A case of γ-butyrolactone associated with severe withdrawal delirium and acute renal failure

γ-Butyrolactone (GBL) is a popular drug of abuse which is easily available over the internet. Following a UK classification change to a class C drug in January 2010, internet supply has become difficult. Some of the effects have resulted in sourcing GBL from industrial solvents. We report a case of a 24-year-old man who was admitted for detoxification from GBL. He reported having sourced the GBL by diluting the contents of nail varnish remover pads with water. During his admission he developed a severe withdrawal delirium and acute renal failure. He required admission to the intensive care unit. Physicians and psychiatrists should be aware of toxic sources of GBL leading to renal failure and consider GBL in those presenting with agitation, psychosis or coma.     

A case of PDH-E1α mosaicism in a male patient with severe metabolic lactic acidosis

We have characterized a novel mutation in a male patient that affects the coding sequence of PDH-E₁ gene and changes arginine-141 to a leucine. This nucleotide substitution was found in about 75% of the studied DNA (fibroblasts, liver and muscle), a scenario that would indicate a case of E₁ mosaicism in a male patient. When the mutant E₁ protein was expressed in human skin fibroblasts with zero endogenous pyruvate dehydrogenase complex activity and E₁ protein expression, no significant restoration of activity was recorded, in contrast to the wild-type cDNA, even though both wild-type and mutant protein levels were comparable. We concluded that the R141L mutation is a severe one and that it must have occurred in one of the E₁ alleles during early embryogenesis.     

Dialysis Disequilibrium Syndrome: Brain death following hemodialysis for metabolic acidosis and acute renal failure – A case report

Background

Hepatitis C virus (HCV) infection is a significant problem among patients undergoing maintenance hemodialysis (HD). We conducted a prospective multi-center study to evaluate the effect of dialysis machine separation on the spread of HCV infection.

Methods

Twelve randomly selected dialysis centers in Tehran, Iran were randomly divided into two groups; those using dedicated machines (D) for HCV infected individuals and those using non-dedicated HD machines (ND). 593 HD cases including 51 HCV positive (RT-PCR) cases and 542 HCV negative patients were enrolled in this study. The prevalence of HCV infection in the D group was 10.1% (range: 4.6%– 13.2%) and it was 7.1% (range: 4.2%–16.8%) in the ND group. During the study conduction 5 new HCV positive cases and 169 new HCV negative cases were added. In the D group, PCR positive patients were dialyzed on dedicated machines. In the ND group all patients shared the same machines.

Results

In the first follow-up period, the incidence of HCV infection was 1.6% and 4.7% in the D and ND group respectively (p = 0.05). In the second follow-up period, the incidence of HCV infection was 1.3% in the D group and 5.7% in the ND group (p < 0.05).

Conclusions

In this study the incidence of HCV in HD patients decreased by the use of dedicated HD machines for HCV infected patients. Additional studies may help to clarify the role of machine dedication in conjunction with application of universal precautions in reducing HCV transmission.     

Extreme efficacy of intravenous immunoglobulin therapy for severe burning pain in a patient with small fiber neuropathy associated with primary Sjögren’s syndrome

Abstract

Neurological involvement occurs in approximately 20% of patients with primary Sjögren’s syndrome. Although neurological symptoms can affect the peripheral nervous system and the central nervous system, the most frequent symptom is polyneuropathy. Small fiber neuropathy (SFN) is a form of painful peripheral polyneuropathy that is common in patients with diabetic neuropathy, but may also occur in toxic, infectious, or immune-mediated neuropathy. We show here a patient with Sjögren’s syndrome who developed SFN and was treated with intravenous immunoglobulin (IVIG) therapy, which was immediately and extremely effective. Because of the efficacy of IVIG therapy, we propose that direct immune-mediated mechanisms may be involved in the pathogenesis of SFN complicated by Sjögren’s syndrome.     

