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Background: Both vestibular schwannoma (VS) and Meniere's disease (MD) patients underwent hydrops MRI to clarify the relationship between VS and endolymphatic hydrops (EH)., Methods: Eighty patients with VS or MD underwent an inner ear test battery followed by hydrops MRI, and were then divided into 3 groups. Group A comprised 58 MD patients (62 ears) with positive EH but negative VS. Group B included 18 VS patients (18 ears) with negative EH, while Group C consisted of 4 patients (4 ears) who had VS concomitant with EH. Another 14 MD patients who tested negative for EH on hydrops MRI were initially excluded from this cohort, but were later included for comparison., Results: The decreasing prevalence of EH at the cochlea, saccule and utricle in Group A was identified in 59 (95%), 42 (68%) and 40 (65%) ears, respectively, mimicking a declining sequence of abnormality rates running from audiometry (86%), cervical vestibular-evoked myogenic potential (cVEMP) test (55%) to the ocular (oVEMP) test (53%). However, such decreasing trend was not identified in Groups B and C. In Groups C and A combined, 4 (6%) of 62 EH patients had concomitant VS. Conversely, 4 (18%) of 22 VS patients in Groups C and B combined had concurrent EH., Conclusion: A very low (6%) rate of VS in EH patients indicates that VS in EH patients may be coincidental. In contrast, EH was identified in 18% prevalence of VS patients, mirroring the 22% prevalence of cochlear EH demonstrated in VS donors through histopathological studies., Competing Interests: Declaration of competing interest The authors declare that they have no conflict of interest., (Copyright © 2024 Formosan Medical Association. Published by Elsevier B.V. All rights reserved.)

Objective: To examine the risk of ocular complications following radiotherapy in patients with nasopharyngeal carcinoma (NPC)., Methods: We adopted 1:1 propensity score matching and identified an NPC cohort (n = 736) and a comparison cohort (n = 736) that comprised non-NPC head and neck cancer patients who received radiotherapy in the National Health Insurance Research Database from 1997 to 2010. The follow-up period was terminated upon developing ocular complications (International Classification of Diseases, Ninth Revision, Clinical Modification [ICD-9-CM]360-379) or on December 31, 2010., Results: After adjusting for the confounding factors of the study, the NPC cohort had a higher adjusted hazard ratio (HR) for developing ocular complications than the comparison cohort (adjusted HR = 2.786, 95% confidence interval [CI] = 1.805-4.112, P < 0.001). The NPC cohort was associated with a significantly higher risk of developing ocular complications compared with the comparison cohort within 12 and after 24 months of follow-up (P < 0.05). The most common associated ocular complications were optic nerve disorder and retinopathy., Conclusions: Patients with NPC might be at higher risk of developing ocular complications after radiotherapy than non-NPC head and neck cancer patients in Taiwan. Either further investigation or routine assessments by ophthalmological physician is recommended., Level of Evidence: NA Laryngoscope, 130:1270-1277, 2020., (© 2019 The American Laryngological, Rhinological and Otological Society, Inc.)

Previous researches have shown that anesthesia can affect the outcomes of many kinds of cancer after surgery. Here, we investigated the association between anesthesia and patient outcomes after elective open intrahepatic cholangiocarcinoma surgery.This was a retrospective cohort study of patients who received elective open intrahepatic cholangiocarcinoma surgery between January 2005 and December 2014. Patients were grouped according to the anesthesia received, that is, propofol or desflurane anesthesia. Kaplan-Meier analysis was performed and survival curves were constructed from the date of surgery to death. After propensity matching, univariable and multivariable Cox regression models were used to compare hazard ratios for death. Subgroup analyses were performed for tumor node metastasis staging and postoperative metastasis and recurrence.A total of 34 patients (21 deaths, 62.0%) with propofol anesthesia and 36 (31 deaths, 86.0%) with desflurane anesthesia were eligible for analysis. After propensity matching, 58 patients remained in each group. In the matched analysis, the propofol anesthesia had a better survival with hazard ratio of 0.51 (95% confidence interval, 0.28-0.94, P = .032) compared with desflurane anesthesia. In addition, subgroup analyses showed that patients under propofol anesthesia had less postoperative metastases (hazard ratio, 0.36; 95% confidence interval, 0.15-0.88; P = .025), but not fewer postoperative recurrence formation (hazard ratio, 1.17; 95% confidence interval 0.46-2.93; P = .746), than those under desflurane anesthesia in the matched groups.In a limited sample size, propofol anesthesia was associated with better survival in open intrahepatic cholangiocarcinoma surgery. Prospective and large sample size researches are necessary to evaluate the effects of propofol anesthesia on the surgical outcomes of intrahepatic cholangiocarcinoma surgery.

