Spontaneous tendon or ligament ruptures in patients undergoing dialysis: First pediatric case report and literature review.

Spontaneous tendon or ligament ruptures are quite rare and mostly associated with chronic systemic diseases such as diabetes mellitus, systemic lupus erythematosus, rheumatoid arthritis, and chronic kidney disease (CKD). In this study, we present the first documented case of a spontaneous rupture of the medial patellofemoral ligament (MPFL) in a pediatric patient. The patient was undergoing long-term peritoneal dialysis (PD) and had a history of severe secondary hyperparathyroidism. Additionally, we discussed spontaneous tendon and ligament ruptures associated with CKD or dialysis through a comprehensive literature review. This case report highlights the importance of recognizing that spontaneous tendon or ligament injuries are not exclusive to adults; children with CKD can also be affected. Several factors including poor parathyroid hormone (PTH) and metabolic acidosis control, prolonged CKD duration and presence of malnutrition play role in the pathogenesis. Early diagnosis is crucial as it allows for timely surgical intervention and leads to a favorable functional recovery.

Urine soluble TLR4 levels may contribute to predict urinary tract infection in children: the UTILISE Study.

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. A higher resolution version of the Graphical abstract is available as Supplementary information.

Comprehensive evaluation of patients with primary hyperoxaluria type 1: A nationwide study.

BACKGROUND: Primary hyperoxaluria type 1 (PH1) is characterized by increased endogenous oxalate production and deposition as calcium oxalate crystals. The main manifestations are nephrocalcinosis/nephrolithiasis, causing impaired kidney function. We aimed to evaluate the clinical characteristics and overall outcomes of paediatric PH1 patients in Turkey. METHODS: This is a nationwide, multicentre, retrospective study evaluating all available paediatric PH1 patients from 15 different paediatric nephrology centres in Turkey. Detailed patient data was collected which included demographic, clinical and laboratory features. Patients were classified according to their age and characteristics at presentation: patients presenting in the first year of life with nephrocalcinosis/nephrolithiasis (infantile oxalosis, Group 1), cases with recurrent nephrolithiasis diagnosed during childhood (childhood-onset PH1, Group 2), and asymptomatic children diagnosed with family screening (Group 3). RESULTS: Forty-eight patients had a mutation consistent with PH1. The most common mutation was c.971_972delTG (25%). Infantile oxalosis patients had more advanced chronic kidney disease (CKD) or kidney failure necessitating dialysis (76.9% vs. 45.5%). These patients had much worse clinical course and mortality rates seemed to be higher (23.1% vs. 13.6%). Patients with fatal outcomes were the ones with significant comorbidities, especially with cardiovascular involvement. Patients in Group 3 were followed with better outcomes, with no kidney failure or mortality. CONCLUSION: PH1 is not an isolated kidney disease but a systemic disease. Family screening helps to preserve kidney function and prevent systemic complications. Despite all efforts made with traditional treatment methods including transplantation, our results show devastating outcomes or mortality.

Comparison of echocardiographic aortic stiffness index measurements and pulse wave velocity measurements in obese and overweight children.

BACKGROUND: Aortic/arterial stiffness is a reliable, independent predictor and a risk factor for cardiovascular mortality. Arterial stiffness is assessed by pulse wave velocity and echocardiography. The purpose of this study is to analyse aortic/arterial stiffness in patients using echocardiographic and pulse wave velocity techniques. MATERIALS AND METHODS: The participants of this study consisted of 62 patients who presented to the Gazi University Pediatric Endocrinology and Pediatric Cardiology outpatient clinics, including 21 obese, 20 overweight, and 21 normal-weight patients. Echocardiography was performed on all patients, and echocardiographic measurements were compared to pulse wave velocity measurements. RESULTS: The mean (min-max) arterial strain measurements were 0.146 ± 0.0 (0.06-0.3) in the obese group and 0.106 ± 0.0 (0.05-0.18) in the overweight group. In comparison to the overweight group, the obese group had greater arterial strain measurements. The pulse wave velocity measurements in the obese and overweight groups were greater than those in the normal weight group (p > 0.05). Elastic modulus and aortic stiffness ß index values were shown to be positively correlated with pulse wave velocity measurements in the obese group (r = 0.56, r = 0.53, respectively; p = 0.008, p = 0.01, respectively). Systolic and diastolic blood pressure measurements were correlated with pulse wave velocity measurements in the obese group (r = 0.98, p = 0.0001, respectively). CONCLUSION: In our study, echocardiographic aortic measurements showing the vessel wall were correlated with pulse wave velocity measurements. Echocardiographic evaluation should be included in the routine follow-up of patients because pulse wave velocity measurement devices are not available in all centres, echocardiography is available in many centres, it is easily applicable, and it facilitates the follow-up of patients.

