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Recent studies have indicated that some cases of nonparaneoplastic autoimmune encephalitis in children can be caused by a systemic autoimmune disorder that generates autoantibodies to cell membrane proteins. We describe the clinical features of a 10-year-old girl with autoimmune encephalitis with autoantibodies against the GABAA receptor in whom type 1 diabetes mellitus developed during the course of the disease. The diagnosis was based on the progressive course of disease, pleocytosis in the cerebrospinal fluid (CSF), inflammatory changes in the brain and autoantibodies against the GABAA receptor detected in serum (absent in CSF). The treatment of encephalitis included intravenous immunoglobulins, intravenous methylprednisolone, oral prednisolone, cycles of plasmapheresis; this led to temporary remission. Finally, rituximab was applied as a second-line therapy with positive results., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Measuring sinus volume in a general practice clinic is a complex and time-consuming procedure, requiring experience in the use of radiological methods In the presented research, the automatically estimated maxillary sinus volume was compared with maxillary sinus volume assessed with mathematical formulas used to calculate the volume of spheres and pyramids. The starting point for the statistical analysis were specific measurements of the sinuses. We wanted to discover which geometric shape has the volume that is nearest to the automatically estimated volume. The study was performed using samples of CT scans of pediatric patients age 1-17. The dimensions (maximal width, maximal height, maximal length) were used for manual calculations. For the automatic volume calculation, the CT Image Segmentation algorithm (Syngo Via for Oncology, Siemens) was used. Pearson's correlation coefficient was applied to analyse the interrelationship between automatically and manually calculated volume of maxillary sinus. It was statistically established that the "sphere", "pyramid" and "mean" manually calculated maxillary sinus volume were accurate and strongly correlated with the automatically estimated maxillary sinus volume. The volume of the sphere corresponds better with the automatic measurements than the volume of the pyramid. The variations are significant and they were made reliable with the application of a statistical test. It is quick and easy to calculate the maxillary sinus volume based on its linear dimensions instead of applying advanced software. The manual method for maxillary sinus volume calculation requires three linear measurements of the sinus (length, width, and height) and can be recommended if the automatically estimated volume cannot be obtained., (Copyright © 2017 Elsevier GmbH. All rights reserved.)

The aim of the study was assessment of the usefulness of multiplex real-time PCR tests in the diagnostic and therapeutic process in children hospitalized due to pneumonia and burdened with comorbidities. Methods . The study group included 97 children hospitalized due to pneumonia at the Karol Jonscher Teaching Hospital in Poznań, in whom multiplex real-time PCR tests (FTD respiratory pathogens 33; fast-track diagnostics) were used. Results . Positive test results of the test were achieved in 74 patients (76.3%). The average age in the group was 56 months. Viruses were detected in 61 samples (82% of all positive results); bacterial factors were found in 29 samples (39% of all positive results). The presence of comorbidities was established in 90 children (92.78%). On the basis of the obtained results, 5 groups of patients were established: viral etiology of infection, 34 patients; bacterial etiology, 7 patients; mixed etiology, 23 patients; pneumocystis, 9 patients; and no etiology diagnosed, 24 patients. Conclusions . Our analysis demonstrated that the participation of viruses in causing severe lung infections is significant in children with comorbidities. Multiplex real-time PCR tests proved to be more useful in establishing the etiology of pneumonia in hospitalized children than the traditional microbiological examinations., Competing Interests: All authors declare that they have no conflict of interests.

