Nasal health in Down syndrome: a cross-sectional study.

OBJECTIVE: To investigate peripheral nasal pathology as a contributor to olfactory impairment in DS. STUDY DESIGN: Twenty DS and 16 non-DS subjects were recruited. Nasal history and symptoms were assessed by self-report or informant. Olfactory threshold, odor identification, and nasal endoscopy were assessed on each subject. RESULTS: DS subjects were impaired on olfactory threshold (P<0.0001) and odor identification (P<0.001). Although DS subjects tended toward upper-respiratory infections, sleep-disordered breathing, and nasal itching, differences were not significant (P=0.07, 0.06, and 0.058, respectively). There were no significant differences on self-reported nasal history or symptoms. Endoscopy showed equivalent health in DS and control subjects. CONCLUSION: This DS population shows olfactory impairment. However, nasal health is comparable in DS subjects and controls. Nasal dysfunction is unlikely to contribute to olfactory impairment in DS. SIGNIFICANCE: Olfactory deficits in DS appear to be secondary to central, rather than rhinologic, pathology. EBM rating: B-2b.     

Masculinizing sclerosing stromal tumor of the ovary during pregnancy

Sclerosing stromal cell tumors of the ovary are an uncommon neoplasm that usually does not produce hormonal imbalances. Most patients showing a hormonal effect from this lesion have had menstrual cycle disturbances. Infertility and endometrial hyperplasia have also been described. One other reported case had masculinizing effects. Other authors have documented elevated levels of both estrogenic and androgenic hormones that corrected after surgery. A case of a pregnant 27-year-old Caucasian with hirsutism on her chin and neck and a male suprapubic hair pattern is presented. Elevated androstenedione, dehydroepiandrosterone, and free testosterone levels were present. A 3-cm left ovarian mass was excised and identified as a sclerosing stromal tumor. The histologic features included a pseudolobular pattern with focal areas of sclerosis and a two-cell population of spindled and polygonal cells. Immunohistochemical studies showed a positive vimentin reaction, weakly positive desmin and muscle-specific actin stains, and a negative cytokeratin stain. Following surgery the hormone levels returned to normal and the hirsutism resolved. A normal female infant without evidence of masculinization was delivered from the patient at term.     

Cutaneous postural vasoconstriction is modified by exogenous but not endogenous female hormones in young women

Previously reported attenuation of skin postural vasoconstriction in women during the luteal menstrual cycle phase may be due to a progesterone-mediated decrease in myogenic or veno-arteriolar (VAR) mechanisms. Skin perfusion was measured in the shin and foot dorsum by Laser Doppler Fluxometry during leg dependency that increased vascular transmural pressure below (myogenic constriction only) and above (myogenic and VAR) the 25 mmHg threshold for activation of the VAR, and during venous distension to activate the VAR alone (cuff inflation to 50 mmHg). In six young women with normal menstrual cycles, vasoconstrictor responses to all interventions did not differ between days 7–13 (follicular) and 18–23 (luteal) of the normal menstrual cycle when progesterone levels were low and at their peak respectively. In eight women taking combined oral contraceptives (OC) and tested during pill consumption days, reductions in foot skin perfusion were smaller (P = 0.05) than in the luteal phase of the normal cycle for leg dependency below (−36.9 ± 5.2% OC vs. −52.5 ± 7.8% luteal, mean ± S.E.M.) and above (−43.7 ± 3.4% OC vs. −55.1 ± 4.8% luteal) the VAR threshold, and for venous distension (−53.1 ± 2.6% OC vs. 66.4 ± 5.5% luteal). In women with normal menstrual cycles, impaired postural vasoconstriction may be confined to those who experience pre-menstrual symptoms rather than a direct effect of endogenous hormones. Reduced vasoconstriction in the dependent foot during OC use is consistent with the known vasodilator action of exogenous hormones and its long-term effects     

Evaluation of NaOH treatment of human dura mater implants to obviate Creutzfeldt-Jakob disease transmission.

One of the few disinfectants known to inactivate the causative agent of Creutzfeldt-Jakob Disease is NaOH. In this study, NaOH was evaluated as a possible routine treatment for human dura mater alloimplants. Use of high concentration NaOH (1 M) resulted in protein loss and macroscopic changes to the tissue. Lower concentrations (0.1 M), although exhibiting little direct detrimental effect, greatly increased the susceptibility of the tissue to collagenase digestion. The use of NaOH treated commercial or institutionally prepared human dura mater should be approached with caution.     

Medical problem solving and uncertainty in the emergency department

STUDY OBJECTIVE: To compare the diagnostic processes of experienced emergency physicians with those of novices. DESIGN: Prospective, convenience sample of patients. SETTING: Emergency department of a county university medical center in a large southwestern urban community. PARTICIPANTS: Experienced emergency physicians (attending and senior residents) and novice clinicians (junior residents and senior medical students). INTERVENTIONS: Participants developed initial diagnostic impressions after reviewing the chief complaint, nurse triage notes, and vital signs. Tests were then selected, and a final diagnostic impression was identified after results were known. Clinicians also marked a visual analog scale corresponding to their estimate that each diagnostic possibility was correct. RESULTS: Experienced physicians increased their certainty more than novices (P = .014). They deviated from a standard history-physical-laboratory sequence more often than novices (P = .008). CONCLUSION: Expertise in medical decision making is characterized by a moderate initial level of certainty concerning a diagnosis that significantly increases as the experienced clinician follows a flexible strategy of testing to arrive at a final diagnosis.     

Retroviruses and schizophrenia in Jamaica

Reports of an 18-fold higher incidence of schizophrenia among second-generation Afro-Caribbeans, and especially Jamaican migrants in the United Kingdom were soon called “an epidemic of schizophrenia,” with the inference that a novel virus, likely to be perinatally transmitted, was a possible etiological agent. This intriguing observation led us to explore a possible link with human T-cell lymphotropic virus type one (HTLV-I), because it is a virus that is endemic in the Caribbean Islands, is perinatally transmitted, known to be neuropathogenic, and the cause of a chronic myelopathy (tropical spastic paraparesis/ HTLV-I associated myelopathy). We therefore examined inpatients at the Bellevue Mental Hospital, Kingston, Jamaica and did standard serological tests for retroviruses HTLV-I and HTLV-II and HIV-I and HIV-II on 201 inpatients who fulfilled ICD-9 and DSM III-R criteria for schizophrenia. Our results produced important negative data, since the seropositivity rates for HTLV-I, the most likely pathogen, were no greater than the seropositivity range for HTLV-I carriers in this island population, indicating that HTLV-I and the other retroviruses tested do not play a primary etiological role in Jamaican schizophrenics.