European Federation of Societies for Ultrasound in Medicine and Biology (EFSUMB): An Update on the Pediatric CEUS Registry on Behalf of the "EFSUMB Pediatric CEUS Registry Working Group".

The European Federation of Ultrasound in Medicine and Biology (EFSUMB) created the "EFSUMB Pediatric Registry" (EFSUMB EPR) with the purpose of collecting data regarding the intravenous application of pediatric contrast-enhanced ultrasound (CEUS). The primary aim was to document the current clinical practice and usefulness of the technique and secondarily to assess CEUS safety in children. We issue the preliminary results of this database and examine the overall practice of CEUS in children in Europe.

Langerhans cell histiocytosis in children - a disease with many faces. Recent advances in pathogenesis, diagnostic examinations and treatment.

Langerhans cell histiocytosis is a rare clonal disease characterized by the proliferation of CD1a-positive immature dendritic cells. The purpose of this article was to present an updated review of recent advances in the pathogenesis, clinical features, imaging and treatment of this disease. The discovery of oncogenic BRAF mutations and the presence of proinflammatory cytokines and chemokines confirmed the unusual characteristics of this disease. Currently, children with organ involvement who do not have a good response to chemotherapy and have neurodegeneration or diabetes insipidus are the most problematic patients. Further research is needed to improve the results of treatment.

Role of Contrast-Enhanced Ultrasound (CEUS) in Paediatric Practice: An EFSUMB Position Statement.

The use of contrast-enhanced ultrasound (CEUS) in adults is well established in many different areas, with a number of current applications deemed "off-label", but the use supported by clinical experience and evidence. Paediatric CEUS is also an "off-label" application until recently with approval specifically for assessment of focal liver lesions. Nevertheless there is mounting evidence of the usefulness of CEUS in children in many areas, primarily as an imaging technique that reduces exposure to radiation, iodinated contrast medium and the "patient-friendly" circumstances of ultrasonography. This position statement of the European Federation of Societies in Ultrasound and Medicine (EFSUMB) assesses the current status of CEUS applications in children and makes suggestions for further development of this technique.

Right Ventricle Mass Removal from Tricuspid Valve Apparatus: An Unusual Thromboembolic Complication of Severe Ketoacidosis.

The presence of a pathologic mass in the right ventricle (RV) may lead to hemodynamic consequences and to a life-threatening incident of pulmonary embolism. The diagnosis of an unstable thrombus in the right heart chamber usually necessitates intensive treatment to dissolve or remove the pathology. We present a report of an unusual complication of severe ketoacidosis: thrombus in the right ventricle, removed from the tricuspid valve (TV) apparatus. A four-year-old boy was diagnosed with diabetes mellitus (DM) type I de novo. During hospitalization, a 13.9 × 8.4 mm tumor in the RV was found in a routine cardiac ultrasound. The patient was referred for surgical removal of the floating lesion from the RV. The procedure was performed via midline sternotomy with extracorporeal circulation (ECC) and mild hypothermia. Control echocardiography showed complete tumor excision with normal atrioventricular valves and heart function. Surgical removal of the thrombus from the tricuspid valve apparatus was effective, safe, and a definitive therapy for thromboembolic complication of pediatric severe ketoacidosis.

Usefulness of lung ultrasound in diagnosing causes of exacerbation in patients with chronic dyspnea.

Dyspnea is a non-specific symptom that requires fast diagnostics, accurate diagnosis and proper treatment. The most common causes of dyspnea include exacerbation of chronic obstructive pulmonary disease (COPD) and chronic heart failure (CHF). Distinction between these two medical conditions seems to be critical in diagnostics of emergencies. At the same time, basic diagnostic tools available in emergency room, such as classic radiography (X-ray) of the chest, electrocardiography (ECG) or b-type natriuretic peptide test, are sometimes ambiguous. Therefore looking for additional diagnostic tool seems to be justified and necessary. Transthoracic lung ultrasound assessment is a simple and easily accessible examination, enabling the early and explicit diagnostics of pulmonary oedema and its distinction from other, non-cardiac causes of dyspnea. This review outlines the current knowledge on the subject of transthoracic lung ultrasound (TLUS), particularly in respect of its clinical usefulness in distinction of causes of dyspnea exacerbation.

Hyperthyroidism secondary to disseminated mucormycosis in a child with acute lymphoblastic leukemia: case report and a review of published reports.

