Nuray Özkaya

Blood pressure nomograms for children and adolescents in Turkey

Abstract In order to obtain data on blood pressure (BP) distribution in Turkish children, a total of 5,599 Turkish children from birth to 18 years were studied. BP rises with age, and both systolic and diastolic BP showed a positive correlation with height and weight in both sexes. As the sampling was representative of Turkish children at different ages, the mean systolic and diastolic BP levels were compared for each age with the results reported in the study of the Second Task Force. The mean systolic and diastolic BP of Turkish children and the increase with growth and development were different from the Second Task Force study. Genetic, ethnic, and environmental factors were suggested to be responsible for this variation. In conclusion, normal BP curves should be applied with caution in childhood, and every population should use their own normal standards to define a measured BP level in children.

COLCHICINE TREATMENT IN CHILDREN WITH FAMILIAL MEDITERRANEAN FEVER

Prophylactic colchicine therapy has been shown to be a safe and effective method of eliminating the attacks and preventing the development of amyloidosis in patients with familial Mediterranean fever (FMF). However, information about effective dosages that control FMF attacks and prevent amyloidosis in childhood is not available. The aim of this study is to determine the ‘effective colchicine dose’ for children in terms of body weight and surface area. Sixty-two (34 male, 28 female) children with FMF were selected and colchicine treatment was initiated by giving 0.5–1 mg/day to each patient. The dose was gradually increased up to a maximum 2 mg/day in unresponsive patients; mean duration of therapy was 45.6 ± 35.5 months. When the ‘optimal effective dosage’ (i.e. the one that reduced the frequency of attacks and ESR, CRP and fibrinogen levels during the attack-free period) was achieved, the optimal effective dose was calculated according to the body weight and body surface area for each patient. Based on these values ‘mean colchicine dose’ was computed for the study group and values for different age groups were evaluated. Mean colchicine doses according to the body weight and surface area of the whole group were found to be 0.03 ± 0.02 mg/kg/day and 1.16 ± 0.45 mg/m2/day, respectively. It was shown that children less than 5 years of age might need colchicine doses as high as 0.07 mg/kg/day or 1.9 mg/m2/day. These dosages are approximately 2.5–3 times more than the ‘mean colchicine dose’ for children aged 16–20 years. These results clearly show that small children need higher doses of colchicine in order to control their attacks. Thus, we conclude that colchicine, when given according to body weight or body surface area, would be more effective in childhood.

Antistreptococcal Response is Exaggerated in Children with Familial Mediterranean Fever

Abstract: Familial Mediterranean fever (FMF) is an autosomal recessive disorder. Although the pathogenesis of the disease is not yet completely understood, enhanced acute-phase responsiveness is considered to be one of the most important mechanisms. The presence of high levels of antistreptolysin O (ASO) antibodies and streptococcus-associated diseases, such as acute poststreptococcal glomerulonephritis (AGN) and acute rheumatic fever (ARF), has been reported in patients with FMF. In order to better understand the effect of FMF on antistreptococcal antibody response, we measured ASO and antideoxyribonuclease B (anti-DNAse B) levels in patients with FMF and compared them with those in healthy controls. The study consisted of two parts. In the first step, antistreptococcal antibody levels were analysed in 44 patients with FMF and 165 healthy children who had no history or clinical evidence of upper respiratory tract infection (URTI) for the last 4 months. In the second step, antistreptococcal antibody levels were measured in 15 patients with FMF and 22 healthy controls in response to documented group A β-haemolytic streptococcal pharyngitis. In the first part of the study, ASO and anti-DNAse B levels in patients with FMF were found to be significantly higher than those in healthy controls (P<0.001). In the second part, ASO and anti-DNAse B titres were found to be significantly higher in patients with FMF than in controls (P<0.001 and <0.05, respectively) 4 weeks after a positive throat culture. We concluded that patients with FMF have an exaggerated response to streptococcal antigens and might be prone to poststreptococcal non-suppurative complications, such as ARF.

