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Biomedical subjects

P Goyens

Publications and source records attributed to P Goyens.

At least 19 recordsLinked to original sources
PubMed1997

Oral calcium phosphate: a new therapy for Crigler-Najjar disease?

BACKGROUND & AIMS: Calcium phosphate binds unconjugated bilirubin in vitro, and dietary calcium phosphate supplementation reduces the serum bilirubin level in rats with hereditary unconjugated hyperbilirubinemia (Gunn rats). The aim of this study was to evaluate the effect of oral calcium phosphate supplementation on plasma bilirubin levels in patients with Crigler-Najjar disease. METHODS: A placebo-controlled, double-blind, crossover design was used. Eleven patients, 2-42 years of age, participated. The group included 5 patients with type I disease who were all treated with phototherapy and 6 patients with type II disease who were primarily treated with phenobarbital. In addition to plasma bilirubin levels, dietary intake and urinary and fecal excretion of calcium and phosphate were evaluated. RESULTS: A modest but significant decrease in serum bilirubin was observed in patients with type I disease (18% +/- 6%, P = 0.03) but not in patients with type II disease during treatment with calcium phosphate. Urinary output of calcium and phosphate did not change during the treatment period. CONCLUSIONS: Oral calcium phosphate may be a useful adjuvant to photo-therapy in Crigler-Najjar type I disease.

Administration, Oral↗
PubMed1995

Familial adrenocorticotropin unresponsiveness associated with alacrima and achalasia: biochemical and molecular studies in two siblings with clinical heterogeneity.

UNLABELLED: The syndrome of familial adrenocorticotropin (ACTH) unresponsiveness is a rare form of primary adrenal insufficiency, usually without mineralocorticoid deficiency. It is characterized by elevated plasma ACTH concentrations and undetectable plasma cortisol levels not responding to exogenous ACTH. Alacrima and achalasia have also been occasionally associated with adrenal insufficiency (triple A syndrome). Pathogenetic mutations have been identified in the ACTH receptor gene in families with isolated familial ACTH unresponsiveness. Whether the ACTH receptor represents the locus of the defect for the triple A syndrome is not known. Here we report two siblings with familial ACTH unresponsiveness who were discrepant for skin pigmentation and mineralocorticoid function. In addition, achalasia and alacrima were documented only in the older sibling. The boy, studied at the age of 2 years, was hyperpigmented, in contrast to his normally pigmented sister, studied at the age of 9 years; basal plasma alpha-melanocyte stimulating hormone immunureactivity levels were 79 and 38 pg/ml, respectively (normal < 40 pg/ml). Furosemide-induced diuresis resulted in normal rises of plasma renin activity in both patients; however, plasma aldosterone levels increased only in the boy and not in his sister. Screening for abnormalities of the ACTH receptor gene by single strand conformation polymorphism analysis revealed no abnormality. Direct sequencing of the entire coding area of the ACTH receptor gene was also normal. CONCLUSION: The syndrome of familial ACTH unresponsiveness can vary clinically and biologically within the same family.(ABSTRACT TRUNCATED AT 250 WORDS)

Adrenal Gland Diseases↗
PubMed1993

Selenium and the thyroid: how the relationship was established.

Several hypotheses concerning consequences of selenium deficiency on iodine metabolism can be proposed on the basis of experimental studies in rats and from epidemiological and experimental studies in humans. By decreasing intracellular GSH peroxidase activity, selenium deficiency may increase hydrogen peroxide (H2O2) supply and lead over several weeks to the thyroid atrophy observed in myxoedematous cretins. By improving thyroid hormone synthesis and by decreasing peripheral thyroxin (T4) deiodination, selenium deficiency could protect fetal brain T4 supply and thus prevent neurologic cretinism. Selenium deficiency may protect against iodine deficiency by decreasing T4 metabolism--and thus iodide leakage and--perhaps also by increasing H2O2 supply and thyroid hormone synthesis and thus thyroid efficiency.

Animals↗
PubMed1993

Interferon alfa-2b therapy in children with chronic hepatitis B.

Twenty nine children (mean age 8.3 years, 18 boys, 11 girls) who had biopsy proved chronic hepatitis B virus infection (HBV) with active viral replication were given a 16 week course of interferon alfa-2b treatment (9 million units (MU)/m2, thrice weekly). Fourteen children (48%) showed persistent loss of HBV-DNA 8 months after the end of treatment; 11 (38%) lost hepatitis B e antigen (HBeAg), and two (7%) hepatitis B surface antigen (HBsAg). Alanine aminotransferase activities returned to normal in 12 children. Those who responded had significantly higher initial transaminase activities than those who did not (p < 0.01) but similar serum HBV-DNA. Results were compared with the natural evolution of the disease in a group of 25 children (mean age 8.3 years) with identical initial mean serum HBV-DNA values, followed up during the same period. Two of these (8%) lost HBeAg and one (4%) HBsAg. The 23 remaining control subjects had no decrease in serum HBV-DNA or in transaminase activities compared with values 1 year earlier. It is concluded that treatment with interferon alfa-2b in children may lead to inhibition of HBV replication similar to that described in adults, and may thus shorten disease evolution. Further studies are necessary to establish the best protocols and to identify those patients who are the most likely to respond to treatment.

