Cannabis use frequency and pain interference among people with HIV.

Cannabis is often used by people with HIV (PWH) for pain, yet study results are inconsistent regarding whether and how it affects pain. This study examines whether greater cannabis use frequency is associated with lower pain interference and whether cannabis use modifies the association of pain severity and pain interference among 134 PWH with substance dependence or a lifetime history of injection drug use. Multi-variable linear regression models examined the association between past 30-day cannabis use frequency and pain interference. Additional models evaluated whether cannabis use modified the association between pain severity and pain interference. Cannabis use frequency was not significantly associated with pain interference. However, in a model with interaction between cannabis use frequency and pain severity, greater cannabis use frequency attenuated the strength of the association between pain severity and pain interference (p = 0.049). The adjusted mean difference (AMD) in pain interference was +1.13, + 0.81, and +0.05 points for each 1-point increase in pain severity for those with no cannabis use, 15 days of use, and daily use, respectively. These findings suggest that attenuating the impact of pain severity on pain-related functional impairment is a potential mechanism for a beneficial role of cannabis for PWH.

Medication for addiction treatment and acute care utilization in HIV-positive adults with substance use disorders.

Medication for addiction treatment (MAT) could reduce acute care utilization in HIV-positive individuals with substance use disorders. The study objective was to determine if HIV-positive people with substance use disorders treated with MAT report less acute care utilization than those not receiving MAT. We assessed the association between MAT and acute care utilization among HIV-positive individuals with alcohol or opioid use disorder. Acute care utilization 6 months later was defined as any past 3-month self-reported (1) emergency department (ED) visit and (2) hospitalization. Of 153 participants, 88% had alcohol use disorder, 41% had opioid use disorder, and 48 (31%) were treated with MAT. Fifty-five (36%) participants had an ED visit and 38 (25%) participants had a hospitalization. MAT was not associated with an ED visit (AOR 1.12, 95% CI 0.46-2.75) or hospitalization (AOR 1.09, 95% CI 0.39-3.04). MAT was not associated with acute care utilization. These results highlight the need to increase MAT prescribing in HIV-positive individuals with substance use disorders, and to address the many factors that influence acute care utilization.

Effects on alcohol related fatal crashes of a community based initiative to increase substance abuse treatment and reduce alcohol availability.

OBJECTIVE: This analysis tested whether comprehensive community interventions that focus on reducing alcohol availability and increasing substance abuse treatment can reduce alcohol related fatal traffic crashes. INTERVENTION: Five of 14 communities awarded Fighting Back grants by The Robert Wood Johnson Foundation to reduce substance abuse and related problems attempted to reduce availability of alcohol and expand substance abuse treatment programs (FBAT communities). Program implementation began on 1 January 1992. DESIGN: A quasi-experimental design matched each program community to two or three other communities of similar demographic composition in the same state. MAIN OUTCOME MEASURES: The ratio of fatal crashes involving a driver or pedestrian with a blood alcohol concentration of 0.01% or higher, 0.08% or higher, or 0.15% or higher were examined relative to fatal crashes where no alcohol was involved for 10 years preceding and 10 years following program initiation. RESULTS: Relative to their comparison communities, the five FBAT communities experienced significant declines of 22% in alcohol related fatal crashes at 0.01% BAC or higher, 20% at 0.08% or higher, and 17% at 0.15% or higher relative to fatal crashes not involving alcohol. CONCLUSIONS: Community interventions to reduce alcohol availability and increase substance abuse treatment can reduce alcohol related fatal traffic crashes.

Diagnosis of hyperlipidemia and treatment with a statin in the outpatient stroke clinic.

The aim of this study was to assess a stroke clinic's performance in the diagnosis of hyperlipidemia and more specifically to evaluate the effectiveness of statins in patients with cerebrovascular disease not enrolled in a research study. The records of 370 consecutive patients seen at a stroke clinic over a 4-year period were reviewed, and information regarding neurologic diagnosis, lipid profile, and use and type of cholesterol-lowering medication was abstracted. Hyperlipidemia was defined as a total cholesterol level equal to or more than 200 mg/dL. Forty-eight patients meeting specific criteria were further analyzed to monitor the effects of statins. Cholesterol testing was obtained in 324 patients (88%) and 178 (55%) were hyperlipidemic, but only 86 (48%) patients received treatment. The mean cholesterol level of the 48 patients dropped from 246.2 mg/dL to 197.1 mg/dL (P < .0001) after the initiation of statin therapy, and significant reductions were present in subgroups with pretreatment levels of 200 to 249 mg/dL and 250 to 299 mg/dL. Of the 21 patients with repeated cholesterol testing more than 6 months after the first posttreatment test, only 11 (52%) maintained a level below 200 mg/dL. Effective control of hyperlipidemia can be achieved in patients with cerebrovascular disease, but not all are adequately tested or treated. Improved physician awareness and more effective health care delivery systems are needed.

