BACKGROUND: Congenital heart defects (CHD) being the most common congenital malformation, significantly impact mortality and morbidity in children and adults. Early detection greatly improves treatment and prognosis. Rou...BACKGROUND: Congenital heart defects (CHD) being the most common congenital malformation, significantly impact mortality and morbidity in children and adults. Early detection greatly improves treatment and prognosis. Routine pulse oximetry screening and fetal echocardiography in Germany have advanced early CHD diagnosis. However, a diagnostic gap persists, leaving some CHD cases undetected, worsening prognosis. This study aimed to evaluate echocardiographic screening in newborns at a German university hospital as well as to record numerous potential risk factors in a database. PATIENTS AND METHODS: This monocentric, prospective cohort study included ostensibly healthy newborns delivered between 03/2015 and 04/2016. Echocardiography was performed at an average age of 3.15 days, with follow-up by a specialist within six weeks and by telephone interview in 2019. RESULTS: Of 1006 newborns, 518 (51.49%) underwent echocardiography. CHD were diagnosed in 7.14% (37/518) with the following frequencies: atrial septal defect (ASD) (45.95%), ventricular septal defect (VSD) (35.14%), persistent ductus arteriosus (PDA) (8.11%), coarctation of aorta (CoA) with ASD (2.70%), quadricuspid aortic valve with VSD (2.70%), VSD with PDA (2.70%), and significant tricuspid regurgitation (2.70%). Follow-up in 2019 showed resolution of 31 CHD, while four patients continued regular cardiologist visits, with one requiring urgent surgery for severe CoA. CONCLUSION: This study demonstrates that echocardiographic screening in newborns can detect previously undiagnosed CHD, including critical cases.
OBJECTIVES: Urinary tract infections (UTI) are the most common occult infections in infants under three months of age with high fever, caused by various pathogens, and can progress with different symptoms from mild to se...OBJECTIVES: Urinary tract infections (UTI) are the most common occult infections in infants under three months of age with high fever, caused by various pathogens, and can progress with different symptoms from mild to severe. We planned to explore and compare pathogen-specific systemic inflammatory indices, urinalysis, and radiological findings in newborns. METHODS: The retrospective study included preterm and term neonates with single pathogen growth of≥50.000 colony-forming units (CFU) /mL from urine cultures obtained by first-time catheterization between September 2019 and November 2021. Baseline hemogram parameters, acute phase reactants, systemic inflammatory indexes, urinalysis, and radiological findings were analyzed in pathogen-specific groups. RESULTS: Of the 132 patients included in the study, gastrointestinal symptoms were the most common. C-reactive protein values were higher in the non- E. Coli group (p<0.05). Procalcitonin values were higher in patients with Klebsiella spp. isolates (p<0.05). Systemic inflammatory indexes were higher in patients with Coagulase-Negative Staphylococci isolates (p<0.05). Urinary acidity was higher in the non-E. Coli group (p<0.05). Escherichia Coli and Klebsiella Spp. were isolated in two-thirds of patients with leukocyte esterase positivity. The most common cause of UTI in patients with abnormal renal ultrasound findings was E. Coli (33.3%). CONCLUSION: Our findings imply that unconventional tests could assist in pathogen differentiation, determining clinical presentation, and prognosis.
Duchenne muscular dystrophy is the most common inherited neuromuscular disease in children. In addition to the progressive loss of motor skills and cardiac involvement, respiratory muscle weakness leads to a restrictive...Duchenne muscular dystrophy is the most common inherited neuromuscular disease in children. In addition to the progressive loss of motor skills and cardiac involvement, respiratory muscle weakness leads to a restrictive lung disease and cough insufficiency. Specific respiratory interventions have significantly improved survival and quality of life of the affected boys. The pediatric pulmonologist's tasks include monitoring of the lung function, polysomnography, starting and adjusting of non-invasive or invasive nocturnal ventilation and optimizing of secretion management. Providing of vaccinations, enhancing of nutrition, treatment of intercurrent infections, and provision of advice and training are important tasks for the pediatric pulmonologist. This article emphasizes on the tasks for the pediatric pulmonologist in the treatment of children with neuromuscular diseases i. e. Duchenne muscular dystrophy in interaction with the other specialist disciplines.
