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1.
Diagnostics (Basel) ; 13(12)2023 Jun 06.
Artigo em Inglês | MEDLINE | ID: mdl-37370879

RESUMO

Microbiota composition might play a role in the pathophysiology and course of sepsis, and understanding its dynamics is of clinical interest. Invasive meningococcal disease (IMD) is an important cause of community-acquired serious infection, and there is no information regarding microbiota composition in children with meningococcemia. In this study, we aimed to evaluate the intestinal and nasopharyngeal microbiota composition of children with IMD. Materials and Methods: In this prospective, multi-center study, 10 children with meningococcemia and 10 age-matched healthy controls were included. Nasopharyngeal and fecal samples were obtained at admission to the intensive care unit and on the tenth day of their hospital stay. The V3 and V4 regions of the 16S rRNA gene were amplified following the 16S Metagenomic Sequencing Library Preparation. Results: Regarding the alpha diversity on the day of admission and on the tenth day at the PICU, the Shannon index was significantly lower in the IMD group compared to the control group (p = 0.002 at admission and p = 0.001, on the tenth day of PICU). A statistical difference in the stool samples was found between the IMD group at Day 0 vs. the controls in the results of the Bray-Curtis and Jaccard analyses (p = 0.005 and p = 0.001, respectively). There were differences in the intestinal microbiota composition between the children with IMD at admission and Day 10 and the healthy controls. Regarding the nasopharyngeal microbiota analysis, in the children with IMD at admission, at the genus level, Neisseria was significantly more abundant compared to the healthy children (p < 0.001). In the children with IMD at Day 10, genera Moraxella and Neisseria were decreased compared to the healthy children. In the children with IMD on Day 0, for paired samples, Moraxella, Neisseria, and Haemophilus were significantly more abundant compared to the children with IMD at Day 10. In the children with IMD at Day 10, the Moraxella and Neisseria genera were decreased, and 20 different genera were more abundant compared to Day 0. Conclusions: We first found alterations in the intestinal and nasopharyngeal microbiota composition in the children with IMD. The infection itself or the other care interventions also caused changes to the microbiota composition during the follow-up period. Understanding the interaction of microbiota with pathogens, e.g., N. meningitidis, could give us the opportunity to understand the disease's dynamics.

2.
Pediatr Res ; 94(2): 730-737, 2023 08.
Artigo em Inglês | MEDLINE | ID: mdl-36813951

RESUMO

BACKGROUND: This study evaluated of clinical characteristics, outcomes, and mortality risk factors of a severe multisystem inflammatory syndrome in children admitted to a the pediatric intensive care unit. METHODS: A retrospective multicenter cohort study was conducted between March 2020 and April 2021 at 41 PICUs in Turkey. The study population comprised 322 children diagnosed with multisystem inflammatory syndrome. RESULTS: The organ systems most commonly involved were the cardiovascular and hematological systems. Intravenous immunoglobulin was used in 294 (91.3%) patients and corticosteroids in 266 (82.6%). Seventy-five (23.3%) children received therapeutic plasma exchange treatment. Patients with a longer duration of the PICU stay had more frequent respiratory, hematological, or renal involvement, and also had higher D-dimer, CK-MB, and procalcitonin levels. A total of 16 patients died, with mortality higher in patients with renal, respiratory, or neurological involvement, with severe cardiac impairment or shock. The non-surviving group also had higher leukocyte counts, lactate and ferritin levels, and a need for mechanical ventilation. CONCLUSIONS: In cases of MIS-C, high levels of D-dimer and CK-MB are associated with a longer duration of PICU stay. Non-survival correlates with elevated leukocyte counts and lactate and ferritin levels. We were unable to show any positive effect of therapeutic plasma exchange therapy on mortality. IMPACT: MIS-C is a life-threatening condition. Patients need to be followed up in the intensive care unit. Early detection of factors associated with mortality can improve outcomes. Determining the factors associated with mortality and length of stay will help clinicians in patient management. High D-dimer and CK-MB levels were associated with longer PICU stay, and higher leukocyte counts, ferritin and lactate levels, and mechanical ventilation were associated with mortality in MIS-C patients. We were unable to show any positive effect of therapeutic plasma exchange therapy on mortality.


