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Biomedical subjects

L S Levin

Publications and source records attributed to L S Levin.

At least 19 recordsLinked to original sources

Thalidomide for the treatment of chronic graft-versus-host disease.

BACKGROUND: Allogeneic bone marrow transplantation is an accepted therapy for hematologic cancer, aplastic anemia, and inherited immunodeficiencies. Chronic graft-versus-host disease (GVHD) is the principal complication in patients surviving more than 100 days. Thalidomide has been shown experimentally to be effective in treating GVHD. METHODS: We treated 23 patients with chronic GVHD refractory to conventional treatment and 21 patients with "high-risk" chronic GVHD (identified as having at least two of the following three risk factors: chronic GVHD that has evolved from acute GVHD, lichenoid skin or mucous-membrane changes, and hepatic dysfunction. Such patients have a high mortality rate.) with thalidomide in a dose that produced a plasma level of 5 micrograms per milliliter two hours after administration. Therapy was continued for three months after a complete response or for six months after a partial response. RESULTS: The overall actuarial survival of all enrolled patients was 64 percent. Survival was 76 percent among the patients receiving salvage therapy for refractory GVHD and 48 percent among those with high-risk GVHD. A complete response was observed in 14 patients, a partial response in 12 patients, and no response in 18. Side effects were minor, most notably sedation in almost all patients. CONCLUSIONS: In this preliminary trial, thalidomide appeared to be safe and effective for the treatment of chronic GVHD. A trial comparing thalidomide with prednisone in patients with newly diagnosed chronic GVHD will be required to demonstrate its relative efficacy.

Adolescent

Direct end-to-end repair of flexor pollicis longus tendon lacerations.

Between 1976 and 1986, 38 consecutive acute isolated flexor pollicis longus lacerations were repaired. This study excluded all replanted or mutilated digits and all lacerations with associated fracture. Average follow-up was 26 months. Tendon rehabilitation was standardized. Range of motion and pinch strength were measured postoperatively. Seventy-four percent (28/38) of the flexor pollicis longus injuries occurred in zone II. Neurovascular injury occurred in 82% of the lacerations, and this correlated with the zone of tendon injury. In 21% of the patients (8/38) both digital nerves and arteries were transected. Postoperative thumb interphalangeal motion averaged 35 degrees and key pinch strength was 81% that of the uninjured thumb. One rupture occurred in a child. Laceration of the flexor pollicis longus is likely to involve damage to neurovascular structures, and repair may be necessary. Direct end-to-end repairs within the pulley system do at least as well as delayed tendon reconstruction and do not require additional procedures.

Adolescent

Submandibular cystic hygroma resembling a plunging ranula in a neonate. Review and report of a case.

Cystic hygromas are large lymphangiomas that are most often found in the posterior triangle of the neck and the axilla in children. They are most frequently found before age 2 and may be massive. After upper respiratory infection, they may become infected and enlarged, causing dysphagia and toxemia. The diagnosis can usually be made by history and physical examination and confirmed by biopsy. Treatment is by surgical excision of small lesions and staged debulking excisions in more severe cases. A patient with a cystic hygroma having many clinical characteristics of a plunging ranula is presented. The cyst fluid was aspirated and analyzed for its amylase, sodium, potassium, chloride, urea nitrogen, glucose, and total protein content. The characteristics of the fluid were also compared with those of lymph and saliva. This report demonstrates the difficulty in determining the diagnosis of a tumor that has the clinical features of a cystic hygroma, as well as a plunging ranula. The necessity of a proper presurgical diagnosis is essential since the form of therapy for each is different and conflicting. A method that distinguishes between the cervical cystic hygroma and a plunging ranula by means of aspirated fluid is discussed.

Cleft Lip

Oral mucositis after bone marrow transplantation. A marker of treatment toxicity and predictor of hepatic veno-occlusive disease.

Oral mucositis and hepatic veno-occlusive disease (HVOD) are common complications after bone marrow transplantation (BMT). Forty-seven patients were prospectively examined for development of ulcerative oral mucositis (UOM) and HVOD after allogeneic BMT. In 17 patients (36%) UOM developed between 2 days before and 18 days after transplant (median 4 days after BMT). In seven patients (15%) HVOD developed with onset between 3 and 21 days after transplant (median 18 days after BMT). In a time-dependent analysis, in patients given busulfan plus cyclophosphamide or busulfan, etoposide, and cyclophosphamide UOM was 19 times more likely to develop than in patients treated by cyclophosphamide plus total body irradiation or by cyclophosphamide alone (p less than 0.001). Patients in whom UOM developed were 6.5 times more likely to develop HVOD than those in whom UOM did not develop (p less than 0.03). The sensitivity (86%), specificity (73%), and negative predictive value (97%) of UOM to predict HVOD were high, but the positive predictive value (35%) was low. The association of UOM and HVOD support the concept that both are toxic effects of treatment. Patients with hepatic abnormalities but without UOM are unlikely to have HVOD, and other causes of the hepatic dysfunction should be investigated.

