scholarly journals Adrenal Insufficiency in Children With Nephrotic Syndrome on Corticosteroid Treatment

2020 ◽  
Vol 8 ◽  
Author(s):  
Karmila Abu Bakar ◽  
Khairunnisa Khalil ◽  
Yam Ngo Lim ◽  
Yok Chin Yap ◽  
Mirunalini Appadurai ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Adrenal Insufficiency ◽  
Corticosteroid Treatment

scholarly journals Sphingosine-1-phosphate lyase mutations cause primary adrenal insufficiency and steroid-resistant nephrotic syndrome

2017 ◽  
Vol 127 (3) ◽  
pp. 942-953 ◽  
Author(s):  
Rathi Prasad ◽  
Irene Hadjidemetriou ◽  
Avinaash Maharaj ◽  
Eirini Meimaridou ◽  
Federica Buonocore ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Adrenal Insufficiency ◽  
Primary Adrenal Insufficiency ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome ◽  
Sphingosine 1 Phosphate

scholarly journals ASSOTIATIONS OF PECULIARITIES OF HLA-PHENOTYPE AND THE SENSIBILITY TO THE CORTICOSTEROID TREATMENT IN PATIENTS WITH CHRONIC GLOMERULONEPHRITIS WITH NEPHROTIC SYNDROME

2017 ◽  
pp. 37-44
Author(s):  
M. Kolesnyk ◽  
G. Drannik ◽  
V. Driyanska ◽  
O. Petrina ◽  
M. Velychko
Keyword(s):  
Nephrotic Syndrome ◽  
Hla Antigens ◽  
Corticosteroid Treatment ◽  
Steroid Resistance ◽  
Control Group ◽  
Chronic Glomerulonephritis ◽  
Healthy Donors ◽  
Steroid Sensitive ◽  
Steroid Sensitivity ◽  
Hla Phenotype

The purpose of study was determination of HLA -antigens I and II classes as predictors of ineffectiveness of initial steroid therapy, and according prognozonegative markers of chronic glomerulonephritis with nephrotic syndrome. Methods. In 59 chronic glomerulonephritis with nephrotic syndrome patients (steroid sensitive n=33 (1 gr.) and steroid resistant’s n= 26 (2 gr.)) and 350 healthy donors( control group) studied HLA antigens I and II classes of the special anti- HLA-antigens panel (20 antigens of locus A, 31 – of locus B and 9- of locus DR). Result. In patients with chronic glomerulonephritis, nephrotic syndrome with hormone sensitivity relative risk is high at the presents of A28 (RR=8,5, r р <0,001), it made attributive risk (=0,37). In comparison with a control group, RR>2 for antigens  A11  (RR=2,23), A23 (RR=4,28),  A24 (RR=3,3),  A29 (RR=10,78) that A30 (RR=11,23); attributive risk more than 0,1 for the antigen A11 (=0,16) ; A24 (=0,13), other did not differ from control. Subzero connection is exposed for the antigens of A2 (р<0,001), А9 (р=0,007). In locus antigen B14 (RR=5,65, р =0,001) are exposed, B44 (RR=48,25, р =0,004), B51(RR=12,32, р =0,006) and attributive risk of development of disease (according =0,24, 0,12 ; 0,14); and antigens B38 and B41 (RR=11,57, р=0,05). The steroid sensitivity was associated with the antigens B5 (p=0,033), B12 (p=0,005) and B35 (p=0,021). In locus DR made etiologic faction antigens DR4 (RR=7,0 and =0,24) DRw52 (RR=7,0 and =0,25). Conclusions. For patients with chronic glomerulonephritis with a nephrotic syndrome antigens of HLA-B14,B38, B51, DRw52 are associated with steroid sensitivity. The attributive risk of steroid resistance is high for split A19+31+32, antigens B8, B55.

scholarly journals Corticosteroid-responsive nephrotic syndrome in children with myelodysplastic syndromes

2002 ◽  
Vol 130 (9-10) ◽  
pp. 323-328 ◽  
Author(s):  
Radovan Bogdanovic ◽  
Milos Kuzmanovic ◽  
Jasmina Markovic-Lipkovski ◽  
Milos Ognjanovic ◽  
Dragan Micic ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Myelodysplastic Syndromes ◽  
Rapidly Progressive Glomerulonephritis ◽  
Corticosteroid Treatment ◽  
Refractory Anemia ◽  
Al Amyloidosis ◽  
Glomerular Diseases ◽  
Medline Search ◽  
Haematological Remission ◽  
Repeated Infections

