One of the most serious presentations of preeclampsia is the HELLP (hemolysis, elevated liver enzymes, low platelets) syndrome. Treatment has generally been limited to supportive measures and management of complications, but recent clinical trials-which were not double-blind or placebo-controlled-suggest that dexamethasone may stabilize the disorder and even improve it in the antepartum period, maximizing postpartum recovery. The present randomized, double-blind, placebo-controlled trial enrolled 132 women, 60 of them pregnant and 72 puerperal, who met criteria for complete HELLP syndrome. They were randomly assigned to a treatment or placebo group and in the former case received 10 mg dexamethasone intravenously at 12-hour intervals until delivery and three further doses after delivery. The puerperal women received three doses of dexamethasone. All women received, in addition, magnesium sulfate intravenously and, when needed, an antihypertensive agent, initially nifedipine and subsequently clonidine or amlodipine if necessary to lower the diastolic blood pressure.Hospital time was less for women given dexamethasone, although not to a significant degree. The treatment and placebo groups were similar in the time needed to reach a platelet count exceeding 100,000/mm 3 . There also was no difference in the proportion of women reaching a lactate dehydrogenase level less than 600 U/L before discharge. Treatment did not hasten recovery in patients with an aspartate aminotransferase level less than 70 U/L before discharge. Urine output was comparable in the treatment and control groups. Three of four maternal deaths were in steroid-treated women. No substantial differences were evident when pregnant and puerperal women were analyzed separately.These findings do not support using dexamethasone to treat HELLP syndrome in preeclamptic women who are in late pregnancy or the early postpartum period. EDITORIAL COMMENT(The abstracted study is both well done and important. It is the sixth randomized study to address this issue and by far the best. It is four times as large as the previous largest trial, doubleblind and placebo-controlled (none of the prior ones were), and only women who met stringent criteria for the hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome were enrolled. Additional strengths of this study include the fact that 132 of 144 eligible women participated, and the protocol was carried out with high fidelity. Participating women had serious disease. The average lactate dehydrogenase (LDH) concentration at randomization was over 2000 U/L, the average total bilirubin approximately 3.5 mg/mL, the average aspartate OBSTETRICS ABSTRACTIn Europe, as many as 2.5% of parturients have hepatitis C virus (HCV) infection, and estimates of mother-to-child transmission range from 3% up to 10%. The maternal load of HCV is probably the major determinant of transmission, but risk factors have not been precisely defined. This multicenter European prospective study, based on data provided by the European Ped...
CHARGE syndrome was initially not thought to involve the limb. Several subsequent reports have shown that limb anomalies are not uncommon. To date, there have been no quantitative studies of limb anomalies in CHARGE syndrome. This study was designed to answer several questions: Do CHARGE patients with limb anomalies represent a subgroup within CHARGE syndrome? Are there correlations between certain CHARGE syndrome anomalies and limb anomalies? Are there differences between the two genders and associated limb anomalies? Are certain types of limb anomalies seen with increased frequency in CHARGE syndrome? All described patients were categorized utilizing the AI/GEN Model 2 Criteria proposed by Mitchell [1985a: J Med Syst 9:425–435]. Patients with chromosomal anomalies or familial CHARGE were excluded, as were patients with inadequate clinical descriptions, and patients in large series where individual characteristics could not be ascertained. Multivariate analysis was performed. One hundred seventy two patients with definite or probable CHARGE syndrome were analyzed. Sixty‐four (37.2%) of these patients have at least one limb anomaly. Significant positive associations were seen between limb anomalies and ocular coloboma, urinary tract malformations, and genital anomalies. These associations were not significant when the definite or probable patients were analyzed separately, with the exception of genital anomalies in definite CHARGE. Gender differences were also identified. Females with tracheoesophageal fistula/esophageal atresia, or genital anomalies were more likely to have limb anomalies, while some female subgroups had positive associations between urinary tract malformations, or choanal atresia and limb anomalies. Negative associations were also seen with sensorineural hearing loss and facial paralysis. In contrast, males showed a positive association between coloboma and limb anomalies, while subgroup analysis identified positive associations with DiGeorge sequence or genital anomalies and limb anomalies. Limb anomalies are present in just over one‐third of CHARGE syndrome patients. Limb anomalies are seen more frequently in association with certain CHARGE anomalies, and these associations show gender differences. There is not a common limb anomaly seen in CHARGE syndrome. © 2003 Wiley‐Liss, Inc.
Background: Distress is an unpleasant psychosocial response that may impede the capacity of patients to cope successfully with a disease, its physical symptoms, and its treatment. Measurements of distress have been extensively evaluated in patients with solid tumor malignancies. The National Comprehensive Cancer Network (NCCN) has recommend standards of care for distress management. However the degree of distress in patients with hematologic diseases (both malignant and non-malignant) is under-investigated. Aim: This study was designed to assess and compare distress in patients with malignant or non-malignant hematological disorders. Methods: Of 617 adult hematology patients enrolled between July 2006 and December 2006, 572 reported distress scores and were prospectively followed, while 45 patients who did not rate their distress were excluded. The NCCN Distress Thermometer (scale of 0–10) accompanied by a modified problem checklist was administered to hematology patients 18 years of age and older seen at the Gundersen Lutheran Center for Cancer and Blood Disorders. Additional demographics, diagnoses, and disease-related information were gathered by chart review. Patients reporting a high score were contacted by a social worker to assess the etiology of distress. Results: At initial visit during the study period, 572 patients reported a distress score (mean = 3.4 ± 2.7). Patients with malignant (3.2 ± 2.7) or non-malignant (3.6 ± 2.6) disease reported similar distress (p = 0.125). Females (4.0 ± 2.8) were more distressed than males (2.9 ± 2.4; p < 0.001). Other factors significantly associated with distress were history of anxiety (p < 0.001), depression (p < 0.001), younger age (p = 0.001), and time from diagnosis (visit day 3.6 ± 2.4; 1–90 days 4.2 ± 2.9; 3mo-1 year 2.9 ± 2.6; 1+ years 3.4 ± 2.7; p = 0.028). Distress based on disease status was (relapse 5.0 ± 3.1, remission/stable 3.3 ± 2.6, treatment 3.7 ± 2.7) also significantly different (p = 0.009). Conclusion: We found no significant difference in levels of reported distress between patients with malignant and non-malignant hematologic disorders. Significantly higher distress levels were reported in women, younger patients, those with previous psychiatric disease, date of visit, and those at relapse. Patients with either malignant or non-malignant hematologic disorders should be considered for distress screening and management.
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