Progressive pseudorheumatoid arthritis of childhood (PPAC)

Five patients are described with a hereditary arthropathy affecting major and minor joints. The main features of this progressive connective tissue disorder are restricted joint mobility, osseous swelling of the interphalangeal and other joints, and platyspondyly. The condition is commonly misdiagnosed as chronic juvenile polyrthritis with Scheuermann disease. It differs from the rheumatoid-factor-negative polyarticular form of rheumatoid arthritis and other rheumatoic spondylarthropathies by the absence of arthritic and other inflammatory changes, radiographically by the absence of destructive and the presence of dysplastic bone changes. The disorder does not seem to respond to the usual forms of antirheumatoid treatment. Histological studies showed a peculiar, nest-like clustering of chondrocytes in the resting and growth cartilage suggesting that pathogenetically this is a primary disorder of the articular cartilage.Supported by the Stiftung Volkswagenwerk     

Bombesin and insulin-stimulated pancreatic polypeptide release as a discriminator of vagal integrity

Intact vagi after ulcer operations are often implicated in the cause of recurrent ulcer. The stimulation of gastric acid by insulin hypoglycemia is dangerous and the measurement of acid secretion after gastrectomy unreliable. This study was undertaken to assess and compare PP release by bombesin or insulin as an indicator of vagal integrity. Eight dogs with a chronic gastric fistula were tested with bombesin (100 nanograms per kilogram) and insulin (0.1 unit per kilogram) intravenous bolus after unilateral and, then, bilateral truncal vagotomy. Each study was 120 minutes, and blood was taken at one, three, five, seven and then ten minute intervals. Gastric acid was measured by autobiuret titration. Plasma was stored at minus 20 degrees C. until assayed for PP by radioimmunoassay. Bombesin-stimulated gastric acid secretion was not significantly altered by vagotomy (p greater than 0.05), whereas that stimulated by insulin was significantly inhibited by bilateral truncal vagotomy (p less than 0.05). Bilateral and right hemivagotomy significantly inhibited PP release by bombesin (p less than 0.05), however, only bilateral truncal vagotomy significantly inhibited PP release by insulin (p less than 0.05). These results suggest that the measurement of PP release by insulin or bombesin is a sensitive index of vagal integrity and that bombesin-released PP may specifically delineate the integrity of the right vagus. Since the measurement of gastric acid secretion after operation is both uncomfortable and often difficult to interpret, the value of a simple blood test to determine vagal integrity may be of considerable clinical relevance.     

Bcl-6 p53 mutations in lymphomas carrying the bcl-2/Jh rearrangement

BACKGROUND AND OBJECTIVES: The t(14;18)(q32;q21) chromosomal translocation is the hallmark of follicular lymphomas (FL). The translocation induces the overexpression of the Bcl-2 protein and prolongs the survival of clonogenic cells. Tumor cells may acquire additional molecular alterations that may be associated with histologic progression or with chemo-resistance. DESIGN AND METHODS: We analyzed the distribution and association of bcl-6 and p53 mutations in 55 consecutive bcl-2/Jh+ lymphoma samples derived from 43 patients obtained at the time of diagnosis and, in 5 of these patients, during follow-up. A total of 29 bcl-6 point mutations were detected in seventeen patients (40%) associated with major or minor breakpoints of the bcl-2/Jh fusion gene. In seven cases a p53 mutation was detected. Three cases corresponded to FL with the minor breakpoint in the bcl-2 gene and these patients had a favorable clinical evolution, whereas the 4 patients with p53 mutations and the major breakpoint had a bad clinical outcome with morphologic transformation to high-grade lymphoma in three cases. The sequential analysis of 5 patients showed a different timing in the acquisition of mutations: one patient showed bcl-6 and p53 mutations at diagnosis, another patient showed bcl-6 mutations at diagnosis and acquired a p53 mutation later whereas the third patient had a p53 mutation before the appearance of the bcl-6 mutation. RESULTS: We did not find significant differences in survival between patients with FL who showed exclusively bcl-6 mutations and those without bcl-6 mutations, but those patients with a high International Progostic Index score and p53 mutations showed the lowest overall survival (p = 0.002). INTERPRETATION AND CONCLUSIONS: These findings suggest that bcl-2/Jh lymphomas show molecular heterogeneity and that bcl-6 and p53 mutations may be acquired during the evolution of such lymphomas. Bcl-6 mutations, by themselves, do not seem to be associated with a bad prognosis. Rearrangements at the minor bcl-2 locus may have a different molecular evolution.     

