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Biomedical subjects

D Jan

Publications and source records attributed to D Jan.

At least 19 recordsLinked to original sources
PubMed2005

Outcome and long-term growth after extensive small bowel resection in the neonatal period: a survey of 87 children.

UNLABELLED: This retrospective study aims to analyze the outcome, the prognosis factors and the long-term growth of children after extensive small bowel (SB) resection in the neonatal period. PATIENTS AND METHODS: 87 children, born between 1975 and 1991 who had undergone extensive neonatal small bowel resection, were followed up over a mean period of 15 years. Anatomical data influencing PN dependency and duration were analyzed. Data on height and weight were collected and compared using growth standards. Final heights were studied for patients who achieved their puberty and compared to predicted height based on Tanner's formula. Patients were analyzed according to PN weaning and growth: children still receiving PN (group A), patients weaned from initial PN but requiring PN once again or enteral feeding (group B), and children with permanent intestinal autonomy (group C). RESULTS: The overall survival is 89.7 %, depending on the date of birth. The duration of PN-dependency varies according to the intestinal length and the presence of the ileocaecal valve (ICV). All patients who remain PN dependent had less than 40 cm of small bowel and/or the absence of ICV. Patients in group B had a mean small bowel length of 35 +/- 19 cm, resection of the ICV in 50 % of cases, and a PN duration of 47.4 +/- 23.8 months. There was a significant decrease in height and weight gain within the 4 years after cessation of PN, requiring enteral or parenteral feeding. Patients in group C had a mean small bowel length of 57 +/- 19 cm, presence of ICV in 81 % of cases and a PN duration of 16.1 +/- 11.4 months. After PN weaning, they grow up normally with normal puberty and final height as predicted from genetic target height. CONCLUSION: PN duration is influenced by the length of residual SB and the absence of ICV. With good anatomic prognosis factors and short duration of initial PN, normal long-term growth may be predicted. Conversely, poor anatomical factors and protracted initial PN require careful monitoring of growth and may sometimes require nutritional support to be restarted. The last group, permanently dependent on PN, might be candidates for intestinal transplantation.

Body Size↗
PubMed2003

Feasibility of surgery for patent ductus arteriosus of premature babies in a neonatal intensive care unit.

Twenty-two premature neonates underwent surgical haemoclip closure of patent ductus arteriosus in a neonatal intensive care unit (NICU). Surgery was performed in the NICU in order to prevent hypothermia and interruption of care, and to avoid accidental vascular access removal and extubation. The results suggest that morbidity can be limited by performing the surgery in an NICU, and by switching from conventional to high-frequency mechanical ventilation in case of severe respiratory failure.

Ductus Arteriosus, Patent↗
PubMed2002

Partial elective pancreatectomy is curative in focal form of permanent hyperinsulinemic hypoglycaemia in infancy: A report of 45 cases from 1983 to 2000.

BACKGROUND/PURPOSE: Permanent hyperinsulinemic hypoglycaemia in infancy (PHHI)I is a severe disease that leads to brain damage. Since 1989, pathologists have identified 2 different forms of the disease: a diffuse form (DiPHHI) and a focal form (FoPHHI). The purpose of this study was to adapt surgical techniques in case of FoPHHI to cure these infants without risk of diabetes. METHODS: All patients with PHHI underwent pancreatic venous sampling (PVS) and elective partial pancreatectomy (EPP). Molecular biology and immunohistochemistry were used to ascertain that FoPHHI was a different disease from DiPHHI. RESULTS: 45 EPPs were performed, guided by PVS and peroperative pathology. The lesions were 17 in the head, 4 in the isthmus, 6 in the body, 15 in the tail of the pancreas. Age at surgery ranged from 25 days to 4 years. Two patients already had been operated on elsewhere, and the focal lesion could be found at second operation. All 45 patients except one, were cured with euglycemia at both fasting and hyperglycaemic tests. Molecular biology has shown a specific anomaly in FoPHHI, which never has been encountered in DiPHHI. CONCLUSIONS: PHHI is not a homogeneous disease. In one third of cases, only a small amount of endocrine pancreas is abnormal, and conservative surgery is mandatory. The pre- and perioperative conditions to point out the focal pancreatic lesion are described.

