RESUMEN
Hyperplasia of the pharyngeal tonsils is to be considered pathologic when nasopharyngeal symptoms of mechanical obstruction and/or chronic inflammation occur. Chronic Eustachian tube dysfunction can result in various middle ear diseases such as conductive hearing loss, cholesteatoma, and recurrent acute otitis media. During examination, attention should be paid to the presence of adenoid facies (long face syndrome), with a permanently open mouth and visible tip of the tongue. In the case of severe symptoms and/or failure of conservative treatment, adenoidectomy is usually performed on an outpatient basis. Conventional curettage remains the established standard treatment in Germany. Histologic evaluation is indicated for clinical evidence of mucopolysaccharidoses. Due to the risk of hemorrhage, the preoperative bleeding questionnaire, which is obligatory before every pediatric surgery, is referred to. Recurrence of adenoids is possible despite correct adenoidectomy. Before discharge home, otorhinolaryngologic inspection of the nasopharynx for secondary bleeding should be performed and anesthesiologic clearance obtained.
Asunto(s)
Tonsila Faríngea , Otitis Media con Derrame , Otitis Media , Niño , Humanos , Tonsila Faríngea/patología , Adenoidectomía , Inflamación , BocaRESUMEN
Hyperplasia of the pharyngeal tonsils is to be considered pathologic when nasopharyngeal symptoms of mechanical obstruction and/or chronic inflammation occur. Chronic Eustachian tube dysfunction can result in various middle ear diseases such as conductive hearing loss, cholesteatoma, and recurrent acute otitis media. During examination, attention should be paid to the presence of adenoid facies (long face syndrome), with a permanently open mouth and visible tip of the tongue. In the case of severe symptoms and/or failure of conservative treatment, adenoidectomy is usually performed on an outpatient basis. Conventional curettage remains the established standard treatment in Germany. Histologic evaluation is indicated for clinical evidence of mucopolysaccharidoses. Due to the risk of hemorrhage, the preoperative bleeding questionnaire, which is obligatory before every pediatric surgery, is referred to. Recurrence of adenoids is possible despite correct adenoidectomy. Before discharge home, otorhinolaryngologic inspection of the nasopharynx for secondary bleeding should be performed and anesthesiologic clearance obtained.
Asunto(s)
Tonsila Faríngea , Otitis Media con Derrame , Otitis Media , Niño , Humanos , Tonsila Faríngea/cirugía , Tonsila Faríngea/patología , Adenoidectomía , Inflamación , Hipertrofia/patología , Hipertrofia/cirugíaRESUMEN
The German Society of Pneumology initiated 2021 the AWMF S1 guideline Long COVID/Post-COVID. In a broad interdisciplinary approach, this S1 guideline was designed based on the current state of knowledge.The clinical recommendations describe current Long COVID/Post-COVID symptoms, diagnostic approaches, and therapies.In addition to the general and consensus introduction, a subject-specific approach was taken to summarize the current state of knowledge.The guideline has an explicit practical claim and will be developed and adapted by the author team based on the current increase in knowledge.
Asunto(s)
COVID-19 , Síndrome Post Agudo de COVID-19 , HumanosRESUMEN
The present guideline aims to improve the evidence-based management of children and adolescents with pediatric community-acquired pneumonia (pCAP). Despite a prevalence of approx. 300 cases per 100â000 children per year in Central Europe, mortality is very low. Prevention includes infection control measures and comprehensive immunization. The diagnosis can and should be established clinically by history, physical examination and pulse oximetry, with fever and tachypnea as cardinal features. Additional signs or symptoms such as severely compromised general condition, poor feeding, dehydration, altered consciousness or seizures discriminate subjects with severe pCAP from those with non-severe pCAP. Within an age-dependent spectrum of infectious agents, bacterial etiology cannot be reliably differentiated from viral or mixed infections by currently available biomarkers. Most children and adolescents with non-severe pCAP and oxygen saturation >â92â% can be managed as outpatients without laboratory/microbiology workup or imaging. Anti-infective agents are not generally indicated and can be safely withheld especially in children of young age, with wheeze or other indices suggesting a viral origin. For calculated antibiotic therapy, aminopenicillins are the preferred drug class with comparable efficacy of oral (amoxicillin) and intravenous administration (ampicillin). Follow-up evaluation after 48â-â72 hours is mandatory for the assessment of clinical course, treatment success and potential complications such as parapneumonic pleural effusion or empyema, which may necessitate alternative or add-on therapy.