Serum osmolality and hyperosmolar states.

Serum osmolality is the sum of the osmolalities of every single dissolved particle in the blood such as sodium and associated anions, potassium, glucose, and urea. Under normal conditions, serum sodium concentration is the major determinant of serum osmolality. Effective blood osmolality, so-called blood tonicity, is created by the endogenous (e.g., sodium and glucose) and exogenous (e.g., mannitol) solutes that are capable of creating an osmotic gradient across the membranes. In case of change in effective blood osmolality, water shifts from the compartment with low osmolality into the compartment with high osmolarity in order to restore serum osmolality. The difference between measured osmolality and calculated osmolarity forms the osmolal gap. An increase in serum osmolal gap can stem from the presence of solutes that are not included in the osmolarity calculation, such as hypertonic treatments or toxic alcoholic ingestions. In clinical practice, determination of serum osmolality and osmolal gap is important in the diagnosis of disorders related to sodium, glucose and water balance, kidney diseases, and small molecule poisonings. As blood hypertonicity exerts its main effects on the brain cells, neurologic symptoms varying from mild neurologic signs and symptoms to life-threatening outcomes such as convulsions or even death may occur. Therefore, hypertonic states should be promptly diagnosed and cautiously managed. In this review, the causes and treatment strategies of hyperosmolar conditions including hypernatremia, diabetic ketoacidosis, hyperglycemic hyperosmolar syndrome, hypertonic treatments, or intoxications are discussed in detail to increase awareness of this important topic with significant clinical consequences.

Evaluation of the Claria sharesource system from the perspectives of patient/caregiver, physician, and nurse in children undergoing automated peritoneal dialysis.

BACKGROUND: Automated peritoneal dialysis (APD) is increasingly preferred worldwide. By using a software application (Homechoice with Claria sharesource system (CSS)) with a mod-M added to the APD device, details of the home dialysis treatment become visible for PD nurses and physicians, allowing for close supervision. We aimed to evaluate the perceptions of patients/caregivers, PD nurses, and physicians about the advantages and disadvantages of CSS. METHODS: Three different web-based questionnaires for patients/caregivers, nurses, and physicians were sent to 15 pediatric nephrology centers with more than 1 year of experience with CSS. RESULTS: Respective questionnaires were answered by 30 patients/caregivers, 22 pediatric nephrologists, and 15 PD nurses. Most of the nurses and physicians (87% and 73%) reported that CSS improved patient monitoring. A total of 73% of nurses suggested that CCS is not well known by physicians, while half of them reported reviewing CSS data for all patients every morning. Sixty-eight percent of physicians thought that CSS helps save time for both patients/caregivers and healthcare providers by reducing visits. However, only 20% of patients/caregivers reported reduced hospital visits. A total of 90% of patients/caregivers reported that being under constant monitoring made them feel safe, and 83% stated that the patient's sleep quality improved. CONCLUSIONS: A remote monitoring APD system, CSS, can be successfully applied with children for increased adherence to dialysis prescription by giving shared responsibility and may help increase the patient's quality of life. This platform is more commonly used by nurses than physicians. Its potential benefits should be evaluated in further well-designed clinical studies with larger patient groups. A higher resolution version of the Graphical abstract is available as Supplementary information.

Urinary HSP70 improves diagnostic accuracy for urinary tract infection in children: UTILISE study.

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.

Electrocardiographic measurements in children with pre-dialysis chronic kidney disease and undergoing kidney replacement therapy.

