Renal growth and function 11–28 years after treatment of Wilms' tumour

In order to obtain more information on the long-term effects of treatment of Wilm's tumour we investigated 30 subjects treated at the Children's Hospital between 1960 and 1976. All had been nephrectomized and in 4 the length of the remaining kidney was subnormal. In the other subjects kidney length was related to follow up time and age at follow up. Blood pressure was elevated in 5 subjects. Urinary albumin excretion deviated only slightly from normal. Tubular functions were well preserved in all subjects. In this small series we were unable to establish any relation between the abnormalities observed and the treatment given. Our results suggest that, despite wide interindividual variation those who survive Wilm's tumours seldom have long-term renal complications.     

Coordination dynamics in Parkinson's disease patients and healthy subjects quantified by the coordination dynamics recording method and sEMG

Coordination dynamics were measured in Parkinson's disease patients to quantify central nervous system (CNS) dysfunction. The low-load coordination dynamics in the patients were impaired by 56% for forward and 44% for backward moving in comparison to a control group of similar age. Exercising at higher load was only partly possible. When the disease preferentially affected one side of the body, the coordination dynamics were worse for the affected side. A dexterity test showed that coordination of hand and arm movements could be improved in the short-term memory when exercising on the special coordination dynamics recording and therapy device. Simultaneously taken surface EMG (sEMG) showed that the motor pattern was impaired in the Parkinson's disease patients. sEMG recordings showed further that the fast fatigable muscle fibre activation was impaired. FF-type muscle fibres were already activated for low load in one and not at all in another muscle. In conclusion, coordination between motoneuron firings and between arm and leg movements were found to be impaired in Parkinson's disease patients.     

Integrative re-organization mechanism for reducing tremor in Parkinson's disease patients

A special movement therapy, called coordination dynamics therapy, has been reported to have the potential to improve central nervous system (CNS) functioning in Parkinson's disease patients. Electromyography using surface electrodes (sEMG) showed that the rhythmic muscle activity leading to Parkinsonian tremor was generated in the patients by the impairment of two kinds of inhibition. First, some premotor spinal oscillators organized themselves in the CNS neuronal networks without strong adequate input and second, the oscillators synchronized their firing to give rise to rhythmic muscle activity and tremor. In this paper it will be shown that highly coordinated arm and leg movements, generated when exercising on a special coordination dynamics therapy device, can reduce Parkinsonian tremor in amplitude and frequency and improve CNS functioning in the short-term memory. sEMG measurements showed upon exercising on the special coordination dynamics therapy device that the motor program improved in the short-term memory and tremor muscle activity became coordinated with the volitional motor program and reduced in size and frequency. Higher load exercising seemed to better reduce tremor muscle activity, probably because the physiologic CNS organization was more integrative then and could 'bind' stronger simultaneous pathologic tremor activity. Moreover, the rhythmic synchronized motor unit firing in different arm and leg muscles was synchronized or coordinated and changed in frequency and amplitude. It is concluded that the integrative re-organization mechanism to reduce Parkinsonian tremor is the phase and frequency coordination between neuron firing of the physiologic neuronal network state, generated by the highly coordinated arm and leg movements, and the simultaneous pathologic tremor network state, generated by the uninhibited neurons, firing synchronized oscillatory.     

Distribution and mRNA expression of tenascin-C in developing human lung

Tenascin-C is an extracellular matrix glycoprotein that is spatially expressed during organogenesis, in inflammatory and fibrotic disorders, and in neoplasms. The aim of this study was to analyze its expression in developing human lung tissues during pseudoglandular, canalicular, saccular, and alveolar periods corresponding to Weeks 12 to 40. Lung tissues were obtained at autopsy from 34 nonmalformed cases. An immunohistochemical analysis and a messenger RNA (mRNA) in situ hybridization method combined with light microscopy were used. The extent of tenascin-C immunoreactivity was scored as absent, low, moderate, or strong in and around different types of pulmonary cells. The immunohistochemical expression for tenascin-C was strong beneath the airway epithelium, especially at the sites of airway subdivision during Weeks 12 to 23, whereas its expression was moderate or weak underneath alveolar and bronchiolar epithelia between Weeks 24 and 40. The expression for tenascin-C was strong in the intima of veins, especially in the canalicular period, i.e., Weeks 17 to 28. A moderate or strong immunoreactivity for tenascin-C was also observed around chondrocytes in every case studied during all periods. The increased expression of tenascin-C mRNA was most often seen in the cells below the airway epithelium. Taken together, tenascin-C is expressed in human lung during all developmental periods, and its expression is especially strong below the airway epithelium at the sites of airway subdivision.     

A retrospective study of long-term psychosocial consequences and satisfaction after carrier testing in childhood in an autosomal recessive disease: aspartylglucosaminuria

Genetic carrier testing of children is usually not recommended. However, there are no data concerning long-term psychological consequences, experience, and satisfaction of those tested as well as their recall of the test results. We evaluated these items retrospectively 10–24 years after carrier testing performed in childhood. Study material comprised 25 families with aspatylglucosaminuria (AGU), an autosomal recessive disorder, with 35 healthy sibs from all parts of Finland tested for carriership during childhood between 1973 and 1987. Of these sibs, 25 participated in our study. The questionnaire comprised multiple-choice and open-ended questions. The psychosocial well-being of the study subjects measured by the RAND 36 item Health Survey 1.0 (RAND) was, in general, at least as good as that of controls, and showed no significant differences between carriers and non-carriers (p>0.154). All tested individuals were satisfied with the fact that they had been tested and stated that the decision to perform carrier testing on a child can be made by the parents. Of the 25 tested, 23 knew and understood their test result correctly at the time of our study. Most of the tested individuals (60%) stated that the best time for carrier testing would be in the childhood or in the teen years.
This study indicates that carrier testing in childhood for an autosomal recessive disorder (AGU) had caused no measurable disturbance of quality of life in adulthood, and those tested reported being satisfied. However, we do not recommend testing in childhood, as the result is not needed prior to the time for reproductive decisions.