Nutrient Intake Differs Among Persons With Celiac Disease and Gluten-Related Disorders in the United States

Persons with celiac disease (CD) may develop nutritional deficiencies, while individuals following a gluten-free diet (GFD) may lack essential nutrients. We examined nutrient intake from diet and supplements among persons with CD and GFD in the cross-sectional National Health and Nutrition Examination Survey, 2009-2014. Among 15,610 participants 20 years and older, we identified CD based on positive serology for immunoglobulin A against tissue transglutaminase, health care provider diagnosis, and adherence to a GFD. People without CD avoiding gluten (PWAG) adhered to a GFD without a diagnosis of CD. Two 24-h recalls assessed nutrient intake from diet and supplements. Compared to participants without CD or PWAG, persons with diagnosed CD had lower intake of total energy, carbohydrates, fat, and saturated and monounsaturated fatty acids. In contrast, persons with undiagnosed CD and positive serology had higher intake of those nutrients, sugar, and protein. Total carbohydrate and sugar intake was lower among PWAG. Persons with diagnosed CD had higher vitamin A and E intake, while those with undiagnosed CD had increased intake of calcium, phosphorus, magnesium, iron, zinc, copper, sodium, potassium, vitamin A, alpha-carotene, folic acid, and choline. Higher micronutrient intake with undiagnosed CD was observed more at high latitudes. PWAG had higher beta-carotene and lutein/zeaxanthin and lower folic acid intake. In the U.S. population over a 6-year period, total energy and macronutrient intake was decreased among persons with diagnosed CD, while intake of total energy, macronutrients, and multiple micronutrients was increased among persons with undiagnosed CD. Nutriomics studies of multiple analytes measured simultaneously across affected persons and populations are needed to inform screening for malabsorption and treatment strategies

Miopatia nemalinowa u noworodka – opis rzadkiej choroby

Nemaline myopathy (NM) is a genetically and clinically heterogeneous muscle disorder, defined by the presence of characteristic nemaline bodies on muscle biopsy. The disease has a wide spectrum of phenotypes, ranging from forms with neonatal onset and fatal outcome to asymptomatic forms. The neonatal form is severe and usually fatal. The clinical variability, with differing age of onset and severity of symptoms makes the diagnosis difficult during infancy. There is no curative treatment. L-tyrosine may prevent aspiration by reducing pharyngeal secretions and drooling. Most of the patients die from respiratory and cardiac failure. This article discusses a newborn infant who presented with generalized weakness and respiratory failure. Partial response to L-tyrosine treatment was noted. The case is worth presenting to remind clinicians of congenital myopathies in the differential diagnosis of floppy infant during neonatal period and to emphasize the importance of muscle biopsy in diagnosis.Miopatia nemalinowa jest schorzeniem mięśni, niejednorodnym pod względem genetycznym i klinicznym. Chorobę cechuje obecność charakterystycznych struktur nemalino-wych w bioptacie mięśnia. Fenotyp jest bardzo zróżnicowany i obejmuje zarówno postacie noworodkowe prowadzące do zgonu, jak i postacie bezobjawowe. Postać noworodkowa przebiega ciężko i zwykle kończy się śmiercią. Zmienność kliniczna, łącznie ze zróżnicowanym wiekiem w chwili wystąpienia objawów i z różnym ich nasileniem, może utrudniać rozpoznanie w wieku niemowlęcym. Choroba jest nieuleczalna. Podawanie L-tyrozyny może zapobiec zachłyśnięciu poprzez zmniejszenie produkcji wydzieliny w gardle i śliny. Większość chorych umiera z powodu niewydolności oddechowej i krążenia. W artykule omówiono przypadek noworodka z uogólnionym niedowładem i niewydolnością oddechową. Reakcja na podawanie L-tyrozyny była częściowa. Przedstawiony opis przypadku ma na celu przypomnienie klinicystom o miopatiach wrodzonych, które należy uwzględniać w rozpoznaniu różnicowym zespołu wiotkiego dziecka w okresie noworodkowym, oraz podkreślenie znaczenia biopsji mięśnia w ustalaniu rozpoznania

