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1.
Eur J Pediatr ; 181(1): 207-213, 2022 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-34241684

RESUMO

The COrona VIrus Disease 2019 (COVID-19) pandemic is posing an unprecedented challenge to healthcare systems around the globe. Europe has been struggling for 1 year now, and despite some encouraging progress (above all, the beginning of vaccination), the second wave is ongoing. Even though children are less affected than adults, the COVID-19 pandemic-and in particular the measures to counter it-is having a considerable impact on the paediatric healthcare setting. It is, therefore, the duty of paediatric teams in Europe to prepare for the challenges ahead. We wish to contribute to this necessary preparedness in two ways: firstly, by assessing the direct and indirect impact of the pandemic on children and on the paediatric setting; secondly, and more importantly, by identifying the various responsibilities of paediatric healthcare professionals, in light of established ethical principles. Only abiding by these responsibilities will it be possible to ensure that ill children and their families are properly supported even in these difficult times and to grant that decisions about children's healthcare remain morally justified and lawful. What is Known: • The COVID-19 outbreak is posing an unprecedented challenge to healthcare systems around the globe • Despite the children are less affected than adults, the COVID-19 pandemic is having a huge impact also on paediatric setting What is New: • The COVID-19 pandemic lays out specific responsibilities of paediatric professionals towards our pa-tients, society and ourselves • The paediatric teams in Europe should assess the direct and indirect impact of the pandemic on the chil-dren and on the paediatric settings, ensuring consistency between centres and across regions in Europe.


Assuntos
COVID-19 , Pandemias , Adulto , Criança , Atenção à Saúde , Pessoal de Saúde , Humanos , Pandemias/prevenção & controle , SARS-CoV-2
2.
Pediatr Transplant ; 19(5): E115-20, 2015 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-25929598

RESUMO

UNLABELLED: Rituximab (anti-B CD20 ab.) in recently widely used in renal transplantation. CASE HISTORY: A 10-yr-old patient with end-stage renal failure due to multidrug-resistant NS was transplanted with renal graft from deceased donor and presented immediate recurrence of NS. PF was started on day 3 and patient received MP pulses, however with no effect. Rituximab (4 × 375 mg/m(2)) was administered. Chest radiographs taken at that time were normal. Partial remission was achieved and the patient was discharged in good condition. Sequential recurrence appeared two wk afterward. Twelve sessions of PF were performed and six pulses of MP were given, effecting a partial remission. Three months after the last dose of rituximab, patient was admitted with increasing respiratory failure, requiring mechanical ventilation. Infectious background, including CMV, BKV, mycoplasma, and pneumocystis, was not confirmed. The patient was treated with MP pulses, IVIG, and a variety of antibiotics. Ground-glass opacity was confirmed on lung CT images. Respiratory failure worsened, despite aggressive ventilation and patient passed away after three wk at ICU. A destruction of alveolar epithelium and extended pulmonary fibrosis was confirmed in the autopsy report. The case represents a fatal RALI.


Assuntos
Falência Renal Crônica/cirurgia , Lesão Pulmonar/induzido quimicamente , Pulmão/efeitos dos fármacos , Síndrome Nefrótica/tratamento farmacológico , Síndrome Nefrótica/cirurgia , Rituximab/efeitos adversos , Criança , Evolução Fatal , Humanos , Transplante de Rim/efeitos adversos , Lesão Pulmonar/complicações , Síndrome Nefrótica/etiologia , Complicações Pós-Operatórias , Fibrose Pulmonar/etiologia , Recidiva , Indução de Remissão
3.
Mycoses ; 57(2): 105-9, 2014 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-23834472

RESUMO

Data on the epidemiology of invasive Candida infections in paediatric patients in Europe are still limited. The aim of this retrospective study was to analyse the epidemiology of candidaemia in a tertiary paediatric hospital in Poland from 2000 to 2010. Using microbiological records, a total of 118 episodes of candidaemia were identified in 114 children, with an annual incidence of 0.35 episodes/1000 discharges. The highest incidences were found in the medical intensive care unit (5.28), and in neonatal intensive care (1.47). The mortality rate was 8.5%. Candida albicans and C. parapsilosis were the most prevalent species (39.8% and 35.6% respectively). The prevalence of non-albicans species increased from 12.5% in 2000 to 70% in 2010. No differences were found between C. albicans and C. non-albicans episodes in terms of demographics, risk factors or mortality. The highest resistance rates (overall 7.6%) were observed for fluconazole (4.3% in C. albicans, 7.1% in C. parapsilosis and 13.8% in other Candida species). Resistance to amphotericin B (2.5%) was limited to non-albicans isolates. The dynamic changes in species distribution and increasing resistance of fungal pathogens confirm the importance of epidemiological surveillance.


