Lung Transplantation for Primary Ciliary Dyskinesia and Kartagener Syndrome: A Multicenter Study.

Primary ciliary dyskinesia, with or without situs abnormalities, is a rare lung disease that can lead to an irreversible lung damage that may progress to respiratory failure. Lung transplant can be considered in end-stage disease. This study describes the outcomes of the largest lung transplant population for PCD and for PCD with situs abnormalities, also identified as Kartagener's syndrome. Retrospectively collected data of 36 patients who underwent lung transplantation for PCD from 1995 to 2020 with or without SA as part of the European Society of Thoracic Surgeons Lung Transplantation Working Group on rare diseases. Primary outcomes of interest included survival and freedom from chronic lung allograft dysfunction. Secondary outcomes included primary graft dysfunction within 72 h and the rate of rejection ≥A2 within the first year. Among PCD recipients with and without SA, the mean overall and CLAD-free survival were 5.9 and 5.2 years with no significant differences between groups in terms of time to CLAD (HR: 0.92, 95% CI: 0.27-3.14, p = 0.894) or mortality (HR: 0.45, 95% CI: 0.14-1.43, p = 0.178). Postoperative rates of PGD were comparable between groups; rejection grades ≥A2 on first biopsy or within the first year was more common in patients with SA. This study provides a valuable insight on international practices of lung transplantation in patients with PCD. Lung transplantation is an acceptable treatment option in this population

Midterm results with the use of polycarbonate urethane heterografts for dialysis access

Dialysis access surgery is currently one of the most common vascular operations as the dialysis patient population inceases. Although autogenous arteriovenous fistulae stand as the preferred method of access due to excellent patency and lower complication rates, grafts may provide alternative approaches when an autogenous fistula is impractical usually due to depleted veins. We aimed in this study to assess early and midterm outcome with the use of Expedial (TM) heterografts (LeMaitre Vascular Ltd., Wrexham, UK) for angioaccess between October 2000 and December 2004. Patients were prospectively followed for pre- and peri-operative course with 1st, 6th and 12th month outpatient controls for patency and complication rates. A total of 34 heterografts were implanted in 30 patients (22 males and 8 females; median age 60.6 years, range 32 - 70). The mean follow-up for 30 patients were 16.3 +/- 6.1 (range 4 - 30) months. 28 grafts (82.4%) were placed in forearm position. Primary and secondary patencies were found as 81% and 94%, respectively (p = 0. 15), with the median censored primary and secondary patencies of 27 1.7 months (95% CI, 23.38 to 30.67) and 30.42 +/- 1.1 months (95% CI, 28.30 to 32.54), respectively. Only presence of diabetes was found as a significant risk factor in the regression model (p = 0.01). We suggest that the polycarbonate urethane heterografts with acceptable patency/complication rates and shorter maturation times for dialysis access serve an alternative for these patients. (c) 2005 Tohoku University Medical Press

Cervical lymphadenopathies in children: A prospective clinical cohort study

Aim: Cervical lymphadenopathy (LAP) is a common sign and may raise fears about serious illnesses. The aim of our study was to evaluate the patients with cervical LAPs in a general pediatrics clinic setting, and to evaluate follow-up results for potential causes and risk factors for malignancies. Material and methods: Two hundred-eighteen patients aged between 79.4 ± 46.7 months with LAP were enrolled in this prospective cohort study. The patients were examined in terms of demographics, clinical, radiologic and serologic aspects like Epstein-Barr virus (EBV), cytomegalovirus (CMV), parvovirus B19. A lymph node biopsy was performed in selected patients. The patients were followed-up for 8 weeks and risk factors for malignancy were evaluated. Results: Seventy patients (41.3%) had specific etiology and 6 (2.7%) had malignant causes. The causes were as follows: 27% (n = 59) infections; 2.7% (n = 6) malignancies; 11.4% (n = 25) other causes. EBV was responsible for 27% of infectious causes. The other common infectious etiologies were CMV 4.3%, parvovirus B-19 2.9%, and group-A beta-hemolytic streptococcus (GAS) 10.8%. Four of the six malignancies were lymphomas. Predictive factors for malignancy were having LAP larger than 30 mm, rubbery lymph node, high serum CRP and LDH values, no hilum in ultrasonography, and enlargement of lymph node in follow-up. High uric acid levels and leucopenia were also common in the malignancy group. Conclusion: Etiology of cervical LAPs was diagnosed in 41.3% patients. Infectious causes were the most common cause with 27%. Malignancy was diagnosed in 2.7% and lymphoma was the most common malignancy. © 2016 Elsevier Ireland Ltd

Predictive Value of Doppler Ultrasound in Childhood Pneumonia

This study aimed to determine the predictive value of intercostal and pulmonary artery Doppler flow patterns in the outcome of childhood pneumonia. Pneumonia was classified according to type of pleural effusion and the ultrasound features of consolidations. Doppler flow patterns of intercostal and pulmonary arteries were analysed and correlated with pneumonia type and hospital stay. Of 83 pneumonia cases, 55 were uncomplicated and 28 were complicated. Pleural effusion was present in 54 cases, with 29 non-septated and 25 septated cases. Patients with uncomplicated pneumonia did not have abnormal Doppler flow patterns, compared with 64% (18 of 28) of patients with complicated pneumonia. Doppler ultrasound patterns in childhood pneumonia were correlated with pneumonia type and may be predictive of pneumonia outcome