Johann P, Sturm D, Kortmann R
… +14 more, Bison B, Capper D, El Damaty A, Behrens L, Manea S, Gojo J, Frühwald M, Thomale UW, Schuhmann MU, Schwarz R, Tietze A, Wagner M, Timmermann B, von Zezschwitz B
With the advent of multi-omic molecular profiling techniques, central nervous system tumor types previously not recognized by conventional neuropathological assessment have emerged, particularly among tumors formerly ter...With the advent of multi-omic molecular profiling techniques, central nervous system tumor types previously not recognized by conventional neuropathological assessment have emerged, particularly among tumors formerly termed as "CNS- primitive neuroectodermal tumors." Given the diverse histopathological, molecular, radiological, and clinical characteristics of these tumors, diagnostic approaches and treatment strategies need to be adapted to our increasing knowledge. The small number of patients per year for individual tumor types precludes large cohort studies and mandates international cooperation and harmonization. To this end, the SIOPE Brain Tumor Group together with the European Reference Network for Pediatric Cancers has published the European Standards of Clinical Practice guidelines for rare embryonal and sarcomatous tumors.
INTRODUCTION: Rheumatological diseases may cause interstitial lung damage. This study aimed to investigate the association between pediatric body composition parameters and interstitial lung disease in patients with rheu...INTRODUCTION: Rheumatological diseases may cause interstitial lung damage. This study aimed to investigate the association between pediatric body composition parameters and interstitial lung disease in patients with rheumatological conditions. METHODS: In this cross-sectional study, all patients aged 8-18 years with rheumatological diseases and chronic respiratory symptoms were evaluated for interstitial lung disease using spirometry, thorax computed tomography, and carbon monoxide diffusion tests. Nutritional status and body composition were assessed using bioelectrical impedance analysis, body mass index, and fat-free mass index. Patients were categorized into three groups, group I (normal fat-free mass index and body mass index), group II (sarcopenia-low fat-free mass index but normal body mass index), and group III (malnutrition with both low fat-free mass index and body mass index). RESULTS: A total of 26 patients were included, and the median age was 169.03 (94-216) months. The median body mass index was 21.9 (14.4-33.8) kg/m. Sixteen (62%) patients had normal body mass index and normal fat-free mass index (group I), four (15%) patients had normal body mass index and low fat-free mass index (group II), and six (23%) patients had low body mass index and low fat-free mass index (group III). Among the pulmonary function tests, the median (interquartile range) carbon monoxide diffusion tests -score was -2.3 (-4.9 to 0.06). A positive correlation was found between carbon monoxide diffusion tests -scores and fat-free mass index (=0.589, =0.002). Fat-free mass index was significantly lower in the group with carbon monoxide diffusion tests -scores of <-1.64. CONCLUSIONS: Nutritional status may be associated with pulmonary function test results in children with interstitial lung disease-related rheumatological diseases.
INTRODUCTION: Differentiated thyroid carcinoma is the most common endocrine malignancy in children and typically presents as an isolated tumor. The synchronous occurrence of differentiated thyroid carcinoma with a second...INTRODUCTION: Differentiated thyroid carcinoma is the most common endocrine malignancy in children and typically presents as an isolated tumor. The synchronous occurrence of differentiated thyroid carcinoma with a second, non-thyroid primary malignancy is exceptionally rare and poorly characterized in pediatric patients. PATIENTS AND METHODS: We reviewed 571 pediatric patients with histologically confirmed differentiated thyroid carcinoma and enrolled in the national German Society for Pediatric Oncology and Hematology-malignant endocrine tumor registry between January 1997 and February 2025. Synchronous tumors were defined as two distinct malignancies diagnosed within 3 months prior to disease-specific therapy. Clinical, pathological, and genetic findings were analyzed descriptively. RESULTS: Five patients (0.9%) were diagnosed with synchronous differentiated thyroid carcinoma and a second primary malignancy. All thyroid tumors were papillary thyroid carcinomas, identified in the context of Hodgkin lymphoma (=2), pheochromocytoma, synovial sarcoma, and Ewing sarcoma. In four cases, the thyroid tumor was detected during staging or evaluation for the primary malignancy. In one patient, the papillary thyroid carcinoma was diagnosed and treated prior to the identification of Hodgkin lymphoma. One patient harbored a pathogenic succinate dehydrogenase [ubiquinone] iron-sulfur subunit variant, and two patients had coexisting Hashimoto thyroiditis. All patients achieved complete remission. CONCLUSIONS: Synchronous presentation of differentiated thyroid carcinoma and other malignancies in pediatric patients, while rare, poses significant diagnostic and therapeutic challenges. These cases warrant multidisciplinary evaluation and may reflect underlying cancer predisposition, immune dysregulation, or previously unrecognized risk factors.
