Adolescence-onset atypical hemolytic uremic syndrome: is it different from infant-onset?

BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is a rare, mostly complement-mediated thrombotic microangiopathy. The majority of patients are infants. In contrast to infantile-onset aHUS, the clinical and genetic characteristics of adolescence-onset aHUS have not been sufficiently addressed to date. METHODS: A total of 28 patients (21 girls, 7 boys) who were diagnosed as aHUS between the ages of ≥10 years and <18 years were included in this study. All available data in the Turkish Pediatric aHUS registry were collected and analyzed. RESULTS: The mean age at diagnosis was 12.8±2.3 years. Extra-renal involvement was noted in 13 patients (46.4%); neurological involvement was the most common (32%). A total of 21 patients (75%) required kidney replacement therapy. Five patients (17.8%) received only plasma therapy and 23 (82%) of the patients received eculizumab. Hematologic remission and renal remission were achieved in 25 (89.3%) and 17 (60.7%) of the patients, respectively. Compared with the infantile-onset aHUS patients, adolescent patients had a lower complete remission rate during the first episode (p = 0.002). Genetic analyses were performed in all and a genetic variant was detected in 39.3% of the patients. The mean follow-up duration was 4.9±2.6 years. At the last visit, adolescent patients had lower eGFR levels (p = 0.03) and higher rates of chronic kidney disease stage 5 when compared to infantile-onset aHUS patients (p = 0.04). CONCLUSIONS: Adolescence-onset aHUS is a rare disease but tends to cause more permanent renal dysfunction than infantile-onset aHUS. These results may modify the management approaches in these patients.

Acute kidney injury in pediatric burn patients.

BACKGROUND: Acute kidney injury (AKI) is a common and important complication of burn injury. Although there are numerous adult studies, data regarding AKI in pediatric burn patients are scarce. Here, we aimed to evaluate the frequency, clinical features, and prognosis of AKI among pediatric burn injury patients. METHODS: This is a retrospective cohort study. Patients aged between 1 month and 18 years who had been followed up between the years 2011 and 2017 were included, and patients with previous kidney disease were excluded. Demographic data, laboratory and clinical variables, management strategies, and outcome data were obtained from the hospital records. Factors associated with AKI were determined by logistic regression analysis. RESULTS: A total of 697 patients had been followed up, and 87 (12.5%) had AKI. Older age, refugee status, prolonged duration between the incident and time of hospitalization, presence of sepsis, severity and type of burn, volume of fluid administration, intubation status, and accompanying organ failure were all associated with the development of AKI. According to multivariate logistic regression analysis, the most statistically significant factors associated with the development of AKI were older age and increased serum hemoglobin values. In terms of outcomes, length of stay and mortality increased in patients with AKI when compared with patients without AKI. CONCLUSION: Similar to adults, AKI is an important and common complication of burn injury in pediatric burn patients and is associated with increased length of stay, morbidity, and mortality. Early recognition and prompt and appropriate management are crucial to avoid morbidity and mortality.

Acute kidney injury and risk factors in pediatric patients undergoing hematopoietic stem cell transplantation.

