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Byline: N. Tumer (1), F. Yalcinkaya (1), E. Adegnce (1), M. Ekim (1), K. Kose (2), N. Cakar (1), N. Kara (1), N. Ozkaya (1), C. Ensari (1), S. Onder (1) Keywords: Key wordsaBlood pressure; Turkish children Abstract: aIn 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. Author Affiliation: (1) Department of Pediatrics, Ankara University Faculty of Medicine, Ankara, Turkey, TR (2) Department of Biostatics, Ankara University Faculty of Medicine, Ankara, Turkey, TR (3) Cinar Sitesi 5. Blok No. 62, TR-06530 Umitkoy Ankara, Turkey e-mail: ince@dialup.ankara.edu.tr Fax: +90-312-210-1348, TR Article note: Received: 29 December 1997 / Revised: 22 September 1998 / Accepted: 23 September 1998

Byline: N. Cakar (1) Author Affiliation: (1) University of Istanbul, Anesthesiology and Intensive Care, 34 390 Capa, Istanbul, Turkey, TR Article note: Accepted: 10 November 1998

Introduction: Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disease that can present with various forms of arthritis. This retrospective study aims to evaluate the characteristics of patients with arthritis in a large pediatric cohort of FMF patients., Methods: The demographic and clinical data were extracted from electronic medical records. Patients with arthritis were grouped as arthritis of FMF and arthritis of FMF-associated diseases., Results: A total of 541 patients were followed with a diagnosis of FMF in the last 5 years. Acute arthritis of FMF (n: 138) was the most common cause. It showed a recurrent course in the majority with a longer duration than other attack symptoms. Significantly higher frequencies of biallelic exon 10 and M694V mutations, erysipelas-like erythema, and protracted febrile myalgia were detected in these patients, particularly in those older than 2 years of age. Sacroiliitis of FMF was the second most common cause (n: 19). Patients with acute arthritis and sacroiliitis of FMF needed higher doses of colchicine. One patient with neonatal-onset FMF and M694V homozygosity was diagnosed with protracted arthritis. Arthritides of FMF-associated diseases including IgA vasculitis (n: 10), juvenile idiopathic arthritis (n: 9), chronic nonbacterial osteomyelitis (n: 5), and inflammatory bowel disease (n: 2) were detected in 26 patients., Conclusions: Arthritis is an important clinical finding of FMF mostly associated with M694V mutations. The frequency of protracted arthritis is declined, whereas sacroiliitis of FMF and arthritis of associated diseases expand the spectrum of arthritis. This study represents the changing face and current perspectives of arthritis in FMF. Key Points • Arthritis is an important clinical finding of familial Mediterranean fever (FMF) that can present in various forms • Arthritis is most likely associated with M694V mutations • The frequency of protracted arthritis is declined whereas sacroiliitis of FMF and arthritis of associated diseases expand the spectrum of arthritis in FMF., (© 2022. International League of Associations for Rheumatology (ILAR).)

Background: The Turkish Renal Tubular Disorders Working Group aimed to form a patient registry database and gathered demographic, clinical, and laboratory data in various hereditary renal tubular disorders (HRTDs)., Methods: A questionnaire comprising HRTDs was sent to the centers. The cohort was composed of 226 patients (109 girls, 117 boys)., Results: The distribution of patients according to HRTD was as follows: 45.6% distal renal tubular acidosis (dRTA), 26.6% proximal RTA (pRTA), 3.5% type IV RTA, 21.7% Bartter's syndrome, and 2.6% Gitelman's syndrome. Cystinosis was the most common cause for renal Fanconi syndrome. Age at diagnosis was between 1 month and 16 years. Overall consanguinity rate was as high as 72%. Rate of affected siblings was 28.5%. pRTA and type IV RTA were more common in males. Most common presenting symptoms were failure to thrive, lack of appetite, and vomiting. Nephropathic cystinosis was the most common HRTD leading to renal failure, followed by dRTA. Hearing loss was present in 23% of patients with dRTA and 6.3% of patients with Bartter's syndrome. No other patient had hearing loss. Convulsions were noted in Bartter's syndrome patients with failure to thrive, especially in those with height below 3%. Polyuria and nephrocalcinosis were more common in dRTA patients with deafness compared with patients without deafness., Conclusions: This data reflected a high number of HRTDs as a result of high consanguinity rate in Turkey. Our data serve as a database of demographic, clinical, and laboratory features of this rare disease group.

