An Assessment of Female Prisoners' Perception of the Accessibility of Quality Healthcare: A Survey in the Kumasi Central Prisons, Ghana.

BACKGROUND: Accessibility of quality healthcare across the globe has generated a lot of attention among public health practitioners. AIM: This study explored the background characteristics of female prisoners and how it influences their assessment of the quality of accessible healthcare in the Kumasi Female Prison. SUBJECTS AND METHODS: This descriptive cross-sectional survey was conducted at the Female section of the Kumasi Central Prisons from June to December 2011. We used pretested questionnaires to obtain quantitative data from all 39 inmates of the female Prisons. An in-depth interview was used to obtain qualitative data from the prison healthcare giver. Data were analyzed with Epi Info Version 3.5.1, (Centers for Disease Control and Prevention), Excel, and Graph Pad Prism version 5.00 for Windows (Graph Pad software, San Diego California USA, www.graphpad.com). RESULTS: Using a 12-point scale inventory questionnaire, inmates with no formal education gave the highest mean health provision assessment score (6.0) whereas those with tertiary education gave the lowest (4.5). Females serving prison sentences gave the highest mean health assessment score whereas remand prisoners gave the lowest. Single females' mean health assessment score was 5.7 whereas that of married inmates was 4.9. Unemployed inmates scored 5.8, informal 5.4 while civil servants scored 5.0. CONCLUSION: Access to quality healthcare was poor and demographic characteristics, marital status, educational background, and occupation influenced inmates' perceptions of accessibility to quality healthcare. Inmates should be encouraged to be proactive in seeking healthcare irrespective of their background characteristics.

Protracted course of juvenile ceroid lipofuscinosis associated with a novel CLN3 mutation (p.Y199X).

The juvenile neuronal ceroid lipofuscinosis (JNCL, Batten disease, MIM 204200), is an autosomal recessive lysosomal storage disease, which is characterized by ubiquitous accumulation of the lipopigment material ceroid-lipofuscin. It manifests with loss of vision in childhood due to retinal degeneration, followed by seizures and parkinsonism leading to premature death at around 30 years. Eighty-five percent of JNCL patients carry a disease-causing 1.02 kb deletion in the CLN3 gene on chromosome 16. Here we report on a large consanguineous Lebanese family with five affected siblings. Electron microscopy of lymphocytes revealed the presence of fingerprint profiles suggesting JNCL. However, disease progression, especially of mental and motor function was slower as expected for 'classic' JNCL. We thus confirmed the diagnosis by genetic testing and found a new c.597C>A transversion in exon 8, homozygous in all affected family members and not present in 200 alleles of normal controls. The mutation generates a premature termination codon (p.Y199X) truncating the CLN3 protein by 55%. In heterozygous state mutant mRNA transcripts are expressed at the same levels as the wild-type ones, suggesting the absence of nonsense mediated messenger decay. We discuss a potential residual catalytic function of the truncated protein as a cause for the mild phenotype.

New mutations in the neuronal ceroid lipofuscinosis genes.

Thirty-eight mutations and seven polymorphisms have recently been reported in the genes underlying the neuronal ceroid lipofuscinoses (NCLs) including 11 new mutations described here. A total of 114 mutations and 28 polymorphisms have now been described in the five human genes identified which cause NCL. Thirty-eight mutations are recorded for CLN1/PPT; 40 for CLN2/TTP-1, 31 for CLN3, four for CLN5, one for CLN8. Two mutations have been described in animal genes (cln8/mnd, CTSD). All mutations in NCL genes are contained in the NCL Mutation Database (http://www.ucl.ac.uk/NCL).