BACKGROUND Multisystem inflammatory syndrome in children (MIS-C) can be life threatening in sever... more BACKGROUND Multisystem inflammatory syndrome in children (MIS-C) can be life threatening in severe cases because of uncontrolled inflammation and multi-organ failure. In this study, we report the effect of plasma exchange in the treatment of MIS-C and to emphasize the effect of its early application on outcome. METHOD In this retrospective observational study, the medical records of children with severe MIS-C admitted to pediatric intensive care unit (PICU) between April 2020 and January 2021 were reviewed. Severe MIS-C patients were treated according to protocol consisting of plasma exchange (PE), intravenous immune globulin, steroids, and anakinra which we called the "PISA" protocol referring to the initials. The patients were divided into two groups as early plasma exchange (E-PE) and late plasma exchange (L-PE) according to the elapse time between hospital admission and the administration of PE. Groups were compared in terms of outcome variables. Primary study outcome was 28-day mortality. Secondary outcome variables were acute phase response time, length of immunomodulatory treatment, frequency of patients requiring mechanical ventilation (MV) and inotropic support, length of inotropic support and MV, length of hospital and PICU stays. RESULTS Eighteen pediatric patients with MIS-C were included in the study. Seventeen (95%) of the patients presented with decompensated shock and required inotropic support. One of the 17 patients needed extracorporeal membrane oxygenation support (ECMO) PISA protocol was used in all patients. There was no mortality in the E-PE group while the mortality rate was 20% in the L-PE group. Acute phase reactant response was faster in the E-PE group and immunomodulatory treatments could be reduced earlier; the frequency of patients requiring inotropic and mechanical ventilation (MV) support was lower in the E-PE group; the duration of inotropic support, duration of MV, and length of stay in hospital and PICU were significantly shorter in the E-PE group. CONCLUSION We suggest that in selected cases, timely administration of PE is a beneficial rescue therapy for MIS-C related hyperinflammation presenting with severe cardiovascular collapse.
Objectives Multisystem inflammatory syndrome in children (MIS-C) is a rare but severe condition a... more Objectives Multisystem inflammatory syndrome in children (MIS-C) is a rare but severe condition associated with coronavirus disease 2019. Here we aimed to raise awareness for the symptoms of MIS-C in patients with rheumatic diseases, emphasizing the challenges of the differential features. Methods We retrospectively evaluated the demographic and clinical characteristics, laboratory and imaging findings, treatments, and outcomes of six MIS-C patients with previous rheumatic disease. Results Three of the patients had familial Mediterranean fever (FMF), one had juvenile dermatomyositis, one had systemic juvenile idiopathic arthritis (JIA), and another patient had oligoarticular JIA. All FMF patients presented with fever and abdominal pain, two also had chest pain. The patient with systemic JIA presented with fever, rash, and myalgia. All patients had elevated inflammatory markers and high d-dimer levels. Chest imaging of two FMF patients showed infiltrations compatible with pneumonia. ...
Saygideger okuyucular,Her seyden once size tesekkur etmek istiyorum, bilgi caginda, bilgiden yoks... more Saygideger okuyucular,Her seyden once size tesekkur etmek istiyorum, bilgi caginda, bilgiden yoksun, bilgiden uzak isini yapadurmak yerine, saglik alanindaki inovasyon, teknoloji ve yapay zeka kulvarlarindaki merakiniz ve hekimseniz hastaniza en dogru taniyi koyup, en dogru tedaviyi vermek gayesi yada alan uzmani iseniz de bu alanlarda gelisime inanarak okumayi sectiginiz, konfor alaninizi bozmayi kabul ettiginiz icin.Bu kitapta sagligimizin geleceginin buyuk bir kismini olusturacak olan, sagligin yapay zekasini size sunduk. Yapay zekanin saglik alanindaki gelismelerini, karar destek sistemlerini, buyuk veriyi ve gelecek perspektifini en guncel bilgiler isiginda bu kitapta bulacaksiniz.
