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Biomedical subjects

G C Madern

Publications and source records attributed to G C Madern.

At least 19 recordsLinked to original sources
PubMed2006

Mediastinal germ cell tumors: clinical aspects and outcomes in 7 children.

BACKGROUND: Mediastinal germ cell tumors presenting during childhood are extremely rare. Publications on this entity are very scarce. This paper reports on the clinical presentations, method(s) of treatment, complications, results and outcomes in a series of children with mediastinal germ cell tumors. METHODS: A retrospective chart review of 7 children treated between 1971 and 2001 for mediastinal germ cell tumor was carried out. Age at diagnosis and symptoms were recorded. Each patient's surgical treatment, peri- and postoperative complications, histological staging and final outcome were analysed. RESULTS: The median age of the 4 boys and 3 girls was 3 years (range 21 months-15 years). The most frequent symptoms were respiratory distress, persistent coughing, thoracic pain and anorexia/weight loss. Four patients had histologically benign tumors (mature teratoma). Their sole treatment consisted of complete surgical excision of the tumor and (part of) the thymus using either median sternotomy or left-sided thoracotomy. Recovery was uneventful. No recurrences have been observed. All four are alive with no evidence of disease, between 2.5 and 29 years after treatment. Malignant tumors were observed in three patients (1 yolk sac tumor, 1 choriocarcinoma and 1 malignant teratoma). Treatment consisted of either biopsy or debulking followed by chemotherapy (and radiotherapy in 1 case). Two of them died from uncontrollable metastatic disease. The patient with yolk sac tumor survived; he is now in remission, 4 years after diagnosis. CONCLUSIONS: Both this study and the literature review testify to the extreme rarity of mediastinal germ cell tumors in childhood. Children with this type of tumor usually are severely symptomatic. Histologically benign tumors carry an excellent prognosis provided surgical excision is complete. Histologically malignant tumors, on the other hand, have a worse prognosis. However, the use of platinum-based combination chemotherapy has considerably increased the survival rates.

Adolescent↗
PubMed2001

Quality of life in patients with anorectal malformation or Hirschsprung's disease: development of a disease-specific questionnaire.

PURPOSE: Hirschsprung's disease and anorectal malformation are congenital diseases of the digestive tract with sequelae into adulthood. The quality of life of patients with these diseases is largely unknown. The aim of the study was 1) to construct a self-report disease-specific instrument to assess the quality of life in these patients and 2) to evaluate its psychometric performance. METHODS: An age-specific (6 and 7 years, 8-11 years, 12-16 years, and >17 years) questionnaire called the Hirschsprung's disease/anorectal malformation quality-of-life instrument was constructed. This questionnaire consists of 39 to 42 items, grouped into 10 to 11 scales that cover physical, emotional, and social functions as well as disease-related symptoms. Generic quality-of-life data were obtained in addition. A national sample of 715 patients aged six years and older completed the questionnaire (response rate, 61.9 percent). RESULTS: Multitrait scaling analyses confirmed the hypothesized scale structure with exception of the scales related to diet for the two youngest groups. Cronbach's alpha ranged (with exception of the diet scales) from 0.62 to 0.91 for children (8-11 years), from 0.69 to 0.82 for adolescents (12-16 years) and from 0.57 to 0.91 for adults. Selective scales were able to discriminate between subgroups of adult patients known to differ in disease and disease severity. Relevant scales of the adult version showed substantial correlations (> 0.40) with comparable scales of the SF-36. In the two youngest age groups the differences between subgroups of patients were less significant, but in the expected direction. CONCLUSIONS: With the exception of the scales related to diet, the Hirschsprung's disease/anorectal malformation quality-of-life instrument is an instrument with promising reliability and validity, to measure the disease-specific quality of life of patients with anorectal malformation or Hirschsprung's disease.

Adolescent↗
PubMed2001

Religious circumcision under local anaesthesia with a new disposable clamp.

OBJECTIVE: To compare the results using a new disposable clamp (the Taraklamp Circumcision Device, TCD, Taramedic Europe BV, Bilthoven, The Netherlands), used since 1998 in one clinic, and the conventional dissection technique (CDT) in another clinic, for religious circumcision in infants. SUBJECTS AND METHODS: The TCD and CDT were compared prospectively; the duration of the procedure, complications and postoperative pain were recorded. The cosmetic result and the degree to which the parents were satisfied were evaluated after 6 weeks. After obtaining informed consent, 275 boys were included in the study (median age 3 years). RESULTS: The median operative duration was 8 min less for the TCD (15 vs 7 min; P < 0.001). There was no difference in complication rate (bleeding in one vs two; infection in two vs three) and postoperative pain was comparable in both groups. The cosmetic results were better for the TCD group (P < 0.001). The parents' satisfaction score for the procedure was equal in the groups, at 8, on a scale of 1 (very bad) to 10 (extremely good). CONCLUSION: A religious circumcision outside the hospital with the TCD is quicker and leads to a better cosmetic result than with the CDT, without increasing morbidity.

