[Profile of Activation of Tyrosine Kinases and MAP Kinases in Therapy of Maffucci Syndrome]. / Profil aktivace receptorových tyrozinkináz a mitogenem aktivovaných proteinkináz v terapii Maffucciho syndromu.

BACKGROUND: Maffucci syndrome is a rare congenital nonhereditary disease characterized by multiple hemangiomas and enchondromas, which may progress into malignancy. The causal therapy does not exist, and therapy is aimed at complications. The determination of appropriate therapy is complicated, and a multidisciplinary approach is often essential. CASE: Authors are presenting the case of a 20-year -old patient with Maffucci syndrome. During her life, multiple enchondromas and progressing hemangiomas have been revealed and they have caused many complications, such as limited movement, growth failure, pain, fluidothorax and ascites. A profile of phosphorylation of selected tyrosine kinases and MAP kinases from progressing hemangioma was performed and with consideration of the result, it led to change of treatment strategy with encouraging clinical response lasting for six months.

Metronomic chemotherapy with the COMBAT regimen in advanced pediatric malignancies: a multicenter experience.

BACKGROUND: The outcome of children with refractory/relapsed malignancies remains poor and novel therapies are urgently required. One of the promising approaches is metronomic chemotherapy. We present the clinical results of 74 children with advanced solid tumors treated according to treatment recommendation with data registry in three European pediatric centers. METHODS: COMBAT (Combined Oral Metronomic Biodifferentiating Antiangiogenic Treatment) included low-dose daily temozolomide, etoposide, celecoxib, vitamin D, fenofibrate and retinoic acid. From 2004 to 2010, 74 children were enrolled. RESULTS: The 2-year overall survival (OS) was 43.1% (median 15.4, range 1.3-69.9 months). Of the 74 patients, 50 patients (68%) died and 24 are alive: 6 (8%) with progressive disease, 7 (9%) with stable disease/partial response and 11 (15%) in complete response. Median time to response was 6 months. Of 62 patients with initially measurable disease, 25 (40%) had radiological response or stable disease. Fourteen of 25 showing clinical benefit responded within the first 6 months. The treatment was well tolerated on an outpatient basis. Regarding non-hematological toxicity of grade ≥2, hepatotoxicity of grade 3 occurred in 8 children and grade 3 cheilitis in 16 children. CONCLUSION: COMBAT is a feasible and effective treatment option for patients with relapsing/refractory malignancies. The treatment is well tolerated with a low acute toxicity profile.

Juxtaglomerular cell tumor--a rare cause of secondary hypertension.

Secondary hypertension (SH) is much more common in children than in adults. We report a 17-year-old girl with severe hypertension, hypokalemia and metabolic alkalosis. Because of these findings, primary or secondary hyperaldosteronism was suspected. Her initial treatment with spironolactone and ACE inhibitor was unsuccessful. With consideration of high plasma renin activity, the renal computed tomography angiography was performed and showed tumor mass in the left kidney. An uncomplicated partial left nephrectomy was performed. Histopathological examination and electron microscopy showed typical features of juxtaglomerular cell tumor (JCT). Imunohistochemistry of tumor was positive for CD34 and CD117 and this finding is effective in the diagnosis of JCT if immunostain for renin is unavailable. After the resection of JCT, the patient's blood pressure and hypokalemia returned to normal range. JCT is a rare renal neoplasm and an unusual cause of SH in children or adolescents (Fig. 2, Ref. 12).

[Radiotherapy induced glioblastoma in a child previously treated for cerebellar medulloblastoma (case report and review of the literature)]. / Radioterapií indukovaný glioblastom u dítete léceného pro meduloblastom mozecku (kasuistika a prehled literatury).

Malignant gliomas represent the most frequent radiotherapy induced ("secondary") solid tumor. Their prognosis remains extremely poor despite of aggressive multimodal treatment. We present a case report of a 16 years old boy who developed cerebellar glioblastoma six years following the combined treatment for medulloblastoma. Clinical history, pathological and cytogenetic findings of the case are discussed along with treatment possibilities.