Assuntos
Estado Terminal , Síndrome de Resposta Inflamatória Sistêmica , Humanos , Criança , Estudos de Coortes , Unidades de Terapia Intensiva Pediátrica , Fatores de Risco , Lactatos , Estudos Retrospectivos
3.
Braz. J. Anesth. (Impr.) ; 72(6): 819-822, Nov.-Dec. 2022. tab, graf
Artigo em Inglês | LILACS | ID: biblio-1420633

RESUMO

Abstract Neisseria meningitidis, also known as meningococcus, is a relatively uncommon cause of invasive infection, but when it occurs, it is frequently severe and potentially life-threatening. A ten-year-old female patient developed a purpuric rash with fever. Upon arrival to the pediatric intensive care department, she was unconscious and in a poor general condition. We combined treatment with antibiotics, volume resuscitation, hydrocortisone, and CytoSorb® therapy resulted in a stabilization of hemodynamics, as well as control of hyperinflammation. We observed a significant decrease in vasopressor dosage in this patient.


Assuntos
Humanos , Feminino , Criança , Doenças das Glândulas Suprarrenais , Sepse , Púrpura Fulminante/complicações , Púrpura Fulminante/terapia , Infecções Meningocócicas/complicações , Infecções Meningocócicas/terapia , Miocardite/complicações , Miocardite/terapia , Neisseria meningitidis , Hemorragia
4.
Ulus Travma Acil Cerrahi Derg ; 28(9): 1297, 2022 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-36043936

RESUMO

BACKGROUND: Severe traumatic injuries not only constitute an important population of pediatric intensive care unit (PICU) but they also play a major role in mortality and morbidity. Mortality risk assessment of traumatic injuries in the PICU is a delicate issue as it influences the treatment decisions. BIG score (Base Deficit +[2.5 × INR] + [15-GCS]) and the Pediatric Trauma Score (PTS) are utilized in pediatric trauma centers for the assessment of trauma severity. In this research, we aimed to elucidate the predictivity of trauma severity scores, the PRISM-3 (pediatric risk of mortality), and admission laboratory parameters in pediatric patients with high-energy traumas. METHODS: Children who had been exposed to high-energy polytraumas between 2018 and 2020 and treated in a tertiary care PICU were included in this retrospective analysis. Newly developed mental or motor disabilities, post-traumatic acquired epilepsy, requirement for tracheostomy, and/or extremity loss at PICU discharge were defined as morbidity. The PTS, the BIG score, PRISM-3 score, and admission laboratory parameters were utilized for mortality and morbidity prediction. RESULTS: A total of 155 patients were included in the study. The median age of the participants were 66 months (25-134). The origin of trauma was fall from height in 45.2% (n=70) of the subjects and traffic accident 54.8% (n=85) of the cases. New morbidities had occurred in 8.7% (n=13) and 3.2% (n=5) of the patients deceased in the ICU. The results of logistic regression analysis indicated that BIG score (p=0.01), PTS (p=0.003), PRISM-3 (p=0.02), admission D-dimer (p=0.01), and albumin levels (p=0.001) were significantly associated with mortality. The receiver operating characteristics curve analysis denoted that BIG score (cutoff >21.5, area under the curve [AUC]: 0.984 95% CI: 0.943-0.988), PRISM-3 score (cutoff >18, AUC: 0.997 95% CI: 0.970-1), the PTS (cutoff ≤3, AUC: 0.969 95% CI: 0.928-0.990), admission albumin level (cutoff ≤3 g/dL, AUC: 0.987 95% CI: 0.953-0.998), and D-dimer level (cutoff >13,100 mcg/L, AUC: 0.776 95% CI: 0.689-0.849) all had high predictive values for mortality. CONCLUSION: Regarding the results of this research, one can conclude that BIG score is a strong predictor of mortality and morbidity in high-energy pediatric traumas. Although PRISM-3 score has a similar predictive capability, the earlier and easier calculation as-sets of BIG score positions itself as a more useful and powerful predictor for mortality and morbidity in pediatric high-energy traumas.