Comparison of posterior approaches to the posterior incisural space: microsurgical anatomy and proposal of a new method,the occipital bi-transtentorial/falcine approach

OBJECTIVE: Direct surgical approaches to the posterior incisural space, including the pineal region, remain as challenges for neurosurgeons. The purposes of this study were 1) to compare the surgical views in the various posterior approaches to the posterior incisural space and 2) to propose a new approach, which is a modification of the occipital transtentorial approach. METHODS: Ten adult cadaveric specimens (20 sides) were studied, using x3 to x40 magnification, after perfusion of the arteries and veins with colored silicone. Intraoperative views in the posterior approaches to lesions were examined in stepwise dissections. In addition, the efficacy of the occipital bi-transtentorial/falcine approach was studied. RESULTS: The posterior incisural space has a roof, a floor, and anterior and lateral walls and extends backward to the level of the tentorial apex. The operative views defined by each approach differ in the extent to which they allow observation of the anatomic structures in the posterior incisural space. The occipital bi-transtentorial/falcine approach permits better observation of the contralateral half of the quadrigeminal cistern. CONCLUSION: Precise surgical anatomic knowledge of each approach is required for the treatment of lesions in the posterior incisural space, because the operative fields obtained with different approaches differ significantly. The occipital bi-transtentorial/falcine approach provides greater contralateral exposure of the posterior incisural space than does the occipital transtentorial approach.     

Importance of right subcostal incisions in patients undergoing TRAM flap breast reconstruction

The presence of a preexisting subcostal incision alters the approach to breast reconstruction and is thought to predispose to donor site skin complications and flap loss. The purpose of this study was to determine whether the presence of a subcostal scar affects breast or donor site morbidity adversely after transverse rectus abdominis musculocutaneous (TRAM) flap breast reconstruction. Twenty-six patients with a right subcostal incision (group A) underwent TRAM flap breast reconstruction (13 immediate, 13 delayed). The average age was 51 years, and the patients had an average body mass index of 25.3. There were 15 right, 10 left, and 1 bilateral reconstruction (4 free flaps, 22 pedicled). Outcome measures were compared with 126 age- and risk-matched patients (group B) who underwent TRAM flap reconstruction without any preexisting abdominal scar. The average age in group B was 46.7 years, and the patients had an average body mass index of 24.8. The average length of stay in group A was 5.9 days, compared with 4.8 days in group B ( < 0.05). There were no significant differences in breast-related complications. Donor site complications were higher in group A, with abdominal wall skin necrosis being significantly higher in patients with a subcostal incision (25%) compared with those patients without abdominal wall scars (5%; = 0.02). Multivariate analysis revealed a 6.5-fold increase in donor site complications in patients with a subcostal incision and a smoking history ( < 0.05). When adjusted for radiation treatment, the increased incidence in donor site complication rate was only marginally significant ( = 0.08). TRAM flap breast reconstruction in patients with preexisting right subcostal scars is effective with certain technical modifications; however, there is a slight predisposition to increased abdominal wall complications. Smoking influenced outcome further in patients with a subcostal incision, stressing the importance of proper patient selection.     

