Kaohsiung Journal of Medical Sciences - Articles in Press

A novel SRD5A2 mutation in a Taiwanese newborn with ambiguous genitalia - Corrected Proof

Meng-Che Tsai, Yen-Yin Chou, Shio-Jean Lin, Li-Ping Tsai — 2012-02-20

Abstract: The 5α-reductase type 2 deficiency is a rare autosomal recessive 46,XY disorder of sex development caused by the mutated 5α-reductase type 2 (SRD5A2) gene. In this disease, defective conversion of testosterone to dihydrotestosterone leads to variable presentations of male ambiguous genitalia during fetal development. The most crucial clinical decision for the affected individual is proper gender assignment; therefore, a prompt and correct diagnosis is important. In this present study, we report a normal male karyotype manifesting microphallus, bifid scrotum/labia majora with bilateral palpable gonads, and a blind-ended pseudovagina. The mutation analysis of the SRD5A2 gene revealed one novel C to T transition changing glutamine to a stop codon at codon 71 (p.Q71X) in exon 1 and one known G to A transition changing arginine to glutamine at codon 227 (p.R227Q) in exon 4. The p.Q71X mutation presumably results in a truncated protein, while the p.R227Q mutation is conceived to impair enzyme function and has been reported in patients of East Asian descent. This report demonstrates the essential role of hormonal and molecular studies for genetic counseling and gender assignment in males with pseudovaginal disorder of sex development, and our report helps identify a novel SRD5A2 gene mutation in the Taiwanese population.

Clinical experience in diagnosis and treatment of malignant gastrointestinal stromal tumors - Corrected Proof

2012-02-20

Abstract: This study investigated the clinical pathologic character of malignant gastrointestinal stromal tumors (MGIST), their treatment with surgery, and evaluated the efficacy of imatinib postoperation. A total of 68 MGIST patients were enrolled. Of these, 27 patients underwent imatinib auxiliary therapy (treatment group) and 41 underwent imatinib therapy (control group). The therapeutic effects on the two groups were compared using χ2 test analysis after follow-up of two years. The expressions of CD117, CD34, S100, Vimentin, and alpha smooth-muscle actin (SMA) were detected by immunohistochemistry methods. Of the 68 cases, 28 showed potential MGIST, whereas 40 had MGIST. Haemorrhagia or necrosis, abundant cell, manifest heteromorphism, and caryocinesia were observed in varying degrees. The positive rates of CD117, CD34, Vimentin, S100, and SMA were 89.7% (61/62), 88.2% (60/62), 73.5% (50/62), 41.1% (28/62) and 25.0% (17/62), respectively. The recurrence rate in the treatment group was significantly lower than that in the control group (p < 0.01). We concluded that CD117 and CD34 may be the most valuable markers in the diagnosis of MGIST, and the diagnosis of MGIST depends on the pathology. Surgery is a far better approach in the treatment of such patients, and imatinib is the more efficient target drug in preventing recurrence and metastasis.

Giant cell tumor of the parotid gland - Corrected Proof

Cheng-Jung Wu, Sau-Tung Chu, Wei-wen Yu, Pi-Hsiung Wu — 2012-02-20

In their article “Features of parotid gland diseases and surgical results in southern Taiwan,” Kuo et al discussed the features of parotid tumors in southern Taiwan . The most common benign parotid tumor was the pleomorphic adenoma. In malignant parotid tumor disease, the most common tumor was the acinic cell carcinoma. In their study, 25.7% of patients with a malignant parotid tumor received radiotherapy. No patient in the benign group received radiotherapy. We report a new case in which a patient with a benign parotid tumor received radiotherapy following a radical parotidectomy.

