[Liver transplantation in paediatric patients in the Netherlands; evolution over the past two decades]. / Levertransplantaties bij kinderen in Nederland.

OBJECTIVE: To evaluate the results of the national paediatric liver transplantation programme in the University Medical Centre (UMC) Groningen in the Netherlands during the past two decades. DESIGN: Retrospective cohort study. METHOD: We analysed data from paediatric patients who underwent liver transplantation at UMC Groningen in the period 1995-2016. We compared outcomes from children who had undergone a liver transplantation in the period 1995-2005 (cohort A; n = 126) and in the period 2006-2016 (cohort B; n = 169). We performed a subanalysis in cohort B between liver transplantations with deceased donor livers (n = 132) and living donor liver transplantations (LDLT; n = 37). RESULTS: In cohort A, almost all livers came from deceased donors (99%), whereas in cohort B, 37 LDLTs (22%) were performed. The median age of recipients was significantly higher in cohort A (4.4 vs. 2.5 years; p = 0.015). Postoperative complications were comparable for both cohorts. Re-transplantations within a year after transplantation were more often performed in cohort A than in cohort B (25% vs. 12%; p = 0.004). Following LDLT, there was 2 times (5.4%) an indication for re-transplantation. In cohort B the 5-year survival rate was better than in cohort A (83 vs. 71%; p = 0.014). In cohort B, 5-year survival was higher after LDLT than after transplantation with a deceased donor liver (95 vs. 81%; p = 0.025). CONCLUSION: Outcomes after paediatric liver transplantation in the Netherlands have further improved during the past two decades. With an actuarial 5-year survival of 83% in the most recent cohort, and as high as 95% following LDLT, we can say that the UMC Groningen has a successful national paediatric liver transplant programme.

Endotracheal temperature and humidity measurements in laryngectomized patients: intra- and inter-patient variability.

UNLABELLED: This study assesses intra- and inter-patient variability in endotracheal climate (temperature and humidity) and effects of heat and moister exchangers (HME) in 16 laryngectomized individuals, measured repeatedly (N = 47). Inhalation Breath Length (IBL) was 1.35 s without HME and 1.05 s with HME (P < 0.0001). With HME, end-inspiratory (minimum) humidity values increased 5.8 mg H(2)O/L (P < 0.0001) and minimum temperature values decreased 1.6 degrees C (P < 0.0001). For the temperature and humidity minimums, the inter-patient variability was much smaller than the short- and long-term intra-patient variability. For exhalation breath length and full breath length, the opposite was the case. CONCLUSIONS: (1) Because inter-patient variability is smaller than intra-patient variability, investigating endotracheal climate in a limited number of laryngectomized subjects is justified, provided repeated measurements per patient are accomplished; (2) main contributor to intra-patient variability is the positioning of the catheter tip in the trachea; (3) an HME leads to a shortened IBL which enhances the HME effect.

[Non-alcoholic fatty liver disease in children: a new complication of obesity]. / 'Non-alcoholic fatty liver disease' bij kinderen: een nieuwe complicatie van obesitas.

Non-alcoholic fatty liver disease (NAFLD) comprises a range of chronic liver diseases from simple steatosis to steatohepatitis and cirrhosis with liver failure. In children, NAFLD is mainly associated with obesity and metabolic syndrome, the results of an unhealthy lifestyle. Insulin resistance and free fatty acids play a key role in the pathogenesis of NAFLD. NAFLD can therefore be seen as a metabolic complication of obesity. Since the prevalence of obesity in Dutch children is increasing, the prevalence of NAFLD in children is expected to increase as well. Prevention of obesity and identification of children with an increased risk of NAFLD are important steps in preventing irreversible liver damage. Lifestyle changes aimed at improving insulin sensitivity through healthy food and sufficient physical activity are essential in the treatment of NAFLD. Pharmacological treatment may have additional value.