Recent blood distribution profiles for transfusions in Taiwan have not been comprehensively documented. This study aimed to analyze trends in red blood cell (RBC), platelet, and plasma distribution rates, and compares these profiles with those in other countries. The distribution rates of RBC, platelets, and plasma in Taiwan during 2015 were 47.6, 11.1, and 26.8 units per 1000 population, respectively. At least 1.5 and 2.5-fold higher platelet and plasma distribution rates were observed than other selected countries. During 2007-2015, there was no significant change in RBC distribution. However, we observed a significant increase of 0.20 (95% CI: 0.11-0.30) adult doses of platelets, and a significant decrease of 1.69 (95% CI: 1.45-1.93) units of plasma per 1000 population per annum. Seven other countries showed a general significant decreasing trend of RBC distributions. Higher blood distribution rates were observed in Taiwan. Therefore, the adoption of patient blood management is essential., (Copyright © 2019 Formosan Medical Association. Published by Elsevier B.V. All rights reserved.)

To determine the incidence and risk of Parkinson disease (PD) in patients with Sjögren syndrome (SS) according to a nationwide population-based database.In total, 12,640 patients in the SS cohort and 50,560 in the non-SS cohort were enrolled from Taiwan's National Health Insurance Research Database from 2000 to 2010. We used the Cox multivariable proportional hazards model to determine the risk factors for PD in the SS cohort.We observed an increased incidence of PD in patients with SS, with a crude hazard ratio (HR) of 1.40 and an adjusted HR (aHR) of 1.23. The cumulative incidence of PD was 1.95% higher in the SS cohort than in the non-SS cohort. The SS cohort had an elevated HR under medication use, namely cevimeline and pilocarpine (crude HR, 1.28), hydroxychloroquine (crude HR, 1.43; aHR, 1.46), and methylprednisolone (crude HR, 2.21; aHR, 1.49). Patients receiving other non-hydroxychloroquine immunosuppressant therapies had a lower risk (aHR, 0.86) of PD. Furthermore, patients with SS aged 20 to 49 years had a 1.93-fold higher risk of PD than did those without SS (aHR, 1.93). The risk of PD was higher (aHR, 2.20) in patients with SS without comorbidities than in those with comorbidities. The aHR of PD significantly increased when the follow-up period exceeded 9 years (aHR, 1.93).We determined an increased risk of PD in patients with SS. Further investigation is warranted to determine the possible underlying mechanisms and the potential role of non-hydroxychloroquine immunosuppressants in ameliorating PD.

Background: Pelvic fracture with hypovolemic shock is a known crucial injury in trauma patients. Pelvic fracture with vessel injury often leads to hemodynamic complications; in a trauma scenario, evidence of other systems being affected is often absent. Bleeding cessation and resuscitation are important for these types of trauma patients. For this purpose, pelvic angiographic embolization is frequently used. Multiple studies have reported that angiographic embolization may cause erectile dysfunction (ED) in hemodynamically stable patients with pelvic fracture. However, no study has evaluated a large patient cohort with a long-term follow-up. We hypothesized that angiographic embolization to control bleeding may compromise blood supply to the genitourinary organs or cause secondary neurogenic injury that increases the risk of ED. Our goal was to evaluate the risk of ED following pelvic fractures in male patients treated with pelvic angiographic embolization., Methods: We used data from the National Health Insurance Research Database (NHIRD) from 1997 to 2010 provided by the Bureau of National Health Insurance of the Department of Health in Taiwan. We collected disease histories from inpatient files. The disease diagnoses were based on the International Classification of Diseases (ICD), Ninth Revision, Clinical Modification. These data were all deidentified, and we did not contact the patients. As such, informed consent was not needed., Results: Eighty-five and 82,802 patients were included in the case and control cohorts, respectively. All patients were aged 15-45, and the proportion of pelvic fracture locations was equal between the groups. After investigating the causes of ED among male patients aged 15-45 with pelvic fractures using logistic regression analysis in a generalized estimating equations model and after adjusting for the influence of confounders, we found that these patients had high risks (odds ratio (OR): 32.637; 95% confidence interval: 14.137-75.346; P < 0.001) of developing ED post-angiographic embolization., Conclusions: Male patients in Taiwan with pelvic fractures who undergo angiographic embolization to control bleeding have a higher risk of ED than those who do not undergo the procedure. Physicians should practice caution and inform patients of this connection before the procedure.