The effects of biological disease-modifying antirheumatic drugs (bDMARDs) in the clinical course ... more The effects of biological disease-modifying antirheumatic drugs (bDMARDs) in the clinical course of COVID-19 on children with underlying rheumatologic diseases have not been fully demonstrated. To evaluate the course of COVID-19 infection in patients with rheumatic disease receiving bDMARD treatment. This was a retrospective, multicenter study conducted in pediatric patients infected by SARS-CoV-2 and under bDMARDs therapy. The study population consisted of 113 patients (72 female/41 male). The mean age of the patients was 12.87 ± 4.69 years. The primary diagnosis of the cohort was as follows: 63 juvenile idiopathic arthritis, 35 systemic autoinflammatory diseases, 10 vasculitides, and five cases of connective tissue diseases. The mean duration of the primary disease was 4.62 ± 3.65 years. A total of 19 patients had additional comorbid diseases. Thirty-five patients were treated with canakinumab, 25 with adalimumab, 18 with etanercept, 10 with infliximab, nine with tocilizumab, six with rituximab, four with anakinra, three with tofacitinib, and one with abatacept. The median exposure time of the biological drug was 13.5 months. Seventy-one patients had symptomatic COVID-19, while 42 were asymptomatic. Twenty-four patients required hospitalization. Five patients presented with MIS-C. The hospitalized patients were younger and had a shorter duration of rheumatic disease compared to ambulatory patients, although the difference was not statistically significant. Steroid usage, presence of fever, and dyspnea were more common among the hospitalized patients. A worsening in the course of both COVID-19 and current disease was not noticed under bDMARDs, however, to end with a strong conclusion multicentric international studies are required.
Introduction: Programmed death 1 (PD-1) is a co-receptor which is located at the surface of cells... more Introduction: Programmed death 1 (PD-1) is a co-receptor which is located at the surface of cells like natural killer, monocytes, T and B cells. It has two ligands including programmed death ligand-1 (PD-L1) and ligand-2 (PD-L2). T cell functions are inhibited by activation of PD-1/PD-L1 pathway and this pathway is used by viruses and some tumor cells in order to escape from immune eradication. In our study we evaluated PD-L1 expression in the tissue specimens of patients with Wilms tumor,
Following cessation of the turmeric supplement (on presentation), her liver function tests improv... more Following cessation of the turmeric supplement (on presentation), her liver function tests improved dramatically within a fortnight. This corresponded to an improvement in her rash and myalgias. Her regular medications were continued during her hospital stay, with the addition of insulin for glycaemic control. There has been continued improvement in the liver function tests, with normalisation of bilirubin and transaminases. In the absence of viral and autoimmune disorders, it is highly probable that the aetiology of hepatic injury was secondary to turmeric supplementation. Turmeric is an ancient health supplement that has recently become popularised for its anti-inflammatory and antioxidant effects, commonly used in both the health supplements and foods industry. While touted for its many benefits, including paradoxically, antiinflammatory effect in non-alcoholic steatohepatitis, 1-4 this case illustrates the significant risk of hepatotoxicity associated with turmeric. To date, there has been only one other case reported in current literature to support this finding. 5,6 Our case highlights the importance of clinicians and consumers to remain diligent of adverse effects when utilising natural or alternative medicines.
Immunoglobulin G4-related disease (IgG4-RD) is a systemic, immune-mediated, and fibroinflammatory... more Immunoglobulin G4-related disease (IgG4-RD) is a systemic, immune-mediated, and fibroinflammatory disease that can affect almost any organ system. We aimed to present our single-center experience of pediatric patients with IgG4-RD, a rare disease in children. Pediatric patients diagnosed with IgG4-RD at the Hacettepe University between June 2014 and September 2020 were evaluated retrospectively. Patients with definite, probable, or possible diagnosis of IgG4-RD were included. A total of eight patients with a median age of 13.4 (IQR 9.5–15.0) years were included in the study. Clinical presentations were IgG4-related ophthalmic disease in six patients, IgG4-related lymphadenopathy in one patient, and IgG4-related sialadenitis and lymphadenopathy of several lymph nodes accompanied by pancreatitis, ulcerative colitis, and pulmonary manifestations in one patient. Elevated serum IgG4 was detected in three of eight patients (37.5%). The main histopathological feature was fibrosis and lymphoplasmacytic infiltrates. Corticosteroids were used as first-line treatment in almost all patients with or without steroid-sparing agents. Azathioprine, methotrexate and rituximab were used as steroid-sparing agents. Relapse occurred in two of seven patients. Radiotherapy was used as the last resort in one patient with severe orbital disease. IgG4 RD mainly presents with orbital manifestations in pediatric population but has wide phenotypic clinical variability. Although rare, early recognition and treatment are essential for a better outcome in these patients.
