Well-being and family support among elderly rural Mexicans in the context of migration to the United States

OBJECTIVE: To explore perceptions of well-being, family support, and economic resources in relation to level of contact with migration to the U.S. among a sample of elderly males from rural Mexico. METHOD: The snowballing technique was used to obtain a sample of 372 participants. Four groups were created according to the level of contact with migration among older adults and their children. RESULTS: Greater level of contact with migration was associated with a higher likelihood that an older adult was literate, married or living with someone, self-employed, and retired or pensioned. In addition, greater level of contact with migration to the U.S. was associated with a higher level of perceived well-being, family support, and economic security. DISCUSSION: Elderly, rural Mexican men with a greater degree of contact with migration to the U.S. seem to have more security and well-being in their old age.     

Stable long‐term risk of leukaemia in patients with severe congenital neutropenia maintained on G‐CSF therapy

In severe congenital neutropenia (SCN), long‐term therapy with granulocyte colony‐stimulating factor (G‐CSF) has reduced mortality from sepsis, revealing an underlying predisposition to myelodysplastic syndrome and acute myeloid leukaemia (MDS/AML). We have reported the early pattern of evolution to MDS/AML, but the long‐term risk remains uncertain. We updated a prospective study of 374 SCN patients on long‐term G‐CSF enrolled in the Severe Chronic Neutropenia International Registry. Long‐term, the annual risk of MDS/AML attained a plateau (2·3%/year after 10 years). This risk now appears similar to, rather than higher than, the risk of AML in Fanconi anaemia and dyskeratosis congenita.     

CCL genes in multiple sclerosis and systemic lupus erythematosus

This follow up study aims to refine the roles of previously suggested candidate genes (CC chemokine ligands or CCLs) in multiple sclerosis (MS), and to test these markers in another autoimmune disorder, systemic lupus erythematosus (SLE). After stringent correction for multiple testing, we reject the importance of previously suggested borderline associations with CCLs in MS. A new finding is the differential distribution of CCL8 marker alleles and a haplotype in extreme severity subgroups of MS. In SLE, this study reveals strong associations with a marker and a haplotype encompassing the CCL14 gene, which suggests that a lupus relevant variant may lie within or in the proximity of this haplotype.     

Identification of midbrain floor plate radial glia-like cells as dopaminergic progenitors

The floor plate (FP), a signaling center and a structure rich in radial glia-like cells, has been traditionally thought to be devoid of neurons and neuronal progenitors. However, in the midbrain, the FP contains neurons of the dopaminergic (DA) lineage that require contact with radial glia-like cells for their induction. We, therefore, decided to explore the interaction relationship between radial glia and neurons during DA neurogenesis. Taking advantage of a novel FP radial glia-like cell culture system and retroviruses, DA neurons were lineage traced in vitro. In utero BrdU pulse-chases extensively labeled the midbrain FP and traced DA neurons both in vivo and in FP cultures. Moreover, from E9.5 to E13.5 the midbrain FP contained dividing cells only in the most apical part of the neuroepithelium, in cells identified as radial glia-like cells. We, therefore, hypothesized that midbrain FP radial glia-like cells could be DA progenitors and tested our hypothesis in vivo. Lineage tracing of DA progenitors with EGFP in Tis21-EGFP knock-in mice, and genetic fate mapping in GLAST::CreERT2/ZEG mice identified the neuroepithelium of the midbrain FP, and specifically, GLAST+ radial glia-like cells as DA progenitors. Combined, our experiments support the concept that the midbrain FP differs from other FP regions and demonstrate that FP radial glia-like cells in the midbrain are neurogenic and give rise to midbrain DA neurons.     

Arteriovenous fistula for long-term venous access for boys with hemophilia

OBJECTIVES: Hemophilia is a sex-linked condition affecting about 1 of every 5000 males in the United States. The management of children with hemophilia can be improved with regular intravenous infusion of factor VIII or IX, thus preventing crippling and sometimes fatal hemorrhage. Maintaining this vital intravenous access is often hampered by gradual loss of superficial veins or repeated central catheter sepsis and thrombosis. This study reviewed an experience with arteriovenous fistula in selected hemophilia patients with limited venous access. METHODS: Consecutive patients operated on between October 2000 and July 2006 for venous access with the creation of an arteriovenous fistula were reviewed. They were selected because of repeated problems with other venous access. Patency, ease of use, duplex scan derived brachial artery diameter, and arm length were assessed. RESULTS: During a 69-month period, 10 arteriovenous fistulas (five brachial artery-basilic vein fistulas, 5 brachial artery-cephalic vein fistulas) were created for nine patients. The patients were a median age of 5.5 years (range, 1 to 27 years), and all were <13 except the 27-year-old patient. There were no postoperative hematomas requiring evacuation. One arteriovenous fistula failed to mature and was redone in the opposite arm, which subsequently occluded after 13 months. Of the mature fistulas, patency was 100% at 1 year, 80% (4/5) at 3 years, and 75% (3/4) at 4 years, with mean follow-up of 22 months. Brachial artery diameter increased in the involved arm by a ratio of 1.95 (range, 1.51 to 2.5) compared with the opposite arm. Arm length disparity was increased by 0.5 cm (range, 0.8 to 1.5 cm) in the involved arm. All fistulas allowed good access at home by a care provider. CONCLUSIONS: For hemophilia patients with compromised venous access, arteriovenous fistulas provide good early patency. Brachial artery diameter and arm length require continued follow-up.     

