An audit of perioperative blood transfusions in a regional hospital to rationalise a maximum surgical blood ordering schedule.

Appropriate preoperative blood typing and cross-matching is an important quality improvement target to minimise costs and rationalise the use of blood bank resources. This can be facilitated using a maximum surgical blood ordering schedule (MSBOS) for specific operations. It is recommended that individual hospitals develop a site-specific MSBOS based on institutional data, but this is challenging in non-tertiary centres without electronic databases. Our aim was to audit our perioperative blood transfusions to develop a site-specific MSBOS. A retrospective audit of blood transfusions in surgical patients in our regional referral hospital was conducted using five years' coded administrative data. Procedures with higher transfusion rates warranting preoperative testing (type and screen with or without subsequent cross-matching) were identified. There were about 15,000 eligible surgical procedures performed in our institution over the audit period. The need for preoperative testing was identified for only a few procedures, namely laparotomy, bowel resection, major amputation, joint arthroplasty, hip/femur fracture and humerus surgery, and procedures for obstetric complications. We observed a reduction in transfusion rates over time for total joint arthroplasty. The use of coding data represents an efficient method by which centres without electronic anaesthesia information management systems can conduct large-scale audits to develop a site-specific MSBOS. This would represent a significant improvement for hospitals that currently base preoperative testing recommendations on expert opinion alone. As many procedures in regional centres have very low transfusion rates, hospitals with a similar case mix to ours could consider selectively auditing higher-risk operations where local data is most likely to alter testing recommendations.

The return of thalidomide: can birth defects be prevented?

Thalidomide, the drug that caused a worldwide epidemic of serious birth defects in the late 1950s and early 1960s, was recently approved by the US Food and Drug Administration (FDA) for use in treating the skin disease erythema nodosum leprosum, a complication of leprosy. The drug has also shown promise in the treatment of other serious diseases. If thalidomide is eventually approved for use in the US and other countries for treatment of diseases more prevalent than erythema nodosum leprosum, or if use of the drug for non-approved indications becomes widespread, hundreds of thousands of women with childbearing ability could be treated. If this should happen, can we prevent another epidemic of birth defects? In an effort to prevent fetal exposures to thalidomide, the FDA mandated a comprehensive programme to regulate prescription, dispensing and use of the drug. The programme is designed to require registration of all participating prescribers, pharmacies and patients. It also requires use of effective methods of contraception and periodic pregnancy testing of all patients with childbearing ability during treatment. Prescribers are directed to counsel both female and male patients on the risks, benefits and proper use of the drug, as well as on the proper use of contraceptives during treatment. The patient is required to sign an informed consent form before beginning treatment. Prescription and dispensing of thalidomide will be tightly controlled. Athalidomide registry will monitor prescription. dispensing and use of the drug, and will investigate all reported fetal exposures. This mandatory, but untested, programme promises to be effective at preventing fetal exposures to thalidomide, provided that patients, prescribers and pharmacists comply with all of its provisions. However, even if the programme proves to be successful in the US, there is concern that thalidomide may eventually be widely used in countries that may not require such stringent controls. In Brazil, where thalidomide is commercially available for treatment of leprosy patients, 33 cases of thalidomide embryopathy have already been reported in the literature. Even in countries that may tightly regulate the distribution and use of thalidomide, some patients may obtain the drug through black market sources. Should these events occur, many cases of thalidomide-induced birth defects could appear. Therefore, there is a need to develop nonteratogenic analogues of thalidomide that can provide effective treatment for erythema nodosum leprosum and other serious conditions without increasing the potential for another epidemic of thalidomide-related birth defects.

Increased frequency of cystic fibrosis among infants with jejunoileal atresia.

There appears to be an increased frequency of cystic fibrosis (CF) among infants with jejunoileal atresia (JIA). However, the figures vary widely, and no population-based data exist. The purpose of this study was to quantitate the magnitude of the association between JIA and CF in Atlanta using population-based data from 1968 to 1995. Case subjects included all infants with isolated JIA born during 1968-1995 to mothers residing in the five-county metropolitan Atlanta area at the time of birth. To ascertain cases, we reviewed records of the Metropolitan Atlanta Congenital Defects Program (MACDP), a population-based birth defects registry. Caucasian JIA cases were cross-referenced with patients in the CF registry at the Egleston Cystic Fibrosis Center at Emory University to more completely ascertain the diagnosis of CF among JIA cases. During 1968-1995, MACDP ascertained a total of 94 isolated JIA cases, for a birth prevalence of 1.8/10,000 live births. Among the cases, 38 were Caucasian, 52 were African-American, and 4 were of Asian or Hispanic ethnicity. Four of the 38 Caucasian JIA cases (11%) also had CF. The expected number of JIA cases with CF is 0.019 based on the estimated population incidence of 1/ 2,000 for CF. The observed to expected (O/E) ratio of Caucasian JIA cases with CF is greater than 210 (P<0.0001). Caucasian infants with JIA have more than 210 times the risk for CF compared with Caucasian infants in the general population. The results of this study have implications for the management of infants born with JIA and genetic counseling for families with affected infants.