What We Already Know About This Topic: WHAT THIS ARTICLE TELLS US THAT IS NEW: BACKGROUND:: Previous research has shown different effects of anesthetics on cancer cell growth. Here, the authors investigated the association between type of anesthetic and patient survival after elective colon cancer surgery., Methods: A retrospective cohort study included patients who received elective colon cancer surgery between January 2005 and December 2014. Patients were grouped according to anesthesia received: propofol or desflurane. After exclusion of those who received combined propofol anesthesia with inhalation anesthesia or epidural anesthesia, survival curves were constructed from the date of surgery to death. After propensity matching, univariable and multivariable Cox regression models were used to compare hazard ratios for death. Subgroup analyses were performed for tumor-node-metastasis staging and postoperative metastasis., Results: A total of 706 patients (307 deaths, 43.5%) with desflurane anesthesia and 657 (88 deaths, 13.4%) with propofol anesthesia were eligible for analysis. After propensity matching, 579 patients remained in each group (189 deaths, 32.6%, in the desflurane group vs. 87, 15.0%, in the propofol group). In the matched analyses, the propofol-treated group had a better survival, irrespective of lower tumor-node-metastasis stage (hazard ratio, 0.22; 95% CI, 0.11 to 0.42; P < 0.001) or higher tumor-node-metastasis stage (hazard ratio, 0.42; 95% CI, 0.32 to 0.55; P < 0.001) and presence of metastases (hazard ratio, 0.67; 95% CI, 0.51 to 0.86; P = 0.002) or absence of metastases (hazard ratio, 0.08; 95% CI, 0.01 to 0.62; P = 0.016). Simple propensity score adjustment produced similar findings., Conclusions: Propofol anesthesia for colon cancer surgery is associated with better survival irrespective of tumor-node-metastasis stage.

Background: The purpose of this study was to assess the incidence and risk of depressive disorder among patients with head and neck cancer., Methods: We identified 48 548 patients from the National Health Insurance Research Database (NHIRD) in Taiwan who were newly diagnosed with head and neck cancer between 2000 and 2010. Each patient was randomly frequency-matched with an individual without head and neck cancer, based on index year, sex, age, occupation category, urbanization level, monthly income, and comorbidities. The Cox proportional Registry of Catastrophic Illnesses Patient Database regression analysis was performed to estimate the effect of head and neck cancer on the risk of depressive disorder., Results: Patients with head and neck cancer had a significantly higher risk of depressive disorder than the matched cohort (adjusted hazard ratio [HR] 3.32; 95% confidence interval [CI] 3.05-3.61), with the highest risk seen in the hypopharynx and oropharynx., Conclusion: Patients with head and neck cancer had >3 times the incidence of depressive disorder, relative to the comparison group. Psychological evaluation and support are essential in head and neck cancer survivors., (© 2017 Wiley Periodicals, Inc.)

Esophageal cancers account for majority of synchronous or metachronous head and neck cancers. This study examined the risk of esophageal cancer following percutaneous endoscopic gastrostomy (PEG) in head and neck cancer patients using the Taiwan National Health Insurance Research Database. From 1997 to 2010, we identified and analyzed 1851 PEG patients and 3702 sex-, age-, and index date-matched controls. After adjusting for esophagitis, esophagus stricture, esophageal reflux, and primary sites, the PEG cohort had a higher adjusted hazard ratio (2.31, 95% confidence interval [CI] = 1.09-4.09) of developing esophageal cancer than the controls. Primary tumors in the oropharynx, hypopharynx, and larynx were associated with higher incidence of esophageal cancer. The adjusted hazard ratios were 1.49 (95% CI = 1.01-1.88), 3.99 (95% CI = 2.76-4.98), and 1.98 (95% CI = 1.11-2.76), respectively. Head and neck cancer patients treated with PEG were associated with a higher risk of developing esophageal cancer, which could be fixed by surgically placed tubes.

