Tolmetin and salicylate therapy in acute rheumatic fever: Comparison of clinical efficacy and side-effects

BACKGROUND: The arthritis of rheumatic fever is very responsive to treatment with salicylates, but there are many adverse reactions, especially hepatotoxicity, due to aspirin (acetylsalicylic acid) therapy. These side-effects change the course and duration of rheumatic fever. Other non-steroidal anti-inflammatory drugs may be equally effective, although no reports are available. METHODS: We studied 72 patients with rheumatic fever who were admitted to Dr Sami Ulus Children's Hospital between 1995 and 1999. Twenty patients with arthritis were treated with tolmetin (25 mg/kg per day; group I) and 52 patients with arthritis and/or mild carditis were put on aspirin therapy (75-100 mg/kg per day) for 4-6 weeks (group II). Arthritis had disappeared at the same time in both the aspirin and tolmetin groups (P = 0.675). RESULTS: The erythrocyte sedimentation rates of patients upon admission, at the first week and at the end of therapy were not different in the two groups (P > 0.05). No adverse effect of tolmetin therapy was observed, whereas side-effects of salicylate were observed in 19 patients (36.5%) in the aspirin group. Hepatotoxicity, gastric irritation and salicylism were found in 16, four and three patients, respectively. Renal toxicity and Reye syndrome were not demonstrated. Because of these side-effects of aspirin, therapy had to be stopped for 10-20 days and the duration of hospitalization in this group was lengthened unnecessarily. CONCLUSION: Tolmetin was safe and effective treatment for arthritic rheumatic fever patients without carditis. Tolmetin can be used particularly in patients who cannot tolerate aspirin.     

Acute rheumatic fever in the young: changing prevalence and pattern

Acute rheumatic fever (ARF) is rare in young people. A group of 28 patients is described who were hospitalized during the years 1960–1984. Patients were divided into two groups: children who were admitted between 1960 and 1966 and those admitted between 1966 and 1984. The annual incidence of ARF declined from 1.17 to 0.44 cases per 10,000 population at risk. The incidence of arthritis declined in parallel to the decrease in number of children presenting with ARF, whereas the incidence of carditis remained the same.Abbreviations ARF acute rheumatic fever - RF rheumatic fever     

Rheumatic fever and rheumatic heart disease: Primary prevention is the cost effective option

Objective To measure the economic output/input ratios for the various options of prevention of rheumatic fever/rheumatic heart disease (RF/RHD) and check the viability of primary prevention vis-à-vis secondary and tertiary preventions. Methods Cost accounting of the various prevention options was calculated for each variable as available in literature. Actual data as obtainable for the financial year ending March 2006 were computed for the Pondicherry population. Both direct and indirect costs (including community/social costs) were worked out using mostly primary data, and wherever necessary, secondary data. Certain scientific assumptions were used where exact data was not available. Results Primary prevention is the definite viable economic option (1:1.56) compared to secondary (1: 1.07) and tertiary (1: 0.12) preventions. In fact, the current stress on only secondary and tertiary preventions is found to be economically unviable. Conclusion It is postulated that primary prevention as a practical policy in tackling RF and RHD can be recommended.     

Non‐specific host response markers in the differentiation between pneumococcal and viral pneumonia: What is the most accurate combination?

BACKGROUND: Serum C-reactive protein (CRP), blood white cell count (WBC), serum procalcitonin (PCT) and erythrocyte sedimentation rate (ESR) were measured in 132 children hospitalized for community-acquired pneumonia. Serological evidence for viral infection was found in 38 cases and for pneumococcal infection in 41 cases, and the infiltrate was alveolar in 46 cases and interstitial in 86 cases. The aim of the present paper was to determine if there is a combination of these four host response markers and chest radiograph findings suitable for differentiating pneumococcal from viral etiology of pneumonia. METHODS: The 50th, 75th and 90th percentiles of CRP, WBC, ESR and PCT in the total group of 132 patients were calculated. By using these cut-off limits, the likelihood ratios of a positive test result were calculated for the possible combinations of CRP, WBC, ESR and PCT, and the likelihood ratio was 1.50 or more for six combinations. RESULTS: The highest likelihood ratio (1.74) was achieved with the combination CRP > 90th (80 mg/L) or WBC > 75th (17.0 x 10(9)/L) or PCT > 75th (0.84 microg/L) or ESR > 90th (63 mm/h) percentile. For this combination, the sensitivity was 61% and the specificity 65%. When the 90th percentile cut-off limit was applied also for WBC (>22 x 10(9)/L) and PCT (>1.8 microg/L), the specificity increased to 76%, but the sensitivity decreased to 37%. When the presence of an alveolar infiltration was included in the combination, the likelihood ratio was 1.89; the specificity was as high as 82% and the sensitivity as low as 34%. CONCLUSIONS: CRP, PCT, WBC and ESR have only limited value in differentiating pneumococcal or other bacterial pneumonia from viral pneumonia. If there was a high value in at least one of the markers (CRP > 80 mg/L, PCT > 1.8 microg/L, WBC > 22 x 10(9)/L or ESR > 60 mm/h), viral infections were rare. There was no combination of these markers which was sufficiently sensitive and specific to be used in clinical pediatric practice.     

