Is Level of Serum Ischemia-Modified Albumin A Useful Biomarker in Carbon Monoxide Poisoning Cases?

OBJECTIVES: Ischemia-modified albumin (IMA) is an emerging diagnostic biomarker for many ischemic conditions. The study was conducted to investigate whether there is a change in IMA levels in carbon monoxide poisoning and, if so, the clinical relevance of IMA levels. METHODS: This study was performed between October 2013 and April 2014 to compare levels of serum IMA drawn at the time of admission to the emergency department in 49 patients poisoned with carbon monoxide and 37 healthy controls. Serum IMA, blood carboxyhemoglobin, and lactate levels were analyzed. RESULTS: Ischemia-modified albumin levels of patients with carbon monoxide poisoning were higher than those of controls. In patient group, however, there was no correlation between serum IMA and carboxyhemoglobin levels (r = -0.244, P > 0.05), whereas a negative correlation was detected between serum IMA and lactate levels (r = -0.334, P < 0.05). After all, a positive correlation was present between carboxyhemoglobin and lactate levels (r = 0.399, P < 0.05). CONCLUSIONS: Results from this preliminary study suggest that IMA might have diagnostic value in carbon monoxide poisoning and may be a parameter to be used clinically together with carboxyhemoglobin levels in terms of reflecting tissue hypoxia. In addition, IMA may be a criterion, especially in delayed cases where carboxyhemoglobin level may be normal in deciding hyperbaric oxygen treatment. To clarify this issue, further studies with larger population are needed.

Molecular analysis of the AGXT gene in patients suspected with hyperoxaluria type 1 and three novel mutations from Turkey.

Primary hyperoxaluria type 1 (PH1) is a rare, autosomal recessive disease, caused by the defect of AGXT gene encoding hepatic peroxisomal alanine glyoxylateaminotransferase (AGT). This enzyme is responsible for the conversion of glyoxylate to glycine. The diagnosis of PH1 should be suspected in infants and children with nephrocalcinosis or nephrolithiasis. Early diagnosis and treatment is crucial in preventing disease progression to end stage kidney disease (ESKD). In this study, AGXT gene sequence analyses were performed in 82 patients who were clinically suspected (hyperoxaluria and nephrolithiasis or nephrocalcinosis with or without renal impairment) to have PH1. Disease causing mutations have been found in fifteen patients from thirteen families (18%). Novel mutations have been found (c.458T>A (p.L153X), c.733_734delAA (p.Lys245Valfs*11), c.52 C>T (p.L18F)) in three of 13 families. There were 3-year lag time between initial symptoms and the time of PH1 is suspected; additionally, 5.5-year lag time between initial symptoms and definitive diagnosis. Consanguinity was detected in 77% of the patients with mutation. After genetic diagnosis, one patient received combined kidney and liver transplantation. AGXT gene sequencing is now the choice of diagnosis of PH1 due to its non-invasive nature compared to liver enzyme assay. Early diagnosis and accurate treatment in PH1 is important for better patient outcomes.

Persistent metabolic acidosis and severe diarrhoea due to Artemisia absinthium poisoning.

Herbs have long been used in the treatment of various disorders in traditional medicine since ancient age. Artemisia absinthium, one of these herbs, has traditionally been used in different societies for antibiotic, antiparasitic, antifungal and antipyretic purposes. Here, we report a poisoning case of a 10-month-old male infant progressing with severe diarrhoea and persistent metabolic acidosis after ingesting home-prepared Artemisia absinthium extract which was given for the treatment of common cold.

The Effects of Gluten on Weight Gain, Hematological, Biochemical, and Various Endocrinological Parameters.

BACKGROUND/AIMS:  This study is aimed to compare the effects of nutrition which has been enriched with different amounts of gluten to gluten-free diets on weight gain, diabetogenic state, hematological, and biochemical parameters. MATERIALS AND METHODS:  A total of 40 newly weaned male Wistar albino rats used in the study were randomized into 4 different groups based on the gluten rations they were given. Following 12 weeks of diet they were killed and intracardiac blood samples were collected. Groups were identified as group 1 (n = 10): control group; normal rat ration containing wheat, group 2 (n = 10): gluten-free diet, group 3 (n = 10): ration containing medium level of gluten (normal rat diet+6% vital gluten) and group 4 (n = 10): ration containing high level of gluten (normal rat diet+12% vital gluten). RESULTS:  In groups 3 and 4, high-density lipoprotein was found to be higher than the other 2groups. However, when group 2 results were compared to the other groups; the highest T3, T4, creatinine and B12 levels and the lowest gluten-specific IgE level were observed. alanine aminotransferase and aspartate aminotransferase levels were found to be higher in group 1 compared to the other 3 groups. No statistically significant difference was detected between the groups in terms of other parameters. CONCLUSION:  This study provides evidence that a gluten-containing diet does not cause weight gain, has no diabetogenic effect, and also does not adversely affect general health in relation to hematological, biochemical, and various endocrinological parameters.

The spectrum of diseases causing fever of unknown origin in Turkey: a multicenter study.

