The use of follow up studies was evaluated in 128 patients with coeliac disease during their first visit to a department for adults. The original diagnosis had been made in childhood in all patients. Fifty eight (45%) of the subjects were following a gluten free diet, 23 (18%) were following ...

1. The present paper reports the effects of liposome-entrapped tyrosinase (EC 1.14.18.1. L-Tyrosine, L-3,4-dihydroxyphenylalanine: oxygen oxidoreductase) infusion on the catecholamine contents of rat plasma. The actions of liposomes and free tyrosinase have also been investigated. 2. From the experiments, evidence has been obtained that liposome-entrapped tyrosinase is able to affect ...

We studied the effect of electrical stimulation of centromedian thalamic nuclei (ESCM) on seizure control and paroxysmal EEG activity in 23 patients. We report the effect of chronic ESCM on psychological performance and background EEG activity of patients with various intractable seizure patterns. In each patient, a simple specifically designed ...

Small bowel biopsy in a 4-year-old girl with symptoms suggestive of coeliac disease revealed subtotal villous atrophy. The mucosa healed on a gluten-free diet. From the age of 7 years, the girl was challenged with gluten. Annual biopsies showed normal or nearly normal mucosa specimens. At 21 years of age, ...

IgA-class antiendomysium antibodies (IgA-EmAs) are a very sensitive and specific serological marker of celiac sprue. Using an indirect immunofluorescent method, we evaluated the kinetics of the antibody titers both during a gluten-free diet and after gluten was added, comparing them with the intestinal histological pattern. The IgA-EmA titers were evaluated ...

The diagnosis of food allergy was confirmed in 51 dogs while the responsiveness to a 60-day home-cooked restricted dietary trial (elimination-diet trial) was evaluated. The primary clinical sign of allergy detected and evaluated in all dogs was persistent and nonseasonally pruritic skin disease. The duration of time between starting the ...

Records of 46 patients with classic phenylketonuria (PKU) were used to determine treatment variables associated with intellectual outcome. Patients comprised three groups: phenylalanine-restricted diet started 1) after 3 months and loss of diet control at a mean age of 7 years, 2) before age 3 months and loss of diet ...

Fifty patients with a history of minor recurrent aphthous stomatitis were entered into a 6-month double-blind controlled trial of a gluten-free diet. After hematologic and biochemical investigations and jejunal biopsy, one half of the group was given a gluten-free diet; the other half served as controls and received a gluten-free ...

To study the supplementary effect of Spirulina, pregnant rats were fed 5 different kinds of diets (casein, Spirulina, wheat gluten, Spirulina + wheat gluten, Spirulina-without additional vitamins and minerals), each providing 22% protein during the period of pregnancy. The outcome of pregnancy was assessed from litter and dams' weight and ...

Human and experimental animal studies suggest a relationship between low Mn status and seizures. The genetically epilepsy prone rat (GEPR), which has low tissue Mn levels, was studied in the context of Mn supplementation. Manganese was provided at 45 micrograms/g diet (control) or 1000 micrograms/g diet (supplemented) to dams during ...

The ketogenic diet was developed in the 1920s as a treatment for intractable childhood seizures when few antiepileptic drugs (AEDs) were available. There are still children whose seizures are refractory even to modern therapy, but use of the ketogenic diet appears to be waning. At Johns Hopkins, we continue to ...

Three patients with severe recessive dystrophic epidermolysis bullosa were treated with oral phenytoin and palliative and supportive measures for variable periods. Their progress was compared with that of three milder cases managed only with palliative and supportive measures. The phenytoin-treated group showed marked decrease in blister count, increase in trauma ...

Plasma phenylalanine (Phe) and tyrosine (Tyr) turnover and the rate of conversion of phenylalanine to tyrosine (Phehyd) and of phenylalanine oxidation (Pheox) after reduced intakes of Phe and Tyr were determined in a metabolic study involving five healthy young adult men. In a pilot study, six postabsorptive young men received ...

Six normal subjects each ingested a single 12-oz can of a diet cola (Diet Coke) providing 184 mg aspartame (APM), of which 104 mg is phenylalanine (Phe), and, on another occasion, a single 12-oz can of regular cola (Coke Classic). Neither cola significantly affected plasma concentrations of Phe or tyrosine ...

Most women who began nutrition support as neonates for a diagnosis of phenylketonuria, an inherited defect in phenylalanine metabolism, are of normal intelligence, no longer require a restricted diet, and wish to have children of their own. Phenylketonuria that is untreated when a woman conceives and during gestation results in ...

A one-year-old boy suffering from intermittent lactic acidosis, muscular hypotonia, horizontal gaze paralysis and spasticity in both legs had low activity of the pyruvate dehydrogenase complex associated with low amounts of immunoreactive E 1 alpha and E 1 beta. Leigh syndrome was diagnosed on the basis of the clinical and ...

