Nephrotic Syndrome: Past, Present and Future

Ignatova, M S; Длин, В. В.

doi:10.21508/1027-4065-2017-62-6-29-44

Cited by 12 publications

(10 citation statements)

References 24 publications

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“…Игнатовой и соавт. (2017) при НС возможно наслоение мофетила микофенолата на циклоспорин, что, по-видимому, может усиливать действие обоих препаратов, а также уменьшить нефротоксический эффект ИК [13]. Касаясь патогенетической терапии первичной ФСГС, необходимо указать на возможности использования цитостатиков.…”

Section: лекарственная терапия фсгсunclassified

“…Этот факт подтверждается результатами экспериментальных исследований, где тяжесть повреждения подоцитов и степень подоцитопении тесно коррелирует с гистологической моделью повреждения [11]. Патогенетические механизмы ФСГС все еще полностью не установлены, тем не менее отмечено, что мутации генов (ACTN4, INF2, COQ6, NPHS2, CD2AP, CD2AP, PDSS2, Glepp1, LMBX1, COL4A3/COL4A4, LAMB2, A3243G) кодирующие состояние белков щелевой мембраны подоцитов, лежат в основе развития наследственных форм этого заболевания [12,13]. У нескольких семей обнаружены и описаны разнообразные мутации генетических факторов при ФСГС [14].…”

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Focal segmental glomerulosclerosis: current status of the problem

Муркамилов

Сабиров

Фомин

et al. 2020

Arhivʺ vnutrennej mediciny

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One of the most prognostically unfavorable variants of glomerulopathy is focal segmental glomerulosclerosis (FSHC), which is detected by nephrobiopsy in 5-20% of patients with nephrotic syndrome (NS) and in 15% of adult patients with chronic glomerulonephritis. FSGS recurs in a transplanted kidney in 30-50% of patients. Among adult patients with FSH, men predominate. A poor prognosis of FSHC is explained by the heterogeneity of the disease and is exacerbated by a poor response to treatment. According to current data, FSGS is characterized by sclerosis of the mesangial matrix, hyalinosis, damage to capillaries, an increase in foam cells and their adhesion between the glomerular bundle and the Bowman capsule. In 2004, the following histological variants of FSGS were proposed: apical, perichillary, collaborating, cellular and classical. Each histological variant of FSGS differs in etiology, response to treatment, and prognosis. The clinical diagnosis of primary FSHC should be based on the exclusion of secondary causes of the disease. Focal sclerotic changes in the glomeruli can be caused by various factors and occur in various conditions, including the existing kidney pathology. According to international recommendations for the treatment of FSHS, one should focus on the amount of daily proteinuria. For patients with FSHS without pronounced proteinuria, the use of angiotensin converting enzyme inhibitors (ACE inhibitors) or angiotensin II receptor blockers (ARBs) is recommended. In FSGS and NS, immunosuppressive therapy is used along with ACE inhibitors or ARB II. For adult patients, glucocorticoids (HA) are prescribed daily in a single dose at a dose of 1 mg / kg per day, the maximum dose is 80 mg with a daily intake and 120 mg with an alternating regimen. Resistance to HA is detected in the absence of effect after 16 weeks. In the presence of contraindications or intolerance to HA, calcineurin inhibitors are used. The recommended initial dose of cyclosporine is 2 mg / kg / day, taken twice a day with a gradual increase to 3.5-4 mg / kg / day. The duration of therapy with satisfactory tolerance to cyclosporine is more than six months. After achieving complete remission, the dose of cyclosporin is gradually reduced by 0.5 mg / kg / day to the minimum effective dose (1.5-2 mg / kg / day) and such maintenance therapy is carried out for 1-2 years. A treatment option is possible using lower doses of HA and cyclosporine, or a combination of mycophenolate mofetil with a high dose of dexamethasone.