Assuntos
Albuminas , Unidades de Terapia Intensiva Pediátrica , Criança , Pré-Escolar , Mortalidade Hospitalar , Humanos , Escala de Gravidade do Ferimento , Morbidade , Estudos Retrospectivos
5.
Pediatr Infect Dis J ; 41(9): 742-750, 2022 09 01.
Artigo em Inglês | MEDLINE | ID: mdl-35703298

RESUMO

BACKGROUND: During the coronavirus disease 2019 (COVID-19) pandemic, the world has a large number of reported COVID-19 cases and deaths. Information on characteristics and mortality rate of pediatric intensive care unit (PICU) cases with COVID-19 remains limited. This study aims to identify the risk factors for mortality related to COVID-19 in children admitted to PICU. METHODS: A retrospective multicenter cohort study was conducted between March 2020 and April 2021 at 44 PICUs in Turkey. Children who were 1 month-18-year of age with confirmed COVID-19 admitted to PICU were included in the study. Children with multisystem inflammatory syndrome and asymptomatic for COVID-19 were excluded. RESULTS: Of 335 patients with COVID-19, the median age was 6.8 years (IQR: 1.2-14) and 180 (53.7 %) were male, 215 (64.2 %) had at least one comorbidity. Age and gender were not related to mortality. Among 335 patients, 166 (49.5%) received mechanical ventilation, 17 (5.1%) received renal replacement therapy and 44 (13.1 %) died. Children with medical complexity, congenital heart disease, immunosuppression and malignancy had significantly higher mortality. On multivariable logistic regression analysis, organ failure index [odds ratio (OR): 2.1, 95 confidence interval (CI): 1.55-2.85], and having congenital heart disease (OR: 2.65, 95 CI: 1.03-6.80), were associated with mortality. CONCLUSIONS: This study presents detailed data on clinical characteristics and outcomes of patients with COVID-19 admitted to PICU in the first pandemic year in Turkey. Our study shows that having congenital heart disease is associated with mortality. In addition, the high organ failure score in follow-up predict mortality.


Assuntos
COVID-19/complicações , Síndrome de Resposta Inflamatória Sistêmica , Adolescente , COVID-19/mortalidade , Criança , Pré-Escolar , Estado Terminal , Feminino , Humanos , Lactente , Unidades de Terapia Intensiva Pediátrica , Masculino , Estudos Retrospectivos , Fatores de Risco , Síndrome de Resposta Inflamatória Sistêmica/mortalidade , Turquia/epidemiologia
6.
Transfus Apher Sci ; 61(4): 103421, 2022 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-35305924

RESUMO

BACKGROUND: Therapeutic plasma exchange (TPE) is an extracorporeal treatment that can be used in adult and pediatric patients with acute demyelinating syndromes of the central nervous system. In this study, the efficacy and safety of TPE was evaluated in 10 pediatric patients who underwent TPE that were unresponsive to corticosteroid treatment. METHODS: Records of 10 pediatric patients who underwent TPE in our pediatric intensive care unit (PICU) between May 2017 and June 2020 were used. Expanded Disability Status Scale (EDSS), Gait Scale (GS), and Visual Outcome Scale (VOS) were applied to the patients before and after TPE. RESULTS: Of the 10 patients who underwent TPE, five were diagnosed with multiple sclerosis (MS), three with transverse myelitis (TM), and two with acute disseminated encephalomyelitis (ADEM). The median age of the patients was 13.3 years (IQR 8-15), and the median day from symptom onset to onset of TPE was 12.5 days (IQR 7-28). A total of 104 TPE sessions were performed successfully. While no complications were encountered in three patients during the sessions, the most common complication was hypofibrinogenemia. The decrease in EDSS and GS scores was found to be consistent with the clinical response of the patients. There was no statistically significant decrease in the VOS. CONCLUSIONS: With this study, we can say that TPE is a feasible, effective, and safe treatment modality in children with acute demyelinating syndromes of the central nervous system.


Assuntos
Encefalomielite Aguda Disseminada , Troca Plasmática , Adolescente , Adulto , Sistema Nervoso Central , Criança , Encefalomielite Aguda Disseminada/etiologia , Encefalomielite Aguda Disseminada/terapia , Humanos , Troca Plasmática/efeitos adversos , Plasmaferese , Estudos Retrospectivos , Síndrome
7.
Turk J Anaesthesiol Reanim ; 50(1): 18-23, 2022 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-35256341