The effects of the inflammatory mediators involved in systemic lupus erythematous (SLE) on subsequent Parkinson disease have been reported, but no relevant studies have focused on the association between the 2 diseases. This nationwide population-based study evaluated the risk of Parkinson disease in patients with SLE.We identified 12,817 patients in the Taiwan National Health Insurance database diagnosed with SLE between 2000 and 2010 and compared the incidence rate of Parkinson disease among these patients with that among 51,268 randomly selected age and sex-matched non-SLE patients. A Cox multivariable proportional-hazards model was used to evaluate the risk factors of Parkinson disease in the SLE cohort.We observed an inverse association between a diagnosis of SLE and the risk of subsequent Parkinson disease, with the crude hazard ratio (HR) being 0.60 (95% confidence interval 0.45-0.79) and adjusted HR being 0.68 (95% confidence interval 0.51-0.90). The cumulative incidence of Parkinson disease was 0.83% lower in the SLE cohort than in the non-SLE cohort. The adjusted HR of Parkinson disease decreased as the follow-up duration increased and was decreased among older lupus patients with comorbidity.We determined that patients with SLE had a decreased risk of subsequent Parkinson disease. Further research is required to elucidate the underlying mechanism.

Background: The purpose of this study was to examine the risk of nasopharyngeal carcinoma (NPC) after a diagnosis of allergic rhinitis., Methods: We identified 67,532 patients with allergic rhinitis (allergic rhinitis cohort) and a 135,064 control cohort with the same mean age and sex ratio by using a Taiwan Longitudinal Health Insurance Database (LHID) sample from 2000 to 2005., Results: After adjusting for the possible confounding factors of the study, the allergic rhinitis cohort had a 2.33-fold higher risk of developing NPC than did the comparison cohort. The frequency of allergic rhinitis visits was correlated with the risk of subsequent NPC. Patients with 4 or more allergic rhinitis visits per year were significantly associated with increasingly developing NPC risk., Conclusion: Patients with allergic rhinitis might be associated with subsequent NPC in Taiwan. Those who had repeated visits for allergic rhinitis had even higher risk for NPC. Physicians should be aware of the link when assessing patients with allergic rhinitis., (© 2014 Wiley Periodicals, Inc.)

Background: The ability of a new generation commercial, multiplex, multi-dye test from Roche, the cobas TaqScreen MPX test, version 2.0, to detect and identify occult HBV infections was evaluated using routine donor samples from Kaohsiung Blood Bank, Taiwan., Study Design and Methods: A total of 5973 samples were tested by nucleic acid amplification technology (NAT); 5898 in pools of six, 66 in pools of less than six and nine samples individually. NAT-reactive samples were retested with alternative NAT tests, and follow-up samples from the donors were tested individually by NAT and for all the HBV serological markers., Results: Eight NAT-only-reactive donors were identified, and follow-up samples were obtained from six of the donors. The results indicated that all eight donors had an occult HBV infection with viral loads <12 IU/ml., Conclusion: The cobas(®) TaqScreen MPX test, version 2.0, has an advantage over the current Roche blood screening test, the cobas TaqScreen MPX test, for screening donations in countries with a high prevalence of occult HBV infections since the uncertainty associated with identifying samples with very low viremia is removed by the ability of the test to identify the viral target in samples that are reactive with the cobas TaqScreen MPX test, version 2.0., (© 2013 International Society of Blood Transfusion.)

Background and Objective: Individuals with p phenotype lack P1, P(k) and P antigens on red blood cells, presumably as a result of deficiency in the enzyme α(1,4)galactosyltransferase (A4GALT). The aim of this study was to explore the molecular background of a Taiwanese family with p phenotype., Materials and Methods: Blood samples from two p siblings and seven family members were investigated. The coding region of the A4GALT gene was analysed by polymerase chain reaction and direct sequencing. The wild- and mutant-complementary DNAs (cDNAs) of A4GALT were cloned into an expression vector and transfected to Chinese hamster ovary (CHO) cells. P(k) expression on the transfected cells was analysed by flow cytometry and the activities of A4GALT were measured by high-performance liquid chromatography., Results: The two individuals with p phenotype were homozygous for the complex mutation, which was caused by a combined deletion and insertion between nt 418 and 428. No expression of P(k) and no enzyme activity were observed in cells transfected with the mutant construct., Conclusion: The first case of p phenotype in Taiwan was caused by a non-functional allele resulting from a homozygous complex mutation of A4GALT gene., (© 2010 The Authors. Transfusion Medicine © 2010 British Blood Transfusion Society.)