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Section: лекарственная терапия фсгсunclassified

unclassified

Focal segmental glomerulosclerosis: current status of the problem

Муркамилов

Сабиров

Фомин

et al. 2020

Arhivʺ vnutrennej mediciny

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“…Nephrotic syndrome (NS), which is characterized by proteinuria, hypoalbuminemia and pronounced oedema, is a pathological condition that affects the glomerular apparatus of the kidney in children. According to the literature, the frequency of NS is 0.5 per 10,000 children (1)(2)(3). It is believed that the NS can be a manifestation of glomerulonephritis (1,2).…”

Section: Introductionmentioning

confidence: 99%

“…According to the literature, the frequency of NS is 0.5 per 10,000 children (1)(2)(3). It is believed that the NS can be a manifestation of glomerulonephritis (1,2). NS is a common glomerulopathy among children, while its incidence ranges from 12-16 per 100,000 (4).…”

Section: Introductionmentioning

confidence: 99%

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NPHS2 gene sequencing results in children of the Azerbaijani population with different types of nephrotic syndrome caused by chronic glomerulonephritis

Baylarov¹,

Baylarova²,

Berdelı³

et al. 2019

BLL

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OBJECTIVES: The aim of the study was to determine the mutation of the podocin gene (NPHS2) in children with minimal changes diseases (steroid sensitive nephrotic syndrome (NS)) and steroid resistant NS. BACKGROUND: Despite the fact that the role of genetic factors is a well-known phenomenon, in NS there are still unknown aspects that are yet to be discovered. NS, type 2 is caused by NPHS2 gene and is characterised with proteinuria, minimal change disease on renal biopsy, poor response to steroid treatment, etc. METHODS: Twenty-nine children (65.5 % male, 34.5 % female) with nephrotic syndrome caused by chronic glomerulonephritis were examined and patients were tested for NPHS2 gene with Sanger technique. RESULTS: The average age was 7.2 ± 2.65 years. 82.8 % of patients had NS with minimal changes, 17.2 % had a steroid resistant NS. The analysis of the NPHS2 gene revealed a likely pathogenic (Arg168His), uncertain signifi cance (Pro20Ley, Leu169Pro, Val180Met, Arg229Gln, Val290Met) and benign (Gly34Gly, Ala318Ala) variants. No novel variants were detected. CONCLUSION: This is the fi rst study investigating the nephrotic syndrome related to NPHS2 gene in Azerbaijani population. The high prevalence of uncertain signifi cance variants emphasises the importance of population studies in this region as such data are necessary for classifi cations of the detected genetic variants (Tab. 1, Ref. 25). Text in PDF www.elis.sk.

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Development of hormone dependence in children with hormone-sensitive nephrotic syndrome at the onset of the disease

Nyrkova

Савенкова

2022

Ross. vestn. perinatol. pediatr.

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Objective. To identify the features of the development of hormone dependence in children with hormone-sensitive nephrotic syndrome at the onset of the disease.Characteristics of children and research methods. The authors carried out a retro- and prospective study with age assessment at the onset of the disease and first relapse in 118 children with hormone-sensitive nephrotic syndrome at the onset with a rarely and often recurrent course.Results. The authors revealed a rarely and often recurrent course in 118 children with hormone-sensitive nephrotic syndrome at the onset of the disease, respectively, in 51 and 49% of cases with the development of hormone dependence (64%) and without (36%). They found statistically significant differences: the age of children at the onset of nephrotic syndrome, who developed hormone dependence and who did not develop, was 3.4 [2.2; 4.8] and 4.2 [3.0; 5.6] years accordingly (p<0.05); the age of children with nephrotic syndrome who developed hormone dependence and who did not develop hormone dependence by first relapse, was 3.8 [2.6; 5.1] and 5.7 [4.0; 7.4] years accordingly (p<0.001). The duration of the period until the first relapse after the onset in children with nephrotic syndrome, who developed hormone dependence and who did not develop hormone dependence, was 4 [4; 5] and 11 [7; 17] months, respectively (p<0.00001). Hormone dependence was revealed in 75 and 47% patients, respectively (p<0.05) among children with nephrotic syndrome who had the first relapse before 5 years of age and after 5 years of age.Conclusion. The age of children at the onset and the first relapse of nephrotic syndrome, which developed hormone dependence, is significantly less than those without hormone dependence. Children with nephrotic syndrome who have had their first relapse before 5 years of age were more likely to become hormone-dependent.

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Nephrotic Syndrome: Past, Present and Future

Cited by 12 publications

References 24 publications

Focal segmental glomerulosclerosis: current status of the problem

Focal segmental glomerulosclerosis: current status of the problem

NPHS2 gene sequencing results in children of the Azerbaijani population with different types of nephrotic syndrome caused by chronic glomerulonephritis

Development of hormone dependence in children with hormone-sensitive nephrotic syndrome at the onset of the disease

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