Population study of congenital hypothyroidism and associated birth defects, Atlanta, 1979-1992.

Very little data are available from population-based studies on congenital hypothyroidism (CH) epidemiology and patterns of associated birth defects. By linking data from two population-based registries, we describe the epidemiology of CH and associated defects in Atlanta from 1979-1992. Cases included all infants with CH born from 1979-1992 to mothers residing in the metropolitan Atlanta area at the time of birth. We ascertained CH cases by reviewing newborn screening records and records of the Metropolitan Atlanta Congenital Defects Program (MACDP), a population-based registry of all serious birth defects diagnosed during a child's first year of life. We linked CH cases with MACDP records to ascertain the presence of serious birth defects among infants with CH. Of 97 infants identified with CH through newborn screening and/or MACDP (1:5,000 live births), 87 had primary CH and 10 had secondary. The rate of primary CH was higher among non-hispanic whites than among blacks (1:4,400 vs. 1:10,000) and among females compared with males (1:4,000 vs. 1:7,700). Among infants with primary CH, 77 had isolated CH, 3 had Down syndrome, and 7 had unrelated major structural defects. Based on Atlanta population rates of Down syndrome and major structural anomalies, we infer i) infants with Down syndrome have a 35-fold increased risk for primary CH compared with infants in the general population (P < .0001); ii) infants with primary CH have a 2.2-fold increased risk for major structural anomalies (P < .05). Because this is the first population study of CH in the United States in which data from two population-based registries were linked, the epidemiologic patterns and associated defects are more representative than those found in studies based on newborn screening records only.

Unique mosaicism of tetraploidy and trisomy 8: clinical, cytogenetic, and molecular findings in a live-born infant.

We report on a live-born infant with mosaicism of tetraploidy and trisomy 8 who had craniofacial abnormalities, cardiac and genitourinary defects, agenesis of the corpus callosum, and anomalies of limbs. The infant died at age 14 weeks. Molecular studies were done on peripheral blood lymphocytes and cultured amniocytes to determine the origin of the cytogenetic abnormalities. On the basis of the results, we describe a possible mechanism to explain these abnormalities. To our knowledge, this infant represents the first reported case of mosaic trisomy 8 with a tetraploid cell line.

Impact of prenatal diagnosis on the birth prevalence of neural tube defects, Atlanta, 1990-1991.

OBJECTIVE: To determine the impact of prenatal diagnosis on the birth prevalence of neural tube defects (NTDs) in Atlanta during 1990 through 1991. METHODS: Live-born and stillborn infants with NTDs who were at least 20 weeks' gestation were ascertained by the Metropolitan Atlanta Congenital Defects Program (MACDP), a population-based birth defects registry. Prenatally diagnosed NTD-affected pregnancies were ascertained from the four perinatal centers and the three genetic laboratories operating in Atlanta during 1990 through 1991. Fetal death certificates were also reviewed for potential cases. RESULTS: During 1990 through 1991, MACDP ascertained 59 NTD cases, for a birth prevalence of 0.77/1000 live births. During this period, an additional 28 NTD-affected pregnancies were detected prenatally and terminated before 20 weeks' gestation. The adjusted NTD rate during 1990 through 1991, which includes prenatally diagnosed cases, was 1.13/1000 live births. CONCLUSIONS: Prenatal diagnosis is making a substantial impact on the birth prevalence of NTDs in Atlanta. However, since NTD rates in Atlanta were 2 to 2.5 per 1000 live births in 1970, prenatal diagnosis and termination of pregnancy does not completely account for the declining rate of NTDs.

Surveillance for anencephaly and spina bifida and the impact of prenatal diagnosis--United States, 1985-1994.