Pseudoxanthoma elasticum: a rare cause of hypertension in children.

Abstract. We describe an 11-year-old girl with severe renin-dependent hypertension. She had characteristic cutaneous, ocular, and vascular signs of pseudoxanthoma elasticum (PXE), and the diagnosis was confirmed histologically. Because PXE is a heterogeneous heritable disorder, the family was evaluated for this condition. Since the patient’s mother and brother showed some manifestations of PXE, autosomal dominant inheritance is the most likely inheritance pattern in this family.

Spectrum of renal osteodystrophy in children on continuous ambulatory peritoneal dialysis

Abstract Background: The prevalence of different types of bone disease in chronic renal failure (CRF) has changed significantly during the last decade. The aim of the present study is to evaluate the spectrum of bone disease in children with CRF undergoing continuous ambulatory peritoneal dialysis (CAPD).

Low-dose erythropoietin is effective and safe in children on continuous ambulatory peritoneal dialysis.

Abstract. Hypertension is one of the most important complications of erythropoietin (rHuEPO) therapy in dialysis patients. In this study, the effect of two different dosage regiments of subcutaneous rHuEPO on blood pressure [BP] was evaluated in 20 anemic children on continuous ambulatory peritoneal dialysis (CAPD). Patients were randomized to receive rHuEPO 50 U/kg, either once a week (group 1, 50 U/kg per week) or three times a week (group 2, 150 U/kg per week). At the beginning of the study, 8 patients in group 1 and 8 patients in group 2 were on antihypertensive therapy. In group 1, the hematocrit increased gradually and significantly from 18.98%±1.79% to 30.1%±1.62% after 6 months, while in group 2 it rapidly increased from 19.53%±1.86% to 32.4%±1.11% after 3 months. A significant increase in the mean arterial BP was observed in group 2. Antihypertensive therapy had to be increased in all of the 8 previously hypertensive patients and had to be initiated in 1 of the 2 originally normotensive patients in the same group. None of the patients in group 1 required a change in antihypertensive medication. We conclude that during treatment with rHuEPO pre-existing hypertension and the dose of rHuEPO are the most important risk factors for the development or worsening of hypertension in children on CAPD, and gradual elevation of hematocrit by low-dose rHuEPO avoids the development of severe hypertension.

Primary nephrotic syndrome during childhood in Turkey

Abstract Background : Minimal change nephrotic syndrome (MCNS) is the most common cause of primary nephrotic syndrome (NS) during childhood. However, recent studies from different countries have reported an increasing incidence of focal segmental glomerulosclerosis (FSGS) in children.

Incidence of pericardial effusion during attacks of familial Mediterranean fever

Familial Mediterranean fever (FMF) is an autosomal recessive disorder that affects primarily Jews, Armenians, Turks, and Arabs. It is characterised by recurrent, self limited attacks of fever accompanied by inflammation of the peritoneal, synovial, and pleural surfaces.1 Pericardial involvement is a well known (0.7–1.4%) but rare feature of the disease.2,3 Our initial observation of two patients who had recurrent pericarditis as a sole manifestation of FMF4 has led us to suggest that pericardial inflammation is more prevalent than generally believed. Since echocardiography is a non-invasive and sensitive tool for the detection of pericardial effusion, we undertook an echocardiographic study to assess the exact frequency of pericardial effusions during attacks of FMF. Two dimensional, M mode, and Doppler echocardiographic examinations were performed during 55 consecutive FMF attacks in 42 patients (15 female, 17 male). Echocardiographic study was carried out by one of the authors (ET or SA) and reviewed by the other one, who was aware of the diagnosis of FMF but unaware of the presence or absence of chest pain. Typical attacks of FMF consisted of fever and serositis including peritoneum, synovium, and pleura lasting 1–4 days. Attacks of FMF were recurrent and self limited. FMF was diagnosed according to established clinical criteria or molecular analysis when appropriate (in 37 patients).1 None of the patients had …