Median Number Of Attacks Research Articles

The Effect of Montelukast in Children with Asthma Predictive Index Negative Episodic Viral Wheezing

Background: The effectiveness of montelukast for wheezing in young children is not clear. We aimed to evaluate whether the use of montelukast in the treatment of wheezing affects the number of attacks in children aged 1 to 6 years. Methods: This is a single center study. It was conducted between August 2019-2020. Children with aged 0-6 years old with a wheezing complaint who applied to the pediatric allergy and immunology outpatient clinic were included in the study. Modified asthma predictive index of the patients were evaluated. It was classified as Episodic Viral Wheezing (EVW) or Multiple Trigger Wheezing (MTW) based on the type of wheezing. Montelukast treatment was started and the number of attacks was checked during five months. Results: The total of 141 children who applied with wheezing complaints were included in the study. The majority of the patients were boys (n = 99). The mean age of the children was 4.2 ± 1.5 years. The median number of attacks before treatment was 2 (2-3). In 2.8% of the patients, eczema, in 12.8% of them, rhinitis and in 26.7% of them, elevated eosinophils were detected. IgE levels were 148.25 ± 279.20 IU/mL. According to the number of attacks before treatment, there was a significant decrease in the number of attacks in followup (p < 0.001). Conclusion: In this study, montelukast has been shown to reduce the number of EVW attacks and we believe montelukast may be a potential treatment option for EVW in preschool children.

Mycophenolate mofetil: An alternative disease-modifying agent for MOG-IgG-associated disorders in childhood: A single-center bidirectional cohort study

ObjectiveTo evaluate the efficacy of mycophenolate mofetil (MMF) in the treatment of childhood MOG-IgG-associated disorder (MOGAD). MethodsThirty patients diagnosed with relapsing MOGAD and treated with MMF for >1 year from a childhood MOGAD ambispective cohort were included in the study. The clinical characteristics, therapeutic regimen, side effects, annualized relapse rate (ARR), and Expanded Disability Status Scale (EDSS) scores of these patients were evaluated. ResultsThe median age of disease onset was 7.05 (2.50–12.75) years. The male to female ratio was 1:1.31. All patients used MMF as first-line maintenance treatment. The median time to add MMF from disease onset was 1.08 (0.25−5.00) year. The median number of attacks before MMF initiation was 2 (2 − 8). The median duration of MMF therapy was 2.13 (1.00−3.58) years. Twenty (66.67%) patients did not experience further attacks during MMF therapy. The Kaplan–Meier curves showed a 3-year relapse-free rate of 59.8% (95% CI, 36.62−76.88%). ARR decreased during MMF therapy (0 (0 − 1.72) vs. 1.25 (0.60−4.00); P < 0.05). EDSS stabilized during MMF therapy (1.0 (0 − 2.0) vs. 0 (0 − 2.0); P = 0.206). None of the patients stopped the use of MMF due to intolerable side effects. Onset age, sex, phenotype of the first attack, ARR before MMF, MOG-IgG titers, and combined long-term prednisone (prednisone <10 mg daily for patients >40 kg or <5 mg daily for patients ≤40 kg longer than 6 months) did not predict recurrence during MMF therapy in univariate analysis. ConclusionsMMF was effective and safe for treating childhood MOGAD. No clinical feature that could predict efficacy of MMF was found in pediatric patients with MOGAD.

Assessment of crab fishermen's exposure to rabies virus in a typical Amazonian community.

Outbreaks of human rabies transmitted by hematophagous bats occurred in 2018 in Pará state, Brazil, eastern Amazon, after 12years of no record of the disease. Thus, it is necessary to understand the epidemiological characteristics of these attacks to protect the local population. This study aimed to characterize the bat bite populations in the municipality of São João da Ponta, Pará State, Brazil, in 2013-2015. The Notifiable Diseases Information System (SINAN) database was used to identify the five individuals who sought medical care during the study period (seeds). Other individuals who were attacked during the same period but did not seek medical care (n=61) were reached by snowball sampling, and a descriptive analysis was performed based on information obtained from questionnaires. Majority of the interviewees were men (92.4%; 61/66) and adults aged 20-50years (69.9%; 46/66) and had <4years of formal school education (86.3%; 57/66). Additionally, most of them were rural residents (92.4%; 61/66) and crab fishermen (79.3%; 53/66). The interviewees (92.4%; 61/66) identified mangroves of the Mãe Grande de Curuçá extractive reserve, where groups of fishermen sometimes gather for several days for crab fishing, often living in improvised dwellings without walls and covered by tarps or straw (88.8%; 56/66), conducive to attacks by vampire bats. Overall, 42.4% (28/66) of the participants had been bitten more than four times throughout their life. The median number of attacks over the participant's lifetime was 3.11 (range, 1-23). Participants were unaware of the risk of contracting rabies from the bite (95.4%; 65/66). These results suggest that vampire bat attacks are essentially an occupational hazard in the study region. Moreover, for each reported attack, there were at least 12.2 unreported cases. Thus, the study highlights the need to develop strategies for prophylactic treatment of this population.