Asunto(s)
Antibacterianos/uso terapéutico , Infecciones Comunitarias Adquiridas/tratamiento farmacológico , Neumonía/tratamiento farmacológico , Guías de Práctica Clínica como Asunto , Neumología/normas , Adolescente , Antibacterianos/administración & dosificación , Niño , Infecciones Comunitarias Adquiridas/diagnóstico , Infecciones Comunitarias Adquiridas/virología , Europa (Continente) , Alemania , Humanos , Lactante , Neumonía/diagnóstico , Neumonía/virología , Sociedades MédicasRESUMEN
Viral meningitis is mainly caused by non-polio enteroviruses (NPEV). Large-scale data on the clinical characteristics between different outbreaks within the same region are lacking. This study aimed to analyse a possible influence of the circulating NPEV genotype on the disease outcome of affected children. A retrospective cohort study analysing two major outbreaks of NPEV meningitis in Germany in 2008 and 2013 was conducted in cooperation with the National Reference Centre for Poliomyelitis and Enteroviruses (NRC PE) and five German children's hospitals. A total of 196 patients with laboratory-confirmed NPEV meningitis were enrolled. In 2008, children with NPEV meningitis had significantly higher fever and showed more behavioural changes and less back pain. To better define typical findings in echovirus 30 (E-30) meningitis, patients were split into the following three groups: E-30 positive patients, patients with "Non E-30" infection and patients with "Untyped" NPEV infection. E-30 positive patients were significantly older and their disease course was more acute, with early admission to but also early discharge from hospital. E-30 positive patients showed a significantly higher rate of headache and meningism, and a lower rate of diarrhoea and clinically defined septicaemia when compared to the others. Regarding laboratory testing, E-30 positive patients presented with significantly elevated peripheral blood neutrophil counts when compared to patients with "Non E-30" or "Untyped" NPEV infection. In conclusion, E-30 meningitis in children shows a characteristic pattern of clinical features. To further characterise NPEV strains worldwide, continuous surveillance and typing of NPEV strains causing central nervous system disease is warranted.
Asunto(s)
Brotes de Enfermedades , Enterovirus Humano B , Enterovirus , Meningitis Viral/epidemiología , Meningitis Viral/virología , Niño , Preescolar , Enterovirus/clasificación , Enterovirus Humano B/clasificación , Femenino , Alemania/epidemiología , Historia del Siglo XXI , Humanos , Masculino , Meningitis Viral/diagnóstico , Meningitis Viral/historia , Admisión del Paciente/estadística & datos numéricos , Estudios Retrospectivos , Serogrupo , Evaluación de SíntomasRESUMEN
Respiratory Syncytial Virus (RSV), Human metapneumovirus (HMPV), and Rhinoviruses (RV) are frequent causes of respiratory tract infections in young children. We compared laboratory and clinical findings in children with comparable age distribution and hospitalized due to RSV, HMPV or RV infections. Viral pathogens were detected by a quantitative real time PCR from nasopharyngeal aspirates. No significant differences in the admission diagnosis, laboratory parameters, patient demographics and treatment measures between the three viral causes of respiratory illness were found. No correlation between viral load and disease severity was observed however, there was a significantly lower concentration of the nasopharyngeal interleukin 8 (IL-8) in children with RV compared to HMPV and RSV, indicating a milder proinflammatory reaction. Moreover, RV-infected children had significantly lower body temperature, higher leucocyte counts in peripheral blood, and a tendency to have a shorter stay in hospital than children with either HMPV or RSV infection. Taken together, clinical presentation of the infections with RSV, HMPV, and RV is similar among children of the same age group and not clearly distinguishable by standard clinical or laboratory findings. Therefore, virus specific testing should be included regularly for routine diagnosis of children with respiratory tract infections.