Cardiovascular diseases are the main causes of morbidity in children with chronic kidney disease (CKD). Electrocardiography (ECG) can provide important information about cardiac functions and parameters associated with sudden cardiac death. This study aims to evaluate the potentially dangerous changes in CKD and kidney replacement therapies by ECG and to determine the value of ECG in predicting cardiovascular outcome compared with echocardiography. 101 patients with CKD were divided into subgroups according to treatment modalities as pre-dialysis CKD, hemodialysis (HD), peritoneal dialysis (PD) and kidney transplantation (KTx). Differences in anthropometric measurements, laboratory results, blood pressures, ECG monitoring were compared within groups as well as with 40 healthy controls. Available echocardiographic findings were noted. In the patients, HD group had highest frequency of hypertension. ECG revealed prolonged QTc as more frequent (16.8% vs 0%, p = 0.006) and higher QTcD (56.7 ± 6.5 vs 39.9 ± 5.1 ms, p = 0.001) in the patients compared to controls, especially in dialysis patients, whereas lowest values were in KTx subgroup. Left ventricular (LV) hypertrophy (LVH) was more frequent (47.1%) in HD compared to other CKD subgroups in ECG (p = 0.052). Echocardiography also showed LV mass index as highest in HD and lowest in KTx (121.4 ± 55.7 vs 63.7 ± 18.3 g/m2, p = 0.000), with numerically highest LVH in HD (58.3%, p = 0.063).  Conclusion: ECG can be used to detect cardiovascular problems in patients with CKD, especially in HD. As ECG results were in line with echocardiography, patients with ECG abnormalities suggestive of LVH should be referred for echocardiographic assessment. What is Known: • Cardiovascular diseases such as coronary artery disease, congestive heart failure, arrhythmias and sudden cardiac death are major causes of morbidity and mortality in chronic kidney disease. • Electrocardiography has significant advantages in demonstrating cardiac functions in children because it is readily available, non-invasive and often non-experts can interpret the results. What is New: • The heart rate is higher, QTc is longer and QTcD is higher in dialysis patients and the prolonged QTc is more frequent in patients with underlying glomerular diseases. • Left ventricular hypertrophy is more common in HD patients and those with hypertension, hypercalcemia, anemia or glomerular etiology. The cardiovascular risky conditions are less frequent in the patients with kidney transplantation.

Eosinophilic peritonitis in children undergoing maintenance peritoneal dialysis: A case report and literature review.

Eosinophilic peritonitis (EP) constitutes a significant number of culture-negative peritonitis cases that can affect 16-60% of the patients who are treated with maintenance peritoneal dialysis (PD). Although it is mainly considered to be the hypersensitivity response of the peritoneum to foreign substances, it can also develop following culture-positive peritonitis attacks. Besides the presence of more than 100 white blood cells (WBC)/ml, the diagnosis is made with the high number of eosinophils in the dialysate fluid (>10%), usually accompanied by peripheral eosinophilia. In this study, a 12-year-old male patient, who was diagnosed as EP as early as in the first week of PD catheter placement and treated with systemic antihistamines was reported. Additionally, clinical aspects and treatment modalities of EP are presented with a detailed literature review. Although EP is usually a self-limiting clinical manifestation with a benign outcome, it can be overlooked due to the lack of a routine reporting of the count and percentage of peritoneal eosinophils in most centers. For this reason, a detailed examination in culture-negative peritonitis cases for EP in order to avoid unnecessary antibiotic use for these patients should be the strategy.

The effect of COVID-19 restriction on metabolic syndrome in primary hypertension.

BACKGROUND: Primary hypertension (HT) has been increasingly reported in parallel to the increase in the prevalence of obesity in children, both of which are important components of metabolic syndrome. The aim of this study was to investigate the effects of COVID-19 restrictions, which are believed to induce lifestyle changes and physical inactivity, on the parameters of metabolic syndrome in children with primary hypertension. METHODS: This was an observational, pre-post study conducted on pediatric patients with primary HT. The first phase of the study was the period prior to when COVID-19 restrictions were put in place in Turkey, and the second phase was up to the date when the restrictions were lifted. Anthropometric and blood pressure measurements, laboratory tests, and hypertensive-mediated organ damage at both phases of the study were compared. RESULTS: Severe restrictions due to the COVID-19 pandemic were associated with an increase in mean ± standard deviation body mass index (BMI) (26.4 ± 7.3 vs. 27.2 ± 7.1, P = 0.002), antihypertensive drug use (n = 53 (57.6%) vs. n = 59 (64.1%), P < 0.0001), fasting blood glucose level (89.4 ± 12.6 vs. 94.1 ± 14.2, P = 0.013), and a borderline elevation in total cholesterol (21 [22.8%] vs. 28 [30.4%], P < 0.0001). These increases negatively affected end organs, with an increased frequency of interventricular septum hypertrophy (n = 12 [13%] vs. n = 17 [18.5%], P = 0.031). CONCLUSIONS: COVID-19 restrictions were associated with an increased risk of parameters associated with metabolic syndrome in patients with primary hypertension. Physicians should carefully monitor the weight, blood pressure, fasting plasma glucose level, and total cholesterol levels in patients during periods of movement/activity restrictions such as during the COVID-19 pandemic.