Mood stabilizers for the treatment of behavioral and psychological symptoms of dementia: An update review - Corrected Proof

Yi-Chun Yeh, Wen-Chen Ouyang — 2012-02-15

Abstract: Behavioral and psychological symptoms of dementia (BPSD) are common and debilitating problems, but current treatments are limited. Antipsychotic agents show some efficacy on BPSD, but their use is limited by the associated risk of cerebrovascular events and mortality. Reports have shown the efficacy of mood stabilizers on BPSD, but systemic reviews on this issue are scant. This article aims to review studies of the efficacy of mood stabilizers on BPSD, and the quality of the available evidence. We searched for articles published in English during the period 1990 to 2010 and included in the PubMed database that concerned treatment of BPSD with mood stabilizers, such as carbamazepine, valproate, gabapentin, topiramate, lamotrigine, oxcarbazepine and lithium. The quality of the studies was assessed by considering the trial designs, analyses, subjects and results. We found one meta-analysis and three randomized controlled trials (RCTs) supporting the efficacy of carbamazepine in managing global BPSD, particularly aggression and hostility. With regard to valproate, current evidence from one meta-analysis and five RCTs did not strongly support its efficacy for global BPSD, including agitation and aggression. Only open trials or case series showed some efficacy of gabapentin, topiramate and lamotrigine in controlling BPSD. The single RCT investigating the effect of oxcarbazepine on agitation and aggression showed negative results. Case series reports on lithium tended to show it to be ineffective. Thus far, among mood stabilizers, carbamazepine has the most robust evidence of efficacy on BPSD. More RCTs are needed to strengthen evidence regarding the efficacy of gabapentin, topiramate and lamotrigine. Valproate, oxcarbazepine and lithium showed low or no evidence of efficacy. Large and well designed RCTs focusing on specific symptoms of BPSD are needed to deal with the issue.

Refractive outcome of premature infants with or without retinopathy of prematurity at 2 years of age: A prospective controlled cohort study - Corrected Proof

Ching-ju Hsieh, Jai-wen Liu, Jen-shang Huang, Keh-chung Lin — 2012-02-13

Abstract: This study evaluated the extent to which refractive morbidity is correlated to preterm birth or retinopathy of prematurity (ROP) itself, or both, and examined the risk factors associated with refractive errors in a cohort of preterm infants with and without ROP compared with full-term infants. This longitudinal, prospective, controlled cohort study enrolled 109 infants, including 74 preterm and 35 full-term infants. Infants were divided into the following groups: no ROP, regressed ROP, laser-treated threshold ROP, and full-term. Cycloplegic refraction was determined at 6 and 24 months’ corrected age. Multiple regression models, analysis of variance (ANOVA) with post hoc comparisons, paired t test, and the χ2 test were used for data analysis. ROP status was highly predictive of significant refractive errors in preterm infants. Eyes with laser-treated threshold ROP had significant myopia at both ages (mean spherical equivalent [MSE] in right eye at both refractions −0.72, −1.21 diopters [D]), astigmatism (MSE −1.62, −1.80 D), and anisometropia (MSE 0.82, 1.02 D; ANOVA p 0.05). Thus, the persistent hyperopic status across ages in patients with regressed ROP and in patients without ROP differed significantly (paired t test p > 0.05) from that in the full-term infants, with a reduction in hypermetropia noted for the first 2 years of life (paired t test p < 0.05). The incidence and magnitude of significant refractive errors increased with severe ROP and with age. Although the emmetropization process of preterm birth, including regressed ROP and no ROP, differed from full-term birth in early infancy, we found no differences in the refractive status after 1 year in patients with regressed ROP and in patients without ROP, who were at risk of developing ametropia similar to that of full-term patients. Therefore, apart from laser-treated ROP, children with regressed ROP and without ROP can likely be observed with a verbal vision screening at 3–4 years of age.

Myelodysplasia followed by Good's Syndrome: A unique manifestation associated with thymoma - Corrected Proof

2012-02-13

Abstract: Good’s syndrome, also known as thymoma with combined immunodeficiency, is rare. The immunodeficiency may precede, arise concurrently with or follow the diagnosis of thymoma. In addition to myasthenia gravis and Good’s syndrome, paraneoplastic syndromes associated with thymoma can also be manifested with hematological disorders, such as pure red cell aplasia, aplastic anemia, agranulocytosis, hemolytic anemia, pernicious anemia, and paroxysmal nocturnal hemoglobinuria. Myelodysplastic syndrome is a group of clonal hematopoietic stem cell diseases characterized by cytopenia(s), dysplasia in one or more lineages, ineffective hematopoiesis, and potential precursors of acute leukemia. One proposed pathogenesis of myelodysplasia is autoantibodies that directly reject against hematopoietic cells, but this situation is rare in thymoma. Herein, we report a thymoma patient with unique paraneoplastic syndromes who developed myelodysplasia prior to Good’s syndrome. Early and accurate diagnosis of myelodysplastic syndrome is important for disease management, especially in patients whose myelodysplastic syndrome is possibly derived from autoimmunity. For thymoma patients with recurrent infections, comprehensive immunologic studies to exclude the possibility of Good’s syndrome and prophylactic intravenous immunoglobulin infusion in suitable candidates are warranted.

