Thoracic Organ Transplantation in Children: The Sahlgrenska University Hospital Experience

On the basis of the experience acquired from more than 350 thoracic organ transplantations in adults, the outcome of thoracic organ transplantations in the paediatric age group (0-17 years of age) performed consecutively from 1989 to 1998 at our centre was reviewed. Heart transplantation was performed in 27 patients, heart-lung in 6 and bilateral lung transplantation in 2 patients. The preoperative diagnosis included dilated cardiomyopathy in 17 patients, congenital heart defects in 8, hypertrophic cardiomyopathy in 2, cystic fibrosis in 1 and secondary and primary pulmonary hypertension in 5 and 2 patients, respectively. The median age at transplantation and the follow-up period were 12.7, range 0.3-18.2, and 4, range 0.1-9.2 years, respectively. No early deaths occurred after heart transplantation, but one patient died of coronary artery disease 4.8 years after transplantation. One early death occurred one week after heart-lung transplantation as a result of bleeding complications, and another patient died of obliterative bronchiolitis and pulmonary infection 2.5 years after surgery. The remaining patients are alive and have been functionally rehabilitated. In conclusion, despite a relatively small centre volume, paediatric thoracic organ transplantations can be performed with good short- and medium-term survival and good functional status can be achieved by deriving knowledge and experience from transplantations in adults and by collaboration between the various professionals involved in the caring process.     

Adolescents with inflammatory bowel disease feel ambivalent towards their parents' concern for them

This is a grounded theory study to identify concepts for describing how adolescents with inflammatory bowel disease (IBD) respond to their parents' concern for them. Ten adolescent boys and seven girls were interviewed. In the analysis four main categories emerged: ambivalence, ability/inability, compliance/resistance and trust/distrust. We found ambivalence to be the most distinctive theme to appear in the way in which these young people described how they felt about their parents' response to their disease. The core category ambivalence was expressed as an oscillation between seeking close contact with one's parents or, sometimes, staving them off, one moment feeling anxiously dependent upon them or turning to them for protection and support and the next, trying to achieve a dialogue with them. The core category comprised three subcategories, ability/inability, compliance/resistance and trust/distrust. The clinical support for young individuals with IBD should include an awareness of the simultaneous existence of conflicting attitudes, reactions and emotions.     

Laparoscopic repair of a Bochdalek hernia with incarcerated bowel during pregnancy: report of a case

A Bochdalek hernia is a rare type of diaphragmatic defect, generally presenting in early childhood with acute signs of cardiorespiratory distress. We herein report a case of acute abdomen in a pregnant patient with pain increasing when lying on her right side. Laparoscopic evaluation revealed a right-sided Bochdalek hernia with incarcerated bowel. A successful reduction was possible, and laparoscopic repair appears to be a safe and feasible technique for the repair of these hernias.     

Long‐term outcome of intraventricular conduction delays in the general population

BackgroundPrevious population studies have presented conflicting results regarding the prognostic impact of intraventricular conduction delays (IVCD).MethodsWe studied long‐term prognostic impact and the association with comorbidities of eight IVCDs in a random sample of 6,299 Finnish subjects (2,857 men and 3,442 women, mean age 52.8, SD 14.9 years) aged 30 or over who participated in the health examination including 12‐lead ECG. For left bundle branch block (LBBB) and non‐specific IVCD (NSIVCD), two different definitions were used.ResultsDuring 16.5 years’ follow‐up, 1,309 of the 6,299 subjects (20.8%) died and of these 655 (10.4%) were cardiovascular (CV) deaths. After controlling for known clinical risk factors, the hazard ratio for CV death, compared with individuals without IVCD, was 1.55 for the Minnesota definition of LBBB (95% confidence interval 1.04–2.31, p = .032) and 1.27 (95% confidence interval 0.80–2.02, p = .308) for the Strauss’ definition of LBBB. Subjects with NSIVCD were associated with twofold to threefold increase in CV mortality depending on the definition. While right bundle branch block, left anterior fascicular block and incomplete bundle branch blocks were associated with seemingly higher mortality, this was no longer the case after adjustment for age and sex. The presence of R‐R’ pattern was not associated with any adverse outcome.ConclusionsIn a population study with long‐term follow‐up, NSIVCD and Minnesota definition of LBBB were independently associated with CV mortality. Other IVCDs had no significant impact on prognosis. The prognostic impact of LBBB and NSIVCD was affected by the definition of the conduction disorder.     

