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Biomedical subjects

H E Hamilton

Publications and source records attributed to H E Hamilton.

At least 19 recordsLinked to original sources

Does indirect speech promote nondirective genetic counseling? Results of a sociolinguistic investigation.

To date, research examining adherence to genetic counseling principles has focused on specific counseling activities such as the giving or withholding of information and responding to client requests for advice. We audiotaped 43 prenatal genetic counseling sessions and used data-driven, qualitative, sociolinguistic methodologies to investigate how language choices facilitate or hinder the counseling process. Transcripts of each session were prepared for sociolinguistic analysis of the emergent discourse that included studying conversational style, speaker-listener symmetry, directness, and other interactional patterns. Analysis of our data demonstrates that: 1) indirect speech, marked by the use of hints, hedges, and other politeness strategies, facilitates rapport and mitigates the tension between a client-centered relationship and a counselor-driven agenda; 2) direct speech, or speaking literally, is an effective strategy for providing information and education; and 3) confusion exists between the use of indirect speech and the intent to provide nondirective counseling, especially when facilitating client decision-making. Indirect responses to client questions, such as those that include the phrases "some people" or "most people," helped to maintain counselor neutrality; however, this well-intended indirectness, used to preserve client autonomy, may have obstructed direct explorations of client needs. We argue that the genetic counseling process requires increased flexibility in the use of direct and indirect speech and provide new insights into how "talk" affects the work of genetic counselors.

Decision Making↗

Adult Niemann-Pick disease masquerading as sea blue histiocyte syndrome: report of a case confirmed by lipid analysis and enzyme assays.

We present the clinical, pathologic, and metabolic findings of an adult woman with debilitating coronary artery disease and hepatosplenomegaly who was discovered to have multiorgan infiltration by sea blue histiocytes. A diagnosis of sea blue histiocyte (SBH) syndrome was made and no further workup performed. The patient suffered from progressive heart failure and sepsis following coronary artery bypass surgery and died 9 months after presentation. Tissues examined at autopsy showed pronounced infiltrates of both granular sea blue histiocytes and foamy, vacuolated histiocytes, which were morphologically compatible with Niemann-Pick cells. Ultrastructural examination of these cells revealed lamellar myelin-like figures as described in Niemann-Pick (N-P) disease. Fibroblast enzyme assay studies and liver lipid analyses performed after the patient's death revealed pronounced sphingomyelinase deficiency and a lipid profile diagnostic of N-P disease, type B. This case adds further support to the claim that some cases of apparent SBH syndrome actually represent a type of N-P disease.

Biopsy↗

Recurrent thrombotic microangiopathy in a renal allograft. Case report and review of the literature.

Thrombotic microangiopathy in a renal allograft may either reflect a recurrence of the patient's original disease, i.e., thrombotic thrombocytopenic purpura, hemolytic-uremic syndrome, or more commonly may be a manifestation of allograft rejection. This report describes a patient in whom irreversible renal failure developed during thrombotic thrombocytopenic purpura. Two years later while her condition was in clinical remission, she received a 2 DR-matched cadaveric allograft. Nineteen days following transplantation, thrombotic microangiopathy developed in the graft with eventual loss of allograft function despite vigorous plasmapheresis therapy. Multiple factors in addition to possible recurrent disease that may have contributed to this event were identified. The literature on thrombotic microangiopathy and renal transplantation is reviewed.

Adult↗

An idiopathic factor VIII anticoagulant: resolution following plasmapheresis and cytotoxic therapy.

A case is described in which plasmapheresis and immunosuppressive therapy were employed to treat a patient with a spontaneously occurring idiopathic polyclonal immunoglobulin G factor VIII anticoagulant. The favorable response observed supports the usefulness of the described treatment methods for the acute and chronic management of acquired circulating factor VIII inhibitors.

Blood Coagulation↗

Immune hemolytic anemia associated with sulindac.

A number of adverse reactions have been associated with sulindac, a nonsteroidal anti-inflammatory drug, but acute hemolytic anemia associated with sulindac has not yet been reported, to our knowledge. We encountered two cases of severe immune hemolytic anemia, one fatal, which appear to have been related to sulindac. In vitro studies provided evidence that antibodies to sulindac and its metabolites were present in the serum of both patients.

Aged↗

Incidence of cocaine metabolites in urine specimens from medical examiners' cases.

Isobutane CIMS is useful for determining the molecular weight of morphine and its derivatives, as well as for identifying labile acyl substituents on morphine's O-6 position. Furthermore, this technique will provide information relating to the presence or absence of pi-bonding on the C-7 carbon. The spectra of morphine derivatives can be further simplified by employing ethylenediamine as a reagent gas. This approach proves useful for eliciting or confirming molecular weight information from the CI spectrum. In our laboratory extended use of ethylenediamine has been accomplished without any deleterious effect on the mass spectrometer's source or its vacuum system. The utility of isobutane and ethylenediamine CI rests with its ability to supply the analyst with structure elucidation data that may be used to complement more detailed information extractable from either EI or CE spectra. This aspect of mass spectrometry is especially useful when one is dealing with an unknown member of a particular class of organic compounds.

