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OBJECTIVES: It is safe to use recombinant growth hormone in children. Studies have shown it to be effective and safe, except for a few side effects in the short and long term after treatment. The present study investigated the presence of hypertension in pediatric patients receiving growth hormone treatment using 24â¯h ambulatory blood pressure monitoring (ABPM). METHODS: This study is a single-center, retrospective study. Eighty-four patients aged 5-16 years who received growth hormone treatment for at least 3 months, who underwent 24â¯h ABPM were analyzed. They were compared with 67 patients who had no risk factors for hypertension. RESULTS: In the study, 84 rhGH-treated patients (45.2â¯% male, 54.8â¯% female) and 67 healthy control groups (49.3â¯% male, 50.7â¯% female) were analyzed. The mean age of the patient group was 10.83±2.85 years and the mean age of the healthy control group was 13.1±2.93 years. The diagnostic classification of the patients receiving treatment was as follows: 66.6â¯% (n=56) partial growth hormone deficiency, 22.6â¯% (n=19) growth hormone deficiency, 7.1â¯% (n=6) bioactive growth hormone, 2.3â¯% (n=2) idiopathic short stature, 1.1â¯% (n=1) low birth weight for gestational age (SGA). Body mass index was significantly lower in the treated group (p=0.013). The duration of treatment was 6.04±4.9 months. Daytime diastolic blood pressure was significantly lower in the treated group (p=0.001). There was no correlation between BMI and ABPM parameters in the treatment group and the control group. CONCLUSIONS: The present study shows that growth hormone treatment is safe in terms of high blood pressure.
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Hormona de Crecimiento Humana , Hipertensión , Humanos , Masculino , Niño , Femenino , Adolescente , Hormona del Crecimiento , Monitoreo Ambulatorio de la Presión Arterial , Presión Sanguínea/fisiología , Estudios Retrospectivos , Hormona de Crecimiento Humana/uso terapéuticoRESUMEN
BACKGROUND: Ventricular repolarization (VR) increases the risk of sudden cardiac death due to ventricular arrhythmia. We aimed to evaluate the blood pressure (BP) parameters affecting VR in obese children. METHODS: Obese (BMI ≥ 95p) and healthy children ≥ 120 cm between January 2017 and June 2019 were included. Demographic and laboratory data, peripheral and central BPs evaluated by a device capable of ambulatory blood pressure monitoring (ABPM), and pulse wave analysis were assessed. Electrocardiographic ventricular repolarization indices, left ventricular mass index (LVMI), and relative wall thickness (RWT) were calculated. RESULTS: A total of 52 obese and 41 control patients were included. Uric acid, triglyceride, total cholesterol, LDL, and ALT values, systolic and diastolic office BPs, 24-h, daytime and nighttime systolic and mean arterial BPs, daytime diastolic BP SDS levels, daytime and nighttime systolic loads, daytime diastolic load, 24-h, daytime and nighttime central systolic and diastolic BPs, and pulse wave velocity values were significantly higher, whereas 24-h, daytime and nighttime AIx@75 were similar between the groups. fT4 levels of obese cases were significantly lower. QTcd and Tp-ed were higher in obese patients. Although RWT was higher in obese cases, LVMI values and cardiac geometry classifications were similar. The independent factors affecting VR in obese cases were younger age and higher diastolic load at night (B = - 2.83, p = 0.010; B = 0.257, p = 0.007, respectively). CONCLUSION: Obese patients have higher peripheral and central BP, arterial stiffness, and higher VR indices that develop before an increase in LVMI. It would be useful to prevent obesity from an early age and follow up nighttime diastolic load to control VR associated sudden cardiac death in obese children. A higher resolution version of the Graphical abstract is available as Supplementary information.
