Chronic active gastritis in X-linked lymphoproliferative disease

Gastric lesions in primary constitutive immune deficiencies include multifocal atrophic gastritis, erosive pangastritis, and a pattern of gastric lesions reminiscent of graft-versus-host disease. We describe the genetic anomalies in 2 monozygotic twins with an X-linked lymphoproliferative disease (XLP; MIM 308240), a rare familial setting of high susceptibility to Epstein-Barr virus (EBV). Since early childhood, both twin brothers exhibited a severe chronic active atrophic pangastritis. A germline screening of the SH2D1A (MIM 300490) and BIRC4 (MIM 300079) genes was performed, and also a high-resolution whole-genome SNP profiling (Infinium Sentrix Human-1 Genotyping BeadChip, Illumina). A 3 Megabase deletion in the Xq25 region, encompassing the SH2D1A gene, was defined by SNP array genotyping. Histologic analysis of yearly or twice yearly gastric biopsies in both children showed a Helicobacter pylori-negative, Epstein-Barr virus-negative chronic active atrophic pangastritis, with superficial ulcer formation, foveolar hyperplasia, glandular dilatation and ultimately pseudopyloric and intestinal metaplasia. No such chronic active inflammatory gastric lesions have been reported to date in XLP. The similarities between XLP and common variable immunodeficiency (MIM 240500) underscore the need for early recognition and close monitoring of these gastric lesions, with special regard to their neoplastic potential. No infectious cause was determined. We favor a dysimmune mechanism in the development of this chronic atrophic gastritis, presenting a striking similarity to the recently described atrophic autoimmune pangastritis.     

Detection of anterior interventricular involvement after primary posterior wall infarct

Forty-five patients (average age 53.3 +/- 9.4 years) underwent exercise stress testing coupled with exercise myocardial scintigraphy and right heart catheterisation on exercise during the 3rd week after primary posterior wall infarction. Coronary angiography and ventriculography were carried out 24 to 48 hours later. Significant (greater than or equal to 75%) left anterior descending disease was present in 9 patients (Group I) and absent in 36 patients (Group II). Tolerance of maximal exercise stress testing was good in all patients. The results were uninterpretable in on third of the cases because the theoretical maximal heart rate was not attained. No significant difference was noted between groups I and II with respect to: mean age, percentage of theoretical maximal heart rate attained, mean pulmonary capillary pressures at rest and during exercise, basal and exercise cardiac index, end diastolic index and angiographic left ventricular ejection fraction. The sensitivity and specificity for the detection of LAD disease were 80% and 84% for the exercise stress testing (n = 30), 44% and 75% for exercise right heart catheterisation (n = 30), 60% and 92% for exercise myocardial scintigraphy (n = 30), 100% and 75% for combined exercise stress testing and myocardial scintigraphy (n = 30) and 78% and 64% for combined exercise stress testing and myocardial scintigraphy and exercise right heart catheterisation (n = 45). These results show that in patients capable of performing maximal exercise stress tests, the best method of early detection of significant LAD disease after primary posterior infarction was combined exercise stress testing and exercise myocardial scintigraphy. These investigations are useful for the selection of candidates for coronary angiography.     

A financial analysis of revision hip arthroplasty: the economic burden in relation to the national tariff

Revision arthroplasty of the hip is expensive owing to the increased cost of pre-operative investigations, surgical implants and instrumentation, protracted hospital stay and drugs. We compared the costs of performing this surgery for aseptic loosening, dislocation, deep infection and peri-prosthetic fracture. Clinical, demographic and economic data were obtained for 305 consecutive revision total hip replacements in 286 patients performed at a tertiary referral centre between 1999 and 2008. The mean total costs for revision surgery in aseptic cases (n = 194) were ￡11 897 (sd 4629), for septic revision (n = 76) ￡21 937 (sd 10 965), for peri-prosthetic fracture (n = 24) ￡18 185 (sd 9124), and for dislocation (n = 11) ￡10 893 (sd?5476). Surgery for deep infection and peri-prosthetic fracture was associated with longer operating times, increased blood loss and an increase in complications compared to revisions for aseptic loosening. Total inpatient stay was also significantly longer on average (p < 0.001). Financial costs vary significantly by indication, which is not reflected in current National Health Service tariffs.     

Cardiac paraganglioma: diagnostic and surgical challenges

Primary cardiac paragangliomas are rare extra-adrenal tumors. Though they account for less than 1% of all primary cardiac tumors, they are considerable sources of morbidity and mortality. In this case review, we discuss the challenges associated with the diagnosis and management of cardiac paragangliomas.     

The management of periprosthetic femoral fractures around hip replacements

Periprosthetic femoral fractures are increasing in frequency and in complexity. They occur intra-operatively, or post-operatively, when they are frequently associated with loosening, with or without osteolysis. Periprosthetic femoral fractures usually occur at low energy levels, either after falls or spontaneously during activities of daily living. At present, the Vancouver classification system probably comes closest to the ideal, as it considers the fracture configuration, the stability of the implant and the quality of the bone stock. When the stem is stable, open reduction and internal fixation is suggested. Several authors have used strut grafts for the treatment of periprosthetic femoral fractures, with good results. If the stem is loose, or revision is indicated for other reasons, it is generally advisable to by-pass the most distal fracture line with a longer stem prosthesis by at least two femoral diameters. Augmentation of this intra-medullary fixation with an external cortical strut to improve rotational stability and/or internally with impaction allografting to compensate for bone defects is also advisable. Vigilant post-operative clinical and radiological assessment following total hip replacement should identify those with recurrent dislocation, loosening, subsidence and osteolysis. These patients are at greatest risk of developing femoral periprosthetic fractures.     

