Renal involvement in the Laurence-Moon-Bardet-Biedl syndrome: report of five cases

.We report five patients with Laurence-Moon-Bardet-Biedl syndrome (LMBBS) who had renal involvement. Intravenous pyelography showed bilateral or unilateral calyceal clubbing and blunting in all patients. In addition, one patient had a parapelvic cyst in the left kidney and another had bilateral lobulated renal outlines of the fetal type. One patient had a urinary concentrating defect and two patients showed increased fractional sodium excretion. Estimated tubular phosphate reabsorption values were in normal limits in all of five patients. No patient had a urine acidification defect, proteinuria, glycosuria, or hyperaminoaciduria. One patient died from end-stage renal failure. The remaining four patients had normal serum creatinine values and estimated creatinine clearances. ^99mTechnetium-diethylenetriamine pentaacetate renal scanning showed prolonged and delayed concentration and delayed excretion in three of the four patients who survived. A focal scar was determined on the left kidney of one of four patients by ^99mtechnetium-dimercaptosuccinic acid renal scanning. All LMBBS cases with or without renal symptoms should be routinely evaluated for renal abnormalities. Renal scanning is a valuable method, especially for determining the renal involvement in the early stage of disease. Received November 14, 1995; received in revised form and accepted May 23, 1996     

脑干区占位病变的手术入路

本文报告了1990年5月以来脑干区大型、复杂占位病变15例.其中,位于脑干内3例,脚间窝2例,岩尖部4例,全岩骨区1例,全斜坡区2例,第四脑室内3例.本组采用以扩大了的岩骨入路为主的联合入路,行肿瘤全切除8例,次全切除5例.2例仅作了活检加减压术.除1例死亡外,均恢复良好.本文结合文献,对脑干区手术入路进行了讨论.     

The Mitek mini anchor in the treatment of the gamekeeper's thumb

The fixation of a distally ruptured ulnar collateral ligament of the MP 1 (Metacarpophalangeal) joint without a portion of ligament which can be sutured or a small bony fragment can be accomplished with a variety of methods, most of which require drillholes through borth cortices and a counter incision as well as the removal of the material at a second stage [1, 11, 13, 15]. The Mitek bone mini anchor (Ethicon-Mitek®) proved to be a reliable and quick alternative [10, 12, 16, 18, 19]. It was successfully used in eleven patients with excellent stability of the reconstructed joint.     

便携式野战—Ⅰ型多功能骨科手术器械

一种便携式野战多功能骨科手术器械。重量仅１．１ｋｇ，体积为２１５ｍｍ×１０５ｍｍ×１５ｍｍ，如铅笔盒，而功能多达３３种，具有结构紧凑、操作简便、一械多用、轻巧便携。价廉实用、效价比高的特点。是基层巡诊、抢险救灾救治中适用的骨科器械。     

Outcome Following Bariatric Surgery in Super versus Morbidly Obese Patients: Does Weight Matter?

Background: Numerous investigators have attempted to identify prognostic indicators for successful outcome following bariatric surgery. The purpose of this study was to determine whether degree of obesity affects outcome in super obese [>225% ideal body weight (IBW)] versus morbidly obese patients (160-225% IBW) undergoing gastric restrictive/bypass procedures. Methods: Since 1984, 157 patients underwent either gastric bypass or vertical banded gastroplasty. Super obese (78) and morbidly obese (79) patients were followed prospectively, documenting outcome and complications. Results: Super obese patients reached maximum weight loss 3 years following bariatric surgery, exhibiting a decrease in body mass index (BMI) from 61 to 39 kg/m² and an average loss of 42% excess body weight (EBW). Morbidly obese patients had a decrease in BMI from 44 to 31 kg/m² and carried 39% EBW at 1 year. After their respective nadirs, each group began to regain the lost weight with the super obese exhibiting a current BMI of 45 kg/m² (61% EBW) versus 34 kg/m² (52% EBW) in the morbidly obese at 72 months cumulative follow-up. Currently, loss of 50% or more of EBW occurred in 53% of super obese patients versus 72% of morbidly obese (P < 0.01). Twenty-six percent of super obese patients returned to within 50% of ideal body weight (IBW) while 71% of morbidly obese were able to reach this goal (P < 0.01). Co-morbidities and complications related to surgery were similar in each group. Conclusions: Super obese patients have a greater absolute weight loss after bariatric surgery than do morbidly obese patients. Using commonly utilized measures of success based on weight, morbidly obese patients tend to have better outcomes following bariatric surgery.     

Inhibitory effect of human blood granulocyte glycosaminoglycans on colony-forming activity of bone marrow fibroblasts

Inhibitory effect of glycosaminoglycans isolated from peripheral blood granulocytes of patients with chronic myeloleukemia and normal subjects on increment in colony-forming units of fibroblasts in monolayer cultures of rat bone marrow is determined by the degree of sulfation of their main component chondroitin-4-sulfate and by increased content of heparan sulfate. Translated fromByulleten' Eksperimental'noi Biologii i Meditsiny, Vol. 125, No. 3, pp. 319–322, March, 1998     

Intraoperative radiotherapy in the multidisciplinary treatment of bone sarcomas in children and adolescents

