Snapshots reflecting the lives of siblings of children with autism spectrum disorders.

BACKGROUND: Past research focused on the effects of raising a child with autism spectrum disorder on families. However, most research examined parents' perspectives rather than siblings' perspectives. Therefore, the purpose of this qualitative descriptive design was to use photo elicitation to capture perspectives of siblings living with a child with autism spectrum disorder. METHODS: Fourteen siblings (nine male) of 13 children with autism spectrum disorder received disposable cameras with 24-27 colour exposures, and were asked to photograph what was important to them within 2 weeks. After developing snapshots, investigators interviewed siblings about their photographs, and used open, axial and selective coding to determine photograph categories and subcategories. RESULTS: Two major categories were found: people (family members, non-family members) and non-people (personal items/objects, animals, buildings, scenery). Interviews about photographs reflected experiences siblings had with people/non-people in the snapshots and their normal everyday activities. Most photographs revealed family life and activities any sibling would experience whether or not they lived in a family raising a child with autism spectrum disorder. CONCLUSIONS: Photo elicitation facilitates communication between children and health-care professionals, and provides information about living with a child with autism spectrum disorder from the sibling's perspective. This information contributes to our knowledge base and allows development of specific intervention plans for siblings of these children.

[The endocrine effects of smoking]. / Le tabac et ses effets sur le système endocrinien.

Almost one third of men and women smoke in Belgium. Besides the well known tobacco's neck and cardiopulmonary systems adverse effects as well as associated neoplasms, today we recognize other deleterious consequences of tobacco on the neuroendocrine, thyroid and reproductive systems. Not only active smokers but also the fetus carried by a smoking mother is at risk for important health problems. Tobacco is a recognized risk factor of occurrence of ophtalmopathy. Some of the active components of tobacco as the thiocyanates are goitrogenic. Tobacco is a risk factor for men and women's infertility. Newborns from parents that smoke are at risk for sudden death. These consequences represent a major public health issue. A campaign for smoking cessation has been recently launched by the Federation of Public Health Service and the INAMI in Belgium.

Malignancy and leg ulceration in a community-based leg ulcer clinic in New Zealand.

The high prevalence of non-melanoma skin cancers in this patient population highlights the need for early, accurate diagnosis. This can be achieved by biopsying atypical ulcers early and all chronic ulcers within three months of starting treatment.

Factors influencing concordance with compression stockings after venous leg ulcer healing.

OBJECTIVE: To investigate the factors influencing patient concordance with compression stockings after venous leg ulcer healing. METHOD: In 2001 patients discharged from a specialist leg ulcer service in New Zealand were approached to participate in a structured interview about their use of compression stockings in the first six months following venous leg ulcer healing. Univariate analyses were performed to identify significant associations between factors and stocking use. Multiple logistic regression was employed to model the factors associated with frequency of stocking use. RESULTS: A total of 163 patients were identified, of whom 129 (79%) agreed to participate. Sixty-seven (52%) reported wearing stockings every day for the first six months after their ulcers had healed, 21 (16%) stated that they had worn the most days, seven (5%) had worn them occasionally and 29 (22%) had not worn them at all after their ulcer had healed. Five (4%) did not provide data. Two factors distinguished those who wore stockings from those who did not 75% of the time: the belief that wearing stockings was worthwhile and the belief that stockings were uncomfortable to wear. Commonly cited factors, such as age, sex, difficulty in applying stockings and cosmetic appearance were not significantly related to stocking use. Cost did not seem to influence the decision to purchase compression stockings. CONCLUSION: Many factors previously thought to influence concordance appear to have little impact on compression-stocking use in people whose venous leg ulcers had healed.

Transplantation procedures in primary hyperoxaluria type 1.

