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Biomedical subjects

D Jan

Publications and source records attributed to D Jan.

At least 73 records · Page 4Linked to original sources
PubMed1992

[Intensifying iron chelating therapy with desferrioxamine using implantable venous access catheters (Port-A-Cath)].

Deferoxamine is still today the only preventive and curative treatment of transfusional hemochromatosis. It must be perfused daily, intravenously or subcutaneously, during several hours. Implantable infusion devices (Port-A-Cath) offer intravenous access, allowing to use higher doses, while avoiding local swelling due to subcutaneous injections. This device was inserted in 7 major thalassemic patients who presented with severe complications of iron overload, including 4 of them with signs of cardiac failure. Ferritinemias of all patients were lowered after intensifying iron chelation: cardiac function improved drastically in 2 patients. Devices were responsible for some complications: occlusion in one patient, local infections in two. This way of administration of desferal seems useful in patients with high ferritinemia and/or organic complications related to hemochromatosis.

Acute-Phase Reaction↗
PubMed1991

Neonatal short bowel syndrome.

In this retrospective study the management of infants who had undergone resection of more than 50% of the small bowel as newborn infants between 1970 and 1988 was analyzed to define prognostic factors. Small bowel resections were performed for atresia (36 cases), volvulus (22 cases), gastroschisis (10 cases), necrotizing enterocolitis (11 cases), and other disorders (8 cases). Patients were classified into two groups depending on the length of residual small bowel: group 1 (n = 35) had less than 40 cm of small bowel and group 2 (n = 51) had 40 to 80 cm of residual small bowel. Patients in group 2 had significantly better survival rates than those in group 1 (92.0% vs 66.6%; p less than 0.001). The patients in group 1 who were born after 1980, when home parenteral nutrition was introduced, had better survival rates than those who were treated before 1980 (95.0% vs 65.0%; p less than 0.01). The time required for acquisition of intestinal adaptation depended on the intestinal length (average, 27.3 months for group 1 and 14 months for group 2; p less than 0.01) and on the presence or absence of the ileocecal valve. Parenteral or supportive enteral nutrition, or both, ensured normal growth in both groups. We conclude that more than 90% of infants now survive after extensive small bowel resection with parenteral nutrition and that the remaining small intestine will adapt with time. Home-based parenteral nutrition allowed children to be treated in the best psychosocial environment.

Adaptation, Physiological↗
PubMed1991

[Prevention of vascular thromboses after renal transplantation using low molecular weight heparin].

Vascular thrombosis is one of the main causes of early transplant failure in pediatric patients. This paper reports the results of an open trial of the low molecular weight heparin (Enoxaparine) used to prevent renal graft thrombosis in pediatric recipients with risk factors including donor or recipient age under 5 years, multiple arteries supplying the transplant, and positive history for recurrent thrombosis. During 1989, 42 of 67 children given a renal transplant were prophylactically treated with Enoxaparin. Only one transplant was lost to thrombosis among treated patients (1.5%), versus 9 transplants among 73 (12%) children who received their kidney in 1988 without prophylactic Enoxaparin. Risk factors were comparable in both groups of recipients. Enoxaparine therapy was associated with an increased rate of bleeding (12/42) without severe consequences. In conclusion, Enoxaparin is effective in preventing renal graft thrombosis. Availability of this prophylactic therapy makes it possible to use transplants removed from the youngest donors considered as inadequate by some groups.

Age Factors↗
PubMed1991

[Hepatic and renal transplantation in the treatment of type I hyperoxaluria].

Hyperoxaluria type I (HPI) is a metabolic disorder secondary to liver alanine glyoxylate aminotransferase deficiency. Renal failure occurs due to the excessive production and precipitation of oxalate in the kidney. Combined liver-renal transplantation is the correct treatment for this condition when end-stage renal failure occurs as with renal transplantation alone the risk of recurrence of the same pathology in the transplanted kidney would be high. We report the case of a 4 year-old child with HPI suffering from terminal renal failure in whom a hepato-renal transplantation was performed: six months later, creatinine clearance was 62 ml/min/1.73 m2 and liver function tests were normal.

Actuarial Analysis↗
PubMed1990

Deduction from Wilms' tumour that glomerular podocytes produce the basement membrane material bearing Goodpasture determinants.

Using an indirect immunoperoxidase technique, we tested frozen specimens from one Wilms' tumour composed of numerous glomeruloid bodies devoid of blood vessels, with monoclonal antibodies directed against vimentin, cytokeratin, CALLA/CD10, CD24, CR1/CD35, endothelium factor VIII, class I and II MHC molecules, laminin, fibronectin, and non-collagenic domain NC1 of type IV collagen. Two reagents against Goodpasture determinants were used: P1 monoclonal antibody and serum IgG (GP antibodies) from a biopsy-proven Goodpasture patient. Glomeruloid bodies comprised two cell types: a peripheral layer of parietal epithelial cells (cytokeratin and CD24-positive) and central cell clumps of podocytes (vimentin and CALLA-positive). The basal lamina surrounding the glomeruloid bodies contained laminin and NC1 domain of type IV collagen, while that present between the podocytes reacted strongly with laminin, and P1 and GP antibodies. Endothelium factor VIII was not detected within the glomeruloid bodies and CR1 molecules bound to the basement membrane material within them. These data favour the hypothesis that podocytes produce the basement membrane material which bears Goodpasture determinants recently identified as a novel chain, named the alpha 3 chain, of type IV collagen.

Anti-Glomerular Basement Membrane Disease↗
PubMed1990

Partial splenectomy in homozygous beta thalassaemia.

