Severe haematological involvement in children with systemic lupus erythematosus and clinical associations.

OBJECTIVES: To investigate the severe haematological involvement in children with SLE and assess its clinical associations, treatments, outcome and damage accrual. METHODS: The medical charts of children with SLE in whom haematological involvement was observed were reviewed. Severe haematological indices were defined as autoimmune haemolytic anaemia with a haemoglobin concentration < 8 g/dL, thrombocyte count < 30 000/µL, and neutrophil count < 500/µL. RESULTS: Among the 224 patients included, 102 (45.5%) displayed severe indices, predominantly at the initial involvement, and most frequently as severe anaemia in 54 (24.1%) and severe thrombocytopenia in 45 (20.1%). Disease activity did not differ according to the presence of severe disease indices. In addition, the presence of severe indices at initial involvement did not affect the damage accrual. However, a higher rate of damage (51.1% vs. 29.9%, p = 0.002) and steroid-induced damage (28.9% vs. 8.2%, p < 0.001) was evident in patients with flares of the haematological system. Regression analysis revealed that rituximab treatment during the initial episode (OR:4.5, p = 0.006) and the presence of anticardiolipin antibodies (OR:2.3, p = 0.014) significantly increases the odds for haematological system flare. However, severe indices at initial involvement did not increase the odds of a haematological flare. CONCLUSION: Severe haematological indices at onset are common but not related with disease outcomes. Prevention of flares is important to improve outcomes, and a more rigorous maintenance strategy would benefit most to children who display haematological indices refractory to conventional immunosuppressants and those with anti-cardiolipin antibodies.

Dyslipidemia in children with chronic kidney disease-findings from the Cardiovascular Comorbidity in Children with Chronic Kidney Disease (4C) study.

BACKGROUND: Dyslipidemia is an important and modifiable risk factor for CVD in children with CKD. METHODS: In a cross-sectional study of baseline serum lipid levels in a large prospective cohort study of children with stage 3-5 (predialysis) CKD, frequencies of abnormal lipid levels and types of dyslipidemia were analyzed in the entire cohort and in subpopulations defined by fasting status or by the presence of nephrotic range proteinuria. Associated clinical and laboratory characteristics were determined by multivariable linear regression analysis. RESULTS: A total of 681 patients aged 12.2 ± 3.3 years with a mean eGFR of 26.9 ± 11.6 ml/min/1.73 m2 were included. Kidney diagnosis was classified as CAKUT in 69%, glomerulopathy in 8.4%, and other disorders in 22.6% of patients. Nephrotic range proteinuria (defined by a urinary albumin/creatinine ratio > 1.1 g/g) was present in 26.9%. Dyslipidemia was found in 71.8%, and high triglyceride (TG) levels were the most common abnormality (54.7%). Fasting status (38.9%) had no effect on dyslipidemia status. Except for a significant increase in TG in more advanced CKD, lipid levels and frequencies of dyslipidemia were not significantly different between CKD stages. Hypertriglyceridemia was associated with younger age, lower eGFR, shorter duration of CKD, higher body mass index (BMI-SDS), lower serum albumin, and higher diastolic blood pressure. CONCLUSIONS: Dyslipidemia involving all lipid fractions, but mainly TG, is present in the majority of patients with CKD irrespective of CKD stage or fasting status and is significantly associated with other cardiovascular risk factors.

Personal Experiences of Chief Physician Emir çelebi on Kidney and Bladder Diseases: A Brief Review in Anmudhaj al-Tibb.

