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The article presents questions and answers related to medicine, including the possible diagnosis of a patient with symptoms like cytopenia, hepatosplenomegaly and high fever and whether additional evaluation is need to confirm macrophage activation syndrome (MAS).

Hypertension (HT) is seen with increasing frequency in childhood. Various guidelines have been published to better evaluate these patients. The most frequently used of these are; The 4th Report (The 4th Report-2004) updated and published by the National Heart Lung and Blood Institute (NHLBI) National High Blood Pressure Education Program (NHBPEP) Working Group in 2004, a guideline prepared by the European Society of Hypertension in 2016 (ESH-2016), the last one is the guideline prepared by the American Academy of Pediatrics in 2017 (AAP-2017). Although these guidelines have some similarities, there are serious differences between them. The 4th Report-2004 and ESH-2016 guidelines use blood pressure (BP) percentile tables based on age and height previously determined in American children. Then, new tables were created by extracting the measurements of obese children and these tables were used in AAP-2017. From the age of 16 in ESH-2016 and 13 in AAP-2017, it is recommended that BP evaluations should be made according to adult guidelines. Evaluation of the hypertensive patient, Ambulatory Blood Pressure Monitoring (ABPM) criteria, the timing of laboratory tests and treatment differ according to guidelines. As a result; since universal BP tables covering all children around the world have not yet been created; age, ethnic and geographical conditions should be taken into account when evaluating which guidelines the office BP and ABPM should follow. By following the currently published guidelines, it will be possible to reduce future cardiovascular events. [ABSTRACT FROM AUTHOR]

Aim: Familial Mediterranean Fever (FMF) is one of the most frequent genetic diseases with a frequency of 1/1,000 in our country. We aimed to assess awareness of FMF among caregivers of patients with and without FMF. Materials and Methods: Caregivers of FMF patients (group 1) and other caregivers who were in hospital for other reasons (group 2) were given a questionnaire regarding FMF and the results were compared between the 2 groups. Results: The mean ages were similar between group 1 (n=142) and group 2 (n=207). Female gender and lower educational status were more frequent in group 1. Group 1 was more aware that recurrent fever, joint pain, abdominal pain, and its occurrence in attacks are seen in FMF; and also that the disease is inherited rather than contagious; that a specific treatment exists; that the risk of kidney and heart failure increases when not treated; that attacks are triggered by sleeplessness, tiredness or emotional stress; that colchicine does not provide complete recovery; and that diarrhea is the most common side effect of colchicine (p<0.05). However, awareness about chest pain as a feature, infertility as a complication, non-adherence as a trigger for the disease and the statement that "patients feel healthy between attacks" were similar (p>0.05). Interestingly, group 2 was more aware that different treatment options exist (p=0.04). The total score was higher in group 1 and in participants who only graduated from secondary school or less (p<0.01). Conclusion: Being a caregiver of a patient was associated with a higher level of awareness of FMF regardless of education level, however, they still need to be informed about current developments. [ABSTRACT FROM AUTHOR]

The article describes the differential diagnosis of Fanconi syndrome in a young child. Highlights include corneal examination and intracellular cystine concentration in white blood cells for cystinosis, demographical data, ifosfamide doses, additional treatment, and laboratory examinations of cases, and correction of chronic metabolic acidosis with oral sodium bicarbonate.

The article presents a case of a 16-year-old boy who was rushed to a clinic due to high fever, darkening of urine, and yellowish skin to discuss his possible diagnosis and the possible causes of persistent non-nephrotic proteinuria. He has a medical history of suspected infantile transient hypogammaglobulinemia. Also cited are his abnormal levels of erythrocytes, urobilinogen, and leukocytes.