Assuntos
Candidemia/epidemiologia , Adolescente , Antifúngicos/farmacologia , Candida/classificação , Candida/isolamento & purificação , Criança , Pré-Escolar , Estudos de Coortes , Farmacorresistência Fúngica , Feminino , Hospitais Pediátricos , Humanos , Incidência , Lactente , Recém-Nascido , Masculino , Testes de Sensibilidade Microbiana , Polônia/epidemiologia , Estudos Retrospectivos , Análise de Sobrevida , Centros de Atenção Terciária
4.
PLoS One ; 19(5): e0301612, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38781220

RESUMO

A retrospective observational study was conducted among healthcare workers (HCWs) in a tertiary paediatric hospital. The study covered the period before and after implementation of the vaccination programme and evaluated the incidence of new SARS-CoV-2 infections in both periods. Risk factors of the new SARS-CoV-2 infection and COVID-19 vaccine effectiveness was also assessed in a real-world setting. The overall incidence of SARS-CoV-2 infections among HCWs in the study period was 19.4% with a high proportion of asymptomatic individuals (45.1%). The incidence before vaccination was 16.6% and nurses had a higher risk of infection, while physicians had a reduced risk (OR 1.80, 95% CI 1.29-2.52; and OR 0.45, 95% CI 0.30-0.68). Within two months of implementation, the programme achieved a high (88.9%) vaccination coverage in our cohort, although some disparities in vaccination rates were observed. In particular, older individuals, physicians, those working in clinical settings, and those previously uninfected were more likely to be vaccinated. The overall incidence of SARS-CoV-2 infection after vaccination deployment was 6.4% (40.0% in unvaccinated individuals and 3.2% in individuals vaccinated with at least one dose). The estimated vaccine efficacy was high (95.0%) in fully vaccinated HCWs and similar to those observed previously in clinical trials and real-world settings.


Assuntos
Vacinas contra COVID-19 , COVID-19 , Pessoal de Saúde , Hospitais Pediátricos , SARS-CoV-2 , Centros de Atenção Terciária , Vacinação , Humanos , COVID-19/epidemiologia , COVID-19/prevenção & controle , Estudos Retrospectivos , Feminino , Masculino , Incidência , Pessoal de Saúde/estatística & dados numéricos , Hospitais Pediátricos/estatística & dados numéricos , Adulto , Vacinas contra COVID-19/administração & dosagem , SARS-CoV-2/imunologia , Vacinação/estatística & dados numéricos , Pessoa de Meia-Idade , Fatores de Risco
5.
Biol Trace Elem Res ; 2024 Feb 07.
Artigo em Inglês | MEDLINE | ID: mdl-38321303

RESUMO

In humans, 23 elements have been shown to have biological activity. The purpose of this study was to evaluate the concentrations of iron (Fe), zinc (Zn), sodium (Na), potassium (K), magnesium (Mg), phosphorus (P), and calcium (Ca) in the serum of patients diagnosed with lumbar degenerative stenosis when compared to the concentrations of those elements in the serum of healthy volunteers. The study group consisted of 60 patients who were diagnosed with degenerative stenosis of the lumbosacral spine and who qualified for hemilaminectomy. The control group included 60 healthy volunteers without degenerative spinal stenosis. The clinical specimens studied had sera collected from both groups. The quantitative analysis of the selected elements revealed statistically significant (p < 0.05) lower concentrations of Zn (740 ± 110 µg/L vs. 880 ± 160 µg/L) and Mg (22,091 ± 4256 µg/L vs. 24,100 ± 4210 µg/L) in the serum of the patients from the study group when compared to the controls. By contrast, K (16,230 µg/L ± 1210 µg/L vs. 13,210 µg/L ± 1060 µg/L) and Fe (141.87 µg/L ± 11.22 µg/L vs. 109.1 µg/L ± 26.43 µg/L) levels were significantly higher in the study group compared to the controls (p < 0.05). No statistically significant changes were detected in the concentrations of the assessed micronutrients and macronutrients in both sexes in either the study group, the control group, or those based on body mass index (p > 0.05). In the serum samples from the study group, the strongest correlations were noted between the concentrations. In the study group, we showed a significant relationship between the levels of Fe/Zn (r = 0.41), Fe/Na (r = 0.41), Fe/P (r = 0.55), Zn/P (r = 0.68), Zn/K (r = 0.48), Zn/Ca (r = 0.94), Mg/Ca (r = 0.79), and Na/K (r = 0.67). We showed that only Mg concentration varied statistically significantly with the severity of pain (p < 0.05). These findings suggest that the assessment of Fe, Zn, Mg, and K concentrations can be helpful in predicting the onset of degenerative changes in the spine.

