Nephrocalcinosis in adolescent girl with medullary sponge kidney and mild hemihypertrophy: A case report.

RATIONALE: Medullary sponge kidney (MSK) is a rare congenital abnormality characterized by cystic dilatation of the medullary collecting tubules. The disorder is likely to be complicated by nephrocalcinosis, urolithiasis, tubular dysfunctions, and urinary tract infections. In addition, it may be rarely associated with extrarenal anomalies. PATIENT CONCERN: We present a case of 17-year old girl who was referred for metabolic evaluation of bilateral nephrocalcinosis. Physical examination showed signs of mild, left-sided hemihypertrophy involving the lower limb, buttock, trunk, face, and tongue. The imaging studies of kidneys including intravenous urography and contrast computed tomography showed numerous medullary calcification and a typical picture of MSK-"paint brush"/"bouquet of flowers" appearance of the dilated tubules within the renal medulla. Laboratory evaluation revealed sterile pyuria, hypercalciuria, and hypocitraturia. INTERVENTION: The patient was subsequently treated with potassium citrate, hydrochlorothiazide, low sodium and low oxalate diet accompanied by high fluid intake. OUTCOMES: After a 1-year therapy the normalization of calciuria and citraturia occurred and no progression of nephrocalcinosis was observed. LESSONS: We conclude that MSK should always be considered as a cause of nephrocalcinosis. Since the final diagnosis requires specific imaging techniques, the concomitant extrarenal abnormalities such as hemihypertrophy may facilitate diagnostic decisions.

Premature atherosclerosis after treatment for acute lymphoblastic leukemia in childhood.

INTRODUCTION: Late cardiovascular complications are the leading causes of morbidity and mortality in patients treated for common malignancies of childhood. Late cardiotoxicity include increased development of atherosclerosis and atherosclerosis - related diseases. An evaluation of the endothelium can be made based on the measurement of endothelium-derived blood vasoactive factors, such as cytokines and adhesion molecules. Their elevated serum levels may serve as sensitive indicators of early atherosclerotic lesions in high risk patients. Currently, assessment of common carotid intima-media thickness has emerged as one of the more powerful tools for evaluation of subclinical atherosclerosis. The purpose of this study was to compare these parameters between patients after antineoplatic treatment compared to persons not exposed to such factors. MATERIAL AND METHODS: Early progression of atherosclerotic disease was evaluated in 64 survivors treated for Acute Lymphoblastic Leukaemia (ALL) in childhood, and in a control group of 36 healthy volunteers. Blood serum concentrations of selected new biomarkers, indicative of endothelial damage and inflammatory activity, were measured, including intercellular adhesion molecule-1 (sICAM-1), endothelial leukocyte adhesion molecule-1 (E-selectin), thrombomodulin (TM), interleukin 6 (IL-6), and high-sensitivity C-reactive protein (hs-CRP). The common carotid intima-media thickness (IMT) was also assessed via ultrasound examination. RESULTS: Significantly higher blood concentrations of sICAM-1 adhesive molecule (229.3±62.2 ng/mL vs. 199.9 ± 63.3 ng/ mL, p=0.0072) and IL-6 (2.1 ± 2.7 pg/mL vs. 1.9 ± 3.6 pg/mL, p=0.0414) were found in ALL survivors compared with control subjects. Concentration of hs-CRP was also higher in the ALL group: 1.3 ± 2.2 ug/mL vs. 0.6 ± 0.9 ug/mL. This difference was close to statistical significance (p=0.0599). The mean IMT values for right and left carotid arteries were higher in ALL patients after antineoplastic therapy, compared with healthy subjects (IMT-R 0.056±0.008 mm vs. 0.052±0.003 mm; p=0.0021; IMT-L 0.057±0.009 mm vs. 0.052±0.003 mm; p=0.0051). CONCLUSIONS: Survivors of childhood ALL in the examined group demonstrated elevated concentrations of selected new biomarkers and increased IMT values, compared to controls, which may confirm the occurrence of endothelial injuries in blood vessels. This study indicates that subjects treated for childhood malignancy are at a higher risk of prematurely developing atherosclerosis.

Mistakes in the ultrasound diagnostics of the abdominal cavity in pediatrics.

The diagnostics of the abdominal cavity in children, especially in the neonatal-infantile period, requires knowledge in the field of anatomical and physiopathological differences as well as clinical symptomatology and pathology at every stage of the child's development. Errors and mistakes in ultrasound diagnostics of the abdominal cavity in children result from many factors, including lack of experience in examining children and the knowledge concerning most frequent ailments and pathologies as well as the incidence or no changes in the ultrasound image of the abdominal cavity organs. The assessment of the ultrasound image should be always based on clinical data of the patient, information on the past diseases, surgeries and the results of additional examinations and laboratory tests. Particular attention should be paid to the occurrence of congenital diseases and inflammations, which may have varied clinical manifestation - especially in the case of pediatric diagnostics. The variety and non-specific nature of clinical symptoms may also mask the developing neoplastic process. Mistakes in ultrasound diagnostics, especially among the youngest children, may also be caused by technical difficulties related to carrying out the examination. The above situation results from lack of cooperation with the child, who is uneasy, wailing, fails to perform orders, which may lead to overlooking the existing lesion or overinterpreting, e.g. a full stomach or residual stool in the intestines to be a pathology. It is also of high importance to have a good class of the ultrasound equipment and technical knowledge concerning its operation. When performing an ultrasound examination in children, it is necessary to apply a wide range of phased-array, convex and linear heads and appropriate applications, the so-called pediatric software (stomach, kidneys, true pelvis, organs at the surface).