We evaluated the predictors of renal scar in children with urinary tract infections (UTIs) having primary vesicoureteral reflux (VUR). Data of patients who were examined by dimercaptosuccinic acid (DMSA) scintigraphy between 1995 and 2005 were evaluated retrospectively. Gender, age, reflux grade, presence/development of scarring, breakthrough UTIs, and resolution of reflux, were recorded. The relation of gender, age and VUR grade to preformed scarring and the relation of gender, age, VUR grade, presence of preformed scarring, number of breakthrough UTIs and reflux resolution to new scarring were assessed. There were 138 patients [male/female (M/F) 53/85]. Multivariate analysis showed that male gender [odds ratio (OR) 2.5], age ≥ 27 months in girls (OR 4.2) and grades IV–V reflux (OR 12.4) were independent indicators of renal scarring. On the other hand, only the presence of previous renal scarring was found to be an independent indicator for the development of new renal scar (OR 13.4). In conclusion, while the most predictive variables for the presence of renal scarring among children presenting with a UTI were male gender, age ≥ 27 months in girls, and grades IV–V reflux, the best predictor of new scar formation was presence of previous renal scarring. [ABSTRACT FROM AUTHOR]

The article describes the case of a 22-month-old girl who was referred to a clinic upon detection of glycosuria and proteinuria in urine analysis. Highlights include low body height and weight for her age according to physical examination, a surgical scar tissue observed on umbilicus, and consistency of urine protein electrophoresis with tubular proteinuria.

Objectives The aim of this study is to investigate the effect of anti-interleukin (IL)-1/-6 biologics on systemic juvenile idiopathic arthritis (sJIA)-associated macrophage activation syndrome (MAS). Methods Demographic, clinical and laboratory data of patients followed up with a diagnosis of sJIA-associated MAS assessed from sixteen paediatric rheumatology centres across the country. The clinical and laboratory features of MAS developing while on biological drugs were compared with those without this treatment. Results One hundred and sixty-two patients were included in the study. Forty-five of the MAS events were detected under the effect of anti-IL-1/-6 biologics, while the patients experiencing the remaining 155 events have not received biological treatment in the last three months. Platelet count [128 (72–232) vs 199 (130–371) 109/l], ferritin level on admission [1107 (676–2050) vs 2863 (1193–9562) ng/ml], C-reactive protein level [15.4 (2.9–56) vs 90 (32–160) mg/l], erythrocyte sedimentation rate [13 (3–36) vs 43.5 (13–77) mm/h] and fever duration [5 (4–7.5) vs 10 (7–14.3) days] were found lower in the group under the impact of anti-IL-1/-6 biologics. Among patients treated with biologics, 26.6% did not meet the published 2016 MAS classification criteria at presentation. The rates of hepatomegaly and splenomegaly were relatively lower in the canakinumab-treated group when compared with those receiving other biologicals or to patients, not on biologicals. Conclusion Anti-IL-1/-6 therapies can mask the clinical and laboratory features of MAS, and proposed guidelines for MAS classification criteria may not be met. [ABSTRACT FROM AUTHOR]

Objective: We retrospectively examined the nephrological conditions of the victims who applied to the Pediatric Nephrology Clinic in our hospital in İzmir after the February 6, 2023 Kahramanmaraş earthquake. Method: Age, gender, time spent under debris, number of crushed extremities, presence of acute kidney injury (AKI), creatine kinase (CK) levels and prognosis of all patients were evaluated. 5% dextrose-0.45% NaCl solution was given as 1500 cc/m2 /day if the CK levels of the children were between 1000-3000 U/L, and as 3000 cc/m2 /day for those >3000 U/L. If the bicarbonate value is <25, alkalinization was achieved by applying NaHCO3 treatment to 50 mEq/L. If CK values fell below 3000 U/L, the amount of fluid was reduced by half, and if it was below 1000 U/L, it was discontinued. If blood gas pH is >7.50 and/or bicarbonate ≥30, alkalinization treatment is discontinued; if 25-30 it is halved. Results: Of the total 33 pediatric patients, 48.5% were girls and 51.5% were boys. The children had a mean age of 9.0±3.9 years. The mean stay under the rubble was 17.00 (4.25-48.00) hours. The CK values of 23 patients were >1000 U/L at the time of admission. Six patients had acute kidney injuries at admission. Four patients received hemodialysis and/or hemodiafiltration treatment. The CK values returned to normal in 5.0 (3.0-8.0) days in the patients who received fluid and alkalinization treatments. The serum creatinine values of all patients normalized. Conclusion: Even in the case of concomitant AKI in crush syndrome developing after an earthquake, full recovery can be achieved with aggressive fluid and alkalinization treatment. [ABSTRACT FROM AUTHOR]

