Low-dose methotrexate in the treatment of severe juvenile rheumatoid arthritis and sarcoid iritis.

OBJECTIVE: To assess the efficacy of low-dose oral methotrexate (MTX) therapy for children with severe iritis. METHODS: MTX in a weekly dose of 7.25 to 12.5 mg/m2 was administered orally to four patients (two with juvenile rheumatoid arthritis [JRA] and two with sarcoidosis) with severe iritis not adequately controlled by topical and systemic corticosteroid therapy. The treatment was initiated with half of the total dose and increased every 2 weeks until the final dose was reached. Iritis was graded from 0 to +4 according to the density of cells in the anterior chamber of the eye. RESULTS: There were three girls and one boy with a mean age of 10.5 years. Two patients were African American and two were Caucasian. The mean age at onset of iritis was 6 years. The mean duration of MTX therapy was 28.8 months. Significant improvement was noted in two of the four patients in ocular inflammation, demonstrated by reduction of cell density from +4 to +1. Two patients had a mild improvement of the iritis. However, corticosteroids were significantly reduced in all patients. One patient was completely off steroids within 30 months of MTX therapy. In the remaining three cases, the steroid dose was successfully tapered from 0.82 mg/kg/d to 0.15 mg/kg/d (mean doses) within a mean duration of 20 months. No side effects were observed with MTX therapy. CONCLUSION: Low-dose MTX therapy was effective and safe, and displayed steroid-sparing properties in four children with severe iritis.     

Oral megadose methylprednisolone therapy for refractory Diamond-Blackfan anemia. International Diamond-Blackfan Anemia Study Group

PURPOSE: Several reports describe children with refractory transfusion-dependent Diamond-Blackfan anemia who responded to extremely large doses of methylprednisolone. Limitations in available data prompted further exploration of this treatment approach. PATIENTS AND METHODS: This prospective treatment protocol was designed to test the efficacy and toxicity of oral megadose methylprednisolone in children with Diamond-Blackfan anemia who had previously failed to respond to standard doses of prednisone and who were dependent on regular packed red blood cell transfusions. Patients were treated with oral methylprednisolone, starting at a dose of 100 mg/kg per day, tapering slowly to 5 mg/kg per day at the end of 4 weeks and to 2 mg/kg per day after 7 weeks of induction therapy. Therapy was continued for a total of 23 weeks. Efficacy was assessed by increase in peripheral blood reticulocytes and increase or stabilization in hemoglobin concentration, which was maintained during and after steroid tapering. RESULTS: Nine children with Diamond-Blackfan anemia were registered on the study, and all were evaluable. Disease in four of the children failed to respond to megadose methylprednisolone therapy. The other five patients demonstrated a partial or complete response during the initial 4 to 8 weeks of therapy, but all subsequently experienced relapse during the ensuing 2 months as the corticosteroid dose was tapered. All patients required resumption of transfusions, although one later remitted spontaneously. Toxicity of megadose methylprednisolone was modest. CONCLUSION: None of nine children with refractory Diamond-Blackfan anemia treated with oral megadose methylprednisolone exhibited a clinically significant response. Alternative therapeutic strategies are required.     