Extreme efficacy of intravenous immunoglobulin therapy for severe burning pain in a patient with small fiber neuropathy associated with primary Sj?gren’s syndrome

Neurological involvement occurs in approximately 20% of patients with primary Sjögren’s syndrome. Although neurological symptoms can affect the peripheral nervous system and the central nervous system, the most frequent symptom is polyneuropathy. Small fiber neuropathy (SFN) is a form of painful peripheral polyneuropathy that is common in patients with diabetic neuropathy, but may also occur in toxic, infectious, or immune-mediated neuropathy. We show here a patient with Sjögren’s syndrome who developed SFN and was treated with intravenous immunoglobulin (IVIG) therapy, which was immediately and extremely effective. Because of the efficacy of IVIG therapy, we propose that direct immune-mediated mechanisms may be involved in the pathogenesis of SFN complicated by Sjögren’s syndrome.     

Cross-sectional and longitudinal association of serum alanine aminotransaminase and γ-glutamyltransferase with metabolic syndrome in middle-aged and elderly Chinese people

Background: Although associations of the liver enzymes alanine aminotransaminase (ALT) and γ ‐glutamyltransferase (GGT) with metabolic syndrome (MetS) are well recognized, whether they are independent of insulin resistance and which enzyme is more effective are yet to be clarified. Methods: A total of 5404 subjects aged ≥40 years were recruited from two urban communities in Shanghai for cross‐sectional analyses. A subgroup of 681 participants without MetS at baseline was included in the longitudinal analyses. Insulin resistance was measured using the homeostasis model assessment of insulin resistance (HOMA‐IR), and the modified National Cholesterol Education Program Adult Treatment Panel III criteria were adopted to diagnose MetS. Results: Both GGT and ALT were strongly and positively associated with MetS risks in simple and multivariate analyses. Further adjustment for HOMA‐IR and ALT did not change the association of GGT and MetS materially, whereas adjustment for HOMA‐IR and GGT substantially attenuated the ALT–MetS association. In longitudinal analyses, risks of developing MetS were increased across GGT quartiles in a dose‐dependent manner after extensive adjustments (odds ratios were 1.00, 1.38, 1.62, and 2.29 for GGT, quartile 1 through quartile 4; P for trend = 0.01). In contrast, ALT was no longer associated with MetS development after final adjustment for GGT (P for trend = 0.09). Conclusions: Our study confirmed significant and independent associations of GGT and ALT with MetS in adult Chinese people. Moreover, GGT might be more effective for indicating the future development of MetS.     

Elevated levels of soluble E-selectin in diabetic patients with severe symptomatic peripheral arterial occlusive disease requiring angioplasty. A possible role in diabetic vascular disease?

BACKGROUND: To study levels of E-selectin in patients with peripheral arterial occlusive disease who were undergoing percutaneous transluminal angioplasty. METHODS: Experimental design and setting: Cross sectional case control study, conducted in a teaching hospital. SUBJECTS: Seven patients with type-2 (non-insulin dependent) diabetes mellitus undergoing transluminal angioplasty for symptomatic peripheral arterial occlusive disease, had blood tests for sE-selectin measurement, and were compared to a similar groups of age and sex matched non-diabetic patients with arteriopathy who are undergoing the same procedure. Also evaluated were a group of diabetic patients and healthy non-diabetics with no peripheral arterial disease. RESULTS: The levels of sE-selectin in the two diabetic groups were significantly higher than the non-diabetic groups measuring at 77 ng/ml (53-120) and 79 ng/ml (43-98) (median, range) vs 54 ng/ml (24-104) in the non-diabetic with arteriopathy, and 42 ng/ml (35-66) in the normal healthy controls, p<0.04, p<0.003 respectively, Mann Whitney "U" test. CONCLUSIONS: We have demonstrated significantly high values of soluble E-selectin in patients with diabetes mellitus requiring angioplasty for symptomatic peripheral arterial occlusive disease. This suggests that sE-selectin may be involved in the diabetic angiopathic process. It may act as a precursor for smooth muscle proliferation.     