[A patient with both cocaine-induced nasal septum destruction and antibodies against anti-neutrophil cytoplasmic antibodies (ANCA); potential confusion with Wegener's disease]. / Een patiënt met neusseptumdestructie door cocaïne en antistoffen tegen cytoplastische antigenen van neutrofiele granulocyten (ANCA); mogelijke verwarring met de ziekte van Wegener.

A 37-year-old male cocaine user presented with continual, sanguinolent nasal obstruction and persistant pain following a nasal operation one year ago. Examination showed crustae, granulations and exposed septal cartilage in the right nasal passage in addition to a considerable septal deviation to the left. No other physical abnormalities were found. A biopsy of the nasal mucosa showed acute necrotic inflammation. The serological examination revealed markedly elevated anti-neutrophil cytoplasmic antibodies (ANCA) titres with positive reactions against proteinase-3, indicating Wegener's disease. Additional testing also showed a positive ANCA reaction for human neutrophil elastase, which made cocaine use a more plausible cause for the nasal abnormalities than Wegener's disease. Treatment consisted of nasal flushing with saline and, for a short period, a nasal tampon with hydrocortisone-oxytetracycline-polymyxin B ointment. However, the patient did, ultimately, develop a septal perforation. Cocaine-induced nasal abnormalities can imitate symptoms that may fit Wegener's disease, including relevant serological ANCA findings.

[Serious haemorrhage after conventional (adeno)tonsillectomy: rare and most often on the day of the procedure]. / Ernstige nabloeding na klassieke (adeno)tonsillectomie: zeldzaam en meestal op de dag van de ingreep.

OBJECTIVE: To analyse the incidence and timing of postoperative haemorrhage that requires re-operation following tonsillectomy or adenotonsillectomy, and to formulate a recommendation regarding the optimal duration of postoperative clinical observation. DESIGN: Retrospective. METHOD: Data were collected on the incidence of haemorrhage that required exploratory surgery in all patients who underwent adenotomy (n=3508) or tonsillectomy or adenotonsillectomy (n=4909) in the period 1996-2002 at the Sint Lucas Andreas Hospital in Amsterdam, the Netherlands. The Sluder technique was used on an outpatient basis in 2439 of the 4909 patients who underwent tonsillectomy or adenotonsillectomy; these patients were aged <10 years. The remaining 2470 patients were aged > or =10 years and underwent conventional dissection followed by 24 hours of clinical observation before being discharged. RESULTS: Postoperative haemorrhage was recorded in 0 of the 3508 patients who underwent adenotomy, 12 (0.5%) of the 2439 patients aged <10 years who underwent tonsillectomy using the Sluder technique and 43 (1.7%) of the 2470 patient aged > or =10 years who underwent conventional dissection. Of the 43 cases of haemorrhage after conventional dissection, 31 (75%) occurred within 24 hours, including 2 cases that occurred between 12 and 24 hours (2/2470; 0.08%; 95% CI: 0.001-0.29). CONCLUSION: Reducing the postoperative observation period from 24 hours to 12 hours would have unfavourable consequences in 1 (95% CI: 0-3) per 1ooo patients operated. In regard to the risk of postoperative haemorrhage, outpatient tonsillectomy and adenotonsillectomy appear to be justified for patients aged 10 years or over, provided that surgery is performed in the morning and the patient is observed in a ward that is open until the evening.

[Active management of children after ingestion of a button battery]. / Actiefbeleid bij een kind dat een knoopbatterij heeft ingeslikt.

Four children, three girls in the age range up to 14 months and a boy aged 10 years, were admitted because of button battery ingestion. In two patients, the course was uncomplicated, with spontaneous passage of the batteries. Two other patients, a girl aged 11 months and a girl aged 6 weeks, developed severe complications: stenosis of the oesophagus in one patient and a dramatic clinical course with a tracheo-oesophageal fistula and oesophageal damage in the other. Ingestion of foreign bodies in children is a common problem. With the increased use of miniature electronic devices, the incidence of button battery ingestion is rising. Ingestion of a battery is an indication for urgent referral and radiological examination. Electrochemical tissue damage and impaction may lead to serious complications within hours. If the battery is located in the oesophagus, endoscopic removal should be attempted as soon as possible. A conservative approach can be followed when the battery is located in the stomach or beyond, and complaints are absent.