Background: The Rh blood D group provides a clinically important model of aberrant splicing with skipped exons. Approximately 30% of serologically D-negative Chinese individuals have an intact RHD gene (DEL phenotype) and induce allo-immunization in transfusions. The RHD1227GNA polymorphism occurs in >95% DEL phenotype of Asian descent. The effects of RHD 1227A and a novel allele on exon 9 splicing were examined., Results: Amplified DEL RNA products revealed that 3 transcripts involved skipping of exons 8-9, exon 9, or exon 9 with an inserted 170-bp cryptic exon located between exons 7 and 8. A novel, single nucleotide polymorphism was identified in the 7th intron, (IVS7) 923C>T, and present in all DEL patients. The odds ratio of RHD1227G>A allele with DEL phenotype was 2711. Splicing analysis of transcripts from minigenes containing the 1227GNA allele, but not the (IVS7) 923C>T allele, demonstrated aberrant exon 9 skipping., Conclusions: A combined haplotype of 1227G>A and IVS7 923C>T alleles was apparent in >95% DEL Chinese individuals. RHD1227A mutation significantly increased aberrant mRNA splicing, producing a hybrid RHD mRNA lacking exon 9. These results provide a molecular basis of the DEL phenotype in the Chinese population., (Copyright 2010 Elsevier B.V. All rights reserved.)

Molecular genotyping of the ABO alleles has been widely used in ABO subgroups analysis and has been able to solve the rare ABO blood grouping discrepancies. The genotypes of sixty-one A2B phenotype donors recruited from the middle and south of Taiwan were analyzed by means of molecular methods. The A2B phenotype was initially identified by serological test. The polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) method was used to screen the ABO alleles at nucleotides (nt) 261 and 703 based on the nt differences found in the ABO alleles. The subgroups of the A2 allele were determined by the PCR-RFLP and direct sequencing methods. The discrepancies between the phenotype and genotype of the A2B were then studied by subcloning and nucleotide sequence analysis. Our results show that 55 of the 61 A2B donors (90%) are A205/B allele and two are A201/B allele. Four cases were heterozygotes of the cis-AB/O or B alleles. Two were cis-AB04/O allele, one was cis-AB01/O allele and the other was cis-AB02/B allele. In conclusion, most A2B genotypes belong to the A205/B allele in Taiwan. In this study, we report for the first time the presence of the A205, A201, and cis-AB02 alleles in Taiwan.

Background: It was estimated that approximately 25 percent of Taiwanese residents were ABO blood group A. Many subgroups of A, however, revealed ambiguous serologic typing results. This study aimed to delineate the molecular basis of the A3, Ax, and weak A phenotypes., Study Design and Methods: Serologic analyses including adsorption and elution assay, serum transferases activity assay, and saliva test were performed to determine the unique phenotypes of these samples. DNA sequencing and polymerase chain reaction-restriction fragment length polymorphism were performed to further investigate the relationships between the genetic characteristics and phenotypic features of these samples., Results: Three single-nucleotide transitions (745C>T, 820G>A, and a novel 860C>T) were found in nine A3/A3B cases. In addition, the Ax and A3B subjects shared the same 860C>T mutation. This A(x) allele with 860C>T transition expressed A3B phenotype in A(x)/B101 heterozygote but Ax phenotype in A(x)/O01 heterozygote. This allelic enhancement was also observed in the weak A family with Aw05 allele, which was previously not found in Taiwan., Conclusion: This allelic enhancement phenomenon was prone to cause serologic discrepancy between parents and children. Genotyping could help us to resolve this problem. Thus, a novel mutation is reported among Taiwanese blood donors.

