Milan Urík

Decreased WNT3 expression in chronic lymphocytic leukaemia is a hallmark of disease progression and identifies patients with worse prognosis in the subgroup with mutated IGHV

The canonical Wnt pathway, dependent on β‐catenin‐controlled transcription, is the most explored Wnt pathway, known to drive the malignant transformation of multiple cell types. Several reports have suggested that this pathway also participates in chronic lymphocytic leukaemia (CLL) pathogenesis. To get a better insight into the role of the Wnt/β‐catenin pathway in CLL we analysed in detail the expression of the most overexpressed Wnt ligand, encoded by the WNT3 gene, in a well‐defined cohort of 137 CLL patients. Our analysis demonstrated that (i) untreated patients with more aggressive disease (with a notable exception of patients with 11q deletion) express less WNT3, (ii) WNT3 declines with disease progression in a significant proportion of patients and (iii) low WNT3 was identified as a strong independent marker indicating shorter treatment‐free survival in CLL patients with IGHV mutation. Interestingly, CLL‐related lymphoid cell lines, but not stromal cells, failed to respond to the ligand‐induced activation of the Wnt/β‐catenin pathway. This opens the possibility that CLL cells use Wnt‐3 to communicate with the cells in the microenvironment. We thus propose that the Wnt/β‐catenin pathway plays a more complex role in CLL pathogenesis than previously anticipated.

Exostosis of the optic canal in a child – a rare diagnosis in a paediatric ear, nose and throat setting: a case report

IntroductionOptic nerve compression is an uncommon disorder leading to deterioration or complete loss of vision.Case presentationWe describe the case of a 14-year-old Caucasian girl with a gradual deterioration of vision in her right eye. Using modern imaging techniques and endonasal endoscopic surgery, we identified the cause and removed the pathology.ConclusionsThe cause of optic nerve compression was a rare exostosis in the optic canal. An endonasal endoscopic approach is the optimal choice for management of optic nerve pathologies. It is a gentle and minimally invasive surgical technique. Interdisciplinary cooperation, especially with the eye doctor and radiologist, is required in these cases.

Post-myringotomy oto-liquorrhea in children - a case study and literature review

Myringotomy is a surgical incision of tympanic membrane used mainly as therapy for acute otitis media. It is a safe and simple procedure; however possible complications must be considered. In this article is described a case study of a 2-and-a-half-year-old girl with a massive oto-liquorrhea following myringotomy.

Histological analysis of retraction pocket pars tensa of tympanic membrane in children

AIMS Histological and histochemical analysis of retraction pocket of pars tensa of tympanic membrane in children. Identification of morphological abnormalities in comparison with a healthy tympanic membrane as it is described in standard textbook. Identification of signs typical for cholesteatoma and support for a retraction theory of cholesteatoma formation. STUDY DESIGN A prospective study analysing 31 samples of retraction pockets taken during surgery. DEPARTMENTS University Hospital, Childrens Medical Centre METHODS Samples of retraction pockets were processed by a standard process for light microscopy, stained by haematoxylin-eosin. Van Giesons stain was used for differential staining of collagen, Verhoeffs stain for elastic fibre tissues, Alcian blue for acidic polysaccharides and PAS (Periodic Acid Schiff) method for basement membrane polysaccharides. RESULTS The following findings were observed in the samples of retraction pockets: hyperkeratosis (100%), hypervascularisations (100%), subepithelial fragmented elastic fibres (96%), myxoid changes (87%), subepithelial inflammatory infiltration (84%), rete pegs (71%), papilomatosis (71%), intraepithelial inflammatory cellularizations, (48%), intraepithelial spongiosis (16%) and parakeratosis (3%). No basement membrane continuity interruptions were observed. Thickness of retraction pocket, thickness of epidermis, occurrence of rete pegs and frequency of fragmented elastic fibres was higher in a Grade III stage RP than Grade II stage RP (according to Charachon). CONCLUSION Morphological abnormalities in the structure of retraction pockets in comparison with a healthy tympanic membrane were described. The changes are typical for a structure of cholesteatoma (these changes are common in matrix and perimatrix), supporting retraction theory of its origin. Our observations show that it is inflammation that probably plays a key role in the pathogenesis of retraction pocket. The frequency of some of the changes increases with the stage of retraction pocket (II-III according to Charachon). Basement membrane continuity interruptions are not typical for retraction pockets.