神经元蜡样脂褐质沉积症3例临床及基因变异分析

目的探讨神经元蜡样脂褐质沉积症(NCL)的临床和基因变异特征。方法回顾分析3例NCL患儿的临床资料和基因检测结果。结果 3例女性患儿,多表现为认知和运动倒退、不同程度的癫痫发作、视力受累。全外显子测序发现,例1的PPT1基因存在c.124+1GA及c.413CT复合杂合变异,其中c.124+1GA遗传自父亲,c.413CT遗传自母亲;例2及其哥哥PPT1基因存在c.181CT及c.536+1GA复合杂合变异,其中c.181CT遗传自父亲,c.536+1GA遗传自母亲;例3的CLN8基因存在c.768GT及c.209GT复合杂合变异,其中c.768GT遗传自父亲,c.209GT遗传自母亲。c.768GT和c.209GT是既往未见报道的新的变异位点。结论基因检测有助NCL的诊断及遗传咨询;PPT1基因变异可呈现不同的临床表现,即使是同一家系具有相同变异位点的个体临床表现也各不相同。     

Naproxen as an alternative to aspirin for the treatment of arthritis of rheumatic fever: a randomized trial

We performed a prospective, randomized, open-label equivalence study comparing the use of naproxen to aspirin in 33 patients with rheumatic fever. The mean time until resolution of arthritis was 2.9+/-2.9 days in both groups. Liver enzyme elevations were more frequent in the aspirin group (P=.002). We conclude that naproxen is as effective, is easier to use, and is safer than aspirin in the treatment of the arthritis of rheumatic fever.     

川崎病治疗前发热时间与丙种球蛋白耐药的相关性临床研究北大核心CSCD

目的 探讨川崎病(Kawasaki disease,KD)患儿丙种球蛋白(intravenous immunoglobulin,IVIG)治疗前发热时间与IVIG耐药的关系。方法 回顾性收集2018年1月至2020年12月收治KD患儿317例的病例资料,根据IVIG治疗前发热时间分为短热程组(发热时间≤4 d,n=92)和长热程组(发热时间>4 d,n=225),根据是否发生IVIG耐药将每组再分为耐药组和非耐药组。分析比较不同热程耐药组及非耐药组的基线资料及实验室结果,并采用多因素logistic回归分析IVIG耐药的影响因素。结果 短热程组中IVIG耐药19例(20.7%),并发冠状动脉瘤5例(5.4%);长热程组中IVIG耐药22例(9.8%),并发冠状动脉瘤19例(8.4%);短热程组IVIG耐药率明显高于长热程组(P<0.05),而冠状动脉瘤发生率在两组间差异无统计学意义(P>0.05)。短热程组中,耐药患儿治疗前血钠水平低于非耐药患儿,而降钙素原、C反应蛋白及N末端B型利钠肽原水平则明显高于非耐药患儿(P<0.05)。长热程组中,耐药患儿治疗前血钠及肌酸激酶水平低于非耐药患儿(P<0.05)。多因素logistic回归分析显示,血钠水平降低与长热程组KD患儿IVIG耐药有关(P<0.05)。结论 KD患儿IVIG耐药因IVIG治疗前发热时间不同而异。在治疗前发热时间>4 d的KD患儿中,血钠降低与IVIG耐药具有相关性。     