OBJECTIVES: The aim of this study is to review the kidney and bladder disease sections in Emir çelebi's book called Anmudhaj al-Tibb by presenting and discussing his own experiences with these issues. MATERIALS AND METHODS: A copy of Anmudhaj al-Tibb registered in Istanbul Süleymaniye Manuscript Library, Mihrisah Sultan Collection, no. 342/1, and a printed copy of al-Mujaz fi al-Tibb in Arabic, as well as a copy of the Turkish translation of this work by Sururi registered in Ankara National Library, A1437, were examined. We examined the findings in light of the literature. RESULTS AND CONCLUSION: We discovered that the sections on kidney and bladder diseases in Emir çelebi's Anmudhaj al-Tibb are actually Turkish translations of relevant sections of Ibn al-Nafis's al-Mujaz fi al-Tibb. In some of these chapters, Emir çelebi has added his own observations and experiences, whereas, in others, he has included quotations from physicians such as Ibn Masawayh and Abu Bakr Muhammad ibn Zakariyya al-Razi. One of the most intriguing findings was the drug Yad-Allah, which Emir çelebi claimed was very effective in reducing the kidney stone of the Grand Admiral of the Ottoman Navy Receb Pasha. According to Emir çelebi, Galen and Aflinus [?] both praised this medicine in their own works. However, it has been discovered that Yad-Allah, as described in the literature, is a drug defined by Philagrius.

What Does the Most Celebrated and Pioneer Ottoman Physician, Sanizade Mehmed Ataullah Efendi, Say on Anatomy, Physiology, Diseases, and Surgery of the Urinary System? A Glance at Hamse-i Sanizade or Kanun-i Sanizade.

OBJECTIVES: Hamse-i Sanizade (Khamse Shani-zade) is the most important work of Sanizade Mehmed Ataullah Efendi (1769 or 1771-1826), an Ottoman kadi (qadi), physician, historian, polymath, and polyglot, about medicine and consists of 5 books. The first book, Mir'at al-ebdan fi teshrih a'da' al-insan, is on anatomy. The second book, Usul al-tabi'a, deals with physiology, while the third, Mi'yar al-etibba, mentions diseases and their treatments. These books were published together in Istanbul in 1820. A fourth book, Qanun al-cerrahin, published in Cairo after his death in 1828, discusses surgical treatments of diseases. A fifth book, Mizan al-edviye, is a pharmacopeia. In this study, we examined the information about urinary system anatomy, physiology, diseases, and their medical and surgical treatments in Hamse-i Sanizade. MATERIALS AND METHODS: We examined the first 3 books and the fourth book. The relevant passages were translated into English after being transliterated into the contemporary Turkish alphabet. RESULTS: In the first book, kidney anatomy is discussed under the heading "fi teshrih al-kula" and bladder anatomy under "fi teshrih al-methane." In the second book, the formation and excretion of urine are explained in different headings. In the third book, kidney inflammation and its treatment are discussed, with kidney and bladder stones and their medical treatments explained in detail. Finally, the types and treatments of urinary retention are discussed, with types, causes, symptoms, prognosis, and surgical treatments of bladder stones written in detail. CONCLUSIONS: In the Ottoman Empire, Hamse-i Sanizade is regarded as one of the first gateways to the West in the field of medicine. It was instrumental in modernizing Ottoman-Turkish medicine and contains and reflects the state-of-the-art knowledge of the time regarding the anatomy, physiology, diseases, and medical and surgical treatments of the urinary system.

From a Surgeon's Registry (Dr. Hayri Meriç): Urological Surgeries and Results From Gaziantep Hometown Hospital in 1949.

OBJECTIVES: In this study, we aimed to present the urological surgeries performed in 1949 and their results recorded by Dr. Hayri Meric (1909 to October 8, 1968). MATERIALS AND METHODS: We analyzed the 64-page brochure written by Dr. Hayri Meric that was published in 1950 by Halk Dili Press (Gaziantep, Turkey). RESULTS: Meric reported that 738 patients were hospitalized in the surgical clinic of Gaziantep Hometown Hospital in 1949. Surgical intervention was performed in 473 (64.1%) of these patients. Forty-nine patients had received surgery for urological reasons, of whom 48 (97.9%) were cured and 1 (2.1%) died (diagnosed with prostate enlargement). In addition to his practice as a physician, Meric also brought necessary regulations as a hospital manager and emphasized the importance of communication. CONCLUSIONS: Dr. Hayri Meric was a surgeon who became a role model to his colleagues by his demonstration of the value of proper keeping of medical records for transfer of information to future generations, and he emphasized the importance of patient-physician and physician-physician communication.

Disparities in treatment and outcome of kidney replacement therapy in children with comorbidities: an ESPN/ERA Registry study.

