MO1021HYPERTENSION IN CHILDHOOD MINIMAL CHANGE DISEASE: IGNORED, MISSED OR UNDER-DIAGNOSED?

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Sourabh Sharma ◽  
Neha Sharma
Keyword(s):  
Body Mass Index ◽  
Nephrotic Syndrome ◽  
Family History ◽  
Body Mass ◽  
Minimal Change Disease ◽  
Angiotensin Receptor Blockers ◽  
Tertiary Care ◽  
Minimal Change ◽  
Primary Nephrotic Syndrome ◽  
Steroid Dependent

Abstract Background and Aims Minimal change disease (MCD) is most common form of primary nephrotic syndrome (76.6% as per ISKDC) in children aged 1 to 10 years. As per current literature, blood pressure (BP) is usually normal in childhood MCD while in adults, 50% MCD cases are hypertensive. We hypothesized that hypertension is prevalent in childhood MCD also and most of cases are missed or under-diagnosed due to lack of BP monitoring, inappropriate BP cuff size (larger cuff will inaccurately show lower BP; cuff should cover 75% to 80% of arm circumference between antecubital fossa and axilla) or lack of knowledge about childhood hypertension criteria (systolic or diastolic BP> 95th percentile for age on ≥2 clinical visits at least 1 week apart). Method This cross-sectional study was performed from March 2018 to December 2020 at a tertiary care centre in India. Newly diagnosed and relapses of primary nephrotic syndrome (presumed MCD) in children aged 1 to 10 years were included in the study. All cases were subjected to careful BP monitoring and charting using appropriate BP cuff, while child is calm and relaxed, in quiet room with comfortable temperature and empty bladder. Three measurements were taken at 1 minute interals and taking average of last 2 measurements. Percentile charts for age and height were followed. Results fifty four cases of childhood MCD were studied. Thirty six cases (66.67%) were male. Mean age was 5.48 years (SD ±2.31). Thirty two cases (59.26%) were found to be hypertensive. Fourteen cases (43.75%) were relapses. Seven cases (21.87%) were steroid resistant nephrotic syndrome while three cases were steroid dependent. Five cases were already on calcineurin inhibitors when diagnosed of hypertension. There was family history of hypertension in 9 cases diagnosed of hypertension (p value 0.77). There was no correlation between occurrence of hypertension and degree of proteinuria, hypoalbuminemia or body mass index. All hypertensives were managed with angiotensin receptor blockers and regularly followed-up. Conclusion Results of our study are contrary to traditional belief that hypertension is not common in childhood MCD. Significant number of cases are hypertensive. Correct BP measurement and interpretation is necessary in each childhood MCD case. Its occurrence is not correlated with family history or degree of proteinuria, hypoalbuminemia or body mass index.

Discovery of Autoantibodies Targeting Nephrin in Minimal Change Disease Supports a Novel Autoimmune Etiology

2021 ◽  
pp. ASN.2021060794
Author(s):  
Andrew Watts ◽  
Keith Keller ◽  
Gabriel Lerner ◽  
Ivy Rosales ◽  
A. Collins ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Animal Studies ◽  
Minimal Change Disease ◽  
Molecular Classification ◽  
Minimal Change ◽  
Filtration Barrier ◽  
Steroid Dependent ◽  
Renal Biopsies ◽  
End Stage ◽  
Autoimmune Etiology

Background Failure of the glomerular filtration barrier, primarily by loss of slit diaphragm architecture, underlies nephrotic syndrome in minimal change disease. The etiology remains unknown. The efficacy of B cell-targeted therapies in some patients, together with the known proteinuric effect of antinephrin antibodies in rodent models, prompted us to hypothesize that nephrin autoantibodies may be present in patients with minimal change disease. Methods We evaluated sera from patients with minimal change disease enrolled in the Nephrotic Syndrome Study Network (NEPTUNE) cohort and from our own institutions for circulating nephrin autoantibodies by indirect ELISA and by immunoprecipitation of full-length nephrin from human glomerular extract or a recombinant purified extracellular domain of human nephrin. We also evaluated renal biopsies from our institutions for podocyte-associated punctate IgG colocalizing with nephrin by immunofluorescence Results In two independent patient cohorts, we identified in a subset of patients with minimal change disease circulating nephrin autoantibodies during active disease that were significantly reduced or absent during treatment response. We correlated the presence of these autoantibodies with podocyte-associated punctate IgG in renal biopsies from our institutions. We also identified a patient with steroid-dependent childhood minimal change disease that progressed to end-stage kidney disease; she developed a massive posttransplant recurrence of proteinuria that was associated with high pretransplant circulating nephrin autoantibodies. Conclusions Our discovery of nephrin autoantibodies in a subset of adults and children with minimal change disease aligns with published animal studies and provides further support for an autoimmune etiology. We propose a new molecular classification of nephrin autoantibody minimal change disease to serve as framework for instigation of precision therapeutics for these patients.

