Neonatal Renal Failure Research Articles

Acute Renal Failure in Neonates:Clinical and Laboratory Profile

Acute Renal Failure in Neonates:Clinical and Laboratory Profile

Risk Factors Associated with Acute Renal Failure in Neonates of a neonatal Intensive Care Unit

Objetive: Determining the risk factors associated with acute renal failure in neonates.
 Materials and Methods: A case-control study was carried out, in 65 cases of acute renal failure and 65 without acute renal failure in the Intensive Care Unit Neonatals (NICU) of a hospital in Pachuca Hidalgo, Mexico. The variables studied; prematurity, low weight, type I respiratory distress syndrome, perinatal asphyxia, sepsis, necrotizing enterocolitis, administration of vancomycin, amikacin, amphotericin, cefotaxime and water restriction. The information was collected from the data of the clinical records and the analysis was carried out in the Epi-info software. The statistical analysis, it was performed using non-parametric tests such as Chi2 and Fisher's Exact Test.
 Results: Denote underweight with an OR 2, 95% CI. from 1.1 to 3.3 and <P 0.05 by Chi2 and Fisher's exact test. For; perinatal asphyxia with OR 2.1, 95% CI. from 1.3 to 3.4 and <P 0.05 through Chi2 and Fisher's exact test. For vancomycin administration OR 4.7, 95% CI. From 2.3 to 9.3 and <P 0.05 by Chi2 and Fisher's exact test. For water restriction OR 2, 95% CI. From 1.2 to 3.3 and <P 0.05 by Chi2 and Fisher's exact test.
 Conclusion: A significant association was found between acute renal failure with low weight, perinatal asphyxia, administration of vancomycin and water restriction.

Acute renal failure in newborns in the practice of a pediatrician of the future

Aims: The treatment of an acute renal failure has not received much research in Kazakhstan, whether focusing on adults or children. As a result, there is a lack of awareness about the condition of the field's knowledge as well as its challenges and prospects. Thus the goal is to identify and assess the studies that are currently accessible concerning the treatment of acute kidney failure in newborns or infants in the context of Kazakhstan experience. Study design: Literature review Methodology: MEDLINE, Cochrane Reviews, and Pedro were among the databases used to conduct the literature search. The conjunctions "AND" and "OR" were used with certain keywords. The articles' time range was restricted between 2018 and 2022 in order to only present the most recent facts. Papers discussing acute renal failure in infants only in Kazakhstan were included in the inclusion criteria. Results: Regarding the treatment of AKI in neonates in Kazakhstan, there are incredibly few available studies. The majority of trials involved individuals who were older. ESRD was more widely covered in conjunction with dialysis than AKI. Women are more prone to experience AKI turning into full-blown CKD. Conclusion: To learn more about the state of acute renal failure in neonates in Kazakhstan, more field study is required. The frequency and prevalence of placing new-borns on dialysis should be updated and made publicly accessible. It is important to emphasise the causes and risk factors of AKI in babies of Kazakh ancestry in order to comprehend current trends. Scientific Novelty: This is the first study that attempts to cover the status of pediatric acute renal failure management within Kazakhstan. It will serve as a starting point for prospective research on pediatric illnesses within the country, so as to understand where the health sector of Kazakhstan stands on the global stage.