BACKGROUND: Acute kidney injury (AKI) is a common complication of hematopoietic stem cell transplantation (HSCT) with increased mortality and morbidity. Understanding the risk factors for AKI is essential. This study aimed to identify AKI incidence, risk factors, and prognosis in pediatric patients post-HSCT. METHODS: We conducted a retrospective case-control study of 278 patients who were divided into two groups: those with AKI and those without AKI (non-AKI). The groups were compared based on the characteristics and clinical symptoms of patients, as well as post-HSCT complications and the use of nephrotoxic drugs. Logistic regression analysis was employed to identify the risk factors for AKI. RESULTS: A total of 16.9% of patients had AKI, with 8.5% requiring kidney replacement therapy. Older age (OR 1.129, 95% CI 1.061-1.200, p < 0.001), sinusoidal obstruction syndrome (OR 2.562, 95% CI 1.216-5.398, p = 0.011), hemorrhagic cystitis (OR 2.703, 95% CI 1.178-6.199, p = 0.016), and nephrotoxic drugs, including calcineurin inhibitors, amikacin, and vancomycin (OR 17.250, 95% CI 2.329-127.742, p < 0.001), were identified as significant independent risk factors for AKI following HSCT. Mortality rate and mortality due to AKI were higher in stage 3 patients than those in stage 1 and 2 AKI (p = 0.019, p = 0.007, respectively). Chronic kidney disease developed in 1 patient (0.4%), who was in stage 1 AKI (2.1%). CONCLUSIONS: AKI poses a serious threat to children post-HSCT, leading to alarming rates of mortality and morbidity. To enhance outcomes and mitigate these risks, it is vital to identify AKI risk factors, adopt early preventive strategies, and closely monitor this patient group.

Pediatric kidney care experience after the 2023 Turkey/Syria earthquake.

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.

A Nine-year-old Boy with Renovascular Hypertension: Knowing Where to Look.

Renal artery entrapment (RAE) by hypertrophic diaphragmatic crura is an extremely rare cause of renovascular hypertension (RVH). Here, we report the case of a 9-year-old boy diagnosed with RVH caused by right RAE by a hypertrophic diaphragmatic crus and successfully managed with close medical monitoring. Diagnosis of this entity is easily overlooked if the optimal views are not obtained during imaging, which depends on a high index of suspicion. We would like to remind clinicians to keep this rare condition in mind when evaluating children with RVH.

Spontaneous remission of nephrotic syndrome associated with COVID-19 infection in an 8-year-old boy.

BACKGROUND: It is already known that viral infections, exclusively upper respiratory tract infections may trigger relapses of nephrotic syndrome. Recently, COVID-19 disease has also been reported to be related with relapse of nephrotic syndrome in a few pediatric cases Case. Here we present an 8-year-old boy who had relapse of nephrotic syndrome due to COVID-19 infection. He was asymptomatic except for mild edema. He was managed supportively, no medication was started and went into spontaneous remission in 7 days. CONCLUSIONS: Viral infections particularly upper respiratory tract infections may trigger relapse of nephrotic syndrome. COVID-19 has also been reported to be related with relapses of nephrotic syndrome in a few pediatric cases. Spontaneous remission in our patient indicates the importance of close monitoring of patients before starting long term treatment with steroids.

Premature ovarian failure in juvenile systemic lupus erythematosus patient: A rare presentation.

A 16-year-old girl was admitted to the hospital for the absence of a menstrual bleeding over 10 mounts. She has had regular menstrual periods since she was 13 years old and no history of systemic disease. Laboratory tests showed low estrogen (<11.8 ng/L, range 16-238 ng/L), elevated FSH (follicle stimulating hormone) (138.3 U/L), low AMH (anti-Mullerian hormone) (<0.01 µg/L), normal prolactin (7 µg/L), and thyroid hormone levels (TSH: 1.5 mU/L). The ovaries were prepubertal in size and diagnosed with primer ovarian failure. ANA titers, antiphospholipid antibody, and direct coombs were positive. Microscopic hematuria and nephritic proteinuria (34 mg/m2/hour) were also detected and renal biopsy was revealed stage 3-5 lupus nephritis. Premature ovarian failure is an extremely rare disease in childhood. When POF is detected in the adolescent, it is important to investigate the etiology. In this way, it may be possible to diagnose diseases with high morbidity and mortality that may cause POF.

Can microalbuminuria be an indicator of renal involvement in pediatric Covid 19 patients?

