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J C Molenaar

Publications and source records attributed to J C Molenaar.

At least 19 recordsLinked to original sources

Cost-effectiveness of neonatal surgery: a review.

BACKGROUND/PURPOSE: Technological developments have revolutionized both diagnosis and treatment in neonatal surgery. However, it has been increasingly recognized that financial resources might become insufficient to provide all the medical care that is technically feasible or that patients and families might desire. The purpose of this study is to apply the theory of health economics to neonatal surgery and to explore the extent and the kind of economic evaluation done in neonatal surgery. METHODS: To explore the work done so far, the authors undertook a literature search aimed at costs and effects of surgical interventions in newborns with Ravitch' surgical index diagnoses of congenital anomalies. Common keywords in cost-effectiveness analysis were used to search Medline. RESULTS: Evidence about the cost effectiveness of neonatal surgery is largely lacking. This is probably because of difficulties in long-term tracking of the patients and to the problem that most generic quality-of-life measures are not applicable in children yet. CONCLUSIONS: Further cost-effectiveness research in neonatal surgery is warranted to settle priority discussions in health care when neonatal surgery is part of such discussions. Methodology for generic quality-of-life measurement in children is badly needed.

Congenital Abnormalities↗

Directing portal flow is essential for graft survival in auxiliary partial heterotopic liver transplantation in the dog.

BACKGROUND/PURPOSE: Auxiliary liver transplantation is an attractive alternative for orthotopic liver transplantation in patients with certain inborn errors of metabolism of the liver in which complete resection of the liver is unnecessary or even contraindicated. Because in these diseases portal hypertension is mostly absent, finding a balance in portal blood distribution between native liver and graft is complicated. The objective of this study was to investigate requirements for long-term (180 days) graft survival in auxiliary partial heterotopic liver transplantation (APHLT) in a dog model. METHODS: A metabolic defect was corrected in 26 dalmation dogs with a 60% beagle heterotopic auxiliary liver graft. Four groups of different portal inflow were studied. In the ligation group the portal vein to the host liver was ligated. In the split-flow group graft and host liver received separate portal inflow. In the banding group the distribution of the portal flow was regulated with an adjustable strapband and in the free-flow group the portal blood was allowed to flow randomly to host or graft liver. RESULTS: Metabolic correction increased in all groups after transplantation from 0.19 +/- 0.02 to 0.70 +/- 0.05 (P< .0001) but remained significantly better in the ligation and split-flow groups (graft survival, 135 +/- 27 and 144 +/- 31 days). In the banding group metabolic correction decreased significantly after 70 days, and although the grafts kept some function for 155 +/- 14 days, in 4 of 6 dogs portal thrombosis was found. In the free-flow group, competition for the portal blood led to reduced correction within 12 days and total loss of function in 96 +/- 14 days. Graft function also was assessed with technetium (Tc) 99m dimethyl-iminodiacetic acid uptake. A good linear association between HIDA uptake and metabolic correction was observed (r = 0.74; P < .0005). Grafts that contributed more than 15% to the total uptake of HIDA showed biochemical correction. This indicates a critical graft mass of about 15% to 20% of the hepatocyte volume to correct this metabolic defect. CONCLUSION: Auxiliary partial heterotopic liver transplantation can be a valuable alternative treatment for inborn errors of hepatic metabolism if the native liver and the graft receive separate portal blood inflow.

Animals↗

Intussusception in a tropical country: comparison among patient populations in Jakarta, Jogyakarta, and Amsterdam.

BACKGROUND: Intussusception is the most common cause of intestinal obstruction in young children, and high mortality rates remain a problem in developing countries. The purpose of this study was to describe and elucidate the differences in outcome between groups of children with intussusception in Indonesia, a developing country, and The Netherlands, a developed country. METHODS: In this retrospective review, 176 patients were studied in three types of hospitals. A comparison was made among children treated at a primary care rural hospital in Indonesia, at a secondary care urban hospital in Indonesia, and at a tertiary care urban hospital in The Netherlands. RESULTS: Children in the rural community hospital in Indonesia were more severely ill at arrival and had a significantly longer duration of symptoms, an increased incidence of nonviable bowel, and a mortality rate of 20%, in contrast to a mortality rate of 3% in the urban hospital in Indonesia and no deaths in the Dutch hospital. CONCLUSIONS: The mortality of children with intussusception in rural Indonesia is much higher than in urban Indonesia or in The Netherlands, probably because of delayed treatment, which results in more patients undergoing surgery in worse physical condition.

Child↗

[The profile of a good doctor].

What is a good doctor? From the 12th to the 19th century the doctor was mostly an intellectual largely restricted to observing and describing symptoms and diseases. The 19th century saw the beginning of scientific insight into the causes of diseases. However, the primary objective of medical science is not to collect knowledge but to promote health. This involves both knowledge and art. Both arise from talent and education and are best achieved by early practical introduction to the basic subjects and scientific research, and during contacts with prominent teachers.

Education, Medical↗

Long-term psychological evaluation of intersex children.

Treatment of psychological problems of 59 children with a physical intersex condition is described. The group consisted of 18 female pseudohermaphrodites with congenital adrenal hyperplasia (CAH), 20 male pseudohermaphrodites and 2 true hermaphrodites born with ambiguous external genitalia assigned the female sex (ambiguous girls), 14 male pseudohermaphrodites born with completely female external genitalia and assigned the female sex (completely female group), and 5 male pseudohermaphrodites born with ambiguous external genitalia and assigned the male sex. Despite the sex assignment, genital organ correction soon after birth, psychological counseling of parents and intensive psychotherapy of the children, general psychopathology developed equally in all 4 groups (39% of total group). Although 87% of the girls with a physical intersex condition developed in line with the assigned sex, 13% developed a gender identity disorder though only 1 girl (2%) failed to accept the assigned sex. Gender identity disorder and deviant gender role were in evidence only in girls with CAH and girls of the ambiguous group. Biological and social factors seem responsible for the development of gender identity disorder, such as pre- and postnatal hormonal influences on the brain enabling deviant gender role behavior to develop, and an inability on the part of parents to accept the sex assignment. A reconsideration of the sex assignment in male pseudohermaphrodites and true hermaphrodites born with ambiguous external genitalia is discussed.