INTRODUCTION: The recent use of Somatex shunts in the prenatal drainage of megacystis remains poorly documented. Here, we describe a number of complications affecting the cutaneous and muscular tissues that have not been...INTRODUCTION: The recent use of Somatex shunts in the prenatal drainage of megacystis remains poorly documented. Here, we describe a number of complications affecting the cutaneous and muscular tissues that have not been reported to date. METHODS: The records of all male patients born alive after receiving a vesicoamniotic shunt were studied retrospectively. The primary end point was the presence of a cutaneous partially muscular lesion at birth. In addition, we analyzed the impact of shunt placement timing on the occurrence of these injuries. RESULTS: Thirty patients were included. Seven patients received a Harrison fetal bladder stent, and none of them had skin ulceration visible at birth. Among the 23 patients who received a Somatex shunt, we observed ulcerative lesions of the lower limbs in 12 patients, representing 52% of children. In these patients, a single lesion was found, except for one case showing affected tissues on both lower limbs. Seventeen of these Somatex shunts were placed in the first 17 weeks of gestation. Among them, 10 showed ulcerative lesions whereas among the 6 Somatex shunts inserted later in pregnancy, two patients had an ulcerative skin lesion. There was no statistically significant difference in the occurrence of skin lesions between these two groups (10/17 vs. 2/6, = 0.37). CONCLUSIONS: The implantation of Somatex shunts in the prenatal treatment of megacystis is associated with cutaneous and muscular lesions in more than half of the newborns. Our results underline the importance of antenatal advice prior to the implantation of these shunts, regarding also the esthetic consequences of these lesions.
OBJECTIVES: Several metabolic diseases require indicated diets, often including special foods and supplements. The assessed scenario calculations aim to quantify the additional expenses caused by necessary dietary treatm...OBJECTIVES: Several metabolic diseases require indicated diets, often including special foods and supplements. The assessed scenario calculations aim to quantify the additional expenses caused by necessary dietary treatment in children and adolescents in alignment with a healthy diet based on the 'optimized mixed diet'. The optimized mixed diet fulfils the nutrient requirements for all ages (1-18 y) by adjusting the food amounts to their age-dependent energy requirements. METHODS: For the food cost calculation, a sample patient (age group 4<7 y) was used. To determine additional costs, the 7-day model menu of the optimized mixed diet was modified based on specific requirements for each disease. Nutritional adequacy of all menus was assured using nutrition software 'Diät 2020'. Food amounts for all different age groups 1<18 years were determined by using calculation factors. Menu costs were calculated using current food prices collected in two supermarkets, pharmacies and online; lowest available prices were recorded. RESULTS: Fourteen diseases were expected to cause additional costs and included in the calculations. The average additional costs of all diseases amounted for 48.88 €/mo for the reference group. The range for the expected additional costs for the reference group was between approx. 16 €-101 €/mo. CONCLUSIONS: The results of this first assessment of food costs required by certain metabolic diseases shall provide guidance for possible targeted financial support for caregivers of children with special dietary needs in Germany, from which other countries may benefit likewise.
BACKGROUND: Central venous catheter placement is essential for managing critically ill children. This study aimed to evaluate the factors associated with procedural success and complication rates of central venous cathet...BACKGROUND: Central venous catheter placement is essential for managing critically ill children. This study aimed to evaluate the factors associated with procedural success and complication rates of central venous catheterization in a pediatric intensive care unit setting. MATERIALS AND METHODS: A retrospective, single-center observational study was conducted in the pediatric intensive care unit of the University Faculty of Medicine between 2024 and 2025. A total of 153 central venous catheters were placed in critically ill children using the Seldinger technique. Patient characteristics, procedural variables, and complication data were analyzed using non-parametric tests and multivariable methods, where appropriate. RESULTS: The total complication rate was 27.6%, and the first-attempt success rate was 98.7%. The most common complication was arterial puncture (20.4% of patients). Procedural variables, including the number of attempts (=0.004), catheterization time (=0.001), sedation use (=0.046), and catheter duration (<0.001), were associated with survival status in the unadjusted analyses. However, these findings should be interpreted as associations rather than as causal relationships. Patients who survived tended to have fewer insertion attempts and shorter procedure durations; however, mortality outcomes are likely influenced by underlying clinical severity and confounding factors. CONCLUSIONS: Central venous catheterization is a fundamental procedure in the management of critically ill children, with a high success rate and measurable complication profile. Procedural factors were associated with complication rates; however, no causal relationship with mortality was established. The femoral and internal jugular access sites were more frequently used in this cohort; however, no adjusted comparison supports their superiority over subclavian access. The relatively low number of subclavian catheterizations limits meaningful comparisons between insertion sites; therefore, further prospective multicenter studies are required to define optimal procedural strategies.