Klin Padiatr
· 2024 Nov · PMID 39333049
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Reaching age-appropriate physical activity levels is a significant challenge for many children, adolescents, and young adults (CAYAs) with and following an oncological disease. Many CAYAs require support in addressing ph...Reaching age-appropriate physical activity levels is a significant challenge for many children, adolescents, and young adults (CAYAs) with and following an oncological disease. Many CAYAs require support in addressing physical activity-specific questions and need assistance in accessing sports and exercise offers in their local area. Since many clinics cannot provide such time-intensive counseling, the nationwide operating network ActiveOncoKids (NAOK), funded by the German Cancer Aid, offers individualized support to participate in physical activity (PA). This paper describes the NAOK-Physical-Activity-Counseling (-PAC) concept and evaluates the initial 200 consultations, focusing on recruitment, objectives, and oncology-specific parameters. Ultimately, dimensions of barriers and facilitators and intervention options are discussed.
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...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.
ABSTRACT:BACKGROUND: Renal dysplasia (RD) is a major cause of chronic kidney disease (CKD) in childhood. Herein, we present a single-center experience about distinctive features of different subtypes of dysplasia. ABSTR...ABSTRACT:BACKGROUND: Renal dysplasia (RD) is a major cause of chronic kidney disease (CKD) in childhood. Herein, we present a single-center experience about distinctive features of different subtypes of dysplasia. ABSTRACT:METHODS: All children with RD admitted between January 2018 and June 2019 were included in this cross-sectional study. Patients were classified as having unilateral RD, bilateral RD, posterior urethral valves (PUV), and multicystic-dysplastic kidneys (MCDK). ABSTRACT:RESULTS: A total of 198 patients (108 unilateral RD, 31 bilateral RD, 16 PUV, and 43 MCDK) with a follow-up period of 80 (1.5-240) months were included. The overall consanguinity rate was 22%, whereas it was 56% in bilateral RD patients. Two-thirds had additional urogenital anomalies. Hypertension was detected in 16% of the patients, and 20% had median proteinuria of 16 (5.2-173) mg/m/h, which was significantly higher in the PUV and bilateral RD groups. The overall rate of CKD was 23%, which was more frequent in bilateral RD and PUV groups. The median age at end-stage renal disease was 91.5 (0.5-208) months, including 12 renal transplant patients. ABSTRACT:CONCLUSIONS: As different subtypes of RD have distinct clinical and prognostic features, the follow-up of RD patients should be individualized, and modifiable complications such as proteinuria and HT should be monitored closely.
OBJECTIVE: To evaluate the efficacy of different doses of gammaglobulin combined with aspirin in treating pediatric Kawasaki disease and its impact on C-reactive protein (CRP) and white blood cell (WBC) levels. METHODS:...OBJECTIVE: To evaluate the efficacy of different doses of gammaglobulin combined with aspirin in treating pediatric Kawasaki disease and its impact on C-reactive protein (CRP) and white blood cell (WBC) levels. METHODS: A prospective study was conducted with 150 children (3groups) diagnosed with Kawasaki disease. Group A received a higher dose of intravenous gammaglobulin (2.5 g/kg) combined with aspirin, Group B received a standard dose of intravenous gammaglobulin (2 g/kg) combined with aspirin, and Group C received a lower dose of intravenous gammaglobulin (1 g/kg) combined with aspirin. All groups received oral aspirin therapy (30 mg/kg/day) for a standard duration. The time to symptom improvement, treatment efficacy, laboratory parameters, and adverse reactions were monitored and analyzed. RESULTS: Group B showed a shorter time to symptom relief compared to Groups A and C, particularly in fever reduction, mucosal congestion, cervical lymph node enlargement, and limb symptoms. The total effective rate was higher in Group B compared to Groups A and C. Group B showed improvements in WBC, CRP, ESR, and PLT levels. CONCLUSION: Standard-dose intravenous gammaglobulin combined with aspirin was more effective in treating pediatric Kawasaki disease compared to higher and lower doses. The treatment regimen in Group B resulted in shorter time to symptom relief, higher total effective rate, improved biochemical markers. Incidence of adverse reactions was similar among the three groups, demonstrating the safety of standard dosage.