A randomised clinical trial to assess the effect of total enteral and total parenteral nutritional support on metabolic,inflammatory and oxidative markers in patients with predicted severe acute pancreatitis (APACHEE II ≥ 6)

Background: Total enteral nutrition (TEN) within 48 h of admission has recently been shown to be safe and efficacious as part of the management of severe acute pancreatitis. Our aim was to ascertain the safety of immediate TEN in these patients and the effect of TEN on systemic inflammation, psychological state, oxidative stress, plasma glutamine levels and endotoxaemia. Methods: Patients admitted with predicted severe acute pancreatitis (APACHE II score > 5) were randomised to total enteral (TEN; n = 8) or total parenteral nutrition (TPN; n = 9). Measurements of systemic inflammation (C-reactive protein), fatigue (visual analogue scale), oxidative stress (plasma thiobarbituric acid-reactive substances), plasma glutamine and anti-endotoxin IgG and IgM antibody concentrations were made on admission and repeated on days 3 and 7 thereafter. Clinical progress was monitored using APACHE II score. Organ failure and complications were recorded. Results: All patients tolerated the feeding regime well with few nutrition-related complications. Fatigue improved in both groups but more rapidly in the TEN group. Oxidative stress was high on admission and rose by similar amounts in both groups. Plasma glutamine concentrations did not change significantly in either group. In the TPN group, 3 patients developed respiratory failure and 3 developed non-respiratory single organ failure. There were no such complications in the TEN group. Hospital stay was shorter in the TEN group [7 (4–14) vs. 10 (7–26) days; p = 0.05] as was time to passing flatus and time to opening bowels [1 (0–2) vs. 2 (1–5) days; p = 0.01]. The cost of TEN was considerably less than of TPN. Conclusion: Immediate institution of nutritional support in the form of TEN is safe in predicted severe acute pancreatitis. It is as safe and as efficacious as TPN and may be beneficial in the clinical course of this disease.     

Circulating alanine transaminase (ALT) and γ-glutamyl transferase (GGT), but not fetuin-A,are associated with metabolic risk factors,at baseline and at two-year follow-up: The prospective Cyprus Metabolism Study

Objective

To comparatively evaluate traditional liver tests and fetuin A as predictors of cardiometabolic risk, we studied associations between serum alanine transaminase (ALT), γ-glutamyl transferase (GGT), aspartate aminotransferase (AST) and fetuin-A and anthropometric, metabolic, and cardiovascular parameters cross-sectionally at baseline, and prospectively, after 2-years of follow-up.

Research Design and Methods

616 randomly enrolled young healthy participants in the Cyprus Metabolism Study, including all 93 subjects who participated in the follow-up study 2 years after baseline assessment, were included in this study.

Results

In the cross-sectional study, serum ALT and GGT were strongly correlated with anthropometric, cardiovascular, and metabolic variables, while serum AST was only correlated with waist circumference and waist-to-hip ratio. Fetuin-A was correlated with anthropometric variables, systolic blood pressure (SBP), insulin, and homeostasis model of assessment-insulin resistance (HOMA-IR) in the unadjusted model. In the fully adjusted model, both serum ALT and GGT levels remained positively correlated with total and low-density lipoprotein (LDL) cholesterol. GGT levels also remained correlated with triglycerides. ALT levels remained strongly positively correlated with insulin (r = 0.17, p < .0001) and HOMA-IR (r = 0.16, p = 0.0001). Serum fetuin-A levels were no longer significantly correlated with any variables.Prospectively, ALT and GGT were predictors of anthropometric variables and LDL cholesterol, while baseline levels of AST and fetuin-A were not predictors of any variables at 2-year follow-up.

Conclusions

We confirmed associations of ALT and GGT levels but failed to demonstrate an independent association between fetuin-A and cardiometabolic risk factors in young healthy men. Traditional liver tests (LFTs) are thus better than fetuin-A predictors of metabolic risk factors cross-sectionally and prospectively in young healthy adults.