Primary hyperoxaluria type 1 (PH 1) is complicated by a high rate of early end-stage renal failure (ESRF). In ESRF combined liver kidney transplantation has emerged as treatment of choice for teenagers and adults. In chronic renal failure (CRF) and for small children the situation is less clear. We report on three isolated liver transplantations and show the data of young children from the European Registry for liver transplantation in PH 1. Patient #1 developed ESRF at 3 months of age. Deficiency of alanine:glyoxylate aminotransferase proved PH 1. Progressive bone disease developed and the boy received a living related liver graft (LRLTx) at age two. Due to recurrent cholangitis kidney transplantation (KTx) is currently not feasible. Plasma oxalate decreased after LRLTx indicating correction of the metabolic defect. Patient #2 was diagnosed at the age of 14 months. He had nephrocalcinosis and hyperglycolic hyperoxaluria. Two years later he developed ESRF. At 5 years of age isolated liver transplantation was performed as a first step of therapy. Due to prolonged warm ischemia time organ function was poor. A severe bleeding complicated the course. The child died four weeks after transplantation from untreatable CMV septicemia. Patient #3 was evaluated for failure to thrive at 6 months of age. Urinary oxalate/creatinine ratio was 705 mumol/mol and gave rise to the diagnosis of PH 1. Renal failure slowly progressed to a creatinine clearance of 20 ml/min/1.73 m2 at 8 years, when liver transplantation (LTx) was performed. Four months later, GFR has not changed. Liver function and urinary oxalate/creatinine ratio are normal. Slowly deteriorating chronic renal failure can be stabilized through isolated liver transplantation and thus the rapid need for KTx will at least be delayed. Even more important, normalization of the oxalate metabolism prevents extrarenal oxalate deposits during renal failure.

Selection of the living liver donor.

Living related liver transplantation offers several advantages in comparison to transplantation of cadaver organs. To achieve maximal donor safety evaluation, selection criteria and complications of the donor operation were retrospectively analyzed in living donors of segmental liver transplants. Seventy-three liver donor candidates were evaluated between October 1991 and June 1994. The median age of 42 mothers and 31 fathers was 31 years (range, 19-50 years). The median volume of the left lateral liver lobe comprised 230 ml (100-350 ml). Twenty-four of 73 (33%) donor candidates were not accepted for living donation. Rejection was due to unsuitability of the donor's liver as a graft (n = 13) or due to an increased risk for living donation (n = 11). Of 35 living donations performed so far, one was a full left hemihepatectomy and 34 were left lateral segmentectomies. The length of the donor operation was, on average, 4.3 hr. No heterologous blood was needed. Postoperative complications included death due to pulmonary embolism (n = 1), seizure due to a previously undiagnosed ependymoma (n = 1), bile duct injury (n = 1), incisional hernia necessitating late revision (n = 2), and duodenal ulcer (n = 2). Long-term follow-up revealed no persistent complications. Using our standardized protocol, 33% of young, presumably healthy donor candidates were rejected for living donation.

Living donor for liver transplantation.

Since living related liver transplantation was first performed in 1989, more than 150 cases have been performed worldwide, mostly in the United States and Japan. This paper reports the first series of living related liver transplantation in Europe. Twenty living related liver transplantation surgeries were performed over a 13-mo period, with an overall patient survival of 85%. For patients who underwent elective transplantation (n = 13), the survival rate was 100%. Technical complications included one arterial thrombosis necessitating retransplantation and five bile leaks requiring surgical revision. The technical improvements that permit avoidance of these complications are discussed. A detailed description of the living related liver procurement is given. All procurements yielded grafts of excellent quality. No intraoperative complications occurred, and no reoperations were necessary. No heterologous blood transfusion was needed. In two patients, incisional hernias developed after wound infection. Living related liver transplantation does not absolve the transplant community of efforts to promote cadaveric organ procurement. Nevertheless, living related liver transplantation does have the advantage of a readily available graft of excellent quality, permitting transplantation with optimal timing under elective conditions. Several centers are now preparing living related segmental liver transplants, following the model of our protocol, for three reasons: (a) to obtain superior results compared with cadaveric liver transplantation; (b) to overcome cadaveric organ shortage and further reduce pretransplantation mortality and (c) to provide viable organs in countries where cadaveric organ procurement is not established. When performed by a team experienced in pediatric liver transplantation and in adult liver resection, living related liver transplantation is an excellent modality for the treatment of end-stage liver disease in children.

