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1.
Turk Arch Pediatr ; 59(1): 43-48, 2024 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-38454259

RESUMEN

OBJECTIVE: We aimed to evaluate the clinical significance of the "unclassified" blood pressure phenotypes on left ventricular hypertrophy in children. MATERIALS AND METHODS: All children evaluated with ambulatory blood pressure monitoring in the pediatric nephrology department between October 2018 and January 2021 were included in the study. Prehypertension, normotensive, white coat hypertension, masked hypertension, ambulatory hypertension groups and 2 other groups including increased blood pressure load, normal ambulatory blood pressure measurements, but normal (unclassified group 1) or high (unclassified group 2) office blood pressure measurements were defined according to the American Heart Association 2014 statement. Left ventricular mass index, left ventricular mass index/95 percentile values, and left ventricular hypertrophy ratios were compared between the groups separately to establish the influence of the unclassified cases. RESULTS: A total of 497 children were included. There were 52 cases in normotensive, 47 cases in unclassified group 1, 50 cases in masked hypertension, 79 cases in white coat hypertension, 104 cases in unclassified group 2, and 165 cases in the ambulatory hypertension group. Left ventricular mass index/95 percentile and left ventricular hypertrophy in masked hypertension were significantly higher than normotensive but similar between normotensive and unclassified group 1 groups. Left ventricular hypertrophy was significantly higher in the ambulatory hypertension group compared to white coat hypertension, and similar between white coat hypertension and unclassified group 2 groups. CONCLUSION: Independent of age, we have found that interpretation of blood pressure load not only has a limited predictable effect on left ventricular hypertrophy but also causes a large group of patients to be unclassified. Cite this article as: Kasap-Demir B, Basaran C, Demircan T, et al. The effect of "unclassified" blood pressure phenotypes on left ventricular hypertrophy. Turk Arch Pediatr. 2024;59(1):43-48.

2.
Artículo en Inglés | MEDLINE | ID: mdl-38327222

RESUMEN

BACKGROUND/AIMS: Two earthquakes on February 6th, 2023 destroyed ten cities in Türkiye. We report our experience with pediatric victims during these catastrophes, with a focus on crush syndrome related-acute kidney injury (Crush-AKI) and death. METHOD: A web-based software was prepared. Patient demographics, time under rubble (TUR), admission laboratory data, dialysis, and kidney and overall outcomes were asked. RESULTS: 903 injured children (median age: 11.62 years) were evaluated. Mean TUR was 13 h (Interquartile range-IQR: 32.5), max 240 h). 31 of 32 patients with a TUR of >120 h survived. The patient who rescued after ten days survived.Two-thirds of the patients were given 50 mEq/L sodium-bicarbonate in 0.45% sodium-chloride solution on admission day. 58% of patients were given intravenous fluid (IVF) at a volume of 2000-3000 mL/m2 body surface area (BSA), 40% of 3000-4000 mL/m2 BSA, and only 2% of >4000 mL/m2 BSA. 425 patients had surgeries, 48 suffered from major bleeding. Amputations were recorded in 96 patients. Eighty-two and 66 patients required ventilator and inotropic support, respectively.Crush-AKI developed in 314 patients (36% of all patients). 189 patients were dialyzed. Age > 15 years, creatine phosphokinase (CK)≥20 950 U/L, TUR≥10 h, and the first-day IVF volume < 3000-4000 mL/m2 BSA were associated with Crush-AKI development. 22 deaths were recorded, 20 of 22 occurred in patients with Crush-AKI and within the first 4 days of admission. All patients admitted after 7 days survived. CONCLUSIONS: This is the most extensive pediatric kidney disaster data after an earthquake. Serum CK level was significantly associated with Crush-AKI at the levels of >20 950 U/L, but not with death. Adolescent age and initial IVF of less than 3000-4000 mL/m2 BSA were also asscoiated with Crush-AKI. Given that mildly injured victims can survive longer periods in the disaster field, we suggest uninterrupted rescue activity for at least 10 days.