[Maxillary sinus myxoma]. / Sluzak zatoki szczekowej.

Myxoma is a slow growing, benign neoplasm, which pathogenesis still remains disputed. The lesion has well-defined borders but a true capsule is absent. Because of that myxoma can be locally invasive causing bone destruction. A change is mainly observed among persons between 20-30 years of age and is very uncommon in the pediatric population. Most myxomas are observed in myocardium, but rarely may also manifest in the head and neck region. In the paper we describe an unusual case of myxoma of maxillary sinus in a female infant. Diagnostic challenges, treatment, outcome, post-operative follow-up are discussed as well as a review of the literature in order to present many features of this rare pathology.

Closure of the thoracic duct from the left-side access: A case report.

BACKGROUND: We report a 16-year-old patient with a massive left-sided chylothorax after chemotherapy due to mixed germinal tumor of the testis with massive metastases located in the retroperitoneal space and posterior mediastinum. Chemotherapy resolved the metastases in the mediastinum but evoked a huge pleural effusion in the left pleural cavity, requiring surgical intervention.Left-sided access was used. The 5-mm camera and 3 5-mm working ports were inserted. The parietal pleura was incised and the esophagus located and protected. Behind the esophagus, the thoracic duct and concomitant tissue were clipped with titanium clips, and additionally, thrombin glue was used. Stopping of the lymph leakage was observed during surgery. A local argon pleurodesis was used to finish the procedure. The thoracic tube was removed on the third postoperative day. CONCLUSION: Left-side access may be a good alternative in the left-sided chylothorax, but the crucial points are location and protection of the esophagus during the procedure, which is also the landmark that allows for locating the thoracic duct.

The Estimation of Intima-Media Thickness and Cardiovascular Risk Factors in Young Survivors of Childhood Cancer.

Cancer treatment in childhood is thought to accelerate the development of atherosclerosis, leading to significant cardiovascular complications and, ultimately, increasing cardiovascular mortality in childhood cancer survivors, which explains the need to assess vascular status in this group. The purpose of this paper was to assess early atherosclerotic lesions based on the analysis of intima-media thickness (IMT) of the common carotid artery, as well as to analyze cardiovascular risk factors in young childhood cancer survivors. The analysis of 158 patients aged 6 to29 years, with a history of previous cancer treatment for different childhood malignancies, revealed a statistically significant difference in IMT between them and 66 age-matched healthy controls. The observed higher IMT scores in childhood cancer survivors may be indicative of premature atherosclerosis. The actual scores were 0.056±0.007 versus 0.052±0.003 (P=0.0001) as a mean score for both carotid arteries in the study group and controls, respectively. We did not observe significant differences in IMT between cancer survivors treated with chemotherapy only versus those treated with chemotherapy and radiotherapy. Similar to the general population, childhood cancer survivors are affected by different cardiovascular risk factors. These factors may enhance the direct cardiotoxicity of cancer treatment, leading to symptomatic incidents in further life, which emphasizes the need of early prevention and/or treatment in this subpopulation.

Two-dimensional (2D), three-dimensional static (3D) and real-time (4D) contrast enhanced voiding urosonography (ceVUS) versus voiding cystourethrography (VCUG) in children with vesicoureteral reflux.

BACKGROUND: Two-dimensional (2DUS) contrast enhanced voiding urosonography has been used in the diagnosis and treatment monitoring of the vesicoureteral reflux in children for over 15 years. The opportunity of performing this examination with the use of three-dimensional static (3DUS) and real-time (4DUS) techniques opens up new diagnostic horizons. OBJECTIVE: To analyze if 3DUS/4DUS bring additional information leading to an increased detection rate or change in the grading of reflux compared to 2DUS and voiding cystouretrography. MATERIAL AND METHODS: We evaluated 69 patients (mean 4.1 years) who underwent 2DUS/3DUS/4DUS contrast enhanced voiding urosonography (ceVUS) and voiding cystourethrography (VCUG) for the diagnosis and grading of vesicoureteral reflux. RESULTS: 2DUS and 3DUS/4DUS urosonography diagnosed 10 more refluxes (7.25%) than cystourethrography and in 3 refluxes (2.17%) detected a higher grade. In 9 refluxes (6.52%) 3DUS/4DUS urosonography and cystourethrography diagnosed a higher grade than 2DUS. There was a statistically significant difference between cystourethrography and 3DUS/4DUS urosonography when the number of detected refluxes and differences in grading were compared. 4DUS enabled a better visualization of reflux than 3DUS. CONCLUSIONS: 3DUS/4DUS techniques bring additional information leading to a change in reflux grading compared to 2DUS and a detect higher number of refluxes compared to cystourethrography.