Risk factors for acute neonatal renal failure

Background: Acute neonatal renal failure is a health problem. Its risk factors and its clinical and evolutionary profile remain unknown in the Malagasy context. The main objective of this study was to determine its risk factors.Methods: This was a 14 month, single-center, retrospective, case-control study (November 2018 to December 2019). The cases were represented by newborns hospitalized and presenting an hypercreatininaemia (value >133 µmol/l or according to the KDIGO criteria) and controls by neonates without an hypercreatininaemia. One case was matched to 2 controls.Results: We included 75 cases. The prevalence was 14.5%. The mean age was 5±6.78 days (p=0.006). The sex ratio was 1.27. The mean gestational age was 36.69±3.84 WA (p=0.66). The mean serum creatinine value was 180.32 µmol/l. Thirty-one newborns had died (41.3%). The risk factors were: sepsis (OR=9.37, p≤0.001, CI=3.03, 33.5), perinatal asphyxia SARNAT 2 (OR=4.52, p=0.007, CI=1.53, 13.8) and SARNAT 3 (OR=7.90, p=0.021, CI=1.56, 60.4), increased weight loss (OR=4.04, p=0.006, CI=1.51, 11.2) and respiratory distress (OR=2.76, p=0.005, CI=1.37, 5.77).Conclusions: The risk factors were consistent with the data in the literature. Better management of parturients and the newborn as well as monitoring of serum creatinine in hospitalised newborns are recommended.

Outcome of Acute Renal Failure in Neonates and Its Associating Factors

Objective:To identify various predisposing causes and immediate consequences in new-borns presenting with acute renal failure. Study design: The study design was case series. Place & duration:In the Pediatric department of Shifa International Hospital, Islamabad for one-year duration from September 2020 to August 2021. Material and Methods:A total of 1300 cases were assessed for acute renal failure (ARF)and 98 established cases of ARF being included in the research. The neonates (0-28 days old) who met the criteria for ARF, i.e., anuria/oliguria plus a serum creatinine level more than 1.5 mg/dl, were included in this research. The patient's relevant medical history, physical examination, and laboratory tests were recorded on a separate proforma after given informed permission for the procedure. Each patient's outcome was precisely documented. The statistical software for social sciences was used to examine the data (SPSS20.0). A p-value of less than < 0.05 was considered significant. Results:A total of 98 cases (7.5 percent) were studied, with 68 (69.4 percent) males and 30 (30.6 percent) females. The ratio of males to females was 1.6:1. According to the study results, the mean age was 9.85± 7.20 days and the mean weight was 2.54± 0.65 kg. The majority of the infants were delivered at a hospital (73.5 percent). The most prevalent ARF symptoms in our neonates were birth asphyxia (30.6%), sepsis (24.5%), respiratory distress syndrome (RDS) (11.2%), and dehydration (9.2%), bleeding in (4.1 percent) and meconium aspiration in 3(3.1 percent). CCF and neurogenic bladder were both present in two patients. In 4 individuals, obstructive uropathy was found, including 2 cases of urethral valve (PUV) blockage and 2 cases of pelvic ureteric junction obstruction (PUJO). Neonates with ARF had a 28.5 percent overall death rate (28 cases). Conclusion:When it comes to neonates in critical care, acute renal failure is a frequent concern to observe. More than one predisposing factor for acute renal failure was present in the majority of the patients. Preventing acute renal failure in infants may be accomplished by early identification of risk factors and prompt treatment of underlying diseases. Keywords:Renal failure, Sepsis, Birth asphyxia, Neonates

Perinatal asphyxia and acute renal failure in neonates: presentation and outcomes

Background: Perinatal asphyxia (PNA) is described by the world health organization as a "failure to begin and sustain breathing at delivery." Acute renal failure (ARF), a well-known consequence of birth asphyxia, has a poor immediate prognosis and can leave survivors with lasting renal impairment. The goal of this study was to find out how often ARF is in cases of PNA and how it relates to the severity of the Apgar score and the grading of hypoxic ischemic encephalopathy.Methods: This study was based on APGAR scores and a renal function evaluation done on day 3 of life on neonates with a gestational age of >34 weeks and signs of birth asphyxia.Results: According to the APGAR score, 46 percent of the 75 infants investigated had mild PNA, whereas 42% and 12% of the babies had moderate and severe PNA. ARF was discovered in 24% of PNA patients, with oliguric renal failure (ORF) in 42% and non-ORF (NORF) in 58%. Blood urea was elevated in all instances with ARF (100%) but only 8% of non-ARF patients. ARF was found in 100% of individuals with severe PNA, but only 28% of patients with mild PNA. ARF was not found in any of the individuals with mild PNA. Only 3 (4%) of asphyxiated babies had abnormal renal USG results.Conclusions: Our findings revealed a substantial link between PNA severity and ARF, with no ARF in mild PNA. The incidence of ARF and the stage of HIE were shown to have a linear association in this study. Only ORF instances resulted in death.

