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"Primary" antiphospholipid syndrome evolving into Waldenstrom's macroglobulinaemia: a case report.

A 57-year-old woman with a history of transient ischaemic attacks and six recurrent foetal losses accompanied by elevations of antiphospholipid antibodies was diagnosed as having a "primary" antiphospholipid syndrome. She was followed up for 5 years, and she developed anaemia, leucopenia and splenomegaly. A bone marrow trephine was diagnostic of Waldenstrom's macroglobulinaemia. A false positive serological test for syphilis was demonstrated and apparently had been noted in her second pregnancy more than 20 years prior to her presentation with an antiphospholipid syndrome. There had previously been no indication to perform serum electrophoretic studies. This case illustrates the importance of this investigation in any middle-aged patient presenting with an antiphospholipid syndrome and a monoclonal gammopathy This finding might presage the development of a more serious condition, even years later (as in our patient).

Antiphospholipid Syndrome↗

Minimally invasive, endoscopically assisted colostomy can be performed without general anesthesia or laparotomy.

PURPOSE: Fecal diversion is frequently required in critically ill patients who may not be able to tolerate a laparotomy. Laparoscopic-assisted and trephine colostomies are alternative methods for colostomy without laparotomy, but require general anesthetic. The objective of this study was to evaluate the possibility of performing fecal diversion with the assistance of a colonoscope and without the additional morbidity of abdominal exploration or general anesthesia. METHODS: Patients were diverted using a colonoscope to identify a site of the sigmoid colon that could easily be approximated to the anterior abdominal wall as confirmed by transillumination of the abdominal wall. A small skin disc was then removed at this location and a loop colostomy was made. The colonoscope was also used as a guide to identify the proximal and distal limbs of the loop colostomy. Four patients were considered to be critically ill and local or regional anesthetic with sedation was used in these patients. RESULTS: A total of 15 patients were reviewed during the past five years. All 15 patients were successfully diverted using minimally invasive techniques with the aid of the colonoscope. Four of these patients were diverted using local or regional anesthetic without complication, thus avoiding the morbidity associated with a general anesthetic in critically ill patients. CONCLUSION: No complications related to this technique were noted in this five-year review. Endoscopically assisted colostomy is an acceptable method for fecal diversion without the need for laparotomy and can be accomplished using a local or regional anesthetic with sedation.

Adult↗

Transvitreal chorioretinal biopsy in the rabbit.

Transvitreal chorioretinal biopsy was performed in nine Dutch rabbits by introducing a biopsy instrument through a small eye wall incision, passing the instrument across the eye through the vitreous to the biopsy site, and by trephinating and removing from the eye a 1.6-mm diameter specimen of the posterior eye wall. Biopsy was carried out under stereotaxic control by using a micromanipulator especially designed for ophthalmic surgery. Hemostasis was achieved by circumferential mechanical pressure around the biopsy site. The eye wall defect was sealed by a preplaced explant and repaired by fibrovascular ingrowth from the surrounding choroid and episclera. The normal histologic features of the biopsy specimen were preserved, except for separation of the neurosensory retina from the pigmented epithelium. Complications of the procedure included hemorrhage from the incision site, transvitreal strand formation, and lens abrasion. In no instance, however, did these complications result in significant opacification of the optical media. The biopsy technique has potential use as a clinical procedure in the diagnosis of choroidal tumors and other selected disorders of the choroid and retina and as a technique in investigational animal studies.

Animals↗

Combined scleral ring and blepharostat.

A modified Legrand scleral ring served three important functions: it prevented anterior scleral buckling and collapse, it separated the eyelids to give good surgical field exposure, and it provided firm fixation for the globe during trephining and suturing.

Corneal Transplantation↗

Comparison of partial-thickness and full-thickness filtration procedures in open-angle glaucoma.

We compared the results of partial-thickness (trabeculectomy) vs full-thickness (trephine) filtration surgery when used as the primary surgical treatment of medically uncontrolled open-angle glaucoma. Prospectively acquired data were used from a series of patients who were examined and operated on by one surgeon. Both groups of data were gathered consecutively and concurrently. The choice of operation was made on the basis of the pressure level required for control of the glaucoma, the full-thickness procedure being chosen for eyes with the most advanced cupping and field loss. The partial-thickness group had a significantly higher postoperative pressure and signficiantly lower incidences of postoperative flat anterior chambers and identifiable filtration blebs.

Adolescent↗

The correction of aphakia. XXXVI Edward Jackson Memorial Lecture.