6.
Ginekol Pol ; 82(1): 39-43, 2011 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-21469520

RESUMO

INTRODUCTION: The paper discusses the reliability of the Apgar score for evaluating newborns, particularly its usefulness in assessing the state of preterm or full term newborns born with hypoxia. AIM: The paper provides a sum-up of the opinions on usefulness and reliability of the Apgar score given by doctors from 255 NICUs. MATERIAL AND METHODS: Data for the analysis were gathered by means of a questionnaire opinion poll sent to 158 primary referral centers, 71 secondary referral centers and 26 tertiary referral centers. Respondents answered questions about value of the Apgar score assessment (highly valuable, limited value, always reliable), as well as overrating and underrating children born in good overall condition and children born with clinical and biochemical indicators of hypoxia. In the group of prematurely born babies, the data concerning newborns with very low (VLBW) and extremely low birth weight (ELBW) were analyzed separately RESULTS: 88.5% neonatologists claimed the use of the Apgar score in assessing newborn condition to be of little value and only 11.5% found this indicator useful and reliable. CONCLUSIONS: According to the majority of Polish neonatologists, Apgar score is not reliable in the assessment of term and preterm hypoxic newborns.


Assuntos
Índice de Apgar , Asfixia Neonatal/classificação , Salas de Parto/organização & administração , Recém-Nascido de Baixo Peso , Recém-Nascido Prematuro , Asfixia Neonatal/diagnóstico , Asfixia Neonatal/epidemiologia , Peso ao Nascer , Feminino , Humanos , Recém-Nascido , Unidades de Terapia Intensiva Neonatal/organização & administração , Masculino , Assistência Perinatal/métodos , Polônia/epidemiologia , Vigilância da População , Reprodutibilidade dos Testes , Projetos de Pesquisa , Inquéritos e Questionários
7.
PLoS One ; 16(4): e0249550, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33793673

RESUMO

Data on the prevalence of the SARS-CoV-2 antibody in healthcare workers (HCWs) is scarce, especially in pediatric settings. The purpose of this study was to evaluate SARS-CoV-2 IgG-positivity among HCWs of a tertiary pediatric hospital. In addition, follow-up of the serological response in the subgroup of seropositive HCWs was analysed, to gain some insight on the persistence of IgG antibodies to SARS-CoV-2. We performed a retrospective analysis of voluntary SARS-CoV-2 IgG testing, which was made available free of charge to HCWs of the Children's Memorial Health Institute in Warsaw (Poland). Plasma samples were collected between July 1 and August 9, 2020, and tested using the Abbott SARS-CoV-2 IgG assay. Of 2,282 eligible participants, 1,879 (82.3%) HCWs volunteered to undergo testing. Sixteen HCWs tested positive for SARS-CoV-2 IgG, corresponding to a seroprevalence of 0.85%. Among seropositive HCWs, three HCWs had confirmed COVID-19. Nine (56.3%) of the seropositive HCWs reported neither symptoms nor unprotected contact with confirmed SARS-CoV-2 cases in the previous months. A decline in the IgG index was observed at a median time of 86.5 days (range:84‒128 days) after symptom onset or RT-PCR testing. Further studies are necessary to elucidate the duration of persistence of anti-SARS-CoV-2 antibodies, as well as the correlation between seropositivity and protective immunity against reinfection. Regardless of the persistence of antibodies and their protective properties, such low prevalence indicates that this population is vulnerable to a second wave of the COVID-19 pandemic.


