RESUMEN

Clonal monocytosis reflects a preneoplastic or neoplastic sustained increase in the absolute monocyte count in the absence of reactive causes. Causes of clonal monocytosis include clonal cytopenias with monocytosis and acute and chronic myeloid neoplasms. Chronic myelomonocytic leukemia (CMML) is a prototypical myelodysplastic/myeloproliferative overlap neoplasm in adults, characterized by sustained peripheral blood monocytosis. Renal abnormalities, including acute kidney injury (AKI) and chronic kidney disease (CKD), are frequent in patients with CMML and are predictors of worse outcomes. In addition, AKI/CKD often limits eligibility for allogeneic stem cell transplantation or enrollment in clinical trials. In this review, we highlight clonal monocytosis-related etiologies that give rise to AKI and CKD, with special emphasis on CMML and lysozyme-induced nephropathy (LyN). Monocytes produce lysozyme, which, in excess, can accumulate in and damage the proximal renal tubular epithelium. Early identification of this etiology and a timely reduction in monocyte counts can salvage renal function. Other etiologies of renal injury associated with clonal monocytosis include direct renal infiltration by monocytes, renal extramedullary hematopoiesis, myeloproliferative neoplasm-associated glomerulopathy, auto-immune (membranous nephropathy, minimal change disease) and paraneoplastic manifestations, thrombotic microangiopathy, obstructive nephropathy due to myeloproliferation, and urate nephropathy due to tumor lysis syndrome. We propose to group these mechanistic etiologies of renal injury as clonal monocytosis of renal significance and provide guidance on their diagnosis and management.

RESUMEN

BACKGROUND: The impact of inadequate empirical antibiotic treatment on patient outcomes and hospitalization duration for non-life-threatening infections in children remains poorly understood. We aimed to assess the effects of inadequate empirical antibiotic treatment on these factors in pediatric patients. METHODS: The medical records of children admitted for infectious diseases with bacteria isolated from sterile sites between 2018 and 2020 were retrospectively reviewed. Patients who received adequate empirical treatment were compared with those who received inadequate treatment in terms of demographic, clinical, and laboratory variables. RESULTS: Forty-eight patients who received inadequate empirical antimicrobial treatment were compared to 143 patients who received adequate empirical treatment. Inadequate empirical antimicrobial treatment did not significantly affect the length of hospital stay or the incidence of complications in non-critically ill children with bacterial infections. Younger age and underlying renal abnormalities were identified as risk factors for inadequate antimicrobial treatment, while associated bacteremia was more common in the adequate antimicrobial treatment group. CONCLUSIONS: inadequate antibiotic treatment did not affect the outcomes of non-critically ill children with bacterial infectious diseases. Therefore, routine empirical broad-spectrum treatment may not be necessary for these cases, as it can lead to additional costs and contribute to antibiotic resistance. Larger prospective studies are needed to confirm these findings.

Asunto(s)

Antibacterianos , Infecciones Bacterianas , Tiempo de Internación , Humanos , Antibacterianos/uso terapéutico , Masculino , Femenino , Estudios Retrospectivos , Preescolar , Niño , Infecciones Bacterianas/tratamiento farmacológico , Lactante , Tiempo de Internación/estadística & datos numéricos , Resultado del Tratamiento , Factores de Riesgo , Adolescente

RESUMEN

BACKGROUND: As a population, living kidney donors have a longer life expectancy than the general population. This is generally thought to be an artifact of selection, as only healthy individuals are allowed to donate, and the operative mortality and risk of subsequent renal failure are very low. However, there may also be an additional benefit to the process, as the donor evaluation may uncover an early occult cancer or a potentially serious medical problem. While these problems may preclude donation, they may be lifesaving, as they are likely to be diagnosed and treated before the donor develops symptoms. PATIENTS AND METHODS: We looked at the incidence of occult cancer and other previously undiagnosed medical problems including renal disease, diabetes, hypertension, cardiac disease, and hepatitis C, in individuals volunteering to become a kidney donor at our center who proceeded with the evaluation between January 1, 1996 and May 31, 2011. RESULTS: Of 4088 potential donors, 19 (.46%) were discovered to have an unsuspected cancer, and 286 (7%) were found to have a previously undiagnosed medical problem. CONCLUSIONS: The living donor evaluation may lead to the early diagnosis of a life-threatening illness. This should be considered as one of the potential benefits of living donation.

