Changes in body weight and height in survivors of Menkes disease

ObjectiveTo explore the changes in the body weight and height of Menkes disease (MNK) patients treated with long-term copper–histidine.MethodsA survey involving a retrospective review of medical records or summaries of MNK patients was conducted. Patients were 44 males born after 1994, and their feeding method and genetic analysis of the ATP7A gene were reviewed. We compared the data of body weight and height from birth until 6 years between classical MNK patients and the general population obtained from national data and between patients who received early treatment and patients who received late treatment.ResultsAlthough five patients who received early treatment reached some developmental milestones, the body weight and height did not differ from patients who received late treatment in the mode of oral nutrition, and were lower in comparison to the national data (<3 percentile).ConclusionWe reported changes in the body weight and height of MNK patients who received early and late treatment. Although early treatment with copper–histidine had favorable effects on neurological development, it did not result in improvements in body weight and height. We suggest that the establishment of sufficient nutritional support is necessary along with early parenteral copper treatment to improve whole body condition in MNK patients.     

Variations in the tree-ring structure of Norway spruce (Picea abies) under contrasting climates

Variations in tree-ring structure from pith to bark of mature Norway spruce (Picea abies Karst. (L)) grown under contrasting climate conditions (warm-dry vs. cool-humid) in south-western Germany were investigated. Sample trees were from even-aged stands where no intensive silvicultural treatments had taken place. The cell number (CN), diameter (CD), lumen diameter (CL) and wall thickness (CW) were measured from stem cross-sections taken at breast height. A raw data chronology of each cell parameter was established for the whole annual ring (AR), earlywood (EW), latewood (LW), and transition wood (TW). The long-term trends of the cell parameters were generally non-linear, parameter-specific in AR and EW and similar over sites. Those in LW were site-specific and similar over cell parameters. The variation of the same parameter, expressed by the coefficient of variance (CV), tended to increase from EW towards LW and was clearly higher for CN than for other cell parameters of all tree-ring zones. Trees from the warm-dry site had more LW cells and substantially thicker CW, whereas those from the cool-humid site had larger EW cells. These observed differences are likely due to the hydraulic adaptation mechanisms of trees to different site conditions.     

On the changing concept of evolutionary population stability as a reflection of a changing point of view in the quantitative theory of evolution

 Eighteen different terms, currently employed to define various concepts of evolutionary stability in population dynamics are mentioned in this paper. Most of these terms are used in different connotations and even different meanings by different authors. On the other hand, different terms are often employed by different authors to define quite the same concept. Twenty-five years ago there was only one, well-defined, concept of stability, universally recognized in the field. In this paper I will try to relate the recent confusion, concerning concepts of population stability, with a more serious, though not that well-recognized, confusion in the modern analytic approach to population dynamics and quantitative evolution. Concepts of population stability will be examined in relation to each other on the one hand and, on the other hand, in relation to two dichotomies regarding the dynamic processes to which they correspond: Short-term versus long-term processes and processes concerning phenotypic changes versus process concerning genotypic changes. A hopefully more consistent use of the current terminology is suggested. Received 15 August 1993; received in revised form 15 September 1994