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Hipertensión , Obesidad Infantil , Humanos , Niño , Presión Sanguínea/fisiología , Monitoreo Ambulatorio de la Presión Arterial , Obesidad Infantil/complicaciones , Análisis de la Onda del Pulso , Hipertrofia Ventricular IzquierdaRESUMEN
OBJECTIVE: To evaluate the effects of non-E. coli or extended-spectrum ß-lactamase-positive (ESBL-positive) microorganism growth in the first febrile urinary tract infection (UTI) of infants on laboratory findings or renal parenchymal damage presenting the severity of inflammation, anatomic abnormalities defined by imaging studies, and recurrent UTIs in the follow-up period. METHODS: The data of patients aged between 2 and 24 mo and followed up for at least 6 mo with febrile UTI guideline of the authors' pediatric-nephrology clinic, were retrospectively analyzed. Ultrasonography was performed in all the cases at the time of UTI and dimercaptosuccinic-acid (DMSA) at least 4 mo after the infection. Voiding cystourethrography (VCUG) was performed only if ultrasonography findings were abnormal, the uptake deformity was detected in DMSA scan, or the patients experienced recurrent UTIs. The patients were grouped concerning E. coli or non-E. coli and ESBL-PB or non-ESBL-BP growth in the urine cultures. RESULTS: There were 277 infants followed up for 28.55 ± 15.24 (6-86) mo. The causative microorganisms were non-E. coli in 73 (26.4%) and ESBL-PB in 58 (20.9%) cases. CRP values, pyuria, and leukocyte-esterase positivity were significantly higher in UTIs caused by E. coli compared to non-E. coli bacteria. All clinical and laboratory findings were similar between the ESBL-PB and non-ESBL groups, but abnormal ultrasonography findings were more common in non-E. coli group. CONCLUSION: E. coli causes more severe inflammation, but non-E. coli infections are more frequently associated with ultrasound abnormalities. However, ESBL production did not affect either laboratory or radiological findings in the present cohort.
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Infecciones por Escherichia coli , Infecciones Urinarias , Niño , Humanos , Lactante , Preescolar , Adolescente , Adulto Joven , Adulto , Infecciones por Escherichia coli/tratamiento farmacológico , Infecciones por Escherichia coli/microbiología , Escherichia coli , Estudios Retrospectivos , Estudios de Seguimiento , beta-Lactamasas/farmacología , Infecciones Urinarias/microbiología , Inflamación , Antibacterianos/uso terapéutico , Factores de RiesgoRESUMEN
OBJECTIVES: Renal transplant recipients are at risk for ventricular arrhythmia and sudden death. To assess that risk, we compared the ventricular repolarization markers of pediatric renal transplant recipients with those of healthy children. MATERIALS AND METHODS: We included 30 children and adolescents who were followed for at least 6 months after renal transplant; 30 age- and sex-matched children were included for the control group. Demographic features, medications, and laboratory findings were recorded. Blood pressure measurements, ventricular repolarization indexes including QT dispersion, corrected QT dispersion, T-wave peak-to-end interval dispersion, the T-wave peak-to-end interval∕QT ratio, the T-wave peak-to-end interval∕corrected QT ratio, left ventricular mass index, and relative wall thickness were compared between groups. In addition, the correlations of ventricular repolarization indexes with other variables were evaluated. RESULTS: Blood pressure standard deviation scores, the mean heart rate, QT dispersion, corrected QT dispersion, the T-wave peak-to-end interval∕QT ratio, the T-wave peak-to-end interval/corrected QT ratio, left ventricular mass index, and relative wall thickness values were significantly higher in renal transplant patients, whereas T-wave peak-to-end interval dispersion, ejection fraction, and fractional shortening were similar between groups. Although ventricular repolarization indexes were similar in patients with and without left ventricular hypertrophy, only corrected QT dispersion was significantly higher in patients with hypertension (P = .006). The only variable that significantly predicted prolonged corrected QT dispersion was the systolic blood pressure standard deviation score (P = .005, ß = .403). CONCLUSIONS: Ventricular repolarization anomalies, hypertension, left ventricular hypertrophy, and cardiac geometry irregularity may be observed after renal transplant in pediatric recipients despite acceptable allograft functions and normal serum electrolyte levels. Control of systolic blood pressure would decrease the risk of ventricular repolarization abnormalities, namely, the corrected QT dispersion. Follow-up of cardiovascular risks with noninvasive methods is recommended in all pediatric renal transplant recipients.