Purpose: The aim of the study was to analyze sonographic (US) renal findings in lithium-treated bipolar patients and to correlate them with renal function., Methods: Renal US and renal function tests were performed on 120 patients with bipolar disorder. Ninety patients (30 males, 60 females), aged 36-82 years, had received lithium therapy for an average of 16 years, whereas 30 patients (10 males, 20 females), aged 35-85 years, who had never been exposed to lithium, served as controls., Results: In the lithium-treated group, patients with macrocysts (22%) had poorer renal function with higher creatinine serum concentrations, lower estimated glomerular filtration rates, and lower urine specific gravity, compared with the patients without macrocysts. The US changes characteristic for lithium nephropathy (punctate hyperechoic foci, microcysts < 2 mm, and increased echogenicity) were seen in three patients. These patients had been treated with lithium for more than 20 years and had impaired renal function. Sixteen percent of patients in the control group had macrocysts; however, no correlation between their presence and impaired renal function was found., Conclusions: The presence of macrocysts in the kidneys of lithium-treated bipolar patients is associated with impaired renal function. The US changes characteristic for lithium nephropathy are rare, and in our study, were only found in patients treated with lithium for 20 years or more. © 2016 Wiley Periodicals, Inc. J Clin Ultrasound 44:354-359, 2016., (© 2016 Wiley Periodicals, Inc.)

Background: Ursodeoxycholic acid (UDCA) supplementation is recommended for cystic fibrosis (CF) patients with associated liver disease. However, its effect on fat digestion and absorption is not known., Materials and Methods: In 23 patients with mild liver involvement, a C-mixed triglyceride breath test was performed on UDCA supplementation (with and without pancreatic enzymes - standard and increased dose) and after 1 month of UDCA withdrawal. Cumulative percentage dose recovery [CPDR; median (interquartile range)] has been considered to reflect lipid digestion and absorption., Results: The enzyme supplementation resulted in a significant CPDR improvement [0% (0-0) vs. 4.6% (0.4-6.0); P<0.00046]. With the increased dose of enzymes in 16 patients with abnormal C-mixed triglyceride breath test results and lipase dose less than 3000 U/g of fat, higher CPDR values [8.6% (5.6-12.7); P<0.000027] were observed. However, a 1-month UDCA withdrawal resulted in a significant reduction in (P<0.000031) fat digestion and absorption [2.9% (0.7-5.8)]., Conclusion: UDCA supplementation seems to enhance lipid digestion and absorption in pancreatic insufficient CF patients with mild liver involvement. This finding points toward the potential impact of UDCA supplementation on nutritional status in CF patients with liver disease and underscores the often overlooked role of factors other than pancreatic enzymes on digestion and absorption of fats in CF.

We retrospectively analyzed the medical documentation of 194 children infected with Epstein-Barr virus. The diagnosis was based on clinical symptoms and the presence of the viral capsid antigen IgM antibody. Patients with severe neurologic complications also underwent neurologic examination, magnetic resonance imaging (MRI), and electroencephalography (EEG). There were 2 peaks in incidence of infection; the first one in young children aged 1 to 5 years represented 62.0% of cases. The second peak (24.6% of patients) occurred in teenagers. Febrile seizures were confirmed in 3.1% of affected children younger than 5 years and headaches in 24.2% patients, mostly older children. Ten children presented severe, neurologic complications: meningoencephalitis, acute encephalitis, acute cerebellitis, transverse myelitis, and myeloradiculitis. Our study identified a variety of Epstein-Barr virus-related neurologic complications. Epstein-Barr virus should be routinely tested for when a child presents with an apparent neuroinfection as it is a common pathogen that can induce a wide variety of signs and symptoms., (© The Author(s) 2015.)

Hepatosplenic candidiasis also known as chronic disseminated candidiasis is a rare manifestation of invasive fungal infection typically observed in patients with acute leukemia in prolonged, deep neutropenia. Immune reconstitution inflammatory syndrome (IRIS) is an inflammatory disorder triggered by rapid resolution of neutropenia. Diagnosis and treatment of IRIS are still challenging due to a variety of clinical symptoms, lack of certain diagnostic criteria, and no standards of treatment. The diagnosis of IRIS is even more difficult in patients with hematological malignancies complicated by "probable" invasive fungal infection, when fungal pathogen is still uncertain. We report a case of probable hepatic candidiasis in 4-year-old boy with acute lymphoblastic leukemia. Despite proper antifungal therapy, there was no clinical and radiological improvement, so diagnosis of Candida -related IRIS was made and corticosteroid therapy was added to antifungal treatment achieving prompt resolution of infection symptoms.

