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C Lenaerts

Publications and source records attributed to C Lenaerts.

At least 19 recordsLinked to original sources
PubMed1991

[Dubowitz syndrome. A diagnosis not to be missed].

Dubowitz syndrome is a rare hereditary disorder whose main features are intra-uterine and post-natal growth retardation, characteristic facies, microcephaly, mental retardation and poor feeding. Because of the eczema which was present in half of the cases after 4 years of age, it cannot be mistaken for the more frequent fetal alcohol syndrome. We report 5 cases, among whom two sibs, confirming the recessive autosomal mode of inheritance and the necessity for genetic counseling.

Abnormalities, Multiple↗
PubMed1990

[Results of dietetics and nutritional assistance in cystic fibrosis].

The development of gastric acid-protected pancreatic extracts has radically transformed the diet of patients with cystic fibrosis, who are now provided with a high-caloric, hyperproteinic and normo- or hyperlipidic diet, with a correct supplementation in minerals, vitamins and oligo-elements. Nutrition has a major influence on the course of the disease, and the evaluation of the patients' needs has already shown that these are clearly increased in cystic fibrosis. In the severe and advanced forms of the disease, when refractory anorexia is added to chronic infection, more or less sophisticated techniques of nutritional assistance, such as oral supplementations and enteral or even parenteral feeding, are carried out. These techniques not only improve the anthropometric nutritional parameters, but if they have been prescribed soon enough they also interfere with the outcome of the disease.

Cystic Fibrosis↗
PubMed1990

Liver transplantation for hereditary tyrosinemia: the Quebec experience.

Sixteen tyrosinemic patients were evaluated in our institution for a possible liver transplantation. All patients showed biochemical and/or radiological evidence of liver dysfunction. Renal involvement was found to be more abnormal than expected. Seven patients have been transplanted, with two patients receiving a combined liver-kidney transplant. Hepatocarcinoma was detected in two of eight patients in whom the whole liver was examined. Six (37.5%) of the initial 16 patients have died since evaluation, one of the six dying after combined liver-kidney transplantation. Posttransplantation survival was 86%, with normal liver function, normal growth, and no recurrence of neurological crises on a normal diet.

Adolescent↗
PubMed1989

High incidence of upper gastrointestinal tract involvement in children with Crohn disease.

This retrospective study of Crohn disease in 230 children and adolescents with a mean age of 12.5 years at the time of diagnosis and an average follow-up of 6.6 years showed that 30% had lesions of the esophagus, stomach, and duodenum. Three patients had Crohn disease isolated to the upper gastrointestinal tract. The 169 patients with both small and large bowel disease were at greater risk (33%, P less than .05) of having upper gastrointestinal lesions than the 37 with isolated small bowel disease and the 21 with disease limited to the colon and/or rectum. An aggregate of symptoms and signs more likely present in those with upper gastrointestinal involvement included: dysphagia, pain when eating, nausea and/or vomiting, and aphthous lesions of the mouth. Furthermore, weight loss was more severe and hypoalbuminemia more frequent. Because upper gastrointestinal series x-ray studies failed to detect upper gastrointestinal lesions in 13 patients of 69 of those with upper gastrointestinal disease, endoscopy should be considered in all children and adolescents in whom a diagnosis of Crohn disease is entertained. Endoscopy and biopsy of the upper gastrointestinal tract should be done in any patient with symptoms suggestive of proximal involvement. Finally, in view of the fact that endoscopy established the diagnosis of Crohn disease in five patients previously thought to have chronic ulcerative colitis, the procedure should routinely be performed in all patients with chronic ulcerative colitis or indeterminate colitis before surgery is performed.

Adolescent↗
PubMed1989

Meconium ileus and its equivalent as a risk factor for the development of cirrhosis: an autopsy study in cystic fibrosis.