Asunto(s)

Hepatitis C , Hipertensión , Trasplante de Riñón , Neoplasias , Humanos , Donadores Vivos , Neoplasias/diagnóstico

RESUMEN

Background and objective: Sickle cell disease (SCD) is now a well-established cause of renal damage. In the northeast of the Democratic Republic of Congo (DRC), SCD is common. However, sickle cell nephropathy remains unstudied in this region. Thus, this study aimed to assess renal abnormalities in SCD patients in Kisangani (northeastern DRC). Methods: This cross-sectional study included 98 sickle cell patients selected from six health facilities in Kisangani and 89 healthy non-sickle cell subjects as the control group. Based on a survey form, a clinical examination and biological tests were performed to collect data related to the sex, age, weight, height, pressure, serum creatinine, serum uric acid, urinary albumin/creatinine ratio, and hemoglobin phenotype. We used a spectrophotometer to measure serum creatinine and uricemia, the sickle SCAN® device for hemoglobin phenotype, and an automatic multifunction analyzer for urine albumin/creatinine ratio. Data were entered into an Excel file and analyzed on SPSS 20.0. Results: The mean urine albumin-to-creatinine ratio was 11.79±9.03 mg/mmol in SCD patients, significantly higher than in AA (1.69±1.89 mg/mmol) and AS (2.97±4.46 mg/mmol) subjects. The decrease in glomerular filtration rate was more observed in SCD patients with hyperuricemia compared to those with normal uric acid levels. A significantly elevated prevalence of chronic kidney disease was observed among SCD patients (87.8%) compared to 23.8% in AS and 7.7% in AA subjects. Conclusions: This study highlighted that albuminuria and chronic kidney disease are common in SCD patients in Kisangani. More studies are needed to further document these complications.

RESUMEN

Schistosomiasis has been associated with kidney diseases leading to serious health problems especially in advanced cases. Most studies have used renal biopsy, and ultrasonography in the diagnosis of renal damage among urogenital schistosomiasis affected individuals. This study assessed serum urea, creatinine, and eGFR as biochemical markers of renal abnormalities in children with urogenital schistosomiasis (Schistosoma haematobium) at a resource limited setting in Sorodofo-Abaasa in the Central Region of Ghana. A case-control study was conducted among 116 basic school children aged 9 to 17 years from January 2015 to May 2015 at Sorodofo-Abaasa in the Abura Asebu Kwamankese District of the Central Region of Ghana. A pre-tested questionnaire was used to obtain information on age, sex, guardian's occupation, water contact activities, history of gross haematuria and history of medication. Participants weight and height were measured using a bathroom scale (Zhongshan Camry Electronic Co. Ltd, Guangdong-China) and a wall-mounted ruler to the nearest 0.1 kg and 0.1 cm respectively. Approximately 4 ml of venous blood sample was collected from the median cubital vein of the study participants and used for the estimation of serum urea and creatinine levels. eGFR (mL/min/1.73 m2) was calculated using the Schwartz equation. The average ages of the cases and the controls recruited in this study were 12.7 ± 1.0 and 12. ± 2.6 years respectively. The median (minimum-maximum) did not differ between cases and controls with regards to eGFR [115.92(62.40-164.98) vs 112.50(51.82-170.36; p = 0.806], serum creatinine [57.20(28.91-84.67) vs 58.19(25.17-90.21); p = 0.876], and urea [9.82(5.80-13.74) vs 10.21(7.29-13.03); p = 0.586]. Hyperfiltration though statistically similar (p = 0.787), was observed among a higher proportion of the controls (20.5%) than observed among the cases (18.4%). This study documented no significant differences between children with light (less than 50 ova per 10 ml urine) and heavy (more than 50 ova per 10 ml urine) infection. This study documented no significant variation in the biochemical markers of renal function between the cases and controls. S. haematobium Infection intensity did not significantly alter the renal physiology of the school children studied.

RESUMEN

Proton pump inhibitors (PPIs) have wide pleiotropic action in addition to their therapeutic potential in gastroesophageal reflux diseases. Conversely, recent reports revealed a significant incidence of toxic events of PPIs including nephritis, osteoporosis, and cardiac damage. Thus, the study was designed to reconcile the deceptive contraindications. The present investigation targeted to reveal the toxic impact of sub-acute and sub-chronic administration of pantoprazole (PPZ) with different concentrations (low dose 4 mg/kg, medium-dose 8 mg/kg and high dose 16 mg/kg once a day) on normal vascular endothelium and renal tissue of rats. Vascular endothelial dysfunction (VED) was estimated by the contractility of an isolated aortic ring, nitrite/nitrate concentration, oxidative stress, and integrity of the endothelium layer. Moreover, the renal abnormalities were further confirmed by an increased level of serum creatinine, blood urea nitrogen (BUN), the incidence of microproteinuria, and structural alteration. Sub-acute administration of PPZ treatment did not produce any toxicological impact on endothelium and renal tissue. Whereas, sub-chronic administration of PPZ treatment causes moderate VED and renal dysfunction in a dose-dependent manner. Sub-chronic treatment of PPZ also influences the mitigation of NO and elevation of oxidative stress. Collecting all the evidence, it concludes that decreased nitric oxide availability and increased levels of oxidative stress may be a possible underlying mechanism of causing VED and renal abnormalities from high-dose PPZ treatment.