Assuntos
Anticorpos Antivirais/sangue , COVID-19 , Pessoal de Saúde/estatística & dados numéricos , Imunoglobulina G/sangue , SARS-CoV-2/imunologia , Adulto , COVID-19/epidemiologia , COVID-19/imunologia , Feminino , Hospitais Pediátricos/estatística & dados numéricos , Humanos , Masculino , Pessoa de Meia-Idade , Pandemias , Polônia , Prevalência , Estudos Retrospectivos , Estudos Soroepidemiológicos , Centros de Atenção Terciária/estatística & dados numéricos
8.
Anaesthesiol Intensive Ther ; 53(5): 369-375, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-35100794

RESUMO

In Poland, guidelines for the management of ineffective treatment of children in neonatal and paediatric departments developed by the Polish Neonatal Society and the Polish Paediatric Society, have been published. The specific problems of futile therapy in paediatric anaesthesiology and intensive care units should be defined and solved separately. For this purpose, the guidelines presented below were prepared. They present the principles for managing children for whom therapeutic options available in paedia-tric anaesthesiology and intensive care units have been exhausted and ineffectiveness of maintaining organ functions, i.e. futile therapy, has been suspected. The decision to withdraw futile therapy of a child is undoubtedly one of the most difficult for both doctors and parents, and for this reason, it should be made collectively, respecting the dignity of the child and his/her parents or legal representatives, and continuing the management aimed at relieving the child's pain and suffering, as well as minimising anxiety and fear. Due to the small amount of reliable evidence-based data, the guidelines constitute the consensus of the Group of Experts and are dedicated to minor patients treated in paediatric anaesthesiology and intensive care units.


Assuntos
Anestesiologia , Cuidados Críticos , Criança , Consenso , Feminino , Humanos , Recém-Nascido , Unidades de Terapia Intensiva , Unidades de Terapia Intensiva Pediátrica , Masculino , Futilidade Médica
9.
J Clin Med ; 10(18)2021 Sep 18.
Artigo em Inglês | MEDLINE | ID: mdl-34575352

RESUMO

BACKGROUND: Brain death/death by neurologic criteria (BD/DNC) guidelines are routinely analyzed, compared and updated in the majority of countries and are later implemented as national criteria. At the same time, extensive works have been conducted in order to unify clinical procedures and to validate and implement new technologies into a panel of ancillary tests. Recently evaluated computed tomography angiography and computed tomography perfusion (CTA/CTP) seem to be superior to traditionally used digital subtraction angiography (DSA), transcranial Doppler (TCD) and cerebral perfusion scintigraphy for diagnosis of cerebral circulatory arrest (CCA). In this narrative review, we would like to demonstrate scientific evidence supporting the implementation of CTA/CTP in Polish guidelines for BD/DNC diagnosis. Research and implementation process: In the first of our base studies concerning the potential usefulness of CTA/CTP for the confirmation of CCA during BD/DNC diagnosis procedures, we showed a sensitivity of 96.3% of CTA in a group of 82 patients. CTA was validated against DSA in this report. In the second study, CTA showed a sensitivity of 86% and CTP showed a sensitivity of 100% in a group of 50 patients. In this study, CTA and CTP were validated against clinical diagnosis of BD/DNC supported by TCD. Additionally, we propose our CCA criteria for CTP test, which are based on ascertainment of cerebral blood flow (CBF) < 10 mL/100 g/min and cerebral blood volume < 1 mL/100 g in regions of interest (ROIs) localized in all brain regions. Based on our research results, CTA/CTP methods were implemented in Polish BD/DNC criteria. To our knowledge, CTP was implemented for the first time in national guidelines. CONCLUSIONS: CTA and CTP-derived CTA might be in future the tests of choice for CCA diagnosis, proper and/or Doppler pretest might significantly increase sensitivity of CTA in CCA diagnosis procedures. Whole brain CTP might be decisive in some cases of inconclusive CTA. Implementation of CTA/CTP in the Polish BD/DNC diagnosis guidelines does not show any major obstacles. We believe that in next edition of "The World Brain Death Project" CTA and CTP will be recommended as ancillary tests of choice for CCA confirmation during BD/DNC diagnosis procedures.

