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Notes: Summary A patient with molybdenum cofactor deficiency (producing the biochemical abnormalities associated with deficiencies of sulphite oxidase and xanthine dehydrogenase) clinically expressed Marfan-like habitus with dislocated lenses, vertebral abnormality, learning disability, moderate hemiplegia, increased medial lentiform MRI signal and intermittent microscopic haematuria.S-Sulphocysteine was present in plasma and urine, and the oxidized derivative of a molybdopterin precursor (precursor Z), together with xanthine and hypoxanthine, were elevated in urine. Blood uric acid was 〈1 mg/dl, while urinary urothione was not detected. These data indicate a functionally inadequate terminal enzyme for converting precursor Z to active molybdopterin (complementation group B of general molybdenum cofactor deficiency). Although the biochemical parameters were indicative of a severe deficiency state, the patient has survived into the third decade with a less severe clinical spectrum than has generally been associated with this disease.

Notes: Summary We report a 21-month-old female patient whose urine organic acid profile suggested a biotin utilization abnormality consistent with multiple carboxylase deficiency. For most previously reported patients, holocarboxylase synthetase deficiency has correlated with the early-onset variant of multiple carboxylase deficiency; conversely, biotinidase deficiency has been characteristic of the late-onset form.In vitro enzyme studies revealed that our patient suffered from holocarboxylase synthetase deficiency. We suggest that holocarboxylase synthetase deficiency should be considered in the differential diagnosis of older patients in whom there is suspicion of a defect in biotin metabolism.

Notes: Abstract A survey, conducted in 1990–1991 on agroforestry and forestry-related systems in eight Midwestern states, showed that three traditional and three nontraditional agroforestry systems are practiced in the region. Of 46 traditional systems reported, most common was agrisilviculture (28), then silvipasture (12) and agrisilvipasture (6). These systems often involved corn, soybeans, and hay planted with tree species for nut, timber, or Christmas tree production, and cattle. Non-traditional agroforestry systems were field windbreaks/shelterbelts (29), treeshrub intercropping systems (21), and boundary plantings (11). These systems involved a variety of northern hardwood species evergreens, and shrubs. There was also a preponderance of specialized systems (97) of growing trees with the production of mushrooms, nuts, syrup, wildlife, and other nonwood products. The survey is exploratory in nature and, although the results may not represent a complete count of people involved with agroforestry in the region, the numbers reported may indicate where a practice may be prevalent. The results reflect the fact that much work still needs to be done by agroforesters in this region. They also provide some basis for further research on more suitable systems, and for identifying other researchable questions on agroforestry for the region. The methodology also made possible a bigger nucleus for networking of agroforesters and other interested people in the Midwest. Finally, although not normally considered as agroforestry, the significance of the other specialized systems reported in this survey is that they offer varied ways of connecting forestry with other activities in a largely agricultural landscape, and that they may even be seen as opportunities for easier transition into agroforestry.

Notes: Abstract A multi-species riparian buffer strip (MSRBS) system was designed and placed along a Central Iowa stream in 1990. Bear Creek, is typical of many streams in Central Iowa where the primary land use along the stream's length is row crop (corn and soybeans) production agriculture or intensive riparian zone grazing. The Bear Creek watershed is long (∼ 35 km), narrow (3–6 km), and drains 7,661 ha of farmland. The MSRBS system is a 20 m wide filter strip consisting of four or five rows of fast-growing trees planted closest to the stream, then two shrub rows, and finally a 7 m wide strip of switchgrass established next to the agricultural fields. The 1.0 km long system, is located on an operational farm and is laid out in a split block design on both sides of Bear Creek. An integral part of this system is a streambank stabilization soil bioengineering component and a constructed wetland to intercept NPS pollutants in field drainage tile water flow. It is hypothesized that this system will function effectively as a nutrient, pesticide, and sediment sink for NPS pollutants coming from the upslope agricultural fields. Prior to establishment of the MSRBS system, the riparian zone along Bear Creek was grazed and row cropped to the stream edge. Since 1990 there has been dramatic alteration in the appearance and functioning of this riparian zone. After four growing seasons, the fast-growing tree species (cottonwood, silver maple, willow, and green ash) range in height from 2.4 m to over 5.5 m. Mean (four-year) biomass production of silver maple was 8.4 dry Mg ha−1, more than twice to seven times the yield from other silver maple research plots in Central Iowa. The shrub species, selected because of desired wildlife benefits, have done well in terms of survival and growth with ninebark, Nannyberry viburnum and Nanking cherry doing the best. The switchgrass grass has developed into a dense stand that effectively stops concentrated flow from the agriculture fields and allows for infiltration rates well above the field rate. Early root biomass data indicate significantly more roots below the MSRBS than agricultural fields. This suggests better soil stabilization, absorption of infiltrated water, and soil-root-microbe-NPS pollutant interaction characteristics within the MSRBS system than the cropped fields. Nitrate-nitrogen concentrations in the MSRBS never exceed 2 mg l−1 whereas the levels in the adjacent agricultural fields exceed 12 mg l−1. The water quality data collected suggest that the MSRBS is effective in reducing NPS pollutants in the vadose and saturated zone below the system. The soil bioengineering revetments have stabilized the streambank and minimized bank collapse. Initial results (from 4 months of operation) from the constructed wetland (built in summer 1994) indicate nitrate-nitrogen concentrations of the tile inflow water 〉15 mg l−1 whereas, the outflow water had a nitrate-nitrogen concentration of 〈3 mg l−1. Over time this wetland should become more effective in removing excess nitrogen moving with the tile flow from the agricultural fields because of the accumulation of organic matter from the cattails. Overall the MSRBS system seems to be functioning as expected. This MSRBS system offers farmers a way to intercept eroding soil, trap and transform NPS pollution, stabilize streambanks, provide wildlife habitat, produce biomass for on-farm use, produce high-quality hardwood in the future, and enhance the aesthetics of the agroecosystem. As a streamside best management practice (BMP), the MSRBS system complements upland BMPs and provides many valuable private and public market and non-market benefits.