Uncharted Territory: Frequent Relapsing, Steroid Sensitive Secondary Minimal Change Nephrotic Syndrome Cause by Solid Tumor of the Gastro-Esophageal Junction —(Case Presentation and Review of the Literature)

We reported a biopsy proved case of minimal change nephrotic syndrome in a 72-year-old patient. The minimal change nephrotic syndrome has been steroid sensitive, but the patient had 7 relapses over a span of 5 years. Each time the dose of steroid is tapered, a relapse of the nephrotic syndrome occurred. Eventually, the patient was complaining of dysphagia and difficulty swallowing. Hospital work-up with barium swallow, endoscopy, and CT of the chest, abdomen and pelvis, revealed a focal stenotic lesion with mild to moderate esophageal dysmotility 7/15/2022. A diagnosis of an ulcerating lesion with biopsy confirmed a neuro-endocrine carcinoma of the gastro-esophageal junction was entertained. The CT of the chest/abdomen/pelvis, 7/19/2022, has shown, an esophageal mass of 5.1 × 5.6 × 7 cm of the gastro-esophageal junction with ulceration. No evidence of spread beyond the esophagus and stomach. The histology revealed a poorly differentiated neuroendocrine tumor of the gastro-esophageal junction. The patient underwent several rounds of chemotherapy, radiation, and surgery culminating in tumor control. His nephrotic syndrome was resolved after the tumor has been controlled by surgery and chemotherapy.

Fulminant ulcerative colitis associated with steroid-resistant minimal change disease and psoriasis: A case report

A 43-year-old Chinese patient with a history of psoriasis developed fulminant ulcerative colitis after immunosuppressive therapy for steroid-resistant minimal change disease was stopped. Minimal change disease in association with inflammatory bowel disease is a rare condition. We here report a case showing an association between ulcerative colitis, minimal change disease, and psoriasis. The possible pathological link between 3 diseases is discussed.

Transition from minimal change disease to focal segmental glomerulosclerosis related to occupational exposure:A case report

BACKGROUND Although minimal change disease(MCD)and focal segmental glomerulosclerosis(FSGS)have been described as two separate forms of nephrotic syndrome(NS),they are not completely independent.We report a case of a patient transitioning from MCD to FSGS,review the literature,and explore the relationship between the two diseases.CASE SUMMARY A 42-year-old male welder,presenting with lower extremity edema and elevated serum creatinine,was diagnosed with NS and end-stage kidney disease(ESKD)based on laboratory test results.The patient had undergone a kidney biopsy for NS 20 years previously,which indicated MCD,and a second recent kidney biopsy suggested FSGS.The patient was an electric welder with excessive levels of cadmium and lead in his blood.Consequently,we suspect that his aggravated pathology and occurrence of ESKD were related to metal nephrotoxicity.The patient eventually received kidney replacement therapy and quit his job which involved long-term exposure to metals.During the 1-year follow-up period,the patient was negative for metal elements in the blood and urine and recovered partial kidney function.CONCLUSION MCD and FSGS may be different stages of the same disease.The transition from MCD to FSGS in this case indicates disease progression,which may be related to excessive metal contaminants caused by the patient’s occupation.

Minimal change disease caused by polycythemia vera: A case report

BACKGROUND Polycythemia vera(PV),often attributed to the JAK2 V617F mutation,is characterized by enhanced red blood cell counts in the peripheral blood.PV-associated renal disease is clinically rare;to date,there have been reports of other chronic kidney diseases related to PV,but no reports on PV-associated minimal change disease.CASE SUMMARY A 37-year-old man presented with proteinuria and high red blood cell count on January 4,2021.The patient underwent bone marrow and renal biopsies,then was subsequently diagnosed with PV and minimal change in disease.Hydroxyurea was administered and proteinuria remission was achieved.The patient’s last visit was on April 14,2022.CONCLUSION We inferred that there may be a causal relationship between PV and minimal change disease.

