PubMed Health⌕ Search

Biomedical subjects

F H van der Staak

Publications and source records attributed to F H van der Staak.

16 recordsLinked to original sources

Remodeling of pulmonary arteries in human congenital diaphragmatic hernia with or without extracorporeal membrane oxygenation.

PURPOSE: The aim of this study was to describe in detail the perinatal developmental profile of the pulmonary vasculature in congenital diaphragmatic hernia (CDH) and to examine the potential beneficial effects of extracorporeal membrane oxygenation (ECMO) on the vascular morphology. Additionally the authors aimed to identify the differences in pulmonary vascular morphology among CDH cases according to the primary cause of death: either extreme lung hypoplasia (LH) or persistent pulmonary hypertension (PPH). METHODS: The authors studied autopsy sections from 30 high-risk CDH cases with respect to the pulmonary arteries in relation to gestational age (GA) and ECMO treatment. They were grouped into CDH-I: 20 cases with GA greater than 34 weeks who were not subjected to ECMO and CDH-II: 10 cases with GA greater than 34 weeks, who were subjected to ECMO for an average time of 237 hours. Five age-matched neonates who died from placental insufficiency or birth asphyxia without evidence of lung hypoplasia served as controls (CON). Medial and adventitial thicknesses of pulmonary arteries were measured in lung sections stained with Elastic van Gieson by 2 investigators blinded for the clinical data. Immunohistological staining with anti-alpha-smooth muscle actin (alpha-SMA) was performed to confirm the precise location of the arterial media before morphometry. CDH cases were subgrouped and compared according to the primary cause of death. Unpaired Student t test was used for statistics, with significant P value < or =.05. RESULTS: In CDH newborns, a significant increase in medial, adventitial, and total wall thickness was found in pulmonary arteries with an external diameter of less than 200 microm as compared with age-matched controls (P<.004, .0001, and .0009, respectively). ECMO-treated CDH newborns showed a significantly thinner arterial adventitia than CDH patients who did not receive this treatment (P<.0001), approaching normal values. However, the medial thickness remained increased. Morphometrically, no significant differences in CDH cases between patients dying of PPH or severe LH could be determined. CONCLUSIONS: (1) In CDH, there is failure of the normal arterial remodeling processes occurring in the perinatal period. (2) Pulmonary vascular morphology in CDH does not differ between the groups with lung hypoplasia or persistent pulmonary hypertension as primary cause of death. (3) Adventitial thinning of these arteries might be one of the mechanisms by which ECMO alters PPH in CDH cases.

Extracorporeal Membrane Oxygenation↗

[Results of extracorporeal membrane oxygenation in 100 newborns with cardiorespiratory insufficiency].

OBJECTIVE: To describe the results of treatment with extracorporeal membrane oxygenation (ECMO) in newborns with severe cardiorespiratory insufficiency. DESIGN: Prospective, descriptive. METHODS: For all 100 newborns treated with ECMO in 1989-1997 in the Academic Hospital Nijmegen, department of Neonatology, the Netherlands, indications for treatment, complications during treatment and mortality within 6 weeks after cessation of ECMO were registered. RESULTS: The 100 children comprised 66 boys and 34 girls, with a mean age of 2 days (range: 1-15). Indications for ECMO treatment were: meconium aspiration syndrome: 39 with 37 survivors (95%), congenital diaphragmatic hernia: 31 with 23 survivors (74%), sepsis or pneumonia: 20 with 14 survivors (70%) and 'others' among which persistent pulmonary hypertension of the newborn: 10 with 8 survivors (80%). Eighteen children died (18%). Causes of death were rebound pulmonary hypertension (9 times), intracranial haemorrhage (4), multi-organ failure (3) and pulmonary problems (2). The most important complications during treatment were bleeding problems (29), clotting problems (20) and infections (11).

Cause of Death↗

Surgical repair of congenital diaphragmatic hernia during extracorporeal membrane oxygenation: hemorrhagic complications and the effect of tranexamic acid.

Extracorporeal membrane oxygenation (ECMO) was incorporated in a strategy of delayed repair of congenital diaphragmatic hernia (CDH) and was used for preoperative stabilization in patients who were unresponsive to maximal conventional treatment. If ECMO was required for preoperative stabilization the diaphragmatic defect was repaired while the patient was on ECMO. In the early experience with this approach all patients suffered from bleeding complications. Therefore, we adopted the use of antifibrinolytic therapy with tranexamic acid (TEA) during and immediately after CDH repair on ECMO. The efficacy of TEA was studied in an unblinded study using historical controls by comparing the postoperative blood loss and the transfusion requirements of red blood cells (RBC) in patient groups treated without (n = 9) and with TEA (n = 10). Patients who received TEA had significantly less bleeding at the surgical site than patients not receiving TEA (57 v 390 mL, P = .005) and had significantly lower RBC transfusion requirements than patients not receiving TEA (1.13 v 2.95 mL/kg/h, P = .03). In the very first two patients of the TEA group we encountered fairly severe thrombotic complications. TEA may have contributed to those complications. Based on the authors' experience they conclude: (1) TEA is effective in reducing postoperative blood loss, hemorrhagic complications, and RBC transfusion requirements associated with CDH repair on ECMO. (2) TEA may be responsible for thrombotic complications. (3) The appropriate, empirically established, dosage and administration patterns of TEA for CDH repair during ECMO seem to be one bolus of 4 mg/kg TEA intravenously 30 minutes before the anticipated CDH repair and a continuous infusion of 1 mg/kg/h TEA during the 24 hours after CDH repair.

Antifibrinolytic Agents↗

The midline cervical cleft.

The midline cervical cleft is a rare congenital anomaly of the ventral neck. With reference to two patients with a midline cervical cleft, we discuss the clinical picture, the embryology, and the surgical treatment.

