Human parvovirus B19-induced epidemic acute red cell aplasia in patients with hereditary hemolytic anemia

From March to August 1984, 26 patients with hereditary hemolytic anemia in northeastern Ohio developed acute, profound red cell aplasia. The patients included 14 males and 12 females 2 to 23 years old, with sickle cell anemia (20 cases), hemoglobin SC-disease (4 cases), sickle- beta-thalassemia (1 case), or hereditary spherocytosis (1 case). All had an acute onset of severe reticulocytopenia and anemia and prodromal symptoms of illness including fever, abdominal symptoms, headache, and arthralgias. Twenty-two received transfusions. Reticulocytosis occurred spontaneously within 2 to 14 days of presentation. In five acute-phase sera, 10(8) to 10(12) viral particles/mL were detected by electron microscopy. Human parvovirus B19 DNA was demonstrated in high concentration by hybridization in the same five acute-phase sera and in low concentration in sera of eight additional patients. The five highly viremic sera inhibited erythroid colony formation in vitro. B19- specific IgM was detected in sera of 24/26 patients, and B19-specific IgG in 21 of 22 patients tested. Our results indicate that human parvovirus B19 was the etiologic agent in this large epidemic of life- threatening acute red cell aplasia in patients with hereditary hemolytic anemia.     

In vivo red cell destruction by anti-Lu6

An example is presented of an IgG1, anti-Lu6, that reacted by indirect antiglobulin test and was capable of destroying antigen-positive red cells in vivo. Two methods for the measurement of red cell survival, 51Cr labeling and flow cytometry, gave the same result: 20 percent of the test dose of Lu:6 red cells was destroyed in the first hour after injection and 80 percent in the first 24 hours. The clinical relevance of the antibody was correctly predicted by an in vitro monocyte monolayer assay. The finding that this example of anti-Lu6 was clinically significant should not be taken to mean that all antibodies directed against high-incidence Lutheran and Lutheran system-related antigens will behave similarly. When such antibodies are encountered, in vivo and/or in vitro studies to assess their clinical significance are necessary before rare blood is used for transfusion.     

Risk status at discharge and cause of death for postneonatal infant deaths: a total population study

OBJECTIVES: To obtain population-based, clinical information regarding potentially modifiable factors contributing to death during the postneonatal period (28 to 364 days), we examined all postneonatal infant deaths in four areas of the United States to determine: (1) the cause of death from clinical and autopsy data rather than vital statistics, (2) whether death occurred during initial hospitalization or after discharge, and (3) the portion of postneonatal mortality attributable to infants who left the hospital with identified high-risk medical conditions. DESIGN AND SETTING: Retrospective medical record review of all postneonatal infant deaths with birth weights greater than 500 g (total N = 386) born to mothers residing in: (1) the city of Boston (1984 and 1985, N = 55), (2) the city of St Louis and contiguous areas (1985 and 1986, N = 123), (3) San Diego County (1985, N = 112), and (4) the state of Maine (1984 and 1985, N = 96). Deaths were identified using linked birth and death vital statistics, and medical record audits of infants' and mothers' charts were performed. Causes of death were obtained from medical record review in conjunction with autopsy if performed (72%, N = 278), medical record alone (17%, N = 67), or vital statistics if no other source was available (11%, N = 41). The medical conditions at the time of discharge for each infant were reviewed and, if judged to confer an increased risk of morbidity or mortality, were classified as high risk. RESULTS: The causes of death were sudden infant death syndrome (47%, N = 181), congenital conditions (20%, N = 77), prematurity-related conditions (11%, N = 43), infections (9%, N = 34), external causes (including injuries, drownings, ingestions, and burns) (7%, N = 25), and other (6%, N = 23). In 24% of congenital and 25% to 44% of prematurity-related deaths, infection was the acute or associated cause of death. Infants born to black mothers were more likely than those born to white mothers to die during the postneonatal period of all major causes of death (7.3 per 1000 vs 3.0 per 1000). Overall, 18% (N = 68) of deaths occurred to infants who never left the hospital; 79% (N = 305) of the infants were discharged before death; and discharge status was unknown in 3% (N = 13). Eighty-one percent of all infants with prematurity-related postneonatal deaths were never discharged, and of the total infants who were initially discharged, only 1% (N = 4) subsequently died of prematurity-related causes. Of all postneonatal deaths, only 16% (N = 62) left the hospital with identified high-risk medical conditions. CONCLUSIONS: These findings suggest that the etiology of postneonatal mortality is heterogeneous, with significant complexity in attributing specific causes of death and making designations of "preventability." The vast majority of infants who died of prematurity-related postneonatal causes never left the hospital, and only a small percentage of all infants that left the hospital before death were identified as being at high medical risk. Therefore, strategies for further decreasing postneonatal mortality must link high-risk follow-up programs to more comprehensive strategies that address risk throughout pregnancy and early childhood.     