PROBLEM/CONDITION: The reported prevalence of anencephaly and spina bifida in the United States has steadily declined since the late 1960s. During this time, the ability to diagnose these defects prenatally has progressed rapidly. Many U.S. birth defects surveillance systems ascertain defects only among live-born infants or among infants and fetuses beyond a certain gestational age, thus excluding defects among pregnancies prenatally diagnosed as being affected by a neural tube defect (NTD) and electively terminated before the gestational age limit. The impact of prenatal diagnosis and subsequent pregnancy termination on the reported prevalence of anencephaly and spina bifida in the United States has not been well established. However, assessment of this impact is crucial to the use of surveillance data to monitor trends in the occurrence of NTDs and the effectiveness of interventions for these defects (e.g., increased consumption of folic acid). REPORTING PERIOD: This report presents data from birth defects surveillance systems in six states over different time periods: Arkansas, 1985-1989; California, 1989-1991; Georgia, 1990-1991; Hawaii, 1988-1994; Iowa, 1985-1990; and South Carolina, 1992-1993. DESCRIPTION OF SYSTEMS: Population-based data about a) live-born and stillborn infants with anencephaly and spina bifida and b) pregnancies electively terminated after prenatal diagnosis of these defects were analyzed from the Arkansas Reproductive Health Monitoring System; the California Birth Defects Monitoring Program; CDC's Metropolitan Atlanta Congenital Defects Program; the Iowa Birth Defects Registry, the University of Iowa, and the Iowa Department of Public Health; and the Greenwood Genetic Center in South Carolina. Data also were analyzed from the Hawaii Birth Defects Monitoring Program, which includes data for some women who were not residents of the state. The systems differed in the size and racial/ethnic composition of the populations studied, the surveillance methods used, the completeness of ascertainment, and the availability and utilization of prenatal testing and pregnancy termination. RESULTS AND INTERPRETATION: Among all pregnancies ascertained in which the infant or fetus had anencephaly or spina bifida, the percentages that were electively terminated ranged from 9% in Arkansas to 42% in Atlanta and Hawaii, with a corresponding increase in the adjusted prevalence of these defects compared with the prevalence at birth. In each system, pregnancies associated with anencephaly were terminated more frequently than were those associated with spina bifida. These data indicate that the impact of prenatal diagnosis and subsequent pregnancy termination on the prevalence at birth of anencephaly and spina bifida differs among geographic areas and populations. Comprehensive surveillance for these defects requires inclusion of pregnancies that are prenatally diagnosed and then terminated. ACTIONS TAKEN: CDC will use these data to promote the inclusion of prenatally diagnosed and terminated pregnancies in estimates of the prevalence of anencephaly and spina bifida generated by birth defects surveillance programs in the United States. Including such pregnancies is crucial to the ability of these programs to monitor trends accurately and to establish the effectiveness of interventions, including the use of folic acid, for these defects.

Emergency contraception--why women don't use it.

AIM: The aim of the study was to examine knowledge of and perceived availability of the emergency contraceptive pill as well as reasons for its non use. METHODS: One hundred women each attending Epsom day unit or the Auckland medical aid clinic in Auckland seeking termination of pregnancy, and 100 women seeking contraceptive advice from the Alice Bush centre in Auckland, were asked to take part in the study. RESULTS: At Alice Bush centre 57% of women had previously used the emergency contraceptive pill compared with 43% women at Epsom day unit and 32% women at Auckland medical aid clinic. Only 7% of women attending Epsom day unit or Auckland medical aid clinic had used the emergency contraceptive pill in the month they conceived. When asked why they had not used the emergency contraceptive pill 38% of respondents said they had not heard of it and 41% did not know where to obtain it. Pacific Island women were least likely to have heard of it. Approximately 50% knew the correct time interval for using the emergency contraceptive pill. Sixty two percent attending the abortion clinics would have used the emergency contraceptive pill if they had a supply at home and 57% stated they would have used it if it was available over the counter through pharmacies. CONCLUSIONS: The discrepancy between the numbers of women who knew of the emergency contraceptive pill (72%) and the numbers who used it to try to prevent pregnancy (7%) indicates that there are barriers to obtaining and using the emergency contraceptive pill. This study demonstrated a lack of knowledge of the emergency contraceptive pill in women attending the abortion clinics. The majority of women seeking termination of pregnancy would have used the emergency contraceptive pill if they had it available at home or over the counter through a pharmacy. Doctors prescribing the pill and barrier methods of contraception should consider providing a supply of emergency contraceptive pill at the same time and consideration should be given to over the counter prescribing of the emergency contraceptive pill in New Zealand.

The contraceptive practices of women seeking termination of pregnancy in an Auckland clinic.