Asunto(s)
Infecciones por Paramyxoviridae/patología , Infecciones por Picornaviridae/patología , Infecciones por Virus Sincitial Respiratorio/patología , Infecciones del Sistema Respiratorio/patología , Adolescente , Niño , Preescolar , Femenino , Hospitales , Humanos , Lactante , Recién Nacido , Masculino , Metapneumovirus/genética , Metapneumovirus/aislamiento & purificación , Nasofaringe/virología , Infecciones por Paramyxoviridae/virología , Infecciones por Picornaviridae/virología , Reacción en Cadena en Tiempo Real de la Polimerasa , Infecciones por Virus Sincitial Respiratorio/virología , Virus Sincitial Respiratorio Humano/genética , Virus Sincitial Respiratorio Humano/aislamiento & purificación , Infecciones del Sistema Respiratorio/virología , Rhinovirus/genética , Rhinovirus/aislamiento & purificaciónRESUMEN
The prevalence of influenza A and B virus-specific IgG was determined in sera taken between 2008 and 2010 from 1,665 children aged 0-17 years and 400 blood donors in Germany. ELISA on the basis of whole virus antigens was applied. Nearly all children aged nine years and older had antibodies against influenza A. In contrast, 40% of children aged 0-4 years did not have any influenza A virus-specific IgG antibodies. Eightysix percent of 0-6 year-olds, 47% of 7-12 year-olds and 20% of 13-17 year-olds were serologically naïve to influenza B viruses. By the age of 18 years, influenza B seroprevalence reached approximately 90%. There were obvious regional differences in the seroprevalence of influenza B in Germany. In conclusion, seroprevalences of influenza A and influenza B increase gradually during childhood. The majority of children older than eight years have basal immunity to influenza A, while comparable immunity against influenza B is only acquired at the age of 18 years. Children aged 0-6 years, showing an overall seroprevalence of 67% for influenza A and of 14% for influenza B, are especially at risk for primary infections during influenza B seasons.
Asunto(s)
Anticuerpos Antivirales/sangre , Virus de la Influenza A/inmunología , Virus de la Influenza B/inmunología , Gripe Humana/epidemiología , Adolescente , Niño , Preescolar , Femenino , Alemania/epidemiología , Humanos , Inmunoglobulina G/sangre , Lactante , Gripe Humana/sangre , Gripe Humana/inmunología , Masculino , Prevalencia , Estudios Seroepidemiológicos , Adulto JovenRESUMEN
Since hepatitis A virus (HAV) infection during childhood is mostly asymptomatic, only seroprevalence studies can provide reliable information on incidence of HAV infection in children. The prevalence of anti-HAV antibodies was determined in sera taken in 2008 to 2010 from 1,645 children aged 0-17 years and in sera taken in 2010-2011 from 400 adult blood donors in Germany. For examination of trend over time, 715 sera collected between 1999 and 2006 from children at the age of 0-17 years within the federal state Thuringia were included. Antibody testing was carried out using the test kits ETI-AB-HAVK PLUS and ETI-HA-IGMK PLUS from DiaSorin. In children, the overall prevalence of antibodies was 10.8 %. After the seroprevalence declined from 8.8 % among the 0-2 year-olds to 2.4 % among the 3-4 year-olds, there was a significant increase to 20.5 % in the group of the 15-17 year-olds. Boys had with 12.7 % a significantly higher seroprevalence of anti-HAV antibodies compared to 8.8 % among girls. In adult blood donors, there was a HAV seroprevalence of 19.3 %. The likelihood of past infection or immunization within the age groups of children from 0 to 12 years differed significantly from that of adults. In conclusion, in Germany, only a small number of HAV infections occur in children, especially up to the age of 12 years. The proportion of susceptible children is greater than the proportion of susceptible adults. Thus, during outbreaks, the rate of infection among children would usually be higher than the rate among adults.