The Rhesus (Rh) blood group is the most polymorphic human blood group system, and it is clinically significant in transfusion medicine. About 15% of Caucasoid people are RhD-negative, whereas in the Asian population, the RhD-negative blood type only occurs in 0.1% to 0.5%. However, approximately 30% of apparently RhD-negative Taiwanese people actually were RhD(el). Traditionally, we verify RhD(el) by a serologically adsorption-elution procedure with polyclonal anti-D. In our recent report, RhC phenotype is highly associated with RhD(el), and RHD1227A is a useful genetic marker for RhD(el). For setting up a rapid protocol to detect RhD(el) in clinical laboratory, a total number of 395 Taiwanese serological RhD-negative blood samples, those with RhC (+) phenotypes as selected by serological tests, were further screened by adsorption/elution tests and RHD1227A allele by specific sequence primer-polymerase chain reaction (SSP-PCR) for RhD(el). Among 395 blood samples collected from RhD-negative subjects, the incidence of RhC (+) was 43% (171/395). One hundred and twenty six of the 171 RhC (+) samples were positive for both adsorption/elution for RhD detection and SSP-PCR assay for RHD1227A. The sensitivity and specificity were 96.9% and 97.5%, respectively, for RHD1227A detection as compared with the traditional adsorption/elution test. Our results also indicated that RHD1227A was highly linked to Ce haplotypes (95.2%). In conclusion, combined RhC (+) phenotyping and RHD1227A analysis can be a simple and accurate laboratory screening protocol for RhD(el) detection in RhD-negative population.

Alpha(1,2)-fucosyltransferase catalyzes the transfer of fucose to the C-2 position of galactose on type II precursor substrate Gal beta1-4GlcNAc beta1-R. It plays an important biological role in the formation of H antigen, a precursor oligosaccharide for both A and B antigens on red blood cells. Aberration of alpha(1,2)-fucosyltransferase activity by gene mutations results in decreased synthesis of H antigen, leading to the para-Bombay phenotype. In this study, we collected about 250,000 blood samples in Taiwan during 5 yr and identified the subjects with para-Bombay phenotype. Then we analyzed the sequence of the alpha(1,2)-fucosyltransferase gene by direct sequencing and gene cloning methods, using the blood samples of 30 para-Bombay individuals and 30 control subjects who were randomly selected. The goals of this study were to search for new h alleles, to determine the h allele frequencies, and to test whether the sporadic theory is applicable in Taiwan. Six different h alleles (ha, 547-548 AG-del; hb, 880-881 TT-del; hc, R220C; hd, R220H; he, F174L; and hf, N327T) were observed. Two h alleles, he and hf, were newly discovered in Taiwan. The he allele has a nucleotide 522C>A point mutation, predicting the amino acid 174 substitution of Phe to Leu; the hf allele has missense mutation of nucleotide 980A>C, predicting the amino acid 327 substitution of Asn to Thr. Frequencies of the 6 alleles are ha 46.67%, hb 38.33%, hc 5.00%, hd 1.67%, he 3.33%, and hf 5.00%, respectively. These findings in the Taiwanese population confirm previous observations in other populations that the Bombay and para-Bombay phenotypes are due to diverse, sporadic, nonfunctional alleles, predominantly ha and hb, leading to H deficiency of red blood cells. In contrast to previous reports of non-prevalent associations of h alleles with para-Bombay phenotype, our results suggest a regional allele preference associated with para-Bombay individuals in Taiwan.

Background and Objectives: In addition to the common ABO phenotypes, numerous phenotypes with a weak expression of the A or B antigens on the red blood cells have been found. This study describes the molecular genetic analysis of the Bel phenotype in Taiwanese individuals., Materials and Methods: The exon 6-7 region of the ABO gene of an individual with the Bel phenotype was amplified by the polymerase chain reaction (PCR), cloned, and the sequences of the exons and their adjacent splice sites were analysed. A PCR-based restriction fragment length polymorphism (RFLP) analysis was designed to detect the 502C>T nucleotide change identified in the Bel allele. Six unrelated individuals with the Bel phenotype were analysed, and samples from 40 randomly selected individuals with the common B phenotype were also assessed., Results: All six unrelated Taiwanese individuals with the Bel phenotype were shown to possess a B gene with the 502C>T mutation. The mutation was not detected in the general group B population. The 502C>T nucleotide change predicts an amino acid alteration of Arg168-->Trp in the encoded B transferase., Conclusions: The results suggest a new molecular basis, a 502C>T missense mutation in the B allele, for the Bel phenotype and an association of the Bel502C>T allele with the Bel phenotype in the Taiwanese population., (Copyright 2003 Blackwell Publishing)