Inlay buccal mucosal graft for reoperative posterior urethroplasty - Corrected Proof

Shou-Hung Tang, Chien-Chang Kao, Seng-Tang Wu, En Meng, Tai-Lung Cha — 2012-02-13

Abstract: Posterior urethral distraction injury following major pelvic trauma is a surgical challenge. Although rarely seen, cases of failure after formal urethral reconstruction are even more problematic. We adapted the concept of augmented free buccal mucosal grafts, which have been successful in anterior urethroplasty, for repairing the posterior urethra in these rare cases with the aim of reducing the likelihood of penile chordee postoperatively. During 2007–2009, four patients were candidates for the proposed procedure because they had received formal transperineal urethral reconstruction but were unable to urinate through the urethra. The urethra was approached transperineally and opened in the midline, rather than divided. Buccal mucosal grafts of an appropriate size were placed in the created urethral groove from 4- to 8 o’clock in the lithotomy view. After the procedure, the urethral catheter was kept for 3 weeks. All patients voided through the urethra after the procedure. The maximal postoperative urinary flow rates were between 12–15 ml/seconds in all cases for a follow-up period of 18–30 months. The recurrence rate was 50% (2/4). Recurrent strictures were minor, and they showed a web-like stricture ring near the suture line. Restricture within 6 months of surgery responded well to endoscopic internal urethrotomy plus dilatations. In conclusion, without further compromising urethral length, reoperative posterior urethroplasty with the inlay grafting technique can be considered in selective cases.

An unusual slow-growing malignant orbital solitary fibrous tumor - Corrected Proof

Tai-Chi Lin, Yu-Chi Lin, Shu-Lang Liao, Fenq-Lih Lee — 2012-02-13

In their articles “Solitary fibrous tumor of the orbit”, Liu et al, Luo et al, and Hsu et al reported the first three cases of solitary fibrous tumor of the orbit (SFT) in Taiwan . We report here the fourth case and the first malignant SFT in Taiwan.

Hereditary hemorrhagic telangiectasia presenting as portal hypertension - Corrected Proof

Chia-Hung Chao, Shih-Chang Chuang, Jong-Shyong Chen, King-Teh Lee — 2012-02-13

Hereditary hemorrhagic telangiectasia (HHT), also known as Osler–Weber–Rendu syndrome, is a rare genetic vascular disorder characterized by widespread telangiectases that may involve the skin, mucous membranes of the nasopharynx, lung, brain, liver, and urinary and gastrointestinal tracts .

Soft-tissue profile changes after orthognathic surgery of mandibular prognathism - Corrected Proof

Chun-Ming Chen, Steven Lai, Huey-Er Lee, Ker-Kong Chen, Kun-Jung Hsu — 2012-02-10

Abstract: During surgical correction of facial deformities, accurate prediction of the resulting facial profile is important for the patient and the surgeon. The purpose of the present study was to investigate profile changes after surgical treatment of mandibular prognathism. Thirty patients (20 females and 10 males; ages 17–28 years) with mandibular prognathism underwent vertical ramus osteotomy. Preoperative and postoperative cephalograms were analyzed; landmarks were identified and compared. The mean horizontal setback of the pogonion (Pog) was 11.7mm. The setback ratios of labrale inferius (Li)/incision inferius (Ii), labiomental sulcus (Si)/point B, and soft tissue pogonion (PogS)/pogonion (Pog) were 0.98, 0.99, and 0.95, respectively. There were no sex-related changes in soft tissue. These findings indicate that changes in soft tissue closely correlate with the amount of mandibular setback in the horizontal direction. Such information might facilitate more accurate prediction of the outcome of orthognathic surgery.