Evaluation of non-infectious complications of peritoneal dialysis in children: a multicenter study.

BACKGROUND: Peritoneal dialysis (PD) is the most common kidney replacement therapy in children. Complications associated with PD affect treatment success and sustainability. The aim of this study was to investigate the frequency of PD-related non-infectious complications and the predisposing factors. METHODS: Retrospective data from 11 centers in Turkey between 1998 and 2018 was collected. Non-infectious complications of peritoneal dialysis (NICPD), except metabolic ones, in pediatric patients with regular follow-up of at least 3 months were evaluated. RESULTS: A total of 275 patients were included. The median age at onset of PD and median duration of PD were 9.1 (IQR, 2.5-13.2) and 7.6 (IQR, 2.8-11.9) years, respectively. A total of 159 (57.8%) patients encountered 302 NICPD within the observation period of 862 patient-years. The most common NIPCD was catheter dysfunction (n = 71, 23.5%). At least one catheter revision was performed in 77 patients (28.0%). Longer PD duration and presence of swan neck tunnel were associated with the development of NICPD (OR 1.191; 95% CI 1.079-1.315, p = 0.001 and OR 1.580; 95% CI 0.660-0.883, p = 0.048, respectively). Peritoneal dialysis was discontinued in 145 patients; 46 of whom (16.7%) switched to hemodialysis. The frequency of patients who were transferred to hemodialysis due to NICPD was 15.2%. CONCLUSIONS: Peritoneal dialysis-related non-infectious complications may lead to discontinuation of therapy. Presence of swan neck tunnel and long duration of PD increased the rate of NICPD. Careful monitoring of patients is necessary to ensure that PD treatment can be maintained safely.

Severity of self-reported depressive symptomatology and relevant factors in children with primary monosymptomatic nocturnal enuresis and their mothers.

BACKGROUND: Nocturnal enuresis (NE) may negatively affect social and emotional life as well as mood in both children and their mothers. The aim of this study is to evaluate severity of self-reported depressive symptomatology and determine the relevant factors in children with primary monosymptomatic nocturnal enuresis (MNE) and their mothers by using depression inventories. METHODS: Children Depression Inventory (CDI) for children and Beck Depression Inventory (BDI) for mothers were administered to the study group. The children and mothers in the patient and control groups were compared according to the depression inventory scores. The relationship of various sociodemographic factors with those scores was also investigated. RESULTS: BDI scores of the mothers of children with primary MNE demonstrated minor depressive symptomatology and were significantly higher than the mothers in the control group (p = 0.002). Moreover, although within the normal range, CDI scores of the children with primary MNE were also significantly higher than the controls (p = 0.031). Main factors associated with BDI scores were the presence of primary MNE, maternal educational level, and CDI scores. School achievement of the children, monthly income of the family, and BDI scores were found to be correlated to the CDI scores. CONCLUSIONS: Primary MNE was found to be associated with negative mood of the mothers in the present study. As misinformed parental attitudes adversely affect family dynamics, improved awareness of, and maternal education regarding primary MNE is vital in improving the holistic outcome of families affected by MNE.

An adolescent boy progressing insidiously to end-stage renal disease: Answers.

Hinman syndrome was a non-neurogenic neurogenic bladder and the most severe form of dysfunctional voiding disorder. The bladder-sphincter discoordination causes damage to the bladder and upper urinary tract if it is not diagnosed early and treated adequately. This case emphasizes the following important message: nighttime wetting is not a benign condition in every child. Parental awareness should be raised about voiding disorders, as it may be possible to prevent important renal diseases such as Hinman syndrome.

An adolescent boy progressing insidiously to end-stage renal disease: Questions.

Hinman syndrome (HS), or non-neurogenic neurogenic bladder, is a voiding dysfunction of the bladder of neuropsychological origin that is characterized by functional bladder outlet obstruction in the absence of neurologic deficits. The bladder-sphincter discoordination causes damage to the bladder and upper urinary tract if it is not timely diagnosed and adequately treated. This case emphasizes the following important message; nighttime wetting is not a benign condition in every child. Parental awareness should be raised about voiding disorders, so it may be possible to prevent some important renal diseases such as Hinman syndrome.