Nephrotic Syndrome Induced by Tiopronin in a Male Patient with Cystinuria

As surrogate markers for autoimmune hepatitis (AIH), serum alanine aminotransferase (ALT) and immunoglobulin G (IgG) are convenient to measure under immunosuppression. However, the long-term prognosis of patients who achieve complete biochemical remission (CBR) in comparison with patients who achieve only biochemical remission (BR) is uncertain. A total of 291 patients (89.7% female) diagnosed with AIH were retrospectively reviewed. CBR was defined as normal ALT and IgG levels with immunosuppression, while BR was defined as normal ALT levels. CBR was further divided into early CBR (<1year) and late CBR (≥1year) by the timing of remission. Liver-related adverse outcomes including liver-related death, liver transplantation and hepatocellular carcinoma were evaluated. With immunosuppressive treatment, 222 (76.3%) patients achieved CBR (early CBR: 168 and late CBR: 54). BR was achieved in 55 (18.9%) patients and 14 (4.8%) patients remained non-remission. With a median follow-up duration of 6.6years, the risk of liver-related mortality was the lowest in patients with CBR, followed by patients with late CBR, BR and non-response. The cumulative risk of liver-related adverse outcomes was the highest in patients with non-response (8.51/100 person-years [PYs]), followed by BR (1.95/100 PYs), late CBR (1.89/100 PYs) and early CBR (0.75/100 PYs). By multivariable analysis, age, cirrhosis and treatment responses were independently associated with liver-related adverse outcomes. Patients with CBR within 1year after treatment initiation had the lowest risk of liver-related adverse outcomes. Patients with late CBR and those with only BR had a comparable risk of long-term outcomes.

Renoprotection with antihypertensive agents

Primary aldosteronism (PA) is the most common cause of endocrine hypertensionand adrenalectomy is the firstline treatment for unilateral PA. Suppression of aldosterone secretion of the nondominant adrenal gland at adrenal venous sampling (AVS), that is, contralateral suppression (CLS)has been suggested as a marker of disease severity. However, whether factors such as CLS, age, genderor comorbidities are associated with remission after surgery is controversial. The objective of this study is to investigate the prognostic value of CLS, age, gender, aldosterone-to-renin ratio, antihypertensives and comorbidities for clinical and biochemical remission following unilateral adrenalectomy in patients with PA. A retrospective study of patients with PA referred for AVS at Rigshospitalet from May 2011 to September 2020, who subsequently underwent adrenalectomy. Clinical remission was defined according to the PA surgical outcome criteria, whereascomplete biochemical remission was defined as normalization of hypokalaemia without potassium substitution. Eighty-four patients were available for analysis of primary outcome. Among patients with CLS, 28/58 (48.3%) obtained complete clinical remission after surgery compared with 10/26 (38.5%) without CLS (p = .40). Complete biochemical remission was obtained in 55/58 (94.8%) of patients with CLS compared with 25/28 (89.3%) without CLS (p = .44). Female gender and lower number of antihypertensives at baseline were associated with higher odds for complete clinical remission, whereasnone of the investigated variables were associated with biochemical remission. CLS was not significantly associated with complete clinical or biochemical remission in this cohort. Our results confirmed that female gender and lower number of antihypertensives were predictors of clinical remission.