RESUMO

OBJECTIVE: The objective of this study is to present our experience using the pressure-regulated volume control and the pressure-control ventilation modes in children. METHODS: Patients with acute respiratory failure ventilated with pressure-regulated volume control or pressure-control modes were retrospectively evaluated. The patient's ventilation parameters (of the first 7 days of ventilation or of the whole ventilation period, if the patient had been ventilated less than 7 days), SpO2, blood gases, and demographic data were collected from the pediatric intensive care unit database. RESULTS: Sixty-one patients (median age 12 [4.8-36.4] months) were enrolled in the study. The pressure-control ventilation mode was used on 40 patients (65.6%) and the pressure-regulated volume-control mode was used on 21 (34.4%) patients. Twenty-eight patients (45.9%) had hypoxemic respiratory failure and 44 (72.1%) had hypercapnic respiratory failure. The median positive end-expiratory pressure was higher in pressure-control ventilation mode (5.4 [4.2-6.3] cmH2O) than the pressure-regulated volume-control mode (4.05 [3.68-4.41] H2O, P < .001). Pressure-control mode was used more frequently in hypoxemic cases but both modes were used equally in hypercapnic cases. Hypoxic respiratory failure (yes/no), odds ratio: 3.9 (95% CI 1.2-12.3, P=.02), Ph (nadir), odds ratio: 0.004 (95% CI 0.000-0.275, P=.01), and base excess, odds ratio: 0.88 (95% CI 0.79-0.98, P=.02) were associated with intensive care mortality. CONCLUSIONS: Although the pressure-control ventilation mode was preferred more frequently in hypoxemic respiratory failure, there was no significant difference between the 2 respiratory modes in terms of length of pediatric intensive care unit stay, MV duration, and mortality. The pressure-regulated volume-control mode seems to be a safer option for physicians who do not have enough experience in using pressurecontrol ventilation mode.

8.
Braz J Anesthesiol ; 72(6): 819-822, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-34284056

RESUMO

Neisseria meningitidis, also known as meningococcus, is a relatively uncommon cause of invasive infection, but when it occurs, it is frequently severe and potentially life-threatening. A ten-year-old female patient developed a purpuric rash with fever. Upon arrival to the pediatric intensive care department, she was unconscious and in a poor general condition. We combined treatment with antibiotics, volume resuscitation, hydrocortisone, and CytoSorb.½ therapy resulted in a stabilization of hemodynamics, as well as control of hyperinflammation. We observed a significant decrease in vasopressor dosage in this patient.


Assuntos
Doenças das Glândulas Suprarrenais , Infecções Meningocócicas , Miocardite , Neisseria meningitidis , Púrpura Fulminante , Sepse , Criança , Feminino , Humanos , Púrpura Fulminante/complicações , Púrpura Fulminante/terapia , Miocardite/complicações , Miocardite/terapia , Infecções Meningocócicas/complicações , Infecções Meningocócicas/terapia , Hemorragia
9.
Arch Pediatr ; 28(7): 567-572, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-34393025

RESUMO

BACKGROUND: Research into new markers has been intensified for early diagnosis, prognosis, and differentiation of SIRS, sepsis, and septic shock in recent years. This study aimed to investigate the role of soluble triggering receptor expressed in myeloid cells-1 (sTREM-1) and interleukin (IL)-6 in distinguishing between systemic inflammatory response syndrome (SIRS), sepsis, and septic shock in pediatric intensive care unit (PICU) patients. METHODS: Between June 2014 and July 2015, 90 consecutive patients who were treated in the PICU were included in this prospective observational study. Patients were divided into four groups: control (n = 23), SIRS (n = 22), sepsis (n = 23), and septic shock (n = 22). All patients were evaluated for white blood cell (WBC), serum C-reactive protein (CRP), procalcitonin (PCT), IL-6, and sTREM-1 levels at 0, 24, and 72 h of admission. The prognostic evaluations were made using the Pediatric Risk of Mortality III (PRISM III) and Pediatric Logistic Organ Dysfunction (PELOD) scores. Patients were evaluated in terms of age, gender, prognosis, pathogen growth in culture, PRISM III and PELOD score, WBC, CRP, PCT, IL-6, and sTREM-1 levels and a comparison was made between groups. RESULTS: There was no significant difference between all groups in terms of the 0-, 24-, and 72-h sTREM-1 values (p = 0.761, p = 0.360, and p = 0.822, respectively). CRP and PCT values did not differ between the septic shock, sepsis, and SIRS groups at 0, 24, and 72 h. In the septic shock group, the 0-h IL-6 value was significantly higher than that of the SIRS group (p = 0.025). The 24-h IL-6 value in the septic shock group was significantly higher than the values of the sepsis and SIRS groups (p = 0.048 and p = 0.043, respectively). No significant difference was detected between the septic shock, sepsis, and SIRS groups in terms of IL-6 values at 72 h. CONCLUSION: sTREM-1 is not useful for the diagnosis of infection and for distinguishing between sepsis, septic shock, and SIRS since it does not offer a clear diagnostic value for PICU patients, unlike other reliable markers such as WBC, CRP, and PCT. Elevated IL-6 levels may indicate septic shock in PICU patients. More research on sTREM-1 is needed in this setting.