3.
Pediatr Nephrol ; 39(5): 1509-1519, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-38040872

RESUMEN

BACKGROUND: Myelomeningocele (MMC) is highly prevalent in developing countries, and MMC-related neurogenic bladder is an important cause of childhood chronic kidney disease (CKD). This nationwide study aimed to evaluate demographic and clinical features of pediatric patients with MMC in Turkey and risk factors associated with CKD stage 5. METHODS: Data from children aged 0-19 years old, living with MMC in 2022, were retrospectively collected from 27 pediatric nephrology centers. Patients > 1 year of age without pre-existing kidney abnormalities were divided into five groups according to eGFR; CKD stages 1-5. Patients on dialysis, kidney transplant recipients, and those with eGFR < 15 ml/min/1.73 m2 but not on kidney replacement therapy at time of study constituted the CKD stage 5 group. RESULTS: A total of 911 (57.8% female) patients were enrolled, most of whom were expectantly managed. Stages 1-4 CKD were found in 34.3%, 4.2%, 4.1%, and 2.4%, respectively. CKD stage 5 was observed in 5.3% of patients at median 13 years old (range 2-18 years). Current age, age at first abnormal DMSA scan, moderate-to-severe trabeculated bladder on US and/or VCUG, and VUR history were independent risk factors for development of CKD stage 5 (OR 0.752; 95%; CI 0.658-0.859; p < 0.001; OR 1.187; 95% CI 1.031-1.367; p = 0.017; OR 10.031; 95% CI 2.210-45.544; p = 0.003; OR 2.722; 95% CI 1.215-6.102; p = 0.015, respectively). Only eight CKD stage 5 patients underwent surgery related to a hostile bladder between 1 and 15 years old. CONCLUSION: MMC-related CKD is common in childhood in Turkey. A proactive approach to neurogenic bladder management and early protective surgery in selected cases where conservative treatment has failed should be implemented to prevent progressive kidney failure in the pediatric MMC population in our country.


Asunto(s)
Fallo Renal Crónico , Meningomielocele , Insuficiencia Renal Crónica , Vejiga Urinaria Neurogénica , Humanos , Niño , Femenino , Recién Nacido , Lactante , Preescolar , Adolescente , Adulto Joven , Adulto , Masculino , Meningomielocele/complicaciones , Meningomielocele/epidemiología , Estudios de Cohortes , Vejiga Urinaria Neurogénica/epidemiología , Vejiga Urinaria Neurogénica/etiología , Vejiga Urinaria Neurogénica/terapia , Estudios Retrospectivos , Diálisis Renal/efectos adversos , Insuficiencia Renal Crónica/diagnóstico , Insuficiencia Renal Crónica/epidemiología , Insuficiencia Renal Crónica/etiología , Fallo Renal Crónico/complicaciones
4.
J Clin Res Pediatr Endocrinol ; 15(4): 397-405, 2023 11 22.
Artículo en Inglés | MEDLINE | ID: mdl-37470253

RESUMEN

Objective: To compare pulse wave analysis (PWA) of obese children with and without metabolic syndrome (MS) with healthy, non-obese children and to evaluate the association between PWA findings and additional risk factors present in children with MS and obesity. Methods: From the obese patients examined between June 2019 and June 2021, 41 patients with MS, 36 obese patients without MS, and 34 healthy non-obese children of similar age and gender were evaluated retrospectively. Anthropometric measurements, biochemical evaluation, 24-hour ambulatory blood pressure (BP) measurement (ABPM), left ventricular mass index (LVMI) and PWA measurements were compared. Results: When the three groups were compared, weight standard deviation score (SDS), height SDS and body mass index SDS were all significantly higher in the MS group (p<0.05). The following measurements were significantly higher in both MS and non-MS obese patients compared to the control group: from ABPM measures, the systolic and mean arterial pressure BP SDSs load; from PWA, the night central systolic BP, 24-hour, day and night pulse pressure values and 24-hour, day and night pulse wave velocity (PWV) rates; and from cardiac evaluations, the LVMI and relative wall thickness measurements (all p<0.05). Furthermore, the 24-hour and daytime central systolic (cSBP) and diastolic BP (cDBP) values were significantly different between the three groups, being the highest in the MS group (p<0.05). Conclusion: Obesity causes higher office, ambulatory and central BP, PWV and LVMI. However our results suggest that additional risk factors associated with MS do not contribute to these parameters, except for 24-hour and daytime cSBP and cDBP values.