Background: One of the most common bacterial infections in childhood is urinary tract infection (UTI). Toll-like receptors (TLRs) contribute to immune response against UTI recognizing specific pathogenic agents. Our aim was to determine whether soluble TLR4 (sTLR4), soluble TLR5 (sTLR5) and interleukin 8 (IL-8) can be used as biomarkers to diagnose UTI. We also aimed to reveal the relationship between urine Heat Shock Protein 70 (uHSP70) and those biomarkers investigated in this study. Methods: A total of 802 children from 37 centers participated in the study. The participants (n = 282) who did not meet the inclusion criteria were excluded from the study. The remaining 520 children, including 191 patients with UTI, 178 patients with non-UTI infections, 50 children with contaminated urine samples, 26 participants with asymptomatic bacteriuria and 75 healthy controls were included in the study. Urine and serum levels of sTLR4, sTLR5 and IL-8 were measured at presentation in all patients and after antibiotic treatment in patients with UTI. Results: Urine sTLR4 was higher in the UTI group than in the other groups. UTI may be predicted using 1.28 ng/mL as cut-off for urine sTLR4 with 68% sensitivity and 65% specificity (AUC = 0.682). In the UTI group, urine sTLR4 levels were significantly higher in pyelonephritis than in cystitis (p < 0.0001). Post-treatment urine sTLR4 levels in the UTI group were significantly lower than pre-treatment values (p < 0.0001). Conclusions: Urine sTLR4 may be used as a useful biomarker in predicting UTI and subsequent pyelonephritis in children with UTI. [ABSTRACT FROM AUTHOR]

CONCLUDING REMARKS In this widespread tragic disaster, the TSPN was a part of the national/international collaborative effort to save patients' lives and to decrease crush injury and resultant acute kidney injury-related morbidity. NATIONAL COLLABORATIONS By cooperation with the Turkish Society of Nephrology, continuity of care was assured by contacting adult nephrologists when necessary, especially in centers without pediatric nephrologists. Since an earthquake should be considered a "kidney disaster" because of crush injuries and resultant acute kidney injury [[7]], the Turkish Society of Pediatric Nephrology (TSPN) took primary responsibility during the immediate and early phases of earthquake. Disasters from the perspective of pediatric nephrologist - 9 February 2023 Crush syndrome Disaster preparedness in pediatric nephrology centers Study suggestions After the earthquake - 16 February 2023 "How should I approach pediatric earthquake victims?". [Extracted from the article]