Alanine Transaminase↗
PubMed1993

Achalasia-alacrima-ACTH insensitivity syndrome (Triple-A-syndrome).

Achalasia is a rare pathology in infancy. It is still more infrequent when associated with adrenal insufficiency and alacrima, a disorder known as Allgrove's syndrome, the etiology of which remains unclear. We describe a 9-year-old girl who presents with glucocorticoid insufficiency, partial mineralocorticoid deficiency, achalasia, and alacrima.

Adrenocorticotropic Hormone↗
PubMed1992

[Enzymes and histology of the intestinal mucosa of breast-fed African infants].

Weight gain and statural growth are slow in the Kivu area (Zaïre), as compared with international reference curves. One hypothesis put forward to explain this well-documented but poorly understood fact is inadequate breast-feeding with early intake of foods responsible for the introduction of microorganisms with deleterious effects on the digestive tract. A study of intestinal mucosa specimens from 90 at least partially breast-fed infants (4 to 22 months) showed that the appearance of the jejunal mucosa was consistently abnormal, as compared with histologic standards used in Europe. Furthermore, intestinal disaccharidase activities were always abnormal; lactase activities were extremely low and alpha-glucosidase activities were reduced by one-third to one-half as compared with European reference values. Age apparently influenced the magnitude of these modifications, whereas the correlation with nutritional status was less clear; only a correlation between the serum albumin level and the microscopic appearance of jejunal biopsy specimens was found. Intestinal alpha-glucosidase levels were lower in those patients with giardiasis. These findings demonstrate that in Kivu early atrophy of the intestinal mucosa contributes, together with the protein-calory malnutrition, to the lower growth rates in infants.

Age Factors↗
PubMed1988

[Validation of fast nutritional scoring. Application to 80 cases of "homing" diarrhea].

Pediatricians often have to assess the nutritional status of their patients from unprecise anamnestic information, unreliable clinical signs or biological data difficult to interpret. None of these parameters can be used alone to establish the nutritional status of a sick child. The validity of a simple and rapid nutritional score was tested. The score consisted of 3 anthropometric parameters (weight, height, triceps skinfold) and 4 biological analyses frequently used in pediatric practice (albumin, transferrin, alkaline phosphatase and ferritin serum determinations). The usefulness of the score was established through the important nutritional deficiencies observed in 80 children admitted for "homing diarrhea" requiring early nutritional support.

Alkaline Phosphatase↗
PubMed1987

Selenium deficiency as a possible factor in the pathogenesis of myxoedematous endemic cretinism.

Myxoedematous endemic cretinism is prevalent in African goitre endemies. It has been related to a thyroid 'exhaustion' atrophy occurring near birth. It is proposed that this might result from the low resistance of a fragile tissue to enhanced H2O2 generation under intense thyroid stimulation by thyrotropin. In support of this hypothesis, low selenium and glutathione peroxidase serum levels have been found in the African endemic area of the Idjwi Island (Kivu, Zaire). Serum selenium and plasma glutathione peroxidase were lower in the area of high endemicity of goitre and cretinism (Northern part of the Island). However, only the former difference is statistically significant. These data thus suggest a role of oligoelements and oxygen toxicity in the pathogenesis of endemic cretinism.

Adolescent↗
PubMed1986

[New aspects in the nutrition of newborn infants with low birth weight].

Optimalize the feeding of low birth weight infants is a very important problem in industrial as well as in developing countries where such infants are very numerous. Water need is very important but gastric and vascular capacity is limited in LBW infants. Energy has to be absorbed at a rate of 120 to 130 Kcal/kgBW/day without raising the osmolarity of the food. Protein intake has to be higher than in term babies but due to enzyme and kidney immaturity the amino acid pattern of the protein has to be carefully adapted to the pattern of the proteins to be synthesized. Calcium, phosphorus, iron, zinc and other trace minerals as well as vitamins have to be provided in relatively large quantities but their bioavailability has also to be checked. The utilisation of breast milk and particularly of own mothers milk having delivered prematurely is also discussed.

Dietary Carbohydrates↗
PubMed1985

Copper deficiency in infants with active celiac disease.

Celiac disease was diagnosed in two unrelated infants aged 7 and 7.5 months with severe malnutrition. They showed typical clinical, biological, and histological signs of the disease. Moreover, accompanying copper deficiency was suggested by severe hypocupremia and persistent neutropenia; bone radiographs were also compatible with this diagnosis. Rapid and complete correction of these anomalies could only be obtained after addition of oral copper sulfate to the gluten-free diet. Mechanisms possibly involved in the development of copper deficiency in young infants with celiac disease are: chronic malabsorption; high copper needs in rapidly growing infants; and possibly increased biliary and digestive losses. It is therefore suggested that young children with severe celiac disease should be monitored for their copper status.

Celiac Disease↗