Isocitrate dehydrogenase mutation hot spots in acute lymphoblastic leukemia and oral cancer - Corrected Proof

Jen-Yang Tang, Chun-Chi Chang, Pei-Chin Lin, Jan-Gowth Chang — 2012-01-24

Abstract: Isocitrate dehydrogenase (IDH) encodes a nicotinamide adenine dinucleotide phosphate+-dependent enzyme for oxidative decarboxylation of isocitrate and has an essential role in the tricarboxylic acid cycle. Mutations of IDH1 and IDH2 have been identified in patients with glioma, leukemia, and other cancers. However, the incidence of IDH mutations in acute myeloid leukemia in Taiwan is much lower than that reported in Western countries. The reason for the difference is unknown and its clinical implications remain unclear. Acute lymphoblastic leukemia (ALL) is a heterogenous hematopoietic malignancy. Oral squamous cell carcinoma (OSCC) results from chronic carcinogen exposures and is highly prevalent in trucking workers, especially in southern Taiwan. Subtypes of both diseases require specific treatments, and molecular markers for developing tailored treatments are limited. High-resolution melting (HRM) analysis is now a widely used methodology for rapid, accurate, and low-cost mutation scanning. In this study, 90 adults with OSC and 31 children with ALL were scanned by HRM analysis for IDH1 and IDH2 mutation hot spots. In ALL, the allele frequency was 3.23% in both IDH1 and IDH2. In OSCC, the allele frequency was 2.22% in IDH2. A synonymous mutation over pG313 (c.939A > G) of IDH2 was found in both pediatric ALL and adult OSCC. Therefore, we concluded that mutations of IDH are uncommon in ALL and OSCC and are apparently not a major consideration when selecting treatment modalities.

Comparison of Gd-Bz-TTDA, Gd-EOB-DTPA, and Gd-BOPTA for dynamic MR imaging of the liver in rat models - Corrected Proof

2012-01-20

Abstract: To evaluate the competitive potential of a new lipophilic paramagnetic complex, Gd-Bz-TTDA [4-benzyl-3,6,10-tri (carboxymethyl)-3,6,10-triazado-decanedioic acid] compared with two other commercially available MR hepatobiliary contrast agents, gadobenate dimeglumine (Gd-BOPTA) and gadoxetic acid (Gd-EOB-DTPA), dynamic MR imaging studies were performed on normal and hepatocellular carcinoma (HCC) rat models using a 1.5-Tesla MR scanner. The results indicate that normal rats that were injected with 0.1 mmol/kg Gd-Bz-TTDA showed significantly more intense and persistent liver enhancement than those that were injected with the same dose of Gd-EOB-DTPA or Gd-BOPTA. All of these agents showed similar enhancement patterns in the implanted HCC. The liver-lesion contrast-to-noise ratios were higher and more persistent in rats that were injected with Gd-Bz-TTDA. These results indicate that Gd-Bz-TTDA is comparable with the commercially available hepatobiliary agents, Gd-EOB-DTPA and Gd-BOPTA, and can result in more intense and prolonged liver enhancement while still providing better liver-lesion discrimination. These results warrant further large-scale studies.

Overexpression of annexin 1 in the development and differentiation of urothelial carcinoma - Corrected Proof

Wan-Yi Kang, Wan-Tzu Chen, Ya-Chun Huang, Yue-Chiu Su, Chee-Yin Chai — 2012-01-19

Abstract: This study investigates the expression of annexin 1 in urothelial carcinoma (UC) and its relation with clinicopathologic factors, and evaluates its potential clinical significance. Annexin 1 expression was analyzed by immunohistochemical staining with manual tissue microarrays and Western blot in UC. Immunohistochemical analysis of UC in tissue microarrays showed that annexin 1 protein was 76.5% (150/196) positive, which was markedly increased compared with that in the normal urothelium 20.8% (5/24) (p < 0.01). In addition, the positive expression rate of annexin 1 was higher in the high-grade UC (81.7%; 143/175) than in the low-grade UC (33.3%; 7/21). Western blot revealed that the expression of annexin 1 was low in low-grade UC, and markedly increased in high-grade UC. In conclusion, annexin 1 overexpression is observed in UC, which suggests it may be associated with tumorigenesis and its expression correlates with the differentiation of UC.

