Cüneyt Ensari

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.

Effect of oral levamisole supplementation to hepatitis B vaccination on the rate of immune response in chronic hemodialysis patients.

Accessible online at: www.karger.com/journals/nef Dear Sir, Patients with chronic renal failure on hemodialysis (HD) carry an increased risk of hepatitis B virus infection due to defective immune response, resulting from T cell effector dysfunction [1]. When the vaccine was first introduced in the early 1980s, it was hoped that hepatitis B virus would be completely eliminated from the renal failure population. However, the results of most of the vaccination programs have been disappointing. Due to a specific defect, only 50– 60% of the patients form significant and protective antibodies [2]. Specific antibody formation to infectious agents and vaccines is also deficient in these patients. Thus, standard vaccination protocols were enhanced in order to improve protection. Current recommendations include vaccination with twice the standard dose of vaccine by four intramusculer injections at months 0, 1, 6, and 9. Despite these measures, the proportion of patients protected by vaccination could not be increased. Interleukin 2, gamma interferon, thymopentin, zinc, and colony-stimulating factor were added to standard vaccinations in order to increase the immune response [3–7]. These were, however, experimental protocols and cannot be recommended as routine procedures. We have, therefore, decided to investigate the effects of levamisole, a nonspecific immune modulator, on the immune response following vaccination. Sixty patients (34 males, 26 females), ranging in age from 18 to 63 (mean B SD 41.4 B 2.4) years were included in the study. Patients were on HD (three times per week) for 11.8 B 1.1 months and were HBsAg, anti-HBs, anti-HBc, anti-HBe, and HBeAg negative with no prior vaccination. Four randomized patient groups were formed together with 15 healthy controls (9 males and 6 females with a mean age of 37.4 B 1.7 years). The patients received intramuscular recombinant DNA-B hepatitis vaccine (Engerix B; 20 Ìg) into the deltoid muscle at 0, 1, 6, and 9 months. Group 1 received a single dose, group 2 had a double dose, group 3 had a single dose plus 120 mg oral levamisole after HD, and group 4 had a double-dose vaccine plus 120 mg oral levamisole after HD. HBsAg and anti-HBs were evaluated by means of a microparticle enzyme immunoassay technique using Abbott-Axoym systems. Cases with an antiHBs level 110 mIU/ml were considered as ‘antibody positive’. These tests were performed at 3 and 10 months. Data are given as mean B SD. The results were compared with the paired chiTable 1. Number of patients in each group responding to hepatitis B vaccination

MEFV gene mutations in Henoch-Schönlein purpura.

Coexistence of familial Mediterranean fever (FMF) with various systemic vasculitides, including Henoch–Schönlein purpura (HSP) and other inflammatory disorders has been reported and the MEFV gene has been suggested to play an important role in the pathogenesis of this association. In the present study, the mutation rate of the MEFV gene in HSP and its association with the clinical course of the disease were evaluated.

Congenital microcephaly and infantile nephrotic syndrome — a case report

A 22-month-old girl with nephrotic syndrome and microcephaly is described. The had dismorphic facies and psychomotor retardation. Her parents were first-degree relatives and one of her siblings had died with nephrotic syndrome and renal failure in infancy. An autosomal recessive inheritance is suggested. The diagnosis of this rare combination is discussed and the relevent literature is reviewed.

Response to Delayed Fluid Therapy in Crush Syndrome

Background: Although early treatment is valuable in the prognosis of crush syndrome, the diagnosis and treatment of many victims are inevitably delayed in major disasters. Patients and Methods: Among the 38 victims of the Marmara earthquake with crush injury, 27 were diagnosed as crush syndrome on the basis of findings of acute renal failure. Intensive intravenous fluid treatment was started in all patients on admission. Of these 27 patients, 10 required dialysis treatment while 17 did not. The laboratory data on admission were evaluated and compared between the two groups. Results: The mean admission time of 27 patients was 46.5 ± (SE) 3.08 h. There was no significant difference between the dialysis and the nondialysis groups with regard to patient’s age, trapped time or admission time. A significantly higher number of patients had crush injury in more than one extremity in the dialysis group. The dialysis group had significantly lower systolic blood pressure, central venous pressure but a higher heart rate together with higher levels of serum urea nitrogen, creatinine, creatinine kinase, C-reactive protein, fibrinogen on admission compared to the nondialysis group. Conclusion: Our results suggest that even delayed application of aggressive specific fluid treatment under close monitoring may prevent the development of established acute renal failure.