Asunto(s)

Aorta Torácica/efectos de los fármacos , Endotelio Vascular/efectos de los fármacos , Riñón/efectos de los fármacos , Pantoprazol/toxicidad , Inhibidores de la Bomba de Protones/toxicidad , Administración Oral , Animales , Aorta Torácica/inmunología , Aorta Torácica/metabolismo , Aorta Torácica/patología , Citocinas/sangre , Relación Dosis-Respuesta a Droga , Endotelio Vascular/inmunología , Endotelio Vascular/metabolismo , Endotelio Vascular/patología , Femenino , Riñón/inmunología , Riñón/metabolismo , Riñón/patología , Peroxidación de Lípido/efectos de los fármacos , Masculino , Nitratos/metabolismo , Nitritos/metabolismo , Ratas , Ratas Wistar , Vasodilatación/efectos de los fármacos

RESUMEN

BACKGROUND: In sub-Saharan Africa, renal abnormalities are a major public health concern, especially in children living in Schistosoma haematobium endemic areas. However, there is a dearth of data on renal abnormalities among children living in Schistosoma mansoni endemic areas. The objective of the study was to assess the prevalence of renal abnormalities among school children in a Schistosoma mansoni endemic community in Northwestern Tanzania. METHODS: A cross-sectional study was conducted between January and March 2017 among school children aged 6-13 years, attending three primary schools located along the shoreline of Lake Victoria. A single urine sample was collected from each child and screened for S. mansoni using circulating cathodic antigen and for S. haematobium eggs using a urine filtration technique. A urine dipstick was used to screen for urine protein levels, creatinine levels, microalbuminuria, and red blood cells. Venous blood was obtained for estimation of creatinine level and for malaria diagnosis. The primary outcomes were the prevalence of renal abnormalities, defined by the presence of low estimated glomerular filtration rate (eGFR), proteinuria or microalbuminuria, and hematuria in urine. RESULTS: Of 507 children included in the final analysis, 49.9% (253/507) were male with a mean age of 8.51 ± 1.3 years. Overall, 64.0% (326/507) of the children were infected with S. mansoni, and 1.6% (8/507) of the children were infected with S. haematobium. A total of 71 (14%) of the children had proteinuria, 37 (7.3%) had hematuria, and 8 (1.6%) had a low estimated glomerular filtration rate (eGFR). Overall prevalence of renal abnormalities was 22.9%. Renal abnormalities (proteinuria) were associated with S. mansoni infection (OR = 4.9, 95% CI 2.1-11.2, p < 0.001) and having red blood cells in urine (OR = 5.3, 95% CI 2.5-11.2, p < 0.001). CONCLUSION: Twenty-two percent of school children who participated in this study had renal abnormalities associated with S. mansoni infection. Given the high prevalence of S. mansoni, longitudinal epidemiological surveillance is warranted to measure the burden of renal abnormalities and assess the impact of the praziquantel treatment on these abnormalities.

RESUMEN

OBJECTIVES: Microtia is a congenital ear anomaly that hinders quality of life. Microtia patients, both syndromic and in isolation, may be at-risk for significant renal anomalies that can go undetected at the time of birth. The goal of this study was to characterize the prevalence of renal anomalies among microtia patients at our institution in order to guide optimal patient management. Current guidelines suggest performing a renal ultrasound when there is presence of preauricular pits and ear anomalies in association with dysmorphic features, but not in cases of isolated microtia and atresia. DESIGN: A retrospective review of 237 children with microtia was conducted from 2001 through 2018 at our tertiary-care pediatric institution, of which 98 also had a documented renal ultrasound. Patients were identified as syndromic or non-syndromic. Data endpoints included renal ultrasounds performed, structural anomalies found, and follow-up. RESULTS: Among the 237 patients, 98 had received renal ultrasounds. 12% of the total cohort was found to be syndromic, the most common being Goldenhar. Structural anomalies were detected in 24% of the 98 patients that underwent ultrasound and included disorders such as pelviectasis, renal ectopia, duplicated collecting systems, and renal agenesis. A third of patients with anomalies required follow-up with nephrology for chronic kidney disease or renal failure. Of note, 21% of non-syndromic patients and 43% of syndromic patients screened had an abnormality on ultrasonography. CONCLUSIONS: Children with microtia are at a significant risk of structural renal abnormalities, even when isolated outside of a genetic syndrome. We recommend the strong consideration of performing a screening renal ultrasound in all patients with microtia. Prospective data would be helpful in developing future clinical guidelines regarding the utility of screening ultrasonography.