BACKGROUND Multisystem inflammatory syndrome in children (MIS-C) can be life threatening in sever... more BACKGROUND Multisystem inflammatory syndrome in children (MIS-C) can be life threatening in severe cases because of uncontrolled inflammation and multi-organ failure. In this study, we report the effect of plasma exchange in the treatment of MIS-C and to emphasize the effect of its early application on outcome. METHOD In this retrospective observational study, the medical records of children with severe MIS-C admitted to pediatric intensive care unit (PICU) between April 2020 and January 2021 were reviewed. Severe MIS-C patients were treated according to protocol consisting of plasma exchange (PE), intravenous immune globulin, steroids, and anakinra which we called the "PISA" protocol referring to the initials. The patients were divided into two groups as early plasma exchange (E-PE) and late plasma exchange (L-PE) according to the elapse time between hospital admission and the administration of PE. Groups were compared in terms of outcome variables. Primary study outcome was 28-day mortality. Secondary outcome variables were acute phase response time, length of immunomodulatory treatment, frequency of patients requiring mechanical ventilation (MV) and inotropic support, length of inotropic support and MV, length of hospital and PICU stays. RESULTS Eighteen pediatric patients with MIS-C were included in the study. Seventeen (95%) of the patients presented with decompensated shock and required inotropic support. One of the 17 patients needed extracorporeal membrane oxygenation support (ECMO) PISA protocol was used in all patients. There was no mortality in the E-PE group while the mortality rate was 20% in the L-PE group. Acute phase reactant response was faster in the E-PE group and immunomodulatory treatments could be reduced earlier; the frequency of patients requiring inotropic and mechanical ventilation (MV) support was lower in the E-PE group; the duration of inotropic support, duration of MV, and length of stay in hospital and PICU were significantly shorter in the E-PE group. CONCLUSION We suggest that in selected cases, timely administration of PE is a beneficial rescue therapy for MIS-C related hyperinflammation presenting with severe cardiovascular collapse.
Objectives Multisystem inflammatory syndrome in children (MIS-C) is a rare but severe condition a... more Objectives Multisystem inflammatory syndrome in children (MIS-C) is a rare but severe condition associated with coronavirus disease 2019. Here we aimed to raise awareness for the symptoms of MIS-C in patients with rheumatic diseases, emphasizing the challenges of the differential features. Methods We retrospectively evaluated the demographic and clinical characteristics, laboratory and imaging findings, treatments, and outcomes of six MIS-C patients with previous rheumatic disease. Results Three of the patients had familial Mediterranean fever (FMF), one had juvenile dermatomyositis, one had systemic juvenile idiopathic arthritis (JIA), and another patient had oligoarticular JIA. All FMF patients presented with fever and abdominal pain, two also had chest pain. The patient with systemic JIA presented with fever, rash, and myalgia. All patients had elevated inflammatory markers and high d-dimer levels. Chest imaging of two FMF patients showed infiltrations compatible with pneumonia. ...
Saygideger okuyucular,Her seyden once size tesekkur etmek istiyorum, bilgi caginda, bilgiden yoks... more Saygideger okuyucular,Her seyden once size tesekkur etmek istiyorum, bilgi caginda, bilgiden yoksun, bilgiden uzak isini yapadurmak yerine, saglik alanindaki inovasyon, teknoloji ve yapay zeka kulvarlarindaki merakiniz ve hekimseniz hastaniza en dogru taniyi koyup, en dogru tedaviyi vermek gayesi yada alan uzmani iseniz de bu alanlarda gelisime inanarak okumayi sectiginiz, konfor alaninizi bozmayi kabul ettiginiz icin.Bu kitapta sagligimizin geleceginin buyuk bir kismini olusturacak olan, sagligin yapay zekasini size sunduk. Yapay zekanin saglik alanindaki gelismelerini, karar destek sistemlerini, buyuk veriyi ve gelecek perspektifini en guncel bilgiler isiginda bu kitapta bulacaksiniz.