Background: Data on comorbidities in children on kidney replacement therapy (KRT) are scarce. Considering their high relevance for prognosis and treatment, this study aims to analyse the prevalence and implications of comorbidities in European children on KRT. Methods: We included data from patients <20 years of age when commencing KRT from 2007 to 2017 from 22 European countries within the European Society of Paediatric Nephrology/European Renal Association Registry. Differences between patients with and without comorbidities in access to kidney transplantation (KT) and patient and graft survival were estimated using Cox regression. Results: Comorbidities were present in 33% of the 4127 children commencing KRT and the prevalence has steadily increased by 5% annually since 2007. Comorbidities were most frequent in high-income countries (43% versus 24% in low-income countries and 33% in middle-income countries). Patients with comorbidities had a lower access to transplantation {adjusted hazard ratio [aHR] 0.67 [95% confidence interval (CI) 0.61-0.74]} and a higher risk of death [aHR 1.79 (95% CI 1.38-2.32)]. The increased mortality was only seen in dialysis patients [aHR 1.60 (95% CI 1.21-2.13)], and not after KT. For both outcomes, the impact of comorbidities was stronger in low-income countries. Graft survival was not affected by the presence of comorbidities [aHR for 5-year graft failure 1.18 (95% CI 0.84-1.65)]. Conclusions: Comorbidities have become more frequent in children on KRT and reduce their access to transplantation and survival, especially when remaining on dialysis. KT should be considered as an option in all paediatric KRT patients and efforts should be made to identify modifiable barriers to KT for children with comorbidities.

Urinary DKK3 as a biomarker for short-term kidney function decline in children with chronic kidney disease: an observational cohort study.

BACKGROUND: Childhood-onset chronic kidney disease is a progressive condition that can have a major effect on life expectancy and quality. We evaluated the usefulness of the kidney tubular cell stress marker urinary Dickkopf-related protein 3 (DKK3) in determining the short-term risk of chronic kidney disease progression in children and identifying those who will benefit from specific nephroprotective interventions. METHODS: In this observational cohort study, we assessed the association between urinary DKK3 and the combined kidney endpoint (ie, the composite of 50% reduction of the estimated glomerular filtration rate [eGFR] or progression to end-stage kidney disease) or the risk of kidney replacement therapy (ie, dialysis or transplantation), and the interaction of the combined kidney endpoint with intensified blood pressure reduction in the randomised controlled ESCAPE trial. Moreover, urinary DKK3 and eGFR were quantified in children aged 3-18 years with chronic kidney disease and urine samples available enrolled in the prospective multicentre ESCAPE (NCT00221845; derivation cohort) and 4C (NCT01046448; validation cohort) studies at baseline and at 6-monthly follow-up visits. Analyses were adjusted for age, sex, hypertension, systolic blood pressure SD score (SDS), BMI SDS, albuminuria, and eGFR. FINDINGS: 659 children were included in the analysis (231 from ESCAPE and 428 from 4C), with 1173 half-year blocks in ESCAPE and 2762 in 4C. In both cohorts, urinary DKK3 above the median (ie, >1689 pg/mg creatinine) was associated with significantly greater 6-month eGFR decline than with urinary DKK3 at or below the median (-5·6% [95% CI -8·6 to -2·7] vs 1·0% [-1·9 to 3·9], p<0·0001, in ESCAPE; -6·2% [-7·3 to -5·0] vs -1·5% [-2·9 to -0·1], p<0·0001, in 4C), independently of diagnosis, eGFR, and albuminuria. In ESCAPE, the beneficial effect of intensified blood pressure control was limited to children with urinary DKK3 higher than 1689 pg/mg creatinine, in terms of the combined kidney endpoint (HR 0·27 [95% CI 0·14 to 0·55], p=0·0003, number needed to treat 4·0 [95% CI 3·7 to 4·4] vs 250·0 [66·9 to ∞]) and the need for kidney replacement therapy (HR 0·33 [0·13 to 0·85], p=0·021, number needed to treat 6·7 [6·1 to 7·2] vs 31·0 [27·4 to 35·9]). In 4C, inhibition of the renin-angiotensin-aldosterone system resulted in significantly lower urinary DKK3 concentrations (least-squares mean 12 235 pg/mg creatinine [95% CI 10 036 to 14 433] in patients not on angiotensin-converting enzyme inhibitors or angiotensin 2 receptor blockers vs 6861 pg/mg creatinine [5616 to 8106] in those taking angiotensin-converting enzyme inhibitors or angiotensin 2 receptor blockers, p<0·0001). INTERPRETATION: Urinary DKK3 indicates short-term risk of declining kidney function in children with chronic kidney disease and might allow a personalised medicine approach by identifying those who benefit from pharmacological nephroprotection, such as intensified blood pressure lowering. FUNDING: None.

