Eye-movement disorders and visual-perceptual impairment in diplegia children born preterm: a clinical evaluation

The visual-motor behaviour of 15 preterm diplegic children and 50 control children (age range 4 to 7 years) was recorded on video as they performed a visual-perceptual task (an adaptation of the Animal House subtest of the Wechsler Preschool Primary Scale of Intelligence). The following parameters were analysed and scored: time to perform task; omissions; figure-colour association; sequence direction; sequential scanning order; accuracy of fitting target; and number of anticipatory saccadic movements to next target. The ability of the control children to perform the task improved significantly with age, as measured by performance time, mistakes in sequence direction and scanning order, accuracy of target fitting, and number of anticipatory saccadic movements. The scores of children with diplegia were not related to age and were poorer overall than those of the control group. Children with diplegia made significantly more mistakes of sequence direction and scanning order, and significantly fewer anticipatory saccadic movements than the control group. These results indicate that visual-perceptual impairment in diplegic children born preterm is not attributable only to sensory visual loss and to fine manipulation difficulties but is also related to difficulties in eye movements and in using anticipatory control to process information.     

Therapeutic Approach To Primary Malignant Epithelial Tumors of the Liver in Childhood: Results of the Italian Retrospective Study and Literature Survey

We analyzed the clinical features and treatment of 23 hepatoblastomas (HPBs) and 16 hepatocellular carcinomas (HPCs) occurring in patients less than 19 years old, admitted to the Italian retrospective multicentric study, conducted between 1983 and 1985, on childhood malignant hepatic tumors. The median ages of the patients with HPB and HPC at diagnosis were 22.34 months and 96.23 months, respectively, with a male/female ratio of 0.7 and 1.7, respectively. Fourteen HPBs (61 %) and 5 HPCs (31%) achieved surgical complete remission (CR). Of these, 11 HPB and all 5 HPC are still in CR with a median follow-up of 36 months and 3.5 years, respectively. One HPB and 1 HPC became resectable after a primary course of cis-platinum alone in the case of HPB and used with VP-16 in the case of HPC. All of the 9 HPBs and 11 HPCs, who never achieved CR, died of disease at a median interval from diagnosis of 5 and 2 months, respectively. The published therapeutic approaches for these tumors were also reviewed.     

Effects of 2,4-dichlorophenoxyacetic acid butyl ester on chick liver

Fertilized hen eggs were externally treated with 3.1 mg 2,4-dichlorophenoxyacetic acid butyl ester (2,4-D ester) before starting incubation. Liver lipid composition, (¹⁴C)2,4-dichlorophenoxyacetic acid (¹⁴C-2,4-D) subcellular distribution and covalent binding to proteins and lipids of chicks hatched from these eggs were studied. Furthermore, catalase, glutathione S-transferase and glucose 6-phosphatase activities as well as reduced glutathione content were determined. The herbicide produced a significant decrease on the total lipids, specially on phospholipids, both in total liver and microsomes. (¹⁴C-2,4-D) subcellular distribution in liver demonstrated that the herbicide passed through the plasmatic membrane and it was present in all the studied fractions. Herbicide bound covalently to hepatic proteins and lipids, being the binding to proteins tenfold higher than to lipids. Because these results might be of potential relevance to the understanding of the 2,4-D toxicity, further studies on the mechanisms of these reactions are necessary. On the other hand, the microsomal and cytosolic glutathione S-transferases activities remained unchanged, even though in vitro studies 2,4-D ester caused a decrease of the enzyme activities with an I₅₀ value of 0.2 mM. No significant change in reduced glutathione content between control and treated livers was observed. The catalase activity increased two-fold whereas glucose 6-phosphatase activity decreased 46% with respect to the corresponding control values. These results indicate that 2,4-D ester may have effects on the metabolism of xenobiotics.     

