Idiopathic systemic capillary leak syndrome - An often missed diagnosis.

Idiopathic systemic capillary leak syndrome (ISCLS) is a potentially fatal disorder characterised by 'attacks' of varying intensity of hypovolemic shock in association with haemoconcentration and hypoalbuminaemia. It is a disease of exclusion, and the severity of attacks may mimic sepsis at presentation. We report a case of a lady with recurrent attacks of ISCLS with at least two life-threatening episodes, having been treated elsewhere as a case of steroid insufficiency. The diagnosis is often challenging, and treatment of an acute episode involves the judicious use of fluids and vasopressors, as required. Prophylaxis to prevent further attacks is of varied success.

Perioperative improvement in serum albumin level in patients with left ventricular assist device.

BACKGROUND: The negative impact of baseline hypoalbuminemia on clinical outcome following left ventricular assist device (LVAD) implantation is well known. However, the implications of perioperative change in serum albumin levels on post-LVAD outcomes remain uninvestigated. METHODS: Among consecutive patients with baseline serum albumin <3.5 g/dl who received durable LVAD implantation between April 2014 and August 2017 and were followed for 1 year, the impact of perioperative change in serum albumin level from baseline to 3 months post-LVAD on the incidence of adverse events was investigated. RESULTS: Sixty-eight patients (median 60 years and 69% male) were included. Serum albumin change was an independent predictor of the occurrence of adverse events with an adjusted hazard ratio of 0.32 (95% confidence interval, 0.13-0.78) and a cutoff change of 0.7 g/dl. Those with albumin increase >0.7 g/dl had higher 1-year freedom from adverse events (45% vs. 14%, p = .008), dominantly due to lower incidence of death or sepsis compared with those without (p < .05 for both). CONCLUSION: Among those with baseline hypoalbuminemia, a considerable perioperative increase in serum albumin levels following LVAD implantation was associated with lower mortality and morbidity. The implication of aggressive nutrition intervention on LVAD patients is the next concern.

Substitution of perioperative albumin deficiency disorders (SuperAdd) in adults undergoing vascular, abdominal, trauma, or orthopedic surgery: protocol for a randomized controlled trial.

BACKGROUND: Hypalbuminemia is associated with numerous postoperative complications, so a perioperative albumin substitution is often considered. The objective of SuperAdd is to investigate whether substitution of human albumin, aiming to maintain a serum concentration > 30 g/l, can reduce postoperative complications in normovolemic surgical patients in comparison with standard care. METHODS/DESIGN: SuperAdd is a single-center, prospective, randomized, outcome-assessor blinded, patient blinded controlled trial. The primary outcome is the frequency of postoperative complications identified using the Postoperative Morbidity Survey graded ≥ 2 according to the Clavien-Dindo Score. Adult patients at risk to develop hypalbuminemia, i.e., ASA III or IV or high-risk surgery, are recruited after written informed consent was obtained. The albumin concentration is assessed before the induction of anesthesia and every 3 h until admission to the postanesthesia care unit. If albumin concentrations drop below 30 g/l, patients are randomly allocated to the control or the treatment group. The study intervention is a goal-directed human albumin substitution aimed at a concentration > 30 g/l during surgery and postanesthesia care unit stay. The patients in the control group are treated according to standard clinical care. Postoperative visits are to be performed on days 1, 3, 5, 8, and 15, as well as by telephone 6 months after surgery. DISCUSSION: SuperAdd is the first clinical trial in a surgical population investigating the effect of a goal-directed albumin substitution aiming at a serum level > 30 g/l. The nonrestrictive selection of patients guarantees that the patients without albumin screening will most likely not develop hypalbuminemia, thus ensuring generalizability of the study results. TRIAL REGISTRATION: EudraCT 2016-001313-24. Registered on 5 September 2016. Clinical Trials NCT03167645. Registered on 18 October 2016 and has the Universal Trial Number (UTN) U1111-1181-2625.

Experiencia de hemodiálisis con dializador de mediano poro en la nefropatía por cilindros del mieloma / Experience of using hemodialysis with medium cut-off dialyzer in cast nephropathy

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[Dietary control of metabolic acidosis in chronic kidney disease]. / Contrôle diététique de l'acidose métabolique chez le patient insuffisant rénal chronique.