Major advances in the correction of aphakia have been made in the past few years. Extended-wear contact lenses constitute one advance, if not a perfect one. Intraocular lenses are now safer to use, and new research is seeking ways to improve their safety through protection of the corneal endothelium during surgery and stimulation of endothelial healing where damage does occur. Altering the refractive power of the cornea offers another possible approach to the correction of aphakia. Modifications of Barraquer's keratomileusis procedure using preserved, preground corneas may have definite indications for certain types of aphakia, such as monocular aphakia in children and in individuals who would otherwise require secondary implants. We have experimented with a "living contact lens" of shaped donor tissue attached to the de-epithelialized surface of the eye. Such a lens of preground, preserved cornea tissue can be sutured to the patient's cornea with its central Bowman's membrane intact. A peripheral trephine mark is made, but the central cornea is untouched and the attachment of the living lens is simple. The procedure is apparently safe, and should be usable by any competent ophthalmic surgeon if the long-term results continue to be as encouraging as our early experience now suggests.

Adolescent↗

Temporary keratoprosthesis for use during pars plana vitrectomy.

We have developed a series of temporary keratoprostheses that permit closed pars plana vitrectomy in eyes with abnormal corneas. These devices are placed into a trephined opening in the cornea, and provide a clear, stable view of the intraocular contents. Immediately after vitrectomy, the keratoprosthesis is replaced with a corneal graft. We have used this technique successfully in four eyes with opaque or severely traumatized corneas.

Adult↗

Clinicopathologic studies of keratoplasty eyes obtained post mortem.

Thirty eyes of 25 patients who had undergone penetrating keratoplasties were examined post mortem. Available corneal buttons and donor rims were evaluated for irregularities of the trephine margins. Of 26 eyes with clear grafts, visual acuity had been 20/80 or better in 13, worse than 20/100 in six, and was unknown in seven. In four eyes with cloudy grafts, visual acuity had been 20/400 or worse. Twenty-three eyes had had one keratoplasty and seven eyes had had two or three transplants. Microscopic examination disclosed frequent overriding wound edges with Bowman's layer incarceration in the wound in 11 eyes and Descemet's membrane incarceration in 22 eyes. Wound abnormalities were correlated with the presence and degree of astigmatism. A retrocorneal fibrous tissue layer was localized at the wound in 16 eyes, extended peripherally in 11, and extended centrally in one. Iris and vitreous incarceration in the wound occurred in 14 and eight eyes, respectively. Histopathologic features contributing to visual loss included cystoid macular edema (three eyes), senile macular degeneration (four eyes), and preretinal membranes (four eyes).

Aged↗

An atraumatic technique for harvesting cancellous bone for secondary alveolar bone grafting in cleft palate.

Secondary alveolar bone grafting in cleft palate patients has been popularised by the Oslo group. Harvesting of the bone graft has been carried out by techniques developed initially for cranio-facial surgery. This paper describes a more refined technique applicable to the requirements of alveolar bone grafting. The Craig bone biopsy set is used to trephine cores of autogenous particulate marrow and cancellous bone from the iliac bone. The method was tested in a cadaver and then applied in 10 clinical cases. The aesthetic and functional results of this technique proved to be superior to the conventional approach.

Alveolar Ridge Augmentation↗

Collagen degradation and synthesis in experimental corneal grafts.

Thirty-two weanling New Zealand white rabbits were labelled repeatedly with [2,3-3-H]-proline for 4 weeks. Four weeks after the end of labelling, 32 rabbits underwent bilateral 6 mm trephinations for donor purposes. One control cornea of each pair was frozen at -70 degrees C. The contralateral corneas were used for autografts, allografts, and xenografts. Grafts were observed from 7-200 days, then retrephined out for determination of loss in total radioactivity in hydroxyproline (specific for collagen), increase in newly synthesized collagen, and net change in collagen mass, by comparing with the matched, ungrafted control corneas. For all three transplant groups there was a small, but significant, decrease early in total radioactivity (loss of original collagen) and a significant increase in new collagen. These data also indicated that the loss of original collagen was replaced by an equivalent or greater increase in new collagen in all transplant groups. Significant relationships between graft clarity and collagen turnover were noted in both the auto- and allograft groups. The degradation of old collagen was significantly greater in the cloudy vs. the clear grafts; however, there was no significant difference in the increase in new collagen between these groups. A progressive loss of original collagen over time was noted in the cloudy autografts, but not the allografts. A trend toward a progressive increase in new collagen was noted over time in both the cloudy auto- and allografts. No relation for these variables to time, however, was noted in the clear grafts.

Animals↗

Metabolic labeling of rod outer segment phospholipids in miniature poodles with progressive rod-cone degeneration (prcd).