Inhaled corticosteroid therapy is safe in tuberculin-positive asthmatic children

BACKGROUND: Although treatment with oral corticosteroids can cause reactivation of latent Mycobacterium tuberculosis (TB) infection in purified protein derivative (PPD)-positive individuals with no evidence of clinical disease, little is known about the effects of inhaled corticosteroids in this respect. OBJECTIVE: This study was undertaken to assess whether inhaled corticosteroid (CS) therapy reactivates latent TB infection in PPD-positive asthmatic children. METHOD: We studied 32 PPD skin test-positive (> or =10 mm) children [age (mean +/- SD), 7.9 +/- 4.1 years] with no family history and no evidence of TB infection on chest radiograms who were receiving inhaled budesonide for the treatment of asthma. They were further evaluated with thorax computed tomography (CT) and erythrocyte sedimentation rate and closely observed for an additional 9 months. RESULTS: At enrollment the mean diameter of PPD reaction was 12.8 +/- 2.7 mm. The mean duration of inhaled CS treatment and the mean cumulative CS dose were 9.8 +/- 7.6 months and 275 +/-199 mg, respectively. Thorax CT studies revealed mediastinal lymph nodes in 7 of the 32 patients. There was no significant difference between children with and without mediastinal lymph nodes according to age, gender, size of PPD skin testing, erythrocyte sedimentation rate and duration and cumulative CS dose of inhaled budesonide therapy before study. A second thorax CT was obtained 9 months later in those 7 patients with lymphadenopathy (additional mean cumulative CS dose, 222.57 mg). There was no change in the size of their lymph nodes. CONCLUSION: Long term inhaled budesonide therapy appears to be safe in PPD-positive asthmatic children.     

Bone mineral density in prepubertal asthmatics receiving corticosteroid treatment

OBJECTIVE: To examine whether bone mass is reduced in prepubertal, asthmatics receiving high doses of inhaled corticosteroids. METHODOLOGY: A cross-sectional comparison of lumbar spine-bone mineral density (LS-BMD) was undertaken in 76 subjects after stratifying them according to dosage and administration route of corticosteroid. RESULTS: Weight was the only independent predictor of LS-BMD (r(2) = 0.38). Children receiving greater than 800 microg/day of inhaled corticosteroid plus intermittent oral corticosteroid had a significantly lower weight-adjusted LS-BMD than children treated with 400-800 microg/day of inhaled corticosteroid (mean difference: 0.06 g/cm(2), 95% confidence interval (CI): - 0.02 to - 0.10). A significant difference in weight-adjusted LS-BMD persisted when all children receiving greater than 800 microg/day of inhaled corticosteroid, irrespective of additional oral corticosteroid treatment, were compared with children receiving 400-800 microg/day of inhaled corticosteroid (mean difference: - 0.05 g/cm(2), 95%CI interval: -0.02 to - 0.09). Bone mass was similar in children not receiving any inhaled corticosteroid and those treated with 400-800 microg/day of inhaled corticosteroid. CONCLUSIONS: A reduced bone mass in prepubertal asthmatic children receiving high doses of inhaled corticosteroids may predetermine a compromised peak bone mass and increase osteoporotic fracture risk in adulthood.     

小剂量茶碱配合吸入皮质激素治疗小儿哮喘的疗效评价

目的研究小剂量茶碱与皮质激素治疗小儿哮喘的协同抗炎作用。方法选择80例中度小儿哮喘,随机分为治疗组、对照组(各40例),治疗组口服小剂量茶碱控释片(或胶囊)配合吸入必酮碟,对照组只吸入必酮碟。结果治疗组在皮质激素较快减量过程中的峰值流速(PEFR)始终在预计值的80％以上,皮质激素的维持量明显较对照组小。结论小剂量茶碱配合吸入皮质激素,可使皮质激素减量过程快,维持剂量小,茶碱的不良反应少,不必强调血药浓度的监测。     

Short-term corticosteroids for celiac crisis in infants

We report two infants with celiac crisis who continued to have persistent secretory diarrhea despite gluten and lactose free diet and supportive parenteral nutrition. The children were given corticosteroid therapy. After a five-day oral prednisone in the dose of 2 mg/kg/daily, both patients rapidly recovered.     