Although dehydrated obstructing mucus is thought to account for the obstructive pathology involving the lungs, the pancreas, the reproductive system, and the intestinal tract, its relationship with CF-associated liver disease remains largely hypothetical and little is known about possible risk factors. Complete clinical and autopsy records were available in 38 of 73 deaths occurring over a 10-year period. The liver was normal in only five cases, and they were all infants. Steatosis was the only lesion present in 9, hypoxic liver disease was documented in 8, and biliary cirrhosis in 16 (focal in 10 and multilobular in 6). There was no relationship between the presence of cirrhosis, gallbladder abnormalities, age at death, and clinical status recorded during the year precoding their demise. Mucus plugs characterized by amorphous eosinophilic material within proliferated bile ductules were present in 75% of children with focal or multilobular biliary cirrhosis as opposed to 14% in those without (p = 0.015). A history of meconium ileus or its equivalent was recorded more frequently (p = 0.038) in those with cirrhosis. Finally, biliary cirrhosis was invariably present when there was a history of meconium ileus or its equivalent and when mucus plugs were noted. These findings suggest that patients with intestinal obstruction are at greater risk for the development of cirrhosis and that strategies should be developed to increase the detergent capacity of bile and its flow in order to decrease the viscosity of mucus in the biliary tree.

Bile↗
PubMed1988

Pancrease gastroresistance: in vitro evaluation of pH-determined dissolution.

In order to avoid inactivation in the stomach, pancreatic enzymes have been prepared as pH-sensitive, enteric-coated microspheres (Pancrease). An in vitro study was performed to evaluate the pH-related dissolution of Pancrease and to confirm its resistance to gastric acidity. Two assay methods were used with three different batches of Pancrease: (a) Enzyme absorbency at 280 nm was measured at unit pH intervals from pH 1 to pH 8 and at 0.5 pH intervals from the start of dissolution to pH 8. (b) Proteolytic activity was measured at pH 6.8. Significant enzyme dissolution started at pH 5.5 and was maximal at pH 6.0. At pH 6.8, the pH of simulated intestinal fluid, dissolution was complete in less than 15 min. At pH 5.0, no dissolution occurred within the first 10 min and only 13% dissolution was observed after 2 h. At pH 7.0, 100% dissolution was seen within 10 min. Results of the two assay methods were comparable with all three enzyme batches assayed. This study confirmed the gastroresistance of Pancrease. Because of the enteric coating of Pancrease, liberation of enzymes occurs in the duodenum and jejunum, providing maximal enzymatic efficacy in exocrine pancreatic insufficiency.

Biological Availability↗
PubMed1985

[Long-term follow-up of abused children (apropos of 65 cases)].

The authors work in an area located north of Paris, which contains roughly a population of .75 million, of which 82,000 are children less than 3 years of age. The center and main city of the area is the town of Amiens. The recruitment of their cases under study was essentially hospital based, which explains the relative severity of the trauma observed. The children were followed by various observation systems. Seventy-four children, of which more than two-thirds were less than 3 years of age were followed for a period varying from two to ten years. Forty-three percent of these children have been maintained in their biological families; the others have been placed. With this system only nine recurrences of abuse have been observed (12%), and only two instances of death have been observed. Other studies have demonstrated higher proportions. However, in two-thirds of the children observed, there were minor or major psychological or even psychiatric sequelae. Protection services have tried to stay away from institutional placement as much as possible; when necessary, the foster home system or adoption has been used. Striking is the lack of means available to help abusing families. Efforts should be made to assist the abusing families stay together.

Battered Child Syndrome↗
PubMed1984

[Reactive arthritis in children].

