Klinische Padiatrie最新文献

Short bowel syndrome (SBS) results from a severe reduction in intestinal absorptive capacity, rendering it insufficient to meet the body's nutritional requirements. Teduglutide represents a novel therapeutic approach for patients with SBS. This study aims to share our experiences and outcomes of Teduglutide treatment in children with SBS, focusing on the development of their anthropometric parameters.This study included all pediatric SBS patients in the Czech Republic receiving home parenteral nutrition (PN) who began Teduglutide treatment from May 2019 to June 2024.We analyzed the changes in volume and caloric content of PN and anthropometric parameters for each patient.All 10 patients showed a reduction in PN volume and caloric content during Teduglutide treatment with a mean value 67%+/- 29. There was also a notable decrease in the caloric content of PN, although this did not always correlate with the volume reduction, the mean value was 52.3%+/- 40.6. Despite the reduced reliance on PN, there was a mean improvement in weight and height among the patients. Gastrointestinal complications were the most common side effects observed.Our findings confirm the positive impact of Teduglutide on intestinal absorption in pediatric SBS patients. They also validate its safety and efficacy in this population. Despite the reduction in PN, improvements in weight were evident across the entire patient cohort.

Acute paediatric community-acquired pneumonia (pedCAP) represents a significant burden on child health and highlights the need for accurate diagnostic tools. This study aimed to differentiate between acute community-acquired viral and bacterial pedCAP using TRAIL, IP-10, and MxA markers.The study a single-center prospective study was conducted between January 2019 to January 2020. During the follow-up period, 315 patients with paediatric community-acquired pneumonia were followed up. The analysis was performed with 52 patients aged 5 years and younger in whom pathogens were detected. 24 control groups were also included to see the change of markers in intact subjects. The patients' medical history and samples were obtained upon hospital admission or within the first day.The mean values of MxA, TRAIL, and IP-10 differed significantly among all three groups (all p values p<0.01). The ROC analysis indicated that serum MxA, TRAIL, and IP-10 parameters had diagnostic value in predicting the differentiation of viral pedCAP from bacterial pedCAP .The TRAIL, MxA, and IP-10 markers have diagnostic value in distinguishing viral pneumonia and bacterial pneumonia. Among these markers, MxA exhibits the highest sensitivity. High levels of MxA, TRAIL and IP-10 in combination with low C-reactive protein (CRP), procalcitonin (PCT) and neutrophil lymphocyte ratio (NLR) values to differentiate viral from bacterial pedCAP will significantly contribute to the accurate identification of viral LRTI.

Objective: The present report describes the results of four delphi surveys conducted within the quality improvement project "My Logbook" which aims to translate evidence-based standards for psychosocial care in pediatric oncology into a practical consensus-based tool.

Methods: In four consecutive delphi surveys a total of n=153 international, multi-disciplinary experts rated the content, method, and design of the different booklets of "My Logbook" which a local expert group had conceptualized. After each survey, the feedback was incorporated, and the changes were evaluated in a final consensus vote by the quality assurance panel of the PSAPOH.

Results: While some surveys led to a review on a page level, most booklets as a whole reached the consensus-level of approval. Over the course of the surveys, any revisions and comments were incorporated in the booklets, and approval rates increased steadily.

Discussion: The delphi surveys ensured the integration of multi-disciplinary, international expertise, uncovering issues such as language barriers and the need for a user manual that would not have been evident in first line . The incorporation of the input led to a continuous improvement of the tool, reflected in steadily increasing acceptance rates in the consecutive survey rounds.

Conclusion: The incorporation of the expert input as well as the additional development of a user manual resulted in a final version of the "My Logbook" apt for the interdisciplinary application in pediatric oncology in the entire DACH-region.

Background: Enteroviruses (EV) are most common causes of the etiologically known aseptic meningitis in children. EV can be detected with polymerase chain reaction (PCR) in cerebrospinal fluid (CSF) samples. We aimed to evaluate the clinical and laboratory characteristics of children diagnosed with PCR-confirmed EV meningitis in CSF samples.

Patients: Patients aged 1 month to 17 years who underwent lumbar puncture (LP) with suspected meningitis and had CSF viral PCR and culture results between September 2012 and January 2021 at a tertiary care hospital in Turkey were included.

Methods: Patients with no virus detected in CSF samples by PCR was comprised PCR-negative group. The EV PCR-positive patients were divided into two groups based on CSF pleocytosis as enteroviral meningitis (EVM) with CSF pleocytosis and EVM without CSF pleocytosis, and compared in terms of clinical and laboratory features.

Results: 78 (38.2%) were EV PCR-positive, and 126 (61.8%) were PCR-negative. Pleocytosis was detected in 55 (70.5%) EV PCR-positive patients and 94 (74.6%) of PCR-negative patients. Convulsion was significantly frequent (p=0.017) in EV PCR-positive patients with no pleocytosis. Protein and lactate concentrations in CSF were significantly higher in EV PCR-positive patients with pleocytosis (p=0.048, p=0.001, respectively). Median hospital stay was significantly longer in PCR-negative group (p<0.001).