Effectiveness of inhaled corticosteroids in controlling acute asthma exacerbations in children at home

Many clinicians advise their patients to increase the dose of inhaled corticosteroids during acute asthma exacerbations, without strong clinical evidence supporting this treatment. This study investigates the effectiveness of inhaled corticosteroids in controlling acute asthma exacerbations in children at home. The study population consisted of children with mild intermittent, mild and moderate persistent asthma aged 1 to 14 years who were treated in our outpatient clinic with inhaled budesonide for 1 year. After participating in an asthma education session, the parents were instructed to initiate treatment with inhaled budesonide at the first signs of asthma exacerbation, starting with 200 to 400 microg budesonide, in combination with beta-2 agonists 4 times a day and followed by a decrease in the dose in 4 to 8 days. Asthma status and peak expiratory flow rates were measured in the 3 monthly follow-up visits. Only children who complied with the treatment regimen and came for follow-up visits regularly were included in the final analysis. One hundred fifty children used our treatment protocol with inhaled budesonide to control their asthma attacks. Clinical improvement of asthma symptoms was achieved after a mean of 1.8 +/- 0.7 days from the beginning of treatment. The parents were able to control 94% of the 1,061 episodes of asthma exacerbation occurring during a cumulative follow-up period of 239 years. In the 3-month period before enrollment, 101 children (67%) had used oral corticosteroids to control their asthma attacks and 50 (33%) were hospitalized. During the entire follow-up period, only 11 children (7%) used oral corticosteroids, and none of the children were hospitalized. The present study demonstrates that children with asthma can control their exacerbations at home using inhaled corticosteroids (budesonide). Treatment, starting with relatively high doses followed by a rapid reduction in dose over 4-8 days, resulted in a decrease in the use of oral steroids and in hospitalization. To achieve good results, patient compliance is essential.     

Pyridoxal phosphate is better than pyridoxine for controlling idiopathic intractable epilepsy.

AIM: To study the difference between pyridoxine (PN) and its active form, pyridoxal phosphate, (PLP) in control of idiopathic intractable epilepsy in children. METHODS: Among 574 children with active epilepsy, 94 (aged 8 months to 15 years) were diagnosed with idiopathic intractable epilepsy for more than six months. All received intravenous PLP 10 mg/kg, then 10 mg/kg/day in four divided doses. If seizures recurred within 24 hours, another dose of 40 mg/kg was given, followed by 50 mg/kg/day in four divided doses. For those patients whose seizures were totally controlled, PLP was replaced by the same dose of oral PN. If the seizure recurred, intravenous PLP was infused followed by oral PLP 50 mg/kg/day. RESULTS: Fifty seven patients had generalised seizures (of whom 13 had infantile spasms) and 37 had focal seizure. Eleven had dramatic and sustained responses to PLP; of these, five also responded to PN. Within six months of treatment with PLP or PN, five of the 11 patients were seizure free and had their previous antiepileptic medicine tapered off gradually. Two were controlled with pyridoxine and the other three needed PLP to maintain seizure freedom. The remaining six responders needed PLP exclusively for seizure control. Six of the 11 responders to PLP had infantile spasms (46%); four of them needed PLP exclusively. The other five responders were in the remaining 81 patients with other seizure type. CONCLUSIONS: PLP could replace PN in the treatment of intractable childhood epilepsy, particularly in the treatment of infantile spasms.     

Pyridoxal phosphate is better than pyridoxine for controlling idiopathic intractable epilepsy

Aim: To study the difference between pyridoxine (PN) and its active form, pyridoxal phosphate, (PLP) in control of idiopathic intractable epilepsy in children. Methods: Among 574 children with active epilepsy, 94 (aged 8 months to 15 years) were diagnosed with idiopathic intractable epilepsy for more than six months. All received intravenous PLP 10 mg/kg, then 10 mg/kg/day in four divided doses. If seizures recurred within 24 hours, another dose of 40 mg/kg was given, followed by 50 mg/kg/day in four divided doses. For those patients whose seizures were totally controlled, PLP was replaced by the same dose of oral PN. If the seizure recurred, intravenous PLP was infused followed by oral PLP 50 mg/kg/day. Results: Fifty seven patients had generalised seizures (of whom 13 had infantile spasms) and 37 had focal seizure. Eleven had dramatic and sustained responses to PLP; of these, five also responded to PN. Within six months of treatment with PLP or PN, five of the 11 patients were seizure free and had their previous antiepileptic medicine tapered off gradually. Two were controlled with pyridoxine and the other three needed PLP to maintain seizure freedom. The remaining six responders needed PLP exclusively for seizure control. Six of the 11 responders to PLP had infantile spasms (46%); four of them needed PLP exclusively. The other five responders were in the remaining 81 patients with other seizure type. Conclusions: PLP could replace PN in the treatment of intractable childhood epilepsy, particularly in the treatment of infantile spasms.     