Asunto(s)
Anticuerpos de Hepatitis A/sangre , Hepatitis A/epidemiología , Adolescente , Adulto , Factores de Edad , Anciano , Niño , Preescolar , Femenino , Alemania/epidemiología , Humanos , Lactante , Masculino , Persona de Mediana Edad , Estudios Seroepidemiológicos , Factores Sexuales , Adulto JovenRESUMEN
BACKGROUND: Pregnancies in women with severe relapsing-remitting multiple sclerosis treated with natalizumab constitute a major challenge, because withdrawal of the drug may cause relapses but continuation might have unknown effects on the infantile immune system. AIMS OF THE STUDY: To identify the impact of maternal natalizumab treatment during pregnancy on basic immune functions of the newborn. METHODS: Basic immunological testing and assessment of the chemotaxis rate of freshly isolated T lymphocytes in the presence and absence of CXCL12 was performed in two neonates, whose mothers were treated with natalizumab until the 34th week of pregnancy (pw). RESULTS: Both children had an uneventful birth. However, a reduction in the CXCL12-induced T-cell chemotaxis was found in both children. In contrast, the chemotaxis rate of unstimulated T lymphocytes was not altered. The distribution of the lymphocyte subpopulations was investigated only in case 1 and was normal. CONCLUSIONS: Here, we present to our knowledge the first assessment of T lymphocytes chemotaxis rate in two natalizumab-exposed newborns. A significant reduction in the CXCL12-induced chemotaxis rate of T lymphocytes has been observed and may compromise host defence function in early life. More clinical and immunological data on natalizumab-exposed neonates are warranted.
Asunto(s)
Anticuerpos Monoclonales Humanizados/efectos adversos , Sistema Inmunológico/efectos de los fármacos , Integrina alfa4/inmunología , Efectos Tardíos de la Exposición Prenatal/inmunología , Estudios de Casos y Controles , Femenino , Citometría de Flujo , Humanos , Recién Nacido , Esclerosis Múltiple Recurrente-Remitente/tratamiento farmacológico , Natalizumab , Embarazo , Efectos Tardíos de la Exposición Prenatal/etiología , Linfocitos T/efectos de los fármacosRESUMEN
A prospective clinical study was performed to correlate nasopharyngeal carriage of bacteria with the type of lower respiratory tract infections (LRTI) in hospitalised children. To determine bacterial load in nasopharyngeal aspirates (NPA) we used semiquantitative culturing and quantitative TaqMan-PCR for those pathogens difficult to culture. Specimens and clinical data were obtained from 311 children between 0 and 16 years of age with LRTI during the period of 2006-2008. The most common detected potentially pathogenic colonisers were Haemophilus influenzae (32.1 %), Moraxella catharralis (26.7 %), Staphylococcus aureus (17.7 %) and Streptococcus pneumoniae (16.7 %). As expected S. aureus was the most common coloniser in children less than 4 months of age, whereas H. influenzae detection peaked in older children. Co-colonisation with other bacterial pathogens were more often observed in children with S. aureus (46 %) and S. pneumoniae (49 %) than in those with H. influenzae (30 %) or M. catharralis (27 %). Children with S. aureus co-colonisation had higher levels of C-reactive-protein, received antibiotics more frequently and stayed longer in hospital than those with S. aureus single colonisation. In contrast, children with H. influenzae, M. catharralis or S. pneumoniae colonisation suffered more often from pneumonia than children with S. aureus colonisation. Coloniser specific analysis of bacterial quantity revealed no significant reduction of the bacterial carriage from the first to the second NPA. No correlation of a high bacterial load and occurrence of pneumonia could be detected. In conclusion, clinical characteristics in children with LRTIs are associated with a specific bacterial set of colonisers detected in the nasopharynx rather than on their quantity.