Several reports have documented various forms of glomerular diseases in adults with myelodysplastic syndromes (MDS), but similar reports in children are lacking. We describe two children with MDS-associated with steroid-responsive nephrotic syndrome (NS). Patient 1, who had MDS with myelofibrosis, presented also hepatosplenomegaly, pancytopenia, chronic hepatitis, moderate proteinuria, hypocomplementemia and elevated ANA titer. During initial prednisone treatment proteinuria markedly diminished and partial but transient haematological improvement occurred. Relapse subsequently occurred that was manifested by overt NS and pancytopenia. High doses of prednisolone led to remission of the renal disease but haematological remission did not occur. Persisting pancytopenia and repeated infections terminated in sepsis, two years after the onset of MDS. Patient 2, who had refractory anemia with clonal monosomy 19, manifested bowel disease, hepatospleno- megaly, anaemia and non-organic specific autoantibodies. Prednisone led to both clinical and haematological remission. Haematologic disease relapsed 12 months later, when nephrotic-range proteinuria, haematuria and mild azotaemia were also found. Corticosteroid treatment led to long-lasting renal and haematologic remission, maintained by a small dosage of prednisone. In both patients renal biopsy findings were consistent with those seen in idiopathic NS. A Medline search disclosed 16 cases of glomerulopathy in the course of MDS in adult patients. Clinical features included NS, usually accompanied by renal insufficiency with either acute, chronic, or rapidly progressive glomerulonephritis. On biopsy, membranous nephropathy, crescentic or mesangial proliferative glomerulonephritis and AL amyloidosis, were found. We conclude: (1) that glomerular disease may be present and should be searched for in patients with MDS; (2) that MDS can be added to the list of rare conditions associated with corticosteroid-responsive NS in children.

scholarly journals Risk factors for low bone density in pediatric nephrotic syndrome

2011 ◽  
Vol 51 (2) ◽  
pp. 61
Author(s):  
Corina Lisa ◽  
Madarina Julia ◽  
Pungky A. Kusuma ◽  
Tonny Sadjimin
Keyword(s):  
Risk Factors ◽  
Nephrotic Syndrome ◽  
Bone Density ◽  
Bone Mineral ◽  
Serum Calcium ◽  
Corticosteroid Treatment ◽  
Mean Difference ◽  
Mineral Density ◽  
Lower Serum ◽  
Steroid Dependent

Background Disturbances in bone mineral metabolism and side effects of corticosteroid treatment may cause decreased bone density in patients v.ith nephrotic syndrome (NS).Objectives To compare the prevalence oflow bone mineral density (BMD) in children with and 'Without NS and to assess the effect of corticosteroid treatment on bone density in NS patients. Methods We conducted a retrospective, cohort study in children aged 5-18 years diagnosed 'With NS for more than 2 months prior to data collection, and in children v.ithout NS as a control. BMD was assessed on calcaneal bone wlith ultrasound bone densitometry. Serum calcium, albumin, creatinine and phosphate levels were also assessed.Results The prevalence of low BMD was significantly higher in NS patients than non􀁂NS subjects, 73.3% (22 in 30) vs. 33% (11 in 33), respectively. The prevalence ratio was 6.3 (95% CI 2.1 to 18.9). NS patients had lower serum calcium levels, With mean difference of -0.17 (95% CI -0.27 to -0.07 mMollL), P<0.009, and lower serum albumin, with mean difference of  -0.88 (95% CI -1.27 to -0.49 gIL); P<O.OO 1, than non􀁂NS subjects. After adjusting for other risk factors, we found NS to be an independent risk factor for low BMD. Steroid-resistant and steroid-dependent patients had lower BMD than steroid-sensitive subjects (P=0.02). There was also a significant correlation between the onset of corticosteroid treatment and BMD (r=O.3; P=0.02).Conclusions NS patients had higher risk for low BMD compared to normal subjects. Response to steroid treatment influences the severity of impaired bone density.