吸入糖皮质激素对哮喘儿童骨龄及生长发育的影响

目的：长期吸入糖皮质激素是哮喘的首选治疗方法,但国内外对于年幼儿童长期吸入糖皮质激素治疗的安全性仍有争议,本研究旨在探讨支气管哮喘患儿吸入糖皮质激素对骨龄及生长发育的影响。方法：73例支气管哮喘患儿给予丙酸氟替卡松吸入治疗,剂量250 μg/d,3个月后减量1/3续用3个月,再减为125 μg/d续用6个月,治疗后观察疗效,治疗前后分别测量骨龄、身高、体重。结果：入选患儿治疗后身高、体重及RUS骨龄增长与正常儿童差异无统计学意义（P>0.05）;体重指数(BMI)治疗前后差异无统计学意义（P>0.05）;治疗前后C骨龄与年龄差值分别为-0.2（-0.6,0.8）岁、-0.5（-1.0、0.6)岁,治疗后明显比治疗前延迟（P<0.05）。结论：哮喘患儿吸入糖皮质激素治疗 1年对C骨龄发育有一定抑制作用,对RUS骨龄、身高、体重及BMI无明显影响;长期糖皮质激素治疗的患儿应监测生长发育状况。     

尘螨标准化免疫治疗85例哮喘儿童疗效相关指标分析

目的 观察哮喘特异性免疫治疗(Specific immunotherapy,SIT)过程中变应原注射时间、变应原累积注射量及糖皮质激素吸入剂量、最高呼气流量、总IgE、螨特异性IgE等的变化.方法 根据纳入标准选择2005年2月-2008年6月我院哮喘免疫治疗中心就诊的过敏性哮喘患儿,进行同期对照回顾性分析,其中治疗组接受尘螨标准化变应原特异性免疫治疗,以每4次变应原注射为观察点,历时3.4年,观察治疗时间、变应原累积注射量、糖皮质激素吸入量、最高呼气流量变化、总IgE和螨特异性IgE变化,对照组仅行药物治疗,比较两组糖皮质激素吸入量及哮喘发作情况,并在SIT结束时评估疗效.结果 SIT组85例,男45例,女40例,年龄(7.6±1.4)岁,对照组病例50例,单纯药物治疗,男28例,女22例,年龄(7.7±1.5)岁.SIT第20次注射、治疗(38.7±2.4)周时,变应原累积注射量达(69.7±4.8)μg,糖皮质激素吸入量明显少于对照组(t=2.359,P＜0.05);SIT组最高呼气流量较治疗前升高,有统计学意义(F=7.874,P＜0.05);SIT前后总IgE变化无统计学意义(t=0.313,P＞0.05),螨特异性IgE变化无统计学意义(tDerp=0.517,tDerf=0.717,P＞0.05);SIT组疗程结束时患儿家长对病情主观评价:45.9%表示明显好转,36.5%表示中度好转,16.5%表示稍有好转,1.1%表示无改变;两组患儿在治疗结束时进行疗效评估,SIT组控制率达85.5%,对照组为62.0%,两组比较差异有统计学意义(x2=10.150,P＜0.01),提示SIT有效..结论 哮喘特异性免疫治疗第20次注射后,螨过敏性哮喘患儿糖皮质激素吸入剂量显著减少;85%以上患儿在SIT结束时哮喘得到控制;总IgE和螨特异性IgE变化无统计学意义.     