Metabolic acidosis is a frequent complication of chronic kidney disease. Although it is known to appear at advanced stages, many studies suggest a state of "global protonic retention" starting at early stages of the disease, responsible of tissue damage, particularly musculoskeletal, alteration of protidic metabolism and endocrine disorders, promoting malnutrition and chronic inflammation, and finally increasing mortality. The majority of international recommandations suggest of supplementation by alkali, most of the time by sodium bicarbonate, to struggle against this complication. An interesting alternative to correct acidosis would consist on the modulation of the endogenous production of acid by playing with the alimentary incomes. In fact, it has been demonstrated that some different types of food produce or consume protons during their metabolism. Low protein diet and rich fresh fruits and vegetables diet would manage to correct at least as well as the supplementation by sodium bicarbonate the metabolic acidosis, and to struggle against its complications, noteworthy by slowing the decline of glomerular filtration rate by limiting the toxic adaptative fibrotic mechanisms, demonstrated by the decrease of urinary tubulo-interstitial suffering markers. Of the condition of being well led, those diets do not seem to expose patients to an over-risk of malnutrition or hyperkaliemia. They therefore appear to be an attractive alternative, efficiency and safe, to fight against chronic kidney disease metabolic acidosis and its complications.

Benefit of oral nutritional supplements for children with acute lymphoblastic leukaemia during remission-induction chemotherapy: a quasi-experimental study.

BACKGROUND AND OBJECTIVES: To determine the effect of oral nutritional supplements (ONS) on children with acute lymphoblastic leukaemia undergoing remission-induction chemotherapy. METHODS AND STUDY DESIGN: We included 127 paediatric patients who were diagnosed with acute lymphoblastic leukaemia and undergoing remission- induction chemotherapy in the First Affiliated Hospital of Zhengzhou University. Children from two paediatric wards who met the inclusion criteria were enrolled. One ward was randomly chosen as the intervention group and the other ward as the control group. Children in the two groups were matched for age and sex. The ONS group was administered Peptamen® (n=60) and the control group was administered a low-fat diet (n=67). RESULTS: The baseline information before treatment was not significantly different between groups (p>0.05). In the control group, weight loss at the end of chemotherapy was significantly higher than that of ONS group (p<0.05). The hemoglobin level and the concentrations of total protein, albumin, and pre-albumin were significantly higher in the ONS group than in the control group (p<0.05 and p<0.01, respectively). The incidences of hypoalbuminaemia, gastrointestinal complications, and infection were lower in the ONS group than in the control group (p<0.05). The ONS group also used lower amount of albumin infusion, fewer blood-product infusion, and had lower hospital costs than the control group. CONCLUSIONS: During remission-induction chemotherapy, oral nutritional supplements can improve the nutritional status of children, reduce the incidence of complications, and decrease the costs of hospitalization.

Supplementation with branched-chain amino acids ameliorates hypoalbuminemia, prevents sarcopenia, and reduces fat accumulation in the skeletal muscles of patients with liver cirrhosis.

BACKGROUND: Liver cirrhosis induces marked metabolic disorders, protein-energy malnutrition, and sarcopenia. The objective of the study reported here was to investigate the effects of dietary branched-chain amino acids (BCAAs) on systemic glucose metabolism, skeletal muscle, and prognosis of patients with liver cirrhosis. METHODS: Japanese patients with liver cirrhosis (n = 21) were enrolled into a longitudinal study in which their diets were supplemented with BCAAs. We evaluated glucose metabolism and analyzed the skeletal muscle area index (SAI) and intramuscular adipose tissue content (IMAC) using computed tomography. RESULTS: After 48 weeks of supplementation with BCAAs, there were no changes in glucose metabolism and skeletal muscle findings. In patients with ameliorated hypoalbuminemia, IMAC was significantly decreased and SAI was preserved concomitant with decreasing 90- and 120-min post-challenge plasma glucose levels (P < 0.01 each). In patients without increased albumin levels, IMAC was significantly increased and the SAI was significantly decreased (P < 0.01 each). Liver-related event-free survival rates for 72 months were 63.6% in patients with decreased IMAC and 20.0% in patients with increased IMAC. CONCLUSIONS: Amelioration of hypoalbuminemia associated with BCAA supplementation correlated with decreased fat accumulation in skeletal muscle, maintenance of skeletal muscle mass, and improved glucose sensitivity, all factors which may contribute to improving the survival of patients with liver cirrhosis.

