Acute rheumatic fever: 10-year single-center experience: clinical and laboratory findings, with subclinical carditis and treatment complications.

BACKGROUND: Acute rheumatic fever in childhood continues to cause serious morbidity despite all developments. The objective of this study was to evaluate the clinical and laboratory characteristics of patients with acute rheumatic fever and to determine the frequency of subclinical carditis and the side effects of the drugs used in the treatment. METHODS: The data of patients hospitalised between 2008 and 2018 with the diagnosis of acute rheumatic fever were included in the study. The relationship of gender and age with the frequency of major symptoms and the distribution of the drugs used in the treatment and their side effects were evaluated. RESULTS: Medical records of 102 patients with complete data were reviewed. 56.9% of the patients were male and the mean age was 10.7 ± 1.9 years. The most common distribution of complaints found were arthritis (51%), arthralgia (25.5%) and fever (16.7%). 10.8% of all patients (n = 11) were diagnosed subclinical carditis via echocardiographic evaluation. The frequency of carditis was higher in female patients with a borderline statistical significance (p = 0.05). However, there was no statistically significant difference between gender and arthritis (p = 0.22) and carditis (p > 0.05). Anti-congestive therapy was required in 22% and inotropic treatment was needed in 6.1% cases. Toxic hepatitis developed in four cases during the acetylsalicylic acid treatment. CONCLUSIONS: In a 10-year period, detection of subclinical carditis in 10.8% cases supported that echocardiography should be performed as a standard method for the diagnosis of acute rheumatic fever. Patients should be followed closely in terms of hepatic toxicity due to acetylsalicylic acid used in the treatment.

A novel frameshift mutation of malonyl-CoA decarboxylase deficiency: clinical signs and therapy response of a late-diagnosed case.

We evaluate the clinical findings and the treatment response of a late-diagnosed case with a novel homozygous insertion c.13_14insG (p.P6Afs*202) result in a frameshift mutation in MLYCD gene. Both cardiac and neurologic involvements were mild when compared to previously reported cases, and see low-fat/high-carbohydrate diet treatment is highly effective.

Leptospirosis in a child with acute respiratory distress syndrome.

Yesilbas O, Sevketoglu E, Kihtir HS, Hatipoglu N, Yildirim HM, Akyol MB, Aktay-Ayaz N, Gökçe I. Leptospirosis in a child with acute respiratory distress syndrome. Turk J Pediatr 2017; 59: 688-692. Leptospirosis is an infectious vasculitis, which can occur with different clinical features. While it is generally a subclinical and self-limited infection; kidney and liver dysfunction, pulmonary hemorrhage, thrombocytopenia, and cardiovascular collapse may occurr. A six-year-old boy presented with acute respiratory distress syndrome, shock, thrombocytopenia-associated multiple organ failure, and persistent high fever secondary to leptospirosis. Persistent high fever was resistant to intravenous immunoglobulin and pulse steroid therapy. He was successfully treated with plasmapheresis and hemofiltration with endotoxin-cytokine cleaning filter. In conclusion; leptospirosis may cause thrombocytopenia-associated multiple organ failure, persistent high fever, and acute respiratory distress syndrome. Plasmapheresis and hemofiltration should be considered in cases of severe leptospirosis with multiorgan failure.

Effects of ACE inhibitors on insulin resistance and lipid profile in children with metabolic syndrome.

OBJECTIVE: The aim of this study was to evaluate the effects of using ACE inhibitors on insulin resistance, glucose metabolism, body fat composition, and lipid profile in children over 10 years of age with obesity-associated metabolic syndrome (MS). METHODS: A total of 53 children with MS, who had been followed for at least one year were included in the study. The sample was divided into two groups: Group 1-30 obese children (13 female, 17 male) who were not using an ACE inhibitor and Group 2-23 obese children (13 female, 10 male) who were using an ACE inhibitor. Anthropometric and laboratory data obtained at baseline and at the 3rd, 6th, and 12th months of follow-up were compared in the two groups. RESULTS: Comparison of the data in the two groups at 3rd, 6th, and 12th months revealed no statistically significant differences in terms of weight standard deviation score (SDS), body mass index SDS, weight for height percentile, body fat percentage, and very low-density lipoprotein (VLDL)values. However, there were statistically significant differences in mean glucose and insulin levels, homeostasis model assessment for insulin resistance, LDL and high-density lipoprotein values, and highly significant differences in mean triglyceride values. CONCLUSIONS: The positive effects of ACE inhibitor drugs, particularly on hypertriglyceridemia and insulin resistance, might bring them forth as first-line drugs in the treatment of obese and hypertensive children. Randomized, controlled, double-blind, and long-term studies are needed for a definitive conclusion.

Non-dipping phenomenon in children with monosymptomatic nocturnal enuresis.

PURPOSE: Monosymptomatic nocturnal enuresis is a common disorder seen in childhood, and many factors play a role in its etiopathology to varying degrees. The aim of our study was to investigate the possible association between nocturnal enuresis and 24-h blood pressure profiles of enuretic children. METHODS: A total of 45 children ranging in age from 6 to 15 years with monosymptomatic nocturnal enuresis and 22 age-matched healthy controls were enrolled in our study. The blood pressure measurement was made at 30-min intervals during a 24-h period via an ambulatory blood pressure measurement device. Both groups underwent medical tests that included a complete blood count, blood biochemistry profile, urinalysis and blood renin-aldosterone levels, and all study subjects received an abdominal ultrasound. RESULTS: Statistically significant high nocturnal blood pressure levels were observed in our patients with monosymptomatic nocturnal enuresis compared with the control group (p < 0.05). The mean values of the day-to-night difference (dipping) in the systolic and diastolic blood pressure of the patients were significantly lower than those of control group (p < 0.05). CONCLUSION: Nocturnal enuresis should not only be accepted as a urinary system disorder. Possible systemic causative factors have to be examined, especially in patients that are resistant to first-line therapy. Based on the results of our study, we deduce that one of the factors that plays a role in the pathogenesis of enuresis nocturna is a non-dipping blood pressure profile (the "non-dipping" phenomenon).