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1.
BMC Infect Dis ; 22(1): 921, 2022 Dec 09.
Artigo em Inglês | MEDLINE | ID: mdl-36494632

RESUMO

BACKGROUND: Mycobacterium (M.) chimaera is a non-tuberculous mycobacterium (NTM) that belongs to M. avium complex (MAC). In patients with cystic fibrosis (CF), MAC can cause bronchopulmonary infections that can be prolonged and difficult to treat. MAC infections of sites other than the lungs or central catheters are rare and almost exclusively associated with immunodeficiency. CASE PRESENTATION: We present a case of an 8-year-old CF patient (delF508 homozygous) with recurrent pulmonary exacerbations, gradual clinical deterioration, B-symptoms (fever, fatigue, weight loss, night sweat), elevated transaminases and intermittent detection of M. chimaera in the sputum without radiological signs of NTM-associated lung disease with a central venous port-catheter. Next-generation sequencing (NGS) revealed M. chimaera port infection that was also confirmed by mycobacterial culture. The patient recovered within 4 weeks after removal of the catheter and initiation of MAC targeted antimicrobial therapy. Electron microscopy of the catheter illustrated the presence of mycobacteria in a biofilm. CONCLUSIONS: MAC central venous catheter infection needs to be considered in immunocompetent people. NGS is a valuable tool for rapid identification of rare infections. MAC capability of biofilm formation renders catheter removal the central therapeutic intervention for the clearance of the infection.


Assuntos
Cateteres Venosos Centrais , Fibrose Cística , Infecções por Mycobacterium não Tuberculosas , Infecção por Mycobacterium avium-intracellulare , Mycobacterium , Humanos , Criança , Complexo Mycobacterium avium/genética , Fibrose Cística/complicações , Fibrose Cística/microbiologia , Infecção por Mycobacterium avium-intracellulare/diagnóstico , Infecção por Mycobacterium avium-intracellulare/microbiologia , Infecções por Mycobacterium não Tuberculosas/diagnóstico , Infecções por Mycobacterium não Tuberculosas/tratamento farmacológico , Infecções por Mycobacterium não Tuberculosas/microbiologia , Escarro/microbiologia , Micobactérias não Tuberculosas
2.
BMC Pulm Med ; 19(1): 132, 2019 Jul 18.
Artigo em Inglês | MEDLINE | ID: mdl-31319825

RESUMO

BACKGROUND: Culturing of bronchoalveolar lavage (BAL) fluid is a commonly used method for pathogen detection in pneumonia. However, the sensitivity is low, especially in patients pre-treated with anti-infective agents. The early detection of a pathogen is crucial for the outcome of respiratory tract infections. For bloodstream infections, a multiplex polymerase chain reaction (PCR) assay (SeptiFast®, SF) is available for improved pathogen detection from blood. OBJECTIVE: The aim of the present study was to determine whether the SF assay is applicable to the BAL of children with pulmonary infections and whether the frequency of pathogen detection is enhanced by the use of this multiplex PCR method. METHODS: We investigated 70 BAL samples of 70 children simultaneously by culture and multiplex PCR. The frequency of pathogen detection was compared. RESULTS: Pathogens were detected more frequently by SF than by culture (83% vs. 31%; p < 0.001). This advantage was shown for immunocompetent patients (p = 0.001) as well as for immunocompromised patients (p = 0.003). The majority (38/44; 86%) of the Gram positive cocci were only detected by SF. Fungal organisms were detected in 7/70 patients (10%) by SF and in 2/70 (3%) by culture (p = 0.125). CONCLUSION: Compared to conventional culture, the use of the SF assay on the BAL of children with pneumonia increases pathogen detection rates and therefore adds important information to guide anti-infective therapy.


Assuntos
Infecções Bacterianas/diagnóstico , Líquido da Lavagem Broncoalveolar/microbiologia , Micoses/diagnóstico , Adolescente , Adulto , Infecções Bacterianas/microbiologia , Criança , Pré-Escolar , Feminino , Fungos/isolamento & purificação , Fungos/patogenicidade , Cocos Gram-Positivos/isolamento & purificação , Cocos Gram-Positivos/patogenicidade , Humanos , Hospedeiro Imunocomprometido , Lactente , Recém-Nascido , Masculino , Reação em Cadeia da Polimerase Multiplex , Micoses/microbiologia , Pneumonia/diagnóstico , Pneumonia/microbiologia , Sensibilidade e Especificidade , Adulto Jovem
3.
Klin Padiatr ; 231(1): 35-40, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30231262