To determinate the degree or severity of renal impairment according to HIE stages in PNA patients

Introduction: Perinatal asphyxia is a major cause of acute renal failure in neonates. So it is essential to evaluate the renal function at an early stage to stabilize fluid and electrolyte balance which is of great importance to infants with hypoxic-ischemic encephalopathy. Yet, it is not quite easy to evaluate neonatal renal function accurately. Objective: To Determinate the Degree or Severity of Renal Impairment According To HIE Stages in PNA Patients. Materials and Methods: This cross-sectional study was carried out in the department of neonatology, Chattagram Ma-O-Shishu hospital from January 2016 to June 2016. All Perinatal asphyxia patients admitted in the department of neonatology, Chattagram Maa-Shishu O General Hospital fulfilling the inclusion criteria were included in the study. As the study was done in a single center and due to time constrain 180 cases were enrolled in this study. After taking informed written consent from legal attended a total of 150 asphyxiated and 30 non-asphyxiated newborns were selected by purposive sampling. Results: During the study period, there were total 197 eligible infants. Among them 154 were case and 43 were control. Among the cases 2 infants left against medical advice (LAMA), 1 infant died, and 1 was excluded due to lack of parental consent. Among the control 13 were excluded due to lack of consent. Finally 150 asphyxiated newborn were selected as case and 30 normal infants were included as control in the study. In this study, stage I, 50(60.2%) baby has normal RFI and 33 (39.8%) babies has abnormally high RFI. For stage II, 23 (43.4%) baby has normal RFI and 30 (56.6%) baby has abnormally high RFI and for stage III, only 5 (35.7%) baby has normal RFI and 9 (64.3%) baby has abnormally high RFI. Their P-value is 0.070, which is statistically not significant. The study also shows Mean ± SD of RFI value in stage I is 2.76 ± 0.62, for Stage II is 3.17 ± 0.67, and for stage III, is 3.17 ± 0.28 and “p” value 0.001 which is statistically highly significant. Conclusion: In conclusion, AKI represents a significant problem among asphyxiated neonates. All of the hyperechogenic kidney patients were also hyperuricemic, which supports the possible association between both features. In addition, uric acid itself might be the causative factor for failure in addition to hypoxic and ischemic insult. We recommend kidney functions, and abdominal ultrasonography to be done routinely in asphyxiated neonates to evaluate the possibility of acute kidney injury in them. FENa and RFI are useful parameters for assessing renal function and urinary β2M is a good biomarker for diagnosis and prognosis of acute tubular injury in term babies with perinatal asphyxia.

To Determinate the Degree or Severity of Renal Impairment According to HIE Stages in PNA Patients

