Spontaneous ovarian hyperstimulation syndrome in four consecutive pregnancies.

BACKGROUND: Ovarian hyperstimulation syndrome (OHSS) is almost exclusively associated with ovulation induction with gonadotropins or occasionally, clomiphene citrate. Severe ovarian hyperstimulation associated with a spontaneously conceived pregnancy is rare with only two previous reports. Misdiagnosis as a neoplastic process may result in inappropriate intervention. CASE: A patient with polycystic ovarian disease experienced severe spontaneous OHSS in four consecutive singleton pregnancies. Serial color and pulsed Doppler ultrasonographic imaging facilitated adequate evaluation of the patient and permitted conservative therapy. She achieved live births in two of the pregnancies. This is the third reported case of spontaneous OHSS associated with pregnancy and the first to result in live births. In one previous report, misdiagnosis resulted in inadvertent castration. CONCLUSIONS: Spontaneous ovarian hyperstimulation syndrome and pregnancy may occur in patients with polycystic ovarian disease. Color Doppler ultrasonography is a useful aid in the diagnosis and management of this rare complication, and in avoiding inappropriate intervention.

The limited spectrum of antinuclear antibodies in leprosy.

Sera from 46 consecutive patients with leprosy were collected and tested against an extensive panel of defined nuclear antigens. Antinuclear antibodies (ANA) were present in 16% of patients, but the titer was uniformly low and there was no consistent fluorescence pattern. None of the ANA positive sera contained antibodies which reacted with native DNA, or which were directed against histones, centromeres, SSB, Sm, or ribonucleoprotein. These more specific autoantibody assays thus retained their clinical utility in the differential diagnosis of rheumatologic complaints in patients with leprosy.

Changes in pituitary hormones during and following transsphenoidal removal of prolactinomas.

Prolactin (PRL) and other pituitary hormones (luteinizing hormone, follicle-stimulating hormone, growth hormone [GH], thyrotropin stimulating hormone) were measured before, during, and after transphenoidal pituitary adenomectomy in 16 patients with hyperprolactinemia. The diagnosis of prolactinoma was made in three of the 16 patients by the absence of PRL response to thyrotropin-releasing factor (TRF) and a dopamine receptor antagonist, metoclopramide, without radiologic evidence of an adenoma. Contrary to findings in subjects with normal PRL values, the PRL rise in response to anesthesia and operation was absent. Other pituitary hormones, with the exception of GH, which increased during anesthesia and operation, exhibited no acute changes. In 11 of 16 cases, complete tumor removal was achieved as determined by the rapid decline of PRL levels to normal values within 24 to 48 hours after operation and by subsequent clinical follow-up. This finding documents that the adenoma is the main source of excessive PRL secretion. The circulating half time of immunoreactive PRL determined by frequent sampling in these patients was variable, ranging from 74 to 190 minutes, significantly longer than the previously reported value of 15 minutes determined by bioassay. Although a transient decline was evident, serum PRL levels remained elevated in those patients with incomplete tumor removal. These findings suggest that a single measurement of serum PRL within 24 to 48 following transphenoidal adenomectomy is a reliable indicator of the success or failure of the procedure.

Substituent effects on the electron transfer reactivity of hydroquinones with laccase blue copper.

Stopped-flow kinetic studies of the anaerobic reduction of Rhus vernicifera laccase (monophenol, dihydroxyphenylalanine:oxygen oxidoreductase, EC 1.14.18.1) type 1 copper by 25 mono- and disubstituted hydroquinones (H2Q-X) have been performed at 25 degrees C and pH 7.0 in 0.5 M phosphate. All of the data are compatible with a mechanism involving rapid enzyme-substrate complex formation followed by rate-limiting intra-complex electron transfer. ES complex formation constants (Qp) for many substrates are strikingly insensitive to the electronic characteristics of the substituent X, falling within the range 5--50 M-1. It is shown that this result may be accounted for if only the singly ionized forms of the substituted hydroquinones are bound by the enzyme. All of the substrates exhibiting exceptionally high Qp values (greater than 50 M-1) have X groups capable of functioning as ligands; substituents with lone pairs of electrons may facilitate enzyme-substrate complex formation by enabling hydroquinone to function as a bidentate bridging ligand between the type 2 and type 3 copper sites. Intra-complex electron transfer rate constants for most substrates are remarkably insensitive to the thermodynamic driving force for the oxidation of H2Q-X to the corresponding semiquinone, the average value for ten substrates being 30 +/- 10 s-1. The electron transfer reactivity of polyphenols with laccase blue copper therefore appears to be controlled largely by protein-dependent activation requirements rather than by the oxidizability of the substrate.

Detection of intermediate complexes by evaluation of the difference between gamma-globulin and IgG concentrations.

A simple analysis based on techniques readily available in the routine clinical laboratory has been presented for identification of patients with moderate and high levels of intermediate complexes. If the gamma-globulin concentration determined by serum protein electrophoresis minus the IgG concentration determined by radial immunodiffusion exceeds 1.0 gm/dl, intermediate complexes of IgG should be present in concentrations greater than 0.5 gm/dl. In principle, any disease association with sufficient polymerization of IgG might cause an abnormal result with this analysis. Furthermore, sera from patients with liver disease and marked beta-gamma-bridging on electrophoresis may also give abnormal values.

Effect of immunization on susceptibility to experimental Streptococcus mutans and Streptococcus sanguis endocarditis.