Asunto(s)

Anomalías Congénitas/epidemiología , Microtia Congénita/epidemiología , Enfermedades Renales/congénito , Riñón/anomalías , Anomalías Urogenitales/epidemiología , Niño , Anomalías Congénitas/diagnóstico por imagen , Femenino , Humanos , Incidencia , Riñón/diagnóstico por imagen , Enfermedades Renales/diagnóstico por imagen , Enfermedades Renales/epidemiología , Masculino , Tamizaje Masivo , Prevalencia , Calidad de Vida , Estudios Retrospectivos , Síndrome , Texas/epidemiología , Ultrasonografía , Anomalías Urogenitales/diagnóstico por imagen

RESUMEN

BACKGROUND: Copy number variations (CNVs) involving the 17q12 region are associated with a broad range of clinical phenotypes. Deletion of the 17q12 chromosome results in structural or functional abnormalities in the kidney and urethra, type 5 diabetes (MODY5), and neurodevelopmental or neuropsychiatric disorders. Microduplication of 17q12 is rare and is associated with an increased risk of epilepsy and mental retardation. We studied the prenatal diagnosis of 17q12 microduplication and microdeletion syndrome in fetuses with congenital renal abnormalities. CASE PRESENTATION: We conducted a retrospective analysis of prenatal diagnoses in our hospital from January 2016 to April 2018. Abnormal renal ultrasound findings were present in 126 fetuses and the incidence of chromosomal abnormalities was 10.32%(13/126). Conventional karyotyping detected 7 of 126 fetuses as aneuploid (5.56%). In addition, chromosome microarray analysis (CMA) detected 6 fetuses(4.76%) with copy number variations (CNVs), of which 5 were shown to have 17q12 microdeletion syndrome and 1 had 17q12 microduplication syndrome. We followed up these pregnant women. The results of the testing had a significant impact on pregnancy outcome. The phenotypes of 17q12 microdeletions and microduplications vary widely, affecting patients in different ways, such as language delays, social deficiencies, and even abortion. CONCLUSIONS: The characteristics of 17q12 microdeletions and microduplications are so vague that the condition is often misdiagnosed or missed. This study demonstrated that karyotype analysis combined with CMA can significantly improve the diagnostic rate in prenatal diagnosis of CNVs, which can provide evidence for genetic counseling in such pregnancies.

RESUMEN

The different ailments of heart including myocardial infarction (MI) and ischemic heart diseases are the foremost trigger of high mortality across the world which is instigated by sedentary life style, chronic hyperglycaemia and atherosclerosis. Albeit strenuous exercise itself induces temporary hypoxia which causes myocardial damage and this vitiosus circulus is poorly understood and has been assumed difficult to break. Present investigation targets temporal dynamics of aerobic exercise treatment induced preconditioning against diabetes associated pre- and post- myocardial injury. The persisting high blood sugar level leads to several biochemical alterations at pre- and post-MI phase. Here, we present the assessment of temporal expression of cardiac biomarkers (CKMB, LDH, cTnI and serum nitrite/nitrate), oxidative stress (myocardial TBARS and reduced NBT), inflammatory cytokines (IL-6, TNF-α and IL-10), renal biomarkers (BUN, serum creatinine and microproteinuria) and structural alterations of cardio-renal tissue. Aerobic exercise preconditioning significantly downregulate the pathological events or biomarkers and upsurge the physiological biomarkers at both pre- and post-MI phase. The attenuation or returning of pathological makers to lowest level at different time points endorses the therapeutic management of aerobic exercise against diabetic MI. Furthermore, the temporal expression of various cardio-renal biomarkers pattern elucidates that aerobic exercise preconditioning boost the strength and consolidate the cardiac muscles to work under stress. Despite the presence of traditional knowledge about health benefits of aerobic exercise, it is yet to be brought into the clinical arena. In spite of few impending challenges subjected to additional investigations, aerobic exercise preconditioning shows a high degree of promise.

Asunto(s)

Biomarcadores/análisis , Diabetes Mellitus Experimental/fisiopatología , Precondicionamiento Isquémico Miocárdico/métodos , Infarto del Miocardio/complicaciones , Daño por Reperfusión Miocárdica/prevención & control , Condicionamiento Físico Animal , Animales , Lípidos/análisis , Estrés Oxidativo , Ratas

RESUMEN

Atmis B, Karabay-Bayazit A, Melek E, Bisgin A, Anarat A. Renal features of Bardet Biedl syndrome: A single center experience. Turk J Pediatr 2019; 61: 186-192. Bardet Biedl syndrome (BBS), is a multisystemic disorder which is described as a ciliopathy. BBS is a rare autosomal recessive disorder and 21 different BBS genes have been defined to date. BBS is characterized with dysmorphic extremities, retinitis pigmentosa, obesity, hypogenitalism, intellectual disabilility and renal structural abnormalities. Renal symptoms in patients with BBS, are nonspecific and often undetected until end-stage renal disease. Here, we were reported 23 children with BBS (12 females, 11 males) with renal abnormalities from a single center and defined their features. Age at diagnosis were very variable (2 days-16 years). Median age at diagnosis was 84 months. Mean follow-up period was 42 months. All 23 children had urinary tract abnormalities on renal ultrasonography. These abnormalities were polycysts (34.8%), hyperechogenic kidneys (34.8%), fetal lobulation (21.7%), hypoplasia on at least one kidney (21.7%) and hydronephrosis in at least one kidney (17.4%). Vesicoureteral reflux and neurogenic bladder detected 11.1% and 22.2% of patients who recieved a voiding cystourethrogram, respectively. Proteinuria was found in 39 % of patients. Hypertension was defined in 21.7% of patients. Six of 23 children (26%) in our cohort had proven mutations in BBS genes. Five of them (83.3%) had homozygous mutations in BBS10 gene and one of them had homozygous mutation in BBS2 gene. All of 23 children had retinitis pigmentosa, twenty two of them (95.6%) had learning disabilities/cognitive impairment and seventeen of them (82.6%) had obesity. Renal involvement is now accepted as a cardinal feature and the most important factor causing mortality in BBS.

