We report on 11 new cases of Proteus syndrome to illustrate the broad range of the phenotype in this hamartomatous dysplasia. The cardinal manifestations of this sporadic disorder are hemihypertrophy, macrodactyly, exostoses, scoliosis, cavernous hemangiomas, lipomas, linear sebaceous nevi, and deeply rugated soles of the feet. Intelligence is usually normal. The differential diagnosis includes Klippel-Trenaunay-Weber and partial lipodystrophy syndromes.

Adolescent↗

PubMed1987

Immunochemistry of the HLA class II molecules isolated from a mouse cell transfected with DQ alpha and beta genes from a DR4 haplotype.

Cells from a mouse B lymphoma were transfected by DQ alpha and DQ beta genes derived from a DR4 haplotype. Quantitatively, the resulting expression of human class II molecules was similar to that of human B lymphoblastoid cell lines. Qualitatively, the transformant class II molecules differed from "normal" class II molecules in their carbohydrate moiety. As for their antigenic specificity, they were shown to carry two determinants previously identified on DQ molecules controlled by DR4 haplotypes, i.e., DQw3 and DCHON. The transformant molecules did not carry a third DR4-associated specificity, DC5 (equivalent to TA10), and must possess a structure allelic to DC5. However, no corresponding alloantigenic specificity was detected by a screening of relevant alloantisera.

Animals↗

PubMed1987

Structure and expression of HLA-DQ alpha and -DX alpha genes: interallelic alternate splicing of the HLA-DQ alpha gene and functional splicing of the HLA-DQ alpha gene using a retroviral vector.

The nucleotide sequences of the two closely related HLA-DQ alpha and HLA-DX alpha genes have been determined. Exons coding for the signal peptide, alpha 2 and transmembrane domains are 94-99% homologous, whereas the alpha 1 exon and the promoter region have diverged as much as or more than introns and the 3' untranslated region. The promoter regions of both genes contain two short sequences thought to be important for regulation of transcription by gamma-interferon. Transfection studies established that the DQ alpha and DQ beta genes encode the HLA-DQ antigen. Transcripts of varying length are produced from different alleles as the result of the use of alternate splice and polyadenylation signals at the 3' end of the DQ alpha gene. Thus typing at the DQ alpha locus can be achieved by Northern blot analysis. No transcript of DX alpha was detected in B lymphocytes. The DX alpha gene was accurately spliced when introduced into a retroviral vector, suggesting that the lack of expression of DX alpha is not due to aberrant splice signals.

Alleles↗

PubMed1987

Antibodies in sera from patients with myasthenia gravis do not bind to nicotinic acetylcholine receptors from human brain.

Nicotinic acetylcholine receptors (AChRs) from brains of chickens and rats have recently been purified and characterized (Whiting and Lindstrom, Biochemistry, 25 (1986) 2082-2093; J. Neurosci., 6 (1986) 3061-3069; Proc. Natl. Acad. Sci. U.S.A., 84 (1987) 595-599). Using both antisera and monoclonal antibodies prepared to AChRs from rat brain, we have demonstrated the existence of a homologous AChR in human brain. Here we report that antibodies to muscle AChRs in the sera of patients with myasthenia gravis (MG) do not bind to AChRs from human brain. Similarly, there was no binding of sera from patients with Guillain-Barré, amyotrophic lateral sclerosis, multiple sclerosis, or Lambert-Eaton myasthenic syndrome. Additionally, no binding of any of these sera to the alpha-bungarotoxin (alpha-Bgt) binding protein from human brain could be detected. This data is consistent with other data using antibodies to AChRs from muscle and nerve in demonstrating that the AChR in brain is antigenically distinct from the AChR in skeletal muscle AChR, and, together with the lack of central neurological symptoms in MG, suggests that the low concentrations of anti-AChR antibodies in the cerebrospinal fluid of MG patients do not bind to AChRs in brain.

Antibodies↗

PubMed1987

Investigation into the effects of ingestion of zinc shot by Mallard Ducks (Anas platyrhynchos).

Lead fishing weights and gun shot have been identified as a widespread cause of poisoning of swans and other wildfowl (order Anseriformes). As part of the efforts to find environmentally acceptable substitutes for lead, this paper describes the results obtained from a trial which involved the feeding of zinc shot to groups of mallard (Anas platyrhynchos). Observations of the mallard during the experimental period, post mortem examination, histo-pathological examination and also chemical analysis of liver, kidney and feathers, revealed no ill effects to the dosed birds and indicated that zinc metal in shot from probably presents no detectable health threat to wild birds if ingested.