Rare case of Alstrom syndrome without obesity and with short stature, diagnosed in adulthood (Case Report)

SUMMARY:  Alstrom syndrome is a rare autosomal recessive disorder characterized by retinal degeneration, sensorineural hearing loss, obesity, type 2 diabetes mellitus and chronic nephropathy. It may be associated with acanthosis nigricans, hypergonadotropic hypogonadism, hepatic dysfunction, hepatic steatosis, hyperlipidaemia, dilated cardiomyopathy and short stature. We report a patient with Alstrom syndrome who had hypergonadotropic hypogonadism, hepatic dysfunction, hepatic steatosis and short stature with normal body weight, all of which are seen infrequently with this syndrome.

Are Uraemic Children Immunologically Compromised

Background: Various immunological abnormalities leading to impaired immune status have been described in uraemic adults; however, few data are available for uraemic children. Methods: In this study, peripheral blood total lymphocyte count and lymphocyte subsets (CD3+, CD4+, CD8+, CD16+, CD20+) were evaluated, skin tests with PPD and Candida antigens were performed, and serum immunoglobulin (IgG, IgA, IgM) and complement (C3, C4) levels were measured in 30 children with end-stage renal failure (10 before dialysis, 10 on continuous ambulatory peritoneal dialysis, and 10 on haemodialysis) and the results compared with those of 15 healthy controls. Results: The data showed significant lymphopenia in predialysis and haemodialysis groups. No significant change was observed in the CD4+/CD8+ ratio or in the percentages of lymphocyte subsets in either group studied, while the absolute values of some lymphocyte subsets were significantly lower in all groups as compared with controls. In skin test evaluation, only the patients in the predialysis group showed a significantly decreased response to Candida antigen. The serum immunoglobulin levels were significantly decreased in the continuous ambulatory peritoneal dialysis group as compared with the control group. Conclusion: Our results, together with those of other paediatric studies, reported in the literature, suggest that uraemic children are not immunocompromised, though the effects of uraemia may cause some variation in their immune status.

Effect of Low-Protein Diet Supplemented with Keto Acids on Progression of Disease in Patients with Chronic Renal Failure

Accessible online at: www.karger.com/journals/nef Dear Sir, Dietary intervention is one of the major components of conservative therapy in patients with chronic renal failure (CRF). Various diet protocols have been used in patients with CRF. The aim of diet therapy in CRF patients should be to minimize uremic symptoms and to provide an optimum nutritional status for the patient. It has been shown that low-protein diet regimens, together with a sufficient calorie support, relieve uremic symptoms and slow down the progression of renal failure in patients with CRF [1–4]. Bergström et al. [5] have used diet protocols containing 18 g protein/day, and they have claimed that better responses could be obtained when keto acid preparations containing analogues of valine, leucine, isoleucine and phenylalanine are added to such a regimen. In the present study, we treated compensated predialytic CRF patients with a low-protein diet supplemented with keto analogues of essential amino acids in order to observe their effects on the progression of the disease. Eighteen patients with moderate CRF (8 female and 10 male) were included into the study. The mean age was 45.4 B (BSD) 11.4 (range 28–66) years. The underlying renal disease was chronic glomerulonephritis in 6, diabetic nephropathy in 4, chronic tubulointerstitial nephritis in 2, amyloidosis in 2, autosomal dominant polycystic kidney disease in 1, and of unknown origin in 3 patients. All patients were on dietary protein intakes of 0.8 g/kg/day and 35 kcal/kg/day for 6 months (period 1). Then, the patients were switched to a diet containing 0.4 g protein/ kg/day and 35 kcal/kg/day for 6 months (period 2). This diet was supplemented with a preparation containing keto analogues of essential amino acids (Ketosteril® tablets; 0.2 g/kg/day). In addition, conventional therapy measures had been taken accordingly during the study period. None of the patients were treated with androgenic steroids or erythropoietin. Serum urea, creatinine, albumin, inorganic phosphorus, total calcium, high-density lipoprotein (HDL) cholesterol, low-density lipoprotein (LDL) cholesterol, and triglyceride levels and glomerular filtration rate (GFR) were measured before the study and at the end of periods 1 and 2. Body weight and height were also measured in indoor clothing. The GFR was calculated as the average of urea and creatinine clearances, and was normalized to 1.73 m2 of body surface area. Results were compared with the paired Student t test, and p ! 0.05 was accepted as statistically significant. All patients completed the study, and no side effect leading to withdrawal of the drug Table 1. Study parameters

Skin biopsy in Berger's disease: is it really useful in the diagnosis?