Modified silicone sling assisted temporalis muscle transfer in the management of lagophthalmos

Aim:

To evaluate the efficacy of modified temporalis muscle transfer (TMT) by silicone sling for the management of paralytic lagophthalmos.

Settings and Design:

Prospective interventional study.

Materials and Methods:

Ten patients of lagophthalmos due to facial palsy underwent modified TMT using silicone sling. The patients were followed-up for a period of 3 months. Palpebral aperture in primary gaze and during eye closure were assessed both pre- and postoperatively along with problems associated with lagophthalmos like exposure keratopathy and lacrimation.

Statistical Analysis:

Paired t-test was applied to measure the statistical outcome.

Results:

Eight patients achieved full correction of lagophthalmos with no lid gap on closing the eye. The mean (standard deviation (SD)) lid gap on eye closure was 7.7 (0.86) mm preoperatively, 0.5 (0.47) mm at 1^st postoperative day, and 0.7 (0.75) mm at 3^rd month. There was a reduction in mean lid gap on eye closure of 7 mm at 3 months (P < 0.0001) which is highly significant. The mean (SD) vertical interpalpebral distance during primary gaze was 12.05 (1.12) mm preoperatively, 10 (0.94) mm at 1^st postoperative day, and 10.35 (1.08) mm at 3^rd month. There was a reduction in mean vertical inter palpebral distance of 1.7 mm at 3 months (P = 0.001) which is significant. Exposure keratitis decreased in five out of six patients at 3 months.

Conclusion:

Modified TMT by silicone sling is a useful procedure with lesser morbidity and good outcomes for the treatment of paralytic lagophthalmos due to long standing facial palsy.     

Ethnoveterinary Application of Morinda Citrifolia Fruit Puree on a Commercial Heifer Rearing Facility with Endemic Salmonellosis

We have previously reported that Morinda citrifolia (noni) puree modulates neonatal calves developmental maturation of the innate and adaptive immune system. In this study, the effect of noni puree on respiratory and gastrointestinal (GI), health in preweaned dairy calves on a farm with endemic salmonellosis was examined. Two clinical trials were conducted whereby each trial evaluated one processing technique of noni puree. Trials 1 and 2 tested noni versions A and B, respectively. Puree analysis and trial methods were identical to each other, with the calf as the experimental unit. Calves were designated to 1 of 3 treatment groups in each trial and received either: 0, 15 or 30 mL every 12 hr of noni supplement for the first 3 weeks of life. Health scores, weaning age, weight gain from admission to weaning, and weaned by 6 weeks, were used as clinical endpoints for statistical analysis. In trial 1, calves supplemented with 15 mL noni puree of version A every 12 hr had a higher probability of being weaned by 6 weeks of age than control calves (P = 0.04). In trial 2, calves receiving 30 mL of version B every 12 hr had a 54.5% reduction in total medical treatments by 42 days of age when compared to controls (P = 0.02). There was a trend in reduced respiratory (61%), and GI (52%) medical treatments per calf when compared to controls (P = 0.06 and 0.08, respectively). There were no differences in weight gain or mortality for any treatment group in either trial.     

The effect of physical activity on pelvic organ prolapse

Objective  The objective of this study was to determine whether pelvic organ prolapse increases after physical activity.
Design  Prospective observational study.
Setting  St Mary's Hospital, Manchester, UK.
Sample  Women undergoing surgery for pelvic organ prolapse.
Methods  Fifty-four women were recruited to the study. Symptoms and POPQ findings were assessed after a period of prescribed activity and overnight bedrest.
Main outcome measures  Primary outcome was an increase in Pelvic Organ Prolapse Quantification (POPQ) measurements with activity. Secondary outcomes were association of symptoms or quality-of-life scores (Pelvic Floor Distress Inventory [PFDI] and Pelvic Floor Impact Questionnaire [PFIQ]) with an increase in POPQ measurements.
Results  There was a significant increase in POPQ stage and five vaginal parameters (Aa, Ba, C, Ap and Bp) with physical activity ( P < 0.001). Reported symptoms, higher PFDI and PFIQ scores and higher individual symptom bother scores were not more common in the women with greater pelvic organ descent (measured by the POPQ system) following physical activity.
Conclusions  Greater pelvic organ prolapse was found on POPQ examination following physical activity, but this was not associated with worsening of symptoms and greater impairment of quality of life.     