Hyponatremic-hypertensive Syndrome in a 19-month-old Boy with Renovascular Hypertension.

Hyponatremic-hypertensive syndrome (HHS) is an uncommon disorder rarely seen in children. Herein, we report a 19-month-old boy with HHS. He had severe hypertension, polyuria, polydipsia, vomiting, and seizure at presentation. Laboratory findings revealed hyponatremia, hypokalemia, metabolic alkalosis, proteinuria, hypercalciuria, high levels of renin and aldosterone, and renal artery stenosis. All symptoms resolved after nephrectomy. Clinicians should be aware of this syndrome because prompt recognition can be lifesaving.

Renal biopsy in children with IgA vasculitis.

INTRODUCTION: Henoch-Schönlein purpura nephritis (HSN) is defined as Henoch-Schönlein purpura with kidney involvement, including hematuria and/or proteinuria. The aim of this study was to evaluate the data of HSN patients who underwent renal biopsy, and compare the main clinical and laboratory parameters that may affect renal biopsy findings, treatment protocols, and short- and long-term outcome of those patients. METHODS: Biopsies performed in 72 HSN patients between January 2007 to January 2017 were retrospectively evaluated. They were divided into two groups according to renal biopsy classification of the International Study of Kidney Disease in Children. Renal outcome, clinical and laboratory parameters, treatment protocols, and outcome were compared between groups. Short- and long-term follow-up of patients were evaluated. RESULTS: Of 72 patients, 47 were male (65.3%) and 44 (61.1%) were ≤10 years of age. Neutrophil-lymphocyte ratio was found higher in patients with scrotal involvement (p=0.042). Short-term unfavorable outcome was significantly higher in patients with scrotal involvement (p=0.038). Patients with hypertension and decreased creatinine clearance were found to have more unfavorable outcomes in long-term follow-up (p=0.029, p=0.040). CONCLUSION: Cyclosporin-A and cyclophosphamide could be effective in steroid unresponsive HSN patients. Patients with scrotal involvement, decreased creatinine clearance, and hypertension should be closely monitored for sequelae of HSN.

Kidney disease profile and encountered problems during follow-up in Syrian refugee children: a multicenter retrospective study.

BACKGROUND: Children are one of the most vulnerable groups in conflict zones, especially those with chronic diseases. This study aimed to investigate kidney disease profiles and problems during follow-up in a population of Syrian refugee children residing in Turkey. METHODS: Syrian refugee children aged between 0 and 18 years were included in the study. Demographic data, diagnosis, particular interventions due to nephrological problems, and problems encountered during follow-up were obtained from all participating pediatric nephrology centers. RESULTS: Data from 633 children from 22 pediatric nephrology centers were included. Mean age of the children was 94.8 ± 61.7 months and 375 were male (59%). 57.7% had parental consanguinity and 23.3% had a close relative(s) with kidney disease. The most common kidney diseases were congenital anomalies of the kidney and urinary tract (CAKUT) (31.0%), glomerular disease (19.9%), chronic kidney disease (CKD) (14.8%), and urolithiasis (10.7%). Frequent reasons for CAKUT were nonobstructive hydronephrosis (23.0%), vesico-ureteral reflux (18.4%), and neurogenic bladder (15.8%). The most common etiology of glomerular diseases was nephrotic syndrome (69%). Ninety-four children had CKD, and 58 children were on chronic dialysis. Six children had kidney transplantation. Surgical intervention was performed on 111 patients. The language barrier, lack of medical records, and frequent disruptions in periodic follow-ups were the main problems noted. CONCLUSIONS: CAKUT, glomerular disease, and CKD were highly prevalent in Syrian refugee children. Knowing the frequency of chronic diseases and the problems encountered in refugees would facilitate better treatment options and preventive measures.