Observations of parasitoid behaviour in both no‐choice and choice tests are consistent with proposed ecological host range

AbstractThe solitary larval endoparasitoid Eadya daenerys Ridenbaugh (Hymenoptera: Braconidae) is a proposed biocontrol agent of Paropsis charybdis Stål (Coleoptera: Chrysomelidae, Chrysomelinae), a pest of eucalypts in New Zealand. Eadya daenerys oviposition behaviour was examined in two assay types during host range testing, with the aim of improving ecological host range prediction. No‐choice sequential and two‐choice behavioural observations were undertaken against nine closely related species of New Zealand non‐target beetle larvae, including a native beetle, introduced weed biocontrol agents, and invasive paropsine beetles. No behavioural measure was significantly different between no‐choice and two‐choice tests. In sequential no‐choice assays the order of first presentation (target–non‐target) had no significant effect on the median number of attacks or the attack rate while on the plant. Beetle species was the most important factor. Parasitoids expressed significantly lower on‐plant attack rates against non‐targets compared to target P. charybdis larvae. The median number of attacks was always higher towards target larvae than towards non‐target larvae, except for the phylogenetically closest related non‐target Trachymela sloanei (Blackburn) (Coleoptera: Chrysomelidae, Chrysomelinae). Most non‐target larvae were disregarded upon contact, which suggests that the infrequent attack behaviour observed by two individual E. daenerys against Allocharis nr. tarsalis larvae in two‐choice tests and the frass of Chrysolina abchasica (Weise) was probably abnormal host selection behaviour. Results indicate that E. daenerys is unlikely to attack non‐target species apart from Eucalyptus‐feeding invasive paropsines (Chrysomelinae). Non‐lethal negative impacts upon less preferred non‐target larvae are possible if E. daenerys does attack them in the field; however, this is likely to be rare.

Occipital nerve stimulation and deep brain stimulation for refractory cluster headache: a prospective analysis of efficacy over time.

Occipital nerve stimulation (ONS) and deep brain stimulation (DBS) are widely used surgical treatments for chronic refractory cluster headache (CH). However, there is little literature regarding long-term follow-up of these treatments. The authors describe two prospective cohorts of patients with refractory CH treated with ONS and DBS and compare preoperative to postoperative status at 6 and 12 months after the surgery and at final follow-up. Efficacy analysis using objective and subjective variables is reported, as well as medication reduction and complications. The ONS group consisted of 13 men and 4 women, with a median age of 44 years (range 31-61 years). The median number of attacks per week (NAw) before surgery was 28 (range 7-70), and the median follow-up duration was 48 months. The DBS group comprised 5 men and 2 women, with a median age of 50 years (range 29-64 years). The median NAw before surgery was 56 (range 14-140), and the median follow-up was 36 months. The NAw and visual analog scale score were significantly reduced for the ONS and DBS groups after surgery. However, while all the patients from the DBS group were considered responders at final follow-up, with more than 85% being satisfied with the treatment, approximately 29% of initial responders to ONS became resistant by the final follow-up (p = 0.0253). ONS is initially effective as a treatment for refractory CH, although a trend toward loss of efficacy was observed. No clear predictors of good clinical response were found in the present study. Conversely, DBS appears to be effective and provide a more stable clinical response over time with an acceptable rate of surgical complications.