Thyroiditis due to fungal infection is an extremely rare cause of hyperthyroidism. The most common etiological factor of thyroiditis is Aspergillus. Infections due to members of the Mucorales have been an increasing clinical problem in recent years, and the prognosis in generalized infections due to those fungi is usually very poor. No hyperthyroidism in a child with thyroiditis due to mucormycosis has been reported in the literature so far. We describe a clinical course of generalized mucormycosis with thyroid involvement in a 12-year-old girl treated for acute lymphoblastic leukemia. The child underwent a hyperthyroidism connected with thyroid involvement due to a fungal process. The diagnosis was based on the clinical signs, laboratory findings and typical ultrasound scan; however, later attempt to amplify the fungi DNA from the tissue block has failed. The child died because of multiorgan failure due to general fungal infection 49 days after the invasive fungal infection was diagnosed. The generalized mucormycosis is always connected with poor prognosis and the mortality is high.

Pediatrician! Do you know the symptoms of DRESS syndrome? A case report of a 4-year-old girl.

Drug Rash with Eosinophilia and Systemic Symptoms (DRESS) syndrome is a severe drug-induced hypersensitivity syndrome and may be observed after administration of many drugs. Clinical symptoms usually occur 2 to 8 weeks after drug introduction. Because DRESS syndrome is potentially life threatening, it is especially important to diagnose it early. Withdrawal of the drug which induced symptoms is the most important therapeutic option. DRESS syndrome appears mostly in adults. There are relatively few articles on the DRESS syndrome in children. The article presents a case of a 4-year-old girl with a life-threatening clinical course of DRESS syndrome with massive pulmonary involvement. The knowledge of DRESS syndrome clinical symptoms is essential for doctors of various specialties. It is especially important that general practitioners, pediatricians, and pediatric neurologists should be able to take this life-threatening syndrome into consideration for differential diagnosis.

Sonographic diagnosis and monitoring of localized langerhans cell histiocytosis of the skull.

PURPOSE: Langerhans cell histiocytosis (LCH) is an infrequent disorder of unknown origin associated with histiocytic proliferation. The bones' involvement (including cranial bones) is the most frequent manifestation of pediatric LCH. Imaging of skull LCH is based predominantly on conventional radiography, CT, scintigraphy, and sometimes MRI. The aim of the study was to investigate the role of sonography (US) in the diagnosis and monitoring of bone lesions in children with localized LCH of the skull. METHODS: Twenty-seven children (15 boys/12 girls, mean age 6.1 years) were diagnosed between 1991 and 2005 in the Department of Pediatrics at the Medical University of Gdansk, Poland. Fourteen children (52%) had localized LCH of the skull. RESULTS: All lesions of the skull visible on CT were visualized on US examination. Overall, 30 focal lesions were revealed by US. The largest bone lesions were accompanied by changes within the adjacent soft tissues. CT revealed 26 lesions, the location and size of which corresponded to the lesions seen on US, but soft-tissue changes adjacent to the bony lesions were difficult to visualize. CONCLUSIONS: The US appearance of focal LCH lesions in cranial bones in children is not pathognomonic, but is characteristic enough to suggest an initial diagnosis and to monitor the evolution of lesions during treatment. US should be considered an additional diagnostic tool for LCH of the skull. Although US of localized LCH cannot replace CT, it could reduce the number of follow-up CT examinations.

Contrast-enhanced ultrasound of adrenal hemorrhage: a helpful problem solving tool.

AIMS: A focal lesion in the region of the adrenal gland in a newborn often requires further assessment. Ultrasound (US) is the initial imaging method of choice in young children as it does not use radiation or require sedation and it has excellent spatial resolution. In this case series, we present contrast-enhanced ultrasound (CEUS) as a problem-solving tool in the evaluation of neonatal adrenal lesions. MATERIAL AND METHODS: The imaging and medical records of five patients with adrenal lesions were retrospectively reviewed. All patients underwent US as an initial examination and all had US follow-up. Additionally, two patients had MRI examinations. CEUS was performed in all patients as a follow up examination. The enhancement characteristics of the adrenal masses on CEUS were analyzed with the use of VueBox software. In addition, qualitative analysis of the cine loops for the presence of vascularization within the lesions was performed by consensus between two radiologists. RESULTS: The presence of an adrenal hematoma was correctly detected and characterized by CEUS in all five cases using VueBox perfusion analysis. Adrenal hematomas had no internal perfusion and flat time intensity curves. CONCLUSION: The quantitative and qualitative CEUS assessment of the mass can distinguish hemorrhage from a malignant lesion. Based on our findings, CEUS could serve as an alternative diagnostic tool to magnetic resonance imaging in the diagnosis of slowly resolving NAH lesions.

Recurrent or persistent salivary gland enlargement in children: When is it Sjögren's?