Introduction: Perinatal asphyxia is a major cause of acute renal failure in neonates. So it is essential to evaluate renal function at an early stage to stabilize fluid and electrolyte balance which is of great importance to infants with hypoxic-ischemic encephalopathy. Yet, it is not quite easy to evaluate neonatal renal function accurately. Objective: To Determinate the Degree or Severity of Renal Impairment According to HIE Stages in PNA Patients. Materials and Methods: This cross sectional study was carried out in the department of neonatology, Chattagram Ma-O-Shishu hospital from January 2016 to June 2016. All Perinatal asphyxia patients admitted in the department of neonatology, Chattagram Maa-Shishu O General Hospital fulfilling the inclusion criteria were included in the study. As the study was done in a single center and due to time constrain 180 cases were enrolled in this study. After taking informed written consent from legal attended a total of 150 asphyxiated and 30 non-asphyxiated newborns were selected by purposive sampling. Results: During the study period, there were total 197 eligible infants. Among them 154 were case and 43 were control. Among the cases 2 infants left against medical advice (LAMA), 1 infant died, and 1 was excluded due to lack of parental consent. Among the control 13 were excluded due to lack of consent. Finally, 150 asphyxiated newborns were selected as case and 30 normal infants were included as control in the study. In this study, stage I, 50(60.2%) baby has normal RFI and 33 (39.8%) babies have abnormally high RFI. For stage II, 23 (43.4%) baby has normal RFI and 30 (56.6%) baby has abnormally high RFI and for stage III, only 5 (35.7%) baby has normal RFI and 9 (64.3%) baby has abnormally high RFI. There P value is 0.070, which is statistically not significant. The study also shows Mean ± SD of RFI value in stage I is 2.76 ± 0.62, for Stage II is 3.17 ± 0.67 and for stage III, is 3.17 ± 0.28 and “p” value 0.001 which is statistically highly significant. Conclusion: In conclusion, AKI represent a significant problem among asphyxiated neonates. All of the hyperechogenic kidney patients were also hyperuricemic, which supports the possible association between both features. In addition, uric acid itself might be the causative factor for failure in addition to hypoxic and ischemic insult. We recommend kidney functions, and abdominal ultrasonography to be done routinely in asphyxiated neonates to evaluate the possibility of acute kidney injury in them. FENa and RFI are useful parameters for assessing the renal function and urinary β2M is a good biomarker for diagnosis and prognosis of acute tubular injury in term babies with perinatal asphyxia.

To study renal parameters and serum calcium levels in birth asphyxia

Background: Birth asphyxia is a common neonatal problem and contributes significantly to neonatal mortality and long term morbidity. Any organ can be affected but the brain, heart, kidneys are more sensitive to hypoxic injury. Aim and Objective: To study renal parameters and serum calcium levels in birth asphyxia, to determine the incidence of renal failure in asphyxiated newborns, and to correlate the renal parameters with different severity of birth asphyxia and with different stages of hypoxic-ischemic encephalopathy. Method and material: A comparative study of 69 term newborns, appropriate for gestational age born/admitted to tertiary care Centre RDGMC Ujjain with birth asphyxia and 69 normal newborns without birth asphyxia was done. Perinatal history, physical examinations, New Ballard score, Apgar score was done. Data collected and entered in the master chart for results and analysis. Result: The study included 69 cases and 69 controls with the majority of boys. Mild, moderate, and severe birth asphyxia was7%, 29%, and 64% of all cases respectively. The incidence of acute renal failure was 57% among cases of birth asphyxia. The incidence of acute renal failure was 53% and 50% in hypoxic-ischemic encephalopathy stages III and II respectively. Incidence of renal failure with mild, moderate, and severe asphyxia was 40%, 50%, and 39% respectively Conclusion: Perinatal asphyxia is an important cause of neonatal renal failure. Monitoring of urea, creatinine and urine output helps in the early diagnosis and management of acute renal failure

Correlation of renal function tests with early onset neonatal sepsis.