BACKGROUND: Chronic recurrent multifocal osteomyelitis is a rare autoinflammatory bone disorder in children. Vitamin D regulates immune and inflammatory pathways, but its impact on chronic recurrent multifocal osteomyeli...BACKGROUND: Chronic recurrent multifocal osteomyelitis is a rare autoinflammatory bone disorder in children. Vitamin D regulates immune and inflammatory pathways, but its impact on chronic recurrent multifocal osteomyelitis remains unclear. This study investigated the relationship between serum 25-hydroxyvitamin D levels and clinical, laboratory, and radiological characteristics of pediatric chronic recurrent multifocal osteomyelitis. METHODS: Pediatric chronic recurrent multifocal osteomyelitis patients and age- and sex-matched healthy controls were retrospectively evaluated. Serum 25-hydroxyvitamin D, inflammatory markers, clinical disease activity score, and whole-body magnetic resonance imaging findings were analyzed. RESULTS: Thirty-one chronic recurrent multifocal osteomyelitis patients and 31 controls were included. Chronic recurrent multifocal osteomyelitis patients had significantly lower 25-hydroxyvitamin D levels and a higher frequency of vitamin D deficiency (<0.001). Serum 25-hydroxyvitamin D levels showed no correlation with C-reactive protein, erythrocyte sedimentation rate, or the neutrophil-to-lymphocyte ratio, but were inversely correlated with the clinical disease activity score (=- 0.429 and =0.020) and the number of magnetic resonance imaging-detected skeletal lesions (=- 0.376 and =0.044). Logistic regression confirmed vitamin D deficiency as independently associated with chronic recurrent multifocal osteomyelitis (odds ratio=0.59, 95% confidence interva: 0.41-0.88, and =0.007). CONCLUSIONS: Vitamin D deficiency is highly prevalent in pediatric chronic recurrent multifocal osteomyelitis and correlates with disease severity, reflected by clinical activity and lesion burden, independent of systemic inflammation. These findings suggest vitamin D as a potential biomarker in chronic recurrent multifocal osteomyelitis and highlight the need for prospective studies assessing its therapeutic role.
Breastfeeding is an important factor for the health of both mother and child. A new guideline on breastfeeding duration recommends, for the first time in Germany, six months of exclusive breastfeeding and a total breastf...Breastfeeding is an important factor for the health of both mother and child. A new guideline on breastfeeding duration recommends, for the first time in Germany, six months of exclusive breastfeeding and a total breastfeeding duration of at least twelve months. However, these recommendations contradict other national guidelines and policy recommendations that suggest four to six months of exclusive breastfeeding. At the same time, the guideline rates the underlying evidence predominantly as low to very low. This article questions whether the strong recommendations are sufficiently evidence-based and discusses the methodological limitations of the new guideline on breastfeeding duration.
Trichobezoars represent a rare condition in paediatric patients, and evidence-based treatment recommendations are lacking. Most reported cases describe surgical removal, predominantly by laparotomy.We present a minimally...Trichobezoars represent a rare condition in paediatric patients, and evidence-based treatment recommendations are lacking. Most reported cases describe surgical removal, predominantly by laparotomy.We present a minimally invasive surgical strategy based on two paediatric cases of gastric trichobezoars and review the current literature. A PubMed search was performed including research articles and case reports published between 2016 and 2020. Inclusion criteria were isolated trichobezoars in patients aged ≤18 years, while reports describing combined bezoars were excluded. Extracted data included age, sex distribution, clinical presentation, medical history, imaging, treatment modality, and follow-up management.Thirty-four publications comprising 41 paediatric cases of trichobezoars were identified (age range: 2-17 y; female-to-male ratio: approximately 7:1). Endoscopic removal was attempted in 10 cases, requiring conversion to laparotomy in nine cases and to a modified laparoscopic technique in one case. Overall, 36 bezoars were removed by laparotomy, 5 by a modified laparoscopic technique, and only 1 by purely laparoscopic extraction without auxiliary incision.Our modified minimally invasive approach resulted in an uncomplicated postoperative course and a favourable cosmetic outcome. The technique enables safe removal of trichobezoars while minimising the risk of intra-abdominal contamination.