OBJECTIVE: The purpose of this trial was to determine the spectrum of diseases with fever of unknown origin (FUO) in Turkey. METHODS: A prospective multicenter study of 154 patients with FUO in twelve Turkish tertiary-care hospitals was conducted. RESULTS: The mean age of the patients was 42+/-17 years (range 17-75). Fifty-three (34.4%) had infectious diseases (ID), 47 (30.5%) had non-infectious inflammatory diseases (NIID), 22 (14.3%) had malignant diseases (MD), and eight (5.2%) had miscellaneous diseases (Mi). In 24 (15.6%) of the cases, the reason for high fever could not be determined despite intensive efforts. The most common ID etiologies were tuberculosis (13.6%) and cytomegalovirus (CMV) infection (3.2%). Adult Still's disease was the most common NIID (13.6%) and hematological malignancy was the most common MD (7.8%). In patients with NIID, the mean duration of reaching a definite diagnosis (37+/-23 days) was significantly longer compared to the patients with ID (25+/-12 days) (p=0.007). In patients with MD, the mean duration of fever (51+/-35 days) was longer compared to patients with ID (37+/-38 days) (p=0.052). CONCLUSIONS: Although infection remains the most common cause of FUO, with the highest percentage for tuberculosis, non-infectious etiologies seem to have increased when compared with previous studies.

Treatment of mitochondrial neurogastrointestinal encephalomyopathy with dialysis.

OBJECTIVE: To study the effect of continuous ambulatory peritoneal dialysis on nucleoside levels and clinical course in a patient with mitochondrial neurogastrointestinal encephalomyopathy (MNGIE). Patient We studied a patient with genetically verified MNGIE, who prior to treatment had lost weight progressively, developed amenorrhea, vomited multiple times daily, and had abdominal pain. Intervention The patient was treated with peritoneal dialysis for 3 years, and the effect on symptoms and plasma concentrations of thymidine and deoxyuridine were monitored. RESULTS: Dialysis stopped vomiting and reduced abdominal pain, and the patient gained 5 kg in weight and started to menstruate again. Symptoms returned if dialysis was paused. Dialysis did not affect plasma nucleoside levels. CONCLUSIONS: This study shows an unambiguous clinical benefit of peritoneal dialysis on gastrointestinal symptoms in MNGIE. Dialysis did not affect nucleoside levels, indicating elevated thymidine and deoxyuridine levels are not solely responsible for the pathogenesis of MNGIE.

Effect of long-term Helicobacter pylori infection on growth of children: a cohort study.

BACKGROUND: The effects of Helicobacter pylori (H. pylori) infection on growth are a controversial issue. We investigated the effects of long-term H. pylori infection on height and weight in children. METHODS: A total of 200 children of 7-18 years old suffering from dyspeptic complaints were classified into two groups: H. pylori positive and negative groups, respectively. Whether the infection was impoved was followed up while performing urea breath test, and according to exposure time to the infection, the children were further divided into group 1 (≤1.5 months), group 2 (>1.5-≤6 months) and group 3 (>6 months). Antropometric measurements were obtained and repeated every six months. RESULTS: Mean growth velocity scores in the H. pylori positive and negative groups were 0.49±3.85 [95% confidence interval (CI): -0.21-1.18] and 1.98±4.42 (95% CI: 1-2.96), respectively. The difference between both groups was statistically significant (P=0.012). Mean growth velocity scores in groups 1, 2 and 3 were 0.96±3.84, 0.16±4.51 and -0.85±3.09, respectively. Mean growth velocity scores of group 3 were significantly lower than those of groups 0 and 1 (P=0.005 and P=0.041). The mean weight scores in group 3 were similar to those in group 2, but the scores in group 3 were significantly lower than those in group 1 (-1.75±1.05, -1.21±1.37 and -0.88±1.49, respectively). CONCLUSION: As the duration of exposure is prolonged in children with H. pylori infection, the negative effect of the infection on both height and weight is evident.

Are obesity and metabolic syndrome associated with plasma adropin levels in children?

Studies performed on mice suggest that adropin is a peptide hormone playing a role in metabolic homeostasis and prevention of obesity-associated insulin resistance. Our study was conducted to investigate the role of adropin in children with obesity or metabolic syndrome. The study group consisted of 70 patients, including 42 obese and 28 with metabolic syndrome, and 26 healthy volunteers. After anthropometric variables and blood pressure of all participants were measured, serum lipids were analyzed, liver USG and oral glucose tolerance test were performed, and HOMA-IR values were calculated. Plasma adropin levels were collectively analyzed from collected plasma samples. In patient and control groups, no difference was observed in the levels of adropin (327.7±124.7 vs. 344.6±208.5 ng/L, respectively). The adropin levels of metabolic syndrome, obesity, and control groups also showed no difference (316±142.3, 335.8±112.5, and 344.6±208.5 ng/L, respectively). While the adropin levels of patients with and without hepatic steatosis were 319.6±123.7 and 347.8±128.7 ng/L, respectively, patients with HOMA-IR values of <3.16 and ≥3.16 had levels 342.3±124.8 and 296.5±136.7 ng/L, respectively. Although statistically insignificant, our findings are considered to support the hypothesis suggesting a nexus between adropin and obesity and metabolic syndrome. Small sample size in our study may have prevented our results to reach a more significant level. So, long-term follow-up studies with large population are needed to enlighten the role of adropin in metabolic homeostasis.