10.
Anaesthesiol Intensive Ther ; 53(2): 97-102, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34284550

RESUMO

We would like to invite paediatric intensive care units (PICU) to join our multi-center trial concerning patient population < 12 y/o and aiming at: • validation of computed tomography angiography (CTA)/computed tomography perfusion (CTP) tests for brain death/death by neurological criteria (BD/DNC) diagnosis procedures, • validation of duplex Doppler insonation of extracranial segments of the internal cerebral arteries and the vertebral arteries for choosing an optimal time for CTA/CTP testing.


Assuntos
Morte Encefálica , Parada Cardíaca , Morte Encefálica/diagnóstico por imagem , Criança , Angiografia por Tomografia Computadorizada , Humanos , Perfusão , Tomografia Computadorizada por Raios X
11.
Pneumonol Alergol Pol ; 78(6): 392-8, 2010.
Artigo em Polonês | MEDLINE | ID: mdl-21077031

RESUMO

INTRODUCTION: Home mechanical ventilation (HMV) is increasingly used in the treatment of chronic respiratory failure thanks to rapid technological development, increasing number of elderly people and extension of indications. The aim of the study was to assess: prevalence of HMV in Poland, the proportions of lung disease and neuromuscular patients using HMV and the type of interface (invasive v. non-invasive). MATERIAL AND METHODS: The questionnaire was send to all institutions providing HMV in Poland and to regional departments of National Health System (NHS). RESULTS: All NHS departments responded. They reported 846 HMV users, 31% of children. The prevalence of HMV in Poland was assessed as 2,2 patient per 100.000 population without striking differences between provinces. Among 39 HMV centers in Poland 12 (31%) answered. They reported 206 patients (24% of all HMV users). Proportion of ventilation mode consisted of 59% (122 pts) treated via a tracheostomy and 41% (84 pts) with non invasive ventilation (NIV). 168 patients (82%) had neuromuscular diseases (ND), majority of them muscular dystrophy - 57 patients ( 34% of ND) and amyotrophic lateral sclerosis - 39 patients (23% of ND). There were only 38 patients (18%) with lung and thoracic cage diseases: 17 with COPD and 10 with kyphoscoliosis. CONCLUSIONS: The prevalence of HMV treatment in Poland has developed dramatically in the last decade, but is still very low comparing to other European countries, especially due to very low number of patients with lung and chest wall diseases. The prevalence of invasive mode of ventilation is extremely high. The most important factors which inhibit development of HMV in Poland are: omission of respiratory physicians in the process of qualification, lack of national guidelines, sophisticated demands for HMV providers. The awareness of the need of HMV especially in patients with respiratory failure due to obesity hypoventilation syndrome and restrictive lung diseases should be increased among chest physicians.


Assuntos
Esclerose Lateral Amiotrófica/terapia , Serviços de Assistência Domiciliar/estatística & dados numéricos , Pneumopatias/terapia , Doenças Neuromusculares/terapia , Respiração Artificial/estatística & dados numéricos , Doenças Torácicas/terapia , Ventiladores Mecânicos/estatística & dados numéricos , Adulto , Distribuição por Idade , Idoso , Esclerose Lateral Amiotrófica/epidemiologia , Criança , Feminino , Humanos , Pneumopatias/epidemiologia , Masculino , Pessoa de Meia-Idade , Distrofias Musculares/terapia , Doenças Neuromusculares/epidemiologia , Polônia/epidemiologia , Prevalência , Qualidade de Vida , Testes de Função Respiratória , Inquéritos e Questionários
12.
Orphanet J Rare Dis ; 15(1): 252, 2020 09 21.
Artigo em Inglês | MEDLINE | ID: mdl-32958024

RESUMO

BACKGROUND: Congenital Central Hypoventilation Syndrome (CCHS) is a rare condition characterized by an alveolar hypoventilation due to a deficient autonomic central control of ventilation and a global autonomic dysfunction. Paired-like homeobox 2B (PHOX2B) mutations are found in most of the patients with CCHS. In recent years, the condition has evolved from a life-threatening neonatal onset disorder to include broader and milder clinical presentations, affecting children, adults and families. Genes other than PHOX2B have been found responsible for CCHS in rare cases and there are as yet other unknown genes that may account for the disease. At present, management relies on lifelong ventilatory support and close follow up of dysautonomic progression. BODY: This paper provides a state-of-the-art comprehensive description of CCHS and of the components of diagnostic evaluation and multi-disciplinary management, as well as considerations for future research. CONCLUSION: Awareness and knowledge of the diagnosis and management of this rare disease should be brought to a large health community including adult physicians and health carers.