Severity of fatty liver on ultrasound correlates with metabolic and cardiovascular risk - Corrected Proof

2012-01-19

Abstract: Non-alcoholic fatty liver disease is associated with an increased risk of metabolic and cardiovascular diseases. Whether the severity of fatty liver on ultrasound correlates with metabolic or cardiovascular risk remains unclear. A total of 1000 people receiving health examinations were enrolled, and 126 were excluded due to the presence of HBsAg, anti-HCV, known hepatic disorders or alcohol use (>140 g/wk). Significant fatty liver consisted of moderate and severe fatty liver on ultrasound. The definition of central obesity was modified to a waist circumference of >90 cm in men and >80 cm in women. Framingham risk score was used to estimate the risk of cardiovascular disease. A total of 874 subjects (485 women and 388 men with a mean age of 52.07 ± 11.68 years) were included in the final analysis. By using logistic regression analyses stratified by gender, the odds ratio for the prevalence of diabetes mellitus, metabolic syndrome and risk of cardiovascular disease increased with increasing fatty liver status in both genders (p ≤ 0.001). The difference was not only present between individuals with fatty liver vs. non-fatty liver but also between the mild fatty liver and significant fatty liver groups (p < 0.05). In conclusion, the severity of fatty liver on ultrasound could be useful for the risk stratification of metabolic syndrome, diabetes mellitus and cardiovascular disease in clinical practice.

Adenoma malignum of the uterine cervix: Clinicopathologic analysis of 18 cases - Corrected Proof

2012-01-19

Abstract: The aim of this study was to evaluate the clinical and pathologic features of a rare tumor (adenoma malignum, AM). We retrospectively analyzed the medical records of 18 patients diagnosed with AM at a single institute between March 1992 and November 2009. The median age of the patients was 45.8 years (range 29–76 years) and the mean follow-up period was 49.2 months (range 4–168 months). A preoperative cytologic diagnosis (Papanicolaou smear) of AM was made in 22.2% (4/18) of the cases. Ten (55.6%) of the 18 patients were misdiagnosed with other benign diseases and underwent hysterectomies, which confirmed AM. Sixteen patients with AM were in the early stage (IB1, 11/18; IB2, 5/18) and the other two patients were in the advanced stage. Fourteen of 18 patients (77.8%) had pure AM alone. Adjuvant therapy was administered to eight of the patients (44.4%) with AM. The recurrence rate was zero, but the disease progressed in two of the patients (11.1%), who died of the disease. The 5-year survival rate was 88.8%. A cytological diagnosis of AM based on a Papanicolaou smear is rarely made; a deep biopsy (cone biopsy or endocervical curettage) is necessary to diagnose this rare tumor preoperatively when there is any clinical suspicion of AM.

Evaluation of the biocompatibility of a coating material for an implantable bladder volume sensor - Corrected Proof

2012-01-19

Abstract: As the applications for implantable medical devices have increased, the need for biocompatible packaging materials has become important. Recently, we reported an implantable sensor for real-time monitoring of the changes in bladder volume, which necessitated finding a safe coating material for use in bladder tissue. At present, materials like polyethylene glycol (PEG), polydimethylsiloxane (PDMS) and parylene-C are used in biomedical devices or as coating materials, owing to their excellent safety in various medical fields. However, few studies have assessed their safety in bladder tissue, therefore, we evaluated the biocompatibility of PEG, PDMS and parylene-C in the bladder. All three materials turned out to be safe in in vitro tests of live/dead staining and cell viability. In vivo tests with hematoxylin and eosin and immunofluorescence staining with MAC387 showed no persistent inflammation. Therefore, we consider that the three materials are biocompatible in bladder tissue. Despite this safety, however, PEG has biodegradable characteristics and thus is not suitable for use as packaging. We suggest that PDMS and parylene-C can be used as safe coating materials for the implantable bladder volume sensor reported previously.

Antibiotic-treated Streptococcus sanguinis intracranial mycotic aneurysm - Corrected Proof

Ya-Ting Chang, Cheng-Hsien Lu, Chun-Chung Lui, Wen-Neng Chang — 2012-01-18

Abstract: We report the case of a woman 33 years of age with infective endocarditis-related intracranial mycotic aneurysm (IMA) caused by a Streptococcus sanguinis infection. The clinical diagnosis was confirmed by the findings of magnetic resonance imaging, magnetic resonance angiographic (MRA) studies, echocardiographic studies, and a positive blood culture result. She underwent antimicrobial treatment only (ceftriaxone intravenously for 8 weeks and penicillin V orally for 6 weeks). The regression and disappearance of IMA were demonstrated by a series of MRA follow-up studies.