It has been asserted that humoral immunity is an important potentiating factor in pathogenesis of infective endocarditis, in that prior immunization to certain bacteria may predispose the host to endocarditis caused by those organisms. If so, possible future vaccination of humans with streptococcal antigens for the prevention of dental caries might increase the susceptibility of the population to streptococcal endocarditis. To examine this hypothesis further, we immunized rabbits with killed Streptococcus sanguis or Streptococcus mutans. After complement-fixing antibody had developed, the rabbits were tested for susceptibility to experimental infective endocarditis. Rabbits with high titers of complement-fixing antibody to the infecting organism developed streptococcal endocarditis less often (13%) than animals with lower titers (69%; P less than 0.0002). These findings do not support the hypothesis that pre-immunization predisposes to infective endocarditis and lend no credence to the concept that vaccination of human subjects against dental caries might increase their susceptibility to streptococcal endocarditis. On the contrary, the results of these experiments indicate that specific antibody can confer relative immunity to infective endocarditis.

Synthesis by an established lymphocyte cell line from a rheumatoid synovium.

Lymphocytes derived from the synovium of a patient with rheumatoid arthritis were shown to synthesize predominantly IgG as measured by an equilibrium binding assay. Chromatographic separation of the radiolabeled lymphocyte culture supernatant revealed rheumatoid factor activity associated with IgG and IgM. In addition, immunofluorescence studies on fixed lymphocytes demonstrated that the majority of cells stained positive for both IgG and rheumatoid factor.

Immunologic quantitation of serum immunoglobulins.

The methods available in the clinical laboratory for measuring serum immunoglobulins (G, A, M, and D) include radial immunodiffusion, electroimmunodiffusion and nephelometry. All these immunochemical technics require specific antisera and stable standards for reproducible and accurate results. Discrepancies between the quantity of protein measured by any of these technics and the actual chemically or physically measured quantity arise when the unknown and standard differ in molecular size, electrophoretic mobility, or antigenic valence. These differences may lead to over- or under-estimation of the quantity of immunoglobulin. Quantitation of immunoglobulins, especially in context with protein electrophoresis, does provide useful information for diagnosis and treatment of selective disorders.

Severe systemic lupus erythematosus with nephritis in a boy with deficiency of the fourth component of complement.

A young boy with severe systemic lupus erythematosus was found to be totally deficient in the fourth component of complement. Family studies were consistent with an autosomal recessive mode of transmission and with linkage of the gene(s) determining C4 deficiency to the major histocompatibility complex; no disease states were associated with heterozygosity. This patient has had severe multisystem disease and immune complex glomerulonephritis presumably the alternative pathway of complement was utilized in the pathogenesis of his nephritis.

Linkage between the gene (or genes) controlling synthesis of the fourth component of complement and the major histocompatibility complex.

In an attempt to map the gene (or genes) controlling the synthesis fo the fourth component of complement (C4), we performed linkage studies in a family with hereditary C4 deficiency. The proband, a seven-year-old boy with lupus erythematosus, consistently lacked deteftable serum C4 by both functional and protein measurements. The complement defect was transmitted as an autosomal recessive disorder. Eight of 15 family members were considered to be heterozygotes, seven because of low C4 levels and one because of genetic data (obligate heterozygote). The gene (or genes) coding for C4 deficiency appeared to be linked to the major histocompatibility complex (A2,B12,DW2 on the maternal side and A2,BW15,LD108 on the paternal side) and to other markers known to be in close proximity to the histocompatibility complex on chromosome 6 (phosphoglucomutase-3, glyoxalase-1 and properdin factor B).

Coombs--negative immune hemolytic anemia.

An immune hemolytic anemia occurs in a few patients in whom the concentration of antibody on the red cell is below the level for detection by the usual antiglobulin test. Clinically, these patients are identical to patients with warm type Coombs-positive hemolytic anemia, except for the quantity of antibody on the cell. The course of the hemolytic disease is highly variable. It is postulated that the properties of the antibody in conjunction with the sensitivity of the reticuloendothelial system for antibody-coated cells account for hemolytic anemia occurring with such low concentrations of antibody. The response to steroid therapy and splenectomy, when indicated, is usually favorable.

Uterus didelphys associated with unilateral imperforate vagina.

Two cases of uterus didelphys associated with unilateral imperforate vagina are reported with a review of the literature. In all cases investigated, ipsilateral renal agenesis was found on the affected side. A summary of the embryologic development of the female reproductive tract is included, as well as a possible explanation for this rare anomaly. The authors describe their proposed method of management to achieve freedom from symptoms and maintenance of a relatively intact reproductive tract.

An intraluminal transducer/telemetry system for oviductal motility studies.

A method for measurement of changes in lumen diameter of the oviduct has been developed. Miniature intraluminal "doughnut-shaped" transducers, 1.0-1.2 mm in diameter, constructed from conductive silicone rubber are implanted in the rabbit isthmus and respond to localized changes--increases or decreases--in lumen diameter. Multiple transducer implants in the same tube permit rate as well as direction of contractions to be determined. A custom-designed, micropower dual-channel telemetry system was fabricated to record motility from unrestrained animals. Typical data collected using this system to determine the effects of induced ovulation on instantaneous changes in lumen diameter are presented.

The hyperviscosity syndrome in rheumatoid arthritis due to intermediate complexes formed by self-association of IgG-rheumatoid factors.

Three patients with rheumatoid arthritis and abundant circulating intermediate complexes were studied. Two of these patients presented with the hyperviscosity syndrome. All 3 patients had markedly elevated plasma and blood viscosity, and the intermediate complexes were thought to be responsible for the increased viscosity. Studies on the isolated intermediate complexes revealed that they were formed by self-association of IgG-rheumatoid factors.