Pancreatic exocrine insufficiency in LXRbeta-/- mice is associated with a reduction in aquaporin-1 expression

Liver X receptors (LXRs) α and β are nuclear oxysterol receptors with a key role in cholesterol, triglyceride, and glucose metabolism. In LXRβ^−/− mice on a normal diet, there is a reduction in size of perigonadal fat pad and, on high-fat diet there is resistance to obesity. In the present study, we investigated the reason for the resistance of LXRβ^−/− mice to weight gain. In LXRβ^−/− mice we found pancreatic exocrine insufficiency with reduced serum levels of amylase and lipase, reduced proteolytic activity in feces, chronic inflammatory infiltration, and, in the ductal epithelium, an increased apoptosis without compensatory proliferation. Electron microscopy revealed ductal dilatation with intraductal laminar structures characteristic of cystic fibrosis. To investigate the relationship between LXRβ and pancreatic secretion, we studied the expression of LXRβ and the water channel, aquaporin-1 (AQP1), in the ductal epithelium of the pancreas. In WT mice, ductal epithelial cells expressed LXRβ in the nuclei and AQP1 on the plasma membrane. In LXRβ^−/− mice neither LXRβ nor AQP1 was detectable. Moreover, in WT mice the LXR agonist (T2320) increased AQP1 gene expression. These data demonstrate that in LXRβ^−/− mice dietary resistance to weight gain is caused by pancreatic insufficiency and that LXRβ regulates pancreatic exocrine secretion through the control of AQP1 expression. Pancreatic exocrine insufficiency is the main cause of malabsorption syndrome responsible for weight loss in adults and growth failure in children. Several genes are known to be involved in the pathogenesis and susceptibility to pancreatic insufficiency. LXRβ should be included in that list.     

Urine biomarkers for monitoring juvenile lupus nephritis: a prospective longitudinal study

Background

In juvenile-onset systemic lupus erythematosus (JSLE), renal involvement (lupus nephritis) is frequently seen and can result in long-term morbidity. This prospective longitudinal study aimed to identify the utility of standard and/or novel biomarkers for monitoring and predicting lupus nephritis in a real world setting.

Methods

Using an unselected JSLE cohort, urine samples were collected during routine clinical review. Protein concentrations of urinary monocyte chemo-attractant protein 1 (uMCP1) and neutrophil gelatinase-associated lipocalin (uNGAL) were analysed along with standard disease activity markers, and were compared with current and subsequent disease activity.

Results

JSLE patients (n?=?64; median age 14.1 years) were seen at 3 (interquartile range: 2–5) clinical reviews over 364 (182–532) days. Multivariate analysis demonstrated uMCP1 and serum C3 as independent variables (p?<?0.001) for active renal disease at the time of the current review. uMCP1 was an excellent predictor of improved renal disease over time (AUC: 0.81; p?=?0.013). uNGAL was a good predictor of worsened renal disease activity (AUC 0.76; p?=?0.04) over time.

Conclusion

Biomarkers (uMCP1, serum C3) can indicate current renal involvement in JSLE, whilst uMCP1 and uNGAL are able to predict subsequent renal disease activity changes. Moving towards biomarker-led monitoring may improve the renal outcome for our patients.     

Contralateral suppression of otoacoustic emissions in a clinical sample of children with auditory processing disorder

Objective: The suppression of evoked otoacoustic emissions (EOAE) may serve as a clinical tool to evaluate the medial olivocochlear (MOC) reflex, which is thought to aid speech discrimination (particularly in noise) by selectively inhibiting cochlear amplification. The present study aimed to determine if contralateral transient evoked otoacoustic emission (TEOAE) suppression was present in a clinical sample of children with listening difficulties with and without auditory processing disorder (APD).

Design: A three-group, repeated measure design was used.

Study sample: Forty three children aged 8–14?years underwent an auditory processing assessment and were divided into three groups: children with reported listening difficulties with APD, children with reported listening difficulties without APD, and children with normal hearing. APD was defined as per British Society of Audiology.

Results: TEOAE suppression was present in all three participant groups. No significant group, age or ear effects were observed for TEOAE suppression in dB or as a normalised index.

Conclusion: Contralateral TEOAE suppression method could not be used as a clinical tool to identify APD in this study’s participating children and did not support the hypothesised link between reduced MOC function and general listening difficulties in background noise in children with or without APD.