Background: The accuracy of conventional urinalysis in diagnosing urinary tract infection (UTI) in children is limited, leading to unnecessary antibiotic exposure in a large fraction of patients. Urinary heat shock protein 70 (uHSP70) is a novel marker of acute urinary tract inflammation. We explored the added value of uHSP70 in discriminating UTI from other infections and conditions confused with UTI. Methods: A total of 802 children from 37 pediatric centers in seven countries participated in the study. Patients diagnosed with UTI (n = 191), non-UTI infections (n = 178), contaminated urine samples (n = 50), asymptomatic bacteriuria (n = 26), and healthy controls (n = 75) were enrolled. Urine and serum levels of HSP70 were measured at presentation in all patients and after resolution of the infection in patients with confirmed UTI. Results: Urinary (u)HSP70 was selectively elevated in children with UTI as compared to all other conditions (p < 0.0001). uHSP70 predicted UTI with 89% sensitivity and 82% specificity (AUC = 0.934). Among the 265 patients with suspected UTI, the uHSP70 > 48 ng/mL criterion identified the 172 children with subsequently confirmed UTI with 90% sensitivity and 82% specificity (AUC = 0.862), exceeding the individual diagnostic accuracy of leukocyturia, nitrite, and leukocyte esterase positivity. uHSP70 had completely normalized by the end of antibiotic therapy in the UTI patients. Serum HSP70 was not predictive. Conclusions: Urine HSP70 is a novel non-invasive marker of UTI that improves the diagnostic accuracy of conventional urinalysis. We estimate that rapid urine HSP70 screening could spare empiric antibiotic administration in up to 80% of children with suspected UTI. A higher resolution version of the Graphical abstract is available as Supplementary information [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background: Aripiprazole-associated hypertension is a previously described side effect, but its mechanism has not been clearly elucidated. Here we present a 16-year-old male patient who developed aripiprazole-associated hypertension, and we discuss the mechanisms that may cause hypertension.Antipsychotic drugs are beneficial for the control of mental health conditions. In this report, we present a case of a patient who developed hypertension after using aripiprazole. A 16-year-old male patient was admitted with headache and high blood pressure. In the examinations conducted for etiology, the aldosterone/plasma renin activity (PRA) was found to be high, but in follow-up, hypertension regressed and aldosterone/PRA were detected normal. The patient was questioned again in terms of medical history. It was learned that the patient was initiated on aripiprazole, and hypertension developed after the drug. The aldosterone/PRA was studied 15 days after he stopped the drug. In this patient, hypertension and hyperaldosteronism were associated with aripiprazole.Case diagnosis: Aripiprazole-associated hypertension is a previously described side effect, but its mechanism has not been clearly elucidated. Here we present a 16-year-old male patient who developed aripiprazole-associated hypertension, and we discuss the mechanisms that may cause hypertension.Antipsychotic drugs are beneficial for the control of mental health conditions. In this report, we present a case of a patient who developed hypertension after using aripiprazole. A 16-year-old male patient was admitted with headache and high blood pressure. In the examinations conducted for etiology, the aldosterone/plasma renin activity (PRA) was found to be high, but in follow-up, hypertension regressed and aldosterone/PRA were detected normal. The patient was questioned again in terms of medical history. It was learned that the patient was initiated on aripiprazole, and hypertension developed after the drug. The aldosterone/PRA was studied 15 days after he stopped the drug. In this patient, hypertension and hyperaldosteronism were associated with aripiprazole. [ABSTRACT FROM AUTHOR]

Objective: Primary hyperoxaluria type 1 is an autosomal recessive rare disorder, caused by mutations in the alanine: glyoxylate aminotransferase (AGXT) gene. We aimed to detect the AGXT gene mutations causing primary hyperoxaluria type I in combined liver-kidney and isolated liver transplanted children with phenotypic characteristics of primary hyperoxaluria type 1. Methods: This study was carried out by including 6 Turkish children and their families followed by Dokuz Eylül. University Faculty of Medicine, Department of Pediatric Nephrology and diagnosed as primary hyperoxaluria with their phenotypic features. Clinical features, transplantation characteristics, and AGT catalytic activities of the cases were noted. The entire coding region including exon-intron boundaries of the AGXT gene was sequenced in patients and their family. Results: We detected 6 mutations primary hyperoxaluria type 1 causing and 2 minor allele polymorphism in 6 patients (5 families) The entire patients had at least one primary hyperoxaluria type 1-related mutation. Patient 1 had homozygous minor allele polymorphisms Pro11Leu in exon 1 and Ile340Met in exon 10, and mutation Met195Arg in exon 5. Patient 2 had homozygous mutation c. 33_34insC in exon 1. Patient 3 was compound heterozygous for mutations Gly170Arg in exon 4 and c.846+1G>A in intron 8 and heterozygous minor allele polymorphisms Ile340Met in exon 10. Patient 4 had homozygous mutation c.823-824dupAG in exon 8. Patient 5 and 6 had homozygous mutation c.976delG in exon 10. Conclusions: Our studies emphasize the mutation analysis of the entire coding region instead of targeted (exons 1, 4, and 7) mutation analysis of AGXT. [ABSTRACT FROM AUTHOR]