Early onset neonatal sepsis is invariably very common and serious problem worldwide, especially it is one of the important etiological factor for deaths of neonates in Pakistan. Acute renal failure is frequently seen in neonates with sepsis. Objectives: The aim of present study was to determine the correlation of renal function tests (Blood Urea and creatinine) with early onset neonatal sepsis. Study Design: Descriptive study. Setting: Department of Paediatric Medicine Sir Ganga Ram hospital, Lahore. Period: Six months from 20th March to 20th September 2018. Material & Methods: Total 300 cases of neonatal sepsis with acute renal failure were included in this study after taking informed consent from the parents. Cases selection was done with help of a predefined inclusion and exclusion criteria. Daily blood urea and serum creatinine were calculated from birth to first 7 days of life. If any of blood urea or serum creatinine was deranged, the neonate was labelled as having acute renal failure. Data was entered and analysed using SPSS software version 21. Results: Mean age of all cases was 2.82±1.56 days. The minimum and maximum age limits of the neonates were 1 and 7 days respectively. Gender distribution of neonate showed that 57% of the neonates were male and 43% were females. At the 2nd day of life, mean serum creatinine level was 1.12±0.39, at 3rd day 1.19±0.51, at 4th day 1.41±0.38, at 5th day 1.33±0.39, at 6th day 1.19±0.39, and at 7th day mean serum creatinine level was 1.09±0.31 respectively. At 2nd day of birth mean blood urea was 54.82±34.77, at 3rd day59.50±28.22 at 4th day 74.94±30.37, at 5th day 67.09±26.94, at 6th day 56.09±25.76, at 7th day mean blood urea level was 47.66±22.47 respectively. Frequency of acute renal failure was observed in 28.3% of the neonates while the remaining 71.7% of neonates did not suffer from acute renal failure. Conclusion: Early onset neonatal sepsis contributes significantly to development of acute renal failure in neonates.

Risk estimation of fetal adverse effects after short-term second trimester exposure to non-steroidal anti-inflammatory drugs: a literature review.

Non-steroidal anti-inflammatory drugs (NSAIDs) are not recommended in the 3rd trimester of pregnancy due to known fetal adverse effects in an advanced gestational age. This investigation was performed to assess whether there is a significant risk of NSAIDs being used as an analgesic or antipyretic medication in the 2nd trimester. A systematic search for publications reporting 2nd trimester NSAID exposure was performed in MEDLINE. The search focused on case descriptions reporting defined adverse effects including prenatal ductus arteriosus constriction, oligohydramnios, neonatal renal failure, and primary pulmonary hypertension. Original articles published until February 2018 were considered for evaluation. Out of 681 identified publications, 26 included relevant information on the defined adverse effects. Among these publications, premature labor was the major reason for 2nd trimester indomethacin treatment while other clinical indications and other NSAIDs were underrepresented. Narrowing or closure of the ductus arteriosus in the 2nd trimester was described in 33 fetuses. Only eight publications reported adverse effects after less than 7-day exposure during the 2nd trimester. Based on these results, short-term use of NSAIDs as analgesics or antipyretics in the 2nd trimester does not appear to pose a substantial risk for fetal adverse effects. Long-term use in the late 2nd trimester, however, should always be monitored.

Neonatal renal replacement therapy: An ethical reflection for a crucial decision

ContextTechnological advances in fetal and neonatal medicine, recent changes in the French legal framework, and encouraging results of the long-term outcomes in children with neonatal renal failure provide elements for an ethical reflection. MethodsWe led a nationwide enquiry among French pediatric nephrologists, intensivists, and neonatologists, exploring the decision-making process when contemplating starting renal replacement therapy (RRT) or delivering palliative care to neonates or infants with pre-end-stage or end-stage renal disease; and the ethical quandaries at hand in such scenarios. ResultsA total of 134 responses with complete national coverage were obtained. Care to be delivered to an infant in pre-end-stage or end-stage renal disease did not achieve consensus. Pediatric nephrologists were more prone to initiate a dialysis/graft program than pediatric intensivists. When chronic kidney disease was associated with comorbidities, especially neurological impairment, physicians, regardless of their subspecialty, were more reluctant to initiate conservative treatment. Many of the doctors surveyed did not give their opinion in these prenatal and/or postnatal situations, considered to be unique and warranting a multidisciplinary reflection. ConclusionSuch ethical dilemmas are challenging for parents and physicians. They can only be overcome by taking into account both concrete on the ground realities and general principles and values acknowledged to be a basis for respecting the individual. In this way, it ensures humaneness and humanization of a practice that must meet a variety of challenges, one by one. The answer is not simple; it is always unique to each child and can only be approached by a multidisciplinary, time-consuming, open discussion, which will never totally erase uncertainty.

