Pulmonary Arterial Hemodynamic Assessment by a Novel Index in Systemic Sclerosis Patients: Pulmonary Pulse Transit Time

Objectives

Systemic sclerosis (SSc) is a chronic, inflammatory, and autoimmune connective tissue disease that is associated with vascular lesions, and fibrosis of the skin and visceral organs. Cardiac complications may occur as a secondary effect of SSc as a result of pulmonary arterial hypertension and interstitial lung disease. The objective of this study was to assess whether the pulmonary pulse transit time (pPTT) could serve as a diagnostic marker for pulmonary arterial alterations in patients with SSc, prior to development of pulmonary hypertension.

Methods

Twenty-five SSc patients as a study group and 25 age- and sex-matched healthy volunteers for the control group were recruited to the study. Right ventricle function parameters, such as tricuspid annular plane systolic excursion (TAPSE), estimated pulmonary artery systolic pressure (ePASP), right ventricular dimensions, right ventricle fractional area changes, and myocardial perfusion index (MPI) were measured and calculated. Pulmonary pulse transit time was defined as the time interval between the R-wave peak in the ECG and the corresponding peak late systolic pulmonary vein flow velocity.

Results

Right ventricle myocardial performance index (RVMPI) and eSPAP were significantly higher in the SSc group than the controls (p?=?0.032, p?=?0.012, respectively). Pulmonary pulse transit time and TAPSE was shorter in the patients with SSc (p?=?0.006, p?=?0.015, respectively). In correlation analysis, pPTT was inversely correlated with RVMPI (r?=???0.435, p?=?0.003), eSPAP (r?=???0.434, p?=?0.003), and disease duration (r?=???0.595, p?=?0.003). Conversely, it positively correlated with TAPSE (r?=?0.345, p?=?0.022).

Conclusion

pPTT was found to be shorter in SSc patients. pPTT might serve as a surrogate marker of pulmonary hemodynamics in patients with SSc, even prior to the development of pulmonary hypertension.

     

The spectrum of diseases causing fever of unknown origin in Turkey: a multicenter study.

OBJECTIVE: The purpose of this trial was to determine the spectrum of diseases with fever of unknown origin (FUO) in Turkey. METHODS: A prospective multicenter study of 154 patients with FUO in twelve Turkish tertiary-care hospitals was conducted. RESULTS: The mean age of the patients was 42+/-17 years (range 17-75). Fifty-three (34.4%) had infectious diseases (ID), 47 (30.5%) had non-infectious inflammatory diseases (NIID), 22 (14.3%) had malignant diseases (MD), and eight (5.2%) had miscellaneous diseases (Mi). In 24 (15.6%) of the cases, the reason for high fever could not be determined despite intensive efforts. The most common ID etiologies were tuberculosis (13.6%) and cytomegalovirus (CMV) infection (3.2%). Adult Still's disease was the most common NIID (13.6%) and hematological malignancy was the most common MD (7.8%). In patients with NIID, the mean duration of reaching a definite diagnosis (37+/-23 days) was significantly longer compared to the patients with ID (25+/-12 days) (p=0.007). In patients with MD, the mean duration of fever (51+/-35 days) was longer compared to patients with ID (37+/-38 days) (p=0.052). CONCLUSIONS: Although infection remains the most common cause of FUO, with the highest percentage for tuberculosis, non-infectious etiologies seem to have increased when compared with previous studies.     

The Feasibility of Hepatic Resections Using a Bipolar Radiofrequency Device (Habib?)

The bipolar radiofrequency device (Habib®) has been recently introduced in order to reduce intraoperative bleeding for a safe hepatic resection as an alternative to the conventional tools. However, indications, perioperative findings, and outcome of the device for hepatic resections remain and deserve to be analyzed. The current study aims to analyze the feasibility of the bipolar radiofrequency device (Habib®) for hepatic resections. Information of the patients that underwent hepatic resection using with the Habib® device between 2007 and 2011 was abstracted. Patient, disease, and operation-related findings and perioperative data were investigated. A total of 71 cases (38 [53.5 %] males, mean age was 56.8 ± 11.9) were analyzed. Metastatic disease (n = 55; 77.5 %) was the leading indication followed by primary liver and biliary malignancies (n = 7; 9.9 %), hemangioma (n = 5; 7 %), hydatid disease (n = 3; 2.8 %), and hepatic gunshot trauma (n = 1; 1.4 %). Metastasectomy was the most commonly performed procedure (n = 31; 56.3 %), but in 24 (77.4 %) cases, it was performed in addition to extended resections. Other procedures in the study patients include segmentectomy in 17, bisegmentectomy in 19, trisegmentectomy in 17, right or left hepatectomy in 8, and extended right/left hepatectomy in 3. The mean (±SD) operation time was 241.7 ± 78.2 min. The median amount of bleeding was 300 cc (range 25–2500), and 23 (32.4 %) cases required perioperative transfusion. The median hospitalization period was 5 days (range 1–47). Lengthened drainage (n = 9, 12.7 %) and intraabdominal abscess (n = 8, 11.23 %) were the most common problems. Hepatic resections using the Habib® device seem to be feasible in cases with primary and metastatic hepatic lesions and benign liver masses and even those with hepatic trauma. It may lessen the amount of intraoperative hemorrhage, although lengthened drainage and intraabdominal abscess were the major postoperative problems in these cases.     