Anesthesia, Local↗
PubMed2000

Inguinal hernias containing the uterus, fallopian tube, and ovary in premature female infants.

Inguinal hernias were diagnosed at 42 and 38 weeks' postconceptional age in 2 premature girls. The hernial sac contained the uterus, one Fallopian tube, and one ovary. The diagnosis was made by physical and sonographic examination and was confirmed during surgical correction. We suggest sonography in the diagnostic workup in (premature) female infants with an inguinal hernia.

Fallopian Tubes↗
PubMed2000

Treatment and pain relief of ulcerative hemangiomas with a polyurethane film.

BACKGROUND: Hemangiomas are the most common tumors occurring in young children. The most common complication in the growing phase of hemangioma is ulceration. AIM AND METHOD: We report healing, pain relief and evolutive effects of a polyurethane film in 8 cases with ulcerative hemangiomas. RESULTS: In all 8 infants, prompt pain relief and healing within 1-2 months were observed. An increased regression was also noted within 2-4 months, when the hemangiomas were in the normal proliferative phase. CONCLUSION: As far as the authors know, there is no explanation for the effectiveness of polyurethane film. Explanations could be the occlusive effects of the film inhibiting proliferation or the decrease in blood flow. As primary initial therapeutic approach in ulcerative hemangiomas, we advocate the application of a polyurethane film. This therapy is painless and suitable for children.

Female↗
PubMed1999

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↗
PubMed1997

[Favorable results of percutaneous endoscopic gastrostomy in children in need of long-term tube feeding].

OBJECTIVE: Analysis of indications, weight and peroperative and postoperative complications in 32 consecutive children who underwent percutaneous endoscopic gastrostomy (PEG). DESIGN: Retrospective. SETTING: Sophia Children's Hospital, Rotterdam, The Netherlands. METHOD: Survey of medical records. RESULTS: In 26 children the indication for PEG was necessity of prolonged tube feeding on account of severe psychomotor retardation. The 6 others had no such retardation and had various indications. Mean hospital stay was 6.7 days, median 6 days. Three patients had immediate postoperative complications: two had leakage, one infection. In 22 patients there were no late complications. One patients died following status epilepticus. One patient had a late peritonitis, and two others gastroesophageal reflux severe enough to require surgical intervention. Six patients had mild long-term complications, mainly infectious. All children showed improvement of weight for length. CONCLUSION: The PEG procedure is well-tolerated, effective way of tube feeding in children, with a relatively low complication rate.

Adolescent↗
PubMed1994

Rectoperineal fistula in newborn boys.

Three newborn boys presented with features suggestive of classic low anorectal abnormality. However, during surgery they were found to have an intermediate anorectal abnormality and a rectoperineal fistula rather than an anocutaneous fistula. The surgical treatment of these infants is discussed.

Anus, Imperforate↗
PubMed1993

[Liver rupture in a newborn infant; diagnosis, treatment and prognosis].

Between August 1989 and August 1992 four neonates with rupture of the liver were admitted to the Neonatal Intensive Care Unit of the Sophia Children's Hospital in Rotterdam, the Netherlands. Two neonates were born after breech delivery, two after caesarean section because of foetal distress. All four patients had Apgar scores < 5 after 1 minute and of < 8 after 5 minutes and required artificial ventilation for a prolonged period. All infants collapsed within 6 hours after birth. Surgical treatment was not considered because of the poor clinical condition. All patients were treated conservatively. Clinical signs were: rapid onset pallor, hypotension, tachycardia and abdominal distension. Ultrasonography of the abdomen confirmed the clinical diagnosis of rupture of the liver. Despite rapid diagnosis and maximal non-surgical treatment mortality was 75%. Surgical intervention is indicated in neonatal liver rupture with significant intra-abdominal bleeding.

Female↗
PubMed1991

Evaluation of mortality in surgical neonates over a 10-year period: nonpreventable, permissible, and preventable death.