Relapsing periodic arthritis, palindromic rheumatism and MEFV gene-related variants alleles in children

BackgroundRelapsing periodic arthritis is a general term used for a group of diseases with recurring and periodic nature, in which the joints are intermittently involved. The aim of this study is to evaluation of the possible relationship between MEFV gene mutations in intermittent arthritis of children which has recurring and periodic nature.MethodsIn this cross-sectional study we reviewed medical records of all patients with recurrent and periodic arthritis referred to pediatric rheumatology clinic from 2003 to 2019.We excluded all patients with suspicious anamnesis and/or positive history of FMF, JIA, gout, psoriasis, IBD and SLE. The peripheral blood of these patients was screened for the 12 common pathogenic MEFV gene variants (FMF Strip Assay, Vienna lab, Vienna, Austria) according to manufacturer’s instructions.ResultsAmong 195 recorded files, 11 patients were identified with idiopathic recurrent and periodic feature. 3 patients suffering from recurrent transient synovitis hip (RSH) and 8 patients were fully compatible with the Pasero and Barbieri modified criteria of palindromic rheumatism (PR). Their mean age at diagnosis was 6.5 and 4.6 years and the average follow-up was 2.6 and 6.6 years in PR and RSH patients, respectively. The most frequently involved joints were knees and ankles in PR patients. The mean duration of attacks was 3.3 days in RSH patients and with various courses in PR. The median number of attacks was 4.75 in PR and 3 in RSH patients per year. In PR group 7 patients showed no mutations and all patients of RSH had these mutations; V726A, R761H, A744S as in heterozygote pattern.ConclusionAn idiopathic relapsing periodic arthritis in children with exclusively hip involvement is a MEFV gene related arthropathy; whereas with various joint attacks it could be considered as childhood PR.

A comparative study of the efficacy of using piracetam with iron therapy versus iron therapy alone in children with breath-holding spells

Background Breath-holding spells (BHS) are apparently frightening events occurring in otherwise healthy children. Generally, no medical treatment is recommended and parental reassurance is believed to be enough; however, in some cases BHS can be very stressful for the parents and a pharmacological agent may be desired in some of these children. Objective The aim of this work was to determine the efficacy of using piracetam with iron therapy versus iron therapy alone in children with BHS associated with iron-deficiency anemia. Study design The study design was an interventional randomized controlled one. Patients and methods This study was conducted at the Neuro-Pediatric Outpatient Clinic at Alexandria University Children’s Hospital, Egypt. A total number of 70 children between 6 months and 5 years of age with a diagnosis of BHS and iron-deficiency anemia reporting to the clinic were randomly divided into two groups: group I included 35 children who were given only iron therapy at a dose of 6 mg/kg/day, and group II included 35 children who were given iron therapy at the same dose and piracetam at a dose of 40 mg/kg/day. Follow-up of the cases monthly for 3 months was scheduled to evaluate the frequency of the attacks and the improvement in iron deficiency by evaluating iron profile after 3 months (iron, ferritin, and total iron binding capacity). Results Seventy patients were enrolled in this study (42 boys and 28 girls) and completed the study. The ratio of boys to girls was 3 : 2. The patients’ ages ranged between 6 months and 5 years at the time of presentation with 50% above 2 years of age. The frequency of spells varied widely, ranging from 1 to 3 per day to 3 per month, and the mean frequency was six episodes of BHS in 1 month (about 2/week) for both groups. On comparing the improvement in the frequency of attacks between the two studied groups after treatment, there was a significant difference (P=0.001) between the two groups; the median number of attacks was two attacks per month for group I and one attack per month for group II. Conclusion Treatment with piracetam in addition to iron therapy is better than iron therapy alone in reducing the frequency of breath-holding attacks in children with BHS associated with iron-deficiency anemia.

Idiopathic hypokalemic periodic paralysis: A series of cases clustered in a part of Eastern India

Background: Most of the studies on hypokalemic periodic paralysis (HOPP) in India and abroad had described secondary causes as the origin of the disease. We had observed a series of HOPP cases clustered within 40 kilometers around Bankura, a district town of West Bengal, Eastern India. The aim of our study was to probe into the clinical, biochemical and electrophysiological details of the cases, which were found to be idiopathic. Material and Methods: 32 idiopathic HOPP cases, attending the neurology OPD or admitted in Bankura Sammilani Medical College &amp; Hospital (BSMCH), were selected from October 2006 to May 2008. All the secondary causes of HOPP were excluded. The patients were evaluated and the data was analyzed. Results: Cases were clustered within a 40-kilometer radius from Bankura town, location of BSMCH. Male: female = 4.3:1. Median age of onset was 23.5 years (4-50 years). Median number of attacks was 2.5, (1 to 70); duration 3 hours - 6 days, median 3 days. Strenous physical activity was most common precipitating factor. 25% had bulbar symptoms, 9.4% had bladder dysfunction and 46.9% had diminished to absent deep tendon reflexes. Mean serum potassium = 2.45 mEq/L (1.4 – 3.1 mEq/L). Conclusion: We had observed a group of idiopathic HOPP cases within a relatively small area in a short span of time in contrast to most other Indian studies, where the share of idiopathic aetiology is comparatively smaller. This underscores the importance of a larger study with genetic analysis on the aetiology of the idiopathic HOPP in this area.DOI: http://dx.doi.org/10.3126/ajms.v6i6.12482 Asian Journal of Medical Sciences Vol.6(6) 2015 83-87

Efficacy of interleukin-1 targeting treatments in patients with familial mediterranean Fever.