Asunto(s)
Hipertensión , Síndrome Metabólico , Obesidad Infantil , Rigidez Vascular , Humanos , Niño , Síndrome Metabólico/complicaciones , Obesidad Infantil/complicaciones , Monitoreo Ambulatorio de la Presión Arterial , Estudios Retrospectivos , Análisis de la Onda del Pulso/efectos adversos , Presión Sanguínea/fisiología , Hipertensión/etiología , Rigidez Vascular/fisiología
5.
Indian J Pediatr ; 90(7): 677-682, 2023 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-35727527

RESUMEN

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.


Asunto(s)
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 Riesgo
6.
Pediatr Rep ; 14(4): 533-537, 2022 Dec 07.
Artículo en Inglés | MEDLINE | ID: mdl-36548205

RESUMEN

Aim: The presence and clinical importance of vesicoureteric reflux in patients with a double collecting system are being questioned. Therefore, the role of voiding cystourethrography in the management of patients with ureterocele is unclear. This study aimed to evaluate patients with a ureterocele in terms of urinary tract infection (UTI) and vesicoureteral reflux (VUR). Material Methods: The cases who were admitted to the Pediatric Nephrology Clinic of Health Sciences University Tepecik Training and Research Hospital between 2012 and 2022 and were diagnosed with ureterocele were evaluated retrospectively. Demographic, clinical, and laboratory data were obtained from file records. Results: All patients diagnosed with ureterocele and voiding cystourethrography (VCUG) were evaluated. A total of 24 (female 13 (54.2%)) children were included. The reasons for admission were antenatal hydronephrosis in 13 (54.2%) patients, UTI in 9 (37.5%) patients, and incidentally diagnosed ureterocele in 2 (8.3%) patients. Urinary tract infection was observed in 20 patients at admission, recurrent UTI in 21 patients at follow-up, preoperative pyelonephritis in 12 patients. VUR was found in 11 patients, and severe VUR (≥stage 3) was found in 9 patients. Ten patients had ipsilateral hydronephrosis, and 14 patients had a double collecting system. The presence of VUR was found to be associated with female gender, UTI at admission, and recurrent UTI at follow-up (p < 0.05). However, there was no difference between groups with or without VUR in terms of ipsilateral hydronephrosis, scar formation, and the need for surgery (p > 0.05). Conclusions: We could not demonstrate any criteria to select patients to receive VCUG; on the other hand, VUR did not cause more kidney damage. Our study supports the need for more scientific data to determine management in patients with ureterocele.

7.
Pediatr Int ; 64(1): e15323, 2022 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-36331224

RESUMEN

BACKGROUND: Patients with nephrotic syndrome (NS) are at a high risk of cardiovascular disease, obesity, and dyslipidemia. The aim of this study was to evaluate the formation of epicardial adipose tissue (EAT) and investigate electrocardiographic (ECG) parameters in patients. METHODS: Thirty-two patients aged 0-18 years and 15 control patients were compared. In the patient group, physical examination and laboratory parameters were recorded. Atrial depolarization and ventricular repolarization parameters in ECG were compared between the groups. EAT was evaluated with M-mode measurements on echocardiography. RESULTS: There was no difference between the groups in terms of sex, age, body mass index, systolic and diastolic BP. EAT was found to be significantly higher in the patient group. In ECG evaluations it was determined that atrial depolarization and ventricular repolarization parameters increased in the patient group. CONCLUSIONS: Cardiovascular morbidity and mortality are high in kidney diseases. Measurement and follow-up of EAT and ECG findings as a noninvasive parameter can provide information in NS.


Asunto(s)
Síndrome Nefrótico , Humanos , Síndrome Nefrótico/complicaciones , Síndrome Nefrótico/diagnóstico , Pericardio/diagnóstico por imagen , Tejido Adiposo/diagnóstico por imagen , Ecocardiografía , Arritmias Cardíacas
8.
Turk J Pediatr ; 64(2): 265-273, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35611415

RESUMEN

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.