Assuntos
Sepse/diagnóstico , Síndrome de Resposta Inflamatória Sistêmica/diagnóstico , Receptor Gatilho 1 Expresso em Células Mieloides/análise , Adolescente , Distribuição de Qui-Quadrado , Criança , Pré-Escolar , Diagnóstico Diferencial , Feminino , Humanos , Lactente , Recém-Nascido , Unidades de Terapia Intensiva Pediátrica/organização & administração , Unidades de Terapia Intensiva Pediátrica/estatística & dados numéricos , Masculino , Estudos Prospectivos , Estatísticas não Paramétricas
10.
Turk J Anaesthesiol Reanim ; 49(5): 420-423, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-35110046

RESUMO

A 10-year-old male patient was admitted to the paediatric intensive care unit due to septic shock and oliguric acute renal failure. A haemodialysis catheter (11.5 Fr) was inserted into left subclavian vein for haemodialysis and cytokine-adsorption therapy. Haemodialysis and cytokine adsorption filter was applied to the patient for a total of two days, and then haemodialysis catheter was not used. The catheter was removed from the patient who was decided to transfer to the service on the fifth day of his admission. Tachycardia and hypotension developed and general condition deteriorated immediately after removal of the catheter. With rapid interventions, shock findings were corrected and the patient was reintubated and followed up in mechanical ventilation. On chest X-ray and thorax ultrasonography, the left hemithorax was completely filled, and a total of 1,500 mL of blood was drained by inserting a thorax tube. The patient was transferred to the paediatric pulmonology clinic after nine days of intensive care stay. Haemothorax development after subclavian catheter removal is a rare but a life-threatening condition. For these reasons, we believe that cases with removed subclavian or internal jugular vein catheters should be followed up for a suitable period of time.

11.
Turk J Pediatr ; 62(2): 274-279, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32419420

RESUMO

BACKGROUND: Tetanus is an infectious disease that can be seen in all age groups in underdeveloped and developing countries, where vaccination programs are inadequate. In developed countries, it is reported more frequently in the adult age group, where the protection of vaccination is diminished and the doses are delayed. CASE: In this report, we present generalized tetanus, which was observed in two male patients aged 12 and 6 years, admitted at different times, together with clinical course and treatment approaches. Both patients belong to different nationalities, who immigrated a couple of months before their application to our hospital. They applied with similar histories and complaints and were not vaccinated during infancy. CONCLUSION: With the development of vaccination programs, this disease with high morbidity and mortality can be prevented.


Assuntos
Doenças Transmissíveis , Tétano , Adulto , Humanos , Masculino , Tétano/diagnóstico , Tétano/prevenção & controle , Vacinação
12.
Turk Pediatri Ars ; 54(1): 49-52, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31217710

RESUMO

Nemaline myopathy, which is characterized by the accumulation of ''rod'' bodies in muscle fibers is a very rare inherited muscle disease. According to the underlying mutation, the disease has varying severity of clinical outcomes. Patients with severe forms of the disease die because of hypotonia, feeding difficulties, aspiration pneumonia, and respiratory failure in the neonatal or infancy period. Mild forms of the disease present with walking-swallowing difficulties and respiratory distress in late childhood or adulthood. A two-and-a-half-month-old boy was monitored in our Pediatric Intensive Care Unit with hypotonia, pneumonia, and respiratory distress. Nemaline myopathy was diagnosed as the result of a muscle biopsy. An advanced molecular examination revealed heterozygous mutations in the skeletal muscle α-actin (ACTA1) gene, which is the second most common cause of this disease. Nemaline myopathy should be kept in mind in patients of all age groups with respiratory failure and walking difficulty secondary to muscle weakness.