Distribution of cerebral microembolism in the anterior and middle cerebral arteries.

BACKGROUND AND PURPOSE: Cerebral infarcts occur more frequently along the middle (MCA) than the anterior cerebral artery (ACA) territory. The reason(s) for this difference remains speculative. The objective of this study was to investigate the distribution of cerebral microemboli as detected by transcranial Doppler ultrasound (TCD) along the MCA and ACA territories. METHODS: Records of consecutive patients examined for the presence of cerebral microembolism during a 32-month period at the Neurovascular Laboratory were reviewed. Of the original 375 TCD studies in 268 patients, 28 studies in 24 patients demonstrated microembolic signals (MES) and monitored the MCA and ACA on the same side. TCD studies were performed on TC-2000 or TC-2020 instruments. MES positive studies were saved and off-line reviewed. MES satisfied previously established criteria. RESULTS: MES were more frequent in the MCA than the ACA in 85.7% (24/28) of studies (P < 0.01). Of the total number of MES (n = 979), 29.6% (n = 290) were detected in the ACA and 70.4% (n=689) in the MCA (P<0.01). The mean (+/- SD) intensity of MCA MES of 12.2 (+/- 2.4) dB was significantly lower than that of ACA MES of 14.8 (+/-3.2) dB (P=0.05). The mean (+/-SD) duration of MCA MES of 38.1 (+/- 45.3) ms was longer than that of ACA MES of 30.7 (+/-34.0) ms (P=0.05). CONCLUSIONS: Cerebral microembolism occurs more frequently in the MCA than the ACA, which may explain the uneven distribution of cerebral infarcts along these arterial territories. Furthermore, there are significant differences in the characteristics of ACA and MCA MES.

Influence of the history on physicians' interpretations of girls' genital findings.

OBJECTIVE: Because physicians customarily obtain histories before examining children in cases of possible sexual abuse, and because the resulting diagnostic opinions can influence important social and legal decisions, we investigated whether clinical histories influence physicians' interpretations of girls' genital findings. DESIGN: In mailed questionnaires, 1387 randomly selected Fellows of the American Academy of Pediatrics and all 802 members of four professional groups concerned with child abuse or pediatric gynecology were asked to interpret seven simulated cases. Respondents were asked to interpret seven additional cases in separate questionnaires mailed 4 months later. Both sets of cases involved the same seven photographs of girls' external genitalia. However, in six of the seven case pairs, the histories in the two questionnaires differed in the extent to which they suggested sexual abuse. In the remaining (control) pair, the same history was presented in both questionnaires. RESULTS: Of 2189 physicians, 1114 (50.9%) responded. Responses from 604 physicians (54.2%) were eligible for analysis. Overall, the genital findings were interpreted most consistently by the most experienced physicians and least consistently by the least experienced physicians. The proportion of physicians whose interpretations of a photograph reversed in the direction suggested by the change in the associated history from "no indication of abuse" to "probable abuse," or vice versa, ranged for experienced physicians from none to 5.6%; for moderately experienced physicians from 1.6% to 19.8%; and for inexperienced physicians from 3.6% to 27.2%. This difference between the experience groups was statistically significant in four case pairs. Mean interpretation scores for genital findings changed significantly when the histories changed in two case pairs for the experienced physicians, in five pairs for the moderately experienced physicians, and in all six pairs for the inexperienced physicians. CONCLUSIONS: In some cases and especially for less experienced physicians, diagnostic expectation appears likely to influence physicians' interpretations of girls' genital findings. Physicians should be alert to the possibility of diagnostic expectation bias and its potentially serious social and legal consequences.

Risk factor modification in stroke prevention: the experience of a stroke clinic.