BACKGROUND: Antenatal hydronephrosis refers to the dilation of the renal pelvis and/or calyces in the developing fetus. The challenge lies in distinguishing between cases that warrant long-term follow-up or surgical inte...BACKGROUND: Antenatal hydronephrosis refers to the dilation of the renal pelvis and/or calyces in the developing fetus. The challenge lies in distinguishing between cases that warrant long-term follow-up or surgical intervention and those with transient hydronephrosis that require minimal invasive investigations. MATERIALS AND METHODS: Our study aimed to assess and contrast the efficacy of the 2015 Congenital Anomalies of Kidney and Urinary Tract Guideline from the Turkish Society of Pediatric Nephrology with the Tepecik Antenatal Hydronephrosis Guideline, which was previously employed in our hospital. We conducted a comparative analysis of demographic data, outcome conditions, additional imaging requirements and quantities, radiation exposures, and rates of surgical interventions between two groups. RESULTS: Group 2 had a significantly higher detection rate of Vesicoureteral Reflux via voiding cystourethrogram at 38.5% compared to Group 1's 13.4% (p<0.01). The incidence of abnormal findings with dimercaptosuccinic acid was similar between Group 1 (28.5%) and Group 2 (26.4%) (p>0.01), but Group 2 had a higher rate of obstruction diagnosis at 68.8% versus Group 1's 29.4% (p<0.01). Group 1 had greater median radiation exposure (500 mrem vs. 200 mrem, p<0.01), and a higher proportion of patients underwent surgery (34.2% vs. 21.9%, p<0.01). CONCLUSION: This study showed that the new guideline required fewer tests, was less invasive, and exposed patients to less radiation than the old guideline.
Seasonal respiratory syncytial virus (RSV) lower respiratory infections (RSV-LRI) and bronchiolitis in children remain a high burden to medical facilities. Studies evaluating the practical approach in outpatient settings...Seasonal respiratory syncytial virus (RSV) lower respiratory infections (RSV-LRI) and bronchiolitis in children remain a high burden to medical facilities. Studies evaluating the practical approach in outpatient settings are scarce. We conducted a survey to provide an insight into management in pediatric offices in Germany. Invitations for participation in an online survey were sent to 623 providers of pediatric primary care via email. Recipients were randomly chosen from national databanks based on zip codes. The response rate was 16,2%, n=92 (14,7%) questionnaires were fully answered and analyzed. 93,5% (n=86) are board certified pediatricians. In outpatient setting, every second patient with suspected or confirmed RSV-LRI is presented three times or more. 40,7% of our participants refer less than 10% of all patients to hospital, 83,7% refer less than one third (<30%). 33,7% of all participants never perform virological testing in suspected cases of RSV-LRI. More than 50% of all participants prescribe bronchodilators, hypertonic saline inhalation is prescribed by 17,4%. Antibiotics are prescribed in less than 10% of all cases of RSV-LRI. About 3/4 ask for a clearer, more conclusive guideline regarding prophylaxis. The diagnostic approach varies significantly. Although not recommended in international guidelines, bronchodilators are still frequently used. Considering emerging options for passive immunization, broader inclusion criteria into immunization regimes are requested by the participants. More conclusive guidelines and recommendations could further improve the management of outpatient RSV-LRI in children.
BACKGROUND: In the period from 2019 to 2022, pediatric hemato-oncological patients were cared for in a pilot project in North Rhine-Westphalia (NRW). The project delivered care at the homes of the patients as opposed to...BACKGROUND: In the period from 2019 to 2022, pediatric hemato-oncological patients were cared for in a pilot project in North Rhine-Westphalia (NRW). The project delivered care at the homes of the patients as opposed to care at the clinics and included both general nursing as well as specific treatments that were delegated from physicians. Patients were admitted to this form of care upon referral by a physician. OBJECTIVES: The project objective was to test the feasibility of such a method of care in the context of pediatric hemato-oncologic disease. METHOD: For the evaluation, a documentation system was designed and applied, which made it possible to evaluate relevant health care data. The evaluation was carried out by means of descriptive statistics and content-analytical categorization. RESULTS: From 11/2019 to 12/2022, a total of N=475 hemato-oncological patients were enrolled in the care project and n=4005 home visits were performed in 242 different zip code areas in NRW. The majority of activities that could be delegated to physicians consisted of blood sampling (79.2%). Assessment of general condition (96.5%) represented the largest proportion of care services. Complications related to the method of care did not occur. CONCLUSION: The nurse-led outpatient care of pediatric, hemato-oncological patients was shown to be a patient- and team-oriented form of care and seems to be an alternative to purely clinic based care. Presumably, transfer to other pediatric specialties is possible. In the next step, a prospective multicenter randomized study is needed to draw relevant conclusions about the actual possibilities and limitations.