Progressive diaphyseal dysplasia: evaluation of corticosteroid therapy

Progressive diaphyseal dysplasia is characterized clinically by crippling leg pain, fatigue, headache, poor appetite, muscle weakness, and waddling gait. Twelve affected patients, aged 2 years 4 months to 40 years, were treated with intermittent courses of low doses of prednisone given in a single dose on alternate mornings for periods ranging from 6 months to 10 years. The average initial dose of prednisone was 0.6 mg/kg/d, and average maintenance dose was 0.3 mg/kg/d. Relief of all crippling symptoms was achieved in all patients. No untoward serious side effects have been observed, and the growth of children was not slowed. However, corticosteroid therapy should be restricted to patients suffering from crippling pain. The mechanism through which steroids act remains undefined.     

Cataract and ocular hypertension in children on inhaled corticosteroid therapy

PURPOSE: To ascertain the incidence of posterior subcapsular cataract and ocular hypertension in a cohort of children < or = 12 years on inhaled steroid therapy. PATIENTS AND METHODS: In this prospective study, a detailed history regarding corticosteroid therapy was obtained for children attending an asthma clinic. The presence and type of lens changes (cataract) was recorded and intraocular pressure (IOP) was measured. The children underwent another eye examination 2 years later. RESULTS: Ninety-five patients were enrolled in the study. Mean patient age was 7 +/- 3 years, and mean duration of inhaled steroid therapy was 2 +/- 1 years. Thirty-six percent of patients received inhaled steroids exclusively, 61% received inhaled steroids with a short course of oral steroids, and 3% received inhaled steroids with a long course of oral steroids. Only 3 (3%) patients had cortical changes that were not visually significant, and none had posterior subcapsular or nuclear cataract. There was no significant differences between children with cataract and those without cataract with respect to age; duration of asthma; and duration, average daily dose, and cumulative dose of inhaled steroids. IOP ranged from 11 to 20 mm Hg (mean, 16 +/- 3 mm Hg). None of the children had ocular hypertension or glaucoma. Ninety patients underwent eye examination 2 years later; none was found to develop posterior subcapsular cataract or increased IOP. CONCLUSION: This study indicates the use of inhaled steroids in children with asthma is probably safe as far as not inducing posterior subcapsular cataract or ocular hypertension.     

Intravenous cyclophosphamide is the drug of choice for steroid dependent nephrotic syndrome

BACKGROUND: Steroid dependency is a major problem seen after therapy for idiopathic nephrotic syndrome in childhood. Although there is consensus about the usage of cyclophosphamide (CYC) in frequent relapsers, there is still a controversy concerning its usage in steroid-dependent nephrotic syndrome (SDNS). METHODS: In the present study, nineteen children with SDNS were treated with CYC: ten via the intravenous (i.v.) route, and nine via the oral route. Remission was then maintained with prednisolone. Oral CYC therapy consisted of CYC at a dose of 2 mg/kg per day for 12 weeks. Intravenous (i.v.) CYC therapy consisted of CYC 500 mg/m2 per month (with intravenous 3500 cc/m2 per 24 h one-third saline hydration) for 6 months. RESULTS: The cumulative dose of CYC was 168 mg/kg in the oral group and 132 mg/kg in the IV group. Daily oral CYC dose was 1.96~0.31 mg/kg, whereas i.v. CYC dose was 0.73~0.03 mg/kg. Long-term complications and side-effects such as alopecia, infection and hemorrhagic cystitis were not observed in the i.v. CYC treated group. In the long term, the dosage of prednisolone that held remission after CYC, the annualized relapse rates and the subsequent relapse time were significantly better in the i.v. CYC group, and the number of patients in remission for 2 years was significantly higher in the i.v. treated group (P<0.05). CONCLUSIONS: In SDNS, i.v. CYC has a long lasting effect with lower annualized relapse rates and longer subsequent relapse time with a lower steroid dosage required to maintain remission than oral CYC. The results of the present study showed the safety of the i.v. route, and it is the preferable treatment in noncompliant patients for its long lasting remission and simple and inexpensive follow up.     