BACKGROUND AND PURPOSE: [corrected] We reviewed Stroke Clinic data to determine the extent of risk factor modification achieved in patients with cerebrovascular disease over 2 years. METHODS: Visits to the Stroke Clinic of a tertiary medical center from July 1, 1994, through June 30, 1996, were reviewed. Obesity, smoking, hypertension, hyperlipidemia, hyperglycemia, and lifestyle changes were noted in patients with >/=2 visits (n=61) and measures (number varied) of these parameters. RESULTS: Fifty-six patients (92%) had primary care physicians. In the 49 patients with >/=2 weight measurements, 33 (67%) were moderately or severely overweight by weight-height correlation. Forty-four patients (90%) remained in the same weight category. Of the 60 patients with available blood pressure data, 50 (83%) were hypertensive. At their last visits, 43 of the 50 (86%) were receiving medications, and 22 of the 43 treated (51%) were controlled. Serum glucose remained elevated in 14 of 47 patients (30%) and in 11 of 16 diabetic patients (69%). Thirty-six of 47 patients (55%) had elevated lipid measurements. None of the 21 smokers quit during the study period. Few patients modified dietary and exercise practices. Of 61 patients, 29 (48%) sustained vascular events during the study, with 17 of these 29 patients (59%) having strokes or transient ischemic attacks. CONCLUSIONS: Although most patients were asked to quit smoking, received advice regarding diet and exercise, and were medicated for hypertension, elevated glucose, and cholesterol levels, their risk factor profiles showed little improvement during the 2-year period. More effective methods of controlling stroke risk factors are needed.

Preventing impaired driving.

Although moderate drinking does not necessarily increase a person's blood alcohol concentration (BAC) to the level at which driving is legally prohibited in the United States, any drinking can impair driving tasks. In addition to laws establishing lower legal BAC limits for drivers, legislative approaches for reducing alcohol-impaired driving include imposing sanctions for drinking and driving and restricting alcohol's availability.

Loss of heterozygosity at 11q23 in squamous cell carcinoma of the head and neck is associated with recurrent disease.

Loss of heterozygosity (LOH) at chromosome 11q23 has been found in a variety of epithelial human neoplasms, suggesting that this region contains a tumor suppressor gene(s) important to tumorigenesis. We investigated whether LOH at 11q23 could be detected in squamous cell carcinoma of the head and neck (SCCHN), and whether loss at this site was associated with specific clinical parameters. Fifty-six matched blood and SCCHN tumor samples taken at the time of diagnosis were evaluated for LOH at three microsatellite markers at 11q23. Multiplex PCRs with [alpha-32P]dCTP labeling of the amplified DNA strands were performed. Clinical data were obtained from medical record review. LOH at 11q23 was found in 13 of 52 (25%) evaluable tumors. There was no association between LOH at 11q23 and amplification of the CCND1 (cyclin D1) oncogene or inactivation of the p53 gene, which had been determined previously. With a mean follow-up of 24 months, an association independent of tumor size or stage was found between LOH at 11q23 and recurrent disease (P = 0.04). Among subjects who received radiotherapy (RT) as a component of their treatment, LOH at 11q23 was associated with persistent or recurrent locoregional disease (P = 0.05). LOH at 11q23 occurs in a subset of SCCHN. It is associated with a higher likelihood of recurrent disease, perhaps related to resistance to RT. The specific gene(s) and mechanism(s) responsible remain to be identified. Until then, LOH at 11q23 might become a marker identifying patients likely to do poorly with conventional therapy.

Cerebral microembolism and ischemic changes associated with carotid endarterectomy.

PURPOSE: The purpose of this study is to characterize microembolic signals (MS) that occur during the various phases of carotid endarterectomy (CEA) and to consider their relationship to postoperative changes on magnetic resonance imaging (MRI). METHODS: This was a retrospective study of 76 patients who underwent 78 carotid endarterectomies at a referral center. Perioperative transcranial Doppler monitoring and MRI were performed before and after CEA. The types of MS that occurred during phases of surgery were analyzed and compared with MRI changes. RESULTS: We observed a clinical stroke in one patient (1.3%) and ipsilateral small areas of silent ischemic change on seven postoperative MRI studies (9.0%). In 95% of CEAs, MS were detected. Only those MS observed in the recovery room that occurred at a rate of more than five per 15 minutes were associated with postoperative MRI ischemic changes (p < 0.0001). CONCLUSIONS: Ischemic changes on MRI after CEA are related to postoperative MS.