Plantago asiatica L. Ameliorates Puromycin Aminonucleoside-Induced Nephrotic Syndrome by Suppressing Inflammation and Apoptosis.

OBJECTIVE: Nephrotic syndrome, a kidney disease with a variety of causes, is mainly characterized by heavy proteinuria, hypoproteinemia, and ascites. This study was designed to evaluate the underlying mechanism of action of Plantago asiatica L. (PAL) in treating nephrotic syndrome induced by puromycin aminonucleoside. METHODS: PAL has been used in Asia as a traditional medicine and dietary health supplement. Sprague-Dawley (SD) rats were intravenously injected with puromycin aminonucleoside (75 mg/kg/day), then treated with either Losartan (30 mg/kg/day) or PAL (200 mg/kg/day) by oral gavage for seven days. RESULTS: PAL significantly decreased ascites, proteinuria level, and plasma lipid parameters. In addition, treatment with PAL attenuated histological damage and hypoalbuminemia. Treatment with PAL also restored podocin expression and reduced inflammation markers such as intracellular adhesion molecules (ICAM-1), monocyte chemotactic protein-1 (MCP-1), tumor necrosis factor alpha (TNF-α) and high-mobility group box-1 (HMGB1). Lower expression levels of the apoptosis markers Bax, caspase-3 and capase-9 were documented in SD rats receiving PAL. PAL also significantly decreased the phosphorylation levels of MAPKs such as ERK, JNK and p38. CONCLUSION: As a multifunctional agent, PAL has a renoprotective effect in nephrotic syndrome rat models. The anti-inflammatory and anti-apoptotic properties, along with reductions in hyperlipidemia and ascites, represent important therapeutic effects. These results indicate that Plantago asiatica is likely to be a promising agent in the treatment of nephrotic syndrome.

The Effect of Ophiocephalus striatus Extract on the Levels of IGF-1 and Albumin in Elderly Patients with Hypoalbuminemia.

BACKGROUND: a freshwater fish Ophiocephalus striatus or known locally to Indonesian as haruan,can potentially increases IGF-1 and albumin levels in elderly patients with hypoalbuminemia due to the contents of amino acids, fatty acids, vitamins, and minerals. This study was conducted to investigate the effect of Ophiocephalus striatus extract on the level of IGF-1 and albumin in elderly patients with hypoalbuminemia. METHODS: the study is a double-blind randomized controlled trial involving malnourished elderly inpatients (≥60 years old) recovering from acute condition before hospital discharge, with Mini Nutritional Assessment score ≤23.5 and albumin level <3.5 g/dL. A total of 109 subjects were randomly divided into two groups: one group received 10 g Ophiocephalus striatus extract per day for 14 days and another group received placebo. Albumin and IGF-1 levels were obtained before and after intervention. RESULTS: ninety subjects completed the study (extract group=45 subjects; placebo group =45 subjects) for 14 days. The median of age were 69 (64;75) years and the male to female ratio were 2 : 3. The changes of IGF-1 and albumin levels from before to after intervention between extract group compared to placebo group were 14.7 (0.30;31.5) ng/mL vs 1.0 (-6;13.15) ng/mL (p=0.002) and 0.5 (0.15;0.70) g/dL vs 0.10 (0.0;0.50) g/dL (p=0.003), respectively. There were significant differences in the improvement of IGF-1 and albumin levels between extract and placebo group. CONCLUSION: supplementation of Ophiocephalus striatus extract was associated with a significant increase in IGF-1 and albumin levels in elderly patients with hypoalbuminemia.

Effect of Exogenous Albumin on the Incidence of Postoperative Acute Kidney Injury in Patients Undergoing Off-pump Coronary Artery Bypass Surgery with a Preoperative Albumin Level of Less Than 4.0 g/dl.