Assuntos
Hipoventilação/congênito , Apneia do Sono Tipo Central , Adulto , Criança , Proteínas de Homeodomínio/genética , Humanos , Hipoventilação/diagnóstico , Hipoventilação/genética , Hipoventilação/terapia , Mutação , Apneia do Sono Tipo Central/diagnóstico , Apneia do Sono Tipo Central/genética , Apneia do Sono Tipo Central/terapia , Fatores de Transcrição/genética
14.
Scand J Trauma Resusc Emerg Med ; 27(1): 87, 2019 Sep 18.
Artigo em Inglês | MEDLINE | ID: mdl-31533787

RESUMO

Children form a specific group of patients, as there are significant differences between children and adults in both anatomy and physiology. Difficult airway may be unanticipated or anticipated. Difficulties encountered during intubation may cause hypoxia, hypoxic brain injury and, in extreme situations, may result in the patient's death. There are few paediatric difficult-airway guidelines available in the current literature, and some of these have significant limitations. This position paper, intended for unanticipated difficult airway, was elaborated by the panel of specialists representing the Polish Society of Anaesthesiology and Intensive Care as well as the Polish Neonatal Society. It covers both elective intubation and emergency situations in children in all age groups. An integral part of the paper is an algorithm. The paper describes in detail all stages of the algorithm considering some modification in specific age groups, i.e. neonates.


Assuntos
Manuseio das Vias Aéreas/normas , Algoritmos , Anestesiologia/métodos , Cuidados Críticos/normas , Hipóxia/terapia , Sociedades Médicas , Criança , Humanos
15.
Ann Transplant ; 13(2): 28-31, 2008.
Artigo em Inglês | MEDLINE | ID: mdl-18566556

RESUMO

BACKGROUND: Fulminant Wilson's disease (FWD) is rare and fatal condition in children unless liver transplantation is performed, however introduction of new technologies could change this poor prognosis. The aim of our study was retrospective analysis of clinical course, treatment and outcome of children with FWD treated in our institution. MATERIAL/METHODS: Between 1999-2007 we've treated in our hospital 13 patients with mean age of 15.5 yrs with FWD. We performed retrospective analysis of clinical course, biochemical parameters, MELD/PELD score, Wilson score and Kings'-College criteria for LTx in acute liver failure in all these patients. Type of treatment and final outcome were analyzed, as well as qualification for transplantation was reevaluated in each case in accordance to pathological examination of explanted during transplantation livers. RESULTS: The initial symptoms of FWD were typically weakness, abdominal pain and developing later after 5-60 days (mean 20 days), jaundice. Eleven patients developed neurological symptoms with coma lasting for 2-11 days before transplantation or death. Maximal serum bilirubin concentration ranged between 4.5-71.6 mg% (mean 42.24 mg%), INR 2.9-10.0 (mean 5.4). MELD/PELD score was between 21-58 (mean 38), 10 patients fulfilled general King's-College criteria for transplantation in acute liver failure. Wilson's index ranged between 11 and 17 points (mean 13 points). In 11 children urgent liver transplantation (LTx) was performed, 1 child recovered on albumin dialysis and chelating treatment, 1 child died shortly after very late referral to our center. Actual follow-up of living patients is 0.36-7.43 years (mean 2.57 yrs), all are doing well with good liver function. CONCLUSIONS: FWD lead to death in almost all pediatric patients if LTx can not be performed, however early introduction of albumin dialysis (MARS) and chelating therapy allowed for survival without transplantation in single patient. It seems also that MARS therapy allows for at least prolongation of waiting time for LTx. Wilson's was slightly better predictor of need for LTx in our patients than classical King's-College criteria.