Tyrosine kinase receptor inhibitor-targeted combined chemotherapy for metastatic bladder cancer - Corrected Proof

Chia-Lun Wu, Szu-Yuan Ping, Cheng-Ping Yu, Dah-Shyong Yu — 2011-09-26

Abstract: Overexpression of hypoxia-inducible factor-1 alpha is noted during the invasive and metastatic process of transitional cell carcinoma. It will upregulate vascular endothelial growth factor (VEGF) and drive proliferation, invasiveness, metastasis, and antiapoptotic ability of cancer cells. We proposed that tyrosine kinase receptor inhibitor, sunitinib malate—(Sutent; Pfizer Inc., Taiwan), combined with chemotherapeutic drug may present synergistic cytotoxic enhancement to transitional cell carcinoma cells with subsequent inhibition of their cellular behaviors, including proliferation, invasiveness, and metastatic activity. The contents of VEGF-A in mouse bladder tumor cells (MBT-2) and culture medium were detected by quantification-polymerase chain reaction and Western blot individually. The inhibitory concentrations of various chemotherapeutic drugs, sunitinib, and their combination treatment in MBT-2 were determined by 3-(4,5-dimethyl-2-thiazolyl)-2,5-diphenyl-2H-tetrazolium bromide (MTT) assay. Microchamber transmembrane migration assay was applied in evaluation of the inhibitory effects of different dosages of sunitinib and combination treatment on tumor cells. The cell cycle and apoptosis were analyzed after combination therapy by flow cytometry. Variation in apoptotic pathway was elucidated by Western blot using specific antibodies with cleaved PARP and caspase-3. Metastatic animal model mimicked by tail vein injection of MBT-2 cells was used to evaluate the treatment efficiency in tumor weight and survival rate. The mRNA and protein level of VEGF-A in MBT-2 cells increased by 70% at 48 hours interval under hypoxia stress condition. In MTT assay, MBT-2 cells had shown the highest sensitivity to epirubicin. Sunitinib combined with epirubicin had shown a synergistic cytotoxic effect to MBT-2 cells. Sunitinib and its combination with epirubicin showed significant inhibition on MBT-2 cells migration in microchambers. G2/M phase arrest and increased subG1 in cell cycle was seen in the epirubicin and sunitinib combination treatment group. The activation of apoptosis pathway was confirmed by increased cleaved caspase-3 and cleaved PARP in MBT-2 cells. In tail vein tumor inoculation C3H mice model, epirubicin alone and sunitinib combination therapy decreased tumor growth in lungs with marginal effect. Sunitinib and epirubicin combination had shown a synergistic cytotoxic effect and inhibited cell migration ability in MBT-2 cells. The combination can induce cell cycle arrest at G2/M phase and increase subG1 cells. Metastatic animal study also showed that sunitinib combined with epirubicin has a marginal effect on inhibition of tumor growth of lungs. The tyrosine kinase receptor inhibitor-targeted combined chemotherapy regimen may provide as a new treatment modality for advanced bladder cancer in the future.

The Chinese version of the Severity of Dependence Scale as a screening tool for benzodiazepine dependence in Taiwan - Corrected Proof

2011-09-23

Abstract: The development of an instrument to estimate the incidence, characteristics, and risk factors of benzodiazepine (BZD) dependence broadly in Taiwan is an important task. This study assessed the validity of the Chinese version of the Severity of Dependence Scale (SDS[Ch]) among regular BZD users in Taiwan (n=228). A positive correlation was shown between SDS[Ch] and Mini-International Neuropsychiatric Interview diagnosed of BZD dependence. Thirty-six percent of the users received a Mini-International Neuropsychiatric Interview diagnosis of current BZD dependence. The dependent users tended to be divorced/widowed; not schizophrenic; and have higher SDS[Ch] scores, a longer duration of use, and multiple-BZD use. The SDS[Ch] for BZD dependence was shown to have high diagnostic utility (area under the receiver operating characteristic curve=0.779), a sensitivity of 80.5%, and a specificity of 85.7%, with a cutoff point of 7. The findings support that the SDS[Ch] is a valid brief self-reported questionnaire for the assessment of BZD dependence among chronic users in Taiwan.