Asunto(s)

Síndrome de Bardet-Biedl/complicaciones , Riñón/anomalías , Chaperoninas/genética , Niño , Trastornos del Conocimiento/etiología , Femenino , Estudios de Seguimiento , Humanos , Hidronefrosis/diagnóstico por imagen , Hipertensión/etiología , Riñón/diagnóstico por imagen , Discapacidades para el Aprendizaje/etiología , Masculino , Mutación , Obesidad/complicaciones , Proteínas/genética , Proteinuria/etiología , Retinitis Pigmentosa/etiología , Ultrasonografía , Vejiga Urinaria Neurogénica/diagnóstico por imagen , Urografía , Reflujo Vesicoureteral/diagnóstico por imagen

RESUMEN

OBJECTIVE: Renal abnormalities are commonly considered in the work up of pediatric patients with external ear malformations. However, there is little consensus regarding an appropriate renal screening protocol for patients with microtia. We sought to characterize renal abnormalities detected on ultrasonography in pediatric patients with microtia. METHODS: We conducted a retrospective cohort study of pediatric patients diagnosed with microtia who underwent renal ultrasound from 1991 to 2014 at a single tertiary academic institution. Renal ultrasound reports and medical records were reviewed to assess for renal abnormalities and to determine whether patients required specialist follow-up or interventions. Audiograms and otolaryngology notes were used to determine patterns of hearing loss. The following additional information was recorded from the electronic medical records: patient sex, microtia grade (I-IV), microtia laterality, and known associated syndromes. Characteristics were compared between those who did and did not have renal ultrasound findings using Fisher's exact test. Univariate logistic regression analysis was performed to determine factors associated with renal ultrasound findings. RESULTS: The majority of patients in this cohort were syndromic (n = 51, 64%) with grade III microtia (n = 46, 58%) and conductive hearing loss (n = 58, 72%). Syndromic children with microtia demonstrated a higher crude rate of renal ultrasound abnormalities (22%) than children with isolated microtia (7%). Of these patients, 69% required specialist follow-up. Univariate logistic regression analysis did not identify predictors that were significantly associated with renal ultrasound findings. CONCLUSION: Fairly high rates of abnormalities in syndromic and non-syndromic patients may warrant screening renal ultrasound in all patients with microtia, especially given the high percentage of findings requiring renal follow-up. A prospective study to formally evaluate screening efficacy is needed.

Asunto(s)

Microtia Congénita/epidemiología , Enfermedades Renales/diagnóstico por imagen , Riñón/anomalías , Riñón/diagnóstico por imagen , Niño , Estudios de Cohortes , Microtia Congénita/clasificación , Femenino , Pérdida Auditiva Conductiva/epidemiología , Perdida Auditiva Conductiva-Sensorineural Mixta/epidemiología , Humanos , Enfermedades Renales/epidemiología , Masculino , Estudios Retrospectivos , Ultrasonografía

RESUMEN

BACKGROUND: Fanconi anaemia (FA) is an inherited disease with bone marrow failure, variable congenital and developmental abnormalities, and cancer predisposition. With improved survival, non-haematological manifestations of FA become increasingly important for long-term management. While renal abnormalities are recognized, detailed data on patterns and frequency and implications for long-term management are sparse. METHODS: We reviewed clinical course and imaging findings of FA patients with respect to renal complications in our centre over a 25-year period to formulate some practical suggestions for guidelines for management of renal problems associated with FA. RESULTS: Thirty patients including four sibling sets were reviewed. On imaging, 14 had evidence of anatomical abnormalities of the kidneys. Two cases with severe phenotype, including renal abnormalities, had chronic kidney disease (CKD) at diagnosis. Haematopoietic stem cell transplantation was complicated by significant acute kidney injury (AKI) in three cases. In three patients, there was CKD at long-term follow-up. All patients had normal blood pressure. CONCLUSIONS: Evaluation of renal anatomy with ultrasound imaging is important at diagnostic workup of FA. While CKD is uncommon at diagnosis, our data suggests that the incidence of CKD increases with age, in particular after haematopoietic stem cell transplantation. Monitoring of renal function is essential for management of FA. Based on these long-term clinical observations, we formulate some practical guidelines for assessment and management of renal abnormalities in FA.

