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Deficiency of iduronate-2-sulphatase (IDS) results in the X linked recessive lysosomal storage disorder Hunter syndrome. Determination of carrier status in families affected by this disorder has been performed using a variety of enzymatic tests. None of these tests has proved to be 100% effective at identifying carriers. The aim of this study was to perform carrier testing in a family affected by the disorder, where testing was complicated by the fact that no surviving affected subjects were available for study. Direct dye primer sequencing of PCR products was used to identify mixed bases in an obligate carrier. Two mixed bases were observed within exon VIII. The first base change (T-->A) at nucleotide position 1150 results in a missense mutation (H342Q), while the second base change (G-->T) at nucleotide position 1151 results in a nonsense mutation (G343X). Four additional female family members were screened for the same mutation. Using this approach it is possible to provide unambiguous information about a subject's carrier status and, unlike biochemical testing, this approach will be equally effective when applied to families with the mild form of this disorder.

Golden hamsters of one common laboratory strain had a high incidence of hydrocephalus internus. When a severity score of hydrocephalus was used, a major autosomal recessive locus could be identified. However, when a binary score (hydrocephalus, no hydrocephalus) was used, no such major locus could be detected and results of test matings were not consistent with Mendelian inheritance. Golden hamsters with severe forms of hydrocephalus had a dorsally compressed and ventrally intact hippocampus. Implications for the behavior and well-being of affected hamsters are unknown but researchers using this strain should be aware of the likely presence of hydrocephalus.

Although the growth of towns and trade in medieval England and Wales has received much attention, the national transportation system which developed hand-in-hand with it has largely been ignored. In particular, there has been a paucity of systematic work dealing with the navigable waterways and their relationship to the road network. This article attempts to rectify this deficiency, principally by an analysis of references to navigations in the various State Rolls. About 140 rivers are shown to have been navigated, with a total length of over 2400 miles. Overall, few places were more than 15 miles from the coast or a navigable river, and in particular, large parts of eastern England were well provided with navigable water; some detail is given of the navigable limits of the rivers flowing into the Humber. Finally, the distribution of the prominent boroughs is combined with the river and road networks to reveal a well-integrated national urban, trading and transportation system.

Respiratory macrophages (RM) of the turkey were elicited with a 1:4 (v/v) suspension of incomplete Freund's adjuvant in sterile phosphate-buffered saline injected directly into the abdominal air sacs. RM were purified by passage through a Ficoll-Hypaque gradient resulting in 95.7 ± 5.9% purity and 94.8 ± 12.3% viability. On days 7 and 9 postinjection, adequate numbers (7.15± 5.47× 106macrophages per turkey) of RM for in vitro experiments were obtained. RM of the turkey demonstrated the ability to adhere to glass, phagocytize Zymosan A, and kill Escherichia coli in vitro. /// Se indujeron macrófagos respiratorios de pavo mediante la inyección de una suspensión 1:4 (v/v) de adyuvante incompleto de Freund en una solución salina fosfatada y buferada estéril, aplicada directamente en los sacos aéreos abdominales. Los macrófagos respiratorios fueron purificados a través de un gradiente de Ficoll-Hypaque, resultando en una pureza de 95.7 ± 5.9% y una viabilidad del 94.8 ± 12.3%. Siete y nueve días después de la infección, se obtuvo un número adecuado de macrófagos respiratorios (7.15± 5.47× 106macrófagos por pavo) para los experimentos in vitro. Estos macrófagos respiratorios demonstraron tener capacidad para adherirse al vidrio, fagocitar el Zymosan A y destruir al Escherichia coli in vitro.

Effects of Newcastle disease virus (NDV) infection on the binding, phagocytic, and bactericidal activities of turkey respiratory macrophages were studied. Respiratory macrophages of the turkey demonstrated the presence of immunoglobulin (Ig) G and complement receptors but lacked IgM receptors. Respiratory macrophages from NDV-infected turkeys showed little or no depression of binding of sheep erythrocyte-IgG complexes and sheep erythrocyte-IgM-complement complexes to their appropriate membrane receptors. In contrast, respiratory macrophages from NDV-infected turkeys showed significant (P ≤ 0.05) depression of phagocytosis of similar complexes. Bacterial killing by respiratory macrophages from NDV-infected turkeys was significantly (P ≤ 0.05) inhibited. /// Se estudiaron los efectos de la infección con el virus de la enfermedad de Newcastle sobre las actividades aglutinantes, fagocíticas y bactericidas de los macrófagos respiratorios del pavo. Los macrófagos respiratorios del pavo demostraron la presencia de receptores de inmunoglobulina G (IgG) y complemento pero no tenían receptores para la inmunoglubulina M (IgM). Los macrófagos respiratorios de pavos infectados con Newcastle mostraron poca o ninguna disminución en su capacidad para aglutinar complejos compuestos por IgG-eritrocitos de ovino y complejos de complemento-IgM-eritrocitos de ovino a los receptores apropiados en la membrana. En contraste, los macrófagos respiratorios de pavos infectados con Newcastle mostraron una disminución significante (P ≤ 0.05) en la fagocitosis de los mismos complejos. La destrucción de bacterias por los macrófagos respiratorios de los pavos infectados con Newcastle fué inhibida en forma significante (P ≤ 0.05).