The value of prospective monitoring of Epstein-Barr virus DNA in blood samples of pediatric liver transplant recipients.

UNLABELLED: Post-transplant lymphoproliferative disease (PTLD) is one of the major causes of morbidity and mortality in transplantation patients. A primary Epstein-Barr virus (EBV) infection is a major risk factor for developing PTLD. The aim of this study was to determine circulating EBV DNA after liver transplantation in pediatric patients in relation to primary EBV infection and development of PTLD. EBV serology was performed before transplantation. Every 4 weeks after transplantation a competitive quantitative polymerase chain reaction (PCR) assay for EBV nuclear antigen-1 was performed in 13 patients. Patients were followed for development of a PTLD. Before transplantation four patients were EBV seropositive and nine patients were EBV seronegative. In one of the four patients who were EBV seropositive before transplantation, EBV DNA became detectable after transplantation, with a peak load of 3600 copies/mL. None of these four patients developed a PTLD. Eight of the nine patients who were EBV seronegative before transplantation developed positive EBV DNA samples. EBV DNA was first detected at a mean of 64 days after transplantation (range 38-89). The mean peak EBV DNA load was 79,700 copies/mL (3600-446,000). Two of these patients developed PTLD, but they could not be identified based on prior or concomitant EBV PCR results. CONCLUSIONS: In pediatric liver transplantation EBV DNA load is higher in patients with a primary infection than in patients who were EBV seropositive before transplantation. The EBV PCR cannot be used to identify individual patients who develop PTLD. However, elevated EBV DNA load can be used to detect a group of patients at increased risk for PTLD.

Rapidly progressive adenomatous polyposis in a patient with germline mutations in both the APC and MLH1 genes: the worst of two worlds.

The two most common inherited forms of colorectal cancer are familial adenomatous polyposis and hereditary non-polyposis colorectal cancer. Simultaneous inheritance of both an APC gene mutation and a mismatch repair gene (for example, MLH1) mutation has never been described. In the present case report, we report rapidly progressive adenomatous polyposis in a 10 year old boy with a germline frame shift mutation in the APC gene and a germline splice site mutation in the MLH1 gene. Immunohistochemical investigations showed abnormal expression of beta-catenin in early adenomas with low grade dysplasia, attributed to the APC gene mutation. Subsequent loss of function of the MLH1 gene, as shown by absent immunostaining of its protein in adenomas with high grade dysplasia, may well have caused the rapid progression to high grade dysplasia in many of the adenomas.

Neonatal neurological morbidity in relation to obstetrical and social conditions in Grenada (Caribbean).

Neonatal morbidity, in particular neurological morbidity is a more relevant measure of the effectiveness of obstetrical care than perinatal mortality. Neurological morbidity was assessed in a birth cohort in Grenada, and appeared to be lower than in a reference group examined in Groningen, the Netherlands, in 1975-1978. Perinatal mortality, however, was higher. The results support the thesis that some children may have died who, if they would have survived, would have been neurologically abnormal. It is concluded that whereas a decrease in perinatal deaths is an essential goal in Grenada, a concomitant increase in morbidity should be carefully avoided.

Neonatal neurological morbidity in relation to obstetrical and social conditions in Grenada (Caribbean)

Neonatal morbidity, in particular neurological morbidity is a more relevant measure of the effectiveness of obstetrical care than perinatal mortality. Neurological morbidity was assessed in a birth cohort in Grenada, and appeared to be lower than in a reference group examined in Groningen, the Netherlands, in 1975-1978. Perinatal mortality, however, was higher. The results support the thesis that some children may have died who, if they would have survived, would have been neurologically abnormal. It is concluded that whereas a decrease in perinatal deaths is an essential goal in Grenada, a concomitant increase in morbidity should be carefully avoided. (AU)