[Pulmonary embolism in a girl with nephrotic syndrome and factor V Leiden - case report]. / Zatorowosc plucna u dziewczynki z zespolem nerczycowym i czynnikiem V Leiden ­ opis przypadku.

Thromboembolic complications are found in 2-3% of children with nephrotic syndrome (NS); this increased risk is caused by hypovolemia, hemoconcentration, increased number and activity of platelets, hyperfibrinogenemia and loss of coagulation inhibitors. Risk is even higher in case of additional factors e.g. congenital thrombophilia. CASE REPORT: Girl with NS aged 17 11/12 years was admitted to hospital due to respiratory tract infection with cough and back pain. NS started 9 months earlier and she had two bouts of disease, and was treated only with prednisone (current dose - 60 mg/48h). On admission she was without any abnormalities on auscultation, with BP 111/65 mmHg, HR 80 bpm, satO2 99%. Lab results showed the increase of WBC 18.3×103/µL, D-dimers 23038 µg/L and proteinuria 900 mg/dL. Other values of examined parameters were in normal limits. Chest X-ray and ECG were also normal. Presumptive diagnosis of pulmonary embolism was made and the patient was given 1000IU of antithrombin III and nadroparine (2x90IU/kg/24h s.c.). In ECHO the occlusion of left pulmonary artery and preserved blood flow in right were revealed. In angioCT clot nearly filling lumen of left pulmonary artery, clot in intermediate part of right pulmonary artery, and focus of pulmonary infarction in 10th segment of left lung were found. Doppler USG of lower limb veins did not reveal thrombi or perforator vein incompetence. Treatment with nadroparine was continued, and rapid improvement of clinical condition and disappearance of pain and cough were observed. Mycophenolate mofetil was added, which resulted in subsidence of proteinuria. Rivaroxaban was used in prophylaxis of recurrences of thromboembolism. Tests for thrombophilia revealed factor V Leiden in patient.

Computed tomography urography with iterative reconstruction algorithm in congenital urinary tract abnormalities in children - association of radiation dose with image quality.

PURPOSE: To assess the extent to which a radiation dose can be lowered without compromising image quality and diagnostic confidence in congenital urinary tract abnormalities in children by using a CT scanner with an iterative reconstruction algorithm. MATERIAL AND METHODS: 120 CT urography image series were analysed retrospectively. Image series were divided into four study groups depending on effective radiation dose (group 1: 0.8-2 mSv; group 2: 2-4 mSv; group 3: 4-6 mSv; group 4: 6-11 mSv). Objective and subjective image quality were investigated. In objective analysis, measurements of attenuation and standard deviation (SD) in five regions of interest (ROIs) were performed in 109 excretory image series, and image noise was evaluated. In subjective analysis, two independent radiologists evaluated 138 kidney units for subjective image quality and diagnostic confidence. RESULTS: There were no significant differences in image noise in objective evaluation between the following study groups: 2 vs. 3 and 3 vs. 4 in all ROIs (with the only exception in spleen SD measurement between study groups 2 vs. 3), while there was significantly more image noise in group 2 in comparison to group 4. For all other ROIs in all study groups, there was more image noise on lower dose images. There were no significant differences in pairwise comparisons between study groups in subjective image quality. Diagnostic confidence was not significantly different between all study groups. CONCLUSIONS: Low-dose CT urography can be a valuable method in congenital urinary tract abnormalities in children. Despite poorer image quality, diagnostic confidence is not significantly compromised in examinations performed with lower radiation doses.

Influence of diuretic (furosemide) on contrast medium distribution in computed tomography urography of high-grade hydronephrosis in children.

