Nine patients out of more than 300 with severe Graves' ophthalmopathy followed in this institution were submitted to orbital decompression carried out on 16 eyes by a three-wall procedure consisting of a transfrontal approach with the removal of the roof, the lateral wall and part of the floor of the orbit. All patients had been previously unsuccessfully treated by orbital radiotherapy and/or systemic corticosteroid administration. The main indications for surgery were marked proptosis or sight-threatening optic neuropathy. Results of treatment were evaluated on clinical grounds and by the variation of the ophthalmopathy index (OI). A significative reduction of proptosis was observed in all eyes, with a mean decrease in the Hertel reading of 3.2 mm, from 22.6 +/- 1.8 mm to 19.4 +/- 1.4 mm (p less than 0.001). A complete regression or improvement of inflammatory signs, corneal lesion and, with one exception, extraocular muscle dysfunction was obtained in all cases. Loss of visual acuity and other manifestations of optic neuropathy were present in 11 out of 16 eyes before surgery. A complete restoration or a marked improvement of optic neuropathy was obtained in 7 cases: failure occurred in the two patients (4 eyes) with longstanding sight loss. The OI decreased in all patients after surgery, from a mean pretreatment value of 8.6 to a mean posttreatment value of 3.8 (p less than 0.001). The clinical response to surgery was excellent in 4 cases, good in 3 and slight in 1; no changes were observed in the remaining patient. Bacterial meningitis which resolved with no sequelae occurred in one patient.(ABSTRACT TRUNCATED AT 250 WORDS)
Corticosteroid-binding globulin (CBG) belongs to the superfamily of serine proteinase inhibitors which include alpha 1-antitrypsin, alpha 1-antichymotrypsin, and T4-binding globulin. Interleukin-6 (IL-6), the main mediator of the acute phase phenomenon, increases alpha 1-antitrypsin and alpha 1-antichymotrypsin synthesis and decreases T4-binding globulin synthesis by human hepatoblastoma-derived (Hep G2) cells. This effect is predominantly at a transcriptional level. When Hep G2 cells were exposed to different concentrations of IL-6 for variable time intervals, IL-6 caused a dose- and time-dependent decrease in the amount of [35S]methionine-labeled CBG immunoprecipitated in the culture medium. This effect could be greatly reduced by preincubation of IL-6 with its neutralizing antibody and reversed by removing the cytokine from the culture medium. The secretion rate of CBG was not affected by cell exposure to IL-6. CBG mRNA steady state levels were reduced; changes in mRNA were quantitatively similar to changes in secreted protein. Nuclear run-off assays failed to show a change in the rate of transcription of the CBG gene. These data indicate that IL-6 diminishes CBG synthesis by Hep G2 cells acting at a posttranscriptional level, presumably through a reduced stability of mRNA. In view of the role of IL-6 in the inflammatory process and other acute phase phenomena, these data suggest that its effects on CBG synthesis might influence the bioavailability of cortisol indirectly and play a role in regulating the homeostatic process during these conditions.
The aim of the present study was to correlate laboratory data and postprocedural parameters after conventional transarterial chemoembolization (cTACE) for hepatocellular carcinoma (HCC) with the radiological response. The study consisted of a retrospective analysis of prospectively collected data from 70 consecutive patients who underwent cTACE. Laboratory parameters were assessed daily after cTACE and compared to pretreatment values. Post-treatment radiological response was assessed using mRECIST at one month from cTACE, and factors associated with treatment response (complete and objective response) were assessed by logistic regression analysis. The optimal cutoff points in predicting the complete response of target lesions were a 52% ALT and a 46% AST increase after cTACE compared to the pre-treatment values. Using multivariate analyses, >46% AST and >52% ALT increases with respect to the pre-treatment value were significantly correlated with the objective response (p = 0.03 and p = 0.04, respectively) and the complete response (p = 0.02 and p = 0.02, respectively). No patients experienced liver function deterioration after cTACE, and no specific treatment was required. This study showed that post-treatment transient transaminase elevation was predictive of objective response to superselective cTACE in clinical practice, representing a simple tool to guide treatment strategy of HCC patients in a tailored approach.
CareToy system is an innovative tele-rehabilitative tool, useful in providing intensive, individualized, home-based, family-centred Early Intervention (EI) in infants. Our aim was to evaluate, through a Randomized Clinical Trial (RCT) study, the effects of CareToy intervention on early motor and visual development in preterm infants. 41 preterm infants (range age: 3.0–5.9 months of corrected age) were enrolled and randomized into two groups, CareToy and Standard Care. 19 infants randomized in CareToy group performed a 4-week CareToy program, while 22 allocated to control group completed 4 weeks of Standard Care. Infant Motor Profile (IMP) was primary outcome measure, Alberta Infant Motor Scale (AIMS) and Teller Acuity Cards were secondary ones. Assessments were carried out at baseline (T0) and at the end of CareToy training or Standard Care period (T1). T1 was the primary endpoint. After RCT phase, 17 infants from control group carried out a 4-week CareToy program, while 18 infants from the CareToy group continued with Standard Care. At the end of this phase, infants were re-assessed at T2. In RCT phase, delta IMP total score and variation and performance sub-domains were significantly higher (P<0.050) in CareToy group if compared to Standard Care group. Similar results were found for Teller Acuity Cards, while no differences between groups were found for AIMS. No differences were found in any outcome measure results (T2-T0), between infants who started CareToy training before or after one month of standard care. This RCT study confirms the results of a previous pilot study, indicating that CareToy system can provide effective home-based EI. Trial Registration: This trial has been registered at www.clinicaltrials.gov (Identifier NCT01990183).
This report concerns a male newborn investigated with chromosomal analysis due to dysmorphic and malformative signs. His caryotype was 46,XY but the short arm of one 3 was not normal: parental chromosomes showed a maternal balanced translocation (3;6)(p25;q23) which had led to 6q23----6qter duplication in the patient. The phenotype was like the twenty-one cases found in the literature: it is characterized by facial dysmorphism (bow shaped mouth, high prominent forehead, protruding ocular bulbs, anteverted nostrils), short neck with pterigium, flexion contractures especially at extremities and neurological problems as well as internal malformations. Moreover, it seems not affected, or very little, by monosomies accompanying unbalanced translocations.