Navajo neurohepatopathy: a mitochondrial DNA depletion syndrome?

Navajo neurohepatopathy (NNH) is an autosomal recessive disease of full-blooded Navajo children living in the Navajo Reservation of southwestern United States. Clinical features of NNH include peripheral and central nervous system involvement, acral mutilation, corneal scarring or ulceration, liver failure, and metabolic and immunologic derangement. The cause of NNH is unknown, but the clinical features of NNH are similar to those of patients with mitochondrial DNA (mtDNA) depletion. Therefore, we studied mtDNA concentration in the liver from 2 patients with NNH. Using histochemical, biochemical, and molecular techniques, we found evidence of mtDNA depletion, and we propose that the primary defect in NNH is in the nuclear regulation of mtDNA copy number.     

Chromosome 12-linked autosomal dominant scapuloperoneal muscular dystrophy

Scapuloperoneal syndromes are characterized by their distribution of muscle weakness and wasting. The reported pattern of inheritance has been variable. Both neurogenic and myopathic forms of autosomally dominantly inherited scapuloperoneal syndrome have been described. It has been suggested that these are variants of other neuromuscular diseases. We examined 44 members from a family with 14 members affected with a scapuloperoneal syndrome. Physiological and histological analysis implied that this condition is predominantly myopathic. Linkage analysis was done to confirm the genetic etiology of the disease in this family and to evaluate the possibility that it is an allelic variant of other neuromuscular diseases. Genetic analysis demonstrated linkage of the disease to chromosome 12, which makes it genetically distinct from other loci known to cause neuromuscular disease. Muscle fibers with hyaline desmin-containing cytoplasmic inclusions in combination with focal myopathic changes may be a disease-specific morphological marker of the disease.     

The posterior sagittal approach: further pediatric applications

The posterior sagittal transsphincteric approach is useful for surgical management of tumors, fistulae, and sequelae of failed pull-throughs for congenital anomalies, particularly when these conditions are located too high to be approached conveniently through the perineum or too low to be approached through the abdomen. Except for anorectal malformations, there have been no previous reports of use of this approach in children. Five cases are presented to demonstrate the advantages of this approach: two patients with severe sequelae of pelvic trauma; one with a large tumor invading the rectum and vagina; one case of adrenal hyperplasia, urogenital sinus, and a high implanted vagina; and one case of a failed pull-through for the treatment of Hirschsprung's disease. All patients are continent of stool and urine. The authors suggest using this approach with a protective colostomy.     

Myeloperoxidase release after allergen-specific conjunctival challenge.

BACKGROUND: Allergen-specific conjunctival challenge is a fruitful and complete tool in evaluating pathophysiological phenomena of allergic inflammation. After challenge, a significant neutrophil infiltrate occurred in allergic subjects. The primary (azurophilic) granules of neutrophils contain a variety of enzymes that might potentiate inflammation, such as myeloperoxidase (MPO). It is not known whether allergen-specific conjunctival challenge (ASCC) is able to elicit MPO release. We also investigated the possible role of immunotherapy (IT) in the release of MPO. METHOD: The groups studied included Dactylis glomerata-sensitive adult atopic patients suffering from seasonal allergic rhinoconjunctivitis, and healthy adult nonatopic volunteer controls. One group of allergic patients received no specific hyposensitization (not-IT allergic group). A second group of allergic patients had been immunotherapy-treated with Dactylis glomerata extract for the preceding three years and continued to receive a maintenance dose within the highest potency of the extract (IT-allergic group). ASCC with Dactylis glomerata was performed outside the pollen season in all subjects. Myeloperoxidase was assayed by MPO-enzyme immunoassay method. RESULTS: Thirty minutes after challenge, myeloperoxidase levels in the non-immunotherapy allergic patients were significantly higher compared than in the healthy group (p<0.001). The levels of myeloperoxidase released in the immunotherapy allergic group were significantly lower than those in the nonimmunotherapy allergic group (p<0.001) and higher than those in nonallergic subjects (p<0.001). CONCLUSION: These results indicate that after ASCC there is a release of MPO. Our study suggests that immunotherapy actively modifies the release of MPO after ASCC.