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Hipertensión , Trasplante de Riñón , Humanos , Niño , Adolescente , Electrocardiografía , Hipertrofia Ventricular Izquierda/diagnóstico , Hipertrofia Ventricular Izquierda/etiología , Trasplante de Riñón/efectos adversos , Arritmias Cardíacas/diagnóstico , Arritmias Cardíacas/etiología , Hipertensión/diagnóstico , Hipertensión/etiología , Medición de RiesgoRESUMEN
BACKGROUND: We aimed to evaluate the predictability of extended-spectrum beta-lactamase (ESBL)-producing bacteria (PB) with inflammation markers and hemogram parameters as neutrophil-lymphocyte-ratio (NLR), platelets-lymphocyte-ratio (PLR) and mean-platelet-volume (MPV) in infants with febrile urinary tract infection until the urine cultures are resulted. METHODS: Infants between 2-24 months hospitalized for the first febrile urinary tract infections were grouped as those infected with ESBL-PB and non-ESBL-PB. The demographic and laboratory data (inflammation markers and hemogram parameters) and the ultrasonographical findings were compared between the two groups. RESULTS: A total of 232 patients were included in the study. The mean age was 8.82 ± 5.68 (2-23) months and 114 (49%) of them were female. Escherichia coli was the most common isolated bacteria (79%) followed by Klebsiella pneumoniae (15.5%) in urine cultures. There were 88 patients in ESBL-PB infected group and 144 patients in the non-ESBL-PB group. The hematologic parameters such as white blood cell count (WBC) count, NLR, PLR, MPV and procalcitonin (PCT) were similar between the two groups. Only the rate of ultrasonographic abnormalities was significantly higher in infants infected with ESBL-PB (p=0.012). The risk of ESBL-PB positivity in urine cultures increased with age (OR 1.068, 95% CI 1.002-1.139, p=0.045), PCT (OR 1.094, 95% CI 1.011-1.184, p=0.025), and ultrasonographic abnormalities (OR 3.981, 95% CI 1.792-8.845, p=0.001). CONCLUSIONS: Platelet counts, WBC, MPV, NLR, PLR, and PCT were not reliable markers, however having an ultrasonographic abnormality is the most important independent risk factor for prediction of infection with ESBL-PB.
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Infecciones por Escherichia coli , Infecciones Urinarias , Adolescente , Antibacterianos/uso terapéutico , Bacterias , Niño , Preescolar , Escherichia coli , Infecciones por Escherichia coli/tratamiento farmacológico , Femenino , Humanos , Lactante , Inflamación , Masculino , Estudios Retrospectivos , Factores de Riesgo , Infecciones Urinarias/tratamiento farmacológico , beta-LactamasasRESUMEN
BACKGROUND: Chronic kidney disease (CKD) may lead to increase in serum levels of peptide hormones as a result of changes in peripheral metabolism. The pathogenesis of uremic hyperprolactinemia in CKD is not fully understood. Plasma prolactin levels are elevated in women, pubertal girls, and also in men with chronic kidney disease. But this is not comon in prepubertal boys. Also in prepubertal children and postmenopausal women, hyperprolactinemia rarely results in galactorrhea. We aimed to discuss hyperprolactinemia and galactorrhea in a 12-year-old male with CKD. CASE: A twelve-year-old boy with chronic kidney disease (CKD) suffered from bilateral galactorrhea. He was on follow-up at Pediatric Nephrology Department from the age of two due to bilateral dysplastic kidney. On physical examination, his weight was - 0.59 SDS, height was -2.82 SDS, Blood pressure was 115 / 72 (75p), stretched penis length was 6 cm, testicular volume was 3mL / 3mL, pubic hair was Tanner Stage 1, breast examination did not reveal plaque on bilateral breast. He was receiving recombinant erythropoietin, sodium bicarbonate, polystyrene sulfonate, calcium acetate, and calcitriol treatments. Glomerular filtration rate was 23ml/min/1.73 m2 (CKD stage IV). Serum prolactin (PRL) was > 200 µg/L (N, 2.64-13.13). The pituitary adenoma was excluded with pituitary and cranial magnetic resonance imaging (gadolinium). Cabergoline (0.5 mg/ twice weekly) was initiated to decrease PRL levels and reduce galactorrhea. In the second week of treatment, serum PRL level was suppressed (0.4 µg/L) and galactorrhea was completely resolved. CONCLUSIONS: Although uremic hyperprolactinemia is very rarely seen in childhood, it is important to evaluate, and initiate an appropriate treatment since it is associated with delayed puberty and infertility in adulthood in many cases.