Predictive Factors for Acute Renal Failure in Neonates with Septicemia

Objectives: Septicemia is an important cause of acute kidney injury (AKI) in neonates admitted to neonatal intensive care units. The aim of this study was to identify the predictive factors for AKI in neonates with culture-proven septicemia. Methods: Medical records of 138 newborns with septicemia were evaluated in 2 groups with (n, 65) and without (n, 73) AKI. Results: The mean age at diagnosis of AKI was 11.31 ± 9.92 days. AKI was more common in neonates with lower gestational age, birth weight, age, blood pressure, platelet count, and serum pH at admission. Moreover, AKI was associated with an increased incidence of intubation at birth, inotrope treatment, shock, disseminated intravascular coagulation (DIC), mechanical ventilation, and morality. Gestational age, initial serum pH, and DIC were the only independent predictors of AKI in the multivariate analysis. In addition, serum pH at admission was the most accurate laboratory marker for the prediction of AKI with 83% sensitivity and 60% specificity. Conclusions: Monitoring of renal function is highly recommended in low-birth-weight infants with severe septicemia.

Incidence of acute renal failure in birth asphyxia and its correlation with hypoxic-ischemic encephalopathy staging

Introduction: Acute renal failure (ARF) is a recognized complication of birth asphyxia and it carries a poor prognosis. Early recognition of ARF is particularly important in asphyxiated neonates with hypoxic-ischemic encephalopathy (HIE). Objectives: To determine the incidence of ARF in term asphyxiated neonates and to correlate it with HIE staging. Methods: A total of 50 term (37-42 weeks) neonates born with Apgar score of <7 at 5 min after birth were selected as cases and 50 normal term (37-42 weeks) neonates as controls. All asphyxiated neonates with clinical features of HIE are staged by Sarnat and Sarnat staging. Gestational age, birth weight, relevant perinatal history, and examination findings are recorded in predesigned pro forma. After 72 h and before 96 h of life, blood was collected and sent for relevant investigations, and clinical condition of the neonate and urine output were monitored and neonates were managed according to the standard protocol. Results: Incidence of ARF is significantly more in cases (76.0% vs. 4.0%) than the controls. Among 38 cases with ARF, 32 (84.2%) had pre-renal ARF and 6 (15.8%) had intrinsic ARF. On the basis of urine output, 12 (24.0%) had oliguric ARF and 38 cases (76.0%) had non-oliguric ARF while out of 50 controls, 2 (4%) neonates had pre-renal and non-oliguric type of ARF. Incidence of ARF correlated well with HIE staging. Among the 50 cases, 32 (84.3%) improved clinically after fluid therapy, while 6 (15.7%) did not improve. Conclusion: Perinatal asphyxia is an important cause of neonatal renal failure. ARF in birth asphyxia is predominantly pre-renal and non-oliguric type and it responds to fluid challenge. ARF in birth asphyxia correlates well with HIE staging.

Case report on neonatal renal failure due to prenatal exposure of Telmisartan

Telmisartan is an antihypertensive medication, which acts as an angiotensin II receptor blocker, and has an inhibitory effect on the renin-angiotensin system. Angiotensin receptor blockers(ARBs) are contraindicated in pregnancy (category D); adverse effects have been reported in both animal and human pregnancies, but still pregnant mothers do come with this medication with consequent neonatal adverse complications. We report 2 such cases of adverse neonatal outcomes in hypertensive mothers exposed to Telmisartan during pregnancy.