Glycogen storage disease type I: clinical and laboratory profile

ObjectivesTo characterize the clinical, laboratory, and anthropometric profile of a sample of Brazilian patients with glycogen storage disease type I managed at an outpatient referral clinic for inborn errors of metabolism.MethodsThis was a cross-sectional outpatient study based on a convenience sampling strategy. Data on diagnosis, management, anthropometric parameters, and follow-up were assessed.ResultsTwenty-one patients were included (median age 10 years, range 1–25 years), all using uncooked cornstarch therapy. Median age at diagnosis was 7 months (range, 1–132 months), and 19 patients underwent liver biopsy for diagnostic confirmation. Overweight, short stature, hepatomegaly, and liver nodules were present in 16 of 21, four of 21, nine of 14, and three of 14 patients, respectively. A correlation was found between height-for-age and BMI-for-age Z-scores (r = 0.561; p = 0.008).ConclusionsDiagnosis of glycogen storage disease type I is delayed in Brazil. Most patients undergo liver biopsy for diagnostic confirmation, even though the combination of a characteristic clinical presentation and molecular methods can provide a definitive diagnosis in a less invasive manner. Obesity is a side effect of cornstarch therapy, and appears to be associated with growth in these patients.     

Practitioner review: bridging the gap between research and clinical practice in pediatric obesity

BACKGROUND: Pediatric obesity is a significant public health concern, with rising prevalence rates in both developed and developing countries. This is of particular significance given that overweight children and adolescents are at increased risk for multiple medical comorbidities, as well as psychosocial and behavioral difficulties. The current review highlights findings from the empirical pediatric obesity treatment literature, with particular attention to diet, physical activity, and behavior interventions. Evaluation and treatment considerations relevant to working with overweight children and adolescents with psychiatric comorbidities are reviewed. METHODS: Review of the relevant treatment literature, with a focus on randomized clinical trials, was conducted. Recommendations regarding treatment of children and adolescents with psychiatric comorbidities are based on relevant prospective studies of the relationship between weight status and psychological variables and studies with adult populations. RESULTS: Well-established pediatric weight control interventions have been conducted in research settings. These studies provide a starting point, but are limited by homogeneous samples that may exclude participants with psychiatric comorbidities. Practitioners treating obese children and adolescents with psychiatric disorders are encouraged to assess individual, familial, and contextual variables specific to weight (e.g., motivation and existing support to change current eating and physical activity patterns, extent of weight-related conflict within family, impact of weight on current functioning) in order to prioritize treatment objectives. CONCLUSIONS: The review concludes with a discussion of current empirical and practical challenges, including explicitly targeting obese children and adolescents with psychiatric concerns and determining appropriateness of pursuing weight control interventions in this population.     

Autosomal recessive polycystic kidney disease in 115 children: clinical presentation, course and influence of gender

The clinical course of 66 boys and 49 girls with autosomal recessive polycystic kidney disease recruited from departments of paediatric nephrology was investigated over a mean observation period of 4.92 years. This is a selected study group of children from departments of paediatric nephrology who in most cases survived the neonatal period, since birth clinics did not participate. The median age at diagnosis was 29 days (prenatal to 14.5 years). We observed decreased glomerular filtration rates (GFRs) in 72% (median age at onset of decrease of GFR < 2SD, 0.6 years; range. 0-18.7 years), and 11 patients developed end-stage renal disease. Hypertension requiring drug treatment was found in 70% (median age at start of medication, 0.5 years; range, 0-16.7 years). Kidney length was above the 97th centile in 68% of patients, and kidney length did not increase with age or deterioration of renal function. Urinary tract infections occurred in 30%, growth retardation in 25%. and clinical signs of hepatic fibrosis were detected in 46%. Thirteen patients (11 %) died during the observation period. 10 of them in the first year of life. There was a statistically significant sex difference in terms of a more pronounced progression in girls. The survival probability at 1 year was 94% for male patients and 82'% for female patients ( p < 0.05) in this study. Urinary tract infections occurred more frequently in girls ( p < 0.025) and were observed earlier. In addition, more girls had impaired renal function. developed end-stage renal disease and showed growth retardation; these differences, however. were not significant. For the children in this study, however, our results indicate that the long-term prognosis in the majority of cases is better throughout childhood and youth than often stated.     

