Menstrual suppression for adolescents with developmental disabilities.

The approach to menstrual suppression for adolescents with developmental disabilities has evolved considerably over the years due to changing philosophies and evolving treatment options. We review the medical management options available for menstrual suppression with a focus on the needs and treatment of adolescents with developmental disabilities.

A survey of canadian practitioners regarding diagnosis and evaluation of the hypertensive disorders of pregnancy.

BACKGROUND: How Canadian practitioners are diagnosing and managing the hypertensive disorders of pregnancy (HDP), particularly in relation to the 1997 recommendations published by the Canadian Hypertension Society (CHS), is not known. METHODS: A survey, with French and English versions (and covering diagnosis, evaluation, and management of pregnancy hypertension), was mailed to all members of the Society of Obstetricians and Gynaecologists of Canada (SOGC) (N = 1757, including obstetricians, family doctors practicing obstetrics, and midwives). Additionally, internists [i.e., all nephrologists (N = 191) and a random sample of 25% of general internists (N = 450)] registered with the Royal College of Physicians and Surgeons of Canada were sampled. The survey was distributed in two mailings and one reminder card. Data were entered into Microsoft Access, and Graph Pad Prism used to summarize responses [N (%)]. Differences in practice between specialties were examined, with a Bonferonni correction used to calculate a significant p value based on the number of comparisons and alpha of 0.05. RESULTS: Respondents numbered 1187 (49.5%), with 466 not informative for the purpose of the study (due to retirement, or practices that do not include pregnant women with hypertension). The final analysis included 721 completed surveys. Most (609, 84.5% of) respondents take blood pressure (BP) with women in the sitting position, and use a mercury sphygmomanometer (79%) and the 5th Korotkoff (61%) sound to designate diastolic BP (dBP). To monitor pregnancies complicated by preeclampsia, most clinicians use the proposed laboratory tests of maternal well-being (usually at least once/week), fetal well-being [nonstress test (NST, at least once/week), and ultrasonographic studies (once weekly to every two weeks)]. There is general agreement that women with preeclampsia should be delivered for uncontrolled hypertension, end-organ dysfunction, or fetal compromise (nonreassuring NST, severe oligohydramnios, biophysical profile < 4, estimated fetal weight < 5th centile, and reversed end-diastolic flow by umbilical artery Doppler velocimetry). Less consensus was seen for delivery for preeclampsia at > 34 weeks, mild asymptomatic HELLP syndrome, hyperreflexia, and absent end-diastolic flow by umbilical artery Doppler velocimetry. INTERPRETATION: This survey has clarified the current state of practice with respect to the diagnosis and evaluation of women with all types of HDP. In particular, we have identified areas of potential variability in BP measurement, and provided data on the feasibility of enrolling women with sub types of preeclampsia into intervention studies aimed at prolonging pregnancy.

A survey of Canadian practitioners regarding the management of the hypertensive disorders of pregnancy.

BACKGROUND: How Canadian practitioners are managing the hypertensive disorders of pregnancy (HDP) is not known, particularly in relation to the 1997 guidelines published by the Canadian Hypertension Society (CHS). METHODS: A survey, with French and English versions (and covering diagnosis, evaluation, and management of pregnancy hypertension), was mailed to all members of the Society of Obstetricians and Gynaecologists of Canada (SOGC) (N = 1757, including obstetricians, family doctors practicing obstetrics, and midwives). Additionally, internists [i.e., all nephrologists (N = 191) and a random sample of 25% of general internists (N = 450)] registered with the Royal College of Physicians and Surgeons of Canada were sampled. The survey was distributed in two mailings and one reminder card. Data were entered into Microsoft Access, and Graph Pad Prism used to summarize responses [N (%)]. Differences in practice between specialties were examined, with a Bonferroni correction used to calculate a significant p value based on the number of comparisons and alpha of 0.05. RESULTS: Respondents numbered 1187 (49.5%), with 466 not informative for the purpose of the study (due to retirement, or practices that do not include pregnant women with hypertension). The final analysis included 721 completed surveys. For all types of HDP, most internists, family doctors, and midwives initiate nonpharmacological therapy (most common advice to quit work) at dBP 80-89 mmHg (i.e., primary prevention). Only for preeclampsia do obstetricians most frequently use this threshold; otherwise, dBP 90-99 mmHg is usually chosen. For nonsevere hypertension, antihypertensive drug therapy (most commonly methyldopa or labetalol) is started by most practitioners at dBP 90-99 mmHg, although obstetricians are more likely to choose a higher threshold (p < 0.0001). There is little agreement about dBP treatment goal; most internists and family doctors normalize dBP, whereas obstetricians appear to be divided on dBP goals of 80-89 (46-51%) vs. 90-99 mmHg (41-44%) for all HDP (p = 0.66). Severe hypertension is commonly treated with parenteral hydralazine, labetalol, or magnesium sulphate. Short-acting or sustained release nifedipine is used rarely/never by most practitioners. Approximately one-third of obstetricians and family doctors use diazepam to treat eclampsia. The vast majority use MgSO4 prophylactically in women with preeclampsia. INTERPRETATION: This survey has clarified current stated management of women with HDP, and identified the need for both research into the dBP treatment goal that optimizes pregnancy outcomes among women with HDP, and translation of definitive studies into clinical practice.