BACKGROUND: Proteinuria (both tubular and glomerular in origin) and its implications are well-known features of adult patients with COVID19. However currently studies addressing proteinuria and its role in the outcome of kidney and patients of pediatric COVID 19 is scarce. We aimed to evaluate the presence of microalbuminuria in order to detect early renal involvement in pediatric COVID 19 patients. METHODS: We prospectively evaluated 100 pediatric patients hospitalized with COVID 19 between April and July 2020. Clinical presentations, laboratory findings and outcomes were investigated. Microalbuminuria was compared with the age, gender, disease severity, and hemoglobin, platelet, leukocyte count and serum CRP levels of the patients. RESULTS: Twenty seven out of 100 patients had microalbuminuria. Fourteen patients had mild and fourteen had moderate disease. There was not any significant relation according to age and gender. Microalbuminuria was not related to the severity of the disease. Also the mean microalbuminuria level did not differ according to the disease course. Hemoglobin, platelet, leukocyte counts and serum CRP levels were also were not correlated with microalbuminuria levels. CONCLUSION: Although there was no difference between the groups with different disease course; microalbuminuria is detected in an important ratio of pediatric patients with COVID 19 in this study. In the highlight of our findings we suggest that urinary findings of pediatric COVID patients should be carefully evaluated.

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.

Effect of enuresis on perceived parental acceptance-rejection in children.

OBJECTIVE: Enuresis is a major problem affecting both the child and his family. This study aimed to investigate the effect of enuresis on mother acceptance-rejection perceived by children. MATERIAL AND METHODS: Fourty-six children and their mothers with the diagnosis of primary enuresis were included in the study. Parental Acceptance-Rejection Scale which consisted of 60 questions and consisted of four subscales: affection, hostility, neglect, and undifferentiated rejection was applied to both mothers and children. A dependent Sample t-test was used to compare the scale results of mothers and children, and an independent sample t-test was used to determine the factors affecting perceived high mother hostility in children. RESULTS: The mean age was 10.12±1.34 years and 58.70% of the children were boys. There was no statistically significant difference between total acceptance-rejection, affection, neglect, and undifferentiated rejection scores of mothers and children's perceptions. The perceived hostility score of the children (25.71±8.05) was higher than the mothers' hostility score (22.52±6.26) (p<0.05). The presence of maternal chronic disease was found to increase the perceived high hostility, while other factors were not statistically significant. Thirteen cases with chronic illnesses were excluded and re-analysis revealed that the difference between perceived and mother hostility persisted (p<0.05). CONCLUSION: Children with enuresis perceive their mother's behavior as more hostile than they are. It should be kept in mind that enuresis may affect the mother and child relationship, the family should be informed about the approach to the child.

Could plasma based therapies still be considered in selected cases with atypical hemolytic uremic syndrome?

BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) occurs due to defective regulation of the alternative complement pathway (ACP) on vascular endothelial cells. Plasma based therapy (PT) was the mainstay of the treatment for aHUS for many years until the introduction of therapies targeting blockage of the complement system. The aim of this study was to evaluate patients with aHUS who had been treated with plasma based therapies alone. METHODS: The outcomes of seven genetically confirmed aHUS patients (2 girls, 5 males) were evaluated by means of clinical presentation, response to plasma therapy, course of the disease during the follow-up period and last status. RESULTS: The median age of the patients at admission was 6.7 years (IQR 0.7-7.8). Three patients received plasma exchange therapy and the other four patients were treated with plasma infusions. One patient was lost to follow-up after one year; the median duration of follow-up for other patients was 3.7 years (IQR 2.7-6.5). During the follow up, two patients from our historical records when complement blocking therapies had not been in clinical use yet in Turkey, underwent kidney transplantation. One transplant patient experienced an acute rejection episode without graft loss. The remaining five patients had a glomerular filtration rate of more than 90 ml/min./1.73 m < sup > 2 < /sup > at the last visit. CONCLUSION: Although we had a relatively small patient population, our findings indicate that PT might still be considered in selected patients particularly in countries where complement blocking therapies are difficult to reach due to their unavailability or costs that are not covered by the health care systems.