Clinical features of acute kidney injury in patients with nephrotic syndrome and minimal change disease:a retrospective,cross-sectional study

Background:Minimal change nephropathy(MCD)is a common pathological type of nephrotic syndrome and is often associated with acute kidney injury(AKI).This study aimed to investigate the clinical characteristics and related factors of AKI in patients with MCD and nephrotic syndrome.Methods:Patients from Chinese People’s Liberation Army General Hospital who were diagnosed with pathological renal MCD with clinical manifestations of nephrotic syndrome were included from January 1,2013 to December 31,2017.Patients diagnosed with membranous nephropathy(MN)by renal biopsy from January 1,2013 to December 31,2017 are included as a control population.We retrospectively analyzed the clinical and pathological characteristics of patients as well as the percentages and clinical characteristics of AKI in different age groups.We assessed the correlation of pathological characteristics with serum creatinine using multivariate linear regression analysis.Results:A total of 367 patients with MCD were included in the analysis,with a sex ratio of 1.46:1(male:female)and an age range of 6 to 77 years.Among all the patients,109 developed AKI(29.7%),and of these patients,85 were male(78.0%).In the 586 patients with MN,27(4.6%)patients developed AKI.The percentage of AKI in MCD patients was significantly higher than that in MN patients(χ^(2)=41.063,P<0.001).The percentage of AKI increased with age in the MCD patients.The percentage of AKI in patients aged 50 years or older was 52.9%(46/87),which was significantly higher than that[22.5%(63/280)]in patients under 50 years(χ^(2)=6.347,P=0.013).We observed statistically significant differences in age(43[27,59]years vs.28[20,44]years,Z=5.487,P<0.001),male(78.0%vs.51.4%,χ^(2)=22.470,P<0.001),serum albumin(19.9±6.1 g/L vs.21.5±5.7 g/L,t=2.376,P=0.018),serum creatinine(129.5[105.7,171.1]μmol/L vs.69.7[57.7,81.9]μmol/L,Z=14.190,P<0.001),serum urea(10.1[6.2,15.8]mmol/L vs.4.7[3.6,6.4]mmol/L,Z=10.545,P<0.001),IgE(266.0[86.7,963.0]IU/ml vs.142.0[35.3,516.5]IU/ml,Z=2.742,P=0.007),history of diabetes(6.4%vs.1.2%,P=0.009),and history of hypertension(23.9%vs.5.1%,χ^(2)=28.238,P<0.001)between the AKI group and the non-AKI group.According to multivariate linear regression analysis,among the renal pathological features analyzed,renal tubular epithelial cell damage(β=178.010,95%CI:147.888-208.132,P<0.001)and renal interstitial edema(β=28.833,95%CI:11.966-45.700,P=0.001)correlated with serum creatinine values.Conclusions:The percentage of AKI in MCD patients is significantly higher than that in MN patients.Patients over 50 years old are more likely to develop AKI.Renal tubular epithelial cell injury and renal interstitial edema may be the main pathological lesions that are associated with elevated serum creatinine in patients with MCD.

A sound and complete R-calculi with respect to contraction and minimal change

AGM postulates are for belief revision （revision by a single belief）, and DP postulates are for iterated revision （revision by a finite sequence of beliefs）. R-calculus is given for R-configurations △｜Г, where △ is a set of atomic formulas or the negations of atomic formulas, and Г is a finite set of formulas. We shall give two R-calculi C and M （sets of de- duction rules） such that for any finite consistent sets Г, △of formulas in the propositional logic, there is a consistent set ⊙ Г C of formulas such that △IГ → △, ⊙ is provable and⊙ is a contraction of F by A or a minimal change of F by A; and prove that C and M are sound and complete with respect to the contraction and the minimal change, respectively.

Reversible Chronic Interstitial Nephritis Induced by Tacrolimus —Tacrolimus Chronic Interstitial Nephritis

Calcineurin inhibitors (CNI) are potent immunosuppressive agents in prophylaxis against graft rejection and autoimmune diseases including primary glomerulopathies. Previous research showed reversible;acute afferent arteriolar vasculopathy and irreversible chronic interstitial fibrosis associated with CNI nephrotoxicity. In this case report we describe a patient, with minimal change disease, that had developed chronic and progressive renal disease while receiving therapeutic dose of Tacrolimus. His serum creatinine had reached 537 umol/L and his nephrotic state worsened. Kidney biopsy showed chronic interstitial nephritis. Tacrolimus was discontinued and he was treated with 1 mg/kg prednisone in addition to Mycophenolate mofetil (MMF) 1 g twice daily. By the 2nd month;serum creatinine returned to normal and by the 3rd month serum albumin too. After 1 month of therapy;the dose of Prednisone was tapered down gradually till 5 mg daily by the end of 3rd month. Moreover, the dose of MMF was reduced to 500 mg X2 by the end of 3rd month. After 2 years of follow up;he remained stable and without relapse of NS or renal failure. In conclusion, reversible renal disease, due to chronic interstitial nephritis can be induced by CNI which is amenable to treatment with Prednisone and MMF.