Female↗

Long-gap esophageal atresia: experience with reconstruction in 25 patients.

In a series of 183 patients with esophageal atresia in a period of 20 years, 25 had a "long-gap" esophageal atresia. In most of these cases we succeeded in saving the patients' own esophagus but at the cost of one to two years of intensive treatment. The overall survival was 80%. The result compares favorably with the results of esophageal replacement reported in the literature.

Anastomosis, Surgical↗

Subcutaneous rheumatoid nodules.

We describe seven children with subcutaneous rheumatoid nodules who had no clinical evidence of rheumatoid arthritis. Only one girl was seropositive for antinuclear factors and had a slightly raised erythrocyte sedimentation rate. Clinical aspects, risks for developing rheumatoid arthritis, and treatment of this entity are discussed.

Child↗

Management of pediatric esophageal perforation.

Esophageal perforation is a serious complication necessitating immediate therapy. In a retrospective study we have evaluated the results in 13 children treated for esophageal perforation. Eleven of 13 perforations could be managed conservatively. In one child with extrapleural effusion, tube drainage was performed. The only death in this series occurred in a child who was brought for treatment after a 60-hour delay. Thoracotomy and multiple abscess drainage eventually proved unsuccessful. On the basis of our experience with children with esophageal perforation or with complications after esophageal atresia repair, we conclude that management of esophageal perforation in children differs substantially from therapy in adults and necessitates restrictive treatment guided by clinical symptoms.

Anti-Bacterial Agents↗

[Developments in management of esophageal atresia].

The results of treatment of oesophageal atresia show that attentiveness and knowledge of the referring paediatrician concerning diagnostics, preoperative care and transport, together with concentration of high-standard care and specific experience in centres for paediatric surgery, can lead to a substantial decrease in morbidity and mortality. Between 1955 and 1968 a total of 193 children have been operated for oesophageal atresia. Mortality has declined from 51% in the period 1955-1969 to 10% in the timespan 1980-1986. Mortality is almost solely determined by additional congenital malformations, in which congenital heartdefects form the major cause. Management of oesophageal atresia seems to have evolved to a routine-procedure. New trends in paediatric surgery pose new sorts of specific problems, that require attention.

Esophageal Atresia↗

[Gastroesophageal reflux].

Gastro-oesophageal reflux (GER) in children, causes sometimes aspecific symptoms in children. Not only in the case of regurgitation but also in respiratory problems or even in the 'near missed' - Sudden Infant Death Syndrome GER is to be considered. The most important method of investigation is pH-monitoring during 24 hours preceded by a barium meal and followed by endoscopy, in most cases. Operative treatment is necessary for a hiatal hernia, or after failure of conservative treatment of an oesophagitis. In mentally retarded children, who continue to vomit, a fundoplication can be necessary to improve general care.

Barium Sulfate↗

[Simultaneous bilateral Wilms' tumors].

Wilms' tumors occur in 4-14% bilaterally, partially simultaneously, partially metachronously. Therefore in every case with Wilms' tumor the contralateral kidney has to be examined thoroughly too. 5 children of a series of 52 patients with Wilms' tumor showed simultaneous bilateral occurrence (9,6%). The radiological procedures and their importance in diagnosis of bilateral Wilms' tumor are discussed. The prognosis of simultaneous bilateral Wilms' tumors is much better than that of metachronous ones and almost the same as that of unilateral ones.

Child, Preschool↗

Reliability of the acetylcholinesterase (ACE) reaction in rectal mucosal biopsies for the diagnosis of Hirschsprung's disease.

The ACE reaction in superficial rectal biopsies is a valuable diagnostic procedure for Hirschsprung's disease. Although the literature mentions a reliability as high as 100 percent accuracy, a false negative ACE reaction can occur. So the result of the histochemical examination can never be seen apart from the clinical picture. This paper deals with false-negative ACE reactions in 3 children. A false-negative ACE reaction is suggested to be caused by a developmental disturbance in the 6th or 7th embryonal week. Since this disturbance can be responsible also for an aganglionosis of the total colon and a part of the ileum, the chance of a false negative ACE reaction is postulated to be increased in an extensive aganglionosis in precisely those cases which are already a diagnostic challenge for the clinician.

Acetylcholinesterase↗

Recirculation in double lumen catheter veno-venous extracorporeal membrane oxygenation measured by an ultrasound dilution technique.

Recirculation is a limiting factor for oxygen delivery in double lumen catheter veno-venous extracorporeal membrane oxygenation (DLVV-ECMO). This study compares three different methods for the determination of the recirculation fraction during double lumen catheter veno-venous ECMO at ECMO flow rates of 150, 125, 100, 75, and 50 ml/kg.min in nine lambs: (1) an ultrasound dilution method, in which the change in ultrasound velocity in blood after injection of a saline bolus as a marker is used for determination of recirculation; (2) an SvO2 method using real mixed venous blood oxygen saturation, the gold standard, for determination of recirculation fraction; and (3) the CVL method, in which oxygen saturation of a blood sample of the inferior vena cava is considered to represent mixed venous oxygen saturation. In all methods, the recirculation fraction increased with increasing ECMO flow rate. The correlation coefficient between the ultrasound dilution method and the SvO2 method was 0.68 (p < 0.01); mean difference was -2.4% (p = 0.6). Correlation coefficient between the ultrasound dilution method and the CVL method was 0.48 (p < 0.01); mean difference was -18.1% (p < 0.01). The correlation coefficient between the SvO2 method and the CVL method was 0.51 (p < 0.01); mean difference was -15.7% (p < 0.01). The ultrasound dilution method is a useful method for measurement of the recirculation fraction in DLVV-ECMO and is easier to use than the other methods.

Animals↗