Child, Preschool↗
PubMed2002

Indications and results of surgery in patients with Crohn's disease with onset under 10 years of age: a series of 18 patients.

In order to examine the presentation and course of Crohn's disease (CD) with an onset of CD before the age of 10 and to assess indications and effects of surgery, a retrospective study was performed in 18 children. One hundred and twenty-two children with CD have been followed over the last 22 years in our institution. Thirty of them had the first onset of disease before 10 years of age. Eighteen out of these 30 underwent intestinal resection (IR). The mean age at the onset of CD was 6.4 years and the average duration of follow-up was 11.1 years. Most common symptoms were abdominal pain (72 %), diarrhoea (72 %), and growth retardation (50 %). Colon and ileum were the most frequently involved areas. Most of the children (94.5 %) received extensive medical and or nutritional treatment before surgery. We performed 26 IRs and 14 other operations in 18 patients. Indications for IR were chronic intestinal obstruction (13 cases) and chronic intestinal disability (13 cases). Improvements in the general clinical state were obtained in 17 cases out of 18. IR is beneficial for chronically ill children with ineffective medical therapy. A disease-free interval after surgery may provide an important time for improved growth and pubertal development and a return to nearly normal life for a long time.

Adolescent↗
PubMed2002

Experience of direct coronary stenting at National Institute of Cardiovascular Diseases.

OBJECTIVE: To assess the efficacy, safety and advantage of direct coronary stenting versus traditional angioplasty and stenting. METHODS: From May 2000 to June 2001, 486 patients underwent PTCA procedure. One hundred fifteen (24%) were considered candidates for direct stenting. Out of 150 coronary lesions, 119 (79%) had direct coronary stenting. In 5% patients stent could not be passed across the lesion. These stents were withdrawn without stent damage and after predilatation the same were successfully deployed. RESULTS: Primary success rate was 95%. Angiographically assessed success was achieved in all patients with immediate TIMI 111 flow in 97%. The vessels directly stented were LAD 53%, RCA 27% and LCX 18%. Two saphenous vein grafts were also directly stented. The coronary lesions stented were type A 36%, B1 54% and B2 10%. In borderline lesions IVUS was used for quantification of stenosis. There was no in-hospital death, MI or emergency CABG. Compared to traditional stenting there was 46% reduction in procedure time, 62% reduction in radiation exposure time and 35% decrease in use of contrast media. There was also significant (18%) cost benefit in direct stenting. CONCLUSION: This study supports the view that direct coronary stenting is feasible and a safe procedure in our population with additional benefits of cost reduction by 18%. We recommend direct stenting in appropriately selected patients.

Adult↗
PubMed2001

Long term results of liver-kidney transplantation in children with primary hyperoxaluria.

From 1990 to 2000, we performed eight liver-kidney transplants in eight children, aged 1-16 years, with end-stage renal failure (ESRF) due to primary hyperoxaluria (PH1). The duration of dialysis before transplantation ranged from 2 to 42 months (mean 14 months) and was <1 year in four patients. Only the first patient underwent postoperative hemodialysis; in the other five, we chose to induce maximal diuresis from the first hours with intravenous and intragastric hyperhydration (> or =3 l/m2 per day). High water intake with nocturnal tube hydration was maintained for 6 months to 5 years, as long as oxaluria exceeded 0.5 mmol/day. A quadruple sequential immunosuppressive regimen was used. Two patients died during liver graft surgery. The other six patients are alive and well, with a mean follow-up of 7.4 years (range 5-11 years). Patient and graft survival is 75% at 5 years. At latest follow-up, liver tests were normal in all six patients; creatinine clearance ranged from 55 to 95 ml/min per 1.73 m2 (mean=74). Oxaluria was lower than 0.4 mmol/day in all patients (mean=0.22). The six patients underwent 15 renal biopsies, 1-11 years after transplantation. Chronic transplant nephropathy was present in four patients and mild cyclosporin nephrotoxicity in another. No oxalate crystals were seen and repeat ultrasonography has been consistently normal in all patients. The three patients with bone oxalosis showed progressive complete healing of bone lesions. All six children or adolescents now live a normal life. From this series, we conclude that early combined liver-kidney transplantation is the treatment of choice for children with ESRF due to primary hyperoxaluria.