The effects of biological disease-modifying antirheumatic drugs (bDMARDs) in the clinical course ... more The effects of biological disease-modifying antirheumatic drugs (bDMARDs) in the clinical course of COVID-19 on children with underlying rheumatologic diseases have not been fully demonstrated. To evaluate the course of COVID-19 infection in patients with rheumatic disease receiving bDMARD treatment. This was a retrospective, multicenter study conducted in pediatric patients infected by SARS-CoV-2 and under bDMARDs therapy. The study population consisted of 113 patients (72 female/41 male). The mean age of the patients was 12.87 ± 4.69 years. The primary diagnosis of the cohort was as follows: 63 juvenile idiopathic arthritis, 35 systemic autoinflammatory diseases, 10 vasculitides, and five cases of connective tissue diseases. The mean duration of the primary disease was 4.62 ± 3.65 years. A total of 19 patients had additional comorbid diseases. Thirty-five patients were treated with canakinumab, 25 with adalimumab, 18 with etanercept, 10 with infliximab, nine with tocilizumab, six with rituximab, four with anakinra, three with tofacitinib, and one with abatacept. The median exposure time of the biological drug was 13.5 months. Seventy-one patients had symptomatic COVID-19, while 42 were asymptomatic. Twenty-four patients required hospitalization. Five patients presented with MIS-C. The hospitalized patients were younger and had a shorter duration of rheumatic disease compared to ambulatory patients, although the difference was not statistically significant. Steroid usage, presence of fever, and dyspnea were more common among the hospitalized patients. A worsening in the course of both COVID-19 and current disease was not noticed under bDMARDs, however, to end with a strong conclusion multicentric international studies are required.
Introduction: Programmed death 1 (PD-1) is a co-receptor which is located at the surface of cells... more Introduction: Programmed death 1 (PD-1) is a co-receptor which is located at the surface of cells like natural killer, monocytes, T and B cells. It has two ligands including programmed death ligand-1 (PD-L1) and ligand-2 (PD-L2). T cell functions are inhibited by activation of PD-1/PD-L1 pathway and this pathway is used by viruses and some tumor cells in order to escape from immune eradication. In our study we evaluated PD-L1 expression in the tissue specimens of patients with Wilms tumor,
Following cessation of the turmeric supplement (on presentation), her liver function tests improv... more Following cessation of the turmeric supplement (on presentation), her liver function tests improved dramatically within a fortnight. This corresponded to an improvement in her rash and myalgias. Her regular medications were continued during her hospital stay, with the addition of insulin for glycaemic control. There has been continued improvement in the liver function tests, with normalisation of bilirubin and transaminases. In the absence of viral and autoimmune disorders, it is highly probable that the aetiology of hepatic injury was secondary to turmeric supplementation. Turmeric is an ancient health supplement that has recently become popularised for its anti-inflammatory and antioxidant effects, commonly used in both the health supplements and foods industry. While touted for its many benefits, including paradoxically, antiinflammatory effect in non-alcoholic steatohepatitis, 1-4 this case illustrates the significant risk of hepatotoxicity associated with turmeric. To date, there has been only one other case reported in current literature to support this finding. 5,6 Our case highlights the importance of clinicians and consumers to remain diligent of adverse effects when utilising natural or alternative medicines.
Immunoglobulin G4-related disease (IgG4-RD) is a systemic, immune-mediated, and fibroinflammatory... more Immunoglobulin G4-related disease (IgG4-RD) is a systemic, immune-mediated, and fibroinflammatory disease that can affect almost any organ system. We aimed to present our single-center experience of pediatric patients with IgG4-RD, a rare disease in children. Pediatric patients diagnosed with IgG4-RD at the Hacettepe University between June 2014 and September 2020 were evaluated retrospectively. Patients with definite, probable, or possible diagnosis of IgG4-RD were included. A total of eight patients with a median age of 13.4 (IQR 9.5–15.0) years were included in the study. Clinical presentations were IgG4-related ophthalmic disease in six patients, IgG4-related lymphadenopathy in one patient, and IgG4-related sialadenitis and lymphadenopathy of several lymph nodes accompanied by pancreatitis, ulcerative colitis, and pulmonary manifestations in one patient. Elevated serum IgG4 was detected in three of eight patients (37.5%). The main histopathological feature was fibrosis and lymphoplasmacytic infiltrates. Corticosteroids were used as first-line treatment in almost all patients with or without steroid-sparing agents. Azathioprine, methotrexate and rituximab were used as steroid-sparing agents. Relapse occurred in two of seven patients. Radiotherapy was used as the last resort in one patient with severe orbital disease. IgG4 RD mainly presents with orbital manifestations in pediatric population but has wide phenotypic clinical variability. Although rare, early recognition and treatment are essential for a better outcome in these patients.
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