Acute focal bacterial nephritis is a localized, non liquefied bacterial inflammatory kidney mass affecting one or more lobes of the kidney. It is a midpoint between pyelonephritis and the formation of a kidney abscess. Mild encephalopathy with a reversible splenial lesion is identified by a temporary diffusion restriction in the selenium of the corpus callosum in magnetic resonance imaging. Neurological symptoms sometimes seen in mild encephalopathy with a reversible splenial lesion include changes in consciousness, behavioral changes, and seizures. Many infectious agents, particularly viruses, may be associated with the diagnosis. In recent years, the association between mild encephalopathy and a reversible splenial lesion has been described in some cases with acute focal bacterial nephritis. We present a case of a 9-year-old pediatric patient admitted to the pediatric emergency department with fever and seizures. Even though his urine sample revealed normal findings, he had higher levels of acute-phase reactants. With the aid of the computerized tomography and the magnetic resonance imaging scan, he was diagnosed with mild encephalopathy with reversible corpus callosum splenium lesion associated with acute focal bacterial nephritis. After appropriate treatment, he was discharged home successfully. As a conclusion, mild encephalopathy with reversible corpus callosum splenium lesion should be kept in mind for patients with neurological signs or symptoms accompanied by acute focal bacterial nephritis. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Aim: Kidney transplantation is the most preferred long-term treatment of children with end stage renal disease since 1954. Graft dysfunction has been divided into three categories based on timing of presentation. In this article, we aim to present the etiologic factors of acute graft dysfunction in the first year of renal transplantation in children. Materials and Methods: The patients, diagnosed with acute allograft dysfunction in first year of kidney transplantation, in University of Health Sciences Turkey, Tepecik Training and Research Hospital between March 2005 and October 2017 were analyzed prospectively in this study. Results: Over the 15 year period, 56 pediatric renal allograft patients were followed in University of Health Sciences Turkey, Tepecik Training and Research Hospital. During this period, 25 patients had diagnosed with early allograft dysfunction. Five patients were admitted two times in a year with allograft dysfunction. The mean age of patients was 12.6 (4.0±21.0) years, with a male and female ratio of 17:8. Delayed graft function was occurred in four patients (16%). Among 25 renal allograft recipients showed early graft dysfunction on average of 4.2 months (1-10 months) after transplantation. The etiology of early graft dysfunction showed 10% immunologic diseases and 90% non-immunologic factors. Five patients (16.7%) diagnosed with urinary tract infection, one patient (3.3%) diagnosed with cytomegalovirus nephropathy, five patients (16.7%) diagnosed with BK nephropathy, eight patients (26.7%) diagnosed with acute cyclosporine toxicity, seven patients (23.3%) were diagnosed with dehydration and one patient (3.3%) diagnosed with urologic anomalies. Twelve patients had performed renal allograft biopsy. The histological findings were consistent with T-cell mediated rejection in two patients; B-cell mediated rejection in one patient and viral nephropathy in five patients. Non-specific histological findings were determined in five patients. Conclusion: Early recognition of the etiology of graft dysfunction that develops at 1 year and appropriate treatment will contribute to the preservation of long-term graft dysfunction. [ABSTRACT FROM AUTHOR]

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. Contrastenhanced 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. [ABSTRACT FROM AUTHOR]

Aim: Enuresis (EN) is a common problem in childhood. Family physicians have an important role in revealing children with EN. The aim of this study was to assess the knowledge and attitude of family medicine (FM) residents regarding the diagnosis and treatment of enuresis in children. Materials and Methods: Family medicine residents of the University of Health Sciences, İzmir Tepecik Training and Research Hospital were invited to complete a questionnaire concerning enuresis in children. Those who had completed their paediatrics rotation training were defined as group 1, and those who had not completed their paediatrics rotation as group 2. The responses were compared between the groups. Results: Sixty (88%) of the FM residents agreed to complete the survey. The mean age of the participants was 28.7 (25-35) years, 38 (63%) had completed their paediatrics rotation (group 1). The question about the age of night-time bedwetting was more often answered correctly in group 1 (19/38) (p=0.025). In the question on the symptoms of non-monosymptomatic enuresis; the constipation option was marked as a symptom by only 15 of the participants (25%), with 13 (87%) being in group 1 (p=0.03). The correct response rates to the questions about other symptoms of non-monosymptomatic enuresis, the causes of secondary enuresis, the need for treatment, treatment options and the follow-up of patients with enuresis were similar between groups 1 and 2. Conclusion: As a result of this study, more correct answers, which revealed adequate knowledge and experience regarding enuresis, were obtained from those who had concluded their paediatrics rotation. [ABSTRACT FROM AUTHOR]

Patients with autosomal dominant polycystic kidney disease become symptomatic and are diagnosed usually at adulthood. The rate of nephrolithiasis in these patients is 5-10 times the rate in the general population, and both anatomic and metabolic abnormalities play role in the formation of renal stones. However, nephrolithiasis is rare in childhood age group. In this paper, an 11-year-old child with autosomal dominant polycystic kidney disease presenting with nephrolithiasis is discussed. [ABSTRACT FROM AUTHOR]

The article presents a quiz related to acute kidney injury and dark red-brown urine.