Asunto(s)

Lesión Renal Aguda/terapia , Anemia de Fanconi/terapia , Riñón/anomalías , Cuidados a Largo Plazo/normas , Insuficiencia Renal Crónica/terapia , Lesión Renal Aguda/diagnóstico , Lesión Renal Aguda/epidemiología , Lesión Renal Aguda/etiología , Niño , Preescolar , Progresión de la Enfermedad , Anemia de Fanconi/complicaciones , Anemia de Fanconi/diagnóstico , Femenino , Estudios de Seguimiento , Trasplante de Células Madre Hematopoyéticas/efectos adversos , Humanos , Incidencia , Lactante , Riñón/diagnóstico por imagen , Cuidados a Largo Plazo/métodos , Masculino , Guías de Práctica Clínica como Asunto , Insuficiencia Renal Crónica/diagnóstico , Insuficiencia Renal Crónica/epidemiología , Insuficiencia Renal Crónica/etiología , Estudios Retrospectivos , Ultrasonografía

RESUMEN

INTRODUCTION: Renal involvement in leprosy has previously been described in the literature and can include amyloidosis, glomerulonephritis, nephrosclerosis, tubulointerstitial nephritis, and granulomas. AIM: To evaluate renal involvement in Turkish patients with leprosy. MATERIAL AND METHODS: In total, 32 patients with lepromatous leprosy but without any co-morbidities and 35 healthy control subjects were evaluated for renal involvement at the Elazig Training and Research Hospital in Turkey. The laboratory tests and radiological results concerning renal function were taken from both the patients' medical records and from current examinations. RESULTS: The levels of creatinine, urea, and leukocyturia in the lepromatous leprosy patients were significantly higher than in the controls (p < 0.001, p < 0.001; p = 0.001, p < 0.01; p = 0.036, p < 0.05, respectively). No significant differences in the proteinuria, hematuria, sodium, or potassium levels were found between the leprosy and control groups (p > 0.05). On ultrasonographic examination, the prevalence of renal cortical cysts and renal cortical echogenicity in the leprosy patients was significantly higher than in the controls (p = 0.020, p < 0.05, respectively). There were no significant differences in terms of nephrolithiasis, parapelvic cysts, or hydronephrosis between the leprosy and control groups (p > 0.05). CONCLUSIONS: Evaluating the renal function in all leprosy patients is important to detect abnormalities and to prevent renal failure, which remains a potential cause of death in this disease.

RESUMEN

Wilson disease (WD) is a rare disorder of copper metabolism that presents mainly with hepatic and neuropsychiatric features. Copper accumulates not only in the liver and brain, but also in other organs. Liver injury can also be the cause of secondary impairment of other tissues. Therefore, the clinical manifestation of WD may be renal, cardiac, skin, osteoarticular, or endocrinologic and include other organ disturbances. Renal abnormalities include tubular dysfunction (e.g., renal tubular acidosis, aminoaciduria) and nephrolithiasis. Bone demineralization is a common manifestation in patients with WD. Cardiac injury may include arrhythmia, cardiomyopathy, and autonomic dysfunction. Different endocrine system manifestations, such as infertility or repeated miscarriages, growth and puberty disturbances, and hypoparathyroidism, are observed. Other important clinical aspects of WD include pancreas involvement, immunologic abnormalities, the presence of lipomas, and skin changes. Although other organ involvement is not common in WD and usually not severe, delayed diagnosis may lead to irreversible changes in organs and tissues. Therefore, awareness of other possible WD presentations is important in the differential diagnosis of WD.

Asunto(s)

Degeneración Hepatolenticular/complicaciones , Enfermedades Óseas Metabólicas/etiología , Encéfalo/metabolismo , Cardiomiopatías/etiología , Cobre/metabolismo , Diagnóstico Diferencial , Enfermedades del Sistema Endocrino/etiología , Degeneración Hepatolenticular/diagnóstico , Humanos , Enfermedades Renales/etiología , Hígado/metabolismo , Enfermedades Cutáneas Metabólicas/etiología

RESUMEN

OBJECTIVES: Our objective was to describe the association between unilateral fetal renal abnormalities and other major anomalies that were not apparent in the second trimester. METHODS: A review of the ultrasound database identified fetuses with suspected unilateral renal agenesis, unilateral multicystic dysplastic kidney, and renal ectopia from 2005 to 2014. Neonatal records were reviewed to identify anomalies not suspected in the second trimester, and postnatal imaging studies were reviewed. Categorical data were compared by &x003C7;(2) analysis and the Fisher exact test. RESULTS: We identified 102 cases, including 36 with suspected renal agenesis, 28 with suspected multicystic dysplastic kidney, and 38 with suspected renal ectopia. There were 8 cases (7.8%) with major anomalies not suspected in the second trimester. In 5 cases (4.9%), there were no associated findings in the second trimester. There were no significant differences in the rates of unsuspected abnormalities between the 3 groups. There was a trend toward a higher rate of unsuspected anomalies in the cases with a single umbilical artery compared to those with a 3-vessel cord (28.6% vs 6.3%; P= .09). CONCLUSIONS: In fetuses with unilateral renal abnormalities, major anomalies that were not suspected in the second trimester were uncommon. However, patients should be aware of the possibility that other major anomalies could subsequently be identified, and the outcome may depend on more than postnatal renal function.