Journal Article↗

PubMed1987

Radiotherapy with or without misonidazole for patients with stage IIIB or stage IVA squamous cell carcinoma of the uterine cervix: preliminary report of a Radiation Therapy Oncology Group randomized trial.

Between August 1980 and November 1984, 119 patients with FIGO Stage IIIB or IVA squamous cell carcinoma of the uterine cervix were randomized to receive radiation therapy (4600 cGy pelvis plus 1000 cGy parametrial boost) followed by intracavitary or external boost to the primary with or without misonidazole (MISO) (400 mg/m2 daily 2 to 4 hours prior to radiation therapy). Patients in the two treatment groups were evenly distributed with respect to stratification variables including stage, Karnofsky Performance score, and positivity of para-aortic nodes. Eighty-nine percent of patients had Stage IIIB disease and 88% had a Karnofsky score of 80 or better. Seventy-five percent of patients treated with radiation therapy alone and 79% of patients treated with radiation therapy plus MISO received a boost via intracavitary application. Life threatening (Grade 4) complications occurred in 5 patients receiving radiation therapy alone and one patient receiving radiation therapy plus MISO. MISO toxicity (Grade 3) was limited to severe nausea and vomiting in two patients. With 119 evaluable patients and a median follow-up of 33 months, 64% of patients receiving radiation therapy alone are alive at 18 months compared with 54% for patients assigned to radiation therapy plus MISO. The median survival for patients treated with radiation therapy alone and radiation therapy plus MISO was 1.9 years and 1.6 respectively. At this point in the study the difference in survival is inconsistent with the hypothesis of an improvement associated with MISO. There have been 23 deaths among the 49 patients treated with radiation therapy plus MISO who have been followed for at least 18 months compared with 17 deaths in 48 patients treated with radiation therapy alone. The chance of observing this number of deaths with radiation therapy plus MISO if the addition of MISO improves survival by 10 to 20% is 0.003 and less than 0.001, respectively. The addition of MISO to radiation failed to improve survival for these patients. The results cannot be explained by an uncharacteristically high survival on the radiation therapy alone arm or by an imbalance in the distribution of prognostic factors. Local-regional control remains a problem in the management of patients with advanced cervical carcinoma. More effective and less toxic radiosensitizing agents are needed.

Adolescent↗

PubMed1987

Crystallographic studies of reduced bond inhibitors complexed with an aspartic proteinase.

To aid in the design of an effective inhibitor to human renin, it is essential to have a detailed knowledge of how this aspartic proteinase interacts with its substrate, angiotensinogen. Human renin shows a stringent specificity toward the Leu-Val bond in its natural substrate. The minimal length for an effective substrate has been characterised as an octapeptide sequence derived from the amino terminal portion of angiotensinogen (residues 6----13): His-Pro-Phe-His-Leu-Val-Ile-His (Leu-Val is the scissile bond). This suggests that renin has a fairly extensive active site cleft, as has been observed in homologous enzymes whose three-dimensional structures have been solved using x-ray diffraction methods. The homologous fungal aspartic proteinase, endothiapepsin, has been cocrystallised with human renin inhibitors of the type His-Pro-Phe-His-Leu-R-Val-Ile-His, where R indicates a reduced carbonyl analogue of the scissile peptide bond. The three-dimensional crystallographic structures of two complexes of endothiapepsin with an inhibitor have been solved. The details of inhibitor binding at the active site cleft of endothiapepsin are described. These data allow a rational approach to the design of novel renin inhibitors, through studies of these inhibitors in a three-dimensional model of human renin constructed in our laboratory.

Amino Acid Sequence↗

PubMed1987

Reduction of asthenopia after accommodative facility training.

Five patients reporting asthenopia secondary to accommodative deficiencies underwent automated accommodative facility training. A matched-subjects, crossover design was used to control for placebo effects. All patients receiving automated accommodative training showed a marked increase in accommodative amplitude along with a concurrent reduction of asthenopia. Decreases of blur and increases of reading time were the most frequently reported changes by patients. This experiment shows the effectiveness of automated accommodative training in reducing asthenopia and improving accommodative facility.