Fluticasone propionate in asthma: a long term dose comparison study.

BACKGROUND: Few dose ranging studies have investigated optimal dosing with inhaled corticosteroids in children with asthma. AIMS: To compare the efficacy and tolerability of fluticasone propionate 100 or 200 microg twice daily in children with moderate to severe asthma for one year. METHODS: One year, randomised, double blind, parallel group, multicentre study. Children aged 4-11 years (n = 528) with moderate to severe asthma who had previously received high dose inhaled corticosteroids were given fluticasone propionate 100 or 200 microg twice daily for the 52 week treatment period. Efficacy (exacerbations, lung function, and symptoms) and tolerability (adverse events and cortisol levels) were measured. RESULTS: There was a non-significant decreased risk of experiencing an exacerbation at any time with fluticasone propionate 200 microg twice daily compared with fluticasone propionate 100 microg twice daily. This difference reached significance among patients with more severe asthma (defined by previous inhaled corticosteroid dose >800 microg/day). Daily record card morning peak expiratory flow (PEF) in the total population improved significantly more with the higher dose of fluticasone propionate (between group difference, weeks 1-52: 11.4 l/min). Clinic visit mean PEF improved from baseline with both doses, but the response was significantly greater with the higher dose (between group difference, week 52: 17.8 l/min). Both doses were equally well tolerated and overnight urinary cortisol concentrations were unchanged or slightly increased during treatment with either dose. CONCLUSION: This long term dose comparison study shows that treatment with fluticasone propionate 200 micro g twice daily may offer benefits over a lower dose, particularly in children with more severe asthma.     

Cushing syndrome with secondary adrenal insufficiency from concomitant therapy with ritonavir and fluticasone

We present 2 cases of Cushing syndrome with secondary adrenal insufficiency from concomitant use of ritonavir and inhaled corticosteroids in children with human immunodeficiency virus infection. These cases highlight the need for special consideration when treatment with an inhaled/intranasal corticosteroid is indicated in children receiving antiretroviral therapy.     

Chronic inflammatory demyelinating polyneuropathy in children: follow-up investigation and results of prednisone-azathioprine treatment

We collected 20 children with chronic dysimmune polyneuropathy (median age at onset 7.5 years) and performed a follow-up investigation after a median of 12 years. At onset all had areflexia; cerebrospinal fluid total protein was increased in 17 (85%) of 20. Nerve conduction velocity was pathological in 17 of 18 and sural nerve biopsy in nine showed evidences of de- and remyelinization in eight. An underlying subclinical hereditary polyneuropathy was indicated in one parent in 11 of 14 re-examined children. Onset was preceded by an infection or vaccination in 12 (60%) of 20. The initial impairment was often impressive and one patient was treated on a ventilator.Treatment principles included induction with prednisone 1–2 mg/kg per day for 6 weeks, tapered off during 3–4 weeks and maintenance with azathioprine 2–3 mg/kg per day for 2 years. Accordingly, 17 were given corticosteroids and 15 also azathioprine. Seven (35%) had a monophasic and 13 (65%) a relapsing-remitting course with 35 relapses (mean 1.7 per affected patient in the whole and 2.8 in the relapsing group). The relapses tended to become successively shorter and milder. The group with combined short-term corticosteroid and long-term azathioprine treatment had fewer relapses (1.6 per patient) than the group with corticosteroids and short-term azathioprine (2.3 per patient).At follow-up two of three in the non-treated group, with generally more benign courses, had no remaining handicap, three of six (50%) treated with short-term prednisone and short-term azathioprine, and eight of 11 (73%) given short-term corticosteroids and long-term azathioprine had no remaining handicap according to the WHO handicap score. Strength was most affected in hand, hip and foot muscles. Corticosteroid dependency and long-term side-effects were avoided in all. We consider the outcome encouraging.     