Clinical profile and outcome of acute kidney injury in neonatal sepsis in a tertiary care centre

Background: Septicemia remains a leading cause of morbidity and mortality among neonates with AKI complicating as many as 3.4 to 24% of them. The actual incidence of renal failure in all sepsis cases is not documented. There are several studies in the literature on renal failure in neonates which were based on older definitions using blood urea and urine output as parameters. The major limitation of the older studies was that they did not use the current acute kidney injury network (AKIN) definition. In the present study, we have attempted to investigate AKI in neonatal sepsis using the AKIN definition based on serum creatinine (Table1). We also attempted to determine the predictors of AKI in neonatal sepsis.Methods: The present study was an explorative study conducted in the neonatal unit of Cheluvamba hospital attached to Mysore Medical College and Research Institute in which a total of 50 neonates with clinical/ culture positive sepsis were enrolled during the period of June 2014 to June 2015.Results: These neonates were investigated for the presence of AKI based on serum creatinine values of three consecutive days and were divided into two groups. Group 1 consisted of septic neonates with AKI and group 2 consisted of septic neonates without AKI. Both the groups were followed up till discharge or death. The risk factors associated with sepsis were compared in both the groups and predictors of morbidity and mortality associated with AKI in sepsis were also determined.Conclusions: Incidence and mortality associated with AKI in sepsis was found to be 24% and 75% respectively. Factors like PROM, foul smelling liquor, > 3 unclean vaginal examinations during labour were significant risk factors for development of AKI in sepsis. Culture positivity, associated meningitis, DIC, shock and need for assisted ventilation were poor prognostic indicators and were significantly associated with mortality.

Continuous renal replacement therapy of acute renal failure in neonate

Acute renal failure (ARF) is the common critical disease caused by various factors in neonates, its clinical manifestation is the sharp decline in renal function, in a short period of time, the serum creatinine is increased and urine volume decreases.However, recently, with the progress of blood purification technology, continuous renal replacement therapy (CRRT) has been used from ARF in elderly children to the newborn.In this paper, the risk factors of neonatal ARF, diagnostic criteria, the relevant questions of CRRT treatment were reviewed. Key words: Renal failure; Infant, newborn; Continuous renal replacement therapy

Fetal and Neonatal Renal Failure and Renal Transplant

Major advances have been made over the past few decades in caring for neonates with chronic renal disease. Although dialysis registries report infants as a high-risk population for poor outcomes, these children can be effectively maintained on dialysis until they are ready for receiving a transplant. Early identification of the presence of chronic renal disease is helpful in preparing for postnatal complications and to counsel parents on potential outcomes of infants with chronic kidney disease.

Transient neonatal renal failure and massive polyuria in MEGDEL syndrome

BackgroundMEGDEL (3-methylglutaconic aciduria with deafness, encephalopathy, and Leigh-like syndrome) syndrome is a mitochondrial disorder associated with recessive mutations in SERAC1. ObjectivesTo report transient neonatal renal findings in MEGDEL syndrome. ResultsThis 7year-old girl was the first child of consanguineous Turkish parents. She exhibited an acute neonatal deterioration with severe lactic acidosis and liver failure. Initial evaluation revealed massive polyuria and renal failure with 3-methylglutaconic aciduria. Symptoms and biological findings progressively improved with symptomatic treatment but lactic acidosis and high lactate to pyruvate ratio along with 3-methylglutaconic aciduria persisted. At 8months of age, a subacute neurological degradation occurred with severe hypotonia, dystonia with extrapyramidal movements and failure to thrive. Brain MRI revealed basal ganglia lesions suggestive of Leigh syndrome. At 3years of age, sensorineural deafness was documented. MEGDEL syndrome was further confirmed by the identification of an already reported homozygous mutation in SERAC1. ConclusionTransient neonatal polyuria and renal failure have not been reported to date in SERAC1 defective patients. Such neonatal kidney findings expand the clinical spectrum of MEGDEL syndrome.

Acute Renal Failure in Neonates with Perinatal Asphyxia and its Correlation with HIE Staging: A Prospective Case Control Study

Acute Renal Failure in Neonates with Perinatal Asphyxia and its Correlation with HIE Staging: A Prospective Case Control Study