scholarly journals SGPL1 Deficiency: A Rare Cause of Primary Adrenal Insufficiency

2018 ◽  
Vol 104 (5) ◽  
pp. 1484-1490 ◽  
Author(s):  
Nikolaos Settas ◽  
Rebecca Persky ◽  
Fabio R Faucz ◽  
Nicole Sheanon ◽  
Antonis Voutetakis ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Adrenal Insufficiency ◽  
Brain Mri ◽  
Clinical Manifestations ◽  
Gene Mutations ◽  
Primary Adrenal Insufficiency ◽  
Sphingosine 1 Phosphate ◽  
Recessive Genes ◽  
Initiation Of Therapy ◽  
Glucocorticoid Deficiency

Abstract Context Multiple autosomal recessive genes have been etiologically linked to primary adrenal insufficiency (PAI). Recently, sphingosine-1-phosphate lyase 1 (SGPL1) gene mutations were recognized as a cause of steroid-resistant nephrotic syndrome type 14 (NPHS14), a sphingolipidosis with multisystemic manifestations, including PAI. Objective To check if SGPL1 mutations are involved in the pathogenesis of PAI in patients who do not exhibit nephrotic syndrome. Methods Sequencing of the SGPL1 gene in 21 patients with familial glucocorticoid disease or triple A syndrome. Results We identified two missense SGPL1 variants in four patients, two of whom were first cousins. We describe in detail the proband, a boy born to Saudi Arabian consanguineous parents with a homozygous c.665G&gt;A, p.R222Q SGPL1 variant. The patient presented with hypoglycemia and seizures at age 2 years and was ultimately diagnosed with PAI (isolated glucocorticoid deficiency). Brain MRI showed abnormalities in the basal ganglia consistent with a degenerative process albeit the patient had no neurologic symptoms. Conclusions New genetic causes of PAI continue to be identified. We suggest that screening for SGPL1 mutations should not be reserved only for patients with nephrotic syndrome but may also include patients with PAI who lack other clinical manifestations of NPHS14 because, in certain cases, kidney disease and accompanying features might develop. Timely diagnosis of this specific sphingolipidosis while the kidneys still function normally can lead to prompt initiation of therapy and improve outcome.

scholarly journals Rifampicin-induced non-responsiveness to corticosteroid treatment in nephrotic syndrome.

BMJ ◽  
1979 ◽  
Vol 1 (6159) ◽  
pp. 306-306 ◽  
Author(s):  
W Hendrickse ◽  
J McKiernan ◽  
M Pickup ◽  
J Lowe
Keyword(s):  
Nephrotic Syndrome ◽  
Corticosteroid Treatment

Urinary Albumin and Low Molecular Weight Protein Excretion in the Nephrotic Syndrome—Sequential Studies during Corticosteroid Treatment

1992 ◽  
Vol 29 (4) ◽  
pp. 450-453 ◽  
Author(s):  
Robert Beetham ◽  
David Newman
Keyword(s):  
Molecular Weight ◽  
Nephrotic Syndrome ◽  
Preliminary Investigation ◽  
Corticosteroid Treatment ◽  
Retinol Binding Protein ◽  
Low Molecular Weight ◽  
Proximal Tubular ◽  
Weight Protein ◽  
Tubular Absorption ◽  
Low Molecular Weight Proteins

In a preliminary investigation into the behaviour of low molecular weight proteins in the nephrotic syndrome, we have measured urinary concentrations of albumin, α-1-microglobulin (α1-m) and retinol-binding protein (RBP) in six children for up to 11 days during the course of steroid therapy for nephrotic syndrome. The results in part support the concept of independent proximal tubular absorption of albumin and low molecular weight proteins, and indicate that in the nephrotic syndrome the excretion of RBP and α1-m, two generally accepted markers of tubular proteinuria, is anomalous.

Infantile nephrotic syndrome, immunodeficiency and adrenal insufficiency—a rare cause: Answers

2022 ◽  
Author(s):  
Georgie Mathew ◽  
M. S. Yasmeen ◽  
R. V. Deepthi ◽  
Meenakshi Swain ◽  
Avinash Vattam ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Adrenal Insufficiency ◽  
Infantile Nephrotic Syndrome
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