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Galactorrea , Hiperprolactinemia , Insuficiencia Renal Crónica , Niño , Galactorrea/etiología , Humanos , Hiperprolactinemia/complicaciones , Masculino , Prolactina/metabolismo , Insuficiencia Renal Crónica/complicacionesRESUMEN
OBJECTIVE: We aimed to evaluate the effects of blood pressure (BP) values obtained by 24-h ambulatory BP monitoring (ABPM) of hypertensive children and adolescents on left ventricular mass index (LVMI). METHODS: Patients diagnosed with HT with BP measurements confirmed with ABPM and evaluated with echocardiography for LVMI were included. The patients were divided into two groups according to their BMI as obese and nonobese. SDSs of ABPM parameters were compared between the groups. RESULTS: A total of 158 children with HT were included in the study. Ninety of these patients were obese. In obese and nonobese cases, mean SDS levels were similar in ABPM parameters, whereas LVMI was significantly higher in obese patients ( P = 0.049). There was a significant correlation between LVMI and 24-h SBP SDS, daytime SBP SDS, 24-h SBP load and daytime BP load. In obese cases, there was a statistically significant correlation between LVMI and 24-h SBP SDS, daytime SBP SDS, 24-h SBP load, daytime SBP load as well as nighttime SBP SDS and nighttime SBP load. When the whole group was evaluated, 24-h SBP SDS was the most effective parameter influencing LVMI ( P = 0.001). Similarly, the most effective ABPM parameter on LVMI in obese patients was 24-h SBP SDS ( P = 0.001). CONCLUSION: A significantly higher rate of LVMI in obese patients suggests that obesity itself is an effective factor on LVMI. In addition, systolic hypertension is more effective on cardiac functions compared with DBP measurements and systolic-DBP dipping ratios.
