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.

Rituximab use in paediatric nephrology practice in Türkiye: TRTX.

AIM: Rituximab (RTX) is a genetically engineered chimeric monoclonal antibody which binds directly to CD20 antigen and mediates inhibition of B cell development. Although RTX has been widely used in paediatric nephrology, there is no routine protocol for its use. In this study, paediatric nephrologists in Türkiye were asked to fill out a questionnaire to understand their practice in using RTX. This study aimed to determine common practices and clarify the uncertainties regarding the use of RTX in paediatric nephrology. METHODS: This was a nationwide, multicenter, retrospective cohort study based on data evaluating the use of RTX in paediatric nephrology practice. An online questionnaire was sent to all paediatric nephrology centers in Türkiye. The questionnaire forms included information about how many patients in total applied RTX treatment, for which indications they use RTX, and whether they made any preparations before using RTX. RESULTS: According to this survey on RTX use in Türkiye, paediatric nephrologists use it most commonly in SSNS and followed by SRNS, ABMR, SLE and AAV, respectively. Dosing was highly standard but there is significant heterogeneity in pre-exposure tests and patient monitoring in the clinical practice of RTX. Also, the rate of encountering RTX-related allergic and infectious side effects at least once during the professional experience of our physicians can be quite high. CONCLUSION: There is an increasing need for the preparation of a guideline on the indications for RTX use for each diagnosis, posology, and the practices to be performed before and after infusion.

A rare cause and an even rarer treatment of hypertension in a 5-year-old boy: Mid-aortic syndrome.

Renal artery stenosis is one of the common vascular diseases that cause hypertension in children. However, renal artery aneurysms and abdominal aortic aneurysms, which may be components of mid-aortic syndrome, are rarely associated with renal artery stenosis. Despite its rarity, early diagnosis and treatment are critical to prevent fatal complications. Currently, non-surgical invasive techniques are considered the first choice for treatment, but in some cases, surgery is inevitable. Here, we present a 5-year-old boy with a mid-aortic syndrome. The patient presented with a history of severe headache and epistaxis 5-6 times a day and was diagnosed with hypertension. A 9 × 9 mm saccular aneurysm on the anterior surface of the abdominal aorta at the level of the left renal artery ostium, and a 12 mm aneurysm in the left renal artery after a stenotic segment at the hilum level was detected in the doppler USG and contrast-enhanced imaging techniques. The patient was operated on electively. We used a PTFE patch to repair the abdominal aorta and, saphenous vein which was taken from his father to repair the renal artery. The patient recovered well and was discharged on the 18th day.

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.

Allelic Variants of the Complement Genes in Acute Postinfectious Glomerulonephritis.

Acute postinfectious glomerulonephritis (APIGN) is one of the most common causes of acute glomerulonephritis in children. It may lead to inflammation and proliferation of glomerular tissue through immunologic mechanisms (Balasubramanian R, Paediatr Int Child Health 2017;37:240-247).

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.

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.

COVID-19 in pediatric nephrology centers in Turkey.

BACKGROUND: There is limited data on COVID-19 disease in children with kidney disease. We aimed to investigate the characteristics and prognosis of COVID-19 in pediatric nephrology patients in Turkey. METHODS: This was a national, multicenter, retrospective cohort study based on an online survey evaluating the data between 11th March 2020 and 11th March 2021 as an initial step of a detailed pediatric nephrology COVID-19 registry. RESULTS: Two hundred and three patients (89 girls and 114 boys) were diagnosed with COVID-19. One-third of these patients (36.9%) were between 10-15 years old. Half of the patients were on kidney replacement therapy: kidney transplant (KTx) recipients (n = 56, 27.5%), patients receiving chronic hemodialysis (n = 33, 16.3%) and those on peritoneal dialysis (PD) (n = 18, 8.9%). Fifty-four (26.6%) children were asymptomatic. Eighty-two (40.3%) patients were hospitalized and 23 (28%) needed intensive care unit admission. Fifty-five percent of the patients were not treated, while the remaining was given favipiravir (20.7%), steroid (16.3%), and hydroxychloroquine (11.3%). Acute kidney injury developed in 19.5% of hospitalized patients. Five (2.4%) had MIS-C. Eighty-three percent of the patients were discharged without any apparent sequelae, while 7 (3.4%) died. One hundred and eight health care staff were infected during the study period. DISCUSSION: COVID-19 was most commonly seen in patients who underwent KTx and received HD. The combined immunosuppressive therapy and frequent exposure to the hospital setting may increase these patients' susceptibility. Staff infections before vaccination era were alarming, various precautions should be taken for infection control, particularly optimal vaccination coverage.