Agnieszka Szmigielska

Mycoplasma pneumoniae as a trigger for Henoch-Schönlein purpura in children.

Mycoplasma pneumoniae is one of the most common causes of respiratory tract infections in children. Extrapulmonary manifestations are seen in up to 25% of infected patients. Extrapulmonary complications are associated with the central nervous system, gastrointestinal tract, skin changes, myocarditis, pericarditis, hemolytic anemia, thrombocytopenia and thrombosis. The majority of extrapulmonary symptoms are associated with skin changes such as exanthematous skin eruptions, erythema nodosum, urticaria, Stevens-Jonson syndrome. M. pneumoniae stimulates production of the interleukins and tumor necrosis factor (TNF) α and can cause vasculitis. Henoch-Schönlein purpura (HSP) is a leucoclastic vasculitis that affects small vessels. Clinical manifestations of HSP include typical rash, arthritis, gastrointestinal and sometimes renal involvement. The main feature in HSP is abnormal IgA deposits in vessel walls. Circulating abnormal glycosylated IgA 1 and IgG antibodies form immune complexes: IgA1-IgG and anti-IgA 1. Immune complexes activate cytokines, parts of complement and influence directly the endothelium. We report cases of three children with Henoch-Schönlein purpura with prolonged and recurrent skin and joint changes. The serological analysis (positive serum IgM) confirmed Mycoplasma pneumoniae infection. Treatment with clarithromycin caused complete regression of disease. We suggest that in the case of prolonged symptoms of vasculitis due to Henoch-Schönlein purpura, Mycoplasma pneumonia infection may be a potential cause of exacerbation of the disease.

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

Summary 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.

Urine interleukin-6, interleukin-8 and transforming growth factor β1 in infants with urinary tract infection and asymptomatic bacteriuria

Introduction Urinary tract infection (UTI) occurs in 1.1% of girls and 1.4% of boys during the first year of life. Asymptomatic bacteriuria (ABU) is usually detected incidentally in 0.9% of girls and 2.5% of boys at this age. The aim of the study was to assess the usefulness of measurement of pro-inflammatory urine interleukin (IL)-6 and IL-8 concentrations and anti-inflammatory transforming growth factor β1 (TGF-β1) level in infants with febrile UTI, non-febrile UTI and ABU. Material and methods A total of 35 children, mean age 6.14 ±3.47 months, were divided into three groups: group I – febrile UTI (n = 13), group II – non-febrile UTI (n = 13) and group III – ABU (n = 9). At the time of enrollment urine IL-6, IL-8, TGF-β1 and serum C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), and white blood cell count (WBC) were measured. Renal ultrasound was performed in all children, 99mTc-dimercaptosuccinic acid scintigraphy (DMSA) and voiding cystourethrography in children with UTI. Results Urine concentrations of IL-6 and IL-8 were significantly higher in febrile UTI compared to those with non-febrile UTI and ABU (p < 0.5, p < 0.01) and positively correlated with CRP, ESR and WBC (p < 0.01). Urine levels of TGF-β1 were significantly higher in children with febrile UTI compared to those with ABU (p < 0.05) and positively correlated with WBC (p < 0.01). Inflammatory changes in the DMSA scan were detected in 66.6% of children with UTI. No significant difference in frequency of an abnormal DMSA scan compared to a normal scan was found in groups with febrile and non-febrile UTI. No relations between urine cytokines, systemic inflammatory markers and changes in DMSA scan were observed. The cutoff value for detection of inflammatory changes in the DMSA scan for IL-8 was 120 pg/mg creatinine (Cr) and 40 pg/mg Cr for TGF-β1. Based on this value, the sensitivity for IL-8 was 58.3%, specificity 100% and for TGF-β1 66.7% and 83.7%, respectively. Conclusions We found significant differences in children with febrile UTI and ABU regarding urine IL-6, IL-8 and TGF-β1 levels. Urine cytokines and systemic inflammatory markers do not differentiate between upper and lower UTI in infants.