Herein, we reported our experience in colchicine-resistant familial Mediterranean fever (FMF) patients who are treated with anti-interleukin-1 (IL-1) drugs. A retrospective review of medical records of anti-IL-1 recipients was performed. The main clinical characteristics of these patients and the evolution after anti-IL-1 were recorded. There were 20 patients (11 male [M] and 9 female [F]). Despite regular colchicine treatment, median number of attacks per month and per year was 1 (1-4) and 12 (4-50), respectively. Twelve patients were receiving anakinra, and eight patients were treated with canakinumab. The number of monthly and yearly attacks after IL-1 treatment was significantly decreased after the biologic agent (p < 0.05). One patient did not respond to the treatment, and one patient developed serious infection during anti-IL-1. We also observed a significant decrease in proteinuria in the amyloidosis complicated FMF patients. Anti-IL-1 targeting drugs seem safe and effective therapies in colchicine-resistant FMF.

Aquaporin 4 IgG Serostatus and Outcome in Recurrent Longitudinally Extensive Transverse Myelitis

Studies focused on recurrent longitudinally extensive transverse myelitis (rLETM) are lacking. To determine the aquaporin 4 (AQP4) IgG detection rate using recombinant human APQ4-based assays in sequential serum specimens collected from patients with rLETM categorized as negative by first-generation tissue-based indirect immunofluorescence (IIF) assay and to define the clinical characteristics and motor disability outcomes in AQP4-IgG-positive rLETM. A search of the Mayo Clinic computerized central diagnostic index (October 1, 2005, through November 30, 2011), cross-linked with the Neuroimmunology Laboratory database, identified 48 patients with rLETM, of whom 36 (75%) were positive and 12 (25%) negative for neuromyelitis optica (NMO) IgG (per IIF of serial serum specimens). Stored serum specimens from "seronegative" patients were retested with recombinant human AQP4-based assays, including enzyme-linked immunosorbent, transfected cell-based, and fluorescence-activated cell-sorting assays. Control patients included 140 AQP4-IgG-positive patients with NMO, of whom a subgroup of 20 initially presented with 2 attacks of transverse myelitis (rLETM-onset NMO). AQP4-IgG serostatus, clinical characteristics, and Expanded Disability Status Scale score. Six patients with negative IIF results were reclassified as AQP4-IgG positive, yielding an overall AQP4-IgG seropositivity rate of 89%. Fluorescence-activated cell-sorting, cell-based, and enzyme-linked immunosorbent assays improved the detection rate to 89%, 85%, and 81%, respectively. The female to male ratio was 2:3 for AQP4-IgG-negative rLETM and 5:1 for AQP4-IgG-positive patients. The AQP4-IgG-positive patients with rLETM or rLETM-onset NMO were similar in age at onset, sex ratio, attack severity, relapse rate, and motor disability. From Kaplan-Meier analyses, 36% of AQP4-IgG-positive patients with rLETM are anticipated to need a cane to walk within 5 years after onset. For patients with rLETM-onset NMO, the median time from onset to first optic neuritis attack (54 months) was similar to the median disease duration for AQP4-IgG-positive patients with rLETM (59 months). The median number of attacks was 3 for AQP4-IgG-positive patients with rLETM (range, 2-22), and the first optic neuritis attack for those with rLETM-onset NMO followed a median of 3 myelitis attacks (range, 2-19). Immunosuppressant therapy reduced the relapse rate in both AQP4-IgG-positive and AQP4-IgG-negative patients with rLETM. Recombinant antigen-based assays significantly increase AQP4-IgG detection in patients with rLETM, and AQP4-IgG-negative adults with rLETM are rare. Evolution to NMO can be anticipated in AQP4-IgG-positive patients. Early initiation of immunotherapy may result in a more favorable motor outcome.