INTRODUCTION: Diuretics improve visualization of the urinary tract in computed tomography urography in adults, as well as in magnetic resonance urography in adults and children. Also, diuretics can help to diagnose upper urinary tract obstruction in intravenous urography, ultrasonography or dynamic scintigraphy. However, there are still missing data on evaluation of furosemide usefulness in computed tomography urography examinations in children with suspected congenital anomalies of the urinary tracts.The aim of this study was to compare the homogeneity of contrast medium distribution in high-grade hydronephrosis in pediatric computed tomography urographies performed with and without use of diuretic (furosemide). MATERIALS AND METHOD: We have restrospectively analyzed computed tomography urography image series performed in the Department of Pediatric Radiology, in children with suspected congenital anomalies of the kidney and the urinary tract. Kidney units with high-grade hydronephrosis were divided in two groups: non-furosemide (n = 25) and furosemide (n = 28) group, where diuretic in dose 1 mg/kg, with maximum 20 mg, was administered intravenously 3-5 min before contrast medium administration. Subjective image quality and diagnostic confidence were evaluated by two independent radiologists and compared between study groups. RESULTS: There were no significant differences in subjective image quality and diagnostic confidence between furosemide and non-furosemide groups. CONCLUSIONS: Addition of furosemide to computed tomography urography does not improve homogeneity of contrast medium distribution in hydronephrotic kidneys in children.

Postnatal Evaluation of Congenital Chest Pathologies Using a Low-Dose Computed Tomography (CT) Protocol - a Pictorial Review.

Computed tomography with its excellent spatial and temporal resolution remains a valuable diagnostic modality in pediatrics. On the other hand an increasing attention has been placed on the radiation risks associated with CT imaging, especially in children. In recent years, many advances in CT hardware and software, for example, automatic exposure control tools and iterative reconstruction techniques, have allowed for a reduction of applied radiation doses while maintaining image quality. The purpose of this paper is to present our protocol for chest CT imaging in the youngest age group, together with a pictorial review of congenital pathologies of the chest, and to emphasize factors that optimize postnatal CT imaging in infants. In our opinion, modern CT imaging with the use of dose reduction techniques and iterative reconstructions allows for a proper visualization of chest pathologies in small children, which has no influence on observer performance. The presented review of low-dose diagnostic images of a wide spectrum of congenital pathologies may serve as an example of routine utilization of the current concepts in pediatric CT optimization.

Extreme hydronephrosis due to uretropelvic junction obstruction in infant (case report).

BACKGROUND: Hydronephrosis is the one of the most common congenital abnormalities of urinary tract. The left kidney is more commonly affected than the right side and is more common in males. AIM OF THE STUDY: To determine the role of ultrasonography, renal dynamic scintigraphy and lowerdose computed tomography urography in preoperative diagnostic workup of infant with extreme hydronephrosis. CASE REPORT: We presented the boy with antenatally diagnosed hydronephrosis. In serial, postnatal ultrasonography, renal scintigraphy and computed tomography urography we observed slightly declining function in the dilated kidney and increasing pelvic dilatation. Pyeloplasty was performed at the age of four months with good result. CONCLUSIONS: Results of ultrasonography and renal dynamic scintigraphy in child with extreme hydronephrosis can be difficult to asses, therefore before the surgical procedure a lower-dose computed tomography urography should be performed.

Calyceal diverticulum of the kidney - diagnostic imaging dilemma in pediatric patients - case report.

BACKGROUND: Calyceal diverticula are rarely diagnosed in children. They can mimic other renal cystic lesions and correct diagnosis can be difficult to establish. Connection between fluid collection and collecting system confirmed by imaging studies is the key diagnostic finding. CASE REPORT: In this report we present a case of pediatric patient with calyceal diverticulum, with initial ultrasonographic diagnosis of simple renal cyst. Final diagnosis was established after extended diagnostics following infection of a fluid collection. CONCLUSIONS: 1. Differential diagnosis of well-circumscribed solitary renal fluid collections in children should include particularly: simple cyst, calyceal diverticulum and the first demonstration of ADPKD. 2. Diagnosis of calyceal diverticulum should be confirmed by contrast studies. 3. Standard management of calyceal diverticula in children includes ultrasonographic follow-up and conservative treatment and rarely requires surgical intervention.

Lower-dose CT urography (CTU) with iterative reconstruction technique in children - initial experience and examination protocol.