Assuntos
Degeneração Hepatolenticular/cirurgia , Transplante de Fígado , Adolescente , Terapia por Quelação , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Degeneração Hepatolenticular/diagnóstico , Degeneração Hepatolenticular/mortalidade , Humanos , Testes de Função Hepática , Masculino , Recuperação de Função Fisiológica , Estudos Retrospectivos , Taxa de Sobrevida , Resultado do Tratamento , Adulto Jovem
16.
Anaesthesiol Intensive Ther ; 49(5): 336-349, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-29286529

RESUMO

Tracheal intubation may be defined as an artificial airway established in order to provide mechanical ventilation of the lungs during surgical procedures under general anaesthesia, treatment in an intensive care unit, as well as in emergency situations. Difficulties encountered during intubation may cause hypoxia, hypoxic brain injury and, in extreme situations, may result in the patient's death. There may be unanticipated and anticipated difficult airway. Children form a specific group of patients as there are significant differences in both anatomy and physiology. There are some limitations in equipment used for the airway management in children. There are only few paediatric difficult airway guidelines available, some of which have significant limitations. The presented algorithm was created by a group of specialists who represent the Polish Society of Anaesthesiology and Intensive Therapy, as well as the Polish Neonatology Society. This algorithm is intended for the unanticipated difficult airway in children and can be used in all age groups. It covers both elective intubation, as well as rescue techniques. A guide forms an integral part of the algorithm. It describes in detail all stages of the algorithm considering some modifications in a specific age group, e.g. neonates. The main aim of Stage I is to optimise conditions for face mask ventilation, laryngoscopy and intubation. Stage IIA focuses on maximising the chances of successful intubation when face mask ventilation is possible. Stage IIB outlines actions aimed at improving face mask ventilation. Stage IIIA describes the use of a SAD (Supraglottic Airway Device) during effective face mask ventilation or in a CICV (Cannot Intubate, Cannot Ventilate) situation. Stage IIIB outlines intubation through a SAD. Stage IV describes rescue techniques and outlines possible options of either proceeding with surgery or postponing it, depending on clinical situation.


Assuntos
Manuseio das Vias Aéreas/métodos , Intubação Intratraqueal/métodos , Respiração Artificial/métodos , Algoritmos , Anestesiologia/métodos , Criança , Consenso , Cuidados Críticos/métodos , Humanos , Máscaras Laríngeas , Laringoscopia/métodos , Polônia
17.
Folia Neuropathol ; 55(2): 146-153, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28677371

RESUMO

Biotin-thiamine-responsive basal ganglia disease is a severe form of a rare neurogenetic disorder caused by pathogenic molecular variants in the thiamine transporter gene. Nowadays, a potentially effective treatment is known, therefore the early diagnosis is mandatory. The aim of the paper was to assess the contribution of neuropathological and magnetic resonance imaging (MRI) studies to a proper diagnosis. We present the brain study of two Polish patients with SLC19A3 mutations, including (1) an infant with an intriguing "walnut" appearance of the brain autopsied many years before the discovery of the SLC19A3 defect, and (2) a one-year-old patient with clinical features of Leigh syndrome. In patient 2, biotin/thiamine responsiveness was not tested at the time of diagnosis and causal treatment started with one-year delay. The central nervous system lesions found in the patients displayed almost clearly a specific pattern for SLC19A3 defect, as previously proposed in diagnostic criteria. Our study presents a detailed description of neuropathological and MRI findings of both patients. We confirm that the autopsy and/or MRI of the brain is sufficient to qualify a patient with an unknown neuropathological disorder directly for SLC19A3 mutations testing and a prompt trial of specific treatment. .


Assuntos
Doenças dos Gânglios da Base/patologia , Encéfalo/patologia , Proteínas de Membrana Transportadoras/genética , Doenças dos Gânglios da Base/genética , Humanos , Recém-Nascido , Masculino , Mutação
18.
Przegl Lek ; 63 Suppl 3: 78-81, 2006.
Artigo em Polonês | MEDLINE | ID: mdl-16898495

RESUMO

Continuous renal replacement therapy (CRRT) has became a modality of choice in chidren with acute renal failure (ARF), especially in cases of multiorgan failure (MOF) and in hemodynamically unstable patients in whom regular hemodialysis is difficult to reform. Newborns and infants with contraindications to peritoneal dialysis are another group of patients treated with CRRT. Retrospective analysis of CRRT therapy in 112 patients treated with (CVVHD, CVVH, CVVHDF, SCUF-continuous veno-venous hemodialysis/hemofiltration/ hemodiafiltration/ultrafiltration) between 2000-2005 is presented. Indication to CRRT was MOF (n=23, 20%), complications post-liver transplant (n=33, 29%), congenital metabolic defects (n=5, 4.5%), complications of cancer or chemotherapy (n=11, 9.8%) and other causes of ARF (n=40, 36.7%). Overall mortality was 36.6%. The highest rate was seen in children between 0-2 years of age (52,3%) and in patients with congenital metabolic diseases (80%). When adjusted to specific modality--the highest mortality was seen in patients treated with CVVHF (55.4%), while lower was in cases treated with CVVHD (37.8%) and CVVHDF (35.4%). Among older children higher mortality was seen in patients with mean arterial pressure (MAP) <70 mmHg (68.4%), compared to patients with MAP >70 mmHg (23.1%).