AIM: The aim of the study was to assess the contraceptive knowledge and practices of women attending the abortion service at Epsom day unit, Green Lane Hospital. METHODS: Women attending the unit in December 1992 and January 1993 were asked to take part in the study. A questionnaire was administered to consenting women to assess demographic details and previous contraceptive education. If a contraceptive method was used the reason for failure was explored and if no method was used the reason for non use was explored. RESULTS: Sixty-one percent of women were using a contraceptive method in the month of conception. The condom was used by 48% and the pill by 42%. Eight percent of women had never used contraception and 30% were not using a method in the month of conception. Forty-three percent had a household income of less than $22,000 and financial barriers were the reason for non use in 32% of those not using a method. Of women who did not use contraception, only 11% used emergency contraception, whereas 78% of those surveyed said they knew about emergency contraception. Sixty-three percent of women said they had received enough contraceptive education to select and use a method effectively. Pacific Island women were least likely to have received adequate contraceptive education or to have been using a method of contraception. CONCLUSIONS: A number of technical problems were identified with condom use. These problems need to be emphasised by sexuality education programmes and contraceptive prescribers. Omitting pills, diarrhoea, vomiting and drug interactions were important causes of pill failure. The seven day rule needs more emphasis when teaching women how to take the pill and when antibiotics are prescribed. Costs were an important barrier to the use of contraception for a significant proportion of women. Section 99 approval should be utilised more readily and the provision of free contraception, especially to low income groups, needs to be urgently explored.

PIP: The aim was to assess the contraceptive knowledge and practices of women obtaining abortions at Epsom day unit, Green Lane Hospital, Auckland, New Zealand, in December 1992 and January 1993. A questionnaire was administered to consenting women. Of the 553 women asked to participate, 198 (35.89%) declined to enroll, thus leaving 355 participants with a mean age of 23 years. 60% were of European ethnicity. 43% had a yearly income of less than $22,000. Approximately half of those of Maori and Pacific Island descent were in the lowest income bracket. 36% had employment as their primary source of income, while 37% were supported by government benefits. 54.5% of the women were single, and 45% (299) were nulliparous. 63% had received enough contraceptive education to use a method effectively. Pacific Island and Asian women were less likely to have received adequate contraceptive education. 61% (217) of women were using a method in the month of conception. The condom was used by 48% of women, while oral contraception was used by 42%. Of the 9 women who used a diaphragm, 8 did not use it every time. Four women had used an IUD. Two women had used Depo Provera. Some women used a combination of condoms and natural family planning. Eight percent (28/355) had never used contraception, and 31% (111/355) were not using a method at the time of conception. 16 women (4.5%) used the emergency contraception pill within 72 hours of intercourse. Of those women who had not used contraception, only 13 (11.5%) used the emergency contraceptive pill. Of all the women in the study, 34% had used the emergency contraceptive pill previously and 78% had heard of it. Higher income level was associated with greater numbers of women using contraception. A logistic regression model showed an increased use of contraception with increased education (p=0.02). Maori and Pacific Islanders were less likely to use contraceptives (p= 0.002, p=0.0001).

Clinical comparison of two low-dose oral contraceptives, Minulet and Mercilon, in women over 30 years of age.

A comparative study of two low-dose oral contraceptives, gestodene (GES) 75 mcg/ethinyl oestradiol (EE) 30 mcg and desogestrel (DES) 150 mcg/EE 20 mcg, was conducted in women over 30 years of age. This randomised, open-label study was organised in Denmark, Italy, New Zealand and United Kingdom. A total of 505 women received GES/EE and 501 received DES/EE for 6 consecutive menstrual cycles. The two groups were comparable in terms of demographic and gynaecologic characteristics at baseline. However, the menstrual flow length was slightly longer in the GES/EE group before the start of the treatment. The mean age (+/- SD) was 35 +/- 4 years in the GES/EE group and 35 +/- 5 years in the DES/EE group. The subjects in the GES/EE group contributed data for a total of 2800 cycles and those in the DES/EE group, data for 2796 cycles. There were no pregnancies on medication with either preparation. The results showed that there were significantly more normal cycles in the GES/EE group for cycles 1 to 6. Irregular bleeding between withdrawal bleeds occurred in 10% of GES/EE and 18.5% of DES/EE cycles. Absence of all bleeding was reported in 29 (1%) and 63 (2%) cycles, respectively. The incidence of missed pills was low in both groups (11% of cycles). No significant differences were observed in cycle length or withdrawal bleeding episode length. Withdrawal bleeding mean intensity was statistically significantly greater with GES/EE. However, for both preparations, the mean intensity was close to light bleeding. No clinically significant differences were noted in weight, blood pressure, Papanicolaou smears or laboratory data. Sixty-eight (13.5%) subjects in the GES/EE group and 64 (12.8%) in the DES/EE group discontinued before the end of the study. Among them, 37 (7%) and 40 (8%) in the respective groups withdrew because of adverse reactions. There was no difference between groups in terms of primary reasons for withdrawal. The most frequently reported complaints that led to discontinuation in both groups were headache, nausea and metrorrhagia. Breast tenderness led to the discontinuation of 1 subject in the GES/EE group and 3 in the DES/EE group. These results show excellent cycle control, efficacy and very low rate of side effects with both GES/EE and DES/EE. These low-dose oral contraceptives could be well suited to healthy nonsmoking women requiring contraception up to the age of menopause.