Urgent Abdominal Re-Explorations

BACKGROUND: Treatment of a number of complications that occur after abdominal surgeries may require that Urgent Abdominal Re-explorations (UARs), the life-saving and obligatory operations, are performed. The objectives of this study were to evaluate the reasons for performing UARs, outcomes of relaparotomies (RLs) and factors that affect mortality. METHODS: Demographic characteristics; initial diagnoses; information from and complications of the first surgery received; durations and outcomes of UAR(s) performed in patients who received early RLs because of complicated abdominal surgeries in our clinic between 01.01.2000 and 31.12.2004 were investigated retrospectively. Statistical analyses were done using the chi-square and Fisher exact tests. RESULTS: Early UAR was performed in 81 out of 4410 cases (1.8%). Average patient age was 50.46 (13–81) years with a male-to-female ratio of 60/21. Fifty one (62.96%) patients had infection, 41 (50.61%) of them had an accompanying serious disease, 24 (29.62%) of them had various tumors and 57 (70.37%) patients were operated under emergency conditions during first operation. Causes of urgent abdominal re-explorations were as follows: leakage from intestinal repair site or from anostomosis (n:34; 41.97%); hemorrhage (n:15; 18.51%); intestinal perforation (n:8; 9.87%); intraabdominal infection or abscess (n:8; 9.87%); progressive intestinal necrosis (n:7; 8.64%); stomal complications (n:5; 6.17%); and postoperative ileus (n:4; 4.93%). Two or more UARs were performed in 18 (22.22%) cases, and overall mortality was 34.97% (n:30). Interval between the first laparotomy and UAR averaged as 6.95 (1–20) days, and average hospitalization period was 27.1 (3–78) days. Mortality rate was found to be higher among the patients who received multiple UARs. The most common (55.5%) cause of mortality was sepsis/multiple organ failure (MOF). The rates for common mortality and sepsis/MOF-dependent mortality that occured following UAR were significantly higher in patients who received GIS surgery than in those who received other types of surgeries (p:0.000 and 0.010, respectively). CONCLUSION: UARs that are performed following complicated abdominal surgeries have high mortality rates. In particular, UARs have higher mortality rates following GIS surgeries or when infectious complications occur. The possibility of efficiently lowering these high rates depends on the success of the first operations that the patient had received

Histologic Abnormalities in Children with Nonalcoholic Fatty Liver Disease and Normal or Mildly Elevated Alanine Aminotransferase Levels.

Objectives: To investigate the histological spectrum of nonalcoholic fatty liver disease (NAFLD) in children with normal, mildly elevated (26–50 U/L boys, 23–44 U/L girls), or elevated (> 50 boys, > 44 girls) serum alanine aminotransferase (ALT) levels. Study design: The Nonalcoholic Steatohepatitis Clinical Research Network (NASH CRN) enrolls children 5–18 years with NAFLD. We analyzed baseline clinical and histological data from 91 children with suspected NAFLD and normal or mildly elevated ALT and liver biopsy within 180 days of ALT, and compared them with 392 children with elevated ALT. Results: Of 91 children, 17 (19%) had normal and 74 (81%) had mildly elevated ALT levels. Overall, 45% of biopsies had ≥ 33% steatosis, lobular inflammation grade was ≥ 2 in 22%, 81% had portal inflammation, 29% had ballooned hepatocytes, 35% had “suspicious/borderline” steatohepatitis, and 8% had definite NASH, 34% had NAFLD activity score (NAS) ≥ 4. Overall, 46% had fibrosis (38% mild/moderate and 8% bridging/cirrhosis). Marked steatosis (50% vs 24%) and fibrosis (54% vs 12%) were significantly more common in mildly elevated vs normal, with no difference in ballooning, inflammation, or NAS ≥ 4. Fibrosis stage 3/4 was seen in none of the children with normal ALT, and in 9% of the mildly elevated and 15% of the elevated. Conclusions: Liver biopsies of children with NAFLD with normal or mildly elevated ALT levels show significant histologic abnormalities, including advanced fibrosis in children with mildly elevated ALT. ALT thus may underestimate liver injury in NAFLD. Appropriate ALT cut-off levels can help identify children at risk for more severe disease

Success of microvascular surgery; repair mesenteric injury and prevent short bowel syndrome: a case report

This is an Open Access article distributed under the terms of the Creative Commons Attribution Licens

Lack of Association of Childhood Partial Epilepsy with Brain Derived Neurotrophic Factor Gene

Brain-derived factor (BDNF) is a member of neurotrophin family and is localized and upregulated in areas implicated in epileptogenesis. Several lines of evidence make the BDNF gene a plausible candidate gene for predisposition to epilepsy. In this study, we tested that BDNF might be involved in the etiology of childhood PE. To assess whether BDNF gene C270T polimorphism could be implicated in vulnerability to PE, we conducted a case-control association analysis (112 partial epileptic and 100 controls) in Turkish children. Epileptic children were divided into two groups: 1—idiopathic (n=85) and 2—symptomathic epilepsy (n=27). There was no significant difference in genotypic distribution and allelic frequencies of the BDNF gene C270T polimorphism between the PE and control groups. However, the BDNF gene TT genotype was more frequently seen in the epileptic children (15 versus 11 patients, resp.). Interestingly, in the epilepsy group, both two children with TT genotype have posttraumatic epilepsy. The data indicate a possible association with the 270T genotype of the BDNF gene with a posttraumatic epilepsy. To draw any conclusion, further studies using larger sample sizes should be carried out in various ethnic populations in childhood epilepsies