In normal subjects, ingestion of tyrosine or phenylalanine stimulates prolactin (PRL) secretion. In patients with phenylketonuria (PKU), we found normal PRL responses to phenylalanine, demonstrating that conversion of phenylalanine to tyrosine is not necessary for PRL stimulation. PKU patients also showed greater PRL responses to tyrosine during dietary phenylalanine restriction ...

Essentially all patients with dermatitis herpetiformis (DH) and dermal granular IgA deposits have a gluten-sensitive enteropathy as seen in coeliac disease. A gluten-free diet would normally restore mucosal morphology within months. The dapsone medication required to suppress the skin lesions could be gradually reduced and/or finally discontinued in most patients ...

If phenylketonuria is untreated in infancy, it causes irreversible brain damage. No dietary treatment can alter this brain damage. However, it has been shown that dietary treatment can reduce behaviour disturbances often associated with the condition. In this study, a subject's behaviour was monitored over 18 weeks while on a ...

Gluten sensitivity in a naturally occurring enteropathy of Irish setter dogs, and the effects of excluding dietary cereal from birth on the subsequent response to gluten challenge were investigated. Peroral jejunal biopsy specimens were obtained at 1 year of age for morphometric and biochemical examinations, and intestinal permeability was assessed ...

Celiac disease (CD) is characterized by diarrhea, growth retardation, and weight loss in genetically susceptible subjects on a gluten-containing diet. The exact pathogenesis of CD is still obscure, but it is considered to be immunologically mediated. We have previously shown elevated prostaglandin E2 (PGE2) and thromboxane B2 (TxB2) content in ...

Of 63 children with severe atopic dermatitis who were treated with a diet eliminating all but 6 foods for a 6-week period, 9 (14%) abandoned the diet, 21 (33%) completed the diet but did not benefit, and in 33 (52%) there was significant benefit. However, the outcome at 12 months ...

Chronic K depletion (KD) causes hypocitraturia. In the present studies, the effect of KD, induced by a low-K diet for 14 days (serum [K] 4.1 +- 0.1, control vs. 2.2 +/- 0.1 meq/l, KD, P less than 0.01), on renal cortical brush-border membrane (BBM) Na-citrate cotransporter activity was examined. KD ...

Elemental diets are reported to decrease activity of patients with dermatitis herpetiformis. We tested the hypothesis that gluten, given in addition to an elemental diet, is responsible for the intestinal abnormalities, cutaneous immunoreactant deposition, and skin disease activity in dermatitis herpetiformis. At entry eight patients with dermatitis herpetiformis, who were ...

A cohort of 123 patients with coeliac disease, diagnosed in the first three years of life and followed up for at least 10 years, was reevaluated during the teenage period in terms of compliance with the diet and clinical state. Mucosal structure and lymphocytes were assessed in small intestinal biopsy ...

Intestinal permeability was assessed in Irish setters with gluten-sensitive enteropathy by oral administration of an isotonic solution of cellobiose and mannitol, and measurement of their urinary excretion ratio. The cellobiose/mannitol ratio was increased in affected Irish setters fed a wheat-containing diet compared both with littermates reared on a cereal-free diet, ...

Intestinal permeability to 51Cr-EDTA was examined during the development of gluten sensitive enteropathy in dogs bred from affected Irish setters and reared on a normal wheat containing diet. Comparisons were made with litter mates reared on a gluten free diet and with a control group of age matched, clinically healthy ...

This study examined the acceptability ratings of three alternative treatments applied to case descriptions which varied in their level of problem severity. Evaluations were provided by 40 parents seeking services in a Pediatric Preventive Cardiology Clinic for treatment of their children's hypercholesterolemia. Results indicated that both diet and diet plus ...

Failure to thrive may not be a result of organ disease, physical abuse, or intentional neglect. We describe an infant who developed kwashiorkor with a high-fat, low-protein, nondairy coffee creamer diet. The elimination diet was administered on the advice of a family friend for a facial rash. The child presented ...

The plasma lipid levels were investigated in 45 celiac children under 3 years of age and 49 over this age at diagnosis (untreated groups), after gluten-free diet and in comparison with irritable bowel syndrome (IBS) groups matched for size, gender, and age. Total and HDL cholesterol levels were significantly lower ...

We report a case of classic phenylketonuria (PKU) treated by dietary restriction until the age of 5 years. At the age of 18 years, she developed anxious-depressive symptoms and deterioration of school performance. Neurophysiological investigations showed changes in VEPs and BAEPs and brain MRI showed white matter abnormalities. The return ...

A diet excluding wheat, oats, rye and barley is the cornerstone of the treatment of coeliac patients. Such a gluten-free diet (GFD) is recommended irrespective of the presence of symptoms. Maintenance of a strict GFD is not a simple matter, as small amounts of gluten capable of causing relapse have ...

Small intestinal morphology and function were assessed in 82 patients with dermatitis herpetiformis, 51 of whom were taking a normal diet and 31 a gluten free diet. Methods used were histopathological evaluation of jejunal mucosal biopsy specimens, quantitation of intraepithelial lymphocytes, cellobiose/mannitol permeability test, tissue disaccharidase values, serum antigliadin antibodies, ...