Adolescent↗
PubMed2001

Urological complications after renal transplantation using ureteroureteral anastomosis in children.

PURPOSE: Ureterovesical reimplantation is most often performed for renal transplantation in children. We reviewed our experience to evaluate the safety and efficacy of ureteroureteral reimplantation in pediatric renal transplantation. MATERIALS AND METHODS: We retrospectively evaluated the charts of 92 boys and 72 girls who underwent a total of 166 ureteroureteral anastomoses for renal transplantation from January 1990 to December 1999. Spatulated end-to-end anastomosis was performed between recipient and graft ureters without stenting and with a bladder catheter for at least 10 days. RESULTS: Mean patient age at transplantation was 11.2 years (range 1 to 21.5). There were 22 living related donor and 144 cadaveric grafts. Urological anomalies and nephropathy were the cause of end stage renal disease in 146 and 20 patients, respectively. Urological complications were noted in 14 of the 166 transplantations (8.4%) in 10 boys and 4 girls, including 12 initial and 2 repeat grafts from 2 living related and 12 cadaveric donors. Five of these patients had undergone previous urological surgery. The 2 children (1.2%) with acute ureteral obstruction underwent repeat intervention after stent failure. Anastomotic leakage in 7 cases (4.2%) was treated conservatively in 1 and with a Double-J stent (Medical Engineering Corp., New York, New York) only required in 3. Reoperation was required in 3 cases. One patient (0.6%) with late ureteral stenosis underwent repeat anastomosis, 1 (0.6%) required reimplantation for recurrent pyelonephritis due to vesicoureteral reflux in the graft, 1 (0.6%) with a valve bladder required bladder augmentation and ureteral reimplantation, and 1 (0.6%) with lymphocele and 1 (0.6%) with lithiasis were successfully treated conservatively. Complications were associated with acute rejection in 6 cases. Mean followup without graft loss in patients who presented with versus without complications was 58.3 months (range 1 to 112) versus 75 (range 1 to 118). In the former patients with a mean age of 16 years 9 months versus those without urological complications mean serum creatinine was 116 and 108 mol./l., respectively. Two grafts were lost in patients with urological complications, including 1 who died of pulmonary embolism and 1 with refractory chronic rejection. Seven patients were lost to followup after 54 months (range 12 to 113) of adequate graft function. CONCLUSIONS: Ureteroureteral anastomosis is a safe and effective technique for pediatric renal transplantation with a low complication rate, which may be due to better vascularization of the shorter ureteral end of the graft. Our results should encourage the use of this technique in pediatric renal transplantation. Efforts to preserve the recipient ureters should be made at nephrectomy.

Adolescent↗
PubMed2000

Value of open lung biopsy in immunocompromised children.