Background: Cat-scratch disease (CSD) is a common infection in children; however, the wide spectrum of its clinical picture may lead to delayed diagnosis. An unusual presentation of CSD includes in the differential diagnosis malignant diseases, Epstein-Barr and cytomegalovirus infections, tuberculosis, and mycobacterioses. The diagnostic procedure is difficult, and it is important to consider CSD as the etiology of untypical lesion., Patients and Method: We present the analysis of 22 immunocompetent children treated with the clinical diagnosis of CSD in our hospital. Their ages were 2 to 16 years (mean 9.15 ± 2.2 years). Four of them presented classical papulas at admission time. Asymmetric, local lymphadenopathy was present in 16 patients. Five children, who presented an untypical course of CSD mimicking the oncological process, were analysed carefully. There were 3 patients with skull osteomyelitis, 1 with inflammation of the parotid gland, and 1 with an extra peripharyngeal mass. The diagnosis in these children was based on epidemiological, radiological, serological, and histological factors., Results: About 25 % of children with bartonellosis present an untypical spectrum of symptoms, including the lack of documented cat contact, primary lesions, or peripheral lymphadenopathy. Radiological methods like USG, CT, MRI present the unspecific masses, but they are not enough to distinguish the Bartonella inflammatory and oncological process. The final diagnosis was based on a histological method with additional polymerase chain reaction test., Conclusion: CSD should be considered in differential diagnosis of any patient with untypical lesions located on the head, neck, and upper extremities.

Objectives: Anatomical and developmental descriptions of the maxillary sinus may be of great clinical importance. An understanding of age-related changes in the dimensions and volume of the normal maxillary sinus may help in the evaluation of radiographs and identification of sinus abnormalities. The aim of the present study was to define growth patterns of maxillary sinuses in children up to the age of 18 years and evaluate the correlation between normal age-related changes in dimensions and volume., Methods: The research sample consisted of CT scans of 170 patients subdivided into 17 groups based on age. Normal developmental changes were investigated and linear dimensions measured., Results: The maxillary sinus, present at birth, increases in size until the end of the 18th year. The growth pattern includes changes in vertical, horizontal and antero-posterior directions. No bilateral dimorphism was observed, but gender-related differences were found in children over the age of 8 years. The most extensive period of growth occurs during the first 8 years and by the end of the 16th year the maximal values of all diameters and volume are reached., Conclusions: A CT study of developing maxillary sinuses allowed a precise evaluation of age-related changes in all diameters and volume to be made., (Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.)

Background: The occurence of aneurysms in young patients, under 18 years of age, is estimated at 0.5-2% of all diagnosed aneurysms., Case Report: We reported on a case of a 16-year-old patient with subarachnoid hemorrhage diagnosed due to a ruptured cerebral vessel aneurysm. The angio-CT revealed an aneurysm of the middle cerebral artery, in its distal branch. An ad hoc coil embolization was performed with angiographic success. After 6 months following the ictus, the patient underwent a control angiography which confirmed total occlusion of the aneurysm with no residual inflow. Clinical examination revealed no neurological deficits and the patient was rated 0 in mRS (modified Rankin Scale)., Conclusions: In experienced departments of interventional neuroradiology the endovascular treatment should be the treatment of choice.