The presence of calprotectin in feces may be a consequence of neutrophil migration into the gastrointestinal tissue thanks to an inflammatory process. Fecal calprotectin is a non-invasive, cost-effective, specific, and s...The presence of calprotectin in feces may be a consequence of neutrophil migration into the gastrointestinal tissue thanks to an inflammatory process. Fecal calprotectin is a non-invasive, cost-effective, specific, and sensitive test used to compare fecal calprotectin concentrations between cerebral palsy children and healthy children and to investigate the relationship between fecal calprotectin levels and cerebral palsy.A total of 25 patients with cerebral palsy and 27 healthy children were included in the study. We conducted a 3-month cohort study following two groups of children. One stool sample was collected from each participant. Subjects provided a single fecal sample for calprotectin measurements. Fecal samples were frozen on receipt at -80°C for further analysis.Median fecal calprotectin levels were found to be statistically significantly higher in the cerebral palsy group than in the control group (=0.01). Albumin levels in the cerebral palsy group were found to be statistically significantly higher than in the control group (=0.04). Total protein levels were statistically significantly higher in the cerebral palsy group than in the control group (=0.01). This study confirms the possible association between increased calprotectin and cerebral palsy disease. The cut-off point, determined as 23.5 μg/g based on fecal calprotectin levels, yielded a sensitivity value of 0.92 and a specificity value of 0.96.Children with cerebral palsy exhibit elevated fecal calprotectin levels compared to healthy peers, suggesting altered intestinal inflammatory activity. Although fecal calprotectin is not proposed as a diagnostic marker for cerebral palsy, it may serve as an adjunctive indicator of subclinical intestinal inflammation in this population.
Influenza is one of the most common vaccine-preventable infectious diseases in Germany, with sometimes severe, even fatal courses. Young children in particular, with or without a prevalent primary disease, have an increa...Influenza is one of the most common vaccine-preventable infectious diseases in Germany, with sometimes severe, even fatal courses. Young children in particular, with or without a prevalent primary disease, have an increased risk of contracting influenza and play a crucial role in transmitting the virus to others. Vaccinations are the most effective medical measure to reduce the risk for influenza infections and complications. However, contrary to WHO recommendations, in Germany, healthy children are not included in the recommendations for influenza vaccinations. Data from various countries demonstrate that routine vaccination of children is well tolerated and reduces the number of influenza-related hospitalizations and deaths. Furthermore, a reduction in influenza infections in the general population and health economic benefits have been observed. The aim of this review is to advance the debate on the necessity of vaccinating healthy children in Germany, taking into account the current challenges.
Remke K, Hoffmann A, Zemlin M
… +10 more, Detemple MC, Shamdeen MG, Drautz F, Thomas W, Schröder N, Nipken ST, de la Rosa-Ramos T, Berger F, Picard D, Simon A
Inpatient treatment of RSV infection involves non-evidence based measures, including excessive antibiotic therapy. The introduction of nirsevimab as a passive immunization is intended to reduce the number of infants requ...Inpatient treatment of RSV infection involves non-evidence based measures, including excessive antibiotic therapy. The introduction of nirsevimab as a passive immunization is intended to reduce the number of infants requiring inpatient treatment.Can inpatient treatment of RSV infection be improved in a regional pediatric infectious diseases network? Does the introduction of nirsevimab have an impact on inpatient epidemiology?Prospective study (2024/2025) in six pediatric hospitals in one region. Agreement on a treatment standard including five interventions that should be avoided (Leave 5). Analysis of epidemiology (≤24 months and 25-36 months) 2022-2025.121 Clinical Review Forms (CRF) were collected, 101 (83.5%) patients were≤24 months old. The length of stay was 4±3 days vs. 3.5±2 days (≤24 months vs. 25-36 months). At least one risk factor was documented in 24% (≤24 months) and 25% (25-36 months; p=0.8). In 56% of cases, all 5 Leave 5 measures were avoided (≤24 months 58%, 25-36 months 45%). In patients≤24 months, only 9% received antibiotics. After the introduction of nirsevimab, there was a decrease in the number of RSV treatment cases≤24 months (over 60%; p<0.0001).Evidence-based, predominantly supportive therapy for RSV infection in hospitalized children≤24 months can be promoted through a coordinated approach in a pediatric infectious disease network. Shortly after the introduction of nirsevimab, a clear effect on inpatient epidemiology is already evident.
Hamelmann E, Schorlemer C, Derichs N
… +17 more, Eber E, Gerstlauer M, Jung A, Kabesch M, Kopp MV, Lau S, Lex C, Möller A, Schaub B, Schwerk N, Spindler T, Taube C, Vogelberg C, Zacharasiewicz A, Zielen S, Schuster A, Gappa M