Background: The adverse effects of peritonitis and of the duration of dialysis on dialysis adequacy and clinical outcome were evaluated in this study., Methods: The study comprised 24 chronic peritoneal dialysis patients who were followed up at least for 12 months. Casual blood pressure (BP) measurements, echocardiographic evaluation, peritonitis rate, hemoglobin (Hb), serum albumin, normalized protein catabolic rate (nPCR), total Kt/Vurea, weekly creatinine clearance (CCr), residual renal function (RRF), removal of fluid and dose of recombinant human erythropoietin (EPO) were evaluated., Results: Mean age of the patients was 15.3 +/- 3.6 years. Mean follow-up was 50.4 +/- 26.8 months. Peritonitis rate was calculated as 1 episode/32.7 patient-months. Systolic hypertension was detected in 14 patients (58%) and diastolic hypertension in 15 (63%). RRF showed a negative correlation with duration on dialysis (r=-0.623, p=0.006). There was no significant correlation between RRF and nPCR, Hb, hematocrit, albumin and dose of EPO. A negative correlation was found with left ventricular mass index and fluid removal (r=-0.461, p=0.041). Higher doses of Kt/Vurea are associated with higher protein intake (r=0.503, p=0.024). A positive correlation was found between Kt/Vurea and Hb and Hct levels (r=0.460, p=0.009, and r=0.528, p=0.017, respectively). Dialysis adequacy tests were found not to be affected by the frequency of peritonitis., Conclusion: The most important factor for the prevention of hypervolemia in chronic peritoneal dialysis patients is RRF. The concept of adequate dialysis should include normal volume homeostasis, control of blood pressure and adequate nutrition.

Background: To examine whether CO2 pneumoperitoneum and positive end expiratory pressure (PEEP) in mechanical ventilation affect the systemic spread of intraabdominal infection., Methods: Sprague-Dawley male rats weighing 200-300 g were allocated to three groups of 12 animals in each. All rats received mechanical ventilation under general anesthesia. An intraabdominal infection model was established by injecting with 1 ml of Escherichia coli (10(9) CFU/mL) intraperitoneally. Half of the animals in each group were exposed to PEEP (10 cmH2O). CO2 pneumoperitoneum at 13 mmHg was applied to the rats in group 1. Group 2 rats underwent laparotomy. Group 3 served as controls. In addition, TNF-alpha serum levels were measured at baseline and 3 h. A peritoneal specimen for histopathological examination were obtained after the rats were killed at the end of 3 h. For the assessment of data, descriptive statistical methods (mean, standard deviation) as well as Friedman test for repeated measurements in multiple groups, Kruskal-Wallis test for intergroup comparisons, Dunn's multiple comparison test for subgroup comparisons, Mann-Whitney U test for comparisons between paired groups, chi-square and Fisher's exact test for comparison of qualitative data, and McNemar's test for assessment of changes in group variables over time were used. The results were considered statistically significant if probability (p) values were <0.05., Results: Grades of peritonitis in group 1 and 2 were seen to differ nonsignificantly. In group 1, baseline blood cultures were not included in the assessment between the subgroups that received PEEP or not, as there was no growth in any of the subgroups. No significant difference was detected between growth in blood cultures at 1, 2, and 3 h ( p > 0.05). Application of PEEP in subgroups did not alter the blood culture results ( p > 0.05). Significant differences were seen between the initial and final TNF-alpha values of groups (KW: 18.94, p < 0.0001). The values in control group were observed to be significantly lower than those in groups 1 and 2 ( p < 0.01, p < 0.001). Bacteremia and systemic spread of the intraabdominal infection did appear to be different according to the PEEP application. After the assessments of ventilation parameters in our study, significant reductions in pH and HCO3 levels were detected in group 1 as a result of pneumoperitoneum, which was consistent with the literature. There is a significant difference between pH values at baseline and at the end of 1 h because of pneumoperitoneum (Fr: 10.01, p < 0.05). PEEP application in subgroups did not create significant differences in terms of respiratory parameters ( p < 0.01)., Conclusion: No difference was found between the applications of CO2 pneumoperitoneum and laparotomy with regard to bacteremia and infection-induced peritonitis. It was determined that pneumoperitoneum along with PEEP application had neither a positive nor a negative impact on intraabdominal infection.