Asunto(s)
Bacterias/clasificación , Bacterias/aislamiento & purificación , Infecciones Bacterianas/patología , Portador Sano/microbiología , Nasofaringe/microbiología , Infecciones del Sistema Respiratorio/patología , Adolescente , Infecciones Bacterianas/microbiología , Carga Bacteriana/métodos , Niño , Preescolar , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Reacción en Cadena de la Polimerasa/métodos , Estudios Prospectivos , Infecciones del Sistema Respiratorio/microbiologíaRESUMEN
BACKGROUND: Cat-scratch disease (CSD) is common in children, however the wide spectrum of the clinical presentation of CSD may lead to delayed diagnosis. An atypical presentation of CSD includes in its differential diagnosis diseases such as tuberculosis, other mycobacterioses, Epstein-Barr-Virus infection (EBV) or malignant disease. Since, in a small number of cases, these diseases may be present concurrently with an active CSD, it is important to consider CSD early in the differential diagnosis and order the appropriate tests. These tests include serology and, where possible, histology including molecular diagnostic methods on tissue specimens. PATIENTS AND METHOD: We performed a case series of five patients treated in our hospital with a clinical diagnosis of cat-scratch disease, confirmed by serology. An analysis of the history and clinical symptoms associated specifically with an atypical presentation of CSD was performed. RESULTS: The clinical presentation of CSD no longer encompasses the original typical description from 1950, but rather presents with a wide spectrum of signs and symptoms, including the absence of a documented cat scratch, fever, primary lesions or peripheral lymphadenopathy. Low density lesions in spleen, liver and lymph nodes are typical findings in ultrasound, MRI, or CT. Ignoring CSD as a possibility in investigating possible malignancy or tuberculosis could lead to unnecessary hospitalisation and delay in the proper treatment. CONCLUSION: CSD should also be considered in differential diagnosis of any patient with intraabdominal lymphadenopathy, abdominal pain and fever of unknown origin. A careful history is important, however, often patients with CSD have no history of contact with cats. Therefore in atypical cases of CSD the finding of other clinical symptoms and performance of specific diagnostic tests is important. Our experience suggests that early serological testing for Bartonella henselae should be performed and may avoid invasive diagnostic procedures.
Asunto(s)
Bartonella henselae , Enfermedad por Rasguño de Gato/diagnóstico , Adolescente , Animales , Antibacterianos/uso terapéutico , Biopsia , Mordeduras y Picaduras/complicaciones , Sedimentación Sanguínea , Proteína C-Reactiva/metabolismo , Enfermedad por Rasguño de Gato/tratamiento farmacológico , Gatos , Niño , Preescolar , Diagnóstico Diferencial , Quimioterapia Combinada , Femenino , Humanos , Laparoscopía , Hígado/patología , Ganglios Linfáticos/patología , Imagen por Resonancia Magnética , Masculino , Bazo/patología , Tomografía Computarizada por Rayos X , UltrasonografíaRESUMEN
Although chronic courses of norovirus infection have been described in immunocompromised patients, little is known about noroviral shedding and correlation with clinical symptoms in these patients. In this report, the quantitative courses of norovirus excretion in nine pediatric patients with hematologic and oncologic disorders and prolonged gastroenteritis were investigated. In a retrospective study multiple fecal samples from nine pediatric cancer patients were examined by a one-step real-time PCR. Clinical data of the patients were reviewed and virological data were correlated with clinical symptoms. All nine patients presented with prolonged illness and prolonged noroviral shedding. Vomiting and diarrhea were associated with high norovirus concentrations and norovirus excretion declined slowly in the patients. Retrospectively, initial PCR-testing for norovirus was performed with a median of 7 days after onset of symptoms. This finding hints at the difficulty of obtaining early diagnosis of the infection in these children. The patients were shedding high norovirus concentration over a long period of time. Results of sequential quantitative PCR-testing for norovirus correlated with clinical symptoms. Both clinical symptoms and quantitative PCR-testings help to define the severity of norovirus infection and to estimate the risk for transmission. To prevent the spread of the disease, usage of virocidal disinfectants and isolation procedures should be maintained as long as patients are positive for noroviruses. Since vomiting is frequent in pediatric patients with oncological conditions, a screening program for rapid detection of norovirus infection in this group of patients should be considered.