A case of May-Thurner syndrome with inconsistent radiological and surgical findings.

May-Thurner syndrome is the result of compression of the left common iliac vein between the right common iliac artery and the overlying vertebrae. In this case report, we describe an 11-year-old boy presenting with swelling of the left lower extremity. An iliac MR venography showed compression of the left proximal iliac vein between the vertebra and the left iliac artery. In surgery, it was seen that the left common iliac vein was connected to the postero-inferior part of the inferior vena cava, and it was compressed between the right common iliac artery and the columna vertebralis, which was inconsistent with the radiological findings. An interposition of the great saphenous vein graft between the left common iliac vein and the inferior vena cava was made, with a successful outcome. Our case is interesting in that it showed inconsistent findings between the radiological images and surgery.

The effects of gemfibrozil on hyperlipidemia in children with persistent nephrotic syndrome.

Persistent nephrotic syndrome is frequently accompanied by severe hyperlipidemia, and this may pose a substantial risk for cardiovascular disease. Lipid-lowering drugs are prescribed by many nephrologists for adult patients but rarely for nephrotic children. The present investigation was designed to evaluate the safety and efficacy of gemfibrozil in nephrotic children. Eight girls and four boys aged from 5 to 17 years were enrolled in this study. They were all steroid and immunosuppressive resistant patients with nephrotic range proteinuria. Placebo was administered to five patients and gemfibrozil was administered to seven patients for four months. Blood samples were taken for the determination of cholesterol, triglyceride, low-density lipoprotein (LDL), high-density lipoprotein (HDL), BUN, serum creatinine (Scr), ALT, AST, CPK, apolipoprotein A (apo A), apoliporotein B (apo B), and serum albumin levels during the initial and subsequent examinations. At the end of the fourth month, gemfibrozil reduced total cholesterol by 34%, LDL by 30%, apo B by 21% and triglycerides by 53% (p < 0.05). HDL cholesterol and apo A levels were not significantly altered. Renal function and urine protein excretion were not affected by gemfibrozil. In this study gemfibrozil therapy had no side effects and had favorable effects on the lipoprotein profile of nephrotic patients.

A case of homozygous familial hypercholesterolemia with focal segmental glomerulosclerosis.

Familial hypercholesterolemia (FH) is a common autosomal dominant inherited disorder characterized by increased levels of circulating plasma low-density lipoprotein cholesterol (LDL-C), tendon xanthomas, and premature atherosclerotic cardiovascular disease. Homozygous FH occurs in only one in a million people. Focal segmental glomerulosclerosis (FSGS) is clinically characterized by proteinuria, which is marked in the majority of cases and accompanied by nephrotic syndrome, high incidence of hypertension, and progression to renal failure. To our knowledge, we herein report for the first time a case of homozygous FH associated with FSGS. A seven-and-a-half-year-old boy was referred to our hospital due to cutaneous xanthomata and growth retardation. He had multiple nodular yellowish cutaneous xanthomatous lesions each 1 cm in size over his knees and sacral region. Laboratory data included cholesterol level of 1,050 mg/dl, low density lipoprotein cholesterol (LDL-C) 951 mg/dl, high-density lipoprotein cholesterol (HDL-C) 29 mg/dl, triglycerides 168 mg/dl, total protein 6.3 g/dl, and albumin 3.2 g/dl. Urinary protein excretion was 78 mg/m(2) per hour. A percutaneous renal biopsy was performed, and histological findings showed FSGS. Treatment with cholestyramine and atorvastatin was unsuccessful in terms of lowering lipids, and he was placed on weekly sessions of plasmapheresis. Total cholesterol was reduced from 1,050 mg/dl to 223 mg/dl, LDL-C from 951 mg/dl to 171 mg/dl, and urinary protein excretion from 78 mg/m(2) per hour to 42 mg/m(2) per hour after eight sessions of plasmapheresis. It is our belief that plasmapheresis is a treatment of choice in patients with FSGS associated with FH.

Peripheral gangrene complicating hemolytic uremic syndrome in a child.

A patient with hemolytic uremic syndrome (HUS) developed peripheral gangrene involving all fingers and toes. There was no history of bloody diarrhea. Hypocomplementemia was present, with a serum C3 concentration of 41 mg/dl. Acute renal failure was treated with peritoneal dialysis for 4 months. He received daily fresh-frozen plasma infusions and plasmapheresis on alternate days for ten sessions, followed by once-weekly sessions. He was anuric for 9 weeks. All medial and distal phalanxes became necrotic and were removed surgically. The renal biopsy findings were consistent with HUS. This is the second report of peripheral gangrene during the course of HUS in childhood.