Fall in mean arterial pressure and fetal growth restriction in pregnancy hypertension: a meta-analysis.

BACKGROUND: We investigated the relation between fetoplacental growth and the use of oral antihypertensive medication to treat mild-to-moderate pregnancy hypertension. METHODS: The study design was a metaregression analysis of published data from randomised controlled trials. Data from a paper that was regarded as an extreme statistical outliner were excluded from primary analyses. The change in (group) mean arterial pressure (MAP) from enrolment to delivery was compared with indicators of fetoplacental growth. FINDINGS: Greater mean difference in MAP with antihypertensive therapy was associated with the birth of a higher proportion of small-for-gestational-age (SGA) infants (slope: 0.09 [SD 0.03], r2=0.48, p=0.006, 14 trials) and lower mean birthweight significant after exclusion of data from another paper regarded as an extreme statistical outliner (slope: -14.49 [6.98] r=0.16, p=0.049, 27). No relation with mean placental weight was seen (slope -2.01 [1.62], r2=0.15, p=0.25, 11 trials). INTERPRETATION: Treatment-induced falls in maternal blood pressure may adversely affect fetal growth. Given the small maternal benefits that are likely to be derived from therapy, new data are urgently needed to elucidate the relative maternal and fetal benefits and risks of oral antihypertensive drug treatment of mild-to-moderate pregnancy hypertension.

Dramatic improvement of left ventricular function after cytotoxic therapy in lupus patients with acute cardiomyopathy: report of 6 cases.

Although lupus cardiomyopathy is thought to be clinically uncommon, we encountered 6 patients with systemic lupus erythematosus (SLE) over a 10 year period who had severe left ventricular dysfunction and showed remarkable improvement in their cardiac function after cytotoxic therapy. All patients met the American College of Rheumatology criteria for classification of SLE and presented with signs of severe biventricular failure relatively early in their disease. Concurrent manifestations of SLE at the time of cardiomyopathy included rash, arthritis, myalgias, pleuritis, pericarditis, and nephritis. Four of the 6 patients were taking prednisone 20 mg/day at the time heart failure developed. In all cases the CPK were normal. Evaluation of cardiac function by echocardiogram and/or radionuclide gated blood pool scintigraphy revealed a severe depression of ventricular function with initial left ventricular ejection fraction (LVEF) ranging from 11 to 34% (mean 19%). Within 6 months of initiation of cytotoxic treatment all patients showed a dramatic response: the post-treatment LVEF ranged from 25 to 55%. This series of patients suggests that cardiomyopathy may be a more common complication of SLE than previously reported. Cardiomyopathy occurs relatively early in the course of SLE, may lead to severe cardiac dysfunction despite corticosteroid therapy, and appears to be responsive to cytotoxic therapy.

The antiinflammatory and antiviral effects of hydroxychloroquine in two patients with acquired immunodeficiency syndrome and active inflammatory arthritis.

OBJECTIVE: To report the antiinflammatory and antiviral effects of hydroxychloroquine (HCQ) treatment in 2 patients with AIDS and inflammatory arthritis. METHODS: Two patients with AIDS and inflammatory arthritis were treated with HCQ, which was given in a loading dose of 600 mg/day. The maintenance dosage was calculated to remain below 6.5 mg/kg/day. Both patients had initial T cell subset studies; 1 patient, had serum and plasma collected before and after 1 year of HCQ treatment. Assays were performed for T cell subsets, recoverable human immunodeficiency virus type 1 (HIV-1) RNA, mitogen- and antigen-specific proliferation, and interleukin-6 (IL-6) levels. New studies on the use of HCQ as an anti-HIV-1 agent are reviewed. RESULTS: Both patients had a dramatic decrease in their arthritis activity. Neither patient required immunosuppressive therapy or developed an opportunistic infection. In the patient who was studied after 1 year of therapy, there was a 1-log decrease in recoverable HIV-1 RNA, improved mitogen- and antigen-specific immune responses, and a large decrease in the IL-6 level while taking HCQ. Recent in vitro and in vivo assays in patients with HIV infection have shown similar antiviral and antiinflammatory effects from HCQ. CONCLUSION: HCQ may exert simultaneous anti-inflammatory and antiviral effects in patients with HIV infection and inflammatory arthritis. If larger studies confirm this observation, it may be the drug of choice in this population of patients.