PIP: At 66 sites in Denmark, Italy, New Zealand, and the UK, clinicians randomly allocated 1006 women 30 years old, some of whom were in their early 50s, into 1 of 2 groups receiving a low-dose oral contraceptive (OC): Minulet containing 75 mcg gestodene (GES)/30 mcg ethinyl estradiol (EE) and Mercilon containing 150 mcg desogestrel (DES)/20 mcg EE. The study aimed to compare these 2 low-dose OCs to help physicians prescribe an OC that could be continued into later years. Before treatment, the 2 groups had similar demographic and gynecologic characteristics. The mean menstrual flow length in the GES/EE group was longer than that of the DES/EE group (4.7 days vs. 4.5 days; p = .035) though. None of the women during 2800 cycles of GES/EE use and 2796 cycles of DES/EE use conceived, even though women forgot to take at least 1 pill in 11% of cycles. The GES/EE OC had significantly better cycle control than did the DES/EE OC. For example, the GES/EE group was more likely to have normal cycles than the DES/EE group (84-93% vs. 73-83%; p .001). The DES/EE group experienced a significantly lower withdrawal bleeding mean intensity than the GES/EE group in all 6 cycles, but the bleeding for both groups was close to light bleeding. The 2 groups were similar in weight, blood pressure, Papanicolaou smears, and laboratory data. Discontinuation rates for the GES/EE and DES/EE groups were 13.5% and 12.8%, respectively. Adverse reactions accounted for discontinuation in 7% of the GES/EE group and 8% of the DES/EE group. The major complaints leading to discontinuation were headache, nausea, and breakthrough bleeding. Both GES/EE and DES/EE had very good cycle control and efficacy and a very low rate of side effects. These results suggest that both these low-dose OCs would be acceptable for healthy nonsmoking women needing contraception up to menopause.

Clozapine treatment of psychosis in Parkinson's disease.

Treating psychosis in patients with Parkinson's disease (PD) is one of the more difficult problems in clinical psychiatry. In this case report, a patient with PD and psychosis was treated with the novel neuroleptic clozapine and sustained improvement in both behavior and PD symptoms.

Pattern of arteries in the hindlimbs of hybrid mice.

The pattern of the arterial system has been studied in the hindlimbs of adult male and female mice in a hybrid strain. A technique was developed to inspect the distribution pattern after the vessels were injected with a blue polymer, the bone was stained with alizarin red S, and the soft tissue was cleared to transparency. Substantial variations were identified in the point of origin of 6 of 41 arterial branches; extra vessels and absence of vessels were uncommon. The types of arterial differences identified in normal adult mice were different from those identified in mice with absence of the tibia, which have absence of major arteries, including the popliteal and posterior tibial arteries.

Frequency and clinical description of high-cost patients in 17 acute-care hospitals.

To assess the potential impact of national "catastrophic" health insurance on the medical-care system, the frequency and clinical characteristics of high-cost patients were surveyed at 17 acute-care hospitals in the San Francisco Bay Area. The percentage of patients whose yearly hospital charges exceeded $4000 in 1976 ranged from 4 at a community hospital to 24 at a referral hospital. Hospital costs charged to these patients ranged from 20 to 68 per cent of total billings, with the highest percentages generally occurring at large referral hospitals. Forty-seven per cent of adult high-cost patients had chronic medical conditions, and only one in six suffered from an acute medical "catastrophe." In addition, more than 13 per cent of high-cost patients died in the hospital. National catastrophic health insurance is likely to pay for much chronic illness and terminal care and divert resources toward acute-care hospitals.