Background: The ABO system includes many variant subgroups. Some of them are difficult to identify serologically, leading to mistyping of blood groups. For example, Bel is often typed as O blood group., Study Design and Methods: DNA sequencing and a molecular approach were explored to accurately determine the genotypes of Bel subgroups. Seven Bel blood donors and 106 individuals with other blood groups were analyzed serologically and molecularly., Results: The serologic results of these seven Bel blood donors showed that their RBCs do not react with anti-B or anti-A,B, and their B antigen was detected by adsorption and elution methods. Sequencing results for exons 6 and 7 of ABO genes showed a new Bel allele with a C>T substitution at nucleotide position 502 in exon 7 of the ABO gene in all seven cases but not in other blood groups. Consequently, an amplification-created restriction site protocol was designed to detect the 502C>T genotype in Bel subgroup cases., Conclusion: A novel 502C>T mutation was found in the Bel subgroup in Taiwan and successfully developed a rapid and accurate molecular protocol to detect this mutation. To our knowledge, the new Bel allele that was found is unique in Taiwanese residents.

A total of 50,000 patients were surveyed for the presence of monoclonal immunoglobulins during the past two decades. There were 411 cases of monoclonal gammopathies including 243 cases of plasma cell neoplasms and 168 cases of secondary plasma-cell dyscrasia. Among the 227 cases of multiple myeloma and Waldenström's macroglobulinemia, there were 49.3% IgG class, 22.9% IgA class, 9.7% IgM class and 13.2% light chain type. In addition, there were 1.3% of nonexcretory myeloma including an IgM type. A relatively high frequency (4.8%) of IgD M-proteins was detected but heavy chain disease was not encountered in the present series. Purified M-components from patients with possible autoimmune manifestations were subjected to immunofluorescence studies. Autoimmune activity of M-proteins was found in a patient of Waldenström's macroglobulinemia with peripheral neuropathy, and another patient of cryofibrinogenemia with recurrent purpura and gangrene. In conclusion, a high frequency of IgD myeloma is found in Chinese patients of this area. M-components may have autoimmune activity resulting in unusual clinical manifestations.

The first case of AIDS positively identified in a non-foreigner in Taiwan was a 25-year-old unmarried male who had practiced homosexuality for ten years. The patient began to have abdominal pain accompanied with loose stools and weight loss in June 1985, followed by fever, cough, headache, dizziness, and loss of memory. Facial hyperpigmentation and extensive oroesophageal candidiasis were noted. Laboratory studies showed severe lymphopenia with a reversed T-helper to T-suppressor ratio, cutaneous anergy and polyclonal gammopathy. Human immunodeficiency virus (HIV) antibodies were positive by ELISA and Western blot, and the virus was isolated from the blood. At autopsy, disseminated cytomegalovirus infection, extensive CNS toxoplasmosis and early lesions of Kaposi's sarcoma were demonstrated. The detection of HIV in the adrenal medulla supports the consensus that the virus is neurotropic.

A total of 2321 serum samples were collected from a variety of AIDS risk groups. The samples were tested for anti-HTLV-III using ELISA. Low prevalences of seropositivity were detected in the high-risk groups. Among 120 homosexuals/bisexuals, one seropositive suffering from full-blown AIDS was an American traveler. Another native homosexual who yielded a high ELISA reading was a "possible" AIDS. Three hemophiliacs with strongly positive reactions received injections of a U.S.-produced factor VIII; all are asymptomatic currently. A patient with hairy cell leukemia had a markedly elevated ELISA reading. Another patient with syphilis also had a markedly high reading, despite having no other known risk factor. Among the low-risk groups a patient who had suffered from oral candidiasis for eight years had no risk factor, yet he yielded repeatedly high ELISA readings. Another patient with a weak positive reaction was a symptomless blood donor. This preliminary study concludes that Taiwan is several years behind the U.S. and is at present not an endemic area for AIDS. To achieve successful AIDS control in the future, however, the homosexuals in Taiwan should change their sexual behavior now. Since many initially weakly-reactive samples became negative on repeated testing it appears that non-specific or false positive ELISA reactions occur frequently. Accordingly when ELISA is used for diagnostic purposes, additional confirming tests are mandatory.