A new silent germline mutation of the TSH receptor: coexpression in a hyperthyroid family member with a second activating somatic mutation.

BACKGROUND: Up to date, three thyroid-stimulating hormone receptor (TSHR) germline variants have been reported for which no functional consequences have been detected by in vitro characterizations. However, familial nonautoimmune hyperthyroidism and hot nodules are clearly associated with constitutively activating TSHR germline mutations. We describe a family with a new TSHR germline mutation that is associated with euthyroidism in 13 family members and hyperthyroidism in 1 family member. METHODS: Mutation analysis of the TSHR gene was performed by denaturing gradient gel electrophoresis. TSHR constructs were characterized by determination of cell surface expression, 3'-5'-cyclic adenosine monophosphate (cAMP) accumulation, and constitutive cAMP activity. RESULTS: A novel TSHR germline mutation (N372T) was found in a man who presented with thyrotoxicosis. The mutation was also detected in 13 family members, all of whom were euthyroid. Interestingly, an additional constitutively active somatic mutation (S281N) was identified on the second parental TSHR allele of the hyperthyroid index patient. Linear regression analysis showed a lack of constitutive activity for N372T. Moreover, coexpression studies of N372T with S281N did not reveal any evidence for a functional influence of N372T on the constitutively active mutation (CAM). CONCLUSIONS: N372T is unlikely to cause altered thyroid function. This is consistent with the finding that only the index patient with the additional somatic mutation S281N was hyperthyroid.     

Urothelial cancer of bladder in young versus older adults: Clinical and pathological characteristics and outcomes

Bladder urothelial carcinoma is rare in young adults and occurs more commonly in older individuals. The aim of this study was to compare the clinical behavior, pathologic characteristics, and prognosis of urothelial carcinoma of urinary bladder in young versus older adults. A retrospective review of our records between 2007 and 2013 identified 56 patients (42 males and 14 females) with transitional cell carcinoma of the bladder who were less than 40 years old. Clinical and pathological parameters of patients who were less than 40 years of age were compared with those of a series of patients older than 40 years of age (the control group) during the same period. A survival analysis was performed using the Kaplan–Meier method and log-rank test, and Cox regression was performed to identify clinical parameters that affected the clinical outcomes. The mean age was 29.21 years (range, 5–40 years) for patients less than 40 years old and 61.66 years (range, 41–75) for those older than 40 years. The mean follow-up was 40.26 months (range, 12–65 months) for young patients and 42.57 months (range, 12–72 months) for the older patients. Young bladder cancer patients had smaller-sized tumors (less than 3 cm), less high-grade cancers, higher papillary urothelial neoplasms of low malignant potential, and low-grade tumors than patients older than 40 years. Multivariate logistic regression analysis predicted tumor recurrence in young patients with high-grade tumors [odds ratio (OR), 1.959; 95% confidence interval (CI), 1.235–2.965; p = 0.046] and tumors larger than 3 cm (OR, 1.772; 95% CI, 1.416–1.942; p = 0.032). The 5-year overall survival rate was 100% for young patients and 88.1% for older patients. No difference was observed in the recurrence-free (p = 0.321) and progression-free (p = 0.422) survival rates between the two groups. We concluded that although the clinical stage distribution, natural history, and outcomes of bladder urothelial cancer in young adults are similar to those in their older counterparts, clinicians must be aware that patients under 40 years of age presented with higher-grade and larger (>3 cm) tumors and are more likely to experience tumor recurrence.     

Is there a role for surgery in the management of isolated secundum atrial septal defect in adults?

Isolated atrial septal defect (ASD) is the most common form of congenital heart abnormalities in adults and approximately 80% are located in the region of the fossa ovalis (ostium secundum ASD).1 Indications for closure are in cases where the ratio of pulmonary-to-systemic flow (Qp/Qs) is higher than 1.5, without significant elevation of pulmonary vascular resistance.Surgical closure of ASDs has been performed for over 60 years and techniques have steadily improved, using smaller incisions and minimally invasive techniques. On the other hand, in the last 20 years, various transcatheter ASD closure techniques and devices have been developed, among which, percutaneous treatment with a septal occluder device is the most popular.2,3Despite increasing use of occluder devices and the fact that studies have been published internationally pointing out some of the advantages and disadvantages compared with surgery in adults, no formal comparison of efficacy, morbidity and complications has been published.2-4 We present a retrospective comparison of short-term (three months) results for transcatheter and surgical closure of 163 ostium secundum ASD patients in a university hospital.