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Monitoreo Ambulatorio de la Presión Arterial , Hipertensión , Adolescente , Presión Sanguínea/fisiología , Niño , Humanos , Hipertrofia Ventricular Izquierda , Obesidad/complicacionesRESUMEN
AIM: Urinary tract infections (UTIs) represent a common febrile illness in infancy. The study compared two UTI guidelines in terms of number of imaging studies, presence of parenchymal damage and radiation exposure in patients with the first febrile UTI between 2 and 24 months of age. METHOD: The results of Tepecik UTI Guideline-1 used until 2012 (Group 1, n = 105) were retrospectively compared with Tepecik UTI Guideline-2 (Group 2) used after 2013. In Group 1, urinary tract ultrasonography (US), dimercaptosuccinic acid (DMSA) and voiding cystourethrography (VCUG) were made in all patients. In Group 2, if the US result was abnormal, patients were evaluated with VCUG and DMSA. If the US was normal, only DMSA was performed. If the DMSA was abnormal, the VCUG was undergone (n: 43, 40.9%). RESULTS: The abnormal VCUG detection rate was 69.2% in Group 1 and 30.8% in Group 2 (p = 0.09). Sensitivity and specificity of US in the diagnosis of vesicoureteral reflux (VUR) was 15.9% and 96.7% in Group 1 and 61.5% and 70.5% in Group 2, respectively. Abnormal DMSA findings were observed among 33.3% (Groups 1) and 66.7% (Groups 2) subjects, respectively (p > 0.05). The median radiation exposure (500 mrem) of patients in Group 1 was statistically significantly higher than those in Group 2 (200 mrem) (p < 0.001). CONCLUSION: The VCUG should not be the first examination to be considered in such patients. We think that Tepecik UTI Guideline-2 reduces unnecessary invasive procedure and radiation exposure and not missed VUR in the management of children with UTI at 2-24 months. Needs prospective follow-up studies before considering this recommendation.
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Infecciones Urinarias , Reflujo Vesicoureteral , Humanos , Niño , Lactante , Estudios Prospectivos , Estudios Retrospectivos , Infecciones Urinarias/diagnóstico por imagen , Succímero , Reflujo Vesicoureteral/complicaciones , Reflujo Vesicoureteral/diagnóstico por imagenRESUMEN
BACKGROUND: Nephrotic syndrome (NS) is a common kidney disease associated with an increased risk of thrombotic events. The aim of this study was to assess the prothrombotic potential of patients with NS using the thrombin generation assay (TGA). METHODS: A total of 35 patients with NS, who were followed in the Division of Pediatric Nephrology in Behcet Uz Children`s Hospital, were included in the study. After the patients with Steroid Resistant NS (n:3) were excluded, 32 patients in total were evaluated for TGA. Patients were primarily classified according to their response to corticosteroid therapy. The control group consisted of 34 healthy volunteers with similar gender and age distribution to the patients. Blood urine nitrogen, creatinine, albumin, triglyceride, cholesterol, 24-hour proteinuria, platelets, erythrocyte sedimentation rate, C-reactive protein and thrombin generation values in activation and remission period of NS were compared. Moreover, TGA values of the patients in their remission period were compared with the values of those in the control group. RESULTS: Endogenous thrombin potential (ETP) and peak thrombin levels were significantly higher in the activation period than remission period of NS. Additionally, after the patients achieved remission, their ETP was still higher than the control group. There was a negative correlation between both ETP and peak thrombin levels of patients with serum albumin, whereas a significant positive correlation was detected with platelet levels. Thromboembolic events were not observed in any of the patients during follow-up. CONCLUSIONS: Nephrotic syndrome is strongly associated with hypercoagulopathy as assessed by TGA during active NS. The present study reinforces the usefulness of TGA as a marker of hypercoagulability in pediatric patients with NS. Further studies are needed in this regard.
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Síndrome Nefrótico , Trombosis , Pruebas de Coagulación Sanguínea , Niño , Humanos , Síndrome Nefrótico/diagnóstico , Proteinuria , TrombinaRESUMEN
Patients with solitary kidneys (SKs) are at risk of hypertension (HT) and associated end-organ damage. The authors aimed to evaluate whether children with congenital SKs (CSKs) have higher office, ambulatory, or central blood pressure (BP), increased arterial stiffness or left ventricular mass index, or any risk for arrhythmia. With this purpose, patients with CSK and healthy controls being followed up between January 2018 and June 2019 were enrolled in the study. Demographic, biochemical, and office blood pressure (BP) data were recorded. Then, ambulatory blood pressure monitoring (ABPM) and measurements of central BP (cBP), pulse wave velocity (PWV), and augmentation index (AIx@75) were obtained. Ventricular repolarization parameters were acquired by 12-lead electrocardiography. Left ventricular mass index (LVMI) and abdominal aortic stiffness parameters including strain, pressure strain elastic modulus (Ep), and normalized Ep (Ep*) were calculated with echocardiographic measurements. Finally, 36 children with CSK and 36 healthy controls were included. Serum creatinine, uric acid, total cholesterol levels, ABPM parameters, cBP levels, and PWV values were significantly higher, and eGFR levels were significantly lower in the CSK group. VR parameters, abdominal aortic stiffness indices, and LVMI were similar between the groups. CSK increased the risk of HT in ABPM (HTABPM ) by 6 times. PWV was significantly correlated with Ep and Ep* in cases with CSK. Determination of cBP and PWV along with 24-hour ABPM would be a useful tool in children with CSK.