Microbiome is not linked to clinical disease severity of familial Mediterranean fever in an international cohort of children.

OBJECTIVES: The severity of familial Mediterranean fever (FMF) may vary in different areas, suggesting a role for environmental factors. We analysed the composition of gut microbiota among children with FMF and healthy controls from Turkey and the USA and determined its effect on disease severity. METHODS: Children with FMF with pathogenic MEFV mutations and healthy controls from Turkey and the USA were enrolled. FMF disease activity was evaluated with the Autoinflammatory Disease Activity Index (AIDAI). Gut bacterial diversity was assessed by sequencing 16S rRNA gene libraries. RESULTS: We included 36 children from Turkey (28 patients with FMF, 8 healthy controls), and 21 patients and 6 controls from the USA. In the Turkish group, 28.6% of patients had severe disease, while 13.3% of US group patients had severe disease. As expected, we observed substantial differences between the gut microbiota of children from the two geographic regions, with Turkish patients and controls exhibiting higher relative abundances of Bacteriodia, while US patients and controls exhibited higher relative abundances of Clostridia. Alpha- and betadiversity did not differ significantly between FMF patients and controls, and neither was predictive of disease severity within each geographic region. We observed differences between FMF patients and controls in the relative abundance of some bacterial taxa at the amplicon sequence variant (ASV) level, but these differences received mixed statistical support. CONCLUSIONS: Among an international cohort of children with FMF, we did not find a strong effect of gut microbiota composition on disease severity. Other environmental or epigenetic factors may be operative.

Rituximab treatment for difficult-to-treat nephrotic syndrome in children: a multicenter, retrospective study

Background/aim: This study aimed to evaluate the efficacy of rituximab in children with difficult-to-treat nephrotic syndrome, considering the type of disease (steroid-sensitive or ­resistant) and the dosing regimen. Materials and methods: This multicenter retrospective study enrolled children with difficult-to-treat nephrotic syndrome on rituximab treatment from 13 centers. The patients were classified based on low (single dose of 375 mg/m2) or high (2-4 doses of 375 mg/m2) initial dose of rituximab and the steroid response. Clinical outcomes were compared. Results: Data from 42 children [20 steroid-sensitive (frequent relapsing / steroid-dependent) and 22 steroid-resistant nephrotic syndrome, aged 1.9­17.3 years] were analyzed. Eleven patients with steroid-sensitive nephrotic syndrome (55%) had a relapse following initial rituximab therapy, with the mean time to first relapse of 8.4 ± 5.2 months. Complete remission was achieved in 41% and 36% of steroid-resistant patients, with the median remission time of 3.65 months. At Year 2, eight patients in steroid-sensitive group (40%) and four in steroid-resistant group (18%) were drug-free. Total cumulative doses of rituximab were higher in steroid-resistant group (p = 001). Relapse rates and time to first relapse in steroid-sensitive group or remission rates in steroid-resistant group did not differ between the low and high initial dose groups. Conclusion: The current study reveals that rituximab therapy may provide a lower relapse rate and prolonged relapse-free survival in the steroid-sensitive group, increased remission rates in the steroid-resistant group, and a significant number of drug-free patients in both groups. The optimal regimen for initial treatment and maintenance needs to be determined.

Legislative proposal in Italy to facilitate contacts between deceased organ donor families and transplant recipients.

Contacts between organ donors and recipients might be possible in the near future in Italy. As suggested by The Italian Committee of Bioethics "anonymity is requested by the Italian National Transplant Centre" before transplantation anonymity shall be strict in order to grant privacy, gratuity, justice, solidarity and benefits and avoids organ trafficking. Following a period that is ethically correct and justifiable, organ donor families and recipients can meet after signing a valid declaration of consent, expressed on a template valid for the whole country. A third party within the body of the National Health Systems shall control the validity of the consent. The opinion stresses that contacts are not a right but a possibility justifiable on ethical grounds if the procedure is followed appropriately. A legislative proposal has been presented before the Chamber of deputies incorporating all suggestions made by the National Committee of Bioethics. The agreement between parties might be signed a year after transplantation. This is a long enough period of time for the recipients to fully appreciate the benefits of the procedure and for the donor families to see the effects of their decision (the opinion and the Law proposal hit the Zeitgeist, and keep Italy in the regulation of European Union).

