ABSTRACT
BACKGROUND: Normal-hearing children show signs of various phonological processes during language development. These processes represent simplifications of articulation, which are overcome at different time points. For the German language, there are currently no reliable data regarding whether these developmental stages also apply to deaf children with cochlear implants (CI). MATERIALS AND METHODS: Phonological development in deaf children with CI was examined and evaluated with the PLAKSS ("Psycholinguistischen Analyse kindlicher Sprechstörungen"). The results of this analysis (time of test 1 = T1, n = 33) were compared to those of a PLAKSS diagnostic evaluation performed 1 year previously (time of test 0 = T0, n = 31). RESULTS: At T1, 76 % of the whole group showed a phonological development that did not correspond to their hearing age (as measured from the time of the first CI implantation). The most frequently observed phonological processes were the reduction of consonant clusters and fronting. However, 83 % of the group had fewer phonological processes inappropriate to their hearing age at T1 than they did at T0. CONCLUSION: The phonological development of children with CI is not equivalent to their hearing age and is structured differently to that of normal-hearing children.
Subject(s)
Aging , Child Development , Cochlear Implants , Deafness/physiopathology , Deafness/rehabilitation , Language Development , Child , Child, Preschool , Deafness/diagnosis , Female , Humans , Male , Treatment OutcomeABSTRACT
Neurofibromatosis type 1 (NF1) is one of the most common inherited disorders in humans. Most of the NF1 gene mutations result in a reduction of the amount of neurofibromin to about 50%. Recently, we found that the level of neurofibromin can be regulated post-translationally through the alteration of its half-life. Here, we investigated whether lysosomes are involved in this post-translational regulation in cultured melanocytes of NF1 patients and controls. When the lysosomal degradation was inhibited by chloroquine, an increase of neurofibromin by a factor of 2 to 3, correlating with an increased half-life, was measured. Incubation with phosphoprotein-phosphatase inhibitors also increased the neurofibromin content in melanocytes. Investigations on phosphorylation of neurofibromin revealed a basal phosphorylation in melanocytes cultured with growth factor-deprived medium that increased upon incubation with the growth stimulators PMA or bFGF. Because both factors are also able to increase the half-life of neurofibromin, we suggest its phosphorylation to be an important step in protecting neurofibromin against specific lysosomal degradation.
Subject(s)
Lysosomes/metabolism , Melanocytes/metabolism , Proteins/metabolism , Animals , Cells, Cultured , Chloroquine/pharmacology , Enzyme Inhibitors/pharmacology , Half-Life , Humans , Melanocytes/drug effects , Molecular Sequence Data , Neurofibromin 1 , Okadaic Acid/pharmacology , Phosphorylation , Rats , Tetradecanoylphorbol Acetate/pharmacologyABSTRACT
A case of acute nonlymphocytic leukemia (ANLL) occurring 2 years after the diagnosis of multiple myeloma (MM) that had been treated by only one course of melphalan/prednisone chemotherapy is reported. Cytogenetic and fluorescence in situ hybridization analysis of peripheral blood cells revealed trisomy 8 as the sole cytogenetic defect at the time of diagnosis of ANLL. Two years earlier, when MM was diagnosed without any cytological evidence of co-existent myelodysplasia, chromosomal analysis of bone marrow cells showed the same pathological karyotype 47, XY, +8 in 14 of 20 mitoses studied. Our interpretation of this unusual cytogenetic finding is that at the time of diagnosis of MM, in spite of lacking cytological signs of myelodysplasia, an unrecognizable myelodysplastic syndrome must have been present which then evolved to ANLL.
Subject(s)
Chromosomes, Human, Pair 8 , Leukemia, Myeloid, Acute/genetics , Multiple Myeloma/genetics , Neoplasms, Second Primary/genetics , Paraproteinemias/genetics , Trisomy , Aged , Aged, 80 and over , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Bone Marrow/pathology , Disease Progression , Fatal Outcome , Follow-Up Studies , Humans , In Situ Hybridization, Fluorescence , Leukemia, Myeloid, Acute/pathology , Male , Melphalan/administration & dosage , Multiple Myeloma/drug therapy , Multiple Myeloma/pathology , Myelodysplastic Syndromes/diagnosis , Myelodysplastic Syndromes/genetics , Myelodysplastic Syndromes/pathology , Neoplasms, Second Primary/pathology , Paraproteinemias/pathology , Prednisone/administration & dosageABSTRACT
The number of electrodes employed, the frequency of reapplication, the technical quality of monitoring and the complications of use of spiral electrodes and Copeland electrodes for cardiotocographic monitoring of deliveries are assessed in a prospective randomized investigation. The number of electrodes employed and the frequency of reapplications were significantly lower employing Copeland electrodes. Similarly, the electrode signal was significantly better as assessed by the percentage of the duration of monitoring in which the cardiotocogram did not register during the second stage of labour on account of poor electrode signals. No differences were found in the frequencies of complications or subjective discomfort in the mother on employing the two types of electrodes.