OBJECTIVES: To determine the value of open lung biopsy (OLB) in terms of diagnosis, morbidity, mortality, and benefits in immunocompromised children with pulmonary involvement. STUDY DESIGN: We retrospectively reviewed 36 OLBs performed in 32 immunocompromised children between 1985 and 1998. Seventeen biopsies were performed in patients with primary immunodeficiency syndromes and 19 in patients with secondary immunodeficiency syndromes. Twenty-eight biopsies were performed because of a lack of response to ongoing antimicrobial treatments with negative or positive findings on bronchoalveolar lavage (BAL) and a deteriorating clinical or radiologic course, and 8 biopsies were performed because of persistent chest x-ray infiltrates. RESULTS: Diffuse pulmonary infiltrates were observed on chest x-ray in 28 cases, hyperinflation in 3 cases, and nodular infiltrates in 5 cases. A histopathologic diagnosis was possible for all 36 OLBs. Specific diagnosis was obtained in 22 (61%) (12 infectious agents, 6 tumors, 4 bronchiolitis obliterans) and non-specific diagnosis in 14 (39%). Fungi were the main infectious agents (8 of 12). For the diagnosed infections, BAL provided 4 true-positive, 3 false-positive, and 6 false-negative results. Specific treatment was changed in 77% of cases, providing real benefits in 12 (33%) cases. The morbidity and overall mortality rates were 31% and 33%, respectively. The mortality rate was significantly higher in the first 30 days after OLB in patients receiving ventilatory assistance (58%). CONCLUSIONS: OLB in immunocompromised children with deteriorating clinical or radiologic course is a sensitive diagnostic tool.

Adolescent↗
PubMed2000

Intestinal transplantation: indications, results and strategy.

The term 'intestinal failure' is now often used to describe gastrointestinal function insufficient to satisfy body nutrient and fluid requirements. The first recognized condition of intestinal failure was short bowel syndrome. Severe motility disorders such as chronic intestinal pseudo-obstruction syndrome in children as well as congenital intractable intestinal mucosa disorders are also forms of intestinal failure, because no curative treatment for these diseases is yet available. Parenteral nutrition and home parenteral nutrition remain the mainstay of therapy for intestinal failure, whether it is partial or total, provisional or permanent. However, some patients develop complications while receiving standard therapy for intestinal failure and are considered for intestinal transplantation. Indeed, recent advances in immunosuppressive treatment and the better monitoring and control of acute rejection have brought intestinal transplantation into the realm of standard treatment for intestinal failure. Although it has been used in humans for the past two decades, this procedure has had a slow learning curve. According to the current results, this challenging procedure may be performed in children or adults, only under certain conditions.

Adult↗
PubMed1999

Liver transplantation in urea cycle disorders.

We report here our experience in the long-term management of 28 patients with citrullinaemia, 13 patients with carbamoyl phosphate synthase deficiency and 15 patients with argininosuccinic aciduria. In addition, we report a national French survey of 119 patients with ornithine transcarbamylase (OTC) deficiency enzymatically characterized in our laboratory. We also include in this report four personal patients (two with OTC and two with citrullinaemia) who were liver transplanted, and one OTC patient from the National French survey. Although this retrospective series is not really representative of the modern treatment combining low protein diet and arginine, sodium benzoate and sodium phenylbutyrate, it is obvious that the long-term outcome of all urea cycle disorders remains very guarded. We highlight the severity of the neonatal forms of such disorders, and mostly for OTC-deficient males. According to this evidence, our policy is not to treat such severely affected patients in the neonatal period who die anyway spontaneously within 2 to 3 days. At the present time, we only have three patients with neonatal citrullinaemia, aged 1, 6 and 10 years respectively, who are still doing well. One of them has been successfully liver transplanted at 5 years. Another transplanted patient died in the post-surgical phase. We emphasize the unexpected severity of argininosuccinic aciduria in which there is no one patient doing well. This is a rather surprising finding as this disorder is easy to manage and rarely presents with recurrent attacks of hyperammonaemia when it is treated by arginine supplementation. This consideration would suggest to extend the indication of orthotopic liver transplantation in this disorder. Finally, the most difficult indication is in the late onset symptomatic female OTC group. In this last group, despite a significant residual activity due to heterozygote status, even with a variable lyonisation, only seven girls are still mentally and neurologically normal. Interestingly, three of these seven were liver-transplanted before the constitution of irreversible neurological damage. These three girls and their family declare their well-being, their feeling to be cured and enjoy their normal life.

Amino Acid Metabolism, Inborn Errors↗