Conclusion: Diagnosing EVM with PCR prevents long-term hospitalization, unnecessary antibiotic use and healthcare-related complications.

Lysosomal storage disorders (LSD) are a group of inherited inborn metabolism errors that are characterized by a deficiency in the lysosomal enzyme. In patients with suspected lipid storage disorders, confirmation of the diagnosis relies predominantly on the measurement of specific enzymatic activities and molecular genetic studies. New approaches to the measurement of lysosphingolipids have been developed that may serve as a rapid first-tier screening tests for the evaluation of lysosomal storage disorders. The present study evaluates the results of lysosphingolipid screening tests in patients with suspected lysosomal storage diseases. Lysosphingolipid elevation was detected in five patients examined with suspected lysosomal storage disease, and a definitive diagnosis was reached based on genetic analysis. Our data support recent evidence of the primary role of LysoSLs in the diagnosis of sphingolipidosis, and suggest that these biomarkers may be used for diagnosis and treatment monitoring in the future.

Obesity, linked to various comorbidities like metabolic syndrome (MS) due to its proinflammatory nature, contributes to elevated exhaled nitric oxide levels, impacting multiple physiological systems, including the respiratory system. This study aimed to examine the impact of obesity and MS on lung function and airway inflammation in adolescents. We investigated the relationship between body fat distribution and pulmonary function parameters, and assess fractional exhaled nitric oxide (FeNO) levels as an indicator of airway inflammation.This study included 100 children aged 10 to 18 years, comprising 50 obese individuals and 50 non-obese healthy controls. Participants were categorized based on their relative body mass index (RBMI), presence of MS, and body fat distribution. Pulmonary function tests and FeNO levels were compared between the groups.The FEV1/FVC and FRC values were significantly lower in the obese group. Among obese participants, MS was present in 30%, and those with MS had higher ERV. In male subjects, FEV1/FVC was lower and FeNO levels were higher in those with increased waist circumference. There was a positive correlation between RBMI and FVC, TLC, and a negative correlation between RBMI and FEV1/FVC, FRC. Negative correlations were also detected between waist and neck circumferences and FEV1/FVC, FRC, RV values.Obesity and MS negatively impact pulmonary function, particularly reducing FEV1/FVC and FRC. Furthermore, central adiposity may contribute to airway inflammation, as indicated by increased FeNO levels.

Background: This study analyzes the RSV season 2021/2022 in a referral children's hospital, compares the epidemiology and illness severity with RSV-infected inpatients from 2016 to 2020 and audits the adherence to our internal therapy standard for RSV bronchiolitis.

Material and methods: Inpatients with rtPCR-confirmed RSV infection (Jan. 2016 to Jan. 2022).

Results: The audit comprises 306 RSV inpatients, on average 50 hospitalizations per year; in 03/2020, a rapid RSV Season-Offset was observed. In the winter season 2020/2021, no patient with RSV was hospitalized. Beginning in July, we noticed a rapid increase of RSV-admissions (most cases in Sept./Oct, duration until Dec. 2021; n=53). In 2021-2022, a significant larger share needed PICU admission (9.4% vs 3.2%, p=0.040). Adherence to the internal guidance was low; only 11.8% (n=36) of all patients received supportive treatment without inhalative or systemic medications, 37% of all patients received antibiotics.

Conclusions: This audit confirms the strong impact of public preventive measures directed against SARS-CoV-2 transmission on RSV epidemiology. Few weeks after easing public COVID-19 restrictions (summer 2021), RSV inpatient cases rapidly increased, lasting until Dec. 2021. The audit of bronchiolitis management revealed surprisingly low adherence to the internal guidance, despite a face-to-face educational session with the attending pediatricians in Oct. 2021. Low adherence resulted in an unnecessary exposure of RSV patients to systemic medications of questionable benefit including antibiotics.

Background: Chronic hepatitis B (CHB) remains an important global public health problem. Seroconversion is highly correlated with favourable long-term outcome. There is no consensus on the treatment method, onset time and duration for paediatric CHB patients. In this study, we aimed to evaluate the course of children with CHB, to determine the characteristics of the patients who developed spontaneous seroconversion and treatment indications, and to compare the effectiveness of different treatment modalities.

Patients and methods: Patients aged 1-18 years with a diagnosis of CHB were included in this study and evaluated for the development of spontaneous hepatitis B envelope antigen (HBeAg)/surface antigen (HBsAg) seroconversion and treatment modalities. The treated patients were divided into two groups according to their treatment regimen.

Results: Of the 114 patients, the median age at diagnosis was 8.4 years and median follow-up period was 5.2 years. Spontaneous HBeAg and HBsAg seroconversion developed in 18 (20.6%) of 87 HBeAg positive patients and two (1.8%) patients, respectively. Thirty-one patients were treated. The follow-up period was higher in the group with HBeAg seroconversion (p:0.005). There was no statistical difference between the patients in terms of seroconversion development and treatment status. The serum alanine aminotransferase (ALT) values at the time of diagnosis were statistically higher in treated patients.

Conclusion: HBV infection and CHB continue to be an important problem for children in our country. Consensus on the appropriate ALT ULN is needed for the treatment of hepatitis in children.