Evolution of renal replacement therapy in Central and Eastern Europe 7 years after political and economical liberation

Purpose of the study: The conditions of renal replacement therapy (RRT) were very poor in the countries located in Central and Eastern Europe (CEE) when they were members of the so-called 'socialist bloc'. The aim of the present analysis was to document the impact of the socioeconomic changes on dialysis therapy in the CEE countries. Design: This was a special survey with the participation of 12 CEE countries, with data obtained through national registries (with the exception of Russia). Results: during the period 1990-1996 the number of haemodialysis units increased by 56% and the number of centres performing peritoneal dialysis by 296%. The number of patients increased respectively by 78% (haemodialysis) and 306% (peritoneal dialysis). The percentage of patients with diabetic nephropathy and elderly patients rose dramatically during this period. One of the main reasons of such expansion was the rapid development of peritoneal dialysis programmes in the majority of the CEE countries. The introduction of modern haemodialysis machines and a wider choice of different dialysers and concentrates permitted individualization of dialysis procedures. These points and the wider use of erythropoietin had a positive influence on quality of life and treatment outcome. There was also a notable increase in the number of transplant centres, but less so of the number of transplanted patients. Conclusion: Renal replacement therapy experienced a major expansion in the CEE countries. Despite progress achieved, the level of RRT is not yet completely satisfactory in most CEE countries.     

The cognitive development of children born preterm and affected by spastic diplegia

In a prospective study the intellectual development of 20 premature children affected by spastic diplegia was compared with that of 10 preterm lowrisk children. The assessment was carried out with the Griffiths scale at the age of 3 years and with the WPPSI scale at the age of 6 years. The analysis of data collected in the 2 psychometric evaluation of the preterm-born diplegic children showed a disharmonic profile of neuropsychological functions, already present at the age of 3 years and confirmed at the age of 6 years. The average scores in diplegic children were poorest in the subscales locomotor, eye-hand coordination, and performance on Griffiths scale, and in the performance subtests of the WPPSI scale. The mean scores of subscales for hearing and speech, and practical reasoning on the Griffiths scale and of the verbal subscale of the WPPSI were near to the lower range of the normal distribution. Significant differences in performance subtests were found between the groups of preterm diplegic children and the group of low-risk preterm children, both at 3 and 6 years of age.     

Results of the Susanna implant in patients with refractory primary congenital glaucoma

PURPOSE: To evaluate the results of the Susanna implant in children with primary congenital glaucoma. PATIENTS AND METHODS: The authors report a retrospective noncomparative interventional case series of data from 24 eyes from 24 pediatric patients who underwent Susanna implant surgery for primary congenital glaucoma. All the patients were operated by the same surgeon. The postoperative follow-up time was 24 months for all patients. Success was defined by an IOP higher than 6 mm Hg and less than or equal to 15 mm Hg, with the concomitant use of a single medication if necessary. RESULTS: The age range of the patients was 1 to 15 years and the mean age was 5.04 +/- 4.12 years. At 12 months, 87.5% of the patients were considered as success, and at 24 months, the proportion dropped to 79.16%. Mean preoperative and postoperative intraocular pressure were 26.17 +/- 5.16 and 14.04 +/- 4.19 mm Hg ( p < 0.001), respectively. All 24 children had undergone previous glaucoma surgeries. Cataract was the only complication found during follow-up, occurring in three patients. Cox's regression model did not show influence of gender ( P = 0.740) and previous surgery ( P = 0.262) on the outcome through time. Age at the time of surgery was the only variable which was statistically associated with success through time, younger patients presenting higher probability of failure ( P = 0.05). CONCLUSIONS: The results of the present study reinforce the role of drainage implants as a safe alternative in difficult cases of primary congenital glaucoma, after failure of the initial approach.     

Unusual segregation of t(11;22) resulting from crossing-over followed by 3:1 disjunction at meiosis I

Reciprocal translocation t(11;22)(q23;q11) is of particular interest because the unbalanced offspring of the translocation carriers usually present with a supernumerary derivative chromosome 22. This common unbalanced karyotype is the result of 3:1 chromosome segregation during meiosis. We report the third case of a rare segregation pattern of a paternal 11; 22 translocation. The proband's karyotype revealed the presence of a der(11) and two copies of a der(22), i.e. 47, XX, t(11; 22)(q23;q11), +der(22) t(11;22)pat. The karyotype is the result of paternal 3:1 segregation after crossing-over involving the derived and the normal chromosome 22, as revealed by chromosome polymorphism analysis. Contrary to the preferential maternal transmission of this common unbalanced translocation, the data from the literature, including our case, may suggest preferential paternal transmission of this rare type of unbalanced translocation.     