Background: Alport syndrome (AS) is an inherited glomerular disease caused by mutations in COL4A3, COL4A4, or COL4A5. Associations between clinical manifestations and genotype are not yet well defined. Our study aimed to define clinical and genetic characteristics, establish genotype–phenotype correlations, and determine prognosis of AS in children. Methods: A total of 87 children with AS from 10 pediatric nephrology centers, whom had genetic analyses performed at the Hacettepe University Nephrogenetics Laboratory between February 2017 and February 2019, were included. Data regarding demographics, family history, clinical and laboratory characteristics, histopathological and genetic test results, treatments, and yearly follow-up results were retrospectively analyzed. Results: Of 87 patients, 16% presented with nephrotic syndrome. In patients with nephrotic syndrome, kidney biopsy findings showed focal segmental glomerulosclerosis (FSGS) in 79%, and COL4A3 mutations were the leading genetic abnormality (50%). Twenty-four percent of all patients progressed to chronic kidney disease (CKD). The rate of progression to CKD and the decline in the glomerular filtration rate of the patients with COL4A3 mutation were higher than other mutation groups (p < 0.001 and p = 0.04, respectively). In kidney survival analysis, nephrotic syndrome presentation, histopathology of FSGS, COL4A3 mutations, and autosomal recessive inheritance were found as independent risk factors for earlier progression to CKD. Cyclosporin A treatment did not improve kidney survival. Conclusions: We emphasize that genetic testing is important for patients suspected as having AS. Furthermore, COL4A mutations should be considered in patients with FSGS and steroid-resistant nephrotic syndrome. This approach will shed light on the prognosis of patients and help with definitive diagnosis, preventing unnecessary and potentially harmful medications. [ABSTRACT FROM AUTHOR]

Aim: Acute bronchiolitis is a lower respiratory tract infection caused by viral agents in children aged under two years. Treatment includes hydration, oxygen, nebulized salbutamol, and intravenous steroids. This study aimed to determine the clinically related factors, the effect of viral agents on the clinical picture, and the efficacy of treatment methods in patients admitted with acute bronchiolitis. Material and Methods: Patients aged under two years of age who were hospitalized with a diagnosis of moderate/severe acute bronchiolitis between March 2015 and March 2019 were included in the study. Demographic data, hospitalization time, body temperature, presence of congenital heart disease, history of atopy, acute-phase reactants, mean platelet volume values, and respiratory virus panel results were recorded. The treatment modalities, length of hospitalization, intensive care hospitalization, and high-flow nasal cannula oxygen therapy (HFNC) were recorded. Results: Four hundred twenty-two patients were included in the study. The duration of hospitalization was found to be significantly longer in patients aged under one year and in patients with acyanotic congenital heart disease. A single viral agent was detected in 69 (51.9%) patients. Rhinovirus was detected in 70 patients and RSV was detected in 37. The duration of hospitalization was found to be significantly shorter in patients who received only oxygen and/or intravenous fluid treatment compared with those who received nebulized salbutamol and/or intravenous steroids. In addition, and there was no significant difference between the groups in terms of HFNC and hospitalization in the intensive care unit. Conclusion: Rhinovirus was the most common cause of acute bronchiolitis in our study. It was observed that congenital heart disease prolonged the length of hospitalization. In the treatment approaches, it was observed that hydration and oxygen therapy were sufficient treatment methods for the patients, in accordance with the recommendations of the American Academy of Pediatrics, and giving nebulized therapy prolonged the hospitalization period due to the treatment discontinuation steps. [ABSTRACT FROM AUTHOR]

The article provides answers to a quiz which asked about the most likely diagnosis, pathologies that should be considered and further investigations that should be done to an eight-year-old boy who presented with intermittent red urine during the previous week and abdominal pain for the previous 2 days.