Cerebral microembolism in patients with retinal ischemia.

BACKGROUND AND PURPOSE: We investigated the frequency of cerebral microembolism detected by transcranial Doppler ultrasonography in patients with clinical evidence of retinal ischemia, including transient monocular blindness, central and branch retinal artery infarction, and ischemic oculopathy, and assessed its correlation with carotid artery stenosis. METHODS: Records of 331 consecutive patients examined during a 47-month period at the Neurovascular Laboratory were reviewed. Of the original 453 intracranial arteries, 186 middle cerebral arteries (MCAs) satisfied qualifying criteria that excluded patients with cardiac embolic sources. Forty-five MCAs ipsilateral to the symptomatic eye constituted the study group. The control group consisted of 141 asymptomatic MCAs. Microembolus detection studies were performed on transcranial Doppler instruments equipped with special software, and the degree of carotid artery stenosis was measured by cerebral or MR angiography or by color duplex studies. RESULTS: Microembolism was detected in 40.0% of study MCAs and 9.2% of controls (P < 0.001). In the study group, microembolic signals were detected in 61.9% of MCAs tested within a week of symptom onset and 20.8% of those tested afterward (P < 0.001). Severe (> or = 70%) carotid stenosis or occlusion was more frequent in the study group (P < 0.001). Microembolic signals were detected in 25.3% and 11.2%, respectively, of MCAs distal to carotid arteries with 70% to 100% and 0% to 69% stenosis (P = 0.013). CONCLUSIONS: In patients without cardiac embolic sources, cerebral microembolism is frequently present on the side of retinal ischemia, particularly during the week after onset of symptoms. It is often associated with severe stenosis or occlusion of the ipsilateral carotid artery.

Assessments of girl's genital findings and the likelihood of sexual abuse: agreement among physicians self-rated as skilled.

OBJECTIVES: To measure agreement about genital examination findings among physicians who rate themselves as skilled in evaluating children for suspected sexual abuse, to compare these physicians' descriptions and interpretations with consensus standards developed by an expert panel, and to investigate the effects of physician and case characteristics on agreement. STUDY DESIGN: Questionnaires including 7 simulated cases, each consisting of a brief history and 1 photograph of a girl's genitalia, were mailed to random samples of 2 groups: the members of 4 physician organizations concerned with child abuse or pediatric gynecology, and pediatricians at large. Among the surveyed physicians who rated their own skill in evaluating cases of suspected sexual abuse as higher than average, we measured agreement, both overall and between those with the most and with less clinical experience, and assessed their conformity with consensus standard descriptions and interpretations. RESULTS: We received responses from 548 (50.9%) of 1076 physicians; 414 responses (75.5%) were analyzable. Two hundred six physicians (50%) rated themselves as skilled in assessing children for sexual abuse. On average, 45% of these physicians' descriptions and 72.6% of their interpretations conformed with the consensus standards. In 4 cases, between 5% and 20.7% of these physicians described genital findings that the expert panel had considered absent from the photographs. Conformity with standard interpretations tended to be higher in cases with photographs concordant with the accompanying, unambiguous histories (P=.06). The most experienced physicians resembled the expert panel more closely than did the less experienced self-rated skilled physicians in interpreting 3 simulated cases (P< or =.001). CONCLUSIONS: Assessments of girls' genital findings by physicians who rate themselves as skilled in examining children for suspected sexual abuse often differ. In some cases, among physicians who all rate themselves as skilled, assessments made by very experienced physicians may conform more closely to consensus standards than do assessments made by less experienced physicians.

Factors associated with HIV testing among sexually active adolescents: a Massachusetts survey.