Asunto(s)
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-Lactamasas
9.
Urolithiasis ; 50(4): 411-421, 2022 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-35482085

RESUMEN

We evaluated the demographic features, etiologic risk factors, treatment strategies, and outcome of the infants and children with urolithiasis (UL). A retrospective multicenter study was conducted including 23 Pediatric Nephrology centers in Turkey. The medical records of 2513 children with UL were reviewed. One thousand, three hundred and four boys and 1209 girls (1.1:1) were reported. The mean age at diagnosis was 39.5 ± 35 months (0.4-231 months), and 1262 patients (50.2%) were in the first year of life (infants). Most of the cases with infantile UL were diagnosed incidentally. Microlithiasis (< 3 mm) was found in 794 patients (31.6%), and 64.5% of the patients with microlithiasis were infants. Stones were located in the pelvis-calyces in 63.2% (n: 1530) of the cases. The most common stone type was calcium oxalate (64.6%). Hypocitraturia was the most common metabolic risk factor (MRF) in children older than 12 months, but in infancy, hypercalciuria was more common. Fifty-five percent of the patients had received at least one medical treatment, mostly potassium citrate. At the end of a year's follow-up, most of the patients with microlithiasis (85%) showed spontaneous remission. The rate of spontaneous stone resolution in infants was higher than in children. Spontaneous remission rate was higher in cases with MRF ( - ) stones than in MRF ( +) stones. However, remission rate with medical treatment was higher in cases with MRF ( +) stones. This study represents the results of a large series of infants and children with UL and showed that there are several differences such as underlying metabolic and anatomic abnormalities, clinical course, and stone remission rates between infants and children with urinary stone disease.


Asunto(s)
Cálculos Urinarios , Urolitiasis , Niño , Femenino , Humanos , Hipercalciuria/complicaciones , Lactante , Masculino , Citrato de Potasio , Remisión Espontánea , Estudios Retrospectivos , Factores de Riesgo , Cálculos Urinarios/complicaciones , Urolitiasis/epidemiología , Urolitiasis/etiología , Urolitiasis/terapia
10.
Hypertens Res ; 45(6): 1047-1057, 2022 06.
Artículo en Inglés | MEDLINE | ID: mdl-35365799

RESUMEN

We aimed to evaluate the agreements between the guidelines used for both office blood pressure (OBP) and ambulatory blood pressure monitoring (ABPM). Our secondary aim was to define the best threshold to assess children at risk of left ventricular hypertrophy (LVH). Thresholds proposed by the Fourth Report (FR), European Society of Hypertension (ESH), and American Academy of Pediatrics (AAP) for OBP and the Wühl, ESH, and American Heart Association (AHA) for ABPM were used, and nine different BP phenotype combinations were created. The agreements between the thresholds, the sensitivity of the thresholds, and the BP phenotypes used to predict LVH were determined in 949 patients with different ages and body mass indices (BMIs). The agreements between the guidelines for OBP and ABPM were "good" and "very good" (κ = 0.639; 95% CI, 0.638-0.640, κ = 0.986; 95% CI, 0.985-0.988), respectively. To classify OBP and ABPM into BP phenotypes, we obtained nine different combinations, which had "very good" agreement (κ = 0.880; 95% CI, 0.879-0.880). The sensitivity of AAP for detecting LVH was the highest in <12-year-old obese children (S = 75.8, 95% CI, 56.4-89.7). The sensitivity of ABPM in detecting LVH was similar among different age and BMI groups. The sensitivity of different BP phenotypes tended to be higher in the groups where OBP was evaluated according to AAP. The highest sensitivity was detected in the 13- to 15-year-old normal weight group.(S: 88.8, 95% CI, 51.7-99.7). The AAP guideline is more sensitive and decisive for BP phenotypes to detect LVH, especially in normal-weight children ≤ 15 years, while ABPM thresholds for children have limited effect.