Central neurocytoma: the neuroradiological perspective.

BACKGROUND: Central neurocytoma (CN) represents a rare, relatively recently described primary central nervous system tumor. It ranks among intraventricular tumors due to its predominant location within the lateral brain ventricles. CN occurs mostly in young adults around the 3rd decade of life; almost a fifth of the cases are children under 18 years of age. OBJECTIVES: The authors present three cases of patients with histopathologically confirmed CN, emphasizing diagnostic imaging issues. A review of the literature concerning differential diagnosis and clinical and therapeutic aspects is also presented. CONCLUSION: Literature reports of CN comprise most likely case reports, small cohorts of patients, and meta-analytic studies due to the generally low incidence of this tumor. In the current paper, the authors summarize up-to-date knowledge of this rare disease on the background of their own observations. CN should be included in the list for differential diagnostics of intraventricular brain tumors, especially those located in lateral ventricles.

[Malignant tumors of long bones in children: differential diagnosis and the role of imaging methods]. / Maligní tumory dlouhých kostí u detí, diferenciální diagnostika, prínos zobrazovacích metod.

PURPOSE OF THE STUDY: The aim of the study was to analyze primary malignant tumors and tumor-like lesions of long bones, in relation to their localization, characteristics and distribution in age groups, in children and young adults, and to assess the role of radiography and magnetic resonance imaging (MRI) in their diagnosis. MATERIAL: Sixty-four patients, aged between 3 and 20 years, who were referred to us with the diagnosis of a suspected malignant long-bone tumor of osseous or cartilage origin in the period from 2000 to 2004 were included in the study. METHODS: Plain radiography and MRI were carried out on the same day. For MRI, the Magnetom Open Viva system (magnetic field strength of 0.2 T) was used and examination comprised a conventional STIR (corresponding to fat saturation) and a T-weighed sequence. Most patients also underwent post-contrast examination with paramagnetic contrast medium infusion. RESULTS: In 26 children (40.6 %) a primary malignant tumor of osseous or cartilage origin was diagnosed; one child (1.5 %) had a giant-cell tumor. The definitive diagnoses in 37 children (57.9 %) included osteomyelitis in 12, fatigue fracture in 11, posttraumatic myositis ossificans in three children, and miscellaneous diagnoses in the remaining 11 children (one, cartilaginous exostosis; three, unicameral bone cyst; two, non-ossifying fibroma; one, fibrous dysplasia; one, subperiosteal abscess; one, histiocytosis; one, foreign body; one, negative MRI findings). CONCLUSIONS: To confirm the diagnosis of a malignant long-bone tumor, high quality X-ray and MRI are essential; CT examination is recommended in specific indications. The results of imaging methods cannot be assessed without reference to each other. Primary malignant tumors of long bones usually involve a large soft-tissue mass, and an exclusively intraosseous localization is rare. Osteosarcoma often invades the epiphysis. In making the differential diagnosis of primary malignant bone tumors, special attention must be paid to differentiation from osteomyelitis or fatigue fractures. The majority of juvenile bone lesions of any origin are usually detected between 10 and 13 years of age. These pathologic lesions are most frequently localized in the distal or proximal transition zones between the diaphysis and metaphysis.

[Synovial sarcoma in child -- case report]. / Synoviální sarkom u dítete -- kazuistika.

Soft tissue tumors occur rarely and account for 7 to 10% of all malignant neoplasms in children under 15 years of age. They constitute a heterogeneous group of tumors arising from primitive mesenchymal tissue. According to their origin they are classified as rhabdomyosarcomas and non-rhabdomyosarcomas of soft tissues. Synovial sarcoma is a rare fibroblastic tumor of soft tissue. In children under 5 years it is found only exceptionally. This study reports on a synovial sarcoma localized on the right lower extremity in a boy aged 4 years and 5 months. Differential diagnosis, prognostic factors and the role of imaging methods are discussed.