RESUMO

BACKGROUND: In general anesthesia, cartoon watching and playing video games reduce anxiety in children. It is unknown whether watching a video in the intervention room has a similar effect, and therefore is able to reduce sedative doses in children undergoing small medical procedures. Aim of this prospective study was to determine the effect of watching a standardized cartoon immediately before and during sleep induction for deep sedation on consumption of propofol in children. PATIENTS AND METHODS: 50 children aged 2-14 years undergoing deep sedation were randomly assigned to 2 groups: (A) watching or (B) not watching a standardized videoclip during sleep induction with propofol. The achievement of predefined sedation depth was objectified by measuring Comfort Score (aim 10-14) and Bispectral Index (aim 50-60). RESULTS: The median sleep induction dose of propofol did not differ in both groups (A: 1.76 (0.62-4.37) mg/kg; B: 1.37 (0.66-5.26) mg/kgKG; p=0.65). Cartoon watching was associated with shorter sleep onset latency (A: 120 (60-480)s; B: 240 (40-600)s; p=0.043). Subgroup analysis reveals this especially for school children and girls. In both groups no complications occurred. CONCLUSION: Children watching a standard cartoon during sleep induction for deep sedation have a shorter sleep onset time but sedative dose is not reduced.


Assuntos
Anestesia/psicologia , Ansiedade/prevenção & controle , Sedação Profunda/métodos , Hipnóticos e Sedativos/administração & dosagem , Cuidados Pré-Operatórios/psicologia , Propofol/administração & dosagem , Jogos de Vídeo/psicologia , Adolescente , Anestesia/métodos , Criança , Pré-Escolar , Estado de Consciência/efeitos dos fármacos , Sedação Profunda/psicologia , Feminino , Humanos , Hipnóticos e Sedativos/farmacologia , Cuidados Pré-Operatórios/métodos , Propofol/farmacologia , Estudos Prospectivos
4.
Ann Hepatol ; 17(4): 631-637, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29893707

RESUMO

INTRODUCTION AND AIM: Procalcitonin is widely used as a biomarker to distinguish bacterial infections from other etiologies of systemic inflammation. Little is known about its value in acute liver injury resulting from intoxication with paracetamol. MATERIAL AND METHODS: We performed a single-center retrospective analysis of the procalcitonin level, liver synthesis, liver cell damage and renal function of patients admitted with paracetamol-induced liver injury to a tertiary care children's hospital. Children with acute liver failure due to other reasons without a bacterial or fungal infection served as the control group. Twelve patients with acute paracetamol intoxication and acute liver injury were compared with 29 patients with acute liver failure. RESULTS: The procalcitonin levels were higher in children with paracetamol intoxication than in patients with acute liver failure without paracetamol intoxication (median 24.8 (0.01-55.57) ng/mL vs. 1.36 (0.1-44.18) ng/mL; p < 0.005), although their liver and kidney functions were better and the liver cell injury was similar in both groups. Outcome analysis showed a trend towards better survival without transplantation in patients with paracetamol intoxication (10/12 vs. 15/29). Within each group, procalcitonin was significantly correlated with alanine aminotransferase and aspartate aminotransferase but was not correlated with the International Normalized Ratio or paracetamol blood levels in the paracetamol group. In conclusion, paracetamol intoxication leads to a marked increase in procalcitonin serum levels, which are significantly higher than those seen in acute liver failure. CONCLUSION: The underlying mechanism is neither caused by infection nor fully explained by liver cell death alone and remains to be determined.


Assuntos
Acetaminofen/intoxicação , Analgésicos não Narcóticos/intoxicação , Doença Hepática Induzida por Substâncias e Drogas/sangue , Falência Hepática Aguda/sangue , Pró-Calcitonina/sangue , Adolescente , Fatores Etários , Biomarcadores/sangue , Doença Hepática Induzida por Substâncias e Drogas/diagnóstico , Doença Hepática Induzida por Substâncias e Drogas/etiologia , Criança , Pré-Escolar , Feminino , Alemanha , Humanos , Lactente , Testes de Função Renal , Falência Hepática Aguda/induzido quimicamente , Falência Hepática Aguda/diagnóstico , Testes de Função Hepática , Masculino , Estudos Retrospectivos , Fatores de Risco , Regulação para Cima
5.
Klin Padiatr ; 230(2): 68-72, 2018 03.
Artigo em Inglês | MEDLINE | ID: mdl-29045997

RESUMO

BACKGROUND: The use of Propofol and Remifentanil for analgosedation in children is common and safe. For sedation monitoring clinical scores as Comfort Score (CS) as well as bispectral index (BIS) are frequently applied. The impact of BIS for sedation monitoring in pediatric patients is still under debate. This prospective study aims to investigate whether dual sedation monitoring of CS and BIS compared with monitoring of CS alone during muscle biopsies in children can reduce sedative doses, reduce awakening time and prevent complications. METHODS: 50 pediatric patients requiring sedation for open muscle biopsy were prospectively enrolled. Analgosedation was performed with remifentanil and propofol. Patients were randomly assigned to 2 groups: In 25 patients, sedation was monitored using CS alone, and in 25 patients CS and BIS monitoring were simultaneously applied. The primary outcome was the propofol dose applied during muscle biopsy. Secondary outcome parameters were recovery time and the frequency of complications. RESULTS: The median CS during the intervention in both groups was equal (11, P=1.000). The median BIS in group 2 was 59. No complications occurred in either group. There was no difference in propofol dose in either group (8.4 vs. 7.2 mg/kg/h; P=0.58) and no difference in the duration until eye opening (9 vs. 11 min; P=0.081). CONCLUSION: For children undergoing minor surgical procedures under analgosedation, BIS monitoring does not affect the sedative dose, the time until eye opening or the frequency of complications.