Asunto(s)
Hipertensión , Obesidad Infantil , Presión Sanguínea , Monitoreo Ambulatorio de la Presión Arterial , Niño , Humanos , Hipertrofia Ventricular Izquierda/complicaciones , Obesidad Infantil/complicaciones , Estados Unidos
11.
Blood Press Monit ; 27(4): 213-219, 2022 Aug 01.
Artículo en Inglés | MEDLINE | ID: mdl-35258018

RESUMEN

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.


Asunto(s)
Monitoreo Ambulatorio de la Presión Arterial , Hipertensión , Adolescente , Presión Sanguínea/fisiología , Niño , Humanos , Hipertrofia Ventricular Izquierda , Obesidad/complicaciones
15.
Pediatr Int ; 64(1): e14887, 2022 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-34131997

RESUMEN

BACKGROUND: Since the emergence and worldwide spread of the new coronavirus (COVID-19) pandemic, it has caused people to experience adverse psychological effects. This study aimed to assess anxiety levels during COVID-19 in children with chronic kidney disease (CKD), including nephrotic syndrome (NS) and kidney transplantation (Tx). METHODS: A case-controlled, cross-sectional study was conducted with children aged 10-18 years, who had a diagnosis of CKD or NS, or Tx, and followed in our center between April and July 2020. A healthy control group was recruited with age- and gender-matched children. A questionnaire with printed and online versions was designed in three parts: the first addressed demographic characteristics, the second addressed opinions about the pandemic, and the third was the Turkish version of the Revised Child Anxiety and Depression Scale - Child Version. RESULTS: A total of 88 children completed the questionnaire. The patient and control groups were similar in terms of gender, age, household members and history of psychiatric treatment. Both groups stated that coronavirus is a risky disease for children (63.6%), and that they were afraid of contagion (69.3%). Only half of them were receiving realistic and informative answers from family members. In the Revised Child Anxiety and Depression Scale - Child Version, 66% of them received a high score on at least one subscale. The social phobia scores of the control group were higher than those of the patient group, although the proportion of high scores was similar in both groups. The ratio of high-scored participants was higher in CKD patients for panic disorder, and was lower in the immunosuppressive agent group for social phobia. CONCLUSION: The current COVID-19 pandemic is a disaster that children encounter for the first time in their lives. It does not exclusively cause anxiety among children with chronic kidney diseases but also affects healthy children.


Asunto(s)
COVID-19 , Insuficiencia Renal Crónica , Niño , Humanos , COVID-19/epidemiología , Pandemias , Estudios Transversales , SARS-CoV-2 , Depresión/psicología , Ansiedad/epidemiología , Ansiedad/etiología , Ansiedad/diagnóstico , Insuficiencia Renal Crónica/complicaciones , Insuficiencia Renal Crónica/epidemiología
18.
Germs ; 11(4): 617-624, 2021 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-35096681

RESUMEN

INTRODUCTION: Multisystem inflammatory syndrome in children (MIS-C) is a rarely seen severe complication of coronavirus disease-2019 (COVID-19). Although fever is one of the indispensable symptoms, other infections should be considered in the differential diagnosis during the pandemic. CASE REPORT: An 8-year-old and a 16-year-old female patient were admitted with fever, vomiting, headache. Both had fulfilled the criteria and were diagnosed with MIS-C. However, they both had remarkable persistent costovertebral angle tenderness, which was unexpected in MIS-C. In Case-1, urine analysis showed microscopic hematuria without pyuria, and urine culture showed no bacterial growth. Case-2 had microscopic hematuria and pyuria with Escherichia coli growth in urine culture. Contrast-enhanced computed tomography showed wedge-shaped hypodense multiple lesions in bilateral kidneys for Case-1, in the right kidney for Case-2. They diagnosed acute focal bacterial nephritis (AFBN). CONCLUSIONS: The diagnostic criteria of MIS-C can overlap with the symptoms of other severe septic infections such as AFBN, which is a rare urinary tract infection, diagnosed by imaging of localized renal inflammatory mass-like or wedge-shaped lesion. A detailed anamnesis and careful physical examination may help differential diagnosis.

19.
Turk Arch Pediatr ; 56(6): 610-617, 2021 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-35110061

RESUMEN

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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