CD1d expression on B-precursor acute lymphoblastic leukemia subsets with poor prognosis.

Acute lymphoblastic leukemia (ALL) is the most frequent malignancy of childhood. Although therapeutical advances have been achieved, some ALL subgroups still fare poorly. CD1d is a monomorphic molecule that provides a suitable target for immunotherapy in view of the characterization of a glycolipid, alpha-galactosylceramide (alpha-GalCer), capable of being presented to CD1d-restricted T cells with cytotoxic potential. We investigated CD1d expression in 80 pediatric B-cell precursor (BCP) ALL cases defined according to immunophenotype, cytogenetic features and age at onset. CD1d was detected on ALL cells in 15% of the patients. CD1d+ ALLs were significantly associated with infant leukemia, pro-B phenotype and mixed-lineage leukemia (MLL)/AF4 gene rearrangement. Accordingly, overall survival of patients with CD1d+ ALL was significantly shorter. CD1d+ leukemic blasts were able to present alpha-GalCer via CD1d to cytotoxic CD1d-restricted T cells, which induced apoptosis of ALL cells that was inhibited by mAb to CD1d. CD1d+ blasts loaded with alpha-GalCer elicited cytokine secretion by CD1d-restricted T cells. Analysis of bone marrow (BM) cells derived from normal donors revealed that CD19+/CD1d+ cells were mostly mature B lymphocytes. However, a minority of BCPs expressed CD1d. Thus, expression of CD1d in ALL cases heralds an adverse prognosis but may provide a therapeutic tool.

Neonatal lupus erythematosus.

Neonatal lupus erythematosus (NLE) is a rare disease characterized by the transplacental passage from the mother to the fetus of autoantibodies, in particular anti-Ro(SS-A), anti-La(SS-B), or both. The majority of infants with NLE exhibit isolated congenital heart block, cutaneous lesions analogous to those of adult subacute cutaneous lupus erythematosus, or both. We report a case of NLE in a 3-month-old male infant, born to a clinically asymptomatic mother, presenting small, annular, erythematous plaques with sharp, hyperkeratotic borders and central clearing localized at the eyebrow region. Both the infant and the mother were positive for anti-Ro(SS-A).

[Two-dimensional echotomography in the prevention of congenital hip dysplasia]. / L'ecotomografia bidimensionale nella profilassi della displasia congenita dell'anca.

Examination of the infant hip with real time ultrasonography is spreading and affirming in these last years. It allows accurate and reliable investigation of the anatomic structures which are not visualized on a radiograph, so that it appears of great interest in the early screening of congenital dislocation of the hip (CDH). In this paper, the authors examine and discuss some particular features of the sonograms which are usually employed for the investigation of neonatal hips, attempting to dissolve some doubts and perplexities which commonly come out in the first approach to a new diagnostic image technique. The systematic examination of infant hips with real time ultrasonography promises a decisive improvement in the problem of the early diagnosis of CDH.

Foot alterations in the hemodialyzed patient.

On the basis of their experience, the authors refer to complications observable at the foot level in chronic uremic patients in long-term hemodialytic treatment. These complications, termed renal osteodystrophy, are attributed to alterations in the phosphocalcium metabolism and to hyperparathyroidism. From an anatomopathologic viewpoint, they are characterized by alteration in bone tissue (osteomalacia and/or fibrous osteitis) and by soft tissue alterations. Alterations in the foot are the same as those observed in other osteoarticular regions. They consist of bone subperiosteal reabsorption of the phalanges, vascular calcifications, articular and para-articular calcifications, spontaneous disinsertion of the Achilles tendon and peripheral canalicular neuropathies specifically represented by the tarsal tunnel syndrome. Another complication of this pathology is represented by "Calciphylaxis," characterized by calcifications of the middle tunica of arteries and small arteries, by ulcerations and tissue necrosis at the foot level.

[Radiographic course of inverted osteochondral graft using the Regnauld technic in the surgical treatment of hallux valgus]. / Evoluzione radiografica dell'innesto osteocondrale "invertito" secondo Regnauld per il trattamento chirurgico dell'alluce valgo.

The osteocartilaginous bone graft, conceived by Regnauld for surgical treatment of hallux valgus, is to-day widely known and practised. We have preferably employed the so called "inverted graft", while Regnauld, in his recent monography, defines it less satisfactory than the "cork" or "hat" shaped grafts. Therefore, we have observed the radiological evolution of our inverted grafts, to evaluate the possible differences of their "rootage" in comparison to the cork grafts, studied by Valenti in 1976. Some possible causes of the infrequent failures of this grafting are also examined and discussed. We conclude that the osteocartilaginous graft evolution do not show radiological differences referable to the graft morphology and that unfavourable evolution, when is present, concern at first the articular (cartilaginous) side of the graft, being independent of failed rootage or aseptic necrosis of the bony side.

[Osteoid osteoma of the foot]. / L'osteoma osteoide del piede.

We relate about our direct clinical experience on 8 cases of osteoid osteoma localized in the bones of the foot. Some atypical appearances, clinical and roentgenographic, of the osteoid osteoma in this bony district may sometimes cause problems in diagnosis and localization, which are exposed and discussed in this paper. Particularly, we underline the importance of bone scan in the detection of the lesion.

[Neurogenic deformities of the foot due to congenital malformations of the lumbosacral spine. Their clinical and therapeutic characteristics]. / Le deformità neurogene del piede da malformazioni congenite del rachide lombo-sacrale. Peculiarità cliniche e terapeutiche.

The authors illustrate the different types of nervous disorders of the foot caused by congenital deformations of the lumbosacral spine. They classify these clinical forms according to the level of the neurological lesions, although emphasizing that more complex neurological damage may cause very atypical deformities that are difficult to classify. Today the treatment of nervous disorders of the foot caused by congenital deformations of the lumbosacral spine seems almost promising when compared to neurological and urological problems which have therapeutic priority. Possible methods of surgical correction, as well as indications that differ according to the age of the patient and the type and extent of the deformity, are discussed. These deformities of the foot evolve very quickly; treatment must be timely or reduction is difficult. Furthermore, their peculiar tendency to relapse necessitates periodic comprehensive follow-up exams and regular maintenance therapy (orthetic and physiatric). Special care must be taken before and during treatment to prevent trophic skin lesions. For this reason, solutions other than casts (external fixation, for example) may be preferable for the maintenance of the correction. The treatment of these deformities, whether conservative or surgical, should aim primarily to achieve adequate plantar support for future aided or autonomous ambulation.

[Apert's syndrome: the clinico-radiographic picture]. / La sindrome di Apert: quadri clinico-radiografici.

Apert's syndrome is a rare malformation (dysostosis), probably of a hereditary nature. It is characterised by craniostenosis, caused by synostosis of the coronal sutures, and by bilateral and symmetrical membranous syndactyly of the hands and feet, to which metacarpal, metatarsal and phalangeal synostoses are associated. Less frequent are dysplastic modifications in other osteo-articular regions and malformations of the viscerae. The authors expose and discuss the etiopathogenetic, clinical and radiographic aspects of this syndrome, in relation to three clinical cases which came to their observation.