BACKGROUND: Conventional X-ray urography is one of the basic imaging techniques in urinary tract diseases in children. CT urography (CTU) employing standard Filtered Back Projection (FBP) reconstruction algorithms is connected with higher radiation dose. Advanced iterative reconstruction techniques enable lowering the radiation dose to the level comparable with conventional X-ray urography with better visualization of the urinary tract. Study protocol and indications for this modified technique should be discussed. MATERIAL/METHODS: Introduction of iterative image reconstruction techniques allowed to significantly reduce the radiation dose delivered during examinations performed at our Department, including CT examinations of urinary tract in children. During the last two years, CT urography replaced conventional X-ray urography and became the basic imaging technique in our Department. We discuss the study protocol regarding pediatric CTU examinations. The main goal is to receive an optimal image quality at reduced radiation dose. RESULTS: CTU examinations performed using the standard filtered back projection (FBP) reconstruction technique are associated with radiation doses about 1.5 times higher than those in conventional X-ray urography. Implementation of iterative reconstruction algorithms in advanced CT scanners allow to reduce the radiation dose to a level comparable or even lower than that in X-ray urography. In addition, urinary tract can be evaluated more precisely in multiplanar reformatted (MPR) and volume rendered (VR) images. CONCLUSIONS: 1. Advanced iterative reconstruction techniques allow to reduce radiation dose in CT examinations and to extend indications for CT urography in children. 2. Urinary tract can be evaluated more precisely in multiplanar reformatted and volume rendered images. 3. CTU may replace conventional X-ray urography in children.

[Congenital laryngeal stridor - an interdisciplinary problem]. / Wrodzony stridor krtaniowy­ problem interdyscyplinarny.

UNLABELLED: The most common causes of laryngeal stridor are laryngomalacia (60%), vocal cord paralysis (VCP) (10 %) and subglottic laryngeal stenosis. Majority of cases of VCP are idiopathic, less frequently it is the effect of abnormalities in central nervous system (Arnold - Chiari syndrome, hydrocephalus, neonatal hypoxia). Differential diagnosis should also include anomalies of aortic arch and its branches (vascular rings). The authors present two cases of neonatal congenital laryngeal stridor. In the first case the girl presented with VCP of unknown etiology. The perinatal period was normal, ultrasound of central nervous system and neurologic examination revealed no abnormalities. Due to sustained VCP in control laryngeal ultrasound examinations, tracheostomy was performed in the third month of life. In the second case, stridor was caused by laryngomalacia and subglottic laryngeal stenosis of first grade according to Meyer-Cotton scale (larynx lumen diameter <4 mm). The diagnosis was established by laryngotracheobronchoscopy. In both children angiotomography was performed and vascular ring was diagnosed (aberrant right subclavian artery). Vascular anomaly was suspected in barium X-ray. In both cases echocardiographic examination did not visualize the fourth vessel of the aortic arch. Chest X- rays were normal. Both children had no symptoms caused by vascular ring. CONCLUSION: Diagnosis of congenital laryngeal stridor is an indication for complete evaluation to establish the cause of the airway obstruction. The differential diagnosis should include laryngomalacia, vocal cord paralysis, subglottic laryngeal stenosis, congenital anomalies of large vessels and abnormalities of the central nervous system.

Chronic rhinosinusitis in children--bacteriological analysis in terms of cytological examination.

INTRODUCTION: The mechanisms of inflammatory response occurring in chronic rhinosinusitis in children are multifactorial. Besides the history and a physical examination, amongst diagnostic tools there are cytological and bacteriological examinations. OBJECTIVES: 1 Determining the nature of the bacterial flora present in the nasal cavities and paranasal sinuses in children with chronic rhinosinusitis amongst patients of The Department of Paediatric Otolaryngology,Warsaw Medical University. 2 Determining the relation between bacterial strains and cytological examination of nasal mucosa in children with chronic rhinosinusitis. MATERIALS AND METHODS: The study group included 64 patients with chronic rhinosinusitis without polyps. The control group included 30 randomly chosen children. Diagnostic tests performed in both groups were: middle meatal culture and cytological examination from the inferior nasal concha and middle meatus. Statistical analysis was accomplished with Statistica 8.0. CONCLUSIONS: Damage to the respiratory epithelial surface is understood as damage to the innate immune barrier, and repeated antibiotic therapy with the subsequent repopulation of the epithelium accidentally by various bacteria can become responsible for the pathogenic effect of bacteria in chronic rhinosinusitis.