We report the case of a patient with pseudophakic cystoid macular edema whose visual acuity improved promptly with oral administration of 500 mg of acetazolamide extended-release capsules BID for two weeks. However, vision worsened again within two weeks of discontinuation of the treatment. On resumption of acetazolamide therapy for three ...

We describe agoraphobia as a complication of phenylketonuria (PKU) in young adults. The five patients have classic PKU and received phenylalanine-restricted diet only in childhood. Only one has normal intelligence. All but one were also depressed. All were anxious. Three of the five had initiated the phenylalanine-restricted diet after 3 ...

Intestinal permeability was assessed before and 1, 2, 4, 8 and 12 weeks after commencing a gluten-free diet (GFD) in eight coeliac subjects. Intestinal morphology was quantified in six coeliac subjects on a normal diet, six coeliac subjects on a GFD, and 21 normal subjects. T-cell activity was measured in ...

The serum levels of beta 2-microglobulins (beta 2-m) were studied in 65 celiac children. Significant statistical differences (p less than 0.05) were found between the values of patients on a gluten-containing diet (mean +/- SD, 1.92 +/- 0.64 mg/L) and those on a gluten-free diet for less than (mean +/- ...

7 patients with phenylketonuria who developed neurological disability in adolescence or early adult life are described. 4 had been diagnosed by routine neonatal screening and started a low phenylalanine diet in infancy. 3 were diagnosed in early childhood because of developmental delay, and then started dietary treatment. Dietary control deteriorated ...

Medium-chain triglycerides (MCT) are an important component of an enteral ketogenic diet for seizure control. Previously, it was difficult to maintain ketosis when parenteral (iv) nutrition therapy was necessary. The use of iv MCT in a 5-year-old girl with Lennox-Gastaut syndrome who had diarrhea and dehydration is reported. Conventional 20% ...

Phenylpropanolamine (PPA) is contained in about 106 products, over half of which are available over-the-counter (OTC). Most are cough/cold remedies; nine are OTC diet aids. More than nine million Americans were using OTC diet aids in 1981, making PPA the fifth most used drug in the United States, responsible for ...

Ten PKU subjects were treated with Product 196 for one year. Product 196 is a special phenylalanine-free dietary supplement consisting primarily of essential amino acids, carbohydrate, Vitamin C and some minerals, made by Scientific Hospital Supplies Limited, Liverpool, England. It is intended for persons with phenylketonuria who are not consuming ...

We surveyed phenylpropanolamine (PPA) use and overuse among 309 diet center clients. Fifty-one percent of all subjects surveyed reported using PPA drugs: 44 percent used cold medicines and 16 percent used diet aids. Twenty-two percent of diet aid users and 7 percent of cold medicine users reported that they deliberately ...

We describe a case of collagenous colitis in a young man with coeliac disease who had responded clinically and histologically to a gluten-free diet three years previously. The collagenous colitis responded initially to oral corticosteroid therapy and he is now asymptomatic (and with normal rectal mucosa) on oral mesalazine. Collagenous ...

In an uncontrolled study a gluten-free diet was given to 29 patients affected by primary IgA nephropathy (IgAGN). All of them followed the diet for 6 months, 23 patients for 1 year and 9 for 2 to 4 years. Mean levels of IgA containing circulating immune complexes (IgAIC), detected by ...

In 46 adult patients with coeliac disease, 41 (89%) of whom were positive for IgA and/or IgG antigliadin antibodies (AGA) when untreated, we investigated after a gluten-free diet the relationship between the persistence of AGA, the persistence of jejunal lesions, and the duration and compliance with the diet. IgG AGA ...

The outcome of 48 pregnancies in 18 women with elevated phenylalanine was studied. The women were divided into two groups, diet and non-diet. All the women on diet had severe hyperphenylalaninaemia and only six were on the amino acid mixture at conception. In the non-diet group four women had concentrations ...

Millions of newborn infants are screened for phenylketonuria (PKU) to prevent the inherited metabolic error by dietary treatment. For some PKU patients a relaxed low-phenylalanine diet will be lifelong. PKU-women must return to a strict low-phenylalanine diet before conception and during pregnancy to insure the delivery of a normal child. ...

The effect of protein malnutrition, induced by a diet containing 10% plant wheat gluten, on the immune response system was studied in growing rats. The rats were fed wheat gluten or casein (control) diets, and were the offspring of mothers fed a commercial stock rat diet, a gluten diet, or ...

The effects of diets containing low biologic value plant protein wheat gluten and the high biologic value protein casein on skin allograft survival were studied in growing Charles River (C.D. strain) rats. Five groups of weanling rats were fed 16% casein or gluten with or without supplementation with the amino ...

A new type of acquired C1-inhibitor (C1-INH) deficiency has been recognized (AAE type II) which is characterized by the presence of autoantibodies to C1-INH and by a circulating 96 KD C1-INH molecule. The clinical manifestations and biochemical abnormalities of this novel autoimmune disease resemble those found in the other forms ...