OBJECTIVE: To assess sexually active adolescents' knowledge, attitudes, and behaviors associated with human immunodeficiency virus (HIV) testing and to determine the factors important in their decision to obtain voluntary HIV testing. DESIGN: Anonymous, random, digit-dial telephone survey undertaken in 1993. SETTING: Massachusetts households. PARTICIPANTS: Adolescents, 16 to 19 years of age. RESULTS: Of the 567 adolescents surveyed who had sexual intercourse within the past year, 127 (22%) had received HIV testing, with 54 (10%) stating that this testing was for personal reasons. A "great deal" or "some" worry about getting HIV/acquired immunodeficiency syndrome (AIDS) was expressed by 51%, and 56% felt that it was at least a little likely that they will get AIDS. Misconceptions were common about aspects of HIV testing: 35% did not believe or did not know that the HIV test results were kept in confidence, 19% thought that AIDS testers informed partners if the results were positive, and 30% did not think that the HIV test was very accurate. Although 92% (452/490) had seen a physician in the past year, only 30% (136/452) had ever discussed AIDS with a doctor. Multivariable analysis identified five factors as independently associated with voluntary adolescent HIV testing: 1) having had more than one sexual partner within the past year [odds ratio (OR): 2.9; 95% confidence interval (CI): 1.5, 5.5]; 2) believing that condoms are only somewhat effective at preventing the spread of AIDS (OR: 2. 6; 95% CI: 1.4, 4.8); 3) having discussed AIDS with a doctor (OR: 2. 6; 95% CI: 1.4, 4.8); 4) not having had a teacher discuss AIDS (OR: 2.2; 95% CI: 1.2, 4.2); and 5) believing that a positive test result means one has AIDS as opposed to carrying the virus (OR: 2.0; 95% CI: 1.1, 3.7). High-risk behavior of infrequent condom use and a history of a sexually transmitted disease were not significantly associated with voluntary HIV testing. CONCLUSION: Among sexually active Massachusetts adolescents, voluntary HIV testing is uncommon. Teens who have had multiple sexual partners and who do not believe condoms are effective in preventing transmission were most likely to have been tested. Issues requiring clearer communication to patients include the testing process, its availability, and confidentiality. Physicians can play an influential role in the promotion of HIV testing by discussing HIV risk behaviors with patients and offering those at risk voluntary HIV counseling and testing.

Cerebral microembolism and early recurrent cerebral or retinal ischemic events.

BACKGROUND AND PURPOSE: We investigated whether cerebral microembolism as detected by transcranial Doppler ultrasonography (TCD) identifies patients at an increased risk for early, recurrent cerebral or retinal ischemic events. METHODS: Records of consecutive patients examined during a 40-month period in the Neurovascular Laboratory were reviewed for the presence of cerebral microembolism. Of the original 302 patients, 229 with 310 arteries met inclusionary criteria. Follow-up information was obtained from the laboratory's database as well as the hospital records. Microembolus detection studies were performed on TC-2000 or TC-2020 instruments equipped with special software, and criteria established a priori were used for microembolus selection. TCD testing was performed a median interval of 9 days after the initial symptoms of cerebral ischemia. Severity of arterial stenosis was determined by cerebral angiography or noninvasive methods. RESULTS: Microembolic signals were detected more frequently in symptomatic (40/140; 28.6%) than asymptomatic (21/170; 12.4%) arteries (P < .001). Ten recurrent ischemic events occurred during a median follow-up of 8 days after TCD examination, all in the territories of symptomatic arteries. Nine events occurred in the territories of microembolic signal positive arteries (9/61; 14.8%) and one in the territory of a microembolic signal-negative artery (1/249; 0.4%) (P < .00). No association was detected in the subgroup with known cardiac lesions. Microembolic signals were more frequent in arteries with lesions causing 70% or more stenosis or occlusion (26/99; 26.3%) than in those with a degree of stenosis less than 70% (17/126; 13.5%) (P = .016). CONCLUSIONS: In this retrospective study, microembolic signals were more common in the territories of symptomatic arteries and particularly those with severely stenotic lesions. During a short follow-up, recurrent ischemic events were more common along the territories of arteries with TCD-detected microembolism and previous symptoms of cerebral or retinal ischemia.

Home birth in the United States, 1989-1992. A longitudinal descriptive report of national birth certificate data.

This study was conducted to profile home birth in the United States from 1989 to 1992 using two birth certificate data sources from the Natality Branch of the National Center for Health Statistics (NCHS). Analysis included published and unpublished descriptive tables about all U.S. home births from 1989 to 1992, and a subset of the 82,210 U.S. home births from 1989 to 1991 that were drawn from NCHS national birth certificate data tapes. Results indicated that less than one-third of reported home births were attended by nurse-midwives or physicians. Distinct regional patterns in the frequency of home births were observed, with higher concentrations in the southwestern and western states. When compared with the average childbearing woman in the United States, mothers who gave birth at home were more likely to be older, have fewer years of education, be married, and be white; they were also more likely to be of higher parity and to receive less prenatal care. Home birth mothers were less likely than average to smoke or drink alcohol prenatally, to have a prenatal medical risk condition or an obstetric complication, or to receive certain prenatal tests. The outcomes of newborns born at home compared favorably to the national average during the same period. Several findings varied considerably by race or ethnicity of the mother.