BACKGROUND: Hypoalbuminemia may increase the risk of acute kidney injury (AKI). The authors investigated whether the immediate preoperative administration of 20% albumin solution affects the incidence of AKI after off-pump coronary artery bypass surgery. METHODS: In this prospective, single-center, randomized, parallel-arm double-blind trial, 220 patients with preoperative serum albumin levels less than 4.0 g/dl were administered 100, 200, or 300 ml of 20% human albumin according to the preoperative serum albumin level (3.5 to 3.9, 3.0 to 3.4, or less than 3.0 g/dl, respectively) or with an equal volume of saline before surgery. The primary outcome measure was AKI incidence after surgery. Postoperative AKI was defined by maximal AKI Network criteria based on creatinine changes. RESULTS: Patient characteristics and perioperative data except urine output during surgery were similar between the two groups studied, the albumin group and the control group. Urine output (median [interquartile range]) during surgery was higher in the albumin group (550 ml [315 to 980]) than in the control group (370 ml [230 to 670]; P = 0.006). The incidence of postoperative AKI in the albumin group was lower than that in the control group (14 [13.7%] vs. 26 [25.7%]; P = 0.048). There were no significant between-group differences in severe AKI, including renal replacement therapy, 30-day mortality, and other clinical outcomes. There were no significant adverse events. CONCLUSION: Administration of 20% exogenous albumin immediately before surgery increases urine output during surgery and reduces the risk of AKI after off-pump coronary artery bypass surgery in patients with a preoperative serum albumin level of less than 4.0 g/dl.

Effect of branched-chain amino acids in patients receiving intervention for hepatocellular carcinoma.

AIM: To investigate the usefulness of branched-chain amino acids (BCAA) before transarterial chemoembolization (TACE) or radiofrequency ablation (RFA). METHODS: We investigated the usefulness of pre-intervention with BCAAs by comparing patients treated with BCAAs at 12.45 g/d orally for at least 2 wk before TACE or RFA and those not receiving such pretreatment. A total of 270 patients with hepatocellular carcinoma complicated by cirrhosis were included in the study. Mean changes from baseline (Δ) in serum albumin (Alb), C-reactive protein (CRP), and transaminase levels, as well as peak body temperature were also determined and compared at days 2, 5, and 10 after the start of TACE or RFA. RESULTS: In patients who underwent TACE or RFA, BCAA pre-intervention significantly suppressed the development of post- intervention hypoalbuminemia and reduced inflammatory reactions during the subsequent clinical course. After TACE, the ΔAlb peaked on day 2, remained constantly lower in BCAA-treated patients, compared to the control group, and was -0.13 ± 0.42 g/dL in BCAA-treated patients and -0.33 ± 0.51 g/dL in untreated patients on day 10. The ΔCRP was also significantly lower in BCAA-treated patients on days 2, 5 and 10 after TACE. Like the trends noted after TACE, a similar tendency was noted as to the ΔAlb and ΔCRP after RFA. The changes in serum Alb level were inversely correlated with CRP changes; therefore, a possible involvement of the anti-inflammatory effect of BCAAs was inferred as a factor contributory to the suppression of decrease in serum Alb level. CONCLUSION: Pre-intervention with BCAAs may hasten the recovery of serum Alb level and mitigate post-operative complications in patients undergoing TACE or RFA.

Tolvaptan increases urine and ultrafiltration volume for patients with oliguria undergoing peritoneal dialysis.