Quantitative clearance of aerosolized Escherichia coli from the trachea, lung, and air sacs was measured in turkeys infected with Bordetella avium. Clearance of E. coli in turkeys with B. avium-induced tracheitis was minimally affected early in infection. Sixteen to 23 days after infection with B. avium, sporadic, mild depressions in clearance of E. coli were observed in the tracheas, which had large areas of deciliated tracheal epithelium or replacement of normal epithelium by immature hyperplastic epithelium or metaplastic squamous epithelium. Clearance of E. coli from the lung and air sacs was minimally affected in turkeys infected with B. avium. /// En pavos infectados con Bordetella avium se midió cuantitativamente la eliminación de Escherichia coli a partir de tráquea, pulmones y sacos aéreos. La eliminación de E. coli en pavos con traqueítis inducida por B. avium fué afectada en forma mínima durante los estados iniciales de la infección. Entre 16 y 23 días después de la infección con B. avium, se observó una disminución esporádica y leve en la eliminación de E. coli de la tráquea. Se observaron en este órgano grandes áreas de epitelio sin cilias o reemplazo del epitelio normal por epitelio inmaduro hiperplásico o epitelio escamoso metaplásico.

Using the radiopharmaceutical99 mtechnetium-sulfur colloid, the tracheal mucus transport rate (TMTR) was measured in healthy unanesthetized turkeys and in turkeys infected with Bordetella avium. The TMTR of uninfected turkeys was 35.6 ± 14.4 cm/min. The TMTR of B. avium-infected turkeys was normal on days 0 through 14 postexposure (PE), despite heavy bacterial colonization of the tracheal epithelium. On day 21 PE, the TMTR of B. avium-infected turkeys was significantly depressed (P ≤ 0.01) compared with that of control turkeys. Depressed transport was associated with extensive loss of ciliated epithelium from the tracheal mucosa and replacement of the normal mucosa by immature nonciliated epithelium or metaplastic squamous epithelium. /// Se determinó el índice de transporte del moco traqueal en pavos sanos sin anestesia y en pavos infectados con Bordetella avium, usando el coloide radiofarmacéutico99 mtecnetio-azufre. El índice en pavos sanos fué de 35.6 ± 14.4 cm/minuto mientras que en pavos infectados con B. avium el índice fué normal durante los días 0 a 14 después de la infección, a pesar de la densa colonización del epitelio traqueal. Al día 21 después de la infección, el índice de los pavos infectados fué significativamente menor (P ≤ 0.01) que el de los controles sanos. Se asocia la disminución en el transporte del moco traqueal con la pérdida extensiva del epitelio ciliado de la mucosa traqueal y el reemplazo del mismo con epitelio no ciliado inmaduro o epitelio escamoso metaplástico.

A case of disseminated coccidioidomycosis caused by a dimorphic fungus Coccidioides immitis in a mandrill baboon (Mandrillus sphinx) was diagnosed following radiography, ultrasound-guided aspiration of thoracic lesions, and aspiration cytology of skeletal lesions of the left sixth rib. The diagnosis was confirmed by fungal culture and serum quantitative immunodiffusion for antibodies against C. immitis.

Tracheal mucus transport rate (TMTR) and quantitative clearance of aerosolized Escherichia coli from the trachea, lung, and air sac were measured in healthy unanesthetized turkeys and in turkeys exposed by aerosol to a La Sota vaccine strain of Newcastle disease virus (NDV). The TMTR of uninfected turkeys was 42.4 ± 14.7 cm/min. The TMTR of NDV-infected turkeys was depressed on days 3 through 7 postexposure (PE); depression was significant (P ≤ 0.05) on day 7 PE. Tracheal E. coli clearance in NDV-infected turkeys was reduced on days 4 through 9 PE, significantly so on day 5 PE (P ≤ 0.01). Depression of TMTR and tracheal E. coli clearance were associated histologically with replacement of normal pseudostratified columnar epithelium by 3 to 8 layers of immature nonciliated cells. E. coli clearance by the lung and air sac of NDV-infected turkeys was depressed on days 5 through 9 PE. /// Se determinó el índice de transporte de moco traqueal y la eliminación cuantitativa de Escherichia coli (administrada por aerosol) a partir de la tráquea, pulmón y saco aéreo. Los parámetros se evaluaron en pavos sanos no anestesiados y en pavos expuestos a un aerosol de la vacuna contra Newcastle cepa La Sota. El índice de transporte para los pavos no infectados fué de 42.4 ± 14.7 cm/minuto. El índice en los pavos infectados con el virus vacunal disminuyó durante los días 3 a 7 postinfección (PI), siendo significativo (P ≤ 0.05) al día 7 PI. La eliminación cuantitativa de E. coli de la tráquea en pavos infectados con el virus disminuyó durante los días 4 a 9 PI, siendo significativo al día 5 PI (P ≤ 0.01). La disminución en el índice de transporte y en la eliminación de E. coli de la tráquea estaban asociados histológicamente por el reemplazo del epitelio columnar pseudoestratificado normal, con 3 a 8 capas de células inmaduras sin cilias. La eliminación de E. coli del pulmón y los sacos aéreos de los pavos infectados con el virus disminuyó durante los días 5 a 9 PI.