[3D ultrasonography for examination of the musculoskeletal system]. / 3D ultrasonografické vysetrování pohybového aparátu.

Three-dimensional (3D) sonography is the next logical step in diagnostic ultrasound examination. The true value of 3D ultrasonography, however, becomes evident only if 3D structures can be assessed without preconceptions ensuing from 2D interpretations. 3D ultrasonography can greatly improve our understanding of locomotor apparatus anatomy and pathology. The authors used spatial analysis to evaluate the data obtained by examination of patients with orthopedic diagnoses. The Voluson 530 MT and SONOReal system were used for examination. The Voluson permits a choice of either a 2D or a 3D imaging program for musculoskeletal system examination. The SONOReal, owing to a positional sensor of the probe, can be attached to any ultrasound transducer. In the period from 1990 to 2004, a total of 19 000 patients were examined by ultrasonography and, in 6 500 of them, the diagnosis was verified by another method, which showed a 99 % reliability of ultrasound examination. In 350 patients 2D imaging was followed by 3D examination; in 53 of them, 3D coronal and multiplanar imaging made the diagnosis based on 2D imaging more accurate and, in 12 patients, it provided new information on the patient's diagnosis. 3D reconstructions were made in 101 patients, of these 40 had been examined by other imaging methods (magnetic resonance, computer-assisted tomography) or arthroscopy. The results of examination showed a 100% correlation. Spatial reconstruction is based on the volume rendering method. This is an extension of the planar reconstruction method. Additional image processing techniques are used for a region of interest within a 3D volume data set. 3D ultrasound revealed a spatial relationship between lesions and their surfaces. The surface mode requires that the interface between tissues with different acoustic impedances should be a start line of 3D rendering. The acoustic threshold is a condition that restricts imaging circumstances in which surface rendering will be successful. Exploring 3D reconstructions with power Doppler scanning, which is more sensitive for tracking vessels, is a unique technique that can hardly be compared with any other imaging modality. 3D-volume imaging gives the examiner freedom to generate anatomical views from an infinite number of perspectives and allows us to explore anatomic relationships in the ways not available in any conventional 2D imaging. A spatial reconstruction presents a nearly perfect anatomical model. The possibility of storing volume data is considered a further progressive trend. It greatly contributes to enhancement of the scope of follow-up examinations, permits comparisons of expert conclusions and can serve educational purposes. The digital technology offers various networking solutions and plays a role in the development of 3D telemedicine. Although the diagnostic efficacy of 3D imaging is not greatly enhanced when compared with a 2D examination done by a well-trained specialist, the features of coronary sections and spatial reconstructions represent great progress of this imaging technology.

[Endoscopic investigations in children under 3 years of age at the clinic of paediatric surgery]. / Endoskopické výkony u detí do 3 let na Klinice detské chirurgie.

BACKGROUND: Retrospective study of gastroscopic investigations in children under 3 years of age. METHODS AND RESULTS: Since 1.1.2002 till 31.10.2004 we performed 119 endoscopic investigations in children of this age. 21 patients were excluded because of missed documentation. The average weight of our children was 11.8 kg. (min. 2.8, max 20 kg). In 55 cases suspicion for the oesophageal or gastric corrosion was the reason of investigations. in 24 cases swallowed alien bodies, in 10 patients we performed dilatation of oesophageal stenosis in repeated sessions in 1-6 moths periods. In 4 children we introduced percutaneous endoscopic gastrostomy (PEG), in 3 children ERCP was performed because of cystic dilatation of the bile duct. In I child the supposed hematemesis was the reason of gastroscopy (negative) and in one patient oesophageal varicula were assumed (negative). CONCLUSIONS: Oesophageal or gastric corrosions were assured in 12 cases (Grade 1.-2.:20.2%). 5 alien bodies couldn't be removed because of peristalsis, 1 object because of its dimensions (25% failures). Our dilations were performed in oesophageal stenosis with bougies or balloon dilator. 9 children were first operated for oesophageal atresia (Vogt IIIb), 1 child was after an operation for oesophageal corrosion with perforation.