Clinical correlates of high-intensity transient signals detected on transcranial Doppler sonography in patients with cerebrovascular disease.

BACKGROUND AND PURPOSE: High-intensity transient signals detected by transcranial Doppler sonography have been associated with particulate cerebral emboli. Their clinical correlates are poorly understood. This study was undertaken to assess their relation to cerebral ischemia and to determine whether the severity of cerebral arterial stenosis has an impact on their occurrence. METHODS: We studied 96 arteries in 75 consecutive patients with extracranial or intracranial arterial lesions or potential cardiac sources of cerebral embolism. Sixty patients had histories of cerebral or retinal transient ischemic attacks or infarcts, and 15 were asymptomatic. The diagnosis of ischemia was based on the clinical presentation and was supported by extensive laboratory testing. A transcranial Doppler sonography unit equipped with special software for emboli detection was used. Signals were selected based on criteria established a priori. RESULTS: Signals were detected in the territories of 28.3% of symptomatic and 11.6% of asymptomatic arteries. The difference was significant (P = .045). When patients with suspected cardiac embolic sources were excluded, the difference between symptomatic (27.9%) and asymptomatic (2.9%) arteries remained significant (P = .003), and signals were more frequent distal to arteries with more than 50% area stenosis (23.5%) than arteries with stenoses equal to or less than 50% (3.7%) (P = .028). In patients with only extracranial internal carotid artery stenoses, the difference between these degrees of stenosis remained significant (P = .043). CONCLUSIONS: We conclude that high-intensity transient signals are significantly more common in the territories of symptomatic arteries and distal to lesions causing more than 50% stenosis. These findings may have diagnostic and therapeutic applications.

A controlled trial of diazepam administered during febrile illnesses to prevent recurrence of febrile seizures.

BACKGROUND: Phenobarbital, once widely prescribed to prevent febrile seizures, is now in disfavor because of its side effects and lack of efficacy. Diazepam, administered only during episodes of fever, may be a safe, effective agent to prevent the recurrence of febrile seizures. METHODS: We conducted a randomized, double-blind, placebo-controlled trial among 406 children (mean age, 24 months) who had at least one febrile seizure. Diazepam (0.33 mg per kilogram of body weight) or placebo was administered orally every eight hours during all febrile illnesses. RESULTS: During a mean follow-up of 1.9 years (a period during which 90 percent of febrile seizures recur), our intention-to-treat analysis showed a reduction of 44 percent in the risk of febrile seizures per person-year with diazepam (relative risk = 0.56; 95 percent confidence interval, 0.38 to 0.81; P = 0.002). A survival analysis of the length of time to the first recurrent febrile seizure did not show a significant difference between the treatment groups (P = 0.064 by the log-rank test), but after adjustment for covariates, diazepam was found to have a benefit (P = 0.027 by Cox regression analysis). An analysis restricted to children who had seizures while actually receiving the study medication (7 in the diazepam group and 29 in the placebo group) showed an 82 percent reduction in the risk of febrile seizures with diazepam (relative risk = 0.18; 95 percent confidence interval, 0.09 to 0.37; P < 0.001). Of the 153 children who took at least one dose of diazepam, 39 percent had ataxia, lethargy, or irritability or at least one other moderate side effect that was reversed after a reduction in the dose. There were no severe side effects. CONCLUSIONS: Oral diazepam, given only when fever is present, is safe and reduces the risk of recurrent febrile seizures.

Factor X deficiency in the neonatal period.

An infant with a severe deficiency of factor X presened in the neonatal period with uncontrollable bleeding from heel prick sites, spontaneous bruising, and haematoma. The deficiency was controlled by infusions of dried human factors II, IX, and X concentrate; the half-life of the infused factor X material is only 18 hours. Despite prophylactic weekly infusions of factor X concentrate, the child developed a fatal intracerebral haemorrhage when only 4 months old. Coagulation studies on both parents and the elder sister showed no obvious coagulation abnormality.