Ectopic ureter, renal dysplasia, and recurrent epididymitis in an infant: case report and review of the literature

The most common etiologies of acute scrotum in boys <1 year of age are torsion of the testis or an appendix, urogenital anomalies, and epididymitis. We report an infant with recurrent epididymitis associated with single‐system ectopic ureter opening into the seminal vesicle and dysplastic right kidney. Treatment included nephroureterectomy.

Bland-White-Garland syndrome – a rare and serious cause of failure to thrive

Patient: Male, 0 Final Diagnosis: Bland-White-Garland syndrome Symptoms: Cardiomegaly, feeding problems Medication: — Clinical Procedure: Reimplantation of the left coronary artery to the aorta Specialty: Pediatrics and Neonatology Objective: Rare disease Background: Bland-White-Garland syndrome (BWGS) is a very rare disease characterized by anomalous origin of the left coronary artery from the pulmonary trunk (ALCAPA). WBGS affects 1 in every 300 000 live births. Children typically present with dyspnea, pallor, and failure to thrive. Without surgical repair, most of these children die during the first months of life. Case Report: This case report describes 3-month-old boy admitted to the hospital because of feeding problems. The boy was born at term, with birth weight 3200 g, and was 10 points in Apgar score. He was breast-fed from birth. From the seventh week of age, his mother observed his increasing difficulties with feeding. Physical examination revealed pale skin, diminished heart sounds, tachycardia, cardiomegaly, and hepatomegaly. Results of urine and blood tests and ultrasonography of the central nervous system and abdomen were normal. The chest radiography showed cardiomegaly and electrocardiogram revealed anterolateral myocardial infarction. On echocardiography, an anomalous left coronary artery arising from the pulmonary artery was found. The life-saving treatment of choice was immediate surgical reimplantation of the left coronary artery to the aorta. Conclusions: Children with congenital heart disease are often prone to malnutrition, but in rare cases failure to thrive and breast-feeding problems can be the first symptoms of life-threatening diseases like myocardial infarction secondary to Bland-White-Garland syndrome (BWGS).

Calyceal Diverticulum of the Kidney – Diagnostic Imaging Dilemma in Pediatric Patients – Case Report

Summary 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.

Hypertension in a girl with severe coarctation of the aorta and renal agenesis.

A 10-year-old previously healthy girl was admitted to our hospital’s Nephrology Department with hypertension. Elevated blood pressure (133/96-154/117 mm Hg) was measured incidentally at 2 months before this admission. Physical examination revealed a loud systolic ejection murmur (3/6) at the left sternal border radiating to the interscapular region. A diffuse apex beat was displaced laterally and inferiorly (to the 6th left intercostal space), and there was no palpable pulse in the lower limbs. The blood pressure gradient between the upper and lower extremities was 20-45 mm Hg. Plasma renin activity was normal. Abdominal ultrasound revealed no left kidney; the right kidney was 109 mm long, with normal corticomedullary differentiation and normal blood flow on power Doppler. An electrocardiogram showed left ventricular hypertrophy, and a chest radiograph revealed bilateral rib notching. Two-dimensional echocardiography showed an abnormal blood flow pattern in the abdominal aorta, with low amplitude and diastolic flow prolongation. A ring-like narrowing of the aortic isthmus to 4-5 mm was noted, with poststenotic dilatation to 18-20 mm Hg. The maximal blood pressure

Nawracające zakażenie meningokokowe u niemowlęcia

N. meningitidis wystepuje wylącznie u czlowieka i wywoluje zakazenia o roznej lokalizacji. Posocznica meningokokowa związana jest z wysoką śmiertelnością. Przewlekla meningokokcemia rzadko wystepuje u dzieci. Do grupy ryzyka zakazen meningokokowych nalezą niemowleta. Autorzy przedstawiają przypadek 6-miesiecznego niemowlecia, u ktorego dwukrotnie rozpoznano zakazenie meningokokowe oraz opon mozgowo-rdzeniowych. Po dochodzeniu epidemiologicznym ustalono, ze zakazenia zostalo wywolane tym samym szczepem bakterii pochodzącym od jednej osoby. Potwierdza to koniecznośc przeprowadzania skutecznej profilaktyki u osob z najblizszego otoczenia dziecka, aby zapobiec kolejnym zakazeniom.