Relationship between red cell distribution width and echocardiographic parameters in patients with diastolic heart failure - Corrected Proof

2011-09-19

Abstract: Red cell distribution width (RDW) was found to be a prognostic marker in heart failure patients. The aim of the study was to investigate the relationship between RDW and echocardiographic parameters in diastolic heart failure (DHF). Seventy-one consecutive DHF patients (26 men) and 50 controls (21 men) were included in the study. All of the study population underwent echocardiographic evaluation, and blood samples were obtained. RDW and N-terminal pro-B-type natriuretic peptide (NT-proBNP) values were significantly higher, whereas there was an increasing trend for high-sensitivity C-reactive protein levels in DHF patients than those in controls (p<0.001, p<0.001, and p=0.064, respectively). All of the echocardiographic parameters evaluating diastolic function were more deteriorated in the DHF group. Patients who had an RDW value greater than the cutoff point also had higher NT-proBNP levels, an elevated ratio of mitral peak velocity of early diastolic filling to early diastolic mitral annular velocity, and increased estimated pulmonary capillary wedge pressures by tissue Doppler parameters, but lower creatinine clearance (p<0.05 for all). According to the cutoff values calculated using receiver operating characteristic analysis, RDW>13.6% and NT-proBNP>125pg/mL have high diagnostic accuracy for predicting DHF. RDW values were increased in the DHF population. Our results suggest that the high RDW levels in patients with DHF may be related to increased neurohormonal activity, impaired renal functions, and elevated filling pressure, but not to increased inflammation.

Therapy-related acute lymphoblastic leukemia with t(4;11)(q21;q23) masqueraded as marrow lymphocytosis in a patient with breast cancer - Corrected Proof

2011-09-19

Abstract: Therapy-related acute leukemia develops in patients after chemotherapy and/or radiotherapy for a prior cancer, and most cases are acute myeloid leukemia with a much lower frequency of acute lymphoblastic leukemia (ALL). One unique feature of these therapy-related ALL (t-ALL) is an increased incidence of chromosome band 11q23 aberrations as compared with de novo ALL. In adult female patients, breast cancer is the most common primary cancer. Herein, we report the case of a 49-year-old Taiwanese lady who developed t-ALL with t(4;11)(q21;q23) 16 months after cyclophosphamide, epirubicin, and 5-fluorouracil chemotherapy for her breast cancer. The unusual feature is that the t-ALL was heralded 4 months ago by marrow lymphocytosis comprising atypical small lymphocytes with condensed chromatin mimicking a B-cell chronic lymphoproliferative disorder. Retrospective studies using additional antibodies for immunophenotyping and PCR-based clonality study for immunoglobulin gene rearrangement showed that these atypical small lymphocytes shared similar features with the leukemic blasts at the frank leukemic stage. Our results suggest that these atypical small lymphocytes are lymphoblasts in disguise and that the clinicopathological correlations with ancillary pathological studies are important to reach a definitive diagnosis of such an unusual case.

Thermal welding versus cold knife tonsillectomy: A prospective randomized study - Corrected Proof

Metin Yilmaz, Mehmet Duzlu, Tolgahan Catli, Selin Ustun, Alper Ceylan — 2011-09-12

Abstract: This is a prospective randomized study conducted in a group of children who underwent two methods of tonsillectomy: thermal welding or cold knife tonsillectomy. Parameters, such as postoperative pain scores, intraoperative blood loss, operation time, and postoperative bleeding rates, were analyzed to find out which technique is better. Ninety-one children (aged between 2 years and 13 years) with recurrent tonsillitis, obstructive sleep apnea syndrome, or both were included in the study. According to the type of tonsillectomy procedure, the patients were divided into two groups: cold knife and thermal welding procedure. The two groups were compared on the basis of postoperative pain scores, intraoperative blood loss, operation time, and postoperative bleeding. Fifty-seven patients underwent thermal welding tonsillectomy and 34 had cold knife tonsillectomy. The mean pain score in thermal welding group was significantly lower (p<0.001). There was no remarkable blood loss intraoperatively in the thermal welding procedure. The operation time was not significantly different between two groups. No postoperative bleeding was encountered in the thermal welding group. Compared with the cold knife technique, thermal welding was found to be a relatively new and safe technique for tonsillectomy as it results in significantly less postoperative pain and no remarkable blood loss.