[Use of magnetic resonance in preoperative evaluation of urinary tract obstruction in children--initial results]. / Vyuzití magnetické rezonance pri predoperacním vysetrení obstrukcí mocových cest u detí--první výsledky.

OBJECTIVE: To evaluate hitherto assembled experience with the application of magnetic resonance in the preoperative diagnosis of patients with obstruction of the upper urinary pathways. METHOD: Urography using magnetic resonance was performed in 22 children aged 1 month to 17 years with a sonographically assessed dilatation of the upper urinary pathways. The examinations were compared with concurrent excretory urography and formed part of a complex of preoperative examinations. RESULTS: In seven patients magnetic urography was evaluated as more accurate than excretory urography, in five instances excretory urography was more accurate, in the remaining children the results were comparable. CONCLUSION: The advantage of urography implemented by magnetic resonance is the short examination time, its non-invasive character, absence of ionizing radiation, impossible allergenization, and the examination does not depend on the functional fitness of the kidney. The results of examination are comparable with X-ray excretory urography.

[Magnetic resonance urography as an indicator for surgical treatment of anomalies of the upper urinary tract]. / MR urografie jako indikacní kritérium v chirurgické lécbe anomálií horních mocových cest.

AIMS: To compare the quality of MR of urography with intravenous urography, sonography and scintigraphy of urinary tract, presentation of MR urography as a possible substitution of so far used examinations in preoperation diagnostics of anomalies in upper urinary tract. METHODS: Static MR urography was used to examine 51 patients at the age of one month to 18 years with dilation of upper urinary tract detected by sonography. In 12 of them a dynamic MR study of the kidneys was also performed. Magnetic urography was compared with elimination urography, sonography and dynamic kidney scintigraphy. The detection rate was evaluated within the framework of the complex preoperation examinations for different stages of urinary tract in patients up to 2, 6, 12 and 18 years, respectively. RESULTS: In the total number of 36 kidneys, modified by hydronephrosis, magnetic urography was evaluated as more precise in 18 cases, elimination urography in eight and both examinations were considered equal in 10 cases. The imaging of megaureters by MR urography was more precise in 7 cases, elimination urography was at the same level six times and less precise in eight patients. The results differed in individual age categories. Sonography imaging of the kidney was good as well as for the lower portion of ureter but insufficient for the middle portion. The same course of excretion curves in dynamic excretion urography and dynamic scintigraphy of the kidney was reached in 6 patients. CONCLUSION: MR urography is capable to provide information on anatomy and function of the whole urinary tract in a single examination, being different from excretion urography by its ability of imaging and evaluating the hypofunctional part of the kidney. It represents a possibility to significantly reduce present examination which exerts a load upon the patient.

[Treatment of redislocated fragments of long bones using plaster cast wedging]. / Uprava redislokace fragmentu dlouhých kostí klínováním sádrového obvazu.

The authors wish to remind of the advantages of an old conservative therapeutic method used in redislocated long bone fractures--cast wedging, Gipskeilung. This mode of treatment makes it possible to restore axial as well as planar dislocation and it is used in corrections of redislocated fractures of the leg and is applied during the last 3 years also in the treatment of fractures of the forearm in children. In 1997-2000 this procedure was used for treatment at the Clinic of Paediatric Surgery, Orthopaedics and Traumatology, 33 children aged 3-16 years, with a redislocated fracture of the leg, 21 children aged 3-16 years, mean age 9.8 years, and with a redislocated fracture of the forearm 12 children aged 5-15 years, mean age 10.2 years. In no instance this treatment failed or was associated with serious complications. According to experience with our group of patients a method is involved which in correct indications holds a justified place in treatment of fractures of the leg and forearm in traumatology of child an adolescent age.

[Injuries of the cervical spine in children--2 case reports]. / Poranení krcní pátere u detí--2 kazuistiky.