BACKGROUND: Hypoalbuminemia caused by peritoneal dialysate protein loss, frequently occurs in patients on peritoneal dialysis (PD) and is associated with an increased risk of death. We investigate whether PD dialysis exchange volume (PD volume) could be reduced with tolvaptan (TVP) through increased urine volume (UV). METHODS: The study included 11 stable patients with oliguria undergoing PD. The following parameters were examined-diuretic response and the effect of TVP on peritoneal ultrafiltration (UF), body weight, serum albumin, sodium, arm muscle area (AMA), PD volume, dialysis efficiency calculator (K t/V), and urine and serum osmolarity (OSM). RESULTS: The average UV increased from 428 ± 178 to 906 ± 285 mL (p = 0.018 by paired t test). Average weekly PD volume decreased from 28,836 ± 5,699 to 23,872 ± 3,569 mL (p = 0.04 by paired t test). Average UF increased from 283 ± 147 to 575 ± 135 mL (p = 0.019 by paired t test). On the other hand, there was no significant difference in the average dialysate K t/V before and after TVP treatment. Serum sodium, AMA, and serum albumin levels were not statistically different before and after TVP treatment. The urine and serum OSM ratio of effective cases before TVP treatment was higher than that of ineffective cases (p = 0.024 by unpaired t test). CONCLUSION: Our results indicate that TVP is useful for patients on continuous ambulatory PD who have oliguria and high urine osmolarity. Furthermore, we can reduce PD volume to maintain their nutritional status.

Hypoalbuminemia in peritoneal dialysis patients.

Clinicians caring for patients on peritoneal dialysis (PD) have relied on a variety of laboratory measures to assess the health of patients and their response to treatment. Traditionally, serum albumin has been an indicator of nutrition status and has therefore been included in monthly blood testing in most centers. The development of hypoalbuminemia in dialysis patients has been associated with increased mortality and often leads to interventions such as trials of nutritional supplements. In PD, hypoalbuminemia combined with ongoing losses of protein into effluent raise particular concerns with clinicians. Serum albumin may be affected by a variety of non-nutrition factors such as inflammation, volume status, and comorbidities. Albumin synthesis in the liver exceeds, in most cases, albumin losses in urine or effluent. Interpreting the medical implications of declining serum albumin in PD patients can therefore be a challenge. This paper reviews protein balance in PD. The nutritional and non-nutritional factors affecting serum albumin are discussed, with specific emphasis on how membrane physiology contributes to dialysate protein losses. A general clinical approach to the PD patient developing hypoalbuminemia is discussed.

Octreotide in Hennekam syndrome-associated intestinal lymphangiectasia.

A number of disorders have been described to cause protein losing enteropathy (PLE) in children. Primary intestinal lymphangiectasia (PIL) is one mechanism leading to PLE. Few syndromes are associated with PIL; Hennekam syndrome (HS) is one of them. The principal treatment for PIL is a high protein, low fat diet with medium chain triglycerides supplementation. Supportive therapy includes albumin infusion. Few publications have supported the use of octreotide to diminish protein loss and minimize hypoalbuminemia seen in PIL. There are no publications on the treatment of PIL with octreotide in patients with HS. We report two children with HS and PLE in which we used octreotide to decrease intestinal protein loss. In one patient, octreotide increased serum albumin to an acceptable level without further need for albumin infusions. The other patient responded more dramatically with near normal serum albumin levels and cessation of albumin infusions. In achieving a good response to octreotide in both patients, we add to the publications supporting the use of octreotide in PIL and suggest that octreotide should be tried in patients with PIL secondary to HS. To the best of our knowledge, this is the first case report on the use of octreotide in HS-associated PIL.

Nutritional status in relation to adipokines and oxidative stress is associated with disease activity in patients with rheumatoid arthritis.

OBJECTIVE: We assessed whether disease activity was associated with dietary habits, nutritional status, adipokines, and oxidative stress in patients with rheumatoid arthritis. METHODS: The subjects were 37 patients with RA. The assessment of the nutritional status included anthropometric and biochemical parameters. A food-frequency questionnaire and a 3-d diet record to assess dietary intake were used. The serum levels of adipokines and oxidative stress markers in sera and saliva were measured. The disease activity was determined using the 28 Disease Activity Score (DAS28). We divided the subjects into high (DAS28 ≥3.2) and low (DAS28 <3.2) disease activity groups. RESULTS: The serum leptin and albumin levels were significantly lower, whereas the inflammatory markers were increased, in the high disease activity group. The dietary intake assessment showed a lower intake of fish oil and a lower ratio of monounsaturated fatty acid intake in the high disease activity group. There was a negative correlation between the DAS28 and the dietary intake of the ratio of monounsaturated fatty acid to total fatty acid intake. The serum oxidative stress marker (reactive oxygen metabolites) showed a positive correlation to the DAS28. The salivary reactive oxygen metabolites also correlated with C-reactive protein and serum reactive oxygen metabolites. CONCLUSION: Altered serum adipokine levels with decreased albumin may reflect the deterioration that is associated with rheumatoid arthritis. An increased oxidative stress was observed in sera and saliva. Intakes of ω-3 polyunsaturated fatty acids, fish oil, and monounsaturated fatty acid seem to affect disease activity and may have beneficial effects by decreasing inflammation.