Accommodation, Ocular↗

PubMed1987

Termination of spontaneous atrial flutter by transesophageal pacing.

Transesophageal atrial pacing using the constant-rate technique was performed in 26 patients presenting with spontaneous atrial flutter (atrial cycle length between 180 and 270 ms). All but one patient had been treated with one or more antiarrhythmic agents (digoxin, quinidine, procainamide, propranolol, verapamil, diltiazem, and propafenone) within the previous 12 hours. Transesophageal atrial pacing at cycle lengths between 80 and 180 ms was successful in terminating atrial flutter in 22 patients: immediate reversion to sinus rhythm in 16, following transient sinus pause in one, following a brief period of atrial fibrillation in three, and following longer periods of atrial fibrillation in another two. No post-conversion ventricular arrhythmia and no other complications were observed. All patients experienced only a mild burning discomfort during the procedure. It is concluded that atrial pacing via the esophagus is a safe and noninvasive technique of terminating spontaneous atrial flutter. The effectiveness of this technique is comparable to endocardial or epicardial atrial pacing.

Adolescent↗

PubMed1987

Expression of HLA-DR antigen in human class II mutant B-cell lines by double infection with retrovirus vectors.

A new retrovirus vector containing the gene for hygromycin B resistance (hyg) as a selectable marker under the control of an internal simian virus 40 promoter was constructed. It was used, together with an analogous previously described vector, DO1, which contains the gene for G418 resistance, to introduce and express the genes for the two chains of a human class II major histocompatibility complex antigen in NIH 3T3 cells. In addition, these vectors were used to express DR antigens in two human mutant B-lymphoblastoid cell lines, one of which was deleted for both alleles of the DR alpha gene and the other of which expressed no class II antigens because of a genetic defect in a putative trans-acting regulatory factor.

Animals↗

PubMed1987

Depressive syndromes in the year following onset of a first schizophrenic illness*.

A complete, unselected series of 68 patients who were seen during their first episode of an undoubtedly schizophrenic illness, and followed up one year later (for 56 patients) is described clinically. Depressive symptoms were common at onset, and 22% of patients could have been considered cases of depression from these symptoms alone. At follow-up, depressive symptoms had reduced in prevalence and only 7% of subjects were depressed cases. Only two cases of depression at follow-up had not been cases at onset. These changes could not be attributed to the use of antidepressants or ECT. Depressive syndromes could be distinguished from akinesia and the negative syndromes. The findings indicate that depression cannot be attributed solely to the administration of neuroleptics.

Adolescent↗

PubMed1987

Head nurses: their practice and education.

The head nurse has become a critical link in managing successful hospital units in this post-DRG (Diagnosis Related Group) era. As more nursing services move to a decentralized model, the head nurse role has grown in complexity and accountability far beyond entry level nursing preparation. This study explores the patterns of use of head nurses in United States hospitals and how they should be educated.

Clinical Competence↗

PubMed1986

Isomorphism of dc-field-induced interference and laser-induced effects in autoionization.

Explore the source record for details and available documents.

Journal Article↗

PubMed1986

Effect of misonidazole dose on survival in patients with stage IIIB-IVA squamous cell carcinoma of the uterine cervix: an RTOG randomized trial.

Between August 1980 and November 1984, 120 patients with FIGO Stage IIIB or IVA squamous cell carcinoma of the uterine cervix were randomized to receive radiation therapy (RT) (46 Gy pelvis + 10 Gy parametrial boost) followed by intracavitary or external boost to the primary +/- misonidazole (MISO) (400 mg/M2 2-4 hours prior to RT daily, maximum 12 gm/M2). The median at 24-28 hr misonidazole plasma level was 20 micrograms/ml 2-6 hr and 3.5 micrograms/ml. Approximately 60% of the patients on RT + MISO received 100% of expected total Misonidazole dose; peripheral neurologic toxicity was reported for nine patients receiving misonidazole (8 with mild and 1 with moderate paresthesia or pain). Time-dependent regression analyses found that actual cumulative misonidazole dose was not related to duration of survival from start of treatment (p = 0.5). MISO dose expressed as a percent of expected dose was marginally related to increased survival measured from 14 weeks on on study (p = 0.1). No improvement in survival was observed with the addition of misonidazole to RT (64% of the patients on RT alone were alive at 18 months versus 54% of those on RT + MISO).

Carcinoma, Squamous Cell↗