Service users' experiences of obtaining and giving information about disorders of sex development

Objective  To quantify participants' experiences of obtaining and giving information about disorders of sex development (DSD).
Design  Cross-sectional survey study that asked people about their current and past experiences relating to DSD disclosure.
Setting  A large tertiary referral centre for DSD management in the UK.
Population  One hundred of 126 people with a confirmed diagnosis of DSD who were invited to participate in the study formed the usable sample.
Methods  All people who attended clinic for follow-up during the study period and members of a patient support group whose annual meeting fell within the study period were asked to complete the Middlesex Communication Survey.
Main outcome measures  The Middlesex Communication Survey.
Results  Younger participants were more likely to report having been appropriately informed about their diagnosis than older people. Nearly half of the former had been fully informed about their diagnosis by age 15 years, compared with 0% of the older age group. In terms of information sharing, mothers were most likely to be the person with whom the participant had shared (almost/all) DSD information (74%), followed by current partners (71%). Information relating to genital surgery, presence of testes and clitoral anomalies were the least likely aspects to have been unambiguously shared with even the most informed person.
Conclusions  Our results suggest that difficulties in obtaining DSD information from care providers were common, and that communication has improved for younger participants. The study also confirmed that many people with DSD continue to struggle with confiding, even in those closest to them, about aspects of their diagnosis. Care protocol needs to centralise psychological adaptation, which should also be a primary focus for future research.     

Impact of bowel obstruction at the time of initial presentation in women with ovarian cancer

Objective  To determine whether the presence of bowel obstruction at the time of initial presentation has any prognostic significance in these women.
Design  Retrospective cohort study.
Setting  Dedicated gynaecological oncology service of a large tertiary institution.
Population  Women who had a bowel obstruction as part of their initial presentation of ovarian cancer were identified between 1995 and 2007. Each woman was matched with four control women (with disease but no obstruction).
Methods  Women with disease were compared with controls to determine the impact, if any, of bowel obstruction at presentation. Several prognostic variables including bowel obstruction were also evaluated in a Cox proportional hazard model.
Main outcome measures  Progression-free survival (PFS) and overall survival (OS).
Results  Forty-eight women with disease and 192 controls were identified during the study period. The median follow-up period was 19 months among women with disease versus 20 months in controls. No differences were seen in demographics and clinical characteristics of the women. Optimal cytoreduction rate was similar between the two groups (75% versus 78%, P  = 0.7). Patients with bowel obstruction had a shorter PFS and OS compared with controls [19 months versus 21 months ( P  = 0.01) and 22 versus 35 months ( P  = 0.008)], respectively. Bowel obstruction at presentation was an independent prognostic variable with a hazard ratio of 1.5 ( P  = 0.009). Other prognostic variables were age, stage and extent of surgical cytoreduction.
Conclusions  Bowel obstruction at the time of initial presentation is an adverse prognostic factor in women with ovarian cancer.     

Lymphokine abnormalities in aplastic anemia: implications for the mechanism of action of antithymocyte globulin

Anti-thymocyte globulin (ATG) provides effective therapy for many patients with aplastic anemia, and its mechanism of action has been presumed to be secondary to lymphocytotoxicity. However, our studies of lymphocyte function in aplastic anemia show marked abnormalities of lymphokine production, which ATG may modulate. In 12 of 17 patients with aplastic anemia, interleukin 2 (IL2) production was markedly elevated in vitro (P less than .01 by paired statistical analysis). Expression of the IL2 receptor, or Tac antigen, on peripheral lymphocytes assessed by flow microfluorometry was also increased above the normal range in 11 of 15 cases. Studies of ATG suggested that it might act to stimulate lymphocyte function. In vitro, ATG is a mitogen, as measured by incorporation of 3H-thymidine into blood mononuclear cells; the response of cells to ATG from patients with aplastic anemia was exaggerated in comparison with normals. Cell proliferation was accompanied by production of IL2 to levels that were, in some cases, similar to those obtained with lectin stimulation. Finally, supernatants from lymphocytes cultured in the presence of ATG were able to replace adherent cells in providing growth factors for the support of nonadherent cells in methylcellulose hematopoietic colony assays. These results provide a mechanism for an "immunostimulatory" action of ATG in effecting hematopoietic response in some patients with aplastic anemia.