Many new features have recently been incorporated to ÇEDD Çözüm/Child Metrics, an online and freely accessible scientific toolset. Various auxological assessments can now be made with data of children with genetic diseases (Prader Willi syndrome, Noonan syndrome, Turner syndrome, Down syndrome, and Achondroplasia) and preterm and term newborns. More detailed reports for height, weight, and body mass index data of a given child are now available. Last but not least, office and 24-hour ambulatory blood pressure values can be analyzed according to normative data. [ABSTRACT FROM AUTHOR]

Hafnia alvei is a rare bacterium that is generally reported to be an opportunistic infectious agent in adults. There are a limited number of identified pediatric cases in the literature. This article reports H. alvei as the causative agent of urinary tract infection in a healthy 8-year-old girl with no underlying disease. The patient recovered with a 10-day oral trimethoprim/sulfamethoxazole treatment. The aim of this study was to review the characteristics of this rare microorganism, as a causative agent of urinary tract infection, for clinicians. [ABSTRACT FROM AUTHOR]

A quiz concerning the periumbilical swelling and erythema is presented.

Fever of unknown origin has three most common etiologic categories in children including infectious diseases, connective tissue diseases, and neoplasms. Takayasu arteritis is a chronic vasculitis involving the large vessels, which generally presents with clinical features of vascular sequelae such as abdominal pain, hypertension, headaches and fever. In this article, we are presenting a 11 year-old girl admitted with fever lasting for one month and abdominal pain. Her physical examination and biochemical parameters were all within normal limits except for fever, abdominal pain and elevated acute phase reactant. Exclusion of infectious diseases, neoplasms and juvenile idiopatic arthritis, systemic lupus erythematosus; and demonstration of increased wall thickness in superior mesenteric artery and bilateral carotid arteries, established the diagnosis of Takayasu arteritis. It has been emphasized that fever of unknown origin and abdominal pain are atypical clinical manifestations of Takayasu arteritis, and early imaging including carotid Doppler ultrasonography may aid in the establishment of diagnosis of Takayasu arteritis in children. [ABSTRACT FROM AUTHOR]

Home hemodialysis (HD) has been supported by health authorities in many countries due to its nature of providing more freedom to the patients and the possibility to individualize the treatment. The treatment method is notable as a feasible dialysis option due to increase in life quality and improvement in the clinical findings of patients. However, pediatric home HD (HHD) is infrequently performed. In this study, we aimed to share our first year experience with HHD in a 15-year-old male patient. A 15-year-old male patient who had been receiving chronic peritoneal dialysis (CPD) for 13 years due to ESRD was transferred to chronic HD treatment due to treatment-resistant hypervolemia, hypertension, anemia and hyperphosphatemia associated with CPD failure. The education period was started with the consent of patient and his family and no problem was observed except short-term fear during the entry of a needle. Following the education period in the hospital, the treatment of the patient was continued at home. At the end of the first year of HHD treatment, increase in growth percentile and academic success, regression in the symptoms related to uremia, and also decrease in the use of medication and hospitalization were observed. Our patient felt better and had increased appetite, better growth scores and higher academic success, indicating that HHD could be a successful renal replacement treatment technique with its control of uremia-related symptoms and the decrease in medication needs of the patient. [ABSTRACT FROM AUTHOR]

