Complex orofacial movements and the disappearance of cerebellar mutism: report of five cases

A syndrome of mutism and subsequent dysarthria occurs frequently in children after resection of a cerebellar tumour. The role of orofacial and speech motor control in this syndrome has not been studied systematically. We examined simple and complex orofacial movements during the mute phase and shortly after the resumption of speech in five children with mutism and subsequent dysarthria. The recovery of complex orofacial movements coincided with the disappearance of the mutism     

Interleukin-5 is at 5q31 and is deleted in the 5q- syndrome

Human interleukin-5 (IL-5) is a selective eosinophilopoietic and eosinophil-activating growth hormone. By in situ hybridization this gene is mapped to chromosome 5q23.3 to 5q32. It is shown to be deleted in two patients with the 5q-syndrome and in one patient previously diagnosed with myelodysplasia whose condition had progressed to acute myeloblastic leukemia. The clustering of other genes involved in hematopoiesis (IL-3, granulocyte-macrophage colony-stimulating factor, feline sarcoma viral oncogene homolog, colony-stimulating factor 1) to the same region as IL-5 suggests a nonrandom localization and raises interesting questions concerning the evolution and regulation of these genes.     

CQI-2 — a new measure of holistic interpersonal care in primary care consultations

BACKGROUND: The Consultation Quality Index (CQI) is a holistic quality marker for GPs based on patient enablement, continuity of the care and consultation length. AIM: To evaluate the CQI-2, a new version of the CQI incorporating a process measure of GP empathy (the Consultation and Relational Empathy Measure). DESIGN OF STUDY: Cross-sectional questionnaire study. SETTING: General practice in the west of Scotland. METHOD: Empathy, enablement, continuity, and consultation length were measured in 3044 consultations involving 26 GPs in 26 different practices in the west of Scotland. CQI-2 scores were calculated and correlated with additional data on GPs' and patients' attitudes. Comparisons were also made with the UK-wide data from which the original CQI had been calculated. RESULTS: CQI-2 scores were independent of deprivation, access, demographics, and case-mix. GPs with lower CQI-2 scores valued empathy and longer consultations less than these GPs with higher CQI-2 scores. 'Below average CQI-2' GPs (those in the bottom 25%) also felt less valued by patients and colleagues. Patients' showed less confidence in and gained less satisfaction from these doctors. Data ranges from the study were comparable with the UK data ranges used to construct the original CQI. CONCLUSIONS: The CQI-2 is a new measure of holistic interpersonal care. In a small but representative sample of GPs it appears to differentiate between below and above average doctors. CQI-2 scores may reflect important aspects of morale, core values and patient-centred care. There may be potential for its use as part of professional development and as a component of the general medical services contract.     

An analysis of fetal hemoglobin variation in sickle cell disease: the relative contributions of the X-linked factor, beta-globin haplotypes, alpha-globin gene number, gender, and age

Five factors have been shown to influence the 20-fold variation of fetal hemoglobin (Hb F) levels in sickle cell anemia (SS): age, sex, the alpha-globin gene number, beta-globin haplotypes, and an X-linked locus that regulates the production of Hb F-containing erythrocytes (F cells), ie, the F-cell production (FCP) locus. To determine the relative importance of these factors, we studied 257 Jamaican SS subjects from a Cohort group identified by newborn screening and from a Sib Pair study. Linear regression analyses showed that each variable, when analyzed alone, had a significant association with Hb F levels (P < .05). Multiple regression analysis, including all variables, showed that the FCP locus is the strongest predictor, accounting for 40% of Hb F variation. beta-Globin haplotypes, alpha-globin genes, and age accounted for less than 10% of the variation. The association between the beta-globin haplotypes and Hb F levels becomes apparent if the influence of the FCP locus is removed by analyzing only individuals with the same FCP phenotype. Thus, the FCP locus is the most important factor identified to date in determining Hb F levels. The variation within each FCP phenotype is modulated by factors associated with the three common beta-globin haplotypes and other as yet unidentified factor(s).     

Imiquimod 5% cream is an acceptable treatment option for external anogenital warts in uncircumcised males

OBJECTIVES: To determine the safety and efficacy of imiquimod (Aldara) 5% cream in the treatment of prepuce-associated warts in uncircumcised males. METHODS: An open-label study in six UK medical centres with 35 uncircumcised males with prepuce-associated warts treated with imiquimod 5% cream three times per week for up to 16 weeks. Other anogenital warts were also treated. RESULTS: Three times weekly application of imiquimod was found to be safe, with erythema as the most commonly reported local skin reaction. Forty per cent of patients had complete clearance of anogenital warts within 16 weeks. CONCLUSIONS: Imiquimod cream at a dosing regimen of three times per week, is effective and has an acceptable safety profile in the treatment of prepuce associated warts and other external anogenital warts in uncircumcised males.     

H2M3wt-restricted, Listeria monocytogenes-immune CD8 T cells respond to multiple formylated peptides and to a variety of gram-positive and gram- negative bacteria

A subset of H2M3wt-restricted, Listeria monocytogenes (LM)-immune CD8 effectors recognize antigen-presenting cells (APC) preincubated with heat-killed LM. The responsible product, which we have previously designated heat-killed Listeria-associated antigen (HAA), is extremely hydrophobic and resistant to proteolytic degradation. Despite the protease resistance of HAA, we now report that HAA-immune clones are uniformly responsive to fMIGWII, a formylated oligopeptide derived from the recently described LM product, lemA. While fMIGWII was by far the most potent peptide tested, over half our clones also responded to the LM-derived peptide fMIVII and cross-reactive responses to two other unrelated formylated peptides at concentrations of <1 microM were frequently observed. One of these peptides (fBlaZ) did not share any amino acid in common with fMIGWII except N-formyl methionine at position 1. Unformylated variants of the same peptides were inactive. HAA-immune CD8 cells also responded in an H2M3wt-restricted manner to APC pretreated with heat-killed or live preparations of other gram- positive and gram-negative bacteria such as Streptococcus pyogenes (SP) and Proteus vulgaris (PV). Unlike fMIGWII which is water soluble and protease sensitive, the native antigens extracted from SP and PV, like HAA, were very hydrophobic and proteinase K resistant, presumably reflecting in each case the association of cross-reactive polypeptides with bacterial lipid or phospholipid. Thus, HAA/lemA-immune, H2M3wt- restricted effectors can respond to a variety of formylated peptides and bacterial antigens in vitro. Similar cross-reactions in vivo might have physiologically significant implications.