Developmental dysplasia of the hip: Risk markers, clinical screening and outcome

Background: Early detection, diagnosis and treatment of developmental dysplasia/dislocation of hip (DDH) are essential in preventing further disability and quality of life impairment in children. DDH risk markers and association between the age of clinical screening and outcome, were evaluated.
Methods: Clinical screening at ages birth, 6 and 13 weeks was performed in 8145 infants by pediatricians. Infants suspected for DDH were referred to the community hospital clinic for clinical evaluation by a pediatric orthopedic surgeon, imaging procedures and follow up. Demographic and perinatal characteristics of the children with suspected ( n = 77) and diagnosed DDH ( n = 51) were compared to matched controls ( n = 154).
Results: The rate of suspected DDH was 0.95% and that of diagnosed DDH was 0.63%. Female gender, firstborn child and breech presentation were significantly more frequent among cases versus controls (odds ratio [OR]: 4.3, 2.7, and 6 respectively; P < 0.05). The highest positive predictive value (95.5%) in physical evaluation was any evidence of a dislocatable hip. The proportion of DDH among infants referred from the newborn department was significantly higher (OR, 4.4). DDH diagnosis after 6 weeks of age was associated with a higher likelihood of subsequent surgery and motor disability. Untoward outcome was significantly associated with increasing age of referral both at ages of 6 and 13 weeks ( P < 0.05).
Conclusions: Children with DDH have certain specific demographic and perinatal risk markers. Clinical screening targeted towards early diagnosis may lessen the need for surgical intervention and the risk of disability or motor handicap.     

Water retention in the acute phase of Kawasaki disease: relationship between oedema and the development of coronary arterial lesions

Despite intravenous immunoglobulin therapy, a certain percentage of patients with Kawasaki disease (KD) still develop coronary arterial lesions (CAL). In an effort to find new combined therapies to reduce the incidence of CAL, we focused on the oedema which can be an important sign of the increased vascular permeability in KD. A total of 127 patients with KD were included in the retrospective study. Serial weekly changes in serum sodium and albumin levels from the 1st to the 4th week of illness were examined. In addition, the maximum rate of increase in body weight from admission to the 14th day of illness was evaluated. Serum sodium levels (mEq/l) in only the 2nd week of illness were significantly lower in patients with CAL than in those without CAL (mean ± SD, 135.5±4.5 versus 138.0±2.4, P <0.05). Serum albumin levels in all 4 weeks were significantly lower in patients with CAL than in those without CAL ( P <0.001). The maximum rate (%) of increase in body weight from admission to the 14th day of illness was significantly higher in patients with CAL than in those without CAL (ranges and median values, 0–12.3 (7.0) versus 0–10.3 (3.2), P <0.001). Conclusion:these results suggest that water retention in the acute phase of Kawasaki disease may be a risk factor for CAL, and water intake of both infusion and oral intake should be kept to a minimum in order to avoid progressive oedema.Abbreviations ASA acetylsalicylic acid - CAL coronary arterial lesions - HGF hepatocyte growth factor - IVIG intravenous immunoglobulin - KD Kawasaki disease - MBW maximum rate of increase in body weight - VEGF vascular endothelial growth factor     

Regressed retinopathy of prematurity: the relationship between clinical risk factors of the newborn period and regressed retinopathy of prematurity severity in a preterm born population of Stockholm county 1976-81

In a retrospective study, clinical risk factors of the neonatal period were correlated with the severity of regressed retinopathy of prematurity (ROP) in a population of preterm infants (bw less than 1500 g and or gestational age less than 33 weeks). At the age of 5-11 years 134 out of 528 preterm born infants (25.4%) were found to be under ophthalmic care. Reliable information on eye fundus status could be obtained in 105 of them. Regressed ROP was found in 61, the moderate form in 48 (9.1%) and the severe form in 13 (2.5%) patients. Twelve patients (2.3%) had visual acuity of less than 0.3 on the worst eye and two (0.4%) of these patients were blind from ROP. Twenty-four clinical factors of the newborn period were correlated with the severity of regressed ROP. The results suggest that long oxygen exposure in combination with other factors interfering with retinal vasotonus are associated with the degree of the disease developed.