M. Hakan Poyrazoğlu

Genotype–phenotype correlation in children with familial Mediterranean fever in a Turkish population

Background: The aim of the present study was not only to review clinical and demographic features of child‐onset familial Mediterranean fever (FMF) patients but also to investigate whether there is a phenotype–genotype correlation in the same patient population.

Amitraz poisoning in children.

There is no information available in the general paediatric literature about amitraz poisoning. In the present study, which is the largest series in the literature, we report 24 children with amitraz poisoning.

The Effects of Pentoxifylline on Diabetic Renal Changes in Streptozotocin‐Induced Diabetes Mellitus

The aim of the study was to investigate the effects of pentoxifylline on the renal growth, the epidermal growth factor receptor expression, and renal total nitric oxide content in streptozotocin‐induced diabetic rats. Adult male Wistar albino rats were randomly divided into three groups: normal control (the N group), diabetic nephropathy (the DN group), and diabetic nephropathy treated with pentoxifylline at the dosage of 20 mg · kg− 1· d− 1, intraperitoneally (the group DNP). Diabetes was induced by injection of streptozotocin intraperitoneally. The kidney wet weight (KWW) and dry weight (KDW), fractional kidney weight (FKW), glomerular volume (VG), renal tissue protein (RTP) contents, and renal tissue total nitric oxide (NO) production were determined after the rats were sacrificed on 10th day. There was a significant increase in KWW and KWD in the DNP and DN groups when compared to the N group (p = 0.000 for the DNP group, p = 0.000 and p < 0.01 for the DN group). In the DN group, FKW was increased for both wet and dry kidney weight (p < 0.05 and p = 0.001, respectively) while in the DNP group there was increase in FKW only for dry kidney weight. VG was increased in both two diabetic groups (p < 0.05), but this increase was less prominent in the rats treated with pentoxifylline. RTP was significantly decreased in the DNP group when compared with the values in the DN group (p < 0.05). Immunohistochemically epidermal growth factor receptor expression was increased in diabetic rats, and it was not affected by pentoxifylline treatment. In diabetic rats renal content of total NO was decreased (p < 0.05 for the DNP group, p < 0.01 for the DN group). In conclusion, the results provide that pentoxifylline may have some beneficial effects on renal changes in streptozotocin‐induced diabetic rats.

Renal tubular dysgenesis with atypical histology and in-utero exposure to naproxen sodium

Abstract Renal tubular dysgenesis (RTD), a rare, lethal, autosomal recessive disorder, is characterised by short and poorly differentiated proximal convoluted tubules associated with oligohydramnios, Potter sequence and neonatal death from respiratory failure. We report an unusual case of neonatal anuria owing to RTD with normally formed lungs, in-utero exposure to naproxen sodium and atypical histology in that the glomeruli were not as crowded as usually seen in RTD. When there is anuria in an infant following birth in the context of a normal renal ultrasound and an absence of objective evidence of perinatal hypoxia, RTD should be considered.

The hormonal and radiological evaluation of adrenal glands, and the determination of the usefulness of low dose ACTH test in patients with renal amyloidosis.

Amyloidosis is a multisystem disease which may cause organloss. Renal involvement is the most common clinical problem in amyloidosis,however involvement of endocrin organs is possible. In this study to assessadrenocortical function and to evaluate the usefulness of low dose ACTH testin patients with renal amyloidosis, we determined cortisol, 17-hydroxyprogesteron(17-OHP) and 11-deoxycortisol (11-DOC) responses to both 1 μg and 250 μgSynacthen. We also determined the size of adrenal glands radiologically byusing computerized tomography. Twenty one patients with renal amyloidosisand 16 healthy subjects for hormonal evaluation, and 20 patients with renalamyloidosis and 22 healthy subjects for radiologic evaluation were includedin the study. In four patients (19%) peak serum cortisol levels followingstimulation with the low dose of Synacthen were less than 20 μg/dL (550nmol/L). Two of them had also subnormal cortisol response to the 250 μgSynacthen stimulation test. Basal and stimulated levels of 11-DOC were lowerthan those of control values (p = 0.000and p < 0.01 respectively). The mean11-DOC responses to stimulation with 1 μg Synacthen were also significantlylower than the values obtained after the simulation with 250 μg Synacthen(p < 0.01 and p= 0.000). Cortisol responses to the stimulation with 250 μg Synacthen werealso lower than the control responses (p< 0.05). 17-OHP responses were similar to the control values in both tests.In the radiological evaluation the mean maximum width of right adrenal glandsand the mean anterior and maximum width of left adrenal glands were significantlygreater in the patient group (p < 0.01).In conclusion, adrenal involvement and adrenal insufficiency is common inamyloidosis. Low 11-DOC levels in amyloidosis is a new finding and furtherdetailed studies is required to explain its cause.