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Enfermedades Cardiovasculares , Hipertensión , Rigidez Vascular , Adolescente , Presión Sanguínea , Monitoreo Ambulatorio de la Presión Arterial , Enfermedades Cardiovasculares/diagnóstico , Enfermedades Cardiovasculares/epidemiología , Enfermedades Cardiovasculares/etiología , Niño , Factores de Riesgo de Enfermedad Cardiaca , Humanos , Hipertensión/complicaciones , Hipertensión/diagnóstico , Hipertensión/epidemiología , Riñón , Análisis de la Onda del Pulso , Factores de RiesgoRESUMEN
AIM: This study aimed to present the demographic, clinical, and laboratory features of children clinically diagnosed with familial Mediterranean fever (FMF) and to predict more severe mutations by evaluating those findings. METHODS: We enrolled cases diagnosed with FMF with a defined variation in at least one allele. The medical charts of the patients were reviewed retrospectively. The patients were grouped as homozygous, compound heterozygous, and simple heterozygous cases, with and without M694V mutation. We compared the data between the subgroups using logistic regression analysis and determined the risk factors for being homozygous or compound heterozygous for M694V. RESULTS: A total of 263 (M/F =109/154) cases were included. The mean age at the onset of symptoms, follow-up duration, and time to diagnosis were 6.75 ± 3.9 (0.25-17) years, 51.78 ± 39.31 (6-166) months, and 9.23 ± 14.44 (1-132) months, respectively. The rates of parental consanguinity, positive family history for FMF, and FMF in a first-degree relative were 15%, 42%, and 31.4% respectively. The most common symptom was abdominal pain (85%). There was no difference between the growth parameters of the cases during the initial and final control periods. The most frequent alleles were M694V, E148Q, and V726A. The most common accompanying disease was IgA vasculitis (20%). Almost 90% of the cases fulfilled all the defined criteria. The rate of patients having a first-degree relative with FMF was higher, Hb values were lower, and the erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) values were higher during the attack period; the ESR and CRP values were higher in the attack-free period; and Pras disease severity scores were higher in homozygous or compound heterozygous cases carrying M694V. The presence of FMF in a first-degree relative increases the probability of being homozygous and compound heterozygous for M694V by a factor of 2.39; and each 1 unit increase in the Pras score increases this probability by a factor of 1.43. The threshold Pras score for this possibility is 5.5 (AUC = 0.651; 95% CI, 0.545-0.757; P = .006; sensitivity, 65%; specificity, 55%). CONCLUSION: M694V was the most common and severe mutation in our cohort. The presence of a first-degree relative with FMF and Pras scores ≥5.5 may predict a homozygous or compound heterozygous mutation for M694V.
