Daily pattern and rates of whole-body tyrosine oxidation and phenylalanine hydroxylation were determined in young adults (15 men, 1 woman) receiving [13C]tyrosine and [(2)H2]phenylalanine via primed, constant oral infusion and [(2)H4]tyrosine by vein (five subjects also received [(2)H3]leucine simultaneously by vein) continuously for 24 h (12 h fast then 12 ...

Epidemiological data, the appropriateness of the medical approach, the effectiveness of unrestricted diet and the subsequent course of chronic non-specific diarrhoea (CNSD) were evaluated in 20 children, mean age at diagnosis 4.7 y, and mean duration of diarrhoea 24 months. A mean of 2.4 previous hospital admissions and a mean ...

OBJECTIVE: To provide a review of the mechanism of action, clinical efficacy, adverse effects, drug interactions, and therapeutic considerations associated with the use of a ketogenic diet to manage patients with intractable seizures. DATA SOURCES: A MEDLINE search from January 1966 to the present and relevant articles from journals were ...

The ketogenic diet is a high-fat, low-carbohydrate diet that results in ketosis. It has been in use for nearly 70 years. Several modifications of the diet's original form, including the medium-chain triglyceride (MCT) diet, have been made in an attempt to overcome the obstacles of compliance and acceptance, which have ...

The authors present more than 20 years' experience with coeliac disease, with a summary of their published studies. Hair shaft characteristics were determined by scanning electron microscopy. Hair diameter was significantly lower and cuticular erosion scores higher in those who were not on gluten-free diets as compared to controls, showing ...

The phenylalanine-free diet is needed for the treatment of phenylketonuria. Phenylketonuria is an inherited metabolic condition in which there is a deficiency of the enzyme phenylalanine hydroxylase. Lack of this enzyme means the body cannot metabolise the essential amino acid phenylalanine, which then builds up in the blood and causes ...

Transthyretin (TTR), a well-established nutritional marker, was measured in patients with active and inactive celiac disease (CD) in order to assess its value as a marker of disease activity. CD activity was assessed by intestinal biopsy to determine intestinal atrophy histologically and by measuring the level of antiendomysial antibody titer. ...

The aim of our study was to determine whether the combination of an anticholinergic treatment with a beta 2-adrenergic medication is a more effective treatment for acute asthma attack than the two treatments individually. The association of salbutamol-ipratropium was compared to treatment with salbutamol and ipratropium alone. It was a ...

A group of 26 children (13 boys and 13 girls; average age 12.2 years) with CD who had been on a gluten free diet for 5-15 years was examined in order to find out how effectively they could manage their diet. The diagnosis of CD was established on the basis ...

BACKGROUND: People with gluten sensitivity should avoid foods containing wheat, rye, and barley, but there has been debate about whether they should avoid oats. Although patients with celiac disease have recently been shown to tolerate oats, less is known about the effects of oats on patients with dermatitis herpetiformis. METHODS: ...

Celiac disease is an intolerance to protein fractions in wheat, rye, barley, and possibly oats. When these grains are consumed by a person with celiac disease, they damage the mucosa of the small intestine, which eventually leads to malabsorption of nutrients. Patients are therefore advised to remove these grains from ...

We report a 2-year-old boy with phosphofructokinase deficiency presenting in the newborn period with congenital arthrogryposis and severe myopathy, who has had significant improvement on a ketogenic diet since its institution at 4 months of age. We provide a rationale for use of this treatment and hypothesize it may be ...

Inborn errors of the pyruvate dehydrogenase complex (PDC) are associated with lactic acidosis, neuroanatomic defects, developmental delay, and early death. PDC deficiency is a clinically heterogeneous disorder, with most mutations located in the coding region of the X-linked alpha subunit of the first catalytic component, pyruvate dehydrogenase (E1). Treatment of ...

A 33-year-old patient, with pre-existing diagnosis of 'schizophrenic' disorder, came to our observation for severe diarrhoea and weight loss. Use of single photon emission computed tomography, (99mTc)HMPAO SPECT, demonstrated hypoperfusion of the left frontal brain area, without evidence of structural cerebral abnormalities. Jejunal biopsy showed villous atrophy. Antiendomysial antibodies were ...

The ketogenic diet is a high-fat diet that maintains the body's starvation mechanism, with exogenous fat provided for metabolism in lieu of stored fat. Mild dehydration is important to prevent dilution of the level of ketones in circulation at any given time. It is not known why or how ketosis ...

The ketogenic diet is becoming a more recognized method of treating seizures in some children with epilepsy. Texas Scottish Rite Hospital for Children (TSRHC) developed an interdisciplinary team that uses a comprehensive approach to implementing this innovative therapy. Anticipated outcomes of this unique approach are increased family satisfaction and improved ...

BACKGROUND/AIMS: Compliance to a gluten-free diet is currently an important issue in the care of both adolescent and adult coeliac patients. The aim of this study was evaluate the compliance to a gluten-free diet and the dietary habits in general of coeliac teenagers and young adults. PATIENTS AND METHODS: A ...