From September 1984 to December 1989, 38 patients of pediatric age with localized bone sarcomas received intraoperative radiotherapy (IORT) as part of a multidisci plinary treatment program. The age ranged from 6 to 21 years. The tumor histologies were 22 osteosarcomas and 16 Ewing's sarcomas. Thirty-four had initial primary disease (90%) and 4 were treated for local recurrence (10%). IORT was used on 32 untreated patients and in 6 previously treated with external beam radiotherapy (EBR). The IORT field included the surgically exposed tumor bed area. Single radiation doses ranging from 10 to 20 Gy were delivered, using 6–20 MeV electron beams. The median follow-up time for the entire group is 25 months (2–65+ months). The projected 5-year disease-free and overall survival rates are 65% and 69%, respectively. One patient developed a local recurrence in each histological group: one chondroblastic osteosarcoma and one cervical Ewing's sarcoma. Six patients died from metastatic progression: 3 initially recurrent tumors and three primary disease cases. Severe neuropathy and soft tissue necrosis were seen in some patients as IORT related complications. IORT is a feasible technique to be integrated in multidisciplinary programs that may promote local control in pediatric and adolescent patients with bone sarcomas. Peripheral nerves are dose-limiting tissue structures for IORT.     

瘤体骨切除灭活再植治疗四肢长骨巨细胞瘤

体内刮除及灭活植骨治疗骨巨细胞瘤（ＧＣＴ）的复发率高。我科从１９８６年６月～１９９６年２月治疗四肢长骨ＧＣＴ２４例。随访１１个月～８年４个月。其中７例采用局部切刮体内灭活植骨和（或）骨水泥充填治疗。结果１例感染，６例复发，１例恶变后死于肺转移。作者自１９９１年１月采用瘤体骨切除，离体灭活再植，与骨残端体内灭活相结合，骨缺损采用自家腓、髂骨移植，Ｌ－梯形加压钢板（Ｌ－ＴＣＰ）或梯形加压钢板（ＴＣＰ）固定治疗１７例，其中１２例保留关节，５例做膝关节融合。本组无菌创口感染１例，１例复发。在保留关节的１２例中９例关节功能恢复优良，１例尚可，２例差。充分说明瘤段骨切除体外灭活再植术疗效确实，复发率低。保留关节术式适于关节破坏较轻（＜＝１／２）者，结合关节残端体内灭活和有效的骨结构重建，有利于恢复关节功能。如关节面累及＞１／２，宜做骨关节端切除，灭活再植，下肢宜做关节融合或人工关节置换术。     

Cancellous Bone Structure in the Growing and Aging Lumbar Spine in a Historic Nubian Population

There is abundant data on cancellous bone in the aging human spine, but little relating to the growing vertebral cancellous bone in childhood and adolescence. The purpose of this study was to map vertebral cancellous bone in a growth and age series of historic skeletal samples and to make comparisons with data published on recent material. Lumbar vertebral bodies were collected from 65 skeletons (0–60 years) from a medieval Nubian population. Ethnohistoric information was collected to interpret conditions that might have influenced bone structure and metabolism. The cancellous bone was studied three dimensionally, using stereophotography and scanning electron microscopy and morphometrically by performing a semiautomatic structural analysis on digitized backscattered electron images of polymethacrylate-embedded material. The cancellous bone structure in the children consisted mainly of a densely packed, uniform network of small rodlike trabeculae. The greatest bone volume fraction with small, more platelike trabeculae was observed during adolescence. In young adults, larger platelike trabeculae were present in the central zone and smaller trabeculae in the superior and inferior zones, as described for modern skeletal material. Structural changes associated with aging were observed much sooner than in modern man. By the estimated age of approximately 50–60 years, the predominant architectural elements were slender rarified rods in both sexes. The ethnohistorical data suggest that this was essentially a black African population of physically active peasants, not likely to suffer Vitamin D insufficiency or deficient calcium intake. Thus an earlier onset of the biological age changes in cancellous bone found in modern populations was probably prevalent. Received: 1 March 1996 / Accepted: 31 December 1996     

Anterior lumbar fusion using a hybrid interbody graft

Summary This is a radiographic report of 40 patients (20 men, 20 women) who underwent anterior lumbar interbody fusions (73 levels) utilizing a “hybrid” interbody graft composed of femoral cortical allograft (FCA) bone and iliac crest cancellous autograft bone. The average age at surgery was 38 years (range 17–64 years), and follow-up averaged 1.4 years (range 1.0–2.4 years). Nineteen of the patients had undergone previous lumbar surgery. Thirty-two patients (63 levels) underwent anterior fusion combined with some type of posterior fixation, and eight patients (10 levels) had no posterior fixation. Types of posterior fixation included: for 20 patients (36 levels) Steffee variable screw placement fixation, for 10 patients (23 levels) translaminar facet screws (TFS), for 1 patient (3 levels) Knodt rods and for 1 patient (1 level) facet screws. Based on the persistence of lucent lines at the graft-host interface, three patients (one level each) were felt to have non-unions at their latest follow-ups at 1.4, 1.5 and 2.0 years, respectively. Two of these patients had no posterior fixation, and the other had TFS fixation. The overall fusion rate was 96% (70 of 73 levels). The fusion rate for all levels treated with posterior fixation was 98% compared with 75% for those without fixation. Intervertebral disc heights (IVDH) were measured on all films and corrected for magnification with computer assistance. On average, the IVDH was increased postoperatively but returned to preoperative values at follow-up. IVDH loss was independent of the type of instrumentation used. No complications arose from the use of the hybrid graft. Incorporation of the allograft portion of the graft is slow and was felt to be complete in only 7 of the 73 levels at follow-up. We conclude that the hybrid interbody graft technique is a safe and reliable method for performing anterior lumbar interbody fusions and should be combined with some type of posterior fixation. Long-term follow-up will be required to assess the behaviour of the allograft until incorporation is complete.