Nemalin miyopatisi oldukça nadir görülen kalitimsal bir kas hastaligi olup kas liflerinde ''rod''(nemalin) cisimcigi birikimi ile tanimlanmaktadir. Hastalik altta yatan mutasyona ve mutasyonun kalitim biçimine göre degisen agirlikta klinik gidise sahiptir. Agir sekillerinde olgular yutma ve solunum kaslarinin etkilenmesi sonucu beslenme yetersizligi, aspirasyon pnömonisi ve solunum yetmezligi nedeni ile yenidogan ya da süt çocuklugu döneminde kaybedilmektedir. Geç baslangiçli hafif olgular yasam kalitesini bozan yürüme-yutma zorlugu ve solunum sikintisi ile geç çocukluk ya da eriskin yasta bulgu verebilmektedir. Hipotoni, pnömoni ve solunum sikintisi ile Çocuk Yogun Bakim Birimi'nde izlenen iki buçuk aylik erkek bebege kas biyopsisi sonucu nemalin miyopatisi tanisi koyuldu. Ileri moleküler inceleme sonucu hastaligin ikinci en sik nedeni olan "Skeletal Muscle α-Actin" (ACTA1) geninde heterozigot mutasyon saptandi. Yenidogan döneminden eriskin döneme kadar kas güçsüzlügüne bagli solunum yetmezligi ve yutma-yürüme güçlügü varliginda yapisal miyopatiler içinde nemalin miyopatisi akilda bulundurulmali, süphenilen olgulara kas biyopsisi ya/ya da genetik inceleme yapilmalidir.

14.
Turk J Med Sci ; 48(3): 517-524, 2018 Jun 14.
Artigo em Inglês | MEDLINE | ID: mdl-29914246

RESUMO

Background/aim: The purpose of this study is to compare the diagnostic value of hepcidin level with the white blood cell (WBC), C-reactive protein (CRP), procalcitonin (PCT), and interleukin-6 (IL-6) levels in pediatric sepsis and septic shock. Materials and methods: A cohort of 89 individuals were divided into four groups: a healthy control group (HCG, n = 28), pediatric intensive care unit control group (PICUCG, n = 17), sepsis group (SG, n = 23), and septic shock group (SSG, n = 21). WBC, CRP, PCT, IL-6, and hepcidin levels were studied in the PICUCG, SG, and SSG, while hepcidin and IL-6 levels were studied in the HCG. Results: In distinguishing the SG and SSG from the HCG, hepcidin sensitivity and specificity were found to be 100%. Distinguishing between the PICUCG and the SG, hepcidin sensitivity was calculated as 95.6% and specificity was calculated as 100%. The sensitivity of WBC, CRP, and PCT was lower than that of hepcidin, but the sensitivity of IL-6 was higher than that of hepcidin. While the specificity of PCT and IL-6 was the same as hepcidin, the specificity of WBC and CRP was lower than that of hepcidin. Conclusion: Hepcidin is a more reliable indicator than WBC and CRP levels in distinguishing children with sepsis and septic shock from healthy children and nonseptic pediatric ICU patients.

15.
Pediatr Nephrol ; 33(6): 1093-1096, 2018 06.
Artigo em Inglês | MEDLINE | ID: mdl-29558000

RESUMO

BACKGROUND: Hemolytic uremic syndrome (HUS) is a clinical syndrome characterized by hemolytic anemia, thrombocytopenia, and acute kidney injury. Atypical hemolytic uremic syndrome (aHUS) is a devastating disease with significant mortality and high risk of progression to end-stage kidney disease. It is mostly caused by dysregulation of the alternative complement pathway. Cobalamin C (Cbl C) defect is a genetic disorder of cobalamin metabolism and is a rare cause of HUS. CASE-DIAGNOSIS/TREATMENT: We present a 6-month-old male infant who was admitted to the pediatric intensive care unit (PICU) due to restlessness, severe hypertension, anemia, respiratory distress, and acute kidney injury. Metabolic screening revealed elevated plasma homocysteine levels, low methionine levels, and methylmalonic aciduria, and the patient was diagnosed as having HUS secondary to Cbl C defect. Additionally, complement factor H (CFH) and complement C3 levels were decreased. The infant was treated with betaine, hydroxycobalamin, and folic acid. After treatment, the homocysteine and methylmalonic acid levels were normalized but hemolysis and acute kidney failure persisted. He required continued renal replacement treatment (CRRT) and plasma exchange due to thrombotic microangiopathy (TMA). Therefore, we considered a second mechanism in the pathogenesis as complement dysregulation and gave eculizumab to the patient. After eculizumab treatment, the renal and hematologic indices improved and he was free of dialysis. CONCLUSIONS: To the best of our knowledge, our patient is the first to have Cbl C defect-HUS accompanied by complement dysregulation, who responded well to eculizumab therapy.