Reactive arthritis is arthritis in which, although the nature of the responsible infection is known or suspected upon serological grounds, attempts at recovering the pathogen from the synovial fluid have failed. One of the main pathogenetic problems is the multiplicity of etiologic agents. Some are exogenous and may be related to the articular tropism of certain microorganisms, to immunologic depression due to an antecedent or coincident infection, and to successive reinfections by the same pathogen or by others which may promote an exacerbation of the disease. Others are endogenous and attention should be given to the local or systemic presence of an antigen as well as, in some instances, to the persistence of residual forms of infecting agents, which are more readily demonstrated with current bacteriological and serological methods. Although reactive arthritis is to be distinguished from septic arthritis, it can no longer be clearly differentiated from the classical post-infectious rheumatism. Once it has been produced, the antigenic stimulation is responsible for an immunologic response which tends to check systemic extension but may also produce tissue damage in the host. Some patients have circulating immune complexes which may bind to the joint, thereby damaging it. In other patients, particularly those who are HLA B27 positive, host-pathogen cross-reactions are demonstrated. Actually, the most frequent pathogenetic sequence seems to be a combination of two or more of these mechanisms, as there are reasons to believe that presence of the pathogen in situ is not required for the persistence of the inflammatory process. Reactive arthritis was first reported in adults following either sexually transmitted urethritis due to chlamydiae, mycoplasma or gonococci, or hepatitis B or an intestinal infection due to Yersinia, Campylobacter, Shigella, Klebsiella or Salmonella. Later, it was described in pediatric patients, particularly in Scandinavia where, for genetic reasons, the HLA B27 group is prevailing. Reactive arthritis seems less frequent in caucasian ethnic groups and above all in Latin Americans among whom HLA B27 carriers are more uncommon; however, it must be pointed out that they have not been as extensively studied and that other etiologic factors may still remain to be discovered. The course and etiology of the different forms of arthritis share certain characteristics which have been determined through a better knowledge of these conditions: onset occurs one or several weeks after a respiratory, urinary or, most often in children, digestive infection. This episode is unremarkable or latent and often overlooked.(ABSTRACT TRUNCATED AT 400 WORDS)

Antigen-Antibody Complex↗
PubMed1984

[Prenatal ultrasonic diagnosis of malformation uropathies: 13 cases].

The systematic prenatal diagnosis of 5 cases of urinary tract malformations out of 2 500 pregnancies in 1982 in a special care maternity, or 2 per 1 000 is compared with the discovery of 10 cases in 28 months in 17 960 foetuses, or 0.55 per 1 000 whereas the overall frequency varies from 0.3 to 5.25 per 1 000 in infants. We studied 13 children whose prenatal abnormalities were 2 unilateral ureterohydronephrosis and 7 bilateral dilatations associated to 2 ureteroceles, 1 multicystic dysplasia and 1 ureteral duplicity. All of these except two were confirmed at birth. The exceptions consisted in one unilateral dilatation and one bilateral ureterohydronephrosis which turned out to be dysplastic kidneys unknown during pregnancy. Three ureteral duplicities were also ignored. The results are the following: Three cases were severe: one therapeutic abortion after 27 weeks, one intra-uterine decompression followed after few days by a preterm delivery and neonatal death and one provocated preterm delivery (36 weeks). All the others underwent decompression during the first hours of their life. Five cases can be considered unsuccessful: 2 abortions, the death of a 22 days old infant with an unilateral multicystic dysplasia and 2 bilateral malformations with renal failure, one of which was associated to an unilateral dysplastic kidney. Reliability of sonography as well as its interest in prognosis and prenatal evaluation of renal function are demonstrated.

Amniotic Fluid↗
PubMed1983

[Regular dominance of thumb ankylosis with mental retardation transmitted over 3 generations].

A young girl 12 old, sent to us for obesity, and coxa-epiphysiolysis showed signs of mental retardation and bilateral thumb ankylosis. The fact that the mother was also affected by both of these signs, led to a more detailed genetic research. The latter revealed that not only the daughter, the mother, but also their own mother and may be, the sister, the grand-mother and the great-aunt of the patient had a retardation, a slight dysmorphia, a type A brachydactylia, signs of obesity and an identical ankylosis of both thumbs. This vertical inheritance, affecting apparently females only, but not associated with a high rate of miscarriage, has, it seems, never been reported. The characteristics of this family are being considered and discussed.

Adult↗
PubMed1983

[Solitary upper incisor, hypopituitarism and monosomy 18p chromosome aberration].

A prepubertal boy with hypopituitarism, mental retardation, dysmorphia and solitary maxillary central incisor is described, karyotypic studies showed deletion of the short arm of chromosome 18 (46, XY, del (18) (p11). It is suggested that caryotypic studies is of interest among the patients with midline defects and/or hypopituitarism.

Child↗
PubMed1983

[Panhypopituitarism in one of 2 monozygotic twins].

Anterior panhypopituitarism has been observed in one of monozygous twin girls. Etiologic investigations were negative and only suggested an hypothetic perinatal insult. Genetic abnormalities could be excluded. Replacement therapy with human growth hormone was strikingly successful, with a remarkable catch-up growth and, however, a velocity of bone age comparable with the velocity of growth.

Age Determination by Skeleton↗