Congenital nephrotic syndrome: is early aggressive treatment needed? Yes

Hölttä, Tuula; Jalanko, Hannu

doi:10.1007/s00467-020-04578-4

Cited by 30 publications

(27 citation statements)

References 43 publications

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“…We have previously shown in a long-term follow-up study, that in patients with CNF, which accounts majority of the KTx recipients in Finland, the incidence of CVD is relatively low. 30 As in earlier studies, the distributions of causes of death changed according to the time after transplantation in our study. Rejection and infection play major part in post-transplant mortality <1 year after Tx.…”

Section: Discussionsupporting

confidence: 76%

Long‐term mortality in pediatric solid organ recipients—A nationwide study

Salonen

Jahnukainen

Nikkilä

et al. 2023

Pediatric Transplantation

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Background: The present study aimed at investigating long-term mortality of patients who underwent solid organ transplantation during childhood and at identifying their causes of death. Methods:A cohort of 233 pediatric solid organ transplant recipients who had a kidney, liver, or heart transplantation between 1982 and 2015 in Finland were studied.Year of birth-, sex-, and hometown-matched controls (n = 1157) were identified using the Population Register Center registry. The Causes of Death Registry was utilized to identify the causes of death.Results: Among the transplant recipients, there were 60 (25.8%) deaths (median follow-up 18.0 years, interquartile range of 11.0-23.0 years). Transplant recipients' risk of death was nearly 130-fold higher than that of the controls (95% CI 51.9-1784.6).The 20-year survival rates for kidney, liver, and heart recipients were 86.1% (95% CI 79.9%-92.3%), 58.5% (95% CI 46.2%-74.1%), and 61.4% (95% CI 48.1%-78.4%), respectively. The most common causes of death were cardiovascular diseases (23%), infections (22%), and malignancies (17%). There were no significant differences in survival based on sex or transplantation era. Conclusion:The late mortality is still significantly higher among pediatric solid organ recipients in comparison with controls. Cardiovascular complications, infections, and cancers are the main causes of late mortality for all studied transplant groups. These findings emphasize the cruciality of careful monitoring of pediatric transplant recipients in order to reduce long-term mortality.

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Section: Discussionsupporting

confidence: 76%

Long‐term mortality in pediatric solid organ recipients—A nationwide study

Salonen

Jahnukainen

Nikkilä

et al. 2023

Pediatric Transplantation

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“…CNS has a genetic basis in most of the cases, but secondary CNS has been described with toxins (i.e., mercury exposure); maternal systemic lupus erythematosus; and several perinatal infections such as CMV, syphilis, rubella, human immunodeficiency virus, hepatitis B virus, and toxoplasmosis [55,56]. A proper differential diagnosis between the genetic and secondary forms of CNS is critical, since the former do not respond to therapy and require kidney transplantation, while specific therapy in secondary causes of CNS may be curative in some patients [57].…”

Section: Kidney Involvement At the Clinical Levelmentioning

confidence: 99%

Renal Involvement in Congenital Cytomegalovirus Infection: A Systematic Review

Ríos-Barnés¹,

Fortuny

Alarcón

et al. 2021

Microorganisms

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Background: Congenital cytomegalovirus (cCMV) infection is the most frequent mother-to-child transmitted infection worldwide and a prevalent cause of neonatal disease and long-term morbidity. The kidney is a target organ for CMV, which replicates in renal tubules and is excreted in large quantities in urine for years in children with cCMV infection. Nonetheless, kidney disease has rarely been reported in cCMV-infected patients. Objective: We aimed to describe the available data on renal involvement in patients with cCMV infection at the pathologic, functional, anatomical, and/or clinical levels. Methods: A systematic search was performed in the MEDLINE/PubMed, SCOPUS, and Cochrane databases. Studies describing any renal involvement in fetuses or neonates aged ≤3 weeks at diagnosis of microbiologically confirmed cCMV infection were eligible. Results: Twenty-four articles were included, with a very low level of evidence. Pathologic findings in autopsy studies universally described CMV typical inclusion bodies in tubular cells. No functional studies were identified. cCMV infection was not associated with an increased risk of kidney malformations. Congenital nephrotic syndrome was the most common clinical condition associated with cCMV, but a causal relationship cannot be established. Conclusions: Typical pathological features of cCMV infection are very common in renal tissue, but they do not seem to entail significant consequences at the anatomical or clinical levels.

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“…Early treatment of daily albumin infusions, nutrition, and timely bilateral nephrectomy followed by transplantation at the age of 1-2 years showed dramatic improvement in neurodevelopmental skills. 19…”

Section: Discussionmentioning

confidence: 99%

Evaluation of Clinical and Follow-up Results of Patients with Congenital Nephrotic Syndrome

2021

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Congenital nephrotic syndrome (CNS) is characterized by severe proteinuria, hypoalbuminemia, and edema within the first three months of life. Congenital nephrotic syndrome can occur due to perinatal infections or mutation of genes encoding structural or regulatory proteins of the glomerular filtration barrier. Treatment includes albumin infusions, nephrectomy, dialysis, and transplantation. In this study, we aimed to evaluate the demographic, clinical, and follow-up results of patients with CNS followed up in our center between 2010 and 2020. Demographic, clinical, laboratory values of 8 patients diagnosed with CNS between 2010 and 2020, kidney biopsy results, genetic examinations, and follow-up results were retrospectively evaluated. A total of 8 patients (4 girls) were included in this study. The median age at diagnosis was 36 days (3 days-8 months) and the follow-up period was 34 months (7-114 months). There was a history of prematurity and consanguinity in 5 patients. Edema was detected at the admission of all patients. Albumin infusion and captopril therapy were started from the diagnosis. No pathology was seen in the tests for perinatal infection, and ultrasonographic examinations were normal. In the genetic analysis, NPHS1 (Nephrin) homozygous mutation was detected in six patients, and coenzyme Q2 mutation was detected in one patient. Peritoneal dialysis treatment was performed in four patients during the follow-up, and unilateral nephrectomy was completed in one patient. During the follow-up, four of eight patients (three due to sepsis while on dialysis, one on the postoperative after the first day of transplantation) died. Three patients are followed up with kidney transplantation and one with supportive treatment. According to our results, most CNS cases are genetic, and nephrin mutation is the most common cause. Management of complications in CNS is crucial for patient surviva

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Congenital nephrotic syndrome: is early aggressive treatment needed? Yes

Cited by 30 publications

References 43 publications

Long‐term mortality in pediatric solid organ recipients—A nationwide study

Long‐term mortality in pediatric solid organ recipients—A nationwide study

Renal Involvement in Congenital Cytomegalovirus Infection: A Systematic Review

Evaluation of Clinical and Follow-up Results of Patients with Congenital Nephrotic Syndrome

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