Angiotensin II stimulates alpha3(IV) collagen production in mouse podocytes via TGF-beta and VEGF signalling: implications for diabetic glomerulopathy.

BACKGROUND: The podocyte is bathed in an angiotensin II (AngII)-rich ultrafiltrate, but the impact of AngII on podocyte pathobiology is not well known. Because podocytes play a direct role in the glomerular basement membrane (GBM) thickening of diabetes, the alpha3(IV) collagen chain was examined. Podocyte expression of alpha3(IV) collagen may involve the transforming growth factor-beta (TGF-beta) and vascular endothelial growth factor (VEGF) systems. METHODS: Cultured mouse podocytes were treated with various doses of AngII for selected periods of time, with or without inhibitors of TGF-beta and VEGF signalling, SB-431542 and SU5416, respectively. TGF-beta1 and VEGF were assayed by enzyme-linked immunosorbent assay (ELISA); alpha3(IV) collagen, TGF-beta type II receptor and phospho-Smad2 were assayed by immunoblotting. RESULTS: AngII >or=10(-10) M was found to stimulate the production of alpha3(IV) collagen significantly in as short a time as 3 h. The expression of alpha3(IV) collagen was influenced by the TGF-beta system, but AngII did not increase the podocyte's production of TGF-beta1 ligand; rather, it increased the expression of the TGF-beta type II receptor and activated the TGF-beta signalling system through Smad2. Despite the TGF-beta receptor upregulation, synergy between AngII and TGF-beta1 to boost alpha3(IV) collagen production was not observed. However, blockade of TGF-beta signalling with SB-431542 prevented AngII from stimulating alpha3(IV) collagen production. Podocyte expression of alpha3(IV) collagen was also increased by the autocrine activity of VEGF. Podocytes were stimulated to secrete VEGF by 10(-10) M or higher AngII after 48 h. Blockade of the endogenous VEGF activity by SU5416 prevented AngII-stimulated alpha3(IV) collagen production. CONCLUSIONS: AngII stimulates the podocyte to produce alpha3(IV) collagen protein via mechanisms involving TGF-beta and VEGF signalling. Alterations in alpha3(IV) collagen production may contribute to GBM thickening and perhaps proteinuria in diabetes.     

Noninvasive cortical stimulation with transcranial direct current stimulation in Parkinson's disease.

Electrical stimulation of deep brain structures, such as globus pallidus and subthalamic nucleus, is widely accepted as a therapeutic tool for patients with Parkinson's disease (PD). Cortical stimulation either with epidural implanted electrodes or repetitive transcranial magnetic stimulation can be associated with motor function enhancement in PD. We aimed to study the effects of another noninvasive technique of cortical brain stimulation, transcranial direct current stimulation (tDCS), on motor function and motor-evoked potential (MEP) characteristics of PD patients. We tested tDCS using different electrode montages [anodal stimulation of primary motor cortex (M1), cathodal stimulation of M1, anodal stimulation of dorsolateral prefrontal cortex (DLPFC), and sham-stimulation] and evaluated the effects on motor function--as indexed by Unified Parkinson's Disease Rating Scale (UPDRS), simple reaction time (sRT) and Purdue Pegboard test--and on corticospinal motor excitability (MEP characteristics). All experiments were performed in a double-blinded manner. Anodal stimulation of M1 was associated with a significant improvement of motor function compared to sham-stimulation in the UPDRS (P < 0.001) and sRT (P = 0.019). This effect was not observed for cathodal stimulation of M1 or anodal stimulation of DLPFC. Furthermore, whereas anodal stimulation of M1 significantly increased MEP amplitude and area, cathodal stimulation of M1 significantly decreased them. There was a trend toward a significant correlation between motor function improvement after M1 anodal-tDCS and MEP area increase. These results confirm and extend the notion that cortical brain stimulation might improve motor function in patients with PD.