OBJECTIVES: To describe characteristic features in children with recurrent or persistent salivary gland enlargement and to propose a diagnostic algorithm with specific consideration for Sjögren's disease (SD). METHODS: In this single-center, prospective study, 45 patients < 18 years, with recurrent or persistent salivary gland enlargement of unknown etiology were enrolled from 2006 to 2019. We collected detailed clinical information to characterize this group of patients including specific details of their major salivary gland signs and symptoms. We compared clinical, laboratory and radiological parameters between 4 groups based on the results of labial salivary gland biopsy (LSGB) and between patients who met existing SD criteria or not. RESULTS: 44 patients, with a mean age of 6.8 years and female to male ratio 21:23 were observed over a mean of 3.8 years. Characteristics of salivary gland swelling episodes varied considerably between individuals, but the majority experienced ≤5 episodes per year, lasting ≤ 1 week, with swelling affecting either or both glands. Ocular and oral dryness symptoms were observed only in 25% and 59% patients, respectively. The majority were positive for ANA, but negative for SD-specific antibodies. A total of 75% patients fulfilled at least one of the existing SD criteria. CONCLUSION: SD is a major cause of recurrent salivary gland enlargement in children. For children meeting adult criteria, the diagnosis of SD is clear. However, for the many children without dryness symptoms, objective dryness, or SD-specific antibodies, further workup including a combination of salivary gland imaging and histopathological examination can help establish the diagnosis of SD.

Bilateral Ovarian Germ Cell Tumor in a 46,XX Female with Nijmegen Breakage Syndrome and Hypergonadotropic Hypogonadism

Nijmegen breakage syndrome (NBS) is a rare autosomal recessive disease, affecting mainly patients of Slavic origin. It is caused by a defect in the NBN gene, resulting in defective nibrin protein formation. This leads to chromosomal instability, which predisposes to cancer, with lymphoid malignancies predominating. Nibrin is also involved in gonadal development and its disfunction in females with NBS frequently results in a pure gonadal dysgenesis (PGD) causing hypergonadotropic hypogonadism. However, only a few ovarian tumors in NBS patients have been reported to date. We describe the first case of a girl with NBS with PGD, who developed metachronous bilateral ovarian germ cell tumors (dysgerminoma and gonadoblastoma). Pathogenesis of PGD, neoplastic transformation and therapeutic approach in females with NBS are discussed.

Ultrasound of the chest and mediastinum in children, interventions and artefacts. WFUMB review paper (part 3).

Ultrasound (US) is an ideal diagnostic tool for paediatric patients owning to its high spatial and temporal resolution, realtime imaging, and lack of ionizing radiation and bedside availability. The lack of superficial adipose tissue and favourable acoustic windows in children makes US the first line of investigation for evaluation of pleural and chest wall abnormalities.In the first part of the topic the technical requirements were explained and the use of ultrasound in the lung and pleura in paediatric patients were discussed. In the second part lung parenchymal diseases with their subpleural consolidations are reflected. In the third part, the use of ultrasound for chest wall, mediastinum, diaphragmatic diseases, trachea, interventions and artifacts in paediatric patients are summarized.

Candidemia in children after complex congenital heart defects surgery treated with caspofungin--our own experience and a review of literature.

BACKGROUND: Invasive fungal infections due to Candida species constitute an increasing clinical problem. There are no guidelines for the management of candidemia in children undergoing surgical procedures for congenital heart defects (CHD). The aim of the study was to draw attention to the problem of candidemia in children who are operated on due to congenital heart defects. MATERIAL/METHODS: We retrospectively analyzed medical documentation of 307 children with congenital heart defects treated in 1 clinical centre in Poland, from whom we selected those diagnosed with candidemia during the postoperative period. Next, we analyzed in detail the clinical course of invasive candidiasis in each individual and we performed an analysis of risk factors that lead to candidemia. RESULTS: In the analyzed period, among 307 children who underwent surgical procedures, we observed 2 cases of candidemia (0.65%) which were effectively cured with caspofungin. No adverse effects were observed after treatment with the drug. CONCLUSIONS: Candidemia in children who undergo surgical treatment of congenital heart defects is an important factor that can influence final clinical results. Caspofungin may be an effective therapeutic option when treating candidemia in children after extensive cardiosurgical procedures.

Response to Comment on Jaworska, J. et al. Consensus on the Application of Lung Ultrasound in Pneumonia and Bronchiolitis in Children. Diagnostics 2020, 10, 935.

Thank you for the opportunity to respond to the issues raised by Nenna et al [...].

Ultrasound of the pleura in children, WFUMB review paper.

