Neu–Laxova syndrome: a case report and review of the literature

Neu-Laxova syndrome (NLS) is a rare autosomal recessive syndrome, characterized by severe intrauterine growth retardation (IUGR), microcephaly, abnormal brain development, oedema and ichthyosis. It was first reported in 1971 by Neu et al. (Pediatrics 47: 610-612) and since then no more than 60 cases have been reported. A newborn girl delivered from a 29-year-old healthy mother was admitted to hospital with a thick membrane covering her body and dismorphic appearance. The diagnosis of NLS was made according to characteristic features. The syndrome is known to have a poor prognosis and the baby lived for 9 weeks. This case is one of the longest living cases of NLS and the fourth case reported from Turkey.     

肱骨骨不连的手术治疗

目的探讨肱骨骨不连的手术治疗方法及疗效。方法1998年12月～2005年5月共收治肱骨骨不连患者25例,均为肱骨骨折内固定术后发生骨不连,其中3例并发骨髓炎,6例合并不同程度肱骨骨缺损,骨缺损长度为3～6cm。骨不连病程8个月～5年。15例行吻合血管游离腓骨移植,10例采用加压交锁髓内针进行肱骨固定并辅以自体骨植骨。结果术后25例均得到随访,时间6个月～6年2个月。吻合血管游离腓骨移植组中移植的腓骨段均与肱骨干形成骨性愈合,平均骨性愈合时间为3．1个月；交锁髓内针组平均骨愈合时间为3．8个月。按Crates和Whittle肩肘关节功能评价标准,腓骨移植组：优9例,良4例,差2例；交锁髓内针组：优5例,良3例,差2例。结论应用加压交锁髓内针辅以自体骨移植对硬化性肱骨骨不连是一种有效的外科治疗方法；对合并骨髓炎、大段骨缺损及严重骨质疏松的肱骨骨不连,采用吻合血管游离腓骨移植可一期进行修复与重建。     

Pancreatic Panniculitis Associated with Allograft Pancreatitis and Rejection in a Simultaneous Pancreas–Kidney Transplant Recipient

Pancreatic panniculitis is an uncommon condition that can occur in association with pancreatic disease. We present a case of pancreatic panniculitis in a female pancreas-kidney transplant recipient 5 months post-transplant. The patient was on standard immunosuppressive medications and had acute rejection of her renal allograft. The diagnosis of allograft pancreatitis and rejection presenting with pancreatic panniculitis was supported clinically, histopathologically and by laboratory and imaging data. This is the fourth case of pancreatic panniculitis occurring in a transplant recipient and the first in a simultaneous pancreas-kidney transplant recipient. It is also the first case associated with allograft rejection. Clinicians should be aware that pancreatic panniculitis may be a manifestation of underlying allograft pancreatic disease.     

Renal Allograft Biopsies with Borderline Changes: Predictive Factors of Clinical Outcome

The clinical outcome and appropriate management for patients showing 'borderline changes' on allograft biopsy after renal transplantation is still controversial. In an attempt to identify predictive factors of clinical outcome of patients with such lesions, we reviewed the clinical course of 91 patients with borderline changes. Multivariate analysis revealed significant and independent effects of histological stage (i + t < or = or > 2) and time to borderline changes (< or = or > 3 months after transplant) on serum creatinine levels at 1 year from borderline changes episodes (respectively, p = 0.04 and p = 0.02) and only a significant effect of time to borderline changes on serum creatinine levels at 2 years (p = 0.005). Renal function at 1 year and 2 years as 5- and 8-year graft survival were not significantly different in the group of patients treated with antirejection therapy (T group, n = 49) compared with the untreated group (UT group, n = 42). This study strongly suggests that borderline changes with histological score (i + t) > 2 and late episodes of borderline changes should be considered to be of poor prognosis.     

