Dietary supplementation of synbiotic Leuconostoc mesenteroide B4 and dextran improves immune regulation and disease resistance of Penaeus vannamei against Vibrio parahaemolyticus.

White shrimp (Penaeus vannamei) is an important culture species in Taiwan but often encounters disease infection by Vibrio parahaemolyticus that cause acute hepatopancreatic necrosis disease (AHPND). This study investigates the effects of dietary supplementation of Leuconostoc mesenteroide B4 and its fermentate (dextran) on the immune response, intestinal morphology, disease resistance, and immune-related gene expression in white shrimp. In comparison to the control group, the shrimp fed with a diet containing B4+dextran (107 CFU B4/g feed and 0.05% dextran) for 14, 28, 42 and 56 days had a significantly higher feed efficiency, weight gain and specific growth rate. A significantly higher villus height in the intestine and higher survival rate after challenging with V. parahaemolyticus was recorded for the B4+dextran group. Flow cytometry analysis demonstrated that the group that had ingested B4+dextran had a higher total hemocyte count and a higher proportion of semi-granulocytes, but a lower percentage of granulocytes compared to the control group. The shotgun metagenomic results in the midgut revealed that Leuco. mesenteroides was barely found in the midgut of the shrimp, suggesting that this microbe and its transient presence in the midgut is not the direct mechanism underlying the improved shrimp growth in the treated sample. Instead, dextran, a key ingredient in the B4 fermentate, on the dynamic of the microbial populations in shrimp, possibly promoting the diversity of gut microbes, especially the beneficial microbes, and thereby rendering protection against AHPND. In terms of comparing the gene expression between the control and synbiotic groups, pre- and post-bacterial challenge, a higher expression level of immune genes was mostly found in the B4+dextran group after challenging it with V. parahaemolyticus (group B4+dextran-VP) in the hepatopancreas and hemocyte. In contrast, the transcript level of immune-related genes was found to be higher in the B4+dextran group than other combinations in the midgut. Taken together, this study found that dietary addition of synbiotic Leuco. mesenteroides B4 and dextran can improve the growth performance, intestinal morphology and microbiome, regulation of immune genes and disease resistance against V. parahaemolyticus infection in white shrimp.

[A Project to Improve the Time-Effectiveness of Routine Morning Blood Tests].

BACKGROUND & PROBLEMS: Morning blood sampling, conducted around 4am to 5am, is routinely ordered due to nothing-by-mouth (NPO) requirements and clinical practice norms. However, this routine may interrupt the normal sleep of patients and decrease the satisfaction of patients with the care received. However, our night-shift staff is not able to take all blood samples during this time, resulting in a high rate of overdue reporting. PURPOSE: To reduce the overdue rate of laboratory result from 62.3% to less than 19.3%. RESOLUTION: In 2013, we performed interventions including the process reengineering of blood sampling to meet the needs of laboratory result flows and the upgrading of equipment in our neurology ward. The reengineering strategies used included the redistribution of blood sampling times in order to decrease testing for unnecessary items. The equipment upgrades improved the ability of the carrier in the pneumatic tube systems to carry the chilled blood samples. An independent two-sample t-test was used to compare the overdue rate before and after the interventions. RESULTS: The overall overdue rate for the blood sampling to laboratory flow improved from 62.3% before the intervention to 18.0% (t = 7.07, p < .001) after the intervention. Furthermore, the overdue rate for the testing done at the stat laboratory improved from 66.9% to 21.3% (t = 7.36, p < .001), while the overdue rate for the testing done at the non-stat laboratory improved from 52.4% to 17.8% (t = 5.37, p < .001). Besides, the satisfaction of both inpatients and nurses with regard to blood-sampling flow improved from 51% and 54.2%, respectively to 93% (t = 51.10, p < .001) and 94% (t = 12.26, p <.001). CONCLUSIONS: Our process reengineering strategies and the upgrading of the pneumatic tube system successfully reduced the overdue rate for the blood sampling and increased the satisfaction of inpatients.

Cystic fibrosis: experience in one institution.

Cystic fibrosis (CF) is one of the most common autosomal recessive inherited disorders among Caucasians. Comparatively, it is considered to be a rare disease among Asians. To date, only a few cases of Taiwanese CF have been published. We report four CF cases from three families. Case 1 was the first report of CF associated with a homozygosity for the CF transmembrane conductance regulator gene (CFTR gene) mutation 3849+10kb C->T in a Taiwanese patient. Cases 2 and 3 had heterozygous c. 1898+5 G->T and heterozygous p. I1023R (novel mutation) for the CFTR gene mutation. Case 4 was homozygous for the CFTR gene mutation R553X being reported in 2005 and complicated with cor pulmonale. These four patients had received 300 mg bid aerosolized tobramycin treatment every other month.

Clinical manifestations and BTK gene defect in 4 unrelated Taiwanese families with Bruton's disease.