Introduction: The aim of this study was to investigate the associations between radiological findings, blood eosinophilia, hyperimmunoglobulinemia E and G and Toxocara seropositivity in Polish children with newly diagnosed pulmonary infiltration., Material and Methods: We retrospectively analyzed the documentation of 119 patients, aged 1 to 18 years (mean age: 7.21 ± 4.82), who were seropositive in Toxocara sp. antibodies. In all cases, peripheral blood eosinophils and leukocyte counts, serum total IgE, IgG levels and specific IgG antibodies against excretory and secretory Toxocara sp. antigens were measured at the first presentation. After the confirmation of seropositivity, all children had a routine radiological examination., Results: In the documentation of 23 children (mean age 3.58 ± 2.63 years) we found abnormalities in the radiological examination of their lungs. Fifteen children who had abnormalities in radiological findings presented clinical respiratory complaints such as chronic cough, wheezing, asthma and haemoptysis. Eight children were asymptomatic. The analysis of peripheral eosinophils and leukocyte number, the level of IgE and specific anti-Toxocara IgG presented significantly higher values in children with radiological lesions than in children who had correct radiology. The concentrations of total IgG and gamma globulins were not significantly different. In 10 patients CT showed irregular round nodules with and without halo ranging from 1 to 13 mm. The number of nodules varied from a single lesion to multiple, disseminated ones. All nodules were located in peripheral areas of the lungs. None of them were found in the central areas. In 13 patients, CT images showed ground-glass opacities with ill-defined margins. None of the CT images presented lymphadenopathy and pleural effusion., Conclusion: The pulmonary lesions in small children with high eosinophilia and hyperimmunoglobulinemia E could be related to toxocariasis and for this reason they are eligible to undergo therapy with prolonged observation for several months, rather than start invasive malignancy investigations.

Severe combined immunodeficiency (SCID) is characterized by the absence of functional T lymphocytes and impairment of adaptive immunity. While heterogeneity of the genetic background in SCID leads to the variability of immune phenotypes, most of affected newborns appear healthy but within the first few months they develop life-threatening opportunistic respiratory or gastrointestinal tract infections. The objective of the study was to define the presenting features and etiology of infections in children with SCID. We retrospectively reviewed five children in whom the diagnosis of SCID had been established in our pediatric immunology clinic over the last 10-year period. A viral respiratory tract infection was the first manifestation of SCID in all the children studied. Cytomegalovirus (CMV) pneumonia was recognized in as many as 4 cases and coronavirus pulmonary infection was diagnosed in one case, whereas Pneumocystis jiroveci was identified as a co-pathogen in one CMV-infected patient. Severe combined immunodeficiency is a pediatric emergency condition and given the significant impact of pulmonary CMV infection in SCID children, establishing an accurate etiological diagnosis is of essential importance in instituting the specific treatment and improving the outcome.

Primary Epstein-Barr virus infection in children typically presents as infectious mononucleosis and in immunocompetent individuals severe pneumonitis proves to be a rare complication. Chronic active Epstein-Barr virus infection (CAEBV) is associated with multiple life-threatening conditions, including interstitial lung disease with fibrosis and lymphoid and lymphohistiocytic infiltrations. We report on a pediatric patient in whom CAEBV resulted in severe pneumopathy with a fatal outcome.

Background: Hyperimmunoglobulinemia E syndrome (hyper-IgE syndrome, Job syndrome, HIES) is a complex immune deficiency with multiorgan clinical manifestations and diverse genetic background. The clinical triad of symptoms observed in approximately 75% of patients with HIES includes: recurrent abscesses of staphylococcal etiology, recurrent respiratory infections and elevated immunoglobulin E in serum., Case Report: The paper discusses three cases of female patients presenting typical pulmonary complications of the hyper-Ig E syndrome. In the first case, the development of aspergilloma in a postinflamatory cyst was observed, in the other one, pneumonia with pleural effusion, and as a consequence of inflammatory infiltrations - fibrotic changes, giving rise to lobectomy, while in the last of these cases, the course of lung disease was complicated by formation of staphylococcal abscess. In one of the girls, bronchiectasis appeared at follow-up., Conclusions: Complications of pulmonary infections are the most common causes of death in hyper-Ig E syndrome. Late diagnosis significantly worsens the respiratory function and reduces the chance for normal development of a child. Introduction of comprehensive treatment, including prophylaxis, decreases the recurrences. Therefore, the important role is attributed to the radiologist in the multidisciplinary care of patients with this syndrome.