Renal Crisis in Children during Armed Conflict.

Armed conflicts continue to occur in some regions of the globe, mostly in developing countries. These man-made disasters affect all segments of the population; however, some groups are more vulnerable and suffer more seriously from the unfavorable consequences of such conflicts. Among these, the pediatric population deserves special attention because they cannot protect themselves, and hence carry a higher threat of injuries and probability of death during conflicts. In addition, children who do survive the disaster are more prone to exploitation. Pediatric victims, including those who sustain acute kidney injury or those suffering from chronic kidney disease before armed conflicts, face higher risks of morbidity and mortality as a result of treatment problems, specifically limited dialysis options. Displaced children, forced to flee their homes as a result of armed conflicts, are also at risk for various health problems because they may not find ideal circumstances for disease treatment. Making preparations in anticipation of armed conflicts, such as disaster-relief scenarios and action plans, may be useful to decrease the death toll in these children, who are dependent on their caregivers for survival. Adopting principles of disaster nephrology may contribute to improved survival chances of pediatric kidney patients in chaotic circumstances.

Increased potassium excretion in children with monosymptomatic nocturnal enuresis: could it be related to Kir 4.1- KCNJ10 gene polymorphism?

BACKGROUND AND OBJECTIVES: There are controversial results in the literature regarding urinary electrolytes, especially potassium, in enuretic children. KCNJ10 channel protein, a member of the Kir 4.1 family is expressed in renal distal tubules and has an important function in renal ion transport. We investigated whether KCNJ10 gene polymorphisms are associated with clinical and laboratory findings of a group of Turkish children with monosymptomatic primary nocturnal enuresis (MNE). METHOD: Ninety-seven MNE children and 100 healthy controls were tested for three single nucleotide polymorphisms (SNPs) in the KCNJ10 gene. The transversions in SNPs were G to A for intron 1(SNP1), G to A for exon 2 (SNP2), and T to C transition for promoter (SNP3). All SNPs were genotyped by PCR-restriction fragment length polymorphism. RESULTS: SNP3 in promoter of KCNJ10 gene showed strong association with MNE children for distribution of genotype and allele frequency, while SNP1 in intron 1 and SNP2 in exon 2 were noninformative. The distribution of TT, TC, and CC genotypes for SNP3 was 66%, 26.8% and 7.2% respectively in MNE compared with 38%, 59% and 3% respectively in controls (p < 0.0001). In enuretic children, TT genotype was higher and there was an increased potassium excretion in children with TT genotype (P < 0.05). CONCLUSION: We conclude that KCNJ10 gene promoter polymorphism may have a role on potassium excretion in Turkish MNE children. This is the first study in literature evaluating KCNJ10 gene polymorphism in this patient population. Future studies investigating the other SNPs, mutations or altered regulation of Kir4.1 in larger samples would help clarify the role (s) of KCNJ10 gene in enuresis.

Waist to height ratio as a screening tool for identifying childhood obesity and associated factors.

OBJECTIVE: To investigate the prevalence of obesity and associated factors during childhood in Southeastern Turkey. Another objective was to determine the cut-off points of Waist to Height Ratio (WHtR) values for defining obesity/abdominal obesity. METHODS: The community-based descriptive cross-sectional study was conducted in Gaziantep Turkey between November 2011 and December 2011 with 2718 primary school/high schools students aged 6-17 years. The SPSS 22.00 was used for the analysis of data. RESULTS: The prevalence of overweight, obesity, abdominal obesity, was 13.2%, 4.2% ,26.4%, respectively. There was a reverse relationship between BMI/WC values and sleep durations (p<0.05). The BMI/WC values were higher in students with computer usage time ≥1 hours in a day (p<0.05). Parental obesity status has an effective role on the WC/BMI values of children (p<0.05). The WHtR was a good predictor of diagnosis on obesity and abdominal obesity (AUC=0.928, p<0.0001; AUC=0.920, p<0.0001; respectively). The optimal cut-off values for obesity and abdominal obesity were detected as 0.5077, 0.4741, respectively. CONCLUSIONS: The WHtR can be used for diagnosis of obesity/abdominal obesity. Parental obesity, short sleep duration and computer use more than one hour per day are risk factors for the development of obesity in children and adolescents.