HIGH-DOSE ORAL METHYLPREDNISOLONE THERAPY IN CHILDHOOD HEMANGIOMAS

The authors report their experience with high-dose oral methylprednisolone therapy (HDMP)in 15 infants with complicated hemangiomas. The starting dose for methylprednisolone was 30 mg/kg/day for 5 days, then the dose was tapered gradually every 5 days to 20, 10, 5, 2.5, and finally to 1 mg/kg/day. Therapy was then stopped and the patients were followed. An initial response was evident in 12 patients. Nine out of 12 responders showed regrowth signs. After regrowth, 4 cases received prednisolone at doses between 1 to 5 mg/kg/day and 3 patients received a second course with HDMP as additional corticosteroid therapy. Overall, 9 out of 15 cases were responders; very good and good responses were obtained in 5, partial response in 4, and therapy failure in 5 cases. One child was not available for evaluation of response. A very rapid initial response was observed in subglottic and periocular hemangiomas. Side effects were not serious and resolved after discontinuation of treatment. Although the number of patients is small in this study, overall response rate with HDMP regimen seems not to be superior to the regimens that use lower doses (5 mg/kg/day), but it provides a high initial response rate and the duration of therapy is short. Therefore, it may be useful for treating hemangiomas that fail to respond with low doses, especially in centers with limited resources where other treatment modalities cannot be used at the moment.     

HIGH-DOSE ORAL METHYLPREDNISOLONE THERAPY IN CHILDHOOD HEMANGIOMAS

The authors report their experience with high-dose oral methylprednisolone therapy (HDMP)in 15 infants with complicated hemangiomas. The starting dose for methylprednisolone was 30 mg/kg/day for 5 days, then the dose was tapered gradually every 5 days to 20, 10, 5, 2.5, and finally to 1 mg/kg/day. Therapy was then stopped and the patients were followed. An initial response was evident in 12 patients. Nine out of 12 responders showed regrowth signs. After regrowth, 4 cases received prednisolone at doses between 1 to 5 mg/kg/day and 3 patients received a second course with HDMP as additional corticosteroid therapy. Overall, 9 out of 15 cases were responders; very good and good responses were obtained in 5, partial response in 4, and therapy failure in 5 cases. One child was not available for evaluation of response. A very rapid initial response was observed in subglottic and periocular hemangiomas. Side effects were not serious and resolved after discontinuation of treatment. Although the number of patients is small in this study, overall response rate with HDMP regimen seems not to be superior to the regimens that use lower doses (5 mg/kg/day), but it provides a high initial response rate and the duration of therapy is short. Therefore, it may be useful for treating hemangiomas that fail to respond with low doses, especially in centers with limited resources where other treatment modalities cannot be used at the moment.     

Azathioprine in the treatment of children with inflammatory bowel disease

During a 6-year period, we treated 21 patients with azathioprine, 2 mg/kg/day, as an adjunct to their customary regimen. Nine patients had ulcerative colitis and 12 patients had Crohn disease; the patients' ages ranged from 3 to 17 years. The median duration of disease before the start of azathioprine therapy was 2 years, and median follow-up was 2 years. Sixteen patients seemed to respond to azathioprine therapy: six patients in each disease group had complete responses and four patients (one with ulcerative colitis and three with Crohn disease) had partial responses. Two patients with ulcerative colitis and three patients with Crohn disease did not respond. The median time until patients responded was less than 3 months for patients with ulcerative colitis and 4 months for those with Crohn disease. Reduction of corticosteroid dose was possible for all patients who responded to azathioprine therapy. Only minimal side effects were attributable to the drug. We conclude that azathioprine is an effective adjunctive agent for the treatment of inflammatory bowel disease in childhood, but because questions remain regarding its long-term safety, its use should be reserved for children with refractory disease or severe and unacceptable side effects of corticosteroids.