Section 7: Heart Failure in Patients With Left Ventricular Systolic Dysfunction

Background Ulcerative proctitis is a subtype of Ulcerative colitis in which only the rectum is affected. Despite the length of inflammation and ulceration being significantly less than that observed in other ulcerative colitis phenotypes, the clinical impact on patients can be considerable. Our aim was to review clinician adherence to British Society of Gastroenterology (BSG) guidelines in the management of phenotypically E1 patients with active proctitis in a tertiary hospital outpatient setting. The secondary objective was to evaluate rates of biochemical, clinical and endoscopic remission at 6-18 months post endoscopy. Methods Retrospective data was collected from endoscopy reports from a single Australian tertiary centre over a 2-year period between 2022 to 2024. Patients included in this cohort were all above the age of 18 with active proctitis identified on endoscopy. Those who were not phenotypically E1 were excluded. Review of the electronic integrated medical system was then conducted to determine if management was in line with BSG guidelines. Evidence of biochemical, clinical and/or endoscopic remission status at 6-18 months post endoscopy finding were also reviewed. Clinical remission was defined as SCCAI score &amp;lt;2 , endoscopic remission was defined as UCEIS score of ≤ 1 and biochemical remission was defined as CRP ≤ 5 +/- faecal calprotectin ≤ 50. Results A total of 875 colonoscopies and/or sigmoidoscopies were identified with the impression of ulcerative colitis in the report. Of these 172 scopes showed proctitis. Among these, 74 of these patients were phenotypically E1 with no prior evidence of more extensive disease. Exclusion of 6 inpatients and 12 repeat procedures resulted in a total analysis of 56 patients (Figure 1). Fifty percent of patients (n=28) were treated in line with current BSG Guidelines (initial and maintenance). Eighty percent (43 of 56) of the patients reported clinical remission within 1 year. Biochemical remission was confirmed in 73.2% (41) of patients. Of the 56 patients, thirty were assessed for endoscopic remission within 18 months. Thirteen (43%) of these had proven endoscopic remission within 18 months of diagnosis. Conclusion Adherence to BSG guidelines was observed in 50% of cases, highlighting significant variability in the management of proctitis in an outpatient setting. A substantial proportion of patients reported complete clinical and biochemical remission within the 18 months. A relatively lower rate of confirmed endoscopic remission, suggests that this group of patients may be undertreated.

More on isolated systolic hypertension, diabetes, nephropathy and hypertension, and use of low-sodium diets in obese hypertensives.

Prevention of renal stones by a high fluid intake?

Hypocitraturia is a low urinary excretion of citrate and a well-known risk factor for kidney stone development in children. This systematic review aimed to evaluate the dietary management of hypocitraturia in children with urolithiasis. Literature search was performed on 30th September 2022 using Embase, PubMed, and Cochrane Central Controlled Register of Trials. Studies were included if children with stones and hypocitraturia were managed with diet supplements. Six papers were included. Four studies evaluated the role of oral potassium citrate associated with high fluid intake on stone resolution and recurrence. Two studies assessed the impact of oral potassium citrate on long-term stone recurrence after percutaneous nephrolithotomy and shock wave lithotripsy. All studies demonstrated that the association of potassium citrate and high fluid intake was well tolerated with no side effects and restored normal urine citrate excretion, allowed a reduction in stone size, and, following definitive treatments, was associated with a lower rate of stone regrowth and recurrence compared with controls. These effects were demonstrated across all pediatric ages. Our review infers that oral potassium citrate and high fluid assumption are safe and effective in restoring urine citrate excretion, treating and preventing stone recurrence with no serious adverse events, and should probably be the first-line treatment of pediatric patients with asymptomatic stones and hypocitraturia.

Usefulness of biochemical remission and transient elastography in monitoring disease course in autoimmune hepatitis

You are what you eat: dietary salt intake and renin–angiotensin blockade in diabetic nephropathy

Autoregulation of cerebral blood flow (CBF) denotes that CBF is constant despitefluctuation of blood pressure within wide limits. Inhibition of therenin–angiotensin system (RAS) is known to decrease the lower and upper limitsof CBF autoregulation. We have previously shown that this includes inhibition bythe angiotensin receptor blocker (ARB) candesartan. In the present study weinvestigated the influence of the ARB candesartan on the lower limit of CBFautoregulation in two groups of Sprague-Dawley rats, on high (4.0%Na+) and low (0.004% Na+) sodium diet, respectively.Control animals were given the same diet, but no ARB. CBF was studied with thelaser Doppler method. Blood pressure was lowered by controlled bleeding. Resultsrevealed that both high and low sodium diet with low and high renin levelsrespectively block the influence of candesartan on CBF autoregulation. This wasexpected in rats on a high salt diet with a low renin level, but unexpected inrats with a low salt intake with a high renin level.