Partial splenectomy was performed on 30 patients with homozygous beta thalassaemia to reduce blood requirements and to avoid the risk of overwhelming postsplenectomy infections; 24 patients had thalassaemia major and six thalassaemia intermedia. Five patients received a high transfusion regimen before and after surgery and 25 a lower one. Follow up after surgery ranged from one to four years. Partial splenectomy improved the long term haematological state in the six patients with thalassaemia intermedia. Recurrence of hypersplenism occurred in nine of the 24 patients with thalassaemia major, however, and complete splenectomy was required. Serum IgM concentrations were not significantly modified by surgery. The mean (SD) residual spleen after surgery was 4.45 (2.36) cm measured by scintigraphy. No severe infections occurred after surgery; however, most patients were routinely treated with phenoxymethylpenicillin and the protective effect of the remaining spleen could not be exactly determined. Because of the possibility of recurrence of hypersplenism, routine partial splenectomy when splenectomy is needed in thalassaemia major is not advised, except in children under 5 years whose risk of overwhelming postsplenectomy infection is greatest.

Adolescent↗
PubMed1990

[Severe digestive manifestation of rheumatoid purpura. Retrospective study of 19 cases in children].

Digestive and/or nutritional manifestations of Henoch-Schoenlein purpura (HSP) in childhood may be so severe as to require nutritional assistance. The study of 19 cases with this form of the disease allows to suggest a therapeutic protocol. When enteral nutrition cannot be used from the onset, parenteral nutrition is required. Associated steroid therapy may prove helpful for the regression of the digestive signs. The observed slowness of the weight catch-up, in spite of high protein-energy intakes, emphasizes the severity of catabolism in these severe forms of HSP.

Adolescent↗
PubMed1989

[Home parenteral nutrition in children. Evaluation after 8 years' activity with 88 patients].

In cases where an anatomic or functional amputation of the small bowel dictates that parenteral nutrition needs to be prolonged, it is essential to offer the child the best possible quality of life. In this regard, being in his home environment is an important component. Over a 8 year-period (1980-1988), 88 children went home with cyclic parenteral nutrition. In 34, parental nutrition was eventually discontinued: 40 are still parenteral nutrition-dependent and 14 died. In most cases, the growth and quality of life were satisfactory. Complications consisted essentially of infections, with 1 septicemia for 692 days of home parenteral nutrition (HPN). In the light of these results, HPN appears to be the best option for prolonged parenteral nutrition-dependent children. However, it cannot be set up without the help of a suitable center able to follow the children and to ensure the logistic support necessary for this highly technical and demanding type of care.

Adolescent↗
PubMed1989

[Esophagoplasty in caustic esophageal stenosis in children].

Over a period of 30 years, 53 children suffering from caustic stenosis of the oesophagus were treated by oesophagoplasty. The left colon was most often used (49), though on occasions a Gavriliu gastric tube (3) or the right ileo-colon was used (1). Technical failure occurred in 7 cases with left esophagocoloplasty and this was treated with either a gastric tube (4) or right esophagocoloplasty (3). Oesophagectomy to prevent cancer was carried out on 20 occasions after the oesophagoplasty, on 23 occasions at the same time at the oesophagoplasty, and has yet to be carried out in 6 children. Early mortality was zero; 4 uncomplicated cervical fistulae occurred; 12 anastomotic stenoses were cured by dilatation and 5 required further surgery. Death occurred in 3 children as a late event: one at 2 months, due to difficulty with deglutition, one at 2 years, due to meningococcal septicemia, and one at 3 years due to obstruction from adhesions. Peptic reflux was seen in 3/9 Gavriliu plasties and in 6/45 coloplasties. Functional sequelae were absent in 29 cases and 12 cases suffered occasional episodes of obstruction. The best procedure appeared to be posterior mediastinal esophagocoloplasty combined with resection of the damaged oesophagus.

Burns, Chemical↗
PubMed1988

[Organ procurement in children. Surgical aspect].

Today, in every child in situation of organs donor, a multiple organ harvesting is performed which requires surgical techniques more precise, in order to improve the results. Heart, heart-lungs and liver transplantation represent in children, for volume reasons, situations where harvesting must be performed in a child matched for height with the recipient. Today a technique of unique harvesting took place of the initial techniques which required careful dissection of all organs, the preparation of organs being made secondarily, after refrigeration and harvesting. The first step is abdominal and sets up a device of abdominal spraying. The second step is the thoracic dissection, heart or cardio-pulmonary block harvesting being performed by the cardiac surgery team. Finally, the third step consists of the harvesting of the abdominal viscera. It is preceded by a precooling, begun during the period before thoracic dissection. It supposes a global cooling of the liver, kidneys, duodeno-pancreatic block and small bowel. Monoblock dissection then begins with the liberation of the liver and eventually of the duodeno-pancreatic block. After hepatectomy, both kidneys are harvested in monoblock. The intestine, itself, is harvested in last position. Progresses should occur, due to the improvement of the performances of the cooling solutions.

Abdomen↗
PubMed1983

Biochemical variants in dogs.

Biochemical variants of constituents of the blood are considered to be valuable markers of genes in studies on population genetics. They are being used for that purpose for human populations and also for populations of laboratory and farm animals. The present report is concerned with biochemical variants in dogs. In dog breeding an increasing interest is being shown in the origin of the breeds, in their mutual relations, and in the relationship with wild canidae. Since biochemically detectable variation can offer valuable information in this respect, an overall investigation of the possibilities for the detection of these variants has been made. In this study, eleven constituents of the blood of dogs were found to demonstrate clearly detectable genetic polymorphism. The genetic systems for these eleven constituents are described. The observed frequencies of the variants are reported.

Animals↗