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Background/aim: In children with autosomal dominant polycystic kidney disease (ADPKD), clinical manifestations range from severe neonatal presentation to renal cysts found by chance. We aimed to evaluate demographic, clinical, laboratory findings, and genetic analysis of children with ADPKD. Materials and methods: We evaluated children diagnosed with ADPKD between January 2006 and January 2019. The diagnosis was established by family history, ultrasound findings, and/or genetic analysis. The demographic, clinical, and laboratory findings were evaluated retrospectively. Patients <10 years and ≥10 years at the time of diagnosis were divided into 2 groups and parameters were compared between the groups. Results: There were 41 children (M/F: 18/23) diagnosed with ADPKD. The mean age at diagnosis was 7.2 ± 5.1 (0.616.9) years and the follow-up duration was 59.34 ± 40.56 (8198) months. Five patients (12%) were diagnosed as very early onset ADPKD. All patients had a positive family history. Genetic analysis was performed in 29 patients (PKD1 mutations in 21, PKD2 mutations in 1, no mutation in 3). Cysts were bilateral in 35 (85%) of the patients. Only one patient had hepatic cysts. No valvular defect was defined in 12 patients detected. Only 1 patient had hypertension. None of them had chronic kidney disease. No difference could be demonstrated in sex, laterality of the cysts, maximum cyst diameter, cyst or kidney enlargement, follow-up duration, or GFR at last visit between Groups 1 and 2. Conclusion: The majority of children with ADPKD had preserved renal functions and slight cyst enlargement during their follow-up. However, they may have different renal problems deserving closed follow-up.
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Quistes/patología , Riñón/patología , Imagen por Resonancia Magnética/métodos , Riñón Poliquístico Autosómico Dominante/genética , Canales Catiónicos TRPP/genética , Niño , Preescolar , Quistes/diagnóstico por imagen , Quistes/epidemiología , Quistes/genética , Femenino , Predisposición Genética a la Enfermedad , Humanos , Lactante , Riñón/diagnóstico por imagen , Masculino , Riñón Poliquístico Autosómico Dominante/diagnóstico por imagen , Riñón Poliquístico Autosómico Dominante/epidemiología , Reacción en Cadena en Tiempo Real de la Polimerasa , Estudios RetrospectivosRESUMEN
BACKGROUND: Herein we present our experience with abatacept in a patient diagnosed with primary focal segmental glomerulosclerosis (FSGS) and resistant to steroid and other immunosuppressives. CASE: A 17-year-old girl was diagnosed with idiopathic nephrotic syndrome (NS) at the age of 8 years. Kidney biopsy was performed when she did not respond to 6-weeks of steroid (2mg/kg) therapy followed by three doses of pulse methylprednisolone (PMP) and considered as steroid resistant NS. The biopsy revealed focal segmental glomerulosclerosis (FSGS) and cyclophosphamide was added to the steroid treatment but the patient had no response. The genetic analysis revealed G34G/A318A compound homozygous synonym aminoacid variation in NPHS2 gene, thus all immunosuppressive regimes were stopped and she was put on supportive treatment. Throughout this period, she had nephrotic range of proteinuria, however serum albumin levels were > 3g/dl. At the end of two years, the patient had NS with severe edema and hypoalbuminemia. When the genetic analysis was interpreted again, it was found to be consistent with a polymorphism rather than a mutation. Following 3 doses of PMP, oral steroid treatment was resumed and cyclosporine (CsA) was added to the treatment at the fifth year of follow up. However, she was unresponsive to CsA at the end of the first year as well as mycophenolate mofetil used for 12 months and rituximab used for 6 months, respectively. Then abatacept was instituted and proteinuria decreased below 1 gr/day and serum albumin levels increased to 3 g/dl at the end of 6 doses. Serum albumin levels remained stable in the following 7 months. CONCLUSION: Partial remission including the decrease in proteinuria and increase in albumin levels achieved in our patient encourages the usage of abatacept in patients who do not respond to multiple immunosuppressive therapies.