Adolescent

Detection of anti-Ro(SSA) antibodies by gel double diffusion and a 'sandwich' ELISA in systemic and subacute cutaneous lupus erythematosus and Sjögren's syndrome.

A newly described Ro 'sandwich' ELISA was compared to the gel double diffusion technique to detect anti-Ro(SSA) antibodies in Sjögren's syndrome, systemic and subacute cutaneous lupus erythematosus patients. This study demonstrates that the ELISA assay increased the frequency of detection of anti-Ro(SSA) antibodies in these well defined connective tissue disease patients by approximately 5-10% compared to the gel double diffusion anti-Ro(SSA) antibody assay. The study also confirms that some patients make anti-Ro(SSA) antibodies directed solely at unique human Ro(SSA) antigen epitopes. We also detected the existence of a significant Sjögren's syndrome patient population failing to make significant anti-Ro(SSA) antibodies. We conclude from our study that the gel double-diffusion technique employing human spleen extract as a source of the Ro(SSA) antigen is, at present, the most cost-effective test to detect anti-Ro(SSA) antibodies.

Antibodies, Antinuclear

Value of combined phenotypic markers in identifying inheritance of familial adenomatous polyposis.

Familial adenomatous polyposis is an autosomal dominant disease characterised by the development of hundreds of colorectal adenomas in young adults. Occult radio-opaque jaw lesions and pigmented ocular fundus lesions (formerly called congenital hypertrophy of the retinal pigment epithelium) are extraintestinal phenotypic markers for this disorder. We evaluated the usefulness of the combination of these markers for identifying patients who have inherited familial adenomatous polyposis. Forty three affected patients and 12 unaffected first degree relatives from 24 families with familial adenomatous polyposis, including four families without extraintestinal manifestations, were examined for both phenotypic markers. Thirty three of the 43 patients (77%) with familial adenomatous polyposis were positive for both markers, including patients from two families without extraintestinal manifestations. By contrast, only one of 12 (8%) unaffected first degree relatives over 35 years of age had both markers. The sensitivity of the combination of these markers in identifying patients who inherited familial adenomatous polyposis was 77%, the specificity 92%, the predictive value of a positive test 97%, the predictive value of a negative test 52%, and the efficacy 80%. The combined markers had improved efficacy over either marker alone (70% for occult radio-opaque jaw lesions and 67% for pigmented ocular fundus lesions). We conclude that the presence of both occult radio-opaque jaw lesions and pigmented ocular fundus lesions in a person at risk indicates a high probability of inheritance and expression of familial adenomatous polyposis.

Adenomatous Polyposis Coli

Assignment of the Nance-Horan syndrome to the distal short arm of the X chromosome.

There are three types of X-linked cataracts recorded in Mendelian Inheritance in Man (McKusick 1988): congenital total, with posterior sutural opacities in heterozygotes; congenital, with microcornea or slight microphthalmia; and the cataract-dental syndrome or Nance-Horan (NH) syndrome. To identify a DNA marker close to the gene responsible for the NH syndrome, linkage analysis on 36 members in a three-generation pedigree including seven affected males and nine carrier females was performed using 31 DNA markers. A LOD score of 1.662 at theta = 0.16 was obtained with probe 782 from locus DXS85 on Xp22.2-p22.3. Negative LOD scores were found at six loci on the short arm, one distal to DXS85, five proximal, and six probes spanning the long arm were highly negative. These results make the assignment of the locus for NH to the distal end of the short arm of the X chromosome likely.

Abnormalities, Multiple

Distal rupture of the palmaris longus tendon and fascia as a cause of acute carpal tunnel syndrome.

Acute carpal tunnel syndrome is rare compared with its more chronic presentation. Previous reports in the literature have documented the most common causes. Rupture of the distal palmaris longus tendon into the palmaris fascia as a cause of an acute carpal tunnel syndrome has not been reported previous to this case report. Partial rupture of the tendon and hemorrhage around its insertion produced intrinsic compression on the transverse ligament and the underlying nerve.