Helical CT of the spine was done for suspicion of injury in 22 children (from July 1999 to December 2000): cervical spine in 7 children (average age 12.1 years), thoracic spine in 10 children (average age 11.9 years), lumbar spine in 5 children (average age 14.6 years). MR examination were done in 23 children: cervical spine in 3 children (average age 7.5 years), thoracic spine in 16 children (average age 10.5 years), lumbar spine in 4 children (average age 13.3 years). (There were no differences between the distribution of boys and girls in thoracic spine injuries; in cervical and lumbar spine injuries girls predominate). 2 case reports are described.

[Use of central venous catheters in children with hematologic neoplasms from the viewpoint of the pediatric surgeon]. / Pohled detského chirurga na pouzití centrálního zilního katétru u hematologicko-onkologicky nemocných detí.

Venous access by cannulation of the central vein is a necessary prerequisite of the oncological treatment. Within the period 1998-2001 there 395 CVCs (Central Venous Catheters) were introduced using a subcutaneous tunnel in 335 hospitalised patients at the Clinic of the Child Surgery, Orthopaedics and Traumatology FN Brno. In 310 patients Hickman CVC was used and in 25 Groshong CVC. Using the vena subclavia 1. dx., 131 CVCs were introduced, via the vena subclavia 1. sin. 264 CVCs. The average period of CVCs introduction was 3 months. In 78 introduced CVCs infectious complication developed. A local infection was noticed only 8 times, general infection at 70 cases. In 13 patients CVCs had to be removed. The authors present their experience with the introduction of tunneled CVCs in oncological patients and the options for solving the infectious complications when using CVCs.

[Fractures and physeal injuries of proximal humeral end in children.]. / Zlomeniny a fyzární poranení proximálního konce humeru u detí

A total of 102 children and adolescents, 27 girls and 75 boys of the median age of 10.9 years, were treated for injuries to the proximal humerus at the PediatricTrauma Centre of the Clinic of Pediatric Surgery and Traumatology in Brno over the period of 1993-1997. A conservative approach was indicated in 51 cases (50%), percutaneous pinning after conservative reduction in 50 cases (49%) and an open surgical approach in one case (1%). In 97 patients the treatment results were evaluated as excellent, in 5 patients as good. No avascular necrosis of the caput humeri was recorded. The percutaneous pinning management is prefered because of the final management of the fracture by way of stabilizing the retention of fragments, substantially higher patient's convenience in SH II, Neer-Horowitz III, IV injury types and in fractures of the proximal humerus with a long fragment with displacements. Key words: proximal humeral end, fractures, physeal injuries, children, percutaneous pinning.

Selective treatment program for developmental dysplasia of the hip in an epidemiologic prospective study.

Over 1 year of ultrasound (US) mass screening, 4,568 newborns were examined consecutively at age 3-4 weeks using both clinical and ultrasonographic examination methods. The US examination involved both the static and dynamic scanning. In total, 25 babies were treated early (5.5 per 1,000), and 6 babies were given late treatment for postnatally developing acetabular dysplasia (1.3 per 1,000). Early treatment was instituted only for hips remarkably distorted anatomically, as could be seen from their shape on the static scan, and for those that were sonographically unstable with an apparent stress displacement. Clinical examination at age 3-4 weeks failed in more than half of all sonographically abnormal cases. In one case, the postnatal development of the hip joint was associated with the worsening of its formation and a developing subluxation despite its primary normal characteristics.

[Systemic enzyme therapy in the treatment of supracondylar fractures of the humerus in children]. / Systémová enzymoterapie v lécbe suprakondylické zlomeniny humeru u detí.

The authors present their experience with enzyme therapy--the preparation Wobenzym--in comprehensive treatment of supracondylar fractures of the humerus in children. On monitoring the condition of the extremity by Doppler ultrasound, where the flow through the radial artery was quantified they obtained better results in the group of patients treated by systemic enzyme therapy than in the control group. Systemic enzyme therapy is recommended as a suitable supplement in comprehensive treatment where the most important part is played by correct and early treatment along with precise monitoring of the extremity during the postoperative course.