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Background Tuberculosis ( TB) has been reported to increase morbidity after kidney transplantation and pose a therapeutic challenge. However, population-based research, specifically focused on the association between kidney transplantation and subsequent pulmonary or extrapulmonary TB, is lacking. Methods A nationwide population-based study was conducted using Taiwan's National Health Insurance Research Database, which provided claims data belonging to kidney transplant recipients during 1997-2006. Multivariate analysis was used to identify independent risk factors for TB after kidney transplantation. Kaplan-Meier survival analysis was used to assess the outcome of patients with TB. Results Among 4554 kidney transplant recipients over the 10-year period, 109 (2.4%) patients with newly diagnosed TB were identified: 75 patients with only pulmonary involvement, and 34 with extrapulmonary spread. The incidence of kidney transplant recipients developing TB was 638 per 100,000 person-years. The independent risk factors for post-transplant TB included cyclosporine-based immunosuppressant agents during the first year after kidney transplantation (odds ratio [ OR]: 1.98, P = 0.001), hepatitis C infection ( OR: 1.79, P = 0.024), and chronic obstructive pulmonary disease ( OR: 1.50, P = 0.041). Kidney transplant recipients who developed TB had a lower 5-year survival rate than those who did not (78.6% vs. 93.4%, P = 0.001). Conclusions Kidney transplant recipients in Taiwan did have a high risk of TB infection, with high proportion of extrapulmonary spread. Physicians need to be vigilant in surveying for TB in kidney transplantation, especially in high-risk patients. [ABSTRACT FROM AUTHOR]

Fabry disease is an X-linked disorder caused by a deficiency of the lysosomal α-galactosidase A [EC 3.2.1.22]. The molecular diagnosis of Fabry disease is important for genotype/phenotype correlation, pre-natal or early diagnosis, and detection of carrier status. Although more than 200 genotypes of the α-galactosidase A gene have been identified, mutation data on the Chinese population is sparse. We recently identified two unrelated Chinese families with Fabry disease. Mutation analysis was performed by polymerase chain reaction (PCR) sequencing of the seven exons and adjacent introns of the α-galactosidase A gene. Two novel mutations were identified: in family I, a C-to-A transversion resulted in an early termination at amino acid 222 (Y222X), while in family II, an A-to-G transition resulted in a substitution of alanine for threonine at amino acid 410 (T410A). Carrier status was identified in all four females in the two families. The genotype Y222X is associated with classic Fabry disease, with unexpectedly rapid deterioration of visual acuity, while T410A is associated with a milder Fabry disease, with ventricular hypertrophy and neuropathic pain. [ABSTRACT FROM AUTHOR]

Ten cases of tetrahydrobiopterin (BH4) deficiency were identified in 1,337,490 newborns screened in a Chinese population in Taiwan. The high incidence of BH4 deficiency in the Taiwanese population may be explained by a founder effect, since all of the patients revealed 6-pyruvoyltetrahydropterin synthase gene mutations, and grouping N52S and P87S mutations together constituted 88.9% of the disease alleles. BH4 supplementation with restriction of high-protein foods gave control of plasma phenylalanine within normal range, and levadopa itself prevented seizure. However, the average intelligence quotient (IQ) score of these patients was only 76 ± 14 (56–98). Statistically, the age of starting medication, including 5-hydroxytrytophan (5-HTP), was inversely correlated to IQ scores of these patients. We suggest the combination of BH4, levodopa and 5-HTP as the standard protocol to commence the treatment of BH4 deficiency as early as possible, although prenatal brain damage could have existed. [ABSTRACT FROM AUTHOR]

SUMMARY Aims To determine whether diabetic autonomic neuropathy is an important factor contributing to mortality in Type 2 diabetes mellitus. Methods Between 1989 and 1993, 431 men and 181 women with Type 2 diabetes were given diabetic autonomic neuropathy cardiovascular reflex (CVR) tests. These subjects were followed for the subsequent 5–9 years to assess mortality rates. Results The prevalence rate of abnormal CVR tests was 46.1% in patients with the history of diabetes less than 5 years and up to 69.4% when the history of diabetes exceeded 20 years. During the follow-up period from 1 January 1989 to 31 December 1997 (mean 7.7 years), a total of 135 participants died. The 8-year survival rate for patients with abnormal CVR tests was 63.6% in males and 76.4% in females, compared with 80.9 and 93.3% for patients with normal CVR tests. The results were grouped as: group 1, normal CVR tests without postural hypotension (PHT); group 2, normal CVR tests with PHT; group 3, abnormal CVR tests without PHT; and group 4, abnormal CVR tests with PHT. The 8-year survival rate was 85.4% in group 1, 80.9% in group 2, 74.5% in group 3 and 61.1% in group 4. Conclusion Type 2 diabetic patients with abnormal CVR tests may have increased mortality, and those combined with postural hypotension have higher mortality than those without. Abnormal CVR tests may be important predictors of mortality in Type 2 diabetes mellitus. [ABSTRACT FROM AUTHOR]

1. To determine the effectiveness of ongoing immediate concurrent feedback (ICF) in minimizing 'inappropriate' sultamicillin or co-amoxiclav prescribing via the parenteral route (i.e. when the oral route was accessible and not contraindicated), a prospective controlled audit was carried out on hospital inpatients over a 20 month period. 2. After an education programme to promote oral rather than unnecessary intravenous (i.v.) use of sultamicillin, co-amoxiclav and certain other drugs, an ongoing ICF strategy was instituted. 3. ICF entailed issue of memos on the following day to prescribers of i.v. sultamicillin or co-amoxiclav for inpatients in whom this route was deemed 'inappropriate', by a specially trained nurse using strict objective criteria. The memos recommended oral prescribing (particularly of co-amoxiclav, currently the less expensive alternative). 4. After starting ICF, there were consistent, clinically and statistically significant reductions in the monthly proportions of (i) admissions prescribed i.v. sultamicillin or co-amoxiclav (38% P < 0.001), (ii) those in whom the route was 'inappropriate' (75%, P < 0.001), and (iii) corresponding ratios of i.v./oral usage and expenditure, oral sultamicillin/co-amoxiclav usage and expenditure, as well as total and per admission expenditure on i.v. forms (> or = 43%, P < 0.01). 5. For i.v. cefuroxime (for which there was no ICF) and its oral counterpart cefuroxime-axetil, there were no comparable changes in usage or expenditure. 6. This simple, ongoing ICF strategy was effective and well accepted; estimated net monthly savings being HK$26-30,000.