Aim: To assess the relationship between mesangial hypercellularity in various childhood nephropathies and clinical and laboratory parameters. Methods and patients: The reports of the renal biopsies were evaluated retrospectively. The patients with diagnosis of IgA nephropathy (isolated and Henoch-Schönlein nephritis), IgM nephropathy, or isolated mesangial proliferative glomerulonephritis were included. Each nephropathy group was divided into two subgroups according to the severity of mesangial hypercellularity as mild and severe. The biochemical data and histopathological findings of the patients were recorded. Results: When the groups were compared, it was found that the patients with IgA nephropathy had hematuria ( p = 0.043) and the patients with IgM nephropathy had nephrotic syndrome more frequently than the other patients ( p = 0.01). No difference was detected between the groups regarding the severity of mesangial hypercellularity. On the other hand, when the groups were evaluated within themselves, no significant association was detected between the severity of mesangial hypercellularity and clinical and laboratory parameters. It was determined that the renal biopsy was performed earlier in patients with Henoch-Schönlein nephritis compared to the other cases ( p = 0.004). Compared to the isolated IgA nephropathy group, it was found that the number of cases with severe mesangial hypercellularity was higher and the level of proteinuria was more prominent in patients with Henoch-Schönlein nephritis. Additionally, when the patients with Henoch-Schönlein nephritis were evaluated, the degree of proteinuria was found to be higher in patients with severe mesangial hypercellularity compared to those of showing mild mesangial hypercellularity ( p = 0.002). Conclusion: It was observed that there is no direct relation between the severity of mesangial hypercellularity and clinical and laboratory findings in various childhood nephropathies. However, when Henoch-Schönlein nephritis is compared with IgA nephropathy, it was found that the severity of mesangial hypercellularity was higher in cases with Henoch-Schönlein nephritis and the level of proteinuria was more prominent in those cases. However, no difference was detected in glomerular filtration rates and biochemical data with regard to the level of mesangial hypercellularity. [ABSTRACT FROM AUTHOR]

The aim of this retrospective study was to evaluate our neonatal intensive care unit (NICU) patients' characteristics treated with acute peritoneal dialysis (PD) and their risk factors for mortality. We also wanted to share our experience of the application of PD in neonates who required less than 60 mL of dwell volume and their PD-related problems, as well as special solutions for these problems. This study included 27 infants treated in our NICU between February 2008 and December 2011. We retrospectively analyzed these patients' records. The percutaneous PD catheter was placed by us. PD procedure was performed either by manual technique or automated PD. Statistical evaluation was performed by using χ2-tests and Student's t-tests. In these 27 neonates, the average gestational age and birth weight were 35.18 ± 4.02 weeks and 2534.62 ± 897.41 g, respectively. The mean PD duration time was 6.11 ± 6.30 days. Of these, 10 patients were treated by manual technique, whereas 17 patients were treated with automated system. Among 27 neonates, 16 patients died. Overall mortality rate was 59.25%. PD-related complications were seen in 25.92% of patients. In conclusion, PD application is less effective and troublesome for low-birth-weight infants. Each center should create its own solutions to accommodate problematic patients in PD treatment to improve the outcome in this special population. [ABSTRACT FROM AUTHOR]

The article presents a quiz related to acute kidney injury and dark red-brown urine.

A quiz is presented on the most likely diagnosis, pathologies that should be considered and further investigations that should be done to an eight-year-old boy who presented with intermittent red urine during the previous week and abdominal pain for the previous 2 days.

The article presents a case study of a 5-year-old female patient presented with complaints of high fever and urinary turbidity existing for 4 days. Her medical history revealed she was born by cesarean section at 37th gestational week, weighing 2950 g and was operated on for myelomeningocele on the 3rd postnatal day.

Objective: To evaluate the efficiency of mesenchymal stem cells in ameliorating renal scarring in a rat pyelonephritis model. Methods: Three groups each, including 8 Sprague-Dawley rats were formed: Group 1 = sham operated (4 were given mesenchymal stem cells); group 2 = pyelonephritis induced by Escherichia coli; and group 3 = pyelonephritis and mesenchymal stem cells. Rats not given mesenchymal stem cells in group 1 and 4 rats in groups 2 and 3 were sacrificed on the eighth day for evaluation of inflammation, and the remaining rats were sacrificed at the sixth week to determine renal scarring along with migration of mesenchymal stem cells to renal tubules and differentiation to tubular cells expressing aquaporin-1. Results: Rats in group 3 had lower scores of both acute (8th day) and chronic (6th week) histopathological alterations compared with rats in group 2. By contrast, although rats in group 3 were shown to have mesenchymal stem cells expressing aquaporin-1 in their renal tubules, these cells were not detected in kidney tissue of mesenchymal stem cells–treated sham rats. Conclusion: These results indicate that mesenchymal stem cells migrated to renal tissues and ameliorated renal scarring in this rat model of pyelonephritis. [ABSTRACT FROM AUTHOR]