Early psychological stress after forearm nerve injuries: a predictor for long-term functional outcome and return to productivity

Forearm and wrist injuries can result in a nonfunctional hand caused by loss of motor and sensory functions. Psychological stress is known to accompany traumatic hand injuries and may therefore affect functional outcome. The authors conducted a retrospective study of 107 patients diagnosed with a median, ulnar, or combined median-ulnar nerve injury (79% response rate) who completed a questionnaire package consisting of the Impact of Event Scale (IES); Disabilities of Arm, Shoulder, and Hand; and a questionnaire concerning return to work and time off work. In an outpatient setting, motor and sensory recovery were examined. Ninety-four percent of those studied experienced early psychological stress. Thirty-six percent of patients reported sufficient symptoms 1 month postoperatively to be classified as in need for psychological treatment (IES score > 30 points). Combined median-ulnar nerve injuries (mean, 35.0 +/- 20.3 points [standard deviation]) were accompanied by a higher psychological stress compared with single nerve injuries (median injuries: mean, 24.2 +/- 20.6 points; ulnar injuries: mean, 22.6 +/- 19.5 points; p = 0.049 and p = 0.021 respectively). Multiple linear regression adjusting for age, gender, and severity of the trauma revealed an association between the IES score and the functional symptom score (beta = 0.51; 95% confidence interval [CI], 0.35-0.65), mean time off work (beta = 0.44; 95% CI, 0.25-0.75), and motor recovery (grip: beta = 0.37; 95% CI, 0.09-0.65; tip-pinch: beta = 0.46; 95% CI, 0.13-0.80). Patients with higher scores on the IES were found to be at increased risk for incapacity for work (odds ratio, 3.32; 95% CI, 1.60-6.91). Higher education was found to be a protecting variable for posttraumatic psychopathology (beta = -0.23; 95% CI, -6.05--0.246). This study demonstrated a high level of early posttraumatic psychological stress after forearm and wrist nerve injuries. These data provide evidence that functional outcome and work resumption are influenced negatively by early psychological stress, independent from severity of the somatic trauma. This indicates that outcome after upper extremity nerve injuries may be influenced positively by psychological intervention.     

Scope and limitations of limb-sparing surgery in childhood sarcomas

The authors reviewed 43 children with high-grade sarcomas of bone and soft tissue who underwent surgery during the past 9 years. Twenty-six patients had osteosarcoma, 7 had Ewing sarcoma, and 10 had soft tissue sarcomas. Patients ranged in age from 4 months to 13 years. Mean follow-up period was 6 years. The feasibility of resection, results of local resection, and failure of local disease control were reviewed. Limb salvage was feasible in 81% of patients. Of the 35 patients undergoing limb salvage, margins were considered adequate in 88% and inadequate in 12%. Limb-sparing resection was not feasible in 19% of patients. Five patients (four with metastatic disease at presentation) did not undergo surgery and were given palliative care; three other patients underwent amputation. The decision of whether to proceed with limb salvage surgery must depend on the aggressiveness of the underlying tumor, its stage, the feasibility of obtaining tumor-free resection margins, and the response to neoadjuvant therapy.     

Salvage extracorporeal shockwave lithotripsy after failed distal ureteroscopy

BACKGROUND AND PURPOSE: When intervention is necessary, controversy remains as to the best treatment modality for stones of the distal ureter. In general, ureteroscopy is favored over extracorporeal shockwave lithotripsy (SWL) as the treatment of choice for distal ureteral stones. Although uncommon, ureteroscopy failures have traditionally necessitated repeat ureteroscopy to retrieve retained stone fragments. We evaluated the efficacy of salvage SWL for failed primary distal ureteroscopy in the community setting. PATIENTS AND METHODS: From December 1989 to December 2000, 6099 patients underwent SWL with the Dornier HM4 lithotripter at our institution. We retrospectively identified 31 patients who had undergone the SWL after a failed distal ureteroscopy. RESULTS: The average stone size in these patients was 9.4 mm, the average time interval from ureteroscopy to SWL was 17.2 days, and the average number of shockwaves delivered was 2386. All patients had had stents placed after ureteroscopy. Twenty-seven patients (87%) had resolution of their stone burden after one SWL session. The remaining four patients underwent additional procedures. CONCLUSIONS: Ureteroscopy is an effective modality for the treatment of distal ureteral stones. However, when unsuccessful, a salvage procedure may be necessary. Extracorporeal lithotripsy is a less invasive procedure with comparable success rates in the distal ureter. This report suggests that salvage SWL is an appropriate option for patients in whom distal ureteroscopic stone extraction fails.