A patient with antineutrophil cytoplasmic antibody-negative pulmonary capillaritis and circulating primed neutrophils.

We describe an antineutrophil cytoplasmic antibody-negative patient with capillaritis, acute respiratory failure, and primed neutrophils circulating systemically. Thoracoscopic lung biopsy showed capillary destruction, with neutrophils infiltrating the lung interstitium and air spaces but no alveolar damage or hemorrhage. We suggest that this atypical, limited form of pulmonary injury may occur when circulating neutrophils are primed, but, in the absence of an activating factor, not fully activated.

A reexamination of the relationship between active rheumatoid arthritis and the acquired immunodeficiency syndrome.

Three patients with rheumatoid arthritis (RA) that remitted with the development of the human immunodeficiency virus (HIV) infection have been described in the literature, and this has contributed to the belief that RA and HIV infection or the acquired immunodeficiency syndrome (AIDS) cannot coexist. However, a computerized MEDLINE literature search revealed reports of 4 patients who did have active RA and AIDS or HIV infection, as well as other case reports of symmetric polyarthritis compatible with RA in patients with HIV infection. Each of the patients whose RA remitted had received standard disease-modifying antirheumatic drug therapy, and 1 of the 3 had a normal T helper:T suppressor ratio at the time of remission. Of the 4 previously reported patients with active RA and AIDS or HIV infection, all had decreased numbers of T helper lymphocytes. The present report describes a fifth patient with both RA and AIDS and reviews the data concerning the coexistence of these 2 diseases. It appears that active RA may indeed coexist with AIDS. It remains to be seen under what settings HIV may have a disease-modifying effect in RA. These issues have important implications regarding the pathogenesis and therapy of RA, especially in terms of the role of CD4+ lymphocytes and anti-CD4 monoclonal antibody therapy.

Correlation of liver and skin histopathology with serology in a patient with cutaneous vasculitis and hepatitis C infection.

A strong body of evidence supports a viral etiology for many forms of vasculitis. Recent literature supports the association of the hepatitis C virus (HCV) with essential mixed cryoglobulinemia. We describe a case of cutaneous vasculitis associated with simultaneous active hepatic inflammation demonstrated by skin and liver biopsies. Immunofluorescence, hepatitis C serology, and study of the cryoprecipitate all confirmed HCV infection and deposition of immune complexes. Treatment with interferon alpha-2b was extremely effective as compared with prednisone and colchicine. This case provides further evidence that active hepatic infection with HCV may be a cause of cryoglobulinemia and cutaneous vasculitis. It also suggests that further studies of antiviral therapy for vasculitis should be explored.

An association between refractory HELLP syndrome and antiphospholipid antibodies during pregnancy; a report of 2 cases.

The HELLP syndrome (hemolysis, elevated liver enzymes and low platelets) is a thrombotic microangiopathic vasculopathy that presents in pregnancy. HELLP syndrome usually resolves with delivery of the fetus without sequelae and is often managed successfully with conservative care. To our knowledge, there has been no prior report of an association between HELLP syndrome and elevated titers of antiphospholipid antibodies (aPL). We describe 2 patients who each presented with HELLP syndrome that was refractory despite delivery of the fetus, corticosteroids, and anticoagulation. Both patients had elevated levels of anticardiolipin antibodies. Examination of skin and placental pathology revealed diffuse deposition of fibrin with small vessel thrombi, without evidence of vasculitis. Coincident with plasmapheresis, HELLP resolved in both patients. We conclude that there may be an association between the presence of aPL and a protracted HELLP syndrome in pregnancy; this may be a new manifestation of the antiphospholipid syndrome. Plasmapheresis appears to benefit these patients. Further investigation into this association is warranted.

Neonatal follow-up of very low birthweight/extremely low birthweight infants to school age: a critical overview.

Neonatal follow-up studies of school age children, published in the last decade, were critically reviewed. Nine studies examined extremely low birthweight infants (less than or equal to 1000 g) and 16 involved very low birthweight infants (less than or equal to 1500 g). The majority of children had age appropriate I.Q. scores, however, there was a greater variability of test scores. There was an increased need for special education or remedial therapy. Visual-motor integration deficits were frequently reported. Behavioural difficulties were described. Fine and gross motor incoordination was identified. There was no conclusive correlation between perinatal course and school outcome. Gender did appear to influence outcome, in the small percent of studies which examined this variable, with females generally faring better. Low socioeconomic status was the most frequently reported predictor of poor outcome. Identified methodological limitations included heterogeneous samples, lack of control groups, high attrition, variable diagnostic criteria and lack of consensus regarding correction for prematurity.