Fournier's gangrene in a patient after third-degree burns: a case report

<p>Abstract</p> <p>Introduction</p> <p>Fournier's gangrene is characterized by tissue ischemia leading to rapidly progressing necrotizing fasciitis.</p> <p>Case presentation</p> <p>We present the case of a patient with Fournier's gangrene after third-degree burns. Clinical manifestations, laboratory results and treatment options are discussed.</p> <p>Conclusion</p> <p>Fournier's gangrene is a surgical emergency. Although it can be lethal, it is still a challenging situation in the field of surgical infections.</p

Factors related to abdominal pain in gastroparesis: contrast to patients with predominant nausea and vomiting

Background Factors associated with abdominal pain in gastroparesis are incompletely evaluated and comparisons of pain vs other symptoms are limited. This study related pain to clinical factors in gastroparesis and contrasted pain/discomfort‐ with nausea/vomiting‐predominant disease. Methods Clinical and scintigraphy data were compared in 393 patients from seven centers of the NIDDK Gastroparesis Clinical Research Consortium with moderate‐severe (Patient Assessment of Upper Gastrointestinal Disorders Symptoms [ PAGI ‐ SYM ] score ≥3) vs none‐mild ( PAGI ‐ SYM  < 3) upper abdominal pain and predominant pain/discomfort vs nausea/vomiting. Key Results Upper abdominal pain was moderate‐severe in 261 (66%). Pain/discomfort was predominant in 81 (21%); nausea/vomiting was predominant in 172 (44%). Moderate‐severe pain was more prevalent with idiopathic gastroparesis and with lack of infectious prodrome (P ≤ 0.05) and correlated with scores for nausea/vomiting, bloating, lower abdominal pain/discomfort, bowel disturbances, and opiate and antiemetic use (P < 0.05), but not gastric emptying or diabetic neuropathy or control. Gastroparesis severity, quality of life, and depression and anxiety were worse with moderate‐severe pain (P ≤ 0.008). Factors associated with moderate‐severe pain were similar in diabetic and idiopathic gastroparesis. Compared to predominant nausea/vomiting, predominant pain/discomfort was associated with impaired quality of life, greater opiate, and less antiemetic use (P < 0.01), but similar severity and gastric retention. Conclusions & Inferences Moderate‐severe abdominal pain is prevalent in gastroparesis, impairs quality of life, and is associated with idiopathic etiology, lack of infectious prodrome, and opiate use. Pain is predominant in one fifth of gastroparetics. Predominant pain has at least as great an impact on disease severity and quality of life as predominant nausea/vomiting.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/97520/1/nmo12091.pd

Pioglitazone, Vitamin E, or Placebo for Nonalcoholic Steatohepatitis

Background Nonalcoholic steatohepatitis is a common liver disease that can progress to cirrhosis. Currently, there is no established treatment for this disease. Methods We randomly assigned 247 adults with nonalcoholic steatohepatitis and without diabetes to receive pioglitazone at a dose of 30 mg daily (80 subjects), vitamin E at a dose of 800 IU daily (84 subjects), or placebo (83 subjects), for 96 weeks. The primary outcome was an improvement in histologic features of nonalcoholic steatohepatitis, as assessed with the use of a composite of standardized scores for steatosis, lobular inflammation, hepatocellular ballooning, and fibrosis. Given the two planned primary comparisons, P values of less than 0.025 were considered to indicate statistical significance. Results Vitamin E therapy, as compared with placebo, was associated with a significantly higher rate of improvement in nonalcoholic steatohepatitis (43% vs. 19%, P=0.001), but the difference in the rate of improvement with pioglitazone as compared with placebo was not significant (34% and 19%, respectively; P=0.04). Serum alanine and aspartate aminotransferase levels were reduced with vitamin E and with pioglitazone, as compared with placebo (P Conclusions Vitamin E was superior to placebo for the treatment of nonalcoholic steatohepatitis in adults without diabetes. There was no benefit of pioglitazone over placebo for the primary outcome; however, significant benefits of pioglitazone were observed for some of the secondary outcomes. (ClinicalTrials.gov number, NCT00063622.