Acute Disease

Human papillomavirus in squamous cell carcinoma, leukoplakia, lichen planus, and clinically normal epithelium of the oral cavity.

Tissue specimens of carcinoma, leukoplakia, and clinically normal epithelium obtained at sites separate from the lesions were examined for the presence of human papillomavirus (HPV). Twenty-two paraffinized specimens of previously diagnosed oral lichen planus were also studied. The carcinoma and leukoplakia specimens were examined by Southern transfer hybridization and reverse blot hybridization; specimens HPV-positive by Southern hybridization were additionally examined by in situ hybridization and an immunoperoxidase technique. The lichen planus specimens were examined by in situ hybridization and immunoperoxidase techniques only. The HPV identification rates were in the range reported in previous studies, and the detection rates were similar for carcinoma, leukoplakia, histologically normal epithelium, and lichen planus. The clinical significance of HPV presence in carcinoma, leukoplakia, and lichen planus was not evaluable because of the short duration of follow-up.

Adult

Management of severe musculoskeletal injuries of the upper extremity.

Limb salvage was successful in 25 patients treated for severe grade III upper extremity injuries. In a retrospective review of 20 men and five women, follow-up time averaged 26 months. These high-energy injuries were characterized by massive soft-tissue injury, highly comminuted fractures, and significant neurovascular injury. Farm, industrial, and vehicular accidents accounted for 80% of the cases. Over 50% of the patients had concomitant systemic and/or other significant extremity injuries. Initial treatment consisted of irrigation and debridement and fracture stabilization using external and/or internal fixation. An average of four additional surgical procedures was required to provide soft-tissue coverage and maximum possible functional recovery. Forty-eight percent of the extremities underwent free vascularized or pedicular flaps for coverage or reconstruction. At final follow-up observation, 12% of the extremities rated excellent, 20% rated good, 52% fair, and 16% were poor. Experience gained in managing these severe upper extremity fractures supports the following observations. (a) Grade III open fractures of the upper extremities are frequently associated with significant neural, vascular, and musculotendon injuries. (b) External fixation plays an important role in the stabilization of grossly contaminated fractures. (c) Residual functional disability is common, and most patients do not return to their previous occupation. (d) Staged reconstruction directed toward maximum functional return may take several years.

Adolescent

Adenomatoid odontogenic tumor: ultrastructural demonstration of two cell types and amyloid.

A typical adenomatoid odontogenic tumor removed from a 13-year-old female was studied by light and electron microscopy. The tumor was composed of two types of epithelial cells: Type I cells were cuboidal and occurred in nests or formed ductlike structures and Type II cells were smaller and spindle shaped. The formation of extracellular masses of amyloid was found in association with Type I epithelial cells, and amyloid formation was not observed in association with Type II cells. Results suggest that the lesion is of enamel organ origin, derived from cells of the inner enamel epithelium at the pre-ameloblastic stage, stellate reticulum and stratum intermedium. The origin of this amyloid material is unknown; however, it may be of enamel protein origin which, like amyloid, may have a beta-protein conformation.

Adolescent

Treatment of the ameloblastoma: a controversy.

Treatment of the ameloblastoma should be re-evaluated based on microscopic behavior. We suggest that conservative therapy is the initial treatment of choice. Although medullary bone is invaded by tumor cells, compact bone only is eroded. Therefore, treatment should be directed at removal of involved medullary bone, leaving as much medial and lateral cortical plates and inferior mandibular border as possible. Four patients with ameloblastoma treated conservatively are presented. Evaluation from 21 months to seven years after initial therapy revealed marked bone regeneration. Three of the four had smaller lesions remaining, requiring less radical surgery than would normally have been performed initially. No tumor was evident on rebiopsy of the fourth patient. Conservative treatment and proper follow-up were acceptable methods of initial treatment in our cases.

Adult

The Rieger syndrome.

Fourteen patients with hypodontia and the ocular features of the Rieger syndrome were examined for the presence of systemic anomalies. A periumbilical defect that consisted of failure of the periumbilical skin to involute was seen in ten of the thirteen evaluated for the defect. Three others had scars over the umbilical area and had a history of surgery for herniation. In addition, four males in one family and one male from another family had hypospadias. None of several other anomalies reported to be components of the Rieger syndrome by other authors was detected in the fourteen patients. The mode of inheritance in the familial cases studied was compatible with autosomal dominance. The results of this study indicate that the Rieger syndrome is an autosomal dominant syndrome whose cardinal features are hypodontia, goniodysgenesis, and failure of the periumbilical skin to involute properly.

Abnormalities, Multiple