Children with Steroid-resistant Nephrotic Syndrome:Long-term Outcomes of Sequential Steroid Therapy

Objective This study aimed to investigate the long-term outcomes in children with steroid-resistant nephrotic syndrome （SRNS）, who received methylprednisolone pulse therapy （MPT）-based sequential steroid therapy. In particular, we aimed to observe whether these patients had a high risk of adverse events. Methods We conducted a retrospective study over a 5-year period. The long-term outcomes for children with SRNS receiving sequential therapy were observed. Results Sixty-three children were diagnosed with SRNS and underwent MPT-based sequential steroid therapy. Thirty-five （55.6%） achieved complete or partial remission, 19 （30.2%） of whom were in remission even after treatment cessation at last review. The mean time to initial remission after MPT was 24.3±13.1 days. Forty-nine children （77.8%） experienced relapses, of whom 31 （49.2%） demonstrated a frequent relapsing course. Adverse effects relevant to MPT were generally mild and infrequent. Five patients （7.9%） complained of vomiting or nausea during MPT infusion; 25 （39.7%） experienced excessive weight gain and developed an obvious Cushingoid appearance; and 26 （41.3%） had poor growth associated with long-term steroid use. Twenty-eight patients （44.4%） failed to respond to MPT, of whom 21 （33.3%） achieved complete or partial remission with immunosuppressive agents. Conclusion MPT-based sequential steroid therapy appears to be a safe and effective method for inducing rapid remission in childhood SRNS. Further clinical studies are needed to comprehensively evaluate this therapy.

Idiopathic Adult Nephrotic Syndrome: A Clinicopathological Study and Response to Steroid in a Sub-Saharan African Country

Introduction: Idiopathic nephrotic syndrome represents 25% to 30% of glomerulonephritis in adults. These glomerulonephritides are responsible of about the half of chronic kidney failure examined as well in United States as in Europe or Africa. The aim of this study was to determine the anatomoclinic, therapeutic and progression patterns of idiopathic nephritic syndrome in Dakar. Patients and Methods: It is a retrospective ten-year study in the nephrology department of Aristide Le Dantec Hospital. Patients with idiopathic nephrotic syndrome were included. We analyzed anatomoclinic, therapeutic and progression data of idiopathic nephrotic syndrome. Results: On 202 patients with nephrotic syndrome, 156 (77%) were primitive. The mean age was 29.7 ± 12 years with a sex ratio of 2.4. Edema was found in 98 patients (62.8%) and hypertension in 63 patients (40%). The mean proteinuria was 6.8 ± 4.8 g/24h. Histologic lesions found at renal biopsy were focal segmental glomerulosclerosis in 71 patients (45.5%), minimal change disease in 68 patients (43.5%) and membranous nephropathy in 8 patients (5%). 134 patients (85.8%) received steroids alone, 12 patients (7.6%) received cyclophosphamide and 4 patients (2.5%) azathioprine in association with steroids. 44 patients (28.2%) reached remission. The factors of poor prognosis were: age, above 40 years, proteinuria above 10 g/24h, existence of renal failure at admission, absence of use of steroids therapy. Conclusion: This study shows that idiopathic nephrotic syndrome is frequent in our country with a prevalence of 77%. The most common lesion found at the renal biopsy is the focal segmental glomerulosclerosis. Remission is found only in 28% which is very low. 33% of patients progress towards chronic kidney disease due to the lack of early diagnosis and the use of traditional medicine.