Ultrasonography of the brain, abdomen, and heart in neonates born to liver or renal transplant recipient mothers.

BACKGROUND: Pregnancies in graft recipients are associated with increased risk of a number of pathologies. The aim of the study was to analyze results of brain and abdominal ultrasonography and echocardiography (ECHO) in neonates born to liver (LTx) or renal recipients (RTx). MATERIAL/METHODS: The study group consisted of 82 neonates born to transplanted women (46 neonates of liver recipients and 36 neonates of renal recipients), enrolled in a retrospective study. The control group consisted of 74 neonates from the general population. Sonographic examination of the brain was performed to check for the presence of intra-/periventricular hemorrhage (IVH/PVH) according to Papile, and periventricular leukomalacia (PVL).The results of abdominal ultrasonography and 2-dimensional echocardiography (ECHO) were compared between the groups. The immunosuppressive therapy used during pregnancy was also analyzed. RESULTS: No significant differences were observed between the frequency of IVH in LTx and RTx groups and LTx, RTx, and control groups. Abdominal ultrasonography revealed 1 case of suprarenal hemorrhage, 1 case of cystic kidney, and 3 cases of pyelocalyceal system dilatation in the study group. There were no abnormalities in the echocardiography in 97.8% of children born to mothers after LTx and in 94.4% after RTx. There were significant differences in the immunosuppressive therapy between the pregnant women after LTx and RTx. CONCLUSIONS: The risk was not increased in intra-/periventricular hemorrhage and congenital abnormalities of the gastrointestinal tract and heart in neonates of mothers after organ transplantation, regardless of the immunotherapy used, and risk was similar to that of the general population.

Successful complex management of a congenital diaphragmatic hernia--a case report.

Congenital diaphragmatic hernia is a very serious congenital defect associated with high mortality rate. The syndrome is characterized by migration of abdominal viscera to thoracic cavity a variable degree of pulmonary hypoplasia associated with pulmonary hypertension caused by alteration of pulmonary vessels diameter increased muscularisation of pulmonary arteries and decreased compliance of the lungs resulting in increased afterload of the right ventricle, decreased cardiac output and impaired oxygenation. We describe a case of congenital diaphragmatic hernia with migration of the stomach, the small and the large bowels and the left hepatic lobe into the left pleural space, diagnosed by ultrasound in utero at 24 weeks of gestation. The child was initially placed on high-frequency oscillation and nitric oxide and required vigorous inotropic support. The defect was repaired after stabilization of the cardiac output and gas exchange. The patient made successful recovery.

Difficulties in diagnosis of laryngeal cysts in children.

INTRODUCTION: In larynx cysts may be localized in different regions: glottis, laryngeal pouch, epiglottis, aryepiglottic folds and subglottic area. It is difficult to estimate if the cyst is acquired or congenital. Symptoms of laryngeal cyst depend on the size and localization of the cyst and include: change in the tone of voice, dysphonia, hoarseness, dysphagia, laryngeal stridor and dyspnoea. Cysts of the larynx in infants are rare but the treatment is easy once the diagnosis is made. If mismanaged, the resulting respiratory obstruction can lead to serious complications. OBJECTIVE: Analysis of cases of laryngeal cyst in children treated in the Department of Paediatric ENT Medical University of Warsaw. MATERIALS AND METHODS: A retrospective analysis of 10 cases of laryngeal cyst in children treated in the Department of Paediatric ENT in Warsaw between 2000 and 2008 was made. RESULTS: The authors analyzed indications to directoscopy, endoscopic presentation, problems with establishing the right diagnosis and management. CONCLUSIONS: Using a direct laryngoscopy as a golden standard in children with stridor will reduce a number of false diagnosis.