Background: A suspicion of ventriculo-peritoneal shunt failure is classified as the most common indication for CT in children with hydrocephalus. The main target of the study was to evaluate the diagnostic value of a low-dose protocol and to compare a total DLP received by patients in compared protocols., Material/methods: Our retrospective analysis included 256 examinations performed in patients aged from 1 month to 18 years, with body mass ranging from 3 to 100 kg. The examinations were conducted in the years 2009-2011. A total number of 128 examinations were performed on the basis of the low-dose protocol and 128 according to a standard protocol using the Siemens SOMATOM Definition AS 128-slice scanner. RESULTS/CONLUSIONS: The analysis showed a full value of the diagnostic low-dose protocol with a simultaneous decrease of the total dose of DLP to the average of 40%. Application protocol with lower mAs in assessing the causes of ventriculo-peritoneal shunt failure in children with hydrocephalus is coherent with the valid principles of radiation protection in pediatrics and reduces the total DLP while maintaining a very good diagnostic value.

Unlabelled: Although pneumothorax has been thoroughly described since the beginning of 19th century there are few studies investigating its epidemiology in pediatric patients. The aim of this study was to evaluate incidence and clinical picture of pneumothorax in pediatric patients., Materials and Method: A retrospective review of medical records of all patients treated in the Department of Pediatric Pulmonology, Allergy and Clinical Immunology of Karol Marcinkowski Medical University from January 1999 to December 2008 with the diagnosis of pneumothorax. Data analyzed include anthropometric parameters, present comorbidities, clinical presentation, treatment, length of hospital stay as well as presence and type of complications., Results: 27 episodes of pneumothorax in 21 patients were treated in the given period. There were 52% of episodes of spontaneous primary pneumothorax, 37% of secondary spontaneous pneumothorax and 11% of pneumothorax due to non-penetrating trauma. 59% of patients were males. Presenting symptoms included dyspnoe (59%), chest pain (48%) and cough (44%). 74% of cases required treatment with chest tube drainage: 80% episodes of primary spontaneous pneumothorax, 67% episodes of non-penetrating trauma pneumothorax and 71% episodes of secondary spontaneous pneumothorax. Mean time of chest tube drainage was 6.2 +/- 5.1 days: 4.5 +/- 0.7 days for non-penetrating trauma pneumothorax, 5.7 +/- 6.4 days for secondary and 7.2 +/- 1.4 days for primary spontaneous pneumothorax. Chest tube drainage was successful in 80% of cases. 4 patients were referred to thoracic surgeons. One child was treated with chemical pleurodesis. Mean hospital stay was 22.2 +/- 13.7 days: 11.1 +/- 0.7 days for patients with non-penetrating trauma pneumothorax and 29.7 +/- 1.4 days for patients with spontaneous secondary pneumothorax., Conclusions: Primary and secondary spontaneous pneumothorax is a rare event in children and the majority of patients are male. Secondary spontaneous pneumothorax is a complication of underlying chronic pulmonary conditions, most frequently cystic fibrosis and pulmonary infections. Children presenting with spontaneous secondary pneumothorax tended to be younger and required longer hospital stay.

Background: Meningocele or meningocephalocele is a rare defect, which consists in the displacement of the structures of the brain or/and meninges through a congenital cavity in the cranium. Depending on the location, there are hernias of the skull vault and base or external and internal hernias. Some of them can cause breathing disorders in children., Case Report: We described a case of an eutrophic, full-term newborn boy with breathing disorders. A laryngological examination found a tumor of the left nasal meatus, from which a sample was collected for histopathological study. Due to a cyst revealed under the lesion and pulsation of the tumor, it was decided to include imaging examinations into the diagnostic process. Computed tomography (CT) showed a mass of meningocele type, which was confirmed in magnetic resonance imaging (MRI)., Results: In case of breathing disorders and a suspected mass in the nasal meatus in a newborn, a congenital meningocele or menigocephalocele should be assumed. This constitutes a contraindication to sampling of the lesion, without prior imaging tests (CT and/or MRI).