Mycophenolate mofetil or tacrolimus compared with intravenous cyclophosphamide in the induction treatment for active lupus nephritis.

BACKGROUND: Although the use of aggressive immunosuppression has improved both patient and renal survival of patients with lupus nephritis (LN), the optimal treatment of LN remains challenging. The objective of this study is to assess the efficacy and safety of mycophenolate mofetil (MMF) and tacrolimus compared with intravenous cyclophosphamide (IVC) as induction therapies for active lupus nephritis (ALN). METHODS: In this open-label, 24-week prospective study, 60 patients with biopsy-proven ALN (Classes III, IV, V or combination) were randomly assigned to receive MMF, tacrolimus or IVC in combination with corticosteroids. The remission of proteinuria, systemic lupus erythematosus disease active index and adverse events were compared. RESULTS: The response rates at 24 weeks were 70% (14/20) in the MMF group, 75% (15/20) in the tacrolimus group and 60% (12/20) in the IVC group (P>0.05). The complete remission rates were also similar in the three groups (40, 45 and 30%, respectively; P>0.05). There were more cases of infection in the IVC group (8/20) and the MMF group (8/20) than the tacrolimus group (3/20) and more hyperglycemia in the tacrolimus group (5/20) than the other two groups (2 or 3/20), but the results were not statistically significant among the three groups. Proteinuria decreased and serum albumin increased more quickly in the patients treated with tacrolimus (P=0.0051 and P=0.048). CONCLUSIONS: This pilot study suggests that both MMF and tacrolimus are possible alternatives to IVC as induction therapies for ALN in Chinese patients. Tacrolimus possibly results in a faster resolution of proteinuria and hypoalbuminemia. Further studies are necessary to determine the optimal dosage and duration of the therapies.

Albumin loss under the use of the high-performance membrane.

Beta-2-microglobulin (ß(2)M) clearance has been improved in recent dialysis membranes and minimum albumin leakage has been achieved in most membranes today since dialysis membranes are now classified by ß(2)M clearance in terms of the reimbursement by health insurance. Kawanishi et al. suggested that 'the desirable albumin leakage in one treatment is less than 4 g '. Function classification type IV or type V dialysis membranes have leakages as low as 3 g, while most resulted in < 1 g leakage. However, some high-performance membranes (HPMs) have around 8 g of leakage, which requires further study of the clinical efficacy using such dialysis membranes. The comparison of albumin leakage in one dialysis treatment (4 h: blood flow volume of 250 ml/min) revealed that PES-210D has an overwhelmingly high albumin leakage of 7.69 ± 1.01 g. The data were without any significant difference between the PES-D membrane user group and the non-PES-D membrane user group for hemoglobin, hematocrit, ß(2)M protein, catabolic rate, body mass index, and muscle mass. However, the PES-D membrane user group had a significantly lower number of hospitalizations and other complication events. When comparing the event contents, the PES-D membrane user group had no cardiac failure and lower DRA, however it had more vascular access problems. In normal renal function, approximately 10 g of albumin is filtered in the glomerulus per day, decomposed in the renal tubule and reabsorbed as amino acid, and re-synthesized into albumin in the liver. On the contrary, in dialysis patients, albumin that is bound to biologically active substances and/or the oxidized form of albumin that has lost its antioxidant effect cannot be filtered from the kidney and accumulate. Therefore, the idea regarding albumin leakage is to remove biologically active substances that bind to albumin and function as uremic toxin, remove albumin without the antioxidant effect, and facilitate synthesis of new albumin with an antioxidant effect.