The plasma amino acid profile and its relationships to standard quantities of liver function in infants and children with extrahepatic biliary atresia and preterminal liver cirrhosis.

The absolute and relative concentrations of 16 plasma amino acids in 48 mostly dystrophic infants and children (median of age 1 1/2 years) with extrahepatic biliary atresia and mainly stable preterminal cirrhosis were compared with those of controls. Patient plasma amino acid data were analysed statistically for diagnostic usefulness and correlated with standard biochemical quantities of liver function and of liver perfusion. In the patients the total amounts of non-essential and essential amino acids were reduced by 19% and with the same significance (p < 0.0005). Plasma tyrosine was increased (+40%), while taurine (-44%) and branched chain amino acids (+28.8% to -34.7%) were decreased. Methionine values varied widely. In the molar fractional plasma amino acid profile, only alanine, valine, and leucine were decreased, while threonine, methionine, tyrosine, phenylalanine, ornithine, and serine were increased. Discriminate function analysis showed that the plasma amino acid data discriminated 93.8% of the patients from controls. The concentrations of some amino acids in plasma seemed to have been influenced by protein-calorie deficiency in the patients. The valine/tyrosine ratio and the Fischer index (ratio branched chain/aromatic amino acids) were significantly reduced in the patients versus controls (1.54 +/- 0.55 vs 3.08 +/- 0.55 and 1.66 +/- 0.39 vs 3.00 +/- 0.48). A number of significant correlations (range of r: 0.37-0.59, p < 0.05, 30-48 data pairs) were calculated between plasma amino acid data and several standard biochemical quantities of liver function. The statistical analyses also showed that the Fischer index began to decrease gradually and linearly early in the progression of liver failure.(ABSTRACT TRUNCATED AT 250 WORDS)

Pre- and post-transplant assessment of liver function in paediatric liver transplantation.

The pre-operative risk of paediatric liver transplantation candidates (n = 41) was assessed in a prospective study by means of clinical symptoms, conventional static and liver blood flow dependent dynamic liver function tests. Nine patients died during the 365-day waiting period. The data were subjected as covariates to a survival analysis in the Cox proportional hazards model. There was a significant relationship between the results of mono-ethylglycinexylidide (MEGX) formation and ICG test and the 365-day survival rate. In the stepwise analysis, none of the remaining parameters improved the predictive ability when added to the dynamic liver function test results. The assessment of post-transplantation liver function was studied in 27 patients during the first 28 postoperative-day period. In addition, liver function was studied in a cross-sectional study 1-7 years after successful liver transplantation in children with complete or partial rehabilitation. In the early postoperative period severe organ damage was indicated by both static and dynamic liver function tests. In the later course after transplantation no deterioration of liver function measured with MEGX formation was to be observed. These findings demonstrate the usefulness of dynamic liver function tests in the pre- and post-transplant assessment of liver function.

Treatment of inherited metabolic disorders by liver transplantation.

Among the worldwide accepted indications for liver transplantation, inherited metabolic disorders play an increasing role. In some paediatric centres this indication runs second after extrahepatic biliary atresia. The aim of liver transplantation in inherited metabolic disorders is twofold: the first is to save a patient's life, the second is to accomplish phenotypic and functional cure of his disease. These aims may be achieved in disorders presenting with cirrhosis, hepatoma, life-threatening progression or failure of other organs with preserved liver function. The timing of liver transplantation has become easier with development of surgical techniques of reduced-size donor livers. These techniques enable the performance of liver transplantation with ABO blood group compatible organs of almost any size if indicated either by deterioration of liver function or impending complications such as hepatoma or life-threatening progression. In comparison with other indications such as extrahepatic biliary atresia, postnecrotic liver cirrhosis or acute liver failure, the results of transplantation in patients with inherited metabolic disorders seem to be better, reaching up to 78-95% actuarial 1-year survival rates. However, lifelong immunosuppressive therapy is necessary. This seems to be acceptable even in disorders with only partial liver function defects.