Assuntos
Analgésicos Opioides/administração & dosagem , Biópsia/métodos , Monitores de Consciência , Hipnóticos e Sedativos/administração & dosagem , Propofol/administração & dosagem , Remifentanil/administração & dosagem , Criança , Humanos , Monitorização Fisiológica/métodos , Estudos Prospectivos
6.
BMC Pulm Med ; 17(1): 96, 2017 Jun 29.
Artigo em Inglês | MEDLINE | ID: mdl-28662657

RESUMO

BACKGROUND: Bloodstream pathogens can be identified by multiplex PCR (SeptiFast (SF)) or blood culture (BC); whether these pathogens are present in cystic fibrosis (CF) patients during febrile pulmonary exacerbations (FPE) has not been sufficiently studied. METHODS: In this prospective observational study, blood from CF patients experiencing FPE was tested with SF and BC before the initiation of antibiotic treatment. RESULTS: After contaminants had been excluded, 9 of 72 blood samples tested positive by BC or SF. SF exclusively detected four pathogens; BC, one. Pulmonary pathogen transmission was likely in all cases except for 2 cases of candidaemia, which were believed to be caused by catheter-related infections. For three cases, test results caused us to change the antibiotic regimen. Sensitivity (85.7% vs. 42.9%) and negative predictive value (98.4% vs. 87.0%) tended to be higher for SF than for BC. CONCLUSIONS: The results of SF and BC show that bacteraemia and fungaemia are present in CF patients during FPE and may affect antibiotic therapy. SF can help rule out catheter-related bloodstream infections.


Assuntos
Bacteriemia/diagnóstico , Candidemia/diagnóstico , Fibrose Cística/complicações , Infecções por Bactérias Gram-Negativas/diagnóstico , Adolescente , Bacteriemia/tratamento farmacológico , Bacteriemia/microbiologia , Hemocultura , Candida albicans/isolamento & purificação , Candidemia/tratamento farmacológico , Candidemia/microbiologia , Progressão da Doença , Reações Falso-Negativas , Feminino , Febre/etiologia , Infecções por Bactérias Gram-Negativas/tratamento farmacológico , Infecções por Bactérias Gram-Negativas/microbiologia , Humanos , Infecções por Klebsiella/diagnóstico , Infecções por Klebsiella/microbiologia , Klebsiella pneumoniae/isolamento & purificação , Masculino , Valor Preditivo dos Testes , Estudos Prospectivos , Infecções por Pseudomonas/diagnóstico , Infecções por Pseudomonas/microbiologia , Pseudomonas aeruginosa/isolamento & purificação , Reação em Cadeia da Polimerase em Tempo Real , Stenotrophomonas maltophilia/isolamento & purificação , Adulto Jovem
7.
Paediatr Anaesth ; 27(4): 394-398, 2017 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-28177568

RESUMO

BACKGROUND: In pediatric patients, invasive procedures such as the insertion of a central venous catheter or gastroscopy require deep sedation. It is unknown whether listening to parental voice during deep sedation in children can reduce sedative doses. AIM: The aim of this prospective study was to determine the effect of listening to a parent's voice during deep sedation on consumption of sedatives in children. METHODS: Fifty children aged 2-14 years undergoing central line placement or gastroscopy under deep sedation with propofol were randomly assigned to two groups: (A) listening or (B) not listening their parents' recorded voice reading a standardized text by the use of earphones. Depth of sedation was monitored by Comfort Score and by Bispectral Index. RESULTS: Mean sedative dose of propofol in both groups was equal (A 0.25 mg·kg-1 ·min-1 ; B 0.28 mg·kg-1 ·min-1 ; Δ -0.03 mg·kg-1 ·min-1 (CI 95% -0.08 to 0.01); P = 0.089). Furthermore, complication rate (P = 1.0) and recovery time (A 14.5 min; B 16.1 min; Δ = -1.6 min (CI 95% -6.98 to 3.81); P = 0.60) did not differ between the intervention and the control group. CONCLUSION: Listening to parental voice during deep sedation does not result in a reduction of sedative dose in children undergoing short medical procedures.