Asunto(s)

Anomalías Múltiples/diagnóstico por imagen , Anomalías Múltiples/epidemiología , Enfermedades Renales/congénito , Enfermedades Renales/epidemiología , Riñón/anomalías , Riñón/diagnóstico por imagen , Femenino , Humanos , Incidencia , Riñón/embriología , Enfermedades Renales/diagnóstico por imagen , Masculino , New York/epidemiología , Embarazo , Segundo Trimestre del Embarazo , Factores de Riesgo , Ultrasonografía Prenatal/estadística & datos numéricos

RESUMEN

La Infección del Tracto Urinario (ITU) es una de las patologías infecciosas más frecuentes en pediatría. Como factores de riesgo más relevantes se encuentran el reflujo vesicoureteral y las malformaciones urogenitales. El estudio con imágenes es imprescindible para detectar posibles anomalías del tracto urinario, demostrando la ecografía la mejor correlación costo-beneficio. Objetivo: Describir las características y resultados de las ecografías renales y vesicales de pacientes hospitalizados por ITU en el Hospital San Martín de Quillota (HSMQ) en el año 2014. Material y métodos: Estudio descriptivo; estudio de casos, se incluyó a niños hospitalizados en el servicio de pediatría del HSMQ durante el año 2014, ingresados con diagnóstico de ITU. Resultados: Ingresos por ITU: 99 pacientes, excluyéndose 27 casos con un número total de 72 pacientes; de estos, 71 % fueron de sexo femenino, 76% lactantes. Del total de ecografías, 55% resultaron normales, 3 1% compatibles con Pielonefritis Aguda (PNA) y 14% con alteración anatómica, siendo lo más frecuente el doble sistema pielocalicial. Discusión: Hubo similitud entre la información entregada por la literatura de un 12 a 15% de ecografías alteradas y un 14% en este estudio.

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The Urinary Tract Infection (UTI) is one of the most common infectious diseases in children. Vesicouretral reflux and urogenital malformations are amongst the most significant risk factors. The imagenological studies are essential to detect possible anatomical or functional urological abnormalities. Within them, renal ultrasound (US) has the best cost-benefit relation. Objective: Describe the characteristics and results of renal and vesical ultrasound done on patients hospitalized in Hospital San Miguel de Quillota (HSMQ) during 2014, diagnosed with UTI. Material and methods: Descriptive study, cases study includes hospitalized children under the diagnose of UTI in the Pediatric Service of HSMQ during 2014. Results: 99 patients entered with the diagnose of UTI, 27 cases were excluded,with 72 patient which met the inclusion criteria:71% of them were female and 76% were infants. From the total of US, 55% were normal, 3 1% showed acute pyelonephritis (APN) and 14% anatomical abnormalities, where duplicated collecting system was the highest frequency condition. Discussion: There were similar results in literature that showed 12-15% of altered ultrasound compared to 14% obtained in this study.

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Humanos , Lactante , Niño , Ultrasonografía/métodos , Infecciones Urinarias , Enfermedades Urológicas/diagnóstico

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BACKGROUND: Functional and morphological renal lesions have been widely described in leprosy for decades. Nevertheless few studies have assessed renal function pre- and during treatment after the advent of multidrug therapy (MDT). METHODS: This is a prospective study involving 189 consecutive patients, with all forms of leprosy (Ridley-Jopling scale). Laboratory (serum urea and creatinine, estimated GFR, urinalysis, microalbuminuria, urinary RBP) and clinical features of renal disease were evaluated previously and after onset (3 and 8 months later) of MDT. RESULTS: One hundred and eighty-nine patients (M 1.8: F 1; mean age 44 ± 16 years) were included just after diagnosis of leprosy and before the introduction of MDT. Mean time until manifestation of symptoms and/or signs of leprosy was 29 ± 56 months (25 days-480 months). Microhematuria and microalbuminuria were detected in 7.5% and 9.6% of the cases, respectively. Elevated serum creatinine was detected in 34% pre-MDT; this was statistically more frequent in males, hypertensive and frequent users of non-steroidal anti-inflammatory drugs (NSAID), as well as in patients with erythema nodosum lepromatosum, 45.5% by the time of diagnosis, 18% after 3 months and 9% after 8 months of MDT (p = 0.039). CONCLUSIONS: Our results suggest that functional renal lesions in leprosy are currently mild and predominantly of glomerular origin, in opposition to the severe involvement described in the past. This improved outcome of renal disease secondary to leprosy is possibly due to the advent of MDT and effective treatment of episodes of reaction, leading to shorter periods of active infectious disease.