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Glomeruloesclerosis Focal y Segmentaria , Síndrome Nefrótico , Abatacept/uso terapéutico , Adolescente , Niño , Ciclosporina , Femenino , Glomeruloesclerosis Focal y Segmentaria/tratamiento farmacológico , Humanos , Inmunosupresores/uso terapéutico , Metilprednisolona , Síndrome Nefrótico/tratamiento farmacológicoRESUMEN
OBJECTIVE: Calyceal diverticulum (CD) is rarely diagnosed in children. It mimics other cystic lesions of the kidney and may be very difficult to identify. Displaying the communication of these fluid-filled cavities with the collecting system of the kidney via imaging methods is the key diagnostic method. The purpose of this study is to determine the presence of calyceal diverticulum in pediatric patients followed up due to simple renal cyst and/or parapelvic cyst. MATERIAL, AND METHOD: Patients who were newly diagnosed or being followed up with the ultrasonographic diagnosis of simple cyst(s) or parapelvic cyst(s) in the Department of Pediatric Nephrology of Izmir University of Health Sciences Tepecik Training and Research Hospital between December 2015 and July 2018 were included in the study. Age, gender, admission symptoms, recurrent urinary tract infections, stone disease, trauma history, accompanying urinary system and systemic anomalies, localization, and size of the cyst(s) at admission and during follow-up, treatment type and developing complications were noted. Contrast MR urography was applied to all patients. RESULTS: A total of 43 patients [mean age:132 ± 54.9 (12-228) months] including 25 girls were enrolled in the study. The mean follow-up period was 36. 74 ± 19 (1-90) months. The cysts were located mostly in the right kidney and also in the upper pole of the kidney. The mean cyst size was 20.4 ± 11 (8-58) mm at admission, and comparably 20.3 ± 8.6 (10-45) mm at the end of the follow-up. In contrast MR urography, a total of 16 cases were observed at first glance to be associated with pelvicalyceal system. However, in the postcontrast images taken at the 5th minute it was determined that 9 of them were compatible with calyceal diverticulum. CONCLUSION: Although it is thought that well-circumscribed solitary renal fluid collections may be thought to be a simple renal cyst at first glance, the differential diagnosis of calyceal diverticulum (CD) should be absolutely kept in mind. Contrast studies and late-phase postcontrast images are needed for the diagnosis of CD. Although the standard management of CDs in children is conservative, surgical intervention may be rarely required.
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Quistes , Divertículo , Enfermedades Renales Quísticas , Niño , Divertículo/diagnóstico por imagen , Femenino , Humanos , Riñón/diagnóstico por imagen , Enfermedades Renales Quísticas/diagnóstico por imagenRESUMEN
BACKGROUND: Myalgia is one of the presenting symptoms of Familial Mediterranean Fever (FMF), which is reported at a rate of 27-39.6%. Recurrent fasciitis in those cases are extremely rare. We aimed to present a case with FMF having radiologically proven fasciitis. CASE: An 11-year-old male patient with a diagnosis of FMF and M694V homozygote mutation, using colchicine regularly at a dose of 2 mg/day (0.08 mg/kg/day) for 4 years; was admitted to the hospital with severe pain and tenderness on the right calf. There were no accompanying symptoms like fever or abdominal pain. He described three similar episodes with pain and tenderness in left and right calves, which were not accompanied by fever in the last 6 months. The erythrocyte sedimentation rate and C-reactive protein levels were high and serum creatinine kinase was normal. The day after initiation of non-steroidal anti-inflammatory drug (NSAID), his complaints regressed. However, after a week, he again had a severe calf pain. Lower extremity arterial and venous doppler ultrasonography was normal. Increased peripheral signal intensity and fasciitis around the soleus muscle was defined in MRI. With NSAIDs, myalgia disappeared in a few days and acute phase reactants decreased within a week. In the follow up, canakinumab was prescribed due to febrile attacks as frequent as once a month and calf pain observed almost weekly. Thereafter, both febrile attacks and recurrent debilitating calf pain were completely ceased. CONCLUSION: There are three cases in the literature with fasciitis related myalgia. With this case, we wanted to emphasize fasciitis as a cause of FMF associated myalgia on MRI. In such cases MRI may be helpful to demonstrate fascial involvement.