Assuntos
Sedação Profunda/métodos , Sedação Profunda/psicologia , Hipnóticos e Sedativos/administração & dosagem , Pais/psicologia , Propofol/administração & dosagem , Voz , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Estudos Prospectivos
8.
BMC Infect Dis ; 16: 378, 2016 Aug 08.
Artigo em Inglês | MEDLINE | ID: mdl-27503068

RESUMO

BACKGROUND: Antimicrobially pre-treated children with systemic inflammation often pose a diagnostic challenge to the physician. We aimed to evaluate the additional use of SeptiFast multiplex polymerase chain reaction (PCR) to identify causative pathogens in children with suspected systemic bacterial or fungal infection. METHODS: Prospective observational study in 39 children with systemic inflammatory response syndrome (SIRS) under empiric antibiotic treatment. Primary outcome was the rate of positive blood cultures (BC), compared to the rate of positive SeptiFast (SF) results. RESULTS: In total, 14 SF-samples yielded positive results, compared to 4 positive BC (p < 0.05). All blood cultures and 13 of 14 positive SF-tests were considered infection. Median time for positive BC was 2 days, and time to definite result was 6 days, compared to 12 h for SF. Antimicrobial therapy was adapted in 7 of the 14 patients with positive SeptiFast, and in 3 of the 4 patients with positive BC. Best predictive power for positive SF shown by receiver-operating characteristic was demonstrated for procalcitonin PCT (Area under the curve AUC: 0.79), compared to C-reactive protein CRP (AUC: 0.51) and leukocyte count (AUC: 0.46). A procalcitonin threshold of 0.89 ng/ml yielded a sensitivity of 0.82 and a specifity of 0.7. Children with a positive SeptiFast result on day 0 had a significantly higher risk to require treatment on the Pediatric Intensive Care Unit or to be deceased on day 30 (Odds-Ratio 8.62 (CI 1.44-51.72). CONCLUSIONS: The additional testing with SeptiFast in antimicrobially pre-treated children with systemic inflammation enhances the rate of pathogen detection. The influence of multiplex PCR on clinically relevant outcome parameters has to be further evaluated. ( TRIAL REGISTRATION: DRKS00004694).


Assuntos
Bacteriemia/diagnóstico , Fungemia/diagnóstico , Síndrome de Resposta Inflamatória Sistêmica/diagnóstico , Adolescente , Antibacterianos/uso terapêutico , Área Sob a Curva , Aspergillus fumigatus/genética , Bacteriemia/tratamento farmacológico , Bacteriemia/metabolismo , Bacteriemia/microbiologia , Proteína C-Reativa/metabolismo , Calcitonina/metabolismo , Candida/genética , Criança , Pré-Escolar , Enterococcus/genética , Escherichia coli/genética , Feminino , Fungemia/tratamento farmacológico , Fungemia/metabolismo , Fungemia/microbiologia , Humanos , Lactente , Recém-Nascido , Klebsiella/genética , Masculino , Reação em Cadeia da Polimerase Multiplex , Estudos Prospectivos , Pseudomonas/genética , Reação em Cadeia da Polimerase em Tempo Real , Sensibilidade e Especificidade , Sepse/diagnóstico , Sepse/metabolismo , Sepse/microbiologia , Staphylococcus aureus/genética , Streptococcus/genética , Síndrome de Resposta Inflamatória Sistêmica/metabolismo , Síndrome de Resposta Inflamatória Sistêmica/microbiologia
9.
Eur J Pediatr ; 175(10): 1361-9, 2016 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-27631588

RESUMO

UNLABELLED: Granulocyte transfusions for neutropenic patients have been used for over 40 years, although effectiveness, indications, and both patient and donor safety remain debated. This single-center study assessed the side effects, clinical course, and survival of granulocyte transfusions in critically ill pediatric patients, with underlying hemato-oncological disorders, prolonged neutropenia, and proven or suspected severe infection. Donor-specific side effects and influence of donor-specific characteristics on patient outcome were also investigated. A median of 4.02 × 10(10) cells was collected from 39 healthy donors for 118 granulocyte concentrates. Donors reported no significant side effects. Complications for patients were frequent but mostly minor and included vomiting, hypotension, and dyspnea. In one episode of life-threatening dyspnea, association with the granulocyte transfusion could not be ruled out. Overall survival on day 100 was 61.9 %. Patients received a median of 0.13 × 10(10) cells per kg body weight. Doses above this median were associated with a significantly better survival. Lower patient weight and age-/sex-adjusted weight were also associated with better survival. CONCLUSION: Granulocyte mobilization and collection is a safe practice. Transfusions are well tolerated in critically ill patients. Patient weight and transfused cells per kg bodyweight are major determinants of survival in pediatric patients. WHAT IS KNOWN: • Granulocyte transfusions for neutropenic patients have been used for over 40 years • The effectiveness of the technique remains controversial • Patient and donor safety remain debated • New mobilization protocols generate higher yields of granulocytes What is new: • Granulocyte collection can safely be performed • Granulocytes can safely be administered to patients • Lower patient weight and age-/sex-adjusted weight are associated with better survival rates • Patients receiving above 0.13 × 10 (10) cells per kg body weight had an excellent outcome • Further standardized, prospective studies are warranted.