Surgical management of a circumflex aneurysm with fistula to the coronary sinus

We report the successful management of a circumflex coronary artery aneurysm with fistula to the coronary sinus. Our strategy aimed at closing the fistula and grafting the obtuse marginal artery. The calcified aneurysm was left intact, and showed secondary thrombus formation on the postoperative angiogram.     

Patello-femoral joint in total knee replacement

Total knee arthroplasty (TKA) surgery is a widely used treatment and has a high success rate. Despite its success, the patello-femoral articulation has been overlooked as a contributor to the successful outcome of TKA. Patello-femoral complications remain the leading cause of failed knee replacement, accounting for up to 10% of complications. To understand the concept of patellar mal-tracking, we explore the normal anatomy and biomechanics of the patello-femoral articulation, and review the surgical factors and complications related to patellar tracking, with an aim to discussing the concepts on how to avoid such complications. Surgeons should be aware of the frequent occurrence of abnormal patellar tracking, and should pay particular attention to the patello-femoral component of knee replacement surgery.     

Engagement of transferrin receptor by polymeric IgA1: evidence for a positive feedback loop involving increased receptor expression and mesangial cell proliferation in IgA nephropathy

IgA nephropathy (IgAN), the most common primary glomerulonephritis in the world, is characterized by IgA immune complex-mediated mesangial cell proliferation. The transferrin receptor (TfR) was identified previously as an IgA1 receptor, and it was found that, in biopsies of patients with IgAN, TfR is overexpressed and co-localizes with IgA1 mesangial deposits. Here, it is shown that purified polymeric IgA1 (pIgA1) is a major inducer of TfR expression (three- to four-fold increase) in quiescent human mesangial cells (HMC). IgA-induced but not cytokine-induced HMC proliferation is dependent on TfR engagement as it is inhibited by both TfR1 and TfR2 ectodomains as well as by the anti-TfR mAb A24. It is dependent on the continued presence of IgA1 rather than on soluble factors released during IgA1-mediated activation. In addition, pIgA1-induced IL-6 and TGF-beta production from HMC was specifically inhibited by mAb A24, confirming that pIgA1 triggers a TfR-dependent HMC activation. Finally, upregulation of TfR expression induced by sera from patients with IgAN but not from healthy individuals was dependent on IgA. It is proposed that deposited pIgA1 or IgA1 immune complexes could initiate a process of auto-amplification involving hyperexpression of TfR, allowing increased IgA1 mesangial deposition. Altogether, these data unveil a functional cooperation between pIgA1 and TfR for IgA1 deposition and HMC proliferation and activation, features that are commonly implicated in the chronicity of mesangial injuries observed in IgAN and that could explain the recurrence of IgA1 deposits in the mesangium after renal transplantation.     

In-111-labeled leukocyte scintigraphy in suspected orthopedic prosthesis infection: comparison with other imaging modalities

When infection of prosthetic orthopedic implants is suspected, optimal management requires accurate confirmation or exclusion of infection. The authors retrospectively studied 98 patients with possible infection who underwent scanning with indium-111-labeled white blood cells (WBCs) and subsequently underwent surgery within 14 days. At surgery, 50 patients had infections, as determined by means of culture or histologic results. The diagnostic accuracy of In-111 scanning was compared with that of plain radiography, arthrography, three-phase bone scanning, and various clinical and laboratory findings classically associated with infection. Positive findings on In-111 WBC scans and elevated erythrocyte sedimentation rates were found to be the most predictive variables in the diagnosis of septic prostheses (P less than or equal to .001 and P less than or equal to .002, respectively). Likelihood ratio analysis more clearly demonstrated the superiority of In-111 WBC scanning, with positive and negative scans yielding likelihood ratios of 5.0 and 0.16, respectively.     

Injury to the femoral vessels--the Lebanese War experience

Over a 10-year-period extending from January 1976 to September 1986, 800 peripheral vascular injuries were surgically treated at the American University of Beirut Medical Centre. Of these, 150 were operated on because of injury to the femoral vessels. Bullets were the commonest wounding agents (60% of cases). Thirty-seven patients had a femoral artery injury, 27 a femoral vein injury and 86 combined femoral artery and vein damage. Twenty-three per cent of the patients had an associated fracture of the femur. Patients with fractures had an 11% amputation rate compared with 1% for those without fractures. Our data reveals the importance of venous reconstruction in the presence of an arterial injury instead of its ligation (P less than 0.05), particularly when there is an associated fracture of the femur. Thirty-seven per cent of patients were in shock on admission (systolic BP 90 mmHg). The amputation rate in these patients was 6.8% in contrast to 1% in the haemodynamically stable group (P less than 0.05). The overall amputation rate was 3.3%. Complications occurred in 36.6% of patients and the mortality was 4.0%.