scholarly journals Primary Nephrotic Syndrome and Risks of End-Stage Kidney Disease, Cardiovascular Events, and Death: The Kaiser Permanente Nephrotic Syndrome Study

2021 ◽  
pp. ASN.2020111583
Author(s):  
Alan Go ◽  
Thida Tan ◽  
Glenn Chertow ◽  
Juan Ordonez ◽  
Dongjie Fan ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Kidney Disease ◽  
Membranous Nephropathy ◽  
Minimal Change Disease ◽  
Cardiovascular Outcomes ◽  
Minimal Change ◽  
End Stage Kidney Disease ◽  
Kaiser Permanente ◽  
Primary Nephrotic Syndrome ◽  
End Stage

Background Few population-based data exist about adults with primary nephrotic syndrome. Methods To evaluate kidney, cardiovascular, and mortality outcomes in adults with primary nephrotic syndrome, we identified adults within an integrated health care delivery system (Kaiser Permanente Northern California) with nephrotic-range proteinuria or diagnosed nephrotic syndrome between 1996-2012. Nephrologists reviewed medical records for clinical presentation, laboratory findings, and biopsy results to confirm primary nephrotic syndrome and assigned etiology. We identified a 1:100 time-matched cohort of adults without diabetes, diagnosed nephrotic syndrome, or proteinuria as controls to compare rates of end-stage kidney disease (ESKD), cardiovascular outcomes, and death through 2014, using multivariable Cox regression. Results We confirmed 907 cases of primary nephrotic syndrome (655 definite and 252 presumed cases of focal segmental glomerulosclerosis [FSGS, 40%], membranous nephropathy [40%], and minimal change disease [20%]). Mean age was 49 years; 43% were women. Adults with primary nephrotic syndrome had higher adjusted rates of ESKD (adjusted hazard ratio [aHR], 19.63; 95% confidence interval [95% CI], 12.76 to 30.20), acute coronary syndrome (aHR 2.58; 95% CI, 1.89 to 3.52), heart failure (aHR 3.01; 95%CI, 2.16 to 4.19), ischemic stroke (aHR 1.80; 95%CI,1.06 to 3.05), venous thromboembolism (aHR 2.56; 95%CI, 1.35 to 4.85), and death (aHR 1.34; 95%CI, 1.09 to 1.64) versus controls. Excess ESKD risk was significantly higher for FSGS and membranous nephropathy than for presumed minimal change disease. The three etiologies of primary nephrotic syndrome did not differ significantly in terms of cardiovascular outcomes and death. Conclusions Adults with primary nephrotic syndrome experience higher adjusted rates of ESKD, cardiovascular outcomes, and death, with significant variation by underlying etiology in risk for developing ESKD.

The paucity of minimal change disease in adolescents with primary nephrotic syndrome

1998 ◽  
Vol 12 (2) ◽  
pp. 105-107 ◽  
Author(s):  
Noosha Baqi ◽  
Anup Singh ◽  
Shivaiha Balachandra ◽  
Hadi Ahmad ◽  
Anthony Nicastri ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Minimal Change Disease ◽  
Minimal Change ◽  
Primary Nephrotic Syndrome

A case of minimal change disease during pregnancy – Benefits of early diagnosis and use of corticosteroids

2021 ◽  
pp. 1753495X2199021
Author(s):  
Priyanka S Sagar ◽  
Eddy Fischer ◽  
Muralikrishna Gangadharan Komala ◽  
Bhadran Bose
Keyword(s):  
Nephrotic Syndrome ◽  
Early Diagnosis ◽  
Renal Biopsy ◽  
Early Pregnancy ◽  
Minimal Change Disease ◽  
Minimal Change ◽  
Risk Of Bleeding ◽  
Increased Risk ◽  
Prompt Diagnosis ◽  
In Pregnancy

Nephrotic syndrome presenting in pregnancy is rare and poses a diagnostic and therapeutic challenge. Timing of renal biopsy is important given the increased risk of bleeding and miscarriage, and the choice of immunosuppression is limited due to the teratogenicity profiles of standard drugs. We report and discuss a case of minimal change disease diagnosed by renal biopsy during early pregnancy and treated with corticosteroids throughout the pregnancy. Prompt diagnosis and treatment of glomerular disease in pregnancy are vital to prevent poor maternal and fetal outcomes.

