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Notes: Summary An 11-year-old girl with mucopolysaccharidosis I Scheie phenotype (MPS I-S) received a bone marrow transplant (BMT) from her heterozygous HLA-identical LMC-non-reactive mother. Multidisciplinary studies were carried out and results evaluated 21 months after transplantation. Herein we report the ultrastructural findings pre-and post-BMT in skin. Multidisciplinary studies are commonly used to evaluate the benefits of metabolic correction following BMT in some MPS and other inherited metabolic disorders, and changes in morphology have been described in liver and few other tissues. In this case, we elected skin, since connective tissue is universally involved in MPS and is safely and easily obtainable. Comparison of skin biopsy specimens taken before and after BMT showed a considerable change in dermal fibroblast morphology, with marked reduction in cell size and the number and size of abnormal lysosomes, thus indicating the clearance of storage. Our results demonstrate that dermal cells respond to enzyme replacement therapy in MPS I-S, with the clearance of glycosaminoglycan lysosomal accumulation in connective tissue fibroblasts, which had near-normal morphology 21 months after BMT. Therefore, the practice of skin biopsy after BMT in MPS and other metabolic disorders in which dermal cells are involved should be encouraged.

Notes: Abstract We report a 4-year-old boy, in acute renal failure, who had acute encephalopathy with very high serum aluminium levels (135 μg/l [5μmol/l]) after receiving vesical irrigations with alum. We believe that in situations of non-focal neurological deterioration with no apparent cause in patients with impaired renal function receiving aluminium-containing preparations, the possibility of acute aluminium poisoning should be considered.

Notes: A cross-sectional study was performed to assess a series of parameters, especially those related to iron status, that may account for differences in the anaemia of patients treated with continuous ambulatory peritoneal dialysis (CAPD) and haemodialysis (HD). Patients on CAPD (n = 37) and on haemodialysis (n = 27) were selected on the basis of clinical stability and absence of factors that may interfere with iron metabolism. Parameters measured included routine haematological and biochemical profile; serum iron metabolism (serum iron, transferrin saturation capacity, transferrin saturation index, serum ferritin); erythrocyte ferritin; bone marrow erythroid components and semiqualitative analysis of iron deposits in the bone marrow. The CAPD patients showed higher levels of haemoglobin and transferrin saturation capacity as well as lower serum ferritin than patients on haemodialysis. In the bone marrow, CAPD patients had a higher percentage of red blood cells and in those with low iron-containing macrophages a higher percentage of sideroblasts than the haemodialysis group. Erythrocyte ferritin levels were similar in both groups. These data suggest more efficient iron metabolism in CAPD patients than in patients on haemodialysis.

Notes: Triazine-resistant (R) biotypes of Amaranthus albus L. (tumble pigweed), A. blitoides S. Wats. (prostrate pigweed), A. hybridus L. (smooth pigweed), A. retroflexus (redroot pigweed), and Solanum nigrum L. (black nightshade) were found in Spain. The former two R biotypes were found in simazine-treated, non-tilled olive orchards, whereas the latter three came from atrazine-treated maize (Zea mays L.) crops. All R biotypes survived at rates up to 5.0 kg ha−1 of soil-incorporated atrazine or simazine, whereas their susceptible (S) or ‘wild type’ counterparts were completely controlled at levels of 0.25 kg ha−1 atrazine, in the case of A. hybridus, A. retroflexus and S. nigrum, and 2.5 kg ha−1 in the case of A. albus and A. blitoides. The higher tolerance to triazines of these latter S biotypes appears to be attributable to herbicide detoxification. Photosynthetic electron transport in R biotypes was little affected by atrazine and simazine, but was inhibited by diuron, as shown by fluorescence induction measurements in whole leaves. In Hill reaction assays, the R biotypes showed a high level of resistance to atrazine and simazine (resistance factors in the range 350–900), and very slight resistance to diuron (resistance factors in the range 1.1–1.6). It is concluded that all R biotypes have evolved a chloroplastic mode of resistance similar to that previously described for other triazine-resistant weed biotypes. Résistances aux triazines des biotypes de Solanum nigrum et 4 espèces d'Amaranthus trousiées en EspagneDes biotypes résistant aux triazines (R) d'Amaranthus albus L., d'A. blitoïdes S. Wats, d'A. hybrydus et d'A. retroflexus et de Solanum nigrum L. ont été trouvés en Espagne. Les 2 premiers biotypes (R) ont été trouvés dans des oliveraies non labourées traitées à la simazine, tandis que les 3 derniers viennent de cultures de maïs traitées à l'atrazine. Tout les biotypes R ont survécu à des doses jusqu'à 5 kg ha−1 d'atrazine ou de simazine incorporée au sol tandis que leurs équivalents sensibles (S) ou ‘types sauvages’étaient complètement détruits à des doses de 0,25 kg ha−1 d'atrazine dans le cas d'A. hybridus, A. retroflexus et S. nigrum et de 2,5 kg ha−1 dans le cas d'A. albus et A. blitoïdes. La tolérance plus élevée aux triazines de ces 2 derniers biotypes apparaît être attribuable à la détoxification de l'herbicide. Le transport des d'électrons photosynthétiques dans les biotypes R a été peu affecté par l'atrazine et la simazine mais a été inhibé par le diuron, comme l'ont montré des mesures d'induction de fluorescence dans des feuilles entières. Dans des essais de réaction de Hill, les biotypes R ont exprimé une haute résistance à l'atrazine et à la simazine (facteurs de résistance de 350 à 900) et une très faible résistance du diuron (facteurs de résistance de 1,1 à 1,6). Il est conclu que tous les biotypes R ont développé un mode de résistance chloroplastiques identique à celui déjà décrit par d'autres biotypes d'adventices résistant aux triazines. Triazinresistenz bei Biotypen von Solanum nigrum und vier Amaranthus-Arten in SpanienTriazinresistente Biotypen (R) von Amaranthus albus L. (Weißer Amarant), A. blitoides S. Watson (Westamerikanischer A.), A. hybridus L. (Grünähriger A.), A. retroflexus L. (Zurückgekrümmter A.) und Solanum nigrum L. (Schwarzer Nachtschatten) wurden in Spanien gefunden. Die ersten beiden R-Biotypen stammten aus Simazin-behandelten Olivengärten ohne Bodenbearbeitung, die drei anderen von Atrazin-behandeltem Mais (Zea mays L.). Alle R-Biotypen ertrugen biszu 5,0kg ha−1 Atrazin oder Simazin, in den Boden eingcarbeitet, während empfindliche (S) oder ‘wilde’ Pflanzen mit 0,25 kg ha−1 Atrazin (A. hybridus, A. retroflexus, S. nigrum) und 2,5 kg ha−1 (A. albus, A. blitoides) bekämpfbar waren. Die höhere Triazintoleranz der beiden letzten Arten ist wahrscheinlich, auf eine Herbizidde-toxifikation zurückzuführen. Der photosynthetische Elektronentransport in den R-Biotypen wurde durch Atrazin und Simazin nur wenig beeinflußt, durch Diuron jedoch unterbunden, wie durch Messungen der Fluoreszenzinduktion der ganzen Blätter gefunden wurde. Bei der Untersuchung der Hill-Reaktion wurde bei den R-Biotypen für Simazin und Atrazin Resistenzfaktoren zwischen 900 und 350 festgestellt, für Diuron aber nur kleine Faktoren (1,1 bis 1,6). Daraus wurde geschlossen, daß alle R-Biotypen eine chloroplastische Resistenz entwickelt haben, wie sie schon früher für andere Triazin-resistente Unkrautbiotypen beschrieben worden ist.