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The Institute of Haematology and Blood Transfusion was designated a Centre for Rare Disorders of Haematopoiesis in 2014. Most of the patients who are currently treated in the centre suffer from rare congenital or acquired disorders of erythropoiesis. This article reviews patients with congenital disorders of hematopoietic stem cells, red blood cell (RBC) membrane defects, enzymopathies and hemoglobinopathies who have been diagnosed and treated in the centre. The centre also follows patients with paroxysmal nocturnal haemoglobinuria. The increasing number of patients with congenital disorders of erythropoiesis is due to greater implementation of diagnostic molecular genetic methods, the prolonged survival of patients as well as increasing migration from regions with a high incidence of congenital RBC disorders. Diagnosis and long term follow up of patients with rare disorders of erythropoiesis are warranted because of the high incidence of tumours and complications resulting from organ iron overload in adulthood. Moreover, diagnosis of heterozygous forms in parents may preclude the birth of a homozygote with a serious form of the disease. [ABSTRACT FROM AUTHOR]

Copyright of Transfusiology & Haematology Today / Transfuze a Hematologie Dnes is the property of Czech Medical Association of JE Purkyne and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Transfusiology & Haematology Today / Transfuze a Hematologie Dnes is the property of Czech Medical Association of JE Purkyne and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

This study describes the amount and the spatial distribution of the above- and below-ground tree skeleton-defined as the woody structure of stem, branches and roots-in a mature Scots pine ( Pinus sylvestris L.) stand in Belgium. Tree skeleton data were linked to the respective needle area, and as such, this work provides the background framework for modeling the tree hydraulic architecture and the carbon balance of the forest stand. Using validated allometric equations, we were able to calculate the amount of the volume, of the biomass and of the corresponding surface areas of individual trees in the stand. Total woody biomass of the 66-year-old forest stand was 155 Mg ha, i.e., 126 Mg ha above ground and 29 Mg ha below ground. The total bio-volume of the woody mass of the stand was 314 m ha. The highest fraction of this value was the stem bio-volume, i.e., 236 m ha or 75 % of the total. The total volume of all roots was 57 m ha (18 % of the total volume), and the volume of branches was 20 m ha (7 % of the total volume). The surface area of the roots ranged from 38,000 m ha in the winter to 68,000 m ha in the spring. The surface area of the stems was 2,700 m ha, and the surface area of all branches reached 4,400 m ha. The total above-ground water storage in the xylem was 94 m ha (or 9.4 mm), while the accessible stored water was 2 mm of that quantity. A comparative analysis of the biometric parameters showed the balance between the different functionally connected, operational surface areas of the trees. The needle surface area was similar to the root surface area and in the same order of magnitude as the surface area of woody cambium. The results allow to link water uptake with transpiration and assimilation with respiration. [ABSTRACT FROM AUTHOR]