Assuntos
Estado Terminal/terapia , Fator Estimulador de Colônias de Granulócitos/administração & dosagem , Granulócitos/transplante , Neutropenia/terapia , Adolescente , Adulto , Idoso , Criança , Pré-Escolar , Feminino , Fator Estimulador de Colônias de Granulócitos/efeitos adversos , Neoplasias Hematológicas/complicações , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Neutropenia/mortalidade , Análise de Regressão , Estudos Retrospectivos , Estatísticas não Paramétricas , Análise de Sobrevida , Taxa de Sobrevida , Doadores de Tecidos/estatística & dados numéricos
10.
Pediatr Transplant ; 19(1): E11-4, 2015 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-25323120

RESUMO

Bacillus cereus is a spore-forming, gram-positive bacterium that causes food poisoning presenting with either emesis or diarrhea. Diarrhea is caused by proteinaceous enterotoxin complexes, mainly hemolysin BL, non-hemolytic enterotoxin (NHE), and cytotoxin K. In contrast, emesis is caused by the ingestion of the depsipeptide toxin cereulide, which is produced in B. cereus contaminated food, particularly in pasta or rice. In general, the illness is mild and self-limiting. However, due to cereulide intoxication, nine severe cases with rhabdomyolysis and/or liver failure, five of them lethal, are reported in literature. Here we report the first case of life-threatening liver failure and severe rhabdomyolysis in this context that could not be survived without emergency hepatectomy and consecutive liver transplantation.


Assuntos
Bacillus cereus , Doenças Transmitidas por Alimentos/microbiologia , Doenças Transmitidas por Alimentos/cirurgia , Infecções por Bactérias Gram-Positivas/cirurgia , Hepatectomia , Falência Hepática/microbiologia , Falência Hepática/cirurgia , Transplante de Fígado , Adolescente , Tratamento de Emergência , Humanos , Masculino
11.
Paediatr Anaesth ; 25(3): 265-71, 2015 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-25279930

RESUMO

BACKGROUND: Open muscle biopsies in children are generally performed under general anesthesia. Alternatively, deep sedation and analgesia may be required. OBJECTIVES: The aim of our study was to compare the Bispectral Index (BIS) and Comfort Score (CS) with respect to their clinical significance for sedation/analgesia in children undergoing open muscle biopsy. METHODS: Thirty pediatric patients subjected to open muscle biopsy for diagnosis of their underlying disease were prospectively enrolled. Sedation/analgesia was performed in all patients using remifentanil and propofol. The patients were simultaneously monitored using the CS and BIS. RESULTS: All sedations and muscle biopsies were performed uneventfully. The CS and BIS were significantly correlated (R = 0.589; P < 0.01). Receiver operating characteristic (ROC) analysis revealed an area under the curve (AUC) of 0.918 with a maximum cut-off point of BIS 70.5 (sensitivity 0.9; specificity 0.785) for adequate sedation. Sensitivity of 100% was achieved at BIS 60. Accordingly, all patients with BIS ≤60 had CS within the target range of 10-14. The BIS showed substantial intra- and interindividual variability (30 points and 58 points, respectively) during sedation, whereas CS varied only within close ranges during sedation. In 25 patients, sedatives were reduced according to low BIS values (<60). No unintended anesthesia awareness was noted during the study period. CONCLUSION: Bispectral Index provides an additional helpful tool to guide sedation/analgesia in minor surgical procedures in children. BIS values ≤60 correlated with sufficient depth of sedation and prevented unintended awareness. Additionally, BIS measurement allowed for distinct regulation of depth of sedation without prolonged sedation/analgesia due to unintended overdose.


Assuntos
Biópsia/métodos , Sedação Profunda , Monitorização Fisiológica/métodos , Músculo Esquelético/patologia , Adolescente , Analgesia , Anestésicos Intravenosos , Área Sob a Curva , Criança , Pré-Escolar , Monitores de Consciência , Feminino , Humanos , Lactente , Consciência no Peroperatório/epidemiologia , Masculino , Doenças Neuromusculares/diagnóstico , Doenças Neuromusculares/patologia , Piperidinas , Propofol , Estudos Prospectivos , Curva ROC , Remifentanil
12.
Front Pediatr ; 12: 1278047, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38445077