Assuntos
Anticorpos Monoclonais Humanizados/uso terapêutico , Síndrome Hemolítico-Urêmica Atípica/diagnóstico , Homocistinúria/diagnóstico , Deficiência de Vitamina B 12/congênito , Síndrome Hemolítico-Urêmica Atípica/etiologia , Síndrome Hemolítico-Urêmica Atípica/terapia , Complemento C3 , Fator H do Complemento , Homocistinúria/complicações , Homocistinúria/terapia , Humanos , Lactente , Rim/patologia , Masculino , Troca Plasmática/métodos , Diálise Renal/métodos , Vitamina B 12/metabolismo , Vitamina B 12/uso terapêutico , Deficiência de Vitamina B 12/complicações , Deficiência de Vitamina B 12/diagnóstico , Deficiência de Vitamina B 12/terapia
16.
Turk J Pediatr ; 59(6): 688-692, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-30035402

RESUMO

Yesilbas O, Sevketoglu E, Kihtir HS, Hatipoglu N, Yildirim HM, Akyol MB, Aktay-Ayaz N, Gökçe I. Leptospirosis in a child with acute respiratory distress syndrome. Turk J Pediatr 2017; 59: 688-692. Leptospirosis is an infectious vasculitis, which can occur with different clinical features. While it is generally a subclinical and self-limited infection; kidney and liver dysfunction, pulmonary hemorrhage, thrombocytopenia, and cardiovascular collapse may occurr. A six-year-old boy presented with acute respiratory distress syndrome, shock, thrombocytopenia-associated multiple organ failure, and persistent high fever secondary to leptospirosis. Persistent high fever was resistant to intravenous immunoglobulin and pulse steroid therapy. He was successfully treated with plasmapheresis and hemofiltration with endotoxin-cytokine cleaning filter. In conclusion; leptospirosis may cause thrombocytopenia-associated multiple organ failure, persistent high fever, and acute respiratory distress syndrome. Plasmapheresis and hemofiltration should be considered in cases of severe leptospirosis with multiorgan failure.

17.
Turk J Pediatr ; 59(5): 576-580, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-29745120

RESUMO

Complement mediated hemolytic uremic syndrome which is caused by excessive activation of the alternative complement system is a thrombotic microangiopathy. The disease frequently occurs as a result of mutations in the genes that regulates complement proteins. Complement factor H gene has the most common mutations. A nine-month-old male patient was transferred to pediatric intensive care unit with the diagnosis of hemolytic uremic syndrome. Nonsense heterozygous p.Arg1215X mutation in the complement factor H gene was detected. The patient who had pulmonary, intestinal and hepatic involvement accompanying acute renal failure was successfully treated with therapeutic plasma exchange and eculizumab. Nonsense heterozygous p.Arg1215X mutation is extremely rare and can cause severe hemolytic uremic syndrome. As far as we know, our patient is the third case with this mutation in the literature.


Assuntos
Síndrome Hemolítico-Urêmica Atípica/genética , Fator H do Complemento/genética , Anticorpos Monoclonais Humanizados/uso terapêutico , Síndrome Hemolítico-Urêmica Atípica/complicações , Síndrome Hemolítico-Urêmica Atípica/terapia , Heterozigoto , Humanos , Lactente , Masculino , Mutação , Troca Plasmática/métodos , Análise de Sequência de DNA
18.
Balkan Med J ; 33(5): 578-580, 2016 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-27761292

RESUMO

BACKGROUND: Leptospirosis is a zoonotic infectious disease caused by pathogenic spirochetes of the genus Leptospira. Although it is usually asymptomatic and self-limited, severe potentially fatal illness accompanied by multi-organ failure may occur. CASE REPORT: Here we report an unusual case of severe leptospirosis successfully treated with continuous venovenous hemofiltration (CVVHF) and therapeutic plasma exchange (TPE). The patient presented with pericardial tamponade, renal failure and macrophage activation syndrome, and later suffered prolonged jaundice and sclerosing cholangitis during hospitalization in the pediatric intensive care unit (PICU). To the best of our knowledge, sclerosing cholangitis due to leptospirosis has not been reported in the literature. CONCLUSION: Leptospirosis should be kept in mind in the differential diagnosis of sepsis and septic shock with fever, thrombocytopenia, jaundice and renal failure. TPE and CVVHF should start early after the diagnosis of leptospirosis with multiorgan failure.