Modulation of Inflammation by Mesenchymal Stem Cell Transplantation in Peritoneal Dialysis in Rats

Aim: The purpose of this study was to determine the effect of mesenchymal stem cell (MSC) transplantation on the peritoneal morphology and inflammation markers in rat models of peritoneal dialysis (PD). Materials and methods: Wistar albino rats were divided into two groups: control (C) (n = 8) and experimental groups (n = 50). PD solution was given to the experimental group during 6 weeks. Then, experimental group was divided into three groups as PD, MSC, and placebo (P) groups. MSC group was treated with MSC (1.5 × 106 cells/kg) and P group was treated with phosphate buffer solution via intraperitoneal injection. Evaluation was performed to C and PD groups at the end of 6 weeks and to MSC and P groups at second and third week of the treatment (MSC-2, P-2, MSC-3, and P-3 groups). Results: The submesothelial area was significantly thickened in PD and P groups compared to C and MSC groups. Peritoneal fibrosis was seen in P-3 group but not in MSC group. There were no significant differences between the MSC-3 and C groups according to morphological findings. Levels of tumor necrosis factor-α (TNF-α) and interleukin-6 (IL-6) were significantly increased in MSC-2 group compared to the other groups (p-values ranged from 0.0001 to 0.04). TNF-α and IL-6 levels in MSC-3 and P-3 groups were lower than PD and C groups (p < 0.0001 for TNF-α and p = 0.0001–0.002 for IL-6). Conclusion: Giving MSC may protect the peritoneal membrane from the deleterious effect of PD and extend the life of the peritoneal membrane. Our study is the first on this issue and more detailed studies are needed.

Trace Elements in Children Suffering from Idiopathic Nephrotic Syndrome

OBJECTIVE Trace elements play a significant role in several metabolic processes and often circulate in the blood binding to protein. The purpose of this study was to determine the status of selenium, zinc, and boron in idiopathic nephrotic syndrome patients in active and remission phases. MATERIALS AND METHODS Fourteen patients and fourteen healthy age-matched controls were included in the study. The selenium, zinc and boron level in plasma and urine were measured by the inductively coupled plasma mass spectrometry. RESULTS The plasma levels of zinc and selenium were significantly lower in both active and remission patients (for all p=0.0001). The plasma boron level was significantly lower only in patients in active phase (p=0.0002 vs control). The concentrations of urinary boron and selenium were significantly higher during active phase compared with remission (p=0.0003 and 0.0001, respectively). CONCLUSION Supplementation with zinc, selenium and boron may be justified in patients suffering with this disease.

11-year-old boy with chronic renal failure and myelodysplastic syndrome

characterized by progressive impairment in the differentiation of hematopoietic cells, resulting in a wide range of hematologic and clinical features.1 These disorders have been described mainly in adults and rarely in the pediatric age group.2 However, the association of extrarenal malignancy with glomerular diseases is well known.3 The most common abnormalities are membranous glomerulonephritis in patients with carcinoma and minimal change nephropathy in patients with Hodgkin’s disease.3 The number of reported cases with glomerulopathy are very small in myeloid malignancies including acute and chronic myeloid leukemia.3–6 To the best of our knowledge, only five patients with glomerulopathy associated with MDS have been reported.7 In this report we presented an 11-year-old boy with chronic renal failure (CRF) who was subsequently diagnosed with MDS.

Lupus Nephritis in a Child with Type I Diabetes Mellitus

Patients with type 1 diabetes (T1D) are at increased risk for developing other autoimmune diseases, most commonly autoimmune thyroiditis and celiac disease. Few reports have described the association of systemic lupus erythematosus and T1D in the literature. To the best of our knowledge, this is the first report of lupus nephritis in a child with T1D.

Kresentik glomerülonefritle başvuran bir Wegener granülomatozu olgusu

Regional anaesthesia has advantages such as, control of postoperative pain, early mobilization, and does not affect respiratory function. In recent years, regional anaesthesia had improved by the application of the peripheral nerve stimulator and ultrasound guided blocks, new local anaesthetic drugs with the use of additional adjunctive agents. Especially in emergency conditions, combination of regional anaesthesia may avoid the complications of general anesthesia in patients with chronic disease who suffer from upper and lower extremity injuries. In this paper, we present two cases who underwent combined regional anaesthesia techniques for the lower and upper extremity surgery.Iki tarafli ses teli felci (ISTF) siklikla tiroid ve paratiroid cerrahisi gibi iyatrojenik bir hasar sonucu olusur. Fakat serebral infarkt sonrasi hava yolu tikanikligina neden olan BVKP nadir olarak bildirilmistir. Bu yazida tekrarlayan serebral infarktin, gec ortaya cikan ve hayati tehdit eden bir komplikasyonu olarak solunum durmasi ile sonuclanan bir ISTF olgusu sunduk. Atmis yedi yasinda erkek hasta, son 3-4 aydir giderek artan solunum yetmezligi sikayetiyle acil servisimize basvurdu. Ozgecmisinde kalp yetmezligi ve kronik obstruktif akciger hastaligi oykusu olmayan hasta, yaklasik bir yil once bir ayda iki kez serebral infarkt gecirmisti. Yapilan solunum sistemi muayenesinde bilateral wheezing ve stridor; norolojik muayenesinde ise sag tarafta kuvvet kaybi mevcuttu. Ust solunum yolu obstruksiyonunu degerlendirilmek uzere endoskopik laringoskopi yapildi. Laringoskopide iki tarafli ses tellerinin orta hatta sabit ve hareketsiz oldugu goruldu. Solunum yetmezliginde artma ve tekrarlayan solunum durmasi nedeniyle hastaya trakeostomi acildi. Operasyon sonrasi hastanin solunum yetmezligi geriledi. Solunum fonksiyonlari normale donen hasta taburcu edildi.Dysphagia aortica is a rare etiological factor of dysphagia resulting from extrinsic compression of the esophagus by thoracic aorta. There is no gold standard diagnostic proscedure for dysphagia aortica. Dysphagia aortica is usually escaped to attention in the differential diagnosis of dyssphagia. Here, we reported compression of the esophagus by thoracic aorta in a 54 years-old male patient who comsplained from dysphagia.