The paper presents, for the first time, corneal buckling, during the air puff applanation, recorded with use of Ocular Response Analyzer (ORA), when the cornea is deeper deformed after its applanation. Precise numerical analysis of the air pressure curve from the raw data, distinct local disturbances of the curve, which appear almost exactly at the time of the first and the second applanations. Thirty measurements taken on six eyes show clear dependencies between times of both applanations and appearances of local wave disturbances on the air pressure curve as well as between the amplitude of pressure wave disturbances and the respective height of applanation curve. These findings can be interpreted as a result of very fast corneal buckling, that produces the air pressure wave, propagating from the cornea towards the device. The quantitative dependencies measured and described in this study, enable to characterize the individual buckling during respective applanations. Due to these individual characterizations and dependencies it is possible to understand and describe better the ultrafast corneal applanation process. Such phenomena could likely be employed to increase the accuracy of measured parameters by ORA or for identifying new types of biomechanical properties of the cornea.

Nijmegen breakage syndrome (NBS) is a rare disease with an autosomal recessive pattern of inheritance caused by mutations in the NBN gene. We report 2 patients with NBS and T-cell lymphoblastic lymphoma (T-LBL), in whom diagnosis and therapy were difficult challenges. Both patients were diagnosed with NBS by mutation analysis of the NBN gene, which revealed homozygosity for a typical 5 base pair deletion (657del5). The lymph node biopsy revealed T-LBL, and both patients were treated according to EURO-LB 02. Complete remission was achieved in the first patient. In the second case, bone marrow relapse was observed, and the patient died due to disease progression. In conclusion, patients with NBS should be closely monitored because of a higher frequency of lymphoma than in the general population. The described cases indicate the importance of identifying predictive markers of cancers and developing treatment regimens for patients with NBS and malignancies.

sclerosis in a child with neurofibromatosis type Iclinical management of a challenging case. Ann Agric Environ Med.

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