Asunto(s)
Gastroenteritis/fisiopatología , Gastroenteritis/virología , Neoplasias/complicaciones , Norovirus/aislamiento & purificación , Esparcimiento de Virus , Adolescente , Infecciones por Caliciviridae/fisiopatología , Infecciones por Caliciviridae/transmisión , Infecciones por Caliciviridae/virología , Preescolar , Heces/virología , Femenino , Gastroenteritis/epidemiología , Genotipo , Humanos , Huésped Inmunocomprometido , Lactante , Masculino , Norovirus/clasificación , Norovirus/genética , ARN Viral/análisis , ARN Viral/aislamiento & purificación , Índice de Severidad de la Enfermedad , Factores de TiempoAsunto(s)
Enfisema Mediastínico/diagnóstico , Infecciones por Virus Sincitial Respiratorio/diagnóstico , Enfisema Subcutáneo/diagnóstico , Administración por Inhalación , Albuterol/administración & dosificación , Terapia Combinada , Diagnóstico Diferencial , Femenino , Humanos , Lactante , Enfisema Mediastínico/terapia , Terapia por Inhalación de Oxígeno , Admisión del Paciente , Infecciones por Virus Sincitial Respiratorio/terapia , Enfisema Subcutáneo/terapiaAsunto(s)
Infecciones por Bacteroides/etiología , Bacteroides fragilis , Enterococcus faecalis , Absceso Epidural/etiología , Infecciones por Bacterias Grampositivas/etiología , Infecciones por Proteus/etiología , Proteus mirabilis , Espina Bífida Oculta/complicaciones , Antibacterianos/uso terapéutico , Ano Imperforado/complicaciones , Ano Imperforado/cirugía , Infecciones por Bacteroides/diagnóstico , Infecciones por Bacteroides/cirugía , Preescolar , Terapia Combinada , Descompresión Quirúrgica , Quimioterapia Combinada , Absceso Epidural/diagnóstico , Absceso Epidural/cirugía , Infecciones por Bacterias Grampositivas/diagnóstico , Infecciones por Bacterias Grampositivas/cirugía , Humanos , Procesamiento de Imagen Asistido por Computador , Imagen por Resonancia Magnética , Masculino , Complicaciones Posoperatorias/diagnóstico , Complicaciones Posoperatorias/etiología , Complicaciones Posoperatorias/cirugía , Infecciones por Proteus/diagnóstico , Infecciones por Proteus/cirugía , Espina Bífida Oculta/diagnóstico , Espina Bífida Oculta/cirugía , Compresión de la Médula Espinal/diagnóstico , Compresión de la Médula Espinal/etiología , Compresión de la Médula Espinal/cirugíaRESUMEN
Mother-to-child transmission of HIV1 is the main cause for pediatric HIV1-infection. Since 1995 in developed countries the rate of vertical HIV1 transmission was reduced from 40% to 1-2% by the combination of antiretroviral therapy of pregnant women, antiretroviral prophylaxis in the newborn and refraining from breast-feeding. Nowadays the main causes for HIV1-infection in children are 1) not offered (voluntary) HIV1-testing in early pregnancy (in spite of recommendation for prenatal care) and 2) missing knowledge about prophylactic interventions in HIV1-positive pregnant women and their HIV1-exposed newborn. Diagnosis and/or exclusion of HIV1-infection in HIV1-exposed and HIV1-positive infants is difficult, because maternal HIV1-antibodies pass the placenta and can persist in the child up to two years after birth. Since in 1996 the era of "highly active antiretroviral therapy (HAART)", the use of an antiretroviral three-drug-regimen, began, HIV1-infection in children changed from a fatal illness to a chronic disease with decreased mortality and improved qualitiy of life. The lack of drug approvement, absence of adequate drug formulation and of pharmacokinetic data for children make the treatment of HIV1-infection in children much more difficult than in adults. Treatment of children depends on clinical category, CD4 cell count, viral load and age of diagnosis. With the current state of knowledge (failure of treatment interruption studies in adults and pending ones in children) once HAART is started it must be carried on life-long. This implies great challenges in adherence to avoid development of resistance and in confrontation with long-term adverse effects of HIV1-therapy.