scholarly journals Clinical manifestations of focal segmental glomerulosclerosis in Japan from the Japan Renal Biopsy Registry: age stratification and comparison with minimal change disease

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Takaya Ozeki ◽  
Shoichi Maruyama ◽  
Toshiyuki Imasawa ◽  
Takehiko Kawaguchi ◽  
Hiroshi Kitamura ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Renal Biopsy ◽  
Focal Segmental Glomerulosclerosis ◽  
Clinical Diagnosis ◽  
Clinical Characteristics ◽  
Minimal Change Disease ◽  
Multivariable Analysis ◽  
Laboratory Data ◽  
Minimal Change ◽  
Segmental Glomerulosclerosis

AbstractFocal segmental glomerulosclerosis (FSGS) is a serious condition leading to kidney failure. We aimed to investigate the clinical characteristics of FSGS and its differences compared with minimal change disease (MCD) using cross-sectional data from the Japan Renal Biopsy Registry. In Analysis 1, primary FSGS (n = 996) were stratified by age into three groups: pediatric (< 18 years), adult (18–64 years), and elderly (≥ 65 years), and clinical characteristics were compared. Clinical diagnosis of nephrotic syndrome (NS) was given to 73.5% (97/132) of the pediatric, 41.2% (256/622) of the adult, and 65.7% (159/242) of the elderly group. In Analysis 2, primary FSGS (n = 306) and MCD (n = 1303) whose clinical diagnosis was nephrotic syndrome (NS) and laboratory data were consistent with NS, were enrolled. Logistic regression analysis was conducted to elucidate the variables which can distinguish FSGS from MCD. On multivariable analysis, higher systolic blood pressure, higher serum albumin, lower eGFR, and presence of hematuria associated with FSGS. In Japanese nationwide registry, primary FSGS patients aged 18–64 years showed lower rate of NS than those in other ages. Among primary nephrotic cases, FSGS showed distinct clinical features from MCD.

scholarly journals An investigation of the diet, exercise, sleep, BMI, and health outcomes of autistic adults

2021 ◽  
Vol 12 (1) ◽  
Author(s):  
Elizabeth Weir ◽  
Carrie Allison ◽  
Ken K. Ong ◽  
Simon Baron-Cohen
Keyword(s):  
Body Mass Index ◽  
Family History ◽  
Health Outcomes ◽  
Body Mass ◽  
Autism Spectrum ◽  
Lifestyle Factors ◽  
Sleep Patterns ◽  
Seizure Disorders ◽  
Severe Intellectual Disability ◽  
Autistic Adults

Abstract Background Studies of autistic children suggest that restricted eating, reduced physical activity, and sleep disorders are common; however, no studies attempt to broadly describe the diet, exercise, and sleep patterns of autistic adults or consider relationships between lifestyle behaviors and the widely reported increased risks of obesity and chronic conditions. To address this, the authors developed the largest study of lifestyle patterns of autistic adults and assessed their relationships to body mass index, health outcomes, and family history. Methods We administered an anonymized, online survey to n = 2386 adults (n = 1183 autistic) aged 16–90 years of age. We employed Fisher’s exact tests and binomial logistic regression to describe diet, exercise, and sleep patterns; mediation of seizure disorders on sleep; body mass index (BMI); relationships of lifestyle factors to BMI, cardiovascular conditions, and diabetic conditions; and sex differences among autistic adults. Results Autistic adults, and particularly autistic females, exhibit unhealthy diet, exercise, and sleep patterns; they are also more likely to be underweight or obese. Limited sleep duration and high rates of sleep disturbances cannot be accounted for by epilepsy or seizure disorders. Lifestyle factors are positively related to higher risk of cardiovascular conditions among autistic males, even more than family history. Limitations Our sample may not be representative of all autistic and non-autistic people, as it primarily comprised individuals who are white, female, have a high school education or higher, and reside in the UK. Our sampling methods may also exclude some individuals on the autism spectrum, and particularly those with moderate to severe intellectual disability. This is a cross-sectional sample that can test for relationships between factors (e.g., lifestyle factors and health outcomes) but cannot assess the direction of these relationships. Conclusions Autistic adults are less likely to meet minimal health recommendations for diet, exercise, and sleep—and these unhealthy behaviors may relate to excess risk of cardiovascular conditions. Although the present study can only provide preliminary, correlational evidence, our findings suggest that diet, exercise, and sleep should be considered and further investigated as key targets for reducing the now widely reported and dramatically increased risks of health comorbidity and premature death among autistic individuals compared to others. Physicians should work cooperatively with patients to provide health education and develop individualized strategies for how to better manage challenges with diet, exercise, and sleep.

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