Treatment with deferiprone (L1) in a thalassemic patient with bone lesions due to desferrioxamine.

A male thalassemia major patient who developed bone and cartilage abnormalities with a standard dose of desferrioxamine (DFX) given subcutaneously from the age of 4 years was treated with the oral iron chelator deferiprone (L1). During L1 treatment an improvement of genu valgum, evidence of healing and filling in of bone at the periphery of knee metaphysis and improvement in growth velocity were observed. However, the sitting height had decreased further, confirming the irreversibility of platyspondylosis, so affecting the near final standing height (156.8 cm) which was below the mid-parental height (168 cm). Prospective studies are warranted to determine the effect of different iron chelators on the bone metabolism of patients with thalassemia.

[Long-term follow-up and bone imaging in acquired osteochondrodysplasia in a female patient with thalassemia and desferrioxamine hypersensitivity]. / Follow-up auxologico e imaging scheletrico nell'osteocondrodisplasia acquisita in una paziente talassemica con ipersensibilità alla desferrioxamina.

We report a female thalassaemia major patient who developed short stature and osteochondrodystrophic lesions during therapy with desferrioxamine (DFX). Healing was noted after the dose of DFX was decreased for 3 years. Unfortunately the spinal cartilage abnormalities did not change suggesting an irreversibility of the ossification process.

Phase diagram of (R)- and (S)-4-hydroxy-2-pyrrolidone mixtures: a new case of a conglomerate-forming system.

The phase diagram of (R)- and (S)-4-hydroxy-2-pyrrolidone presents a conglomerate in the racemic mixture. The enthalpy of melting extrapolated by the Schröder-van Laar-Le Chatelier equation [change in enthalpy (delta H) = 28410 J/mol; melting temperature (TA) = 429.9 K; solidus temperature (Ts) = 395.4 K] and the value determined by differential scanning calorimetry (delta H = 28494 J/mol; TA = 429.6 K; Ts = 394.6 K) are in excellent agreement. The experimental entropy of the mixing of enantiomers is 5.69 J/mol.K. The conglomerate nature of the racemic mixture was confirmed by X-ray and IR spectroscopy. The presence of solvates was also observed.

Interaction of bilirubin with small unilamellar vesicles of dipalmitoylphosphatidylcholine.

Interaction of bilirubin with phospholipid bilayers was studied at physiological pH above and below the gel-liquid crystalline phase transition of small unilamellar vesicles of dipalmitoylphosphatidylcholine. Chromatographic, calorimetric and 1H-NMR evidences strongly suggest that dianion form of bilirubin binds to the polar heads of the phosphatidylcholines protruding from the outer leaflet of the vesicles, whilst acid bilirubin, which is insoluble in water, is hydrophobically inserted into the lipophilic region of the bilayers. The surface-bound bilirubin is promptly removed from vesicles, whilst the acid form hydrophobically inserted into the vesicles is firmly bound to the membrane in the gel state. This pool of bilirubin could perturb the chemico-physical properties of the membrane (i.e., fluidity, phase transition, etc. ...) thus contributing to perturbation of the biological properties of living cells.

Thermotropic behavior of dipalmitoylphosphatidylcholine-sulfatide liposomes.

The thermotropic behavior of multilamellar liposomes prepared from mixtures of sulfatide and dipalmitoylphosphatidylcholine has been studied by DTA calorimetry. The cooperative unit size and the enthalpy change of the main transition decrease concomitant with the increase of the sulfatide-phospholipid mole ratio. The origin of these effects and their dependence on the sulfatide content suggest that the in-plane distribution of sulfatide and the physical state of the lipid bilayer are affected by the sulfatide-phospholipid mole ratio.