RESUMO

Background/purpose: MEGDHEL syndrome is a rare autosomal recessive metabolic disorder, which is characterized by 3-methylglutaconic aciduria with deafness-dystonia, hepatopathy, encephalopathy and Leigh-like syndrome. It is caused by biallelic pathogenic variants in the SERAC1 gene. Due to the unspecific symptoms and the diverse manifestations of the clinical phenotype, the diagnosis is challenging. Infantile MEGDHEL syndrome often has a severe disease course with acute liver failure. Differentiation from other metabolic disorders is difficult and requires a multidisciplinary approach. Case presentation: A two-day-old small for gestational age neonate was admitted to our pediatric intensive care unit (PICU) due to severe liver failure with distinct hyperammonemia and hypoglycemia without elevation of transaminases or cholestasis. Due to high ammonia level, continuous hemodialysis was established immediately after admission. In addition, protein intake was stopped, and the patient anabolized with intravenous glucose. Temporary stabilization could be achieved after four days. In the further course, severe neurological and cardiocirculatory complications occurred, which ultimately led to the infant's death. In the metabolic diagnostics, a pronounced lactate acidosis and in urine an increased excretion of 3-methylglutaconic acid as well as other metabolites of mitochondrial energy metabolism has been the leading findings besides the hyperammonemia. Post-mortem trio whole genome analysis detected a homozygous pathogenic variant in SERAC1 with evidence of SERAC1 deficiency leading to the diagnosis of infantile MEGDHEL syndrome. Conclusion: When pediatricians are faced with hepatopathy or even acute liver failure without elevation of transaminases or cholestasis in newborns, SERAC1 deficiency should be considered as a potential differential diagnosis. The initial treatment is based on the recommended management of suspected metabolic disorders. Even while no cure is available yet, patients should be offered proper supportive management through a multidisciplinary team. In addition, genetic confirmation of the diagnosis is important for the families, especially regarding further family planning.If a newborn presents with hyperammonemia, hypoglycemia and impaired liver synthesis function without elevation of transaminases or cholestasis, the possible presence of MEGDHEL syndrome due to a SERAC1 mutation should be considered.

13.
J Clin Med ; 13(5)2024 Mar 04.
Artigo em Inglês | MEDLINE | ID: mdl-38592307

RESUMO

Background/Objectives: Precise assessment of hypnotic depth in children during procedural sedation with preserved spontaneous breathing is challenging. The Narcotrendindex (NI) offers uninterrupted information by continuous electrocortical monitoring without the need to apply a stimulus with the risk of assessment-induced arousal. This study aimed to explore the correlation between NI and the Comfort Scale (CS) during procedural sedation with midazolam and propofol and to identify an NI target range for deep sedation. Methods: A prospective observational study was conducted on 176 children (6 months to 17.9 years) undergoing procedural sedation with midazolam premedication and continuous propofol infusion. Statistical analyses included Pearson correlation of NI and CS values, logistic regression, and receiver operating curves. Results: Median NI values varied with CS and age. The correlation coefficient between CS and NI was 0.50 and slightly higher in procedure-specific subgroup analyses. The optimal NI cut-off for deep sedation was between 50 and 60 depending on the analyzed subgroup and displayed high positive predictive values for sufficient sedation throughout. Conclusion: Our study found a moderate correlation between NI and CS, demonstrating reliable identification of adequately sedated patients.

14.
Neuroradiology ; 55(2): 213-6, 2013 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-22983413

RESUMO

INTRODUCTION: This study reports the first cases of neurogenic stunned myocardium in two children with vein of Galen aneurysmal malformation after interventional treatment. PATIENTS: Two newborns with vein of Galen aneurysmal malformation and high output cardiac failure developed a severe reversible left ventricular dysfunction shortly after embolization, concurrently with acute hydrocephalus. RESULTS: There was a resolution of the cardiac symptoms of left ventricular dysfunction within a few days under treatment with milrinone and dobutamine. CONCLUSIONS: Reversible left ventricular dysfunction is observed in adult patients mainly after subarachnoid hemorrhage and is called neurogenic stunned myocardium (NSM). Other forms of brain injuries have also been identified accounting for this condition in adults. In pediatric population especially with specific cerebral diseases, NSM may be underdiagnosed.


Assuntos
Angiografia Cerebral/métodos , Aneurisma Intracraniano/complicações , Aneurisma Intracraniano/diagnóstico , Miocárdio Atordoado/diagnóstico , Miocárdio Atordoado/etiologia , Malformações da Veia de Galeno/complicações , Malformações da Veia de Galeno/diagnóstico , Cardiotônicos/uso terapêutico , Diagnóstico Diferencial , Dobutamina/uso terapêutico , Humanos , Recém-Nascido , Aneurisma Intracraniano/tratamento farmacológico , Masculino , Milrinona/uso terapêutico , Miocárdio Atordoado/tratamento farmacológico , Resultado do Tratamento , Malformações da Veia de Galeno/tratamento farmacológico
15.
Front Biosci (Landmark Ed) ; 27(11): 302, 2022 11 08.
Artigo em Inglês | MEDLINE | ID: mdl-36472101

RESUMO

BACKGROUND: Infections, major surgeries, and hyperinflammatory syndromes are known to trigger Systemic Inflammatory Response Syndrome (SIRS). Discrimination between infectious and noninfectious inflammation often poses a challenge in chronically ill patients with multiple comorbidities. These patients are routinely treated with a variety of anti-infective medications before a pathogen is identified. With the goal of improving pathogen detection rates and interventions, we evaluated Next Generation Sequencing (NGS) as a highly sensitive and fast means of detecting free microbial DNA in a small amount of serum samples from children with ongoing SIRS. METHODS: We describe seven complex pediatric patients of SIRS or prolonged fever (>38.5 °C) >72 hours in which serum samples analyzed by NGS had a major impact on therapy. One patient was analyzed twice. RESULTS: In eight NGS there were six positive results (two bacterial, three viral, one fungal) which were subsequently confirmed by microbiological culture or polymerase chain reaction (PCR) in five of the six NGS. In five of the eight performed NGS, results led to a change of therapy: antibiotic therapy was discontinued in two, escalated in one, an initiated in another; in one an antiviral was administered. CONCLUSIONS: NGS may become a valuable addition to infectious disease diagnostics in cases of pediatric SIRS. However, NGS has not yet been validated as a diagnostic method in pediatric as a diagnostic method in pediatric patients and results should therefore be interpreted with caution. Multi-center NGS evaluation studies are currently being planned.