19.
Mikrobiyol Bul ; 50(1): 159-64, 2016 Jan.
Artigo em Turco | MEDLINE | ID: mdl-27058341

RESUMO

Human bocavirus (HBoV), that was first identified in 2005 and classified in Parvoviridae family, is a small, non-enveloped, single-stranded DNA virus, responsible for upper and lower respiratory tract infections, especially in young children. Although HBoV generally causes self-limited influenza-like illness, it may also lead to pneumonia, bronchiolitis, croup and asthma attacks. In this report, a case of acute bronchiolitis complicated with pneumomediastinum and bilateral pneumothorax caused by HBoV has been presented. A three-year-old boy was referred to our pediatric intensive care unit with a two day history of fever, tachypnea, hypoxia and respiratory failure. On auscultation, there were widespread expiratory wheezing and inspiratory crackles. The chest radiography yielded paracardiac infiltration and air trapping on the right lung and infiltration on the left lung. The patient had leukocytosis and elevated C-reactive protein level. On the second day of admission, respiratory distress worsened and chest radiography revealed right pneumothorax and subcutaneous emphysema in bilateral cervical region and left chest wall. He was intubated because of respiratory failure. In the thorax computed tomography, pneumomediastinum and bilateral pneumothorax were detected and right chest tube was inserted. Repetitive blood and tracheal aspirate cultures were negative. A nasopharyngeal swab sample was analyzed by multiplex real-time polymerase chain reaction method with the use of viral respiratory panel (FTD(®) Respiratory Pathogens 21 Kit, Fast-Track Diagnostics), and positive result was detected for only HBoV. On the ninth day of admission, pneumomediastinum and bilateral pneumothorax improved completely and he was discharged with cure. In conclusion, HBoV bronchiolitis may progress rare but severe complications, it should be kept in mind as an etiological agent of the respiratory tract infections especially children younger than five years old.


Assuntos
Bronquiolite/virologia , Bocavirus Humano/patogenicidade , Enfisema Mediastínico/virologia , Infecções por Parvoviridae/virologia , Pneumotórax/virologia , Bronquiolite/complicações , Pré-Escolar , Bocavirus Humano/genética , Bocavirus Humano/isolamento & purificação , Humanos , Intubação Intratraqueal , Masculino , Enfisema Mediastínico/diagnóstico por imagem , Reação em Cadeia da Polimerase Multiplex , Nasofaringe/virologia , Infecções por Parvoviridae/complicações , Pneumotórax/diagnóstico por imagem , Reação em Cadeia da Polimerase em Tempo Real , Insuficiência Respiratória/terapia , Insuficiência Respiratória/virologia , Tomografia Computadorizada por Raios X
20.
Turk Pediatri Ars ; 51(4): 228-230, 2016 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-28123338

RESUMO

A girl aged six months was hospitalized because of resistant seizures and was discharged with phenobarbital and carbamazepine therapy. She was admitted to a state hospital with symptoms of inability to waken and difficulty in breathing. It was learned that phenobarbital had been used incorrectly and the patient was sent to our pediatric intensive care unit because of severe phenobarbital overdose. The decision was taken for hemodialysis. Single-pass albumin dialysis was planned because phenobarbital can bind to high levels of plasma protein. The process was undertaken with 1% albumin-containing dialysate, which was prepared manually. After 6 hours of dialysis, the phenobarbital blood level measured 62 mcg/mL (>140 mcg/mL on admission) and the patient's clinical findings were markedly regressed. There are no case reports about phenobarbital overdose treated with single-pass albumin dialysis in the literature. We conclude that single-pass albumin dialysis may be a useful treatment, especially with intoxications of drugs that bind protein at high levels.

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