Testicular adrenal rest tumors in boys with congenital adrenal hyperplasia: 3D US and elastography--do we get more information for diagnosis and monitoring?

BACKGROUND: Testicular adrenal rest tumors (TART) are the nodular testicular lesions deriving from the adrenal remnant tissue reported in boys and men with congenital adrenal hyperplasia. Until now, the diagnostics of TART have been based on a combination of clinical features, imaging methods (primarily two dimensional ultrasound--2D US), response of the foci to glycocorticosteroid (GCS) therapy and exclusion of the neoplastic process. Application of 2D US supplies however a limited range of information about the volume, demarcation, structure and vascularization of the lesions. OBJECTIVE: To define whether the use of 3D US, power Doppler and elastography changes the algorithm of the diagnostics and monitoring or treatment of TART. MATERIAL AND METHODS: In this study, modern ultrasound techniques such as 3D US and elastography were introduced in two boys with TART. RESULTS: The 3D power Doppler option gives the opportunity for accurate assessment of the volume of testes and adrenal tissue foci and their vascularization. Sonographic elastography allows the assessment of stiffness of adrenal tissue areas compared to normal testis parenchyma. CONCLUSION: The use of these modern techniques enables more adequate and advanced diagnostics, and more precise monitoring of the effects of treatment in patients with TART.

Ultrasound presentation of abdominal non-Hodgkin lymphomas in pediatric patients.

INTRODUCTION: Burkitt's lymphoma accounts for approximately 25% of lymphomas diagnosed in children of developmental age. The tumor is localized mainly in the intestine (usually in the ileocecal region), mesenteric lymph nodes and extraperitoneal space. The clinical symptoms are non-specific and include: abdominal pain, vomiting, gastrointestinal bleeding, and acute abdomen suggesting appendicitis or intestinal intussusception. On ultrasound examination, Burkitt's lymphoma may manifest itself in various ways, depending on the origin of the lesion. AIM: The aim of this paper was to review the ultrasound manifestation of abdominal Burkitt's lymphoma in children. MATERIAL AND METHODS: The analysis included 15 pediatric patients with Burkitt's non-Hodgkin lymphoma in the abdominal cavity. The mean age of the patients was 9.5. Abdominal and gastrointestinal ultrasound examinations were conducted using a Siemens scanner with a convex transducer of 3.5-5 MHz and linear array transducer of L4 - 7.5 MHz. RESULTS: Ultrasound examinations conducted in the group of 15 patients revealed pathological masses localized in the gastric wall in 3 patients (20%), in the ileocecal region in 10 patients (67%) and a disseminated process in 2 patients (13%). In 12 patients with a diagnosed Burkitt's non-Hodgkin lymphoma in an extragastric localization, differences in the morphology of the lesions were observed. CONCLUSIONS: The clinical and ultrasound picture of abdominal Burkitt's lymphoma in children is variable. A careful ultrasound assessment of all abdominal organs conducted with the use of convex and linear probes increases the chances of establishing an adequate diagnosis.

Solid tumours of perimeningeal region in children--diagnostic and therapeutic difficulties.

Thirteen patients, aged 2-17 years, were treated because of primary solid tumours of head and neck location at the Department of Children Hematology and Oncology in Lublin. The authors analyzed clinical symptoms before diagnosis and the duration of these symptoms as well as the kind of tumours. In all cases the tumour was diagnosed on histopathological examination: soft tissue sarcomas--9 children, lymphoepithelioma--4 ones. The prognosis and treatment were estimated.

The application of ultrasound contrast, 3D imaging and tissue harmonic imaging in the differential diagnosis of lymph nodes enlargement in children.

The application of Power Doppler mode examination introduced the assessment of vascularisation and measurement of blood flow parameters in lymph node's vessels as a differentiating criterion of benign and malignant lymphadenopathy. However, those criteria appeared insufficient in evaluation of the malignancy grade of enlarged lymph nodes, especially in the cases of lymph nodes with invisible or scantly visible vascularisation in Power Doppler mode. Introducing contrast media in ultrasonographic examinations enabling intensification of the Doppler signal even by 20 dB creates the hope of increasing diagnostic efficacy of ultrasonography in evaluation of vascularisation in lymph nodes enlargement. The purpose of the study was to define the usefulness of ultrasonographic contrast media (Levovist), 3D presentation and harmonic imaging in differential diagnosis of lymph nodes enlargement in children. 32 children with cervical lymph nodes enlargement underwent examination with ultrasonography. In the examinations, Levovist by Schering was used in concentration 300, the amount depending on patient's body mass. The analysis of results obtained in the study revealed that application of contrast media enables better visualisation of lymph node vascularisation. Localisation of the vessels which were not shown in conventional Doppler mode enables visualisation of the vessel architecture in the lymph node and better defining of vascularisation pattern. Application of the new methods of THI and 3D imaging and contrast media in Power Doppler examinations increases the diagnostic efficacy of ultrasonography in differentiating lymph nodes alternations.