Assuntos
Sequenciamento de Nucleotídeos em Larga Escala , Síndrome de Resposta Inflamatória Sistêmica , Humanos , Criança , Sequenciamento de Nucleotídeos em Larga Escala/métodos , Síndrome de Resposta Inflamatória Sistêmica/diagnóstico , Estado Terminal , Análise de Sequência de DNA , DNA
16.
Pediatr Pulmonol ; 56(5): 1221-1229, 2021 05.
Artigo em Inglês | MEDLINE | ID: mdl-33336889

RESUMO

INTRODUCTION: In pediatric patients, flexible bronchoscopy requires deep sedation. Different sedation regimes are common, but only some of them include opioids. Due to their antitussive effect, the use of short-acting opioids may be beneficial for this particular indication, but additional respiratory depression may lead to an increase in adverse events. Here, we systematically compared sedation regimes in children undergoing flexible bronchoscopy with either propofol alone, or a combination of propofol and remifentanil. The primary outcome parameter was the frequency of coughing episodes during the intervention. Secondary outcome parameters were frequency and types of complications, patient satisfaction, examiner satisfaction, and recovery time after finishing the sedation. METHODS: Fifty children aged 1-17 years undergoing flexible bronchoscopy under deep sedation with propofol were randomly assigned to two groups: PR receiving propofol and remifentanil and PP receiving propofol only. Sedation depth was predefined as Comfort Score 10-13. RESULTS: We found significantly less coughing episodes ([med (IQR)] PR: 0.73 (0.28-2.45)/min; PP: 1.98 (1.26-3.12)/min; p = .010) and shorter recovery time in Group PR (PR: 13.5 (8-17.5) min; PP: 21.0 (14-27) min; p = .011). Examiner's satisfaction was higher in Group PR (PR: 10 (8-10); PP: 9 (7-9); p = .012). The number of adverse events, patient satisfaction, and required propofol dose during the intervention did not differ between groups. CONCLUSION: We suggest the combination of propofol with remifentanil instead of using propofol alone in pediatric procedural sedation for flexible bronchoscopy.


Assuntos
Broncoscopia , Criança , Sedação Consciente/efeitos adversos , Humanos , Hipnóticos e Sedativos/efeitos adversos , Propofol/efeitos adversos , Estudos Prospectivos , Remifentanil
17.
Front Pediatr ; 7: 358, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31555625

RESUMO

Objective: The aim of our study was to assess the use of aEEG in our pediatric intensive care unit (PICU), indications for neuromonitoring and its findings, utility for seizure detection, and associations with outcome. Design: We retrospectively analyzed non-neonates who were treated in our PICU and received amplitude-integrated EEG (aEEG). Patients: 27 patients aged between 29 days and 10 0/12 years (median 7.3 months) were included, who received a total of 35 aEEGS. Measurements: aEEG tracings were assessed for background (BG) pattern and its evolution, seizures, and side differences using a visual classification (Hellström-Westas). Clinical data were collected from patients' histories and analyzed for correlation with aEEG findings. Main results: While rare in early years, there was an increase in use over time. Most aEEGs were conducted because of (suspected) seizures or for management of antiepileptic treatment. aEEG had low sensitivity but high specificity for recognition of pathological BG pattern with reference to conventional EEG. Worsening of BG pattern or failure to improve was associated with death. Seizure detection rates by aEEG were higher than by clinical observation, especially for identification of non-convulsive epileptic state (ES). Side differences in aEEG were rare, but if present, they were associated with unilateral brain injury. Conclusions: aEEG is useful for the detection of seizures and ES in pediatric intensive care patients. Abnormal BG pattern and poor evolution of BG are negatively associated with survival. aEEG is a potential supplement to conventional EEG, facilitating long-term surveillance of cerebral function when continuous full-channel EEG is not available. Further investigation is needed.