The Beneficial Effect of Combination of Mycophenolate with Low-Dose Corticosteroids and Calcineurin-Inhibitor as Well as Angiotensin-Converting Enzyme Inhibitor or Angiotensin II Blocker in Induction and Maintenance of Remission in Corticosteroid- and Rituximab-Resistant Minimal Change Nephrotic Syndrome in Adults

Management of steroid-resistant minimal change disease remains elusive with international guidelines suggesting high-dose corticosteroids and/or Calcineurin inhibitors for months similar to those with refractory idiopathic FSGS. Unfortunately, with such approach, the overall remission rates were 47% - 66%. Moreover, complete remission rates were 32% - 47% and partial remission ones were 19% - 29%. Those limited options of treatment and their poor outcomes led us to conduct the present study to assess the efficacy and safety of a new combined drug-therapy at induction and subsequent maintenance of such disease. The regimen consisted of an initial induction phase of 3-month Prednisone, Calcineurin-inhibitor, Mycophenolate and ACEI/ARB. The latter was followed by a maintenance phase of minimal dose Prednisone and nearly 1/2 the induction dose of Calcineurin inhibitors to decrease their long-term side effects. The results were satisfactory with 14 of the 22 patients, had complete remission. Moreover, 5 patients manifested partial remission and only 3 did not respond. Creatinine clearance was maintained in patients with complete remission yet, was mildly reduced in the partial and non-responsive ones. The safety and efficacy of such new combined drug-therapy provide new tool and future prospective in management of such relentless disease.

Pathogenesis of childhood idiopathic nephrotic syndrome:a paradigm shift from T-cells to podocytes

Background:Nephrotic syndrome is the most common cause of kidney disease in children,but its pathogenesis remains unclear.This article reviews the novel aspects of the mechanisms underlying massive proteinuria in minimal-change disease,which is the most common form of childhood nephrotic syndrome.Data sources:This article integrates the findings of a PubMed database search for English language articles published in the past 40 years(from September 1974 to February 2014)using the key words"pathogenesis","minimal change nephrotic syndrome"or"idiopathic ne phrotic syndrome".Results:Unknown humoral factors associated with T-cell dysfunction have been thought to play an important role in the pathogenesis of minimal-change disease.However,recent findings are changing this paradigm,i.e,visceral glomerular epithelial cells(podocytes)may be involved via expression of molecules such as CD80 and angiopoietin-like 4.Conclusions:Recent evidence suggests that minimal-change disease results from interactions between humoral factors and dysfunctional podocytes.In addition to immunosuppressant drugs that target lymphocytes,a biological agent such as an antibody against the abnormal molecule(S)expressed by podocytes may provide novel drug treatment for minimal-change disease.

Demonstration of the test-retest reliability and sensitivity of the Lower Limb Functional Index-10 as a measure of functional recovery post burn injury:a cross-sectional repeated measures study design

Background:Lower limb burns can significantly delay recovery of function.Measuring lower limb functional outcomes is challenging in the unique burn patient population and necessitates the use of reliable and valid tools.The aims of this study were to examine the test-retest reliability,sensitivity,and internal consistency of Sections 1 and 3 of the Lower Limb Functional Index-10(LLFI-10)questionnaire for measuring functional ability in patients with lower limb burns over time.Methods:Twenty-nine adult patients who had sustained a lower limb burn injury in the previous 12 months completed the test-retest procedure of the study.In addition,the minimal detectable change(MDC)was calculated for Section 1 and 3 of the LLFI-10.Section 1 is focused on the activity limitations experienced by patients with a lower limb disorder whereas Section 3 involves patients indicating their current percentage of pre-injury duties.Results:Section 1 of the LLFI-10 demonstrated excellent test-retest reliability(intra-class correlation coefficient(ICC)0.98,95%CI 0.96–0.99)whilst Section 3 demonstrated high test-retest reliability(ICC 0.88,95%CI 0.79–0.94).MDC scores for Sections 1 and 3 were 1.27 points and 30.22%,respectively.Internal consistency was demonstrated with a significant negative association(rs=?0.83)between Sections 1 and 3 of the LLFI-10(p<0.001).Conclusions:This study demonstrates that Section 1 and 3 of the LLFI-10 are reliable for measuring functional ability in patients who have sustained lower limb burns in the previous 12 months,and furthermore,Section 1 is sensitive to changes in patient function over time.