Autoimmune hepatitis (AIH) is characterized by interface hepatitis, elevated serum alanine aminotransferase and aspartate aminotransferase levels, circulating autoantibodies, and elevated predominantly immunoglobulin G (IgG)levels. The goal in the treatment of autoimmune hepatitis (AIH) is complete disease remission. Here we took advantage of a large cohort of AIH patients to clarify predictors associated with biochemical and histological remission. Of 705 patients with complete follow-up, 569 (80.7%) patientsachieved complete biochemical remission. Lower IgG levels (17.8 vs. 25g/L, p < 0.001) and less liver cirrhosis (19.3% vs. 33.1%, p < 0.001) at diagnosis were observed in these patients. They also had lower serum IgG levels (13 vs. 18.9g/L, p < 0.001) after 3 months of treatment. Histological remission was achieved in 69.4% of 160 patients with complete biochemical remission after 3 years of treatment. Patients with histological remission had lower IgG levels (16.2 vs. 20.1g/L, p = 0.006) and Ishak fibrosis scores (3.4 vs. 4.1, p = 0.010) at diagnosis, and they appeared to achieve biochemical remission more rapidly (1 vs. 3months, p < 0.001). Of note, patients with histological remission had higher frequency of fibrosis regression than those with persisting histological activity (87.5% vs. 60%, p = 0.004). In conclusion, lower serum IgG levels, less fibrosis in liver histology at diagnosis, and rapid response to immunosuppressive therapy are reliable predictors of biochemical and histological remission. Our study underscores the importance of early diagnosis and appropriate treatment.

Management of Hypertension in Patients With Diabetic Kidney Disease: Summary of the Joint Association of British Clinical Diabetologists and UK Kidney Association (ABCD-UKKA) Guideline 2021

The review presents materials on the prevalence of NS in children, variants of its course: steroid-sensitive (SSNS) and steroidresistant (SRNS) steroid-dependent (SSNS). Minimal change nephrotic syndrome minimal changes (NSMC) is the most common glomerular disorder. Although NSMC has an excellent prognosis with a low risk of progression to t-CRF, its recurrent nature requires children to receive frequent courses of steroid therapy and other medications, many of which are known to affect blood pressure (BP). The interrelation of NS in children with arterial hypertension (AH) is shown. Prevalence of hypertension in children with SCNS, SRNS, SZNS is given. The regulation of hypertension in children is mandatory in the treatment of NS, due to the fact that hypertension is not established in a timely manner, is insufficiently controlled and is often masked. Vascular dysregulation, fluid overload, increased cardiac output and peripheral vascular resistance, alone or in combination, can lead to hypertension in CKD. The use of modern methods to monitor and control blood pressure is critical for improving hypertension management and preventing target organ damage in children. 24-hour blood pressure measurements are an important tool in determining the prognosis and treatment of children with HC. Many comorbidities increase the risk of cardiovascular disease, including obesity, left ventricular hypertrophy (LVH), increased arterial stiffness (increased BMI, endothelial dysfunction), impaired glucose metabolism, and hyperlipidemia. The pathophysiological aspects of hypertension in children with NS are considered. The pathophysiology of hypertension in NS is complex, with many renal and extrarenal factors. Renal factors include sodium retention, fibrosis / decreased GFR, and progression of kidney disease, and a direct link between albuminuria and blood pressure has recently been described. Other factors include drug side effects, comorbidities and genetic predisposition. Sodium metabolism plays an important role in the development of edema and blood pressure regulation in NS. There are two main hypotheses for sodium retention in NS, the hypothesis of underfilling and overfilling. The role of the epithelial sodium channel (ENC), atrial natriuretic peptide (ANP), nitric oxide (NO), steroid hormones and other drugs in sodium retention and the pathogenesis of hypertension is also considered. In children with NS, hypertension leads to target organs damage (TOD): left ventricular hypertrophy (LVH), damage to the organ of vision, cognitive impairment and more rapid progression of chronic kidney disease. Salt restriction and RAAS inhibition are considered integral parts of the treatment of children with proteinuria, and both are known to have blood pressure lowering effects. The RAAS blockade has a renoprotective effect in patients with glomerular damage. Studies have found greater reductions in proteinuria with ACE / ARB combination therapy. This renoprotective effect is explained by both a decrease in blood pressure and mechanisms independent of blood pressure. Lifestyle modifications, weight control, healthy eating, reduced sodium intake, supportive exercise, and basic drug therapy using angiotensin-converting enzyme (ACE) inhibitors, angiotensin receptor blockers (ARBs), diuretics can slow the progression of NS in children.

Interventions for idiopathic steroid-resistant nephrotic syndrome in children.