Cadaver↗

Electron-capture gas-liquid chromatographic determination of ethosuximide and desmethylmethsuximide in plasma or serum.

We describe the determination of ethosuximide and desmethylmethsuximide, simultaneously or separately, in 50 to 100 microliter of plasma or serum. Derivatives of ethosuximide and desmethylmethsuximide formed by reaction with pentafluorobenzoyl chloride are extremely sensitive to the electron-capture detector of a gas-liquid chromatograph. The sample, with added internal standard and ammonium sulfate as a pH-adjusting and salting-out agent, is extracted with ethyl acetate/benzene (20/80 by vol). The extract is evaporated and the derivatives are formed. Analytical recoveries of ethosuximide and desmethylmethsuximide exceed 99%, and the relative standard deviation (CV) between analyses is usually less than 4.0%. alpha-Methyl-alpha-propylsuccinimide is used as the internal standard for ethosuximide, 2-phenylsuccinimide as the internal standard for desmethylmethsuximide.

Chromatography, Gas↗

Sulfisoxazole-induced thrombocytopenic purpura. Immunologic mechanism as cause.

During treatment of brucellosis with sulfisoxazole, tetracycline, and streptomycin sulfate, severe thrombocytopenic purpura developed in a young farmer. Verification for an immune mechanism was provided by clinical challenge with a small dose of sulfisoxazole that caused recurrence of thrombocytopenia and by serologic laboratory test results that detected a serum factor causing platelet agglutination requiring the presence of sulfisoxazole. The original antigenic stimulation was considered to come from drinking cows' milk contaminated with sulfonamide drugs. Cross-reactivity with some other sulfonamide drugs was demonstrated.

Adult↗

A pesticide (dieldrin)-induced immunohemolytic anemia.

The unusual presentation of a factory worker with severe hemolytic anemia which remitted following splenectomy prompted a search for an environmental cause for red cell injury. The investigation showed the presence of an immunoglobulin in the patient's serum and on the red cells and small amounts of complement on red cells. The patient's serum caused agglutination of a normal person's red cells only when dieldrin-coated, a reaction blocked by first reacting the serum with dieldrin. The spleen of the patient had a greater than normal concentration of dieldrin, the source of dieldrin being dietary. It is concluded that dieldrin became immunogenic and provoked a chemical immunohemolytic anemia. The spleen played a major role in destruction of red cells injured by the immunopathic process and in accumulation of the antigenic substance dieldrin.

Anemia, Hemolytic↗

Analysis for carbamazepine in serum by electron-capture gas chromatography.

We describe an analytical method for determining carbamazepine in serum by electron-capture gas chromatography. A single-step extraction is followed by formation of the N-pentafluorobenzamide derivative with pentafluorobenzoyl chloride. For quantitation either of two internal standards may be used: 10-methoxy carbamazepine or 7-chloro-5,11-dihydrodibenz[b,e] [1,4]-oxazepine-5-carboxamide (Squibb Compound No. 10996). The procedure requires 0.5 ml of serum. Analytical recoveries are 96%. Coefficients of variation routinely are less than 2% for concentrations of carbamazepine that are within the therapeutic range.

Carbamazepine↗

Identification of selected antihypertensive drugs by thin-layer chromatography.

A thin-layer chromatographic procedure is described for the qualitative identification of several antihypertensive drugs including certain thiazide diuretics spironolactone, triamterene, methyldopa and their metabolites. Utilization of new solvent developing systems and spray detecting reagents provides a method useful for the identification of these compounds in biologic fluids at low therapeutic concentrations. Sensitivity limits for these antihypertensive drugs are given, and alternate techniques to provide confirmatory analyses are also presented.

Antihypertensive Agents↗

Cocaine and benzoylecgonine excretion in humans.

Maximal urinary excretion of unchanged cocaine occurred within 2 h of the intranasal absorption of 1.5 mg/kg body weight of cocaine hydrochloride, and diminished rapidly thereafter. Excretion of benzoylecgonine was maximal 4 to 8 h following administration of the drug and diminished slowly over an interval of several days. Peak cocaine and benzoylecgonine concentrations observed were 24 and 75 microgram/ml, respectively. Benzoylecgonine/cocaine ratios were too varied to allow estimation of cocaine concentrations from benzoylecgonine concentration data or vice versa. Benzoylecgonine concentrations generally exceeded the corresponding cocaine values by a wide margin, but excretion of free cocaine in the absence of benzoylecgonine was observed in one subject. Cocaine was generally detected for only approximately 8 h, and for a maximum of 12 h, whereas benzoylecgonine was generally detected by chromatographic or enzyme immunologic assays for 48 to 72 h. Benzoylecgonine was positively identified in urine by raidoimmunoassay for 96 to 144 h after dosing.

Adult↗