The mortality pattern and causes of death in surgical neonates were evaluated over the period of 1980 through 1990 in order to assess whether any deaths might have been prevented. In the study period 1,010 neonates were admitted, 104 of whom died (10%). These 104 neonates formed our study population. The Clinical Classification System (CCS) and the Therapeutic Intervention Scoring System (TISS) were used to assess the severity of illness. All 104 nonsurvivors were seriously ill (CCS 3 and 4; TISS greater than 20). The mortality pattern was classified in three groups. Group A (nonpreventable death) included 56 deaths, 48 of which were due to the underlying disease. The other 8 patients died of a nonpreventable complication of treatment. Eleven of the patients in group B (permissible death) had (multiple) congenital anomalies associated with chromosomal anomalies. Three of them were not treated and in the other 8 treatment was initially started but later withdrawn. In most of the other 14 patients further treatment was withdrawn because of serious postoperative complications or inoperable cardiac anomalies. Group C (preventable death) consisted of 23 patients. Six of 10 had irreversible brain damage due to prolonged hypoxemia. In the other 13 patients of group C, death was due to sepsis acquired in the postoperative period and treated inadequately. Staphylococcus epidermidis was the most frequent isolated pathogen (7/13).

Cause of Death↗
PubMed1991

Sequential changes in relative liver function assessed by 99mTc-HIDA scintigraphy after auxiliary heterotopic liver transplantation in dogs.

Auxiliary heterotopic liver transplantation (HLT) was used to achieve functional repair in a dog model with an inborn error of metabolism. For the interpretation of the results, information on separate liver function is essential when a normal host liver is also present. We developed a radionuclear method to quantitate the relative contribution of each liver to the total uptake of intravenously (IV) injected 99mTc-HIDA. The HLT was performed between 20 mismatched pairs of dogs from two different strains. Four surgical procedures were used. After autopsy the outcome of the premortem HIDA-scan was compared with the wet weight of the graft and the host liver. A good linear correlation was noted between the relative contribution of the uptake and weight of the graft to the total HIDA uptake and total liver weight. Therefore, the relative contribution of an auxiliary heterotopic liver graft to the total liver function can be quantitated with a 99mTc-HIDA scan. With this technique, changes in relative function after an HLT under various flow conditions can be sequentially followed.

Animals↗
PubMed1989

Pre-operative stabilisation with delayed repair in congenital diaphragmatic hernia.

Congenital diaphragmatic hernia (CDH) remains one of the major challenges for paediatric surgeons and paediatric intensive care specialists. Death in patients with CDH is 30-60% worldwide due to severe pulmonary hypoplasia or pulmonary hypoplasia associated with persistent pulmonary hypertension, secondary to hypoxia, metabolic acidosis or myocardial insufficiency. Pre-operative stabilisation of CDH patients might reduce the risks of these complications. In a two-year period 16 high-risk patients with CDH (respiratory insufficiency less than 6 hrs after birth) underwent delayed surgery following a stabilisation period (mean 14 hrs). Continuous suctioning on a nasogastric tube resulted in total resolution of the mediastinal shift on repeat x-rays. The use of the ventilation parameters arterial alveolar oxygen gradient (A-aDO2), oxygenation index (OI) and mean airway pressure (MAP) revealed three different groups of patients: I consisting of 6 survivors, II two preventable deaths and III eight non-survivors. In this way selection of patients with CDH is possible. In patients who do not improve during the stabilisation period alternative ways of treatment have to be evaluated such as extracorporeal membrane oxygenation (ECMO), high frequency oscillation (HFO) or high frequency jet ventilation (HFJV). Application of the ventilation parameters in prospective trials of patients with CDH enables comparison between different ways of treatment in the future.

Blood Pressure↗
PubMed1988

Congenital diaphragmatic hernia: impact of preoperative stabilization. A prospective pilot study in 13 patients.

In case of congenital diaphragmatic hernia (CDH), survival generally depends not on prenatal diagnosis, planned delivery, and immediate postnatal operation, but on the gravity of pulmonary hypoplasia and persistent hypertension (PPH). Many vasoactive drugs have become available for lowering PPH, but the mortality rate for CDH still amounts to 40% to 70%. Preoperative stabilization might prevent or at least reduce the risk of PPH. This method was evaluated in a pilot study lasting 15 months and involving 13 patients. All were admitted to the pediatric surgical intensive care unit within six hours of birth, all requiring mechanical ventilation. Continuous suction of the stomach and bowel proved successful in reducing the mediastinal shift. Study parameters were alveolar-arterial oxygenation differences ((A-a)DO2), mean airway pressure (MAP), oxygenation index (OI), and ventilation index (VI), measured on admission and at set times before and after surgery. Eight patients did not survive, but in two cases death was not directly related to CDH. The following conclusions were reached: (1) satisfactory ventilation parameters on admission will remain good during the preoperative stabilization phase and will not be affected by its duration or by subsequent surgery, spelling survival; (2) unsatisfactory ventilation parameters on admission may improve with preoperative stabilization, giving these patients a better chance of survival; and (3) poor ventilation parameters on admission that fail to improve with preoperative stabilization will not improve with surgery or postoperatively, spelling death.

Female↗