±7.85, 24.95±6.33; p=0.740, respectively). CONCLUSIONS: TAPSE, PAcT, MPI and TIVA are useful markers for evaluation of preoperative and postoperative ventricular function in children with ATH who have findings of upper airway obstruction. We think that using these practical and easy-to perform parameters may be relevant for evaluation and postoperative follow-up of patients with ATH who have findings of upper airway obstruction. Besides adenotonsillectomy is a beneficial treatment option for these patients.