The recessive genetic defect in miniature poodles which results in progressive rod-cone degeneration (prcd) has been investigated in an attempt to determine the biochemical abnormality involved. In the present study, the rod outer segments of young prcd affected miniature poodles and normal dogs have been compared with respect to the incorporation of intravitreally injected [3H]palmitic acid. [14C]linolenic acid, and [14C]docosahexaenoic acid into neutral lipids and phospholipids as well as [3H]palmitate and [14C]leucine into rhodopsin. In addition, 3 mm trephined punches of retinas were incubated with [3H]palmitic acid, [3H]arachidonic acid, [14C]linolenic acid, [3H]serine, [14C]glycerol and [14C]leucine. No difference in incorporation of labeled precursors into lipids or rhodospin was noted between prcd affected and normal retinas. Phosphatidyl choline appeared to function as a carrier of fatty acids to the rod outer segment where they were redistributed to other phospholipids. An interesting lack of conversion of the essential fatty acid linolenic acid to docosahexaenoic acid was noted in both normal and affected retinas. This conversion involves elongation and desaturation of linolenic acid and may take place primarily in extraretinal tissues such as the liver. This finding, in conjunction with a parallel study of plasma fatty acids which has shown significantly lower levels of docosahexaenoic acid in prcd affected poodles, points to a possible systemic defect in the metabolism or transport of docosahexaenoic acid, a fatty acid uniquely enriched in the photoreceptor outer segments.

Animals↗

Fluid conductance of cancellous bone graft as a predictor for graft-host interface healing.

The aim of this study is to test the hypothesis that the fluid conductance of cancellous bone graft is a deciding factor in graft-host union. Cylindrical cancellous bone specimens were trephined from fresh porcine femoral heads in a direction either parallel or perpendicular to the femoral neck axes. The graft after a defatting and freeze-drying process was placed in a perfusion apparatus. The pressure drop across the bone graft and the induced flow were measured and the conductance to fluid flow was calculated as the slope of the flow-pressure relation. Grafts of different flow conductance were transplanted into 35 rabbits to replace segments of tibiae. Nine weeks after grafting, 29 rabbits which had completed the follow-up without incidents were sacrificed. The decalcified sections of the grafts and the adjacent tibiae were examined microscopically for histological events during graft-host interface healing. Perfusion data indicated that fluid conductance ranged from 0.05 x 10(-10) to 13.4 x 10(-10) m3 s-1 Pa-1 and was higher in the direction parallel to the femoral neck axis, inversely proportional to the length of the graft and directly proportional to the square of porosity. Data from rabbit model supported the hypothesis stated above. A threshold conductance was found to be 1.5 x 10(-10) m3 s-1 Pa-1, below which revascularisation and the formations of osteoblasts and fibrous tissues could not be attained. How the low conductance led to non-union at the graft-host interface was briefly discussed.

Animals↗

Topographic mapping of the cutaneous microcirculation using two outputs of laser-Doppler flowmetry: flux and the concentration of moving blood cells.

Using a "needle" probe in a template probe holder, we measured the flux and the concentration of moving red blood cell (CMBC) outputs from a Perimed (PF2B) laser-Doppler instrument at 1-mm2 contiguous sites in a 8 x 8-mm area on the flexor forearm of three subjects. Using the means of the flux and CMBC recorded at each spot, a topographic contour map was constructed for each of these parameters. Viewing the two maps together, sites with four different combinations of flux and CMBC could be identified. Trephine biopsies (2 mm) of three representative sites in each subject were performed and the upper plexus was reconstructed in 3 dimensions from serial sections. High flux/high-to-medium CMBC sites were found over the spot where the ascending arterioles entered the upper plexus. Medium flux/medium-to-low CMBC sites and low flux/medium CMBC sites were found in the peripheral part of the vascular unit that was fed by the ascending arteriole. The low flux/low CMBC sites were relatively avascular zones. Video imaging of the upper plexus in the forearm showed the same overall vascular pattern as the contour maps. The highest flux and CMBC signals were recorded when horizontally oriented vessels were present in the upper third of the plexus (400-650 microns below the stratum corneum). Topographic mapping will allow one to selectively identify different microvascular areas in the skin for physiological studies.

Adult↗

Evaluation of 5,000 endodontic treatments: incidence of the opened tooth.

Five thousand endodontic cases treated by three endodontists were reviewed to determine the frequency of the need to leave teeth opened for drainage. The subsequent treatment of these teeth is described. Of the 5,000 cases reviewed, 518 were left open at some point during treatment. Two hundred and eighty-one cases (54.2 percent) remained closed after the first closure. Four hundred and twenty-six (82.2 percent) of the cases opened were eventually completed without surgical intervention. The need to prescribe antibiotics and perform a trephination is also stated.

Alveolar Process↗

Expression of the proliferating cell nuclear antigen in bone marrow cells from patients with myelodysplastic syndromes and aplastic anemia.