Adolescent↗

Long-term pulmonary sequelae in children with congenital diaphragmatic hernia.

Neonates with congenital diaphragmatic hernia (CDH) often suffer from respiratory insufficiency due to lung hypoplasia and pulmonary hypertension. Artificial ventilation is frequently required, and this leads to a high incidence of bronchopulmonary dysplasia. Long-term follow-up studies have shown persisting airway obstruction. To evaluate the long-term pulmonary sequelae in CDH, we studied 40 CDH patients of age 7 to 18 yr (median 11.7 yr) and 65 age-matched controls without CDH and lung hypoplasia who underwent similar neonatal treatment. Mild airway obstruction was found in both groups with more peripheral airway obstruction in CDH patients than in control subjects. Both groups had normal TLC and single-breath carbon monoxide diffusion capacity (DLCO). CDH patients had increased residual volume (RV) and RV/TLC compared with controls. Increased airway responsiveness to methacholine (MCH) was common but bronchoconstriction to inhaled metabisulfite (MBS) was rare both in CDH and control subjects. We conclude that this group of CDH patients has minor residual lung function impairment. Mild airway obstruction and increased airway responsiveness to inhaled MCH but not to MBS suggest that structural changes in distal airways are involved and not autonomic nerve dysfunction. Both artificial ventilation in the neonatal period and residual lung hypoplasia seem important determinants of persistent lung function abnormalities in CDH patients.

Adolescent↗

Function of pediatric Nissen-Rossetti fundoplication followed up into adolescence and adulthood.

BACKGROUND: We analyzed the very long-term results of the Nissen-Rossetti fundoplication performed in young children. Little has been reported about follow-up longer than 5 years in homogeneous populations. This study concerns a homogeneous group with a minimum follow-up of 10 years; these former patients, therefore, are now adolescents or adults. STUDY DESIGN: In 24 consecutive patients without other congenital or acquired anomalies of the esophagus or stomach, we evaluated the primary postoperative diagnoses, symptoms of recurrent reflux, and their state of health in 1994. RESULTS: After a median follow-up of 16 years, the result was excellent in 18 patients, good in 5, and poor in I (graded according to Visick). Patients with recurring reflux symptoms had evidence of failure of the fundoplication. All but 1 had been given a diagnosis of recurrent reflux within 2 years after the operation. The situation after 2 years seems predictive for the later outcome. CONCLUSIONS: In almost all cases, the Nissen-Rossetti fundoplication is a long-lasting, effective treatment for young children with symptomatic gastroesophageal reflux.

Adolescent↗

Staff attitudes towards continuation of life-support in newborns with major congenital anomalies.

UNLABELLED: This study was conducted to gain insight into the attitudes of medical staff towards life-support of newborns with life-threatening problems, seen against the background of these children's expected morbidity and quality of life. The opinions about the mode of life-support were determined by questionnaires and the demographic characteristics of the respondents were noted. Each patient's risk of mortality was scored by means of the standardized Paediatric Risk of Mortality Score (PRISM). Attitudes towards support were unanimous for 39 of the 46 patients. For the other 7 patients at least one of the respondents preferred a different support mode than that given at the time. The attitudes were influenced by the patient's risk of mortality. CONCLUSION: Decisions about life-support of newborns with life-threatening problems should include all the disciplines involved in patient care and should be made at an early stage.

Attitude of Health Personnel↗

Prospective evaluation of faecal fatty acid excretion in short bowel syndrome in newborns.

Short bowel syndrome (SBS) in the newborn results in limited intestinal absorptive capacity, leading especially to fatty acid (FA) malabsorption. It is unknown whether adaptation occurs in time in FA absorption, and whether this adaptation is chain-length dependent. The aid of the present study was to prospectively evaluate FA absorption and excretion during SBS in the newborn. Twenty-one neonates who underwent small bowel resection (of variable length) for various reasons (necrotizing enterocolitis, intestinal atresia, meconium peritonitis, cloacal extrophy, etc) were studied. Eight neonates had SBS, defined as a small bowel remnant of less than 50% of the original small bowel length related to gestational age. The mean remaining small bowel length in the SBS group was 34% (24% to 42%). The non-SBS control group consisted of 13 neonates who had only minor small bowel resections. The mean remaining bowel length for the non-SBS group was 95% (70% to 100%). The results show that the total fractional excretion of FA (FE-FA) at 2 weeks and 1, 2, 3, and 4 months postsurgery was 51% +/- 37%, 33% +/- 24%, 51% +/- 65%, 53% +/- 27%, and 7% +/- 2% in patients with SBS, versus 12% +/- 8%, 24% +/- 10%, 9% +/- 3%, 8% +/- 3% and 17% +/- 14% in the non-SBS controls, respectively (P < .05 by ANOVA). There appeared to be an amelioration in time in FA absorption, especially in the SBS group, after 3 months. FE-FA was chain-length related, being considerably less for C10 and C12 than for C14 and longer amounts. An amelioration of absorption occurred in the SBS patients, especially with the longer-chain FA. On the basis of the study data, the authors conclude that in the initial adaptation phase shorter chain lengths are better absorbed than longer chain lengths; however, in the latter FA group, substantial adaptation occurs with time.

Fatty Acids↗

Cloacal exstrophy.

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Abdominal Muscles↗