Eculizumab in AQP4-IgG-positive relapsing neuromyelitis optica spectrum disorders: an open-label pilot study

Complement activation after binding of an IgG autoantibody to aquaporin 4 (AQP4) is thought to be a major determinant of CNS inflammation and astrocytic injury in neuromyelitis optica. The aim of this study was to investigate the use of eculizumab--a therapeutic monoclonal IgG that neutralises the complement protein C5--in neuromyelitis optica spectrum disorders. Between Oct 20, 2009, and Nov 3, 2010, we recruited patients from two US centres into an open-label trial. Patients were AQP4-IgG-seropositive, aged at least 18 years, had a neuromyelitis optica spectrum disorder, and had at least two attacks in the preceding 6 months or three in the previous 12 months. Patients received meningococcal vaccine at a screening visit and 2 weeks later began eculizumab treatment. They received 600 mg intravenous eculizumab weekly for 4 weeks, 900 mg in the fifth week, and then 900 mg every 2 weeks for 48 weeks. The coprimary endpoints were efficacy (measured by number of attacks [new worsening of neurological function lasting for more than 24 h and not attributable to an identifiable cause]) and safety. Secondary endpoints were disability (measured by expanded disability status scale), ambulation (Hauser score), and visual acuity. At follow-up visits (after 6 weeks and 3, 6, 9, and 12 months of treatment; and 3 and 12 months after discontinuation), complete neurological examination was undertaken and an adverse event questionnaire completed. This trial is registered with ClinicalTrials.gov, number NCT00904826. We enrolled 14 patients, all of whom were women. After 12 months of eculizumab treatment, 12 patients were relapse free; two had had possible attacks. The median number of attacks per year fell from three before treatment (range two to four) to zero (zero to one) during treatment (p<0·0001). No patient had worsened disability by any outcome measure. Median score on the expanded disability status scale improved from 4·3 (range 1·0-8·0) before treatment to 3·5 (0-8·0) during treatment (p=0·0078). Two patients improved by two points and three improved by one point on the Hauser score; no change was recorded for the other patients. Visual acuity had improved in at least one eye by one point in four patients, and by two points in one patient; no change was recorded for other patients. One patient had meningococcal sepsis and sterile meningitis about 2 months after the first eculizumab infusion, but resumed treatment after full recovery. No other drug-related serious adverse events occurred. Eight attacks in five patients were reported within 12 months of eculizumab withdrawal. Eculizumab seems to be well tolerated, significantly reduce attack frequency, and stabilise or improve neurological disability measures in patients with aggressive neuromyelitis optica spectrum disorders. The apparent effects of eculizumab deserve further investigation in larger, randomised controlled studies. Alexion Pharmaceuticals.

Rilonacept for Colchicine-Resistant or -Intolerant Familial Mediterranean Fever

Currently, there is no proven alternative therapy for patients with familial Mediterranean fever (FMF) that is resistant to or intolerant of colchicine. Interleukin-1 is a key proinflammatory cytokine in FMF. To assess the efficacy and safety of rilonacept, an interleukin-1 decoy receptor, in treating patients with colchicine-resistant or -intolerant FMF. Randomized, double-blind, single-participant alternating treatment study. (ClinicalTrials.gov number: NCT00582907). 6 U.S. sites. Patients with FMF aged 4 years or older with 1 or more attacks per month. One of 4 treatment sequences that each included two 3-month courses of rilonacept, 2.2 mg/kg (maximum, 160 mg) by weekly subcutaneous injection, and two 3-month courses of placebo. Differences in the frequency of FMF attacks and adverse events between rilonacept and placebo. 8 males and 6 females with a mean age of 24.4 years (SD, 11.8) were randomly assigned. Among 12 participants who completed 2 or more treatment courses, the rilonacept-placebo attack risk ratio was 0.59 (SD, 0.12) (equal-tail 95% credible interval, 0.39 to 0.85). The median number of attacks per month was 0.77 (0.18 and 1.20 attacks in the first and third quartiles, respectively) with rilonacept versus 2.00 (0.90 and 2.40, respectively) with placebo (median difference, -1.74 [95% CI, -3.4 to -0.1]; P = 0.027). There were more treatment courses of rilonacept without attacks (29% vs. 0%; P = 0.004) and with a decrease in attacks of greater than 50% compared with the baseline rate during screening (75% vs. 35%; P = 0.006) than with placebo. However, the duration of attacks did not differ between placebo and rilonacept (median difference, 1.2 days [-0.5 and 2.4 days in the first and third quartiles, respectively]; P = 0.32). Injection site reactions were more frequent with rilonacept (median difference, 0 events per patient treatment month [medians of -4 and 0 in the first and third quartiles, respectively]; P = 0.047), but no differences were seen in other adverse events. Small sample size, heterogeneity of FMF mutations, age, and participant indication (colchicine resistance or intolerance) were study limitations. Rilonacept reduces the frequency of FMF attacks and seems to be a treatment option for patients with colchicine-resistant or -intolerant FMF. U.S. Food and Drug Administration, Office of Orphan Products Development.