Immunohistochemical evaluation of Cajal-like cells and Caveolin-1 levels at ureteropelvic junction obstruction. / Evaluación inmunohistoquímica de las células de Cajal-like y los niveles de Caveolina-1 en la obstrucción de la unión pieloureteral.

OBJECTIVE: To compare c-kit-positive interstitial Cajal-like cells (ICC) and Caveolin-1 protein levels as a pacemaker and signaling molecules, on ureteropelvic junction (UPJ) specimens, between two groups of pediatric patients with and without ureteropelvic junction obstruction (UPJO). METHODS: We evaluated the UPJ specimens of 45 pediatric patients operated between 2005- 2012 retrospectively. Group 1 included 37 patients who underwent dismembered pyeloplasty due to UPJO. Eight patients underwent nephrectomy by the other reasons (renal tumor, trauma etc) and had normal UPJ were accepted as Group 2. The specimens were examined immunohistochemically with CD117 and Caveolin-1 antibody. According to the total number of ICC; 0-5 cells were accepted as a few (1), 610 cells as moderate (2), and > 10 as many (3). According to the staining intensity of Caveolin-1 at muscle tissue, a subjective evaluation was performed as; mild staining (1), moderate staining (2) and strong staining (3). RESULTS: The mean value of ICC distribution was calculated 1.37 ± 0.54 in Group 1 and 2.13 ± 0.64 in Group 2 (p = 0.003), and the median value of ICC distribution was found 1 [1-3] in Group 1 and 2 [1-3] in Group 2 (p = 0.008). Median values for the intensity of staining with Caveolin-1 were found 2 [1-3] in the Group 1, and 2.5 [2-3] in the Group 2 (p = 0.025). CONCLUSIONS: A decrease in ICC and Caveolin-1 levels support that there may be a relationship between ICC and Caveolin-1 for UPJO associated with signal transduction and peristalsis in urinary system.

OBJETIVO: Comparar las células intersticiales Cajal-like, c-kit positivas, y los niveles de proteína Caveolina -1 como marcapasos y moléculas señalizadoras en piezas de unión pieloureteral (UPU) comparando dos grupos de pacientes pediátricos con o sin obstrucción de la UPU.MÉTODOS: Evaluamos retrospectivamente las piezas obtenidas en la operación de 45 pacientes pediátricos entre 2005-2012. El grupo 1 incluía 37 pacientes sometidos a pieloplastia desmembrada por estenosis de UPU. Ocho pacientes que fueron sometidos a nefrectomía por otras razones (tumor renal, traumatismo, etc) y tenían una UPU normal se incluyeron en el grupo 2. Las piezas fueron examinadas mediante inmunohistoquímica con CD117 y Caveolina-1 en el tejido muscular. Se realizó una evaluación subjetiva como: tinción leve (1), tinción moderada (2) y tinción fuerte (3). RESULTADOS: Se calculó el valor medio de la distribución de las células intersticiales de Cajal (CIC), 1,37 ± 0,54 en el Grupo 1 y 2,13 ± 0,64 en el Grupo 2 (p = 0,003), y la mediana, de 1 [1-3] en el Grupo 1 y 2 [1-3] en el Grupo 2 (p = 0,008). Los valores de la mediana para la intensidad de la tinción con Caveolina- 1 fueron de 2 [1-3] en el Grupo 1 y 2,5 [2-3] en el Grupo 2 (p = 0,025). CONCLUSIONES: Una disminución de las células intersticiales de Cajal y los niveles de Caveolina 1 apoyan que puede haber una relación entre las células intersticiales de Cajal y la Caveolina 1 en la estenosis de la UPU asociada con la transducción de la señal y el peristaltismo en el sistema urinario.