Asunto(s)
Terapia Antirretroviral Altamente Activa/métodos , Infecciones por VIH/prevención & control , VIH-1 , Transmisión Vertical de Enfermedad Infecciosa/prevención & control , Complicaciones Infecciosas del Embarazo/virología , Atención Prenatal , Síndrome de Inmunodeficiencia Adquirida/prevención & control , Recuento de Linfocito CD4/métodos , Niño , Ensayos Clínicos como Asunto , Consejo , Parto Obstétrico/métodos , Femenino , Infecciones por VIH/tratamiento farmacológico , Infecciones por VIH/inmunología , Infecciones por VIH/transmisión , Infecciones por VIH/virología , VIH-1/efectos de los fármacos , Humanos , Recién Nacido , Cooperación del Paciente , Embarazo , Calidad de Vida , Factores de Riesgo , Resultado del Tratamiento , Carga ViralRESUMEN
Chlamydophila pneumoniae is mainly responsible for respiratory tract infections but has also been associated with endocarditis and myocarditis. We report a case of pneumonia in a child with hemorrhagic pericardial effusion with a positive result by a new C. pneumoniae TaqMan PCR, suggesting a pericardial inflammation directly induced by C. pneumoniae. C. pneumoniae should be suspected in patients with community-acquired pneumonia and concurrent pericarditis. Empirical treatment with azithromycin seems feasible.
Asunto(s)
Infecciones por Chlamydophila/complicaciones , Chlamydophila pneumoniae/aislamiento & purificación , Pericarditis/etiología , Pericarditis/microbiología , Neumonía Bacteriana/complicaciones , Enfermedad Aguda , Adolescente , Infecciones por Chlamydophila/microbiología , Chlamydophila pneumoniae/clasificación , Chlamydophila pneumoniae/genética , Femenino , Hemorragia , Humanos , Derrame Pericárdico/microbiología , Neumonía Bacteriana/microbiologíaRESUMEN
Pericardial effusion and cardiomyopathy in the first year of life point to a fatty acid b-oxidation defect as underlying disease after exclusion of infectious causes. We report two patients with the early-onset, cardiac phenotype of very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency; in one patient, severe pericardial effusion was the predominating symptom. Because specific noninvasive treatment is available in fatty acid b-oxidation defects that reverses cardiomyopathy and pericardial effusion, early diagnosis is essential to adjust therapy accordingly.
Asunto(s)
Acil-CoA Deshidrogenasa de Cadena Larga/deficiencia , Acil-CoA Deshidrogenasa de Cadena Larga/metabolismo , Cardiomiopatías/etiología , Errores Innatos del Metabolismo Lipídico/complicaciones , Errores Innatos del Metabolismo Lipídico/dietoterapia , Derrame Pericárdico/etiología , Cardiomiopatías/diagnóstico por imagen , Cardiomiopatías/dietoterapia , Ecocardiografía , Ácidos Grasos/metabolismo , Humanos , Lactante , Errores Innatos del Metabolismo Lipídico/diagnóstico , Errores Innatos del Metabolismo Lipídico/enzimología , Derrame Pericárdico/diagnóstico por imagen , Derrame Pericárdico/dietoterapia , Triglicéridos/uso terapéuticoRESUMEN
OBJECTIVES: Aim of this study was to analyze the feasibility of oncological treatment in pediatric patients belonging to Jehovah's Witnesses and to describe the changing policy in performing transfusions and supportive care measures at two German pediatric cancer institutions. PATIENTS AND METHODS: Over a period of 16 years 21 treatments according to the current cooperative protocols were performed in 14 children of Jehovah's Witnesses. Various hematological supportive care measures such as supplementation with iron, human erythropoietin, interleukin 11, granulocyte colony-stimulating factor and autologous or allogeneic stem cell rescue had been applied. For comparison matched pairs treated in our hospitals not belonging to Jehovah's Witnesses and 50 pediatric and adult oncological patients belonging to Jehovah's Witnesses reviewed from the international literature were analyzed with respect to transfusions and outcome. RESULTS: So far, 9 of 14 children are surviving 16-195 months (median 26 months). During the primary therapy they received markedly less transfusions than the control cohort (-39,1% red blood cell transfusions and -37,5% platelet transfusions). The review of 50 reported cases showed that oncological therapy can also be successfully performed with a restricted transfusion regimen in children and particularly in adults. CONCLUSION: Pediatric cancer patients belonging to Jehovah's Witnesses can be treated similarly to other patients. A restrictive transfusion policy and the broad application of hematopoietic supportive care measures may reduce transfusions. This treatment policy and a continuous collaboration with the Hospital Liaison Committee for Jehovah's Witnesses appears to create an oncological treatment situation with a high compliance of patients and parents where court orders may not be necessary.