Outcomes of Donor Evaluations for Adult-to-Adult Right Hepatic Lobe Living Donor Liver Transplantation

The purpose of this study is to determine the role of liver biopsy and outcome of patients undergoing donor evaluation for adult-to-adult right hepatic lobe living donor liver transplantation (LDLT). Records of patients presenting for a comprehensive donor evaluation between 1997 and February 2005 were reviewed. Liver biopsy was performed only in patients with risk factors for abnormal histology. Two hundred and sixty patients underwent a comprehensive donor evaluation and 116 of 260 (45%) were suitable for donation, 14 of 260 (5.4%) did not complete evaluation and 130 of 260 (50%) were rejected. Four patients underwent unsuccessful hepatectomy surgery due to discovery of intraoperative abnormalities. Between 1997 and 2001, the acceptance rate of donor candidates (63%) was higher than 2002-2005 (36%), p < 0.0001. Sixty-six of the 150 eligible patients (44%) fulfilled criteria for liver biopsy and 28 of 66 (42%) had an abnormal finding. Less than half of the patients undergoing donor evaluation were suitable donors and the donor acceptance rate has declined over time. A large proportion of the patients undergoing liver biopsy have abnormal findings. Our evaluation process failed to identify 4 of 103 who had aborted donor surgeries.     

Racial and Ethnic Disparities in Idiopathic Pulmonary Fibrosis: A UNOS/OPTN Database Analysis

We previously reported poorer survival among non-Hispanic blacks and Hispanics with idiopathic pulmonary fibrosis (IPF) compared to non-Hispanic whites at our center. In the current study, we hypothesized that these disparities would exist in a nationwide cohort of wait-listed patients with IPF. We performed a retrospective cohort study of 2635 patients with IPF listed for lung transplantation between 1995 and 2003 at 94 transplant centers in the United States. The age-adjusted mortality rate was higher among non-Hispanic blacks [hazard ratio (HR) = 1.24, 95% confidence interval (CI) 1.06-1.45, p = 0.009] and Hispanics (HR = 1.29, 95% CI 1.06-1.56, p = 0.01) compared to non-Hispanic whites. These findings persisted after adjustment for transplantation, medical comorbidities and socioeconomic status. Worse lung function at the time of listing appeared to explain some of these differences (HR for non-Hispanic blacks after adjustment for forced vital capacity percent predicted = 1.16, 95% CI 0.98-1.36, p = 0.09; HR for Hispanics = 1.21, 95% CI 0.99-1.48, p = 0.056). In summary, black and Hispanic patients with IPF have worse survival than whites after listing for lung transplant.     

12例开放性腋动脉损伤的诊治

目的探讨开放性腋动脉损伤的诊治体会。方法1996年-2002年，共诊治12例开放性腋动脉损伤的患者，从受伤到入院时间为3～14h，入院时均处于不同程度的休克状态，桡动脉搏动减弱或消失。在积极抗休克治疗的同时，急诊进行清创、血管神经探查和修复术。其中行腋动脉修补5例，腋动脉直接吻合6例，自体静脉移植修复1例。结果伤后8h内腋动脉再通8例，8～16h内腋动脉再通4例，无病例发生截肢或死亡。术后伤口均Ⅰ期愈合，桡动脉搏动良好。结论对开放性腋动脉损伤早期诊断和及时手术探查是控制休克、获取良好疗效的关键。     

The floating hip injury: which should we fix first?

In floating hip injuries, the priority of the steps in fixation has not been well described. We have treated these patients in accordance with the following protocols. In patients with pelvic ring fractures, the external fixation should be performed first, and in patients with acetabular fractures, the femoral fractures should be internally fixated first. The purpose of the present study was to review our experience with these fracture treatments to evaluate our strategy. For 8 years, 31 patients with femoral fractures associated with ipsilateral fractures: 26 unstable pelvic ring fractures and 7 unstable acetabular fractures were surgically treated at our level 1 trauma center. We reviewed the admission, operation, and follow-up records retrospectively and evaluated the order of the fixation and the complications. Two patients with acetabular fractures failed to follow our protocol outlined at the preoperative planning stage. There were no surgical difficulties other than two patients with the hemipelvis dislocated proximally who required traction of the fixated femur. The complications that were thought to be related to our protocol were one fat embolism syndrome and one pubic fracture displacement. One heterotopic ossification seemed to occur due to using a single approach for both fractures. We believe our protocol to be generally effective, however, in patients with acetabular and vertical shear type pelvic ring fractures, the internal or external fixation of the femur should be performed before the internal fixation of the pelvis.