Sodium Oxybate in Narcolepsy with Cataplexy: Zurich Sleep Center Experience

Sodium oxybate (SO; Xyrem®) has been approved in most countries for treatment of narcolepsy and cataplexy. In this study, we present a single-center experience of a series of 18 patients with narcolepsy with cataplexy (18/18 DQB1*0602 positive, 17/17 with low/absent cerebrospinal fluid hypocretin) in whom SO was prescribed. After 26 ± 13 months, 13/18 patients were still on SO at a mean dosage of 6.1 ± 1.2 g (in 8 of them in combination with stimulants). The following significant effects were observed: improved subjective sleepiness (12/13), cataplexy (13/13; median number of attacks from 20 to 1/month), hallucinations (8/10) and sleep paralysis (8/8); increase in mean sleep latency on the Maintenance of Wakefulness Test (from 5.5 to 17.4 min) and sleep/rest efficiency on actigraphy (from 61 to 76%); decrease in Epworth Sleepiness Scale score (from 18 to 14), sleep onset REM periods on the Multiple Sleep Latency Test (from 3.6 to 2.4) and errors in the Steer-Clear Test (from 11 to 2%). Five patients discontinued SO because of insufficient compliance (n = 2), lack of efficiency (n = 1) and side effects (n = 1). These data confirm and expand previous reports on the good effects and tolerability of SO as a treatment for narcolepsy with cataplexy.

C1‐inhibitor concentrate for individual replacement therapy in patients with severe hereditary angioedema refractory to danazol prophylaxis

Hereditary angioedema (HAE) caused by functional deficiency of C1-inhibitor (C1-INH) is a rare disease that manifests with recurrent spontaneous nonallergic edema of the subcutaneous tissues and mucous membranes. In cases of laryngeal edema that are not treated immediately, HAE is associated with high mortality rates. Attenuated androgens (e.g., danazol) are usually administered for prophylaxis, but associated side effects may limit their use. This study investigated the efficacy, safety, and quality of life (QoL) associated with a pasteurized plasma-derived C1-inhibitor (pC1-INH) concentrate for individual replacement therapy (IRT) in patients with severe HAE suffering from frequent attacks who were intolerant or not responding to danazol. Twenty-two patients with severe HAE and danazol incompatibility or insufficient efficacy of danazol were recruited. Intraindividual comparisons of efficacy, safety, and QoL with pC1-INH concentrate IRT versus danazol treatment were made using retrospective and prospective patient data. Pharmacokinetic data were collected for 15 of the 22 patients. In patients receiving pC1-INH regularly, the median number of attacks per year decreased significantly compared to danazol prophylaxis (p < 0.001), and the 24 laryngeal edema episodes per year ceased. Superior efficacy of pC1-INH was found for all QoL variables (e.g., general condition, social activities). No transmission of human immunodeficiency virus or hepatitis A, B, or C was observed. In patients with severe HAE who experience severe side effects and/or lack of efficacy of danazol prophylaxis, very early substitution with pC1-INH can completely abolish the incidence of potentially fatal laryngeal edema and can reduce the incidence of acute attacks.

LHRH Analogue Treatment for the Prevention of Premenstrual Attacks of Acute Porphyria

We have assessed the value of suppressing ovulation with the luteinizing hormone releasing hormone (LHRH) analogue buserelin in seven patients experiencing crises of acute intermittent porphyria related to the menstrual cycle. Clinical course, plasma oestradiol and progesterone levels, and urinary porphyrin and precursor excretion were monitored over a baseline period of approximately one year, and then for a similar period on buserelin treatment. There was a trend towards clinical improvement on buserelin therapy. The median number of attacks fell from seven during the baseline period to three on treatment (p = 0.06). The response to buserelin varied considerably, with those patients in whom the association between baseline attacks and the menstrual cycle was strongest gaining the most benefit. All patients became amenorrhoeic with suppression of plasma oestradiol and progesterone levels. Urinary delta-aminolaevulinic acid and total porphyrin excretion fluctuated widely both before and during treatment. Our experience indicates that ovulation suppression may be of value in the management of young women in whom recurrent attacks of porphyria are related to the menstrual cycle.