To determine the proliferative activity of the hematopoietic cells under nonneoplastic and/or neoplastic conditions, the expression of a cell cycle-related antigen, the proliferating cell nuclear antigen (PCNA), was examined in the bone marrow trephines of 79 individuals, 12 of whom had no hematologic disorder, 32 of whom had a diagnosis of myelodysplastic syndromes (MDSs), 20 of whom suffered from aplastic anemia, and 15 of whom had a diagnosis of acute myeloid leukemia. Most of the patients with MDS had more than 15% PCNA-positive cells (23.5% +/- 1.5%) while patients with no hematologic disorder showed fewer than 15% PCNA-positive cells (11.7% +/- 0.7%). The overall ratio of the PCNA-positive cell fraction in the bone marrow was considered of prognostic value for predicting transition into overt leukemia from MDS. Aplastic anemia cases usually exhibited hypocellular bone marrow and an infrequent labeling with the anti-PCNA antibody (3.3% +/- 0.5%). However, a few aplastic anemia cases showed hypercellular bone marrow and a significantly high PCNA-positive cell ratio (32.0% +/- 4.4%). In the bone marrow of acute myeloid leukemia patients more than 20% of total nucleated cells were positive for PCNA (30.0% +/- 2.2%). The results suggest that the expression of PCNA is associated with the regulation of bone marrow cell proliferation and the bone marrow cellularity, and that these findings would serve as an early indicator of evolution of overt leukemia in MDS and also would be useful in distinguishing MDS cases from aplastic anemia cases when the bone marrow is hypocellular or normocellular.

Acute Disease↗

Assessment of the value of immunohistochemistry in the subtyping of acute leukemia on routinely processed bone marrow biopsy specimens with particular reference to macrophage-associated antibodies.

The value of immunohistochemical staining in the subtyping of acute leukemia was investigated on 36 routinely processed (formalin-fixed and paraffin-embedded) trephine biopsy specimens from the iliac crest containing diffuse infiltrates of acute myelogenous leukemia (AML; n = 23) and acute lymphoblastic leukemia (ALL; n = 13). These were stained with a broad panel of antibodies (n = 23) against various leukocyte antigens, among them 11 macrophage-associated antibodies (MAAs): Ki-M1p, MAC387, HAM56, LN5, KP1 (CD68), PG-M1 (CD68), Ki-M4p, DAKO-DRC (CD35), and antibodies against lysozyme, alpha 1-antichymotrypsin, and S100 protein. The French-American-British (FAB) classification subtypes of the AML cases, as determined by enzyme-cytochemical and/or immunocytological investigation of bone marrow smears, were as follows: M1 = 6, M2 = 5, M4 = 7, M5 = 3, and AML (not classified) = 2. The 13 cases of ALL were classified as follows: c-ALL (pre-B-ALL) = 7, B-ALL = 3, T-ALL = 2, and ALL (not classified) = 1. All the MAAs except LN5, Ki-M4p, and DAKO-DRC stained blast cells in AML. However, the number of stained blast cells varied considerably within and between the individual subtypes (M4/5 > M2/1). Using Fisher's exact test a significant difference in frequency of blast cell staining between AML and ALL was found for four MAAs (anti-lysozyme, MAC387, Ki-M1p, and KP1) and two of the three myeloid cell markers applied (Ki-My2p and anti-neutrophil elastase). Of these six antibodies, the combination of anti-lysozyme and KP1 can be recommended for use in routine diagnostics for the differentiation of AML from ALL on the basis of immunohistochemical staining because both of these antibodies were found to stain a relatively large percentage of cases of AML but none of ALL. However, none of the MAAs were found to discriminate reliably between the FAB M4/5 and M1/2 subtypes of AML.

Antigens, CD↗

Growing fracture of the skull: report of an unusual case.

The authors report a case of a growing fracture of the skull in an elderly woman who remained asymptomatic for over 6 decades and presented at the age of 62 years with symptoms akin to the syndrome of the trephined. Successful duraplasty and cranioplasty were done with disappearance of all symptoms. A brief review of the relevant literature is also presented.

Diagnosis, Differential↗

Bone marrow biopsy during induction chemotherapy for acute myeloid leukaemia identifies only 50% of patients with resistant disease.

A study was carried out to determine whether bone marrow biopsy performed on day 6 of induction therapy for acute myeloid leukaemia (AML) can identify those patients with resistant disease who would need an intensification of the first course of induction. Bone marrow biopsies were performed on day 6 of induction chemotherapy in 44 patients with AML treated with daunorubicin, cytosine arabinoside and thioguanine. Biopsies were assessed for blast count, trephine cellularity and leukaemic index. Discrimination between patients who went on to achieve remission and those with resistant disease was best achieved using the reduction in bone marrow cellularity from pretreatment marrow to day-6 marrow. However, this discriminator identified only 50% of the patients with resistant disease and included 13% of patients who achieved remission with the first course of chemotherapy. The other parameters of response were even less effective at discriminating between chemotherapy-resistant and chemotherapy-responsive disease.

Antineoplastic Combined Chemotherapy Protocols↗