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Creatinina/sangre , Glomerulonefritis/sangre , Fallo Renal Crónico/sangre , Riñón/fisiopatología , Lepra/complicaciones , Lepra/diagnóstico , Mycobacterium leprae/patogenicidad , Adolescente , Adulto , Anciano , Brasil , Niño , Quimioterapia Combinada/métodos , Femenino , Tasa de Filtración Glomerular , Humanos , Lepra/tratamiento farmacológico , Masculino , Persona de Mediana Edad , Estudios Prospectivos , Resultado del Tratamiento , Urinálisis , Adulto Joven

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Introducción: Los niños con malformaciones menores del pabellón auricular son estudiados habitualmente con ecografías renal y vesical, existiendo controversias en cuanto a la validez de esta práctica. Objetivo: Determinar si la prevalencia de malformaciones del tracto urinario es mayor entre niños con mamelones preauriculares aislados que entre aquellos que no los presentan. Población, material y métodos: Entre junio de 1996 y junio de 2006, 92 niños con mamelones preauriculares fueron estudiados con ecografía renal y vesical. Se incluyeron en la evaluación a niños sanos con mamelón preauricular aislado. Se excluyeron aquellos con hoyuelos preauricular , una o más malformaciones menores asociadas, síndromes de malformaciones congénitas múltiples o anomalías mayores identificadas previamente. Ochenta y ocho niños cumplieron con los criterios de inclusión. Para la comparación estadística se utilizó un grupo control histórico de 300 lactantes sanos sin mamelón, estudiados en forma no concurrente con el grupo estudio y que mostró una prevalencia del 1% de malformaciones urinarias. Se realizó una prueba de hipótesis exacta para una proporción utilizando un nivel de significación del 5%, con el software Epidat 3.1. Resultados: Tres de los 88 niños (3,4%) con malformaciones preauriculares aislados presentaron malformaciones renales: ectopia renal derecha e hidroureteronefrosis detectada prenatalmente; agenesia renal izquierda, existiendo antecedentes familiares de malformaciones renales y un quiste cortical simple. No se detectaron diferencias estadísticamente significativas con el grupo considerado control histórico (p=0.11). Conclusión: La prevalencia de malformaciones renales similar entre los niños sanos con y sin mamelones preauriculares aislados, por lo que la indicación rutinaria de ecografías renal y vesical no sería necesaria.

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Introduction: The renal and vesical ultrasound in every patient with preauricular tags is a common practice.However, the benefit of this indication is controversial. Objective: To determine whether the prevalence of urinarytract abnormalities in patients with isolated preauricular tags is higher than in those without preauricular tags.Population, material and methods: From June 1996 to June 2006, 92 patients with preauricular tags had beenreferred consecutively for renal and vesical ultrasound. Healthy infants with isolated preauricular tags were included.Patients who had a preauricular tag associated with other ear anomalies and/ or major anomalies or malformative syndromes were excluded. Eighty eight infants achieved the inclusion criteria. A historical control group of 300 healthy infants, with a prevalence of urinary tract abnormalities of 1%, was considered for a statistical comparison. An exacthypothesis test for one proportion was performed, with the software Epidat 3.1, setting a signification level of 5%.Results: Three out of 88 infants (3.4%) with isolated preauricular tags had renal abnormalities: ectopic kidney withhidroureteronephrosis detected by prenatal ultrasound, solitary kidney with a positive family history of renal malformations and a simple cortical renal cyst. No significant statisticaldifference was found in comparison with the historical control group (p: 0.11). Conclusion: The prevalence of renal malformations was similar in the groups with and without preauricular tags. Renal and vesical ultrasounds seem not to be mandatory in infants with isolated preauricular tags.

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Anomalías Congénitas/diagnóstico , Anomalías Congénitas/etiología , Oído Externo/anomalías , Riñón/anomalías , Sistema Urinario/anomalías , Ultrasonografía , Vejiga Urinaria/anomalías

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Objective To assess the safety and feasibility of minimally invasive percutaneous nephrolithotomy(mPCNL) in the treatment of solitary kidney calculi.Method Thirty-eight patients with solitary kidney calculi,treated with mPCNL from Jan 1998 to May 2004,were retrospectively investigated.Results Of 38 cases,there was 6 cases with pyonephrosis and 32 cases with the treatment of one stage percutaneous nephrolithotomy.With the treatment of 1 to 3 tract was used for calculus removed in all cases,a total of 86% stone-free rate was achieved.Only one case with pyonephrosis suffered from postoperative infection shock while one stage minimally invasive percutaneous nephrolithotomy was performed.Another one patient with diabetes suffered from postoperative ketoacidosis and no major complications were noted in other patients.Conclusion Minimally invasive percutaneous nephrolithotomy is safe and effective in the treatment of solitary kidney calculi.