OBJECTIVES: The aim of the study was to establish whether body composition in patients with celiac disease is normal and influenced by the age at diagnosis or by the duration of the gluten free diet. METHODS: A group of 66 children with celiac disease and 76 healthy controls were studied ...

PURPOSE: Despite use of the ketogenic diet (KD) for >75 years its effectiveness or mechanism of action has been examined in few animal studies. Using the kindling model of epilepsy, we tested the anticonvulsant effectiveness and behavioral consequences of an experimental KD in adult rats. METHODS: Rats fully kindled from ...

We report hyperamylasemia due to macroamylasemia in a 33-year-old-woman with gluten enteropathy. Macroamylasemia was demonstrated by precipitation of 97% of amylase activity with PEG 6000. It was associated with increased serum IgA, with elevated values of specific IgA antibodies against alpha-gliadin and with a high titre of IgA anti-endomysium antibodies. ...

Seventy-two adults with phenylketonuria were evaluated to investigate the genotypic relationship to phenotype. Patient data were collected by chart review and medical follow-up as well as current psychological evaluation. Nineteen diagnosed neonatally had remained on a phenylalanine-restricted diet all their lives, whereas 34 who were also diagnosed on newborn screening ...

The water-based paints now frequently used for house painting still contain small amounts of volatile organic compounds (VOCs), with the potential to exacerbate symptoms of asthma. Because of these potential problems and environmental concerns, some manufacturers have produced paints with no VOC content. We wished to compare the effects on ...

This study investigated whether gluten-sensitive enteropathy (GSE) in Irish setter dogs was associated with underlying structural abnormalities of microvillar membrane proteins. Jejunal biopsies taken from eight-month-old GSE-affected dogs reared on a normal, gluten-containing diet exhibited partial villous atrophy and contained more intra-epithelial lymphocytes than controls. The morphological abnormalities were reversed ...

The daily rates of whole-body phenylalanine oxidation (phe-ox) and hydroxylation (phe-OH) were determined in young men (n = 10) receiving [13C]phenylalanine and [2H2]tyrosine via primed constant oral infusion (four also received simultaneously [2H4]tyrosine and [2H3]leucine via primed constant intravenous infusions) continuously for 24 h (first 12 h fast and then ...

There has been a dramatic resurgence of interest in the ketogenic diet during the past several years. For many children with difficult-to-control epilepsy, the diet presents an alternative approach to trying multiple medications. The ketogenic diet's current success rate, when properly executed, greatly exceeds that of the medications which have ...

A 10-year-old girl with yellow dystrophic nails, bronchiectasis, chronic sinusitis and lower-limb lymphedema is presented. The underlying mechanism remains unknown although it has been postulated to be associated with lymphatic abnormalities. To date no causative treatment exists. Our patient was treated with conservative management, including a low-fat diet supplemented with ...

Failure to thrive is common in children with celiac disease. As alterations in the growth hormone-insulin-like growth factor I (GH-IGF-I) growth axis have been reported in these patients, we studied the behavior of growth hormone-binding proteins (GH-BPs I and II), IGF-I and its binding proteins in 14 children with celiac ...

Forty-three adults with classical phenylketonuria were identified by neonatal screening and treated with a phenylalanine (Phe) restricted diet. Nineteen have remained on dietary treatment with varying levels of blood Phe control and 24 have discontinued the diet at an average age of 7.8 years. Follow up at an average age ...

In our clinic the decision on whether to continue with dietary treatment of phenylketonuria or not is left to each adolescent and adult patient after the advantages and disadvantages, as discussed in this paper, of continuing diet have been presented to them. As a result 61 of 132 patients have ...

Early diagnosis and treatment of phenylketonuria (PKU) in Poland was started in 1965, initially on a voluntary and then on a obligatory basis. Guthrie tests have been used for newborn screening. For confirmation of diagnosis changing with time methods of blood phenylalanine (Phe) and tyrosine estimation were used. In addition, ...

Gluten-sensitive enteropathy is induced by dietary wheat gliadin and related proteins in genetically susceptible individuals. Most evidence suggests that the mucosal lesion represents an immunologically mediated injury triggered by gluten in the context of a particular assortment of major histocompatibility complex genes. The amino acid residues of gliadin and related ...

The daily rates of whole-body phenylalanine oxidation and hydroxylation were determined in young men receiving [1-13C]phenylalanine and [2H2]tyrosine via primed, constant intravenous (n=3) or oral (n=5) infusion for 24 consecutive hours (12-h fast followed by 12-h fed period), and given a generous phenylalanine (100 mg.kg-1.d-1), tyrosine-free, but otherwise adequate L-amino ...

To evaluate the effects of a gluten free diet on bone mineral density in untreated adult patients with coeliac disease, 63 patients (17-79 years, 35 women) were examined at diagnosis and after one year taking a gluten free diet. Bone mineral density was measured in the forearm using single photo ...