Accessible online at: www.karger.com/journals/nef Dear Sir, Berger’s disease (BD) is known as ‘Henoch-Schönlein purpura (HSP) without the rash’ and these two diseases are considered as the clinical variants of the same entity with common pathogenetic mechanisms [1, 2]. It is widely accepted that typical clinical features are sufficient for the diagnosis of HSP and that renal biopsy is needed in a limited number of cases, in contrast to BD where renal biopsy is mandatory for definite diagnosis. Various immune deposits, mainly IgA, have been found in the involved skin of HSP patients [3] who may also show immune deposits in the uninvolved skin [3–5]. In the study of Baart de la Faille-Kuyper et al. [6] and a few others [7–12], however, high levels of IgA positivity were observed in the normal skin in BD which led the investigators to conclude that this finding could be of importance in the diagnosis of some BD cases in whom renal biopsy is either contraindicated or could not be performed. Zawada and Ramirez [13], on the other hand, suggested that these cases should rather be accepted as subclinical or occult HSP, not BD. In reviewing the literature, we could find only one study performed during the last decade, written in Polish [14], reporting a high proportion of negative results in skin biopsy specimens of patients with BD and HSP. We have, therefore, decided to evaluate skin biopsy findings in BD and HSP in order to assess their diagnostic potential in BD. Skin biopsy was performed in 19 BD patients ranging in age between 7 and 34 (mean B SD 14.4 B 8.3) years, with a renal biopsy specimen revealing IgA nephropathy. Other diseases such as systemic lupus erythematosus and liver diseases that could cause IgA-associated nephropathy were verified. Biopsy specimens were taken from clinically normal skin of the tibial surface during gross hematuric attacks in patients with BD. Twenty-one patients with an age range of 5– 33 (11.6 B 6.8) years, diagnosed as having HSP with typical clinical findings, also underwent skin biopsy from purpuric rash within 24 h of its occurrence. The skin biopsy specimens were large enough to include subcutaneous tissue and intradermal blood vessels and were examined by light and immunofluorescence microscopy. In patients with skin rash (HSP group), the skin biopsy specimen showed leukocytoclastic vasculitis and mainly IgA-positive immune depositions. Patients without rash (BD group) showed normal light microscopy with no significant depositions on immunofluorescence microscopy. In contrast to many previous studies [6– 13] (table 1), our results suggest that, in the absence of renal biopsy findings, skin biopsy should not be used as a diagnostic tool in BD. We would like to draw attention to the high incidence of positive skin biopsy specimens in BD reported in the literature which seem rather difficult to interpret. Table 1. Reported results of skin biopsy specimens in BD

A patient with amyloidosis, vesicoureteral reflux and renal cell adenoma complicated by intrarenal calcification and acquired cystic renal disease.

Dr. İlkser Akpolat, Makis Yapi Kooperatifi, 5/2 Mutlukòy, TR-06530 Ankara (Turkey) Dear Sir, Amyloidosis and vesicoureteral reflux (VUR) are well known causes of end-stage renal disease (ESRD) [1,2] and ESRD may be complicated by acquired cystic renal disease (ACRD) and soft tissue calcification such as kidney, lung and heart [3, 4]. The coexistence of amyloidosis, VUR, ACRD and intrarenal calcification is rare but here such a patient with amyloidosis, VUR, ACRD and diffuse intrarenal calcification is presented. As far as we know this is the only case in whom all these lesions are present since 1982 according to the medline facilities of the Ankara University School of Medicine. A 14-year-old boy with growth retardation and ESRD due to amyloidosis secondary to familial Mediterranean fever and VUR was admitted to the Ankara University Hospital for pretransplant preparation. The patient had chronic renal disease since 1989 and had been on a chronic ambulatory peritoneal dialysis program for 2 years, and he had severe hyperparathyroidism with a serum Ca × P product greater than 70. Bilateral nephroureterectomy was performed because of third degree VUR and macroscopic findings were small, shrunken kidneys with many medullary and cortical cysts. The sections from nephrectomy material were stained with hematoxylin and eosin, von Kossa, congo red and gentian violet for microscopic examination and these stains showed dense, homogenous eosinophilic material, sclerosis, hyalinosis, tubular atrophy, multiple tubular cysts, focal renal cell adenoma and amyloidosis and diffuse calcification on glomeruli and vessel wall. Although these renal lesions may not be significant in patients with chronic renal disease, one must always bear in mind that more than one lesion may coexist in the same kidney. It appears that for unknown reasons visceral calcification occurs much less frequently nowadays than it used to [4], but it should not be forgotten that severe intrarenal calcification may still complicate ESRD. References Stone MJ: Amyloidosis: A final common pathway for protein deposition in tissues. Blood 1990;75:531-545. Zucchelli P, Gaggi R: Reflux nephropathy in adults. Nephron 1991;57:2-9. Ishikawa I: Uremic acquired renal cystic disease. Nephron 1991;58:257-267. Alfrey AC: Phosphate, aluminium and