Anticancer Activity of Nonpolar Pt(CH)I{} is Found to be Superior among Four Similar Organoplatinum(IV) Complexes.

The anticancer properties of well-defined molecules serve to bolster the field of metals in medicine. Such compounds, particularly those of platinum and their closely related structural analogs, continue to be potentially highly interesting to researchers and clinicians alike. The four octahedral organoplatinum(IV) compounds [Pt(CH)X{ }] (X = Br, I; = 2,2'-bipyridine, 2,2'-bipyridine-4,4'-dicarboxylic acid) have been isolated and structurally characterized by single-crystal X-ray diffraction.

Newborn screening for spinal muscular atrophy: The Wisconsin first year experience.

Spinal muscular atrophy was recently added to the Wisconsin newborn screening panel. Here we report our screening methods, algorithm, and outcomes. A multiplex real-time PCR assay was used to identify newborns with homozygous SMN1 exon 7 deletion, and those newborns' specimens further underwent a droplet digital PCR assay for SMN2 copy number assessment.

Newborn Screening for Severe Combined Immunodeficiency Using the Multiple of the Median Values of T-Cell Receptor Excision Circles.

All newborn screening programs screen for severe combined immunodeficiency by measurement of T-cell receptor excision circles (TRECs). Herein, we report our experience of reporting TREC assay results as multiple of the median (MoM) rather than using conventional copy numbers. This modification simplifies the assay by eliminating the need for standards with known TREC copy numbers.

Newborn Screening for Severe Combined Immunodeficiency: Do Preterm Infants Require Special Consideration?

The Wisconsin Newborn Screening (NBS) Program began screening for severe combined immunodeficiency (SCID) in 2008, using real-time PCR to quantitate T-cell receptor excision circles (TRECs) in DNA isolated from dried blood NBS specimens. Prompted by the observation that there were disproportionately more screening-positive cases in premature infants, we performed a study to assess whether there is a difference in TRECs between full-term and preterm newborns. Based on de-identified SCID data from 1 January to 30 June 2008, we evaluated the TRECs from 2510 preterm newborns (gestational age, 23-36 weeks) whose specimens were collected ≤72 h after birth.

Development of a routine newborn screening protocol for severe combined immunodeficiency.

Background: Severe combined immunodeficiency (SCID) is characterized by the absence of functional T cells and B cells. Without early diagnosis and treatment, infants with SCID die from severe infections within the first year of life.

Objective: To determined the feasibility of detecting SCID in newborns by quantitating T-cell receptor excision circles (TRECs) from dried blood spots (DBSs) on newborn screening (NBS) cards.

Use of detomidine hydrochloride as an adjunct for studying first-stage Gasterophilus intestinalis (Diptera: Gasterophilidae) in the tongue of the horse.

A synthetic alpha-2 adrenergic agonist, detomidine hydrochloride, was used in the study of in vivo activity of Gasterophilus intestinalis (Diptera: Gasterophilidae) during migration in the tongue of the horse. Use of the drug allowed the investigator to manipulate the tongue and closely observe the movement patterns and tissue disturbance caused by burrowing first-stage larvae. Detomidine hydrochloride should be utilized in studies of drug efficacy and larval biology, whenever possible, to avoid the need to sacrifice the horse.

Field observations of the host-parasite relationship associated with the common horse bot fly, Gasterophilus intestinalis.

Behavior of Gasterophilus intestinalis (Diptera: Gasterophilidae) was observed in the laboratory and field over a period of 10 years. Mating occurred in a frenzied manner as flies emerged from pupae. Males attempted to copulate with females that had not fully emerged from the pupal case.

Inter-relationship between Gasterophilus larvae and the horse's gastric and duodenal wall with special reference to penetration.

The degree of penetration into the stomach and duodenum of the horse by bot fly larvae, Gasterophilus intestinalis (De Geer) and G. nasalis (Linnaeus) (Diptera : Gasterophilidae) was evaluated. Evidence of larval perforation of the stomach or duodenum was not found on gross inspection.

Pattern of inheritance in hereditary myeloperoxidase deficiency associated with the R569W missense mutation.

Myeloperoxidase (MPO) is an essential component of the oxygen-dependent microbicidal system of neutrophils and monocytes. Hereditary deficiency of MPO occurs in 1 in 2,000 to 4,000 individuals in the general population and has been generally considered an autosomal recessive trait. Previous studies have used the peroxidase activity of blood leukocytes to assess the phenotype of affected family members.

Effect of the R569W missense mutation on the biosynthesis of myeloperoxidase.

Human neutrophil microbicidal activity is largely mediated by reactive species generated by the oxygen-dependent myeloperoxidase (MPO) system. Peroxidase-negative neutrophils from many patients with hereditary MPO deficiency possess a 90-kDa MPO-related protein. We recently identified a missense mutation, R569W, in the MPO gene of many subjects with MPO deficiency.

Hereditary myeloperoxidase deficiency due to a missense mutation of arginine 569 to tryptophan.

Hereditary deficiency of myeloperoxidase (MPO) is a common disorder but its genetic basis is unknown. We have reported that neutrophils from individuals with MPO deficiency lack enzymatic and immunochemical evidence for mature MPO but have a 90-kDa precursor protein. We have thus hypothesized that hereditary MPO deficiency reflects a defect in processing of a mutated primary translation product.