Ketosis is beneficial for seizure control, possibly through induction of cerebral acidosis. However, cerebral intracellular pH has not previously been measured in ketotic humans and the animal data are sparse. We describe a high-fat diet, avidly consumed by rats, that induced consistent and moderate ketosis. Adult male rats were fed ...

Bromide treatment was successful in controlling seizures in an 11-year-old Dachshund with epilepsy and presumptive phenobarbital-associated hepatopathy. Because bromide does not induce liver enzyme activity and does not seem to be hepatotoxic, it can be used to control seizures in dogs with concurrent epilepsy and hepatic disease. In this dog, ...

An experimental investigation has lately shown that certain allyl compounds of garlic are able to provoke acantholysis in normal human skin cultured in vitro. The acantholytic effect has been more prominent in the samples from DR4+ donor. We here report a case of superficial pemphigus which appeared spontaneously in a ...

If phenylketonuria (PKU) is not treated from an early age with a reduced phenylalanine diet, irreversible brain damage may occur. Although dietary intervention can do nothing to alter this impairment once it occurs, there is some evidence to suggest that a low phenylalanine diet may help to reduce the level ...

OBJECTIVE: To determine whether ketogenic weight reducing diets have adverse effects on cognitive performance. SUBJECTS: 21 overweight women (mean BMI = 41 kg/m2). DESIGN: Randomized double-blinded study. METHOD: Subjects were randomized to ketogenic or nonketogenic liquid formula very low energy diets, that were comparable in energy and in protein content. ...

Severe mental handicap in phenylketonuria (PKU) can be prevented if dietary treatment is implemented at birth. Controversy remains about the optimum age for terminating treatment. A group of adolescents and young adults with PKU from the West of Scotland Register was identified which had received early treatment, been well-controlled on ...

Eighty five children (median age 2.3 years, range 0.3 to 13.3 years) with refractory atopic dermatitis affecting more than 12% of the body surface area, were randomly allocated to receive a few foods diet (eliminating all but five to eight foods) supplemented with either a whey hydrolysate (n = 27) ...

A 47-year-old man with a flat jejunal mucosa complicated by malabsorption, diarrhoea and lymphocytic colitis is presented. There was no response to gluten withdrawal alone, combination of a gluten-free diet and prednisone therapy, or total parenteral nutrition. Complete clinical remission was only achieved after simultaneous treatment with cyclosporine and a ...

Adolescents with celiac disease often fail to adhere to a strict gluten-free diet. The value of endomysial antibodies in assessing the dietary compliance of such adolescents has been assessed in 23 patients divided into four groups according to their daily gluten intake. Serum endomysial antibodies were absent in all subjects ...

Celiac disease is characterized by a gluten-induced villous atrophy of the upper small intestine which has an active role in the lipoprotein metabolism. In the present study the lipoprotein profiles of different patients were analyzed to determine the effect of impaired enterocyte function in celiac disease. We compared serum lipid ...

We determined the daily rates of whole-body phenylalanine oxidation (phe-Ox) and hydroxylation (phe-OH) in young men receiving [1-13C]phenylalanine and [2H2]tyrosine via primed, constant intravenous (n = 5) or oral (n = 7) infusions for a consecutive 24 h (12-h fast followed by 12-h fed period), and given a low-phenylalanine (21.9 ...

Five hundred ninety two children (6.0%) were diagnosed as persistent diarrhea (PD) out of a total attendance of 9795 cases in the Diarrhea Training and Treatment Unit (DTU) over a period of 1 year. Most of the cases were initially managed as outpatients on dietary advice and treatment of associated ...

The ketogenic diet, a treatment for intractable epilepsy, is rarely initiated because it requires strict compliance with a diet that is perceived to be unpalatable. In a case study of a 15-year-old girl with uncontrolled epilepsy, we used a stimulus-choice procedure to assess relative preferences of 33 foods from the ...

Nineteen early-treated phenylketonuric patients, whose diet was discontinued between 4.5 and 13 years of age, and who have been off the diet for 12-28 years, were reassessed in 1992-93. There was little change in mean IQ between end of diet and follow-up, less than one IQ point on the average, ...

Gluten-free diets have been used in the treatment of patients with dermatitis herpetiformis in our department since 1967. Of the 212 patients with dermatitis herpetiformis attending between 1967 and 1992, 133 managed to take the diet, and 78 of these achieved complete control of their rash by diet alone. Of ...

To investigate body protein turnover and the pathogenesis of increased concentration of plasma phenylalanine in liver cirrhosis, we have studied phenylalanine and leucine kinetics in cirrhotic (diabetic and nondiabetic) patients, and in normal subjects, both in the postabsorptive state and during a mixed meal, using combined intravenous and oral isotope ...

The age at which children suffering from classical phenylketonuria can safely discontinue their dietary therapy has been constantly disputed over the past decades. Recently, most phenylketonuria centers have begun to recommend a life-long diet, especially for female patients. Male patients are also advised to continue their diet until at least ...