Ultrasound (US) is an ideal diagnostic tool for paediatric patients owning to its high spatial and temporal resolution, real-time imaging, and lack of ionizing radiation and bedside availability. The lack of superficial adipose tissue and favorable acoustic windows in children makes US the first line of investigation for the evaluation of pleural and chest wall abnormali-ties. Lung parenchyma was previously thought to be inaccessible to ultrasound due to the presence of the air and bony thorax. The change in attitude and growing awareness of the diagnostic possibilities has led to lung ultrasound (LUS) being accepted as a valuable point of care method. In addition, the application of LUS has widened with improvements in technology such as higher resolution transducers, harmonic imaging and contrast-enhanced ultrasound. In the current World Federation of Societies for Ultrasound in Medicine and Biology (WFUMB) paper series the topic will be introduced, the technical requirements explained and the use of ultrasound in the lung and pleura in pediatric patients are discussed.

Lung ultrasound in children, WFUMB review paper (part 2).

Ultrasound (US) is an ideal diagnostic tool for paediatric patients owning to its high spatial and temporal resolution, real-time imaging, and lack of ionizing radiation and bedside availability. In the current World Federation of Societies for Ultra-sound in Medicine and Biology (WFUMB) paper series so far (part I) the topic has been introduced and the technical require-ments explained. In the present paper the use of US in the lung in paediatric patients is analysed. Lung diseases including the interstitial syndrome, bacterial pneumonia and viral infections, CoViD findings, atelectasis, lung consolidation, bronchiolitis and congenital diseases of the respiratory system including congenital pulmonary airway malformation (CPAM) and sequester but also pneumothorax are discussed.

Cystatin C and parenchymal thickness/kidney length ratio in Wilms tumor survivors.

BACKGROUND: This study presents a clinical, biochemical, and sonographic evaluation of single kidneys in Wilms tumor survivors. PROCEDURE: The function of single kidneys in 26 Wilms tumor survivors (mean age, 11.17 years; mean follow-up, 7.09 years) was evaluated using cystatin C (CysC) levels and compared to serum creatinine concentration and glomerular filtration rate (eGFR), the latter of which was estimated by the Schwartz formula. The length of the kidney, the resistance index (RI) of the renal vessels, and the parenchymal thickness/kidney length ratio (PT/KL) were evaluated by sonographic examination. RESULTS: Group A (n = 15) consisted of children with normal CysC levels, and group B (n = 11) consisted of children with CysC over 0.95 mg/L. No differences were observed between the groups in creatinine concentration, age, follow-up evaluation, age at the time of diagnosis, or kidney size. Children with elevated CysC had statistically lower eGFR (P = 0.02) and PT/KL (P = 0.0065). The correlation rate between CysC and PT/KL in all children was -0.38. Kidney hypertrophy was observed in 23 children and was correlated with CysC (group A, R = 0.46; group B, R = 0.4; P < 0.05). RI was normal in all individuals. CONCLUSIONS: CysC levels may be elevated in people with normal GFR. Hypertrophy of a single kidney increases with deteriorating kidney function. PT/KL should be verified in future studies as a sonographic marker of kidney impairment.

Thyroid isthmus thickness in prepubertal healthy children in an iodine-sufficient region.

The study presented the reference values for thyroid isthmus thickness in prepubertal healthy children. Ultrasound evaluation of the isthmus seems to be very useful in the diagnostic process and monitoring of thyroid diseases, especially the diffuse ones. The authors are not aware of the existence of any other publications on reference values of isthmus depth in children population. In our study, we evaluated isthmus thickness in a healthy population of prepubertal children from a seaside region. A total of 402 healthy children (214 boys and 188 girls) aged 7-12 years, underwent ultrasound examinations of the thyroid. We propose the following maximum values for isthmus depth: from 2.6 mm for boys at the age of 7-9 years to 3.3 mm for those aged 10-12 years and from 2.7 mm to 3.5 mm for girls, respectively.

Sonographic appearance of rhabdomyolysis - a systematic review of the literature.

Rhabdomyolysis is the process of striated muscle cell lysis, during which proteins and microelements such as myoglobin are released into the bloodstream. It is important to diagnose rhabdomyolysis as soon as possible and start the treatment according to severity, as it is a state that significantly increases the mortality of the patients. The current gold standard of rhabdomyolysis diagnosis is the creatine kinase plasma concentration test, but it can be also diagnosed with imaging techniques, such as ultrasound (US). This review aims to gather previously published information regarding sonographic appearance of rhabdomyolysis. We searched through PubMed and ScienceDirect databases for studies using designed queries. After the selection process we were left with 13 studies containing a description of US appearance of rhabdomyolysis confirmed with a CK plasma level test. Findings described in the majority of the cases were muscle thickening, ground glass opacity, traits of edema and anechoic areas. Other than these, there were several less often reported findings. As a conclusion, rhabdomyolysis seems to have its own US appearance, but for now it cannot be precisely specified and needs further research for clarification.