Assuntos
Injúria Renal Aguda/mortalidade , Injúria Renal Aguda/terapia , Terapia de Substituição Renal/mortalidade , Adolescente , Adulto , Criança , Pré-Escolar , Soluções para Diálise/química , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Masculino , Insuficiência de Múltiplos Órgãos/mortalidade , Insuficiência de Múltiplos Órgãos/terapia , Estudos Retrospectivos
19.
Endokrynol Pol ; 67(2): 226-31, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-26884306

RESUMO

ROHHAD syndrome (Rapid-onset Obesity with Hypothalamic Dysfunction, Hypoventilation, and Autonomic Dysregulation) is characterized by rapid-onset obesity in young children, hypoventilation, and hypothalamic and autonomic dysfunction. The exact aetiology of the disease remains unknown, and the number of reported cases seems to be underestimated. We present the case of a nine-year-old male patient suspected of ROHHAD due to weight gain since early childhood, decreased height velocity, hypoventilation, hypodipsia, excessive perspiration, and pyrexial episodes. The presented symptoms, and laboratory and imaging findings met the criteria of ROHHAD syndrome. ROHHAD should be considered in differential diagnosis for obesity in children. Early identification of the disease prevents potential complications specific for the syndrome, in particular a life-threatening cardio-pulmonary arrest. Patients with ROHHAD require regular follow-up by a multidisciplinary team.


Assuntos
Anormalidades Múltiplas/fisiopatologia , Doenças do Sistema Nervoso Autônomo/fisiopatologia , Doenças Hipotalâmicas/fisiopatologia , Hipoventilação/fisiopatologia , Obesidade/fisiopatologia , Criança , Humanos , Masculino , Síndrome
20.
Respir Care ; 60(4): 577-85, 2015 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-25492950

RESUMO

BACKGROUND: Home mechanical ventilation (HMV) is a routine method of treatment for patients with chronic ventilatory failure. Over the last 20 y, a marked development in HMV has been noted in terms of its prevalence and the changing proportion of patients with various indications. However, data on HMV come exclusively from the developed countries of Europe and North America. Nowadays, we can see the emergence of HMV in less developed countries. This study aimed to describe the development of HMV in Poland. METHODS: Data from the largest HMV centers were retrospectively evaluated with regard to cause of respiratory failure, ventilation technique, and characteristics of the HMV-implementing institution. RESULTS: The number of subjects treated with HMV increased from 8 in 2000 to 928 in 2010. Neuromuscular diseases remained the main indication. However, their relative contribution decreased from 100 to 51% in favor of pulmonary diseases (an increase from 0 to 21%) and hypoventilation syndromes (0% in 2000 and 11% in 2010). The majority of the HMV population treated between 2000 between 2008 was ventilated by tracheostomy; however, since 2007, the percentage of subjects on noninvasive ventilation significantly increased and was equal to the number of tracheostomized subjects. HMV was initiated mainly in ICUs. However, their role systematically diminished, and an increasing number of subjects were recruited in respiratory departments. CONCLUSIONS: The prescription pattern of HMV in Poland has evolved, and there is a clear shift from neuromuscular to respiratory diseases. The prevalence of ventilation via tracheostomy still remains very high in comparison with other European countries. The Polish experience could be useful for countries with emerging HMV care systems.


Assuntos
Serviços de Assistência Domiciliar/estatística & dados numéricos , Pneumopatias/epidemiologia , Doenças Neuromusculares/epidemiologia , Respiração Artificial/estatística & dados numéricos , Insuficiência Respiratória/epidemiologia , Adolescente , Adulto , Criança , Humanos , Pneumopatias/complicações , Doenças Neuromusculares/complicações , Polônia/epidemiologia , Prevalência , Respiração Artificial/métodos , Insuficiência Respiratória/etiologia , Estudos Retrospectivos , Traqueostomia/estatística & dados numéricos
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