18.
Crit Rev Oncol Hematol ; 142: 153-163, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31404827

RESUMO

BACKGROUND: Outcomes for children diagnosed with cancer have improved dramatically over the past 20 years. However, although 40% of pediatric cancer patients require at least one intensive care admission throughout their disease course, PICU outcomes and resource utilization by this population have not been rigorously studied in this specific group. METHODS: Using a systematic strategy, we searched Medline, Embase, and CINAHL databases for articles describing PICU mortality of pediatric cancer patients admitted to PICU. Two investigators independently applied eligibility criteria, assessed data quality, and extracted data. We pooled PICU mortality estimates using random-effects models and examined mortality trends over time using meta-regression models. RESULTS: Out of 1218 identified manuscripts, 31 studies were included covering 16,853 PICU admissions with the majority being retrospective in nature. Overall pooled weighted mortality was 27.8% (95% confidence interval (CI), 23.7-31.9%). Mortality decreased slightly over time when post-operative patients were excluded. The use of mechanical ventilation (odds ratio (OR): 18.49 [95% CI 13.79-24.78], p < 0.001), inotropic support (OR: 14.05 [95% CI 9.16-21.57], p < 0.001), or continuous renal replacement therapy (OR: 3.24 [95% CI 1.31-8.04], p = 0.01) was significantly associated with PICU mortality. CONCLUSIONS: PICU mortality rates of pediatric cancer patients are far higher when compared to current mortality rates of the general PICU population. PICU mortality has remained relatively unchanged over the past decades, a slight decrease was only seen when post-operative patients were excluded. This compared infavorably with the improved mortality seen in adults with cancer admitted to ICU, where research-led improvements have led to the paradigm of unlimited, aggressive ICU management without any limitations on resuscitations status, for a time-limited trial.


Assuntos
Mortalidade Hospitalar , Hospitalização , Unidades de Terapia Intensiva Pediátrica , Neoplasias/mortalidade , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Estudos Retrospectivos
19.
Inhal Toxicol ; 19(10): 895-8, 2007 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-17687720

RESUMO

Chronic exposure to chlorine gas has been shown to cause occupational asthma. Acute inhalation of chlorine is known to cause airway inflammation and induce airway nitric oxide formation. Exhaled nitric oxide may therefore be a marker of airway damage after chlorine gas exposure. After accidental chlorine gas exposure in a swimming pool, exhaled nitric oxide and pulmonary function were repeatedly measured in 18 children over a 1-mo period. Symptomatic children with impaired pulmonary function had higher nitric oxide levels on the day after the exposure compared to day 8 and day 28. Differences in exhaled nitric oxide were more pronounced at a higher exhalation flow compared to lower flow, suggesting peripheral rather than central airway damage. This was in accordance with the observed changes in pulmonary function. No changes in exhaled nitric oxide were seen in asymptomatic children. These data suggest that acute chlorine gas exposure results in a mild increase of exhaled nitric oxide in symptomatic children.


Assuntos
Acidentes , Cloro/efeitos adversos , Expiração/efeitos dos fármacos , Exposição por Inalação/análise , Óxido Nítrico/análise , Adolescente , Criança , Cloro/administração & dosagem , Expiração/fisiologia , Feminino , Seguimentos , Humanos , Exposição por Inalação/efeitos adversos , Pneumopatias/induzido quimicamente , Pneumopatias/diagnóstico , Masculino , Óxido Nítrico/biossíntese , Piscinas
20.
Pediatr Pulmonol ; 46(11): 1114-20, 2011 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-21618715

RESUMO

BACKGROUND: There is only sparse data on the use of non-invasive ventilation (NIV) in acute respiratory failure (ARF) in infants and children. For this setting we investigated feasibility and efficacy of NIV and aimed to identify early predictors for treatment failure. PATIENTS AND METHODS: Retrospective chart review was performed for all patients treated with NIV for ARF from 2003 to 2010 on an 8-bed pediatric intensive care unit of a tertiary university hospital. RESULTS: Seventy-four patients were treated with NIV. One patient did not tolerate mask ventilation and needed immediate invasive ventilation. Intubation rate of the remaining patients was 23% and mortality 15%. Institution of NIV led to significant improvement of both respiratory and heart rate in all patients within the first hour and to further stabilization within the next 8-10 hr. In patients with NIV success blood gases improved significantly 1-2 hr after starting NIV. Multivariate analysis identified low pH after 1-2 hr to be an individual risk factor for NIV failure. Other factors tested were age, underlying disease, acute respiratory insufficiency versus post-extubation failure (PEF), and 1-2 hr after starting NIV oxygen saturation, respiratory rate, PCO(2) , and FiO(2) . Patients with PEF tended to show better outcomes compared to those with acute respiratory insufficiency. CONCLUSION: NIV can be effective in infants and children with ARF. Low pH 1-2 hr after start of NIV is associated with NIV failure. It may therefore be useful in the decision to continue or stop mask ventilation.


Assuntos
Unidades de Terapia Intensiva Pediátrica , Respiração Artificial/métodos , Adolescente , Fatores Etários , Dióxido de Carbono/sangue , Criança , Pré-Escolar , Feminino , Frequência Cardíaca , Humanos , Concentração de Íons de Hidrogênio , Masculino , Oxigênio/sangue , Insuficiência Respiratória/mortalidade , Insuficiência Respiratória/terapia , Taxa Respiratória , Estudos Retrospectivos , Fatores de Risco , Falha de Tratamento
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