The frequency of acute rheumatic fever (ARF) in patients with familial Mediterranean fever (FMF) was documented and the effects of preceding streptococcal infections on the exacerbation of FMF were determined. In the first part of the study, 162 individuals with FMF were investigated for a history of ARF in a retrospective study. In the second part of the study, antistreptolysin-O (ASO) titres were measured in 130 individuals with FMF. Thirty-six patients had an arthritic attack (group A1), 55 patients had a typical FMF attack without arthritis (group A2) and 39 patients were in the attack-free interval (group B) during the investigation. Nine patients with FMF (5.5%) were considered to have ARF and three of them (1.85%) also had rheumatic heart disease. This prevalence of rheumatic heart disease in FMF is higher than that of the normal population (0.65%) reported in Turkey. Elevated ASO titres were found in 75%, 42% and 38% of the patients in groups A1, A2 and B, respectively. These percentages were found to be significantly higher in group A1 than in both groups A2 (p<0.01) and B (p<0.01). We concluded that patients with FMF might be more prone to the late complications of streptococcal infections.

Basal erythropoietin (Epo) levels and single dose-pharmacokinetics of recombinant human erythropoietin (rhuEpo) were investigated in 8 predialysis (PD) patients (mean age 11.6 +/- 1.4 years) and in 8 patients on continuous ambulatory peritoneal dialysis (CAPD) (mean age 12.7 +/- 0.6 years). Basal Epo levels were found to be 1.0 +/- 0.0 mu/ml in PD group, 1.6 +/- 0.7 mu/ml in CAPD group and 8.5 +/- 1.8 mu/ml in control group. Following administration of 50 mu/kg rhuEpo (s.c.) serum Epo concentration (Cmax) was 23.2 +/- 2.5 mu/ml in 18.5 +/- 2.6 hours (tmax) in PD patients and 9.9 +/- 0.8 mu/ml in 26.8 +/- 7.7 hours in CAPD patients. Mean elimination half-lives (t1/2) were 13.3 +/- 1.9 hours and 13.5 +/- 3.0 hours in PD patients and CAPD patients, respectively. The volume of distribution (Vd) was 840.0 +/- 100.0 ml/kg; the clearance (Epo Cl) was 37.0 +/- 5.5 ml/kg/hour in PD patients. These values were significantly lower in PD patients than in CAPD patients (p < 0.05) (Vd; 1500 +/- 240.0 ml/kg; Epo Cl 110 +/- 30.0 ml/kg/hour). During the course of CAPD, more efficient clearance of uraemic toxins that inhibit erythropoiesis and more rapid extraction of erythropoietin by erythroid precursors may cause higher Vd in CAPD patients than in PD patients.

A ten-year-old boy with hyperreninaemic hypertension is described. He had a normally functioning right kidney, as confirmed by ultrasonography and renal scintigraphy. Renal angiography was performed but the left kidney and the left renal artery could not be detected. Computerized tomography revealed a rudimentary atrophic left kidney. Nephrectomy was carried out and the blood pressure returned to normal.

Plasma protein C (PC) activity, prothrombin time (PT), partial thromboplastin time (PTT) and platelet count were studied in 23 children with nephrotic syndrome (NS) and AT III activity was determined in 15 children in the same group. All parameters were compared with those obtained in a group of 16 age matched healthy controls. Mean plasma AT III activity was significantly reduced in patients with NS (P < 0.001) correspondingly, plasma AT III levels were found to be directly correlated with serum albumin and inversely correlated with proteinuria. In contrast, mean plasma PC activity, as well as PT, PTT and platelet count were similar in the NS group when compared with the control group and in addition no remarkable difference was found in the mean plasma PC activity between the steroid sensitive and resistant NS groups. In conclusion, this study demonstrated acquired AT III deficiency and normal PC levels in childhood NS. These data suggest that although plasma AT III activity depends on the severity of NS, neither the severity of NS nor the underlying renal disease is an important factor determining the changes of PC activity in childhood NS.

A 12-year-old girl with vaginal ectopic ureter in a duplex system associated with an extremely dysplastic upper segment who presented with a complaint of vaginal discharge is reported. The diagnostic approach with special reference to ultrasonography and computerized tomography is discussed.