BACKGROUND AND OBJECTIVE: X-linked agammaglobulinemia (XLA, also called Bruton's disease) is is an X-linked recessive disorder characterized by recurrent bacterial infections, usually occurring in the first few years of life. Here, we report the results of a BTK gene mutation screening study that was performed in Taiwanese families with the BTK gene defect to further understand the inheritance patterns of XLA patients in Taiwan and to avoid new cases of XLA within families. MATERIALS AND METHODS: In this study, 52 members of 4 unrelated Taiwanese families with the BTK gene defect were enrolled. We studied the immunologic reports of 6 symptomatic living male patients with confirmed BTK gene defects and correlated the findings with their clinical symptoms. The genomic DNA of the subjects was subjected to direct sequencing mutation analysis. RESULTS: We screened 52 members of 4 unrelated Taiwanese families with the BTK gene defect for BTK gene mutation and found that there were 6 symptomatic living patients with a confirmed defect, 7 symptomatic deceased patients highly suspected to have had the defect and 11 asymptomatic female carriers. CONCLUSIONS: This is the first report in a series of the thorough screening for the BTK mutation and its carrier status in 4 unrelated Taiwanese families. One pedigree of our study comprises 4 generations. A complete BTK gene mutation study for the patient's family members is strongly suggested.

Type I hereditary angioedema in Taiwan -- clinical, biological features and genetic study.

BACKGROUND: Hereditary angioedema (HAE) is a rare, autosomal dominant inherited disease which is caused by a genetic deficiency of C1 esterase inhibitor (C1 INH). There have only been a few case reports in Taiwan to date. OBJECTIVE: To describe the clinical features of type I HAE in Taiwanese patients. METHODS: Three unrelated Taiwanese families with type I HAE are reported, and one case of a family from a review of PubMed was reviewed. Clinical manifestations, diagnostic examinations, management and genetic studies were analyzed. RESULTS: Including this report, 19 patients had low C1 INH and low C4 levels and were diagnosed with type I HAE. Only 11 (57.9%) patients were symptomatic. Recurrent skin swelling and edema over the four extremities or trunk were reported in all symptomatic patients (100%). 45.5% of the patients recalled laryngeal attacks and one patient died from asphyxia. 18.2% of the patients experienced abdominal symptoms. The age at the beginning of clinical symptoms ranged from 5 to 30 years (mean +/- SD: 20.82 +/- 7.88 years). The diagnosis tended to be delayed (range from 1 to 39 years; mean +/- SD: 8.45 +/- 11.04 years). Nine patients had a mutant C1 INH gene, and two patients received long-term prophylaxis with danazol. CONCLUSION: The prevalence of hereditary angioedema in Taiwan is low. Persons with low levels of C1 INH who were clinically symptomatic accounted for only 57.9% of the cases in our study, which is far lower than previous reports from other countries. Ethnic differences may be the reason for this finding. Further genomic studies are needed to elucidate the genetic penetrance of C1 INH deficiency in Taiwan.

Factors for high-risk asthma in Taiwanese children.

BACKGROUND: Asthma is one of the major causes of death in otherwise healthy young individuals. However, many of these deaths may have been prevented by more aggressive treatment. To determine factors correlated with a high risk of death in Taiwanese children with atopic asthma. METHODS: Taiwanese children aged 5-18 years, diagnosed with atopic asthma were enrolled in the study. Atopic asthma was diagnosed and immunoglobulin E (IgE) specific to antigens from any 1 of 8 allergens was measured (i.e. Dermatophagoides pteronyssinus, Dermatophagoides farinae, cat and dog dander, cockroach, egg white, milk and fish). High-risk asthma was defined as asthma requiring admission to a hospital or a visit to an emergency department. The study tried to determine the association of high-risk asthma with allergy-related parameters (e.g. asthma severity, asthma score, total serum IgE levels, serum levels of allergen-specific IgE, eosinophil count) and pulmonary function in Taiwanese children. RESULTS: One thousand one hundred and twenty-two Taiwanese children were evaluated. Those with higher asthma severity, asthma symptom score, serum levels of IgE specific to D. pteronyssinus and D. farinae, higher total serum IgE levels, and lower FEF25-75% (forced expiratory flow, 25-75%) values were considered to be members of the high-risk asthma group. CONCLUSIONS: The characterization of risk factors has enabled us to identify high-risk asthma in Taiwanese children, which will facilitate the treatment of these children in the future.

Juvenile scleroderma: experience in one institution.

BACKGROUND: Scleroderma is a chronic connective tissue disease characterized by hardened or scaly skin and widespread abnormalities of the viscera, which is rare in the pediatric age group. OBJECTIVE: In this study, we retrospectively reviewed 23 pediatric patients suffering systemic (SSc) and localized (LS) scleroderma. METHODS: Twenty-three patients were enrolled and were diagnosed with SSc or LS from March 1993 to September 2009 in the Department of Pediatrics at Mackay Memorial Hospital in Taipei, Taiwan. These diagnoses were based on the criteria of the American College of Rheumatology and the clinical manifestations of hard skin. Data recorded included sex, age-at-onset, age-at-diagnosis, laboratory data, family history, trauma history, treatment, and outcomes. RESULTS: Three patients suffered SSc and 20 patients had LS, including 16 girls and 7 boys. Mean age-at-onset was 6.55 +/- 3.28 years old. Antinuclear antibodies were positive in 15 patients. Tests for anti-Scl-70 antibodies were positive in 1 patient with SSc. One boy had en coup de sabre combined with a posterior fossa tumor. Twenty-two patients were treated with D-penicillamine. Oral prednisolone and methotrexate were added, if indicated. One girl with LS developed proteinuria after D-penicillamine treatment. All patients with localized disease ultimately documented a softening of their skin lesions. CONCLUSIONS: While scleroderma is rare in children, the prognosis of SSc is poor but better than for adults. The prognosis for LS is usually benign, however, the skin may become progressively indurated and it may not only be a skin disease. No progression from LS to SSc was observed in our study.