Hip surveillance in Tasmanian children with cerebral palsy

Background:   Children with cerebral palsy (CP) are at risk of hip subluxation. Over time, subluxation can lead to dislocation, deformity and pain. Hip surveillance in the form of an X-ray and clinical examination of this 'at risk group' can identify early subluxation. Early subluxation can be treated, preventing hip dislocation in many cases. Hip surveillance in CP commenced in Tasmania in 1992.
Aims:   To audit the hip surveillance data to date, perform a literature review to understand the emerging evidence for prevention and management of hip subluxation in CP and update hip surveillance guidelines.
Methods:   New guidelines were written and distributed, and an audit of the previous 12 years performed by review of medical files and X-rays.
Results:   Two hundred and eighteen children had been involved in the hip surveillance programme. Fifteen cases of dislocation were recorded in this time. The incidence of subluxation and surgery, as well as the gross motor function classification system (GMFCS) level, was recorded.
Conclusion:   Data from Tasmania showed a similar incidence of hip subluxation according to GMFCS level, and frequency of different surgical interventions as other recent audits. Some children with minor subluxation improved without orthopaedic intervention once weight bearing occurred, which had not before been appreciated. Migration percentage alone is not adequate to fully describe the outcome of hip subluxation. More appropriate measures of outcome in terms of quality of life for children with CP need to be developed.     

Repair of long-gap esophageal atresia: gastric conduits may improve outcome—a 20-year single center experience

Introduction  

Treatment of long-gap esophageal atresia (LEA) is a major challenge. Options for reconstruction include native esophagus, or replacement with stomach, colon, or small intestine. However, debate continues regarding the optimal conduit for esophageal replacement.     

A clinical study assessing the tolerability and biological effects of infliximab, a TNF-alpha inhibitor, in patients with advanced cancer

BACKGROUND: Tumour necrosis factor-alpha (TNF-alpha) is an important regulator of the chronic inflammation contributing to tumour progression. Infliximab, an anti-TNF-alpha monoclonal antibody was investigated in this trial of patients with advanced cancer. The primary objectives were to determine the safety profile and biological response of infliximab in a cancer population. Clinical response was a secondary objective. PATIENTS AND METHODS: Forty-one patients received infliximab at 5 mg/kg (n = 21) or 10 mg/kg (n = 20) i.v. at 0 and 2 weeks and then every 4 weeks. Post-treatment samples were measured for changes in plasma and serum TNF-alpha, CCL2, IL-6 and C-reactive protein (CRP). RESULTS: Infliximab was well tolerated with no dose-limiting toxic effects. At both doses of infliximab, neutralisation of serum TNF-alpha was observed after 1 h while plasma CCL2, IL-6 and serum CRP were decreased 24 and 48 h following infliximab administration. Seven patients experienced disease stablisation (range 10-50+ weeks). There was no evidence of disease acceleration in any patient. CONCLUSIONS: Infliximab treatment was safe and well tolerated in patients with advanced cancer. There was evidence of biological activity with baseline TNF-alpha and CCL2 being correlated with infliximab response.     

The Fremantle Lead Study

Objective: To ascertain blood lead levels in a sample of preschool children from Fremantle, Western Australia, and to correlate these with possible risk factors.
Methodology The study was a cross-sectional prevalence survey of 120 children from day-care centres and 44 hospital inpatients. Blood lead and ferritin levels were determined and a risk factor questionnaire was completed by parents.
Results Of the 164 children 25.6% had lead levels above the NH&MRC goal (<10μg/dL). Nine of 133 (6.7%) had ferritin levels below 10 μg/L suggesting iron deficiency. Excessive blood lead concentrations as defined by the NH&MRC (>9μg/dL) related to: child's presence during house renovation (OR 3.35, P = 0.007, 95% Cl 1.39-8.81); Aboriginality (OR 6.4, P = 0.008, 95% Cl 1.6-24.9), and, in the 9-24 month age group, inversely to distance between home and a road carrying >7000 vehicles/day (r-0.56, P = 0.009, n = 24).
Conclusions A group of Fremantle children with unacceptably high blood lead levels has been identified. Renovation of older housing and Aboriginality are important risk factors.     

Umbilical artery flow velocity waveforms and twin pregnancy outcome

Two hundred seventy-two twin pregnancies were studied by analysis of the fetal umbilical artery waveforms recorded using continuous-wave Doppler ultrasound. After the first 100 cases, a management strategy was adopted whereby all twin gestations would be studied between 28-32 weeks' gestation and the results made available to the referring obstetrician, thereby influencing clinical management. Perinatal mortality and morbidity, in pregnancies with both fetuses alive at 28 weeks' gestation, were compared between the patients studied in the first group of 100 and those studied after the Doppler examination was introduced as a clinical service. There was a decrease in perinatal mortality, both uncorrected (57.9 per 1000 to 17.9 per 1000; P less than .05) and corrected (42.1 per 1000 to 8.9 per 1000). Fetal deaths were reduced from six to one (P less than .05). This decrease in perinatal mortality was achieved without any appreciable change in the gestational age at delivery or mode of delivery between the two groups. There was a reduction in the number of infants requiring neonatal intensive care (from 38% to 24%; P less than .01).     

Reproductive problems and birth defects in survivors of Wilms' tumor and their relatives

In a retrospective cohort study of 47 Wilms' tumor survivors and their 77 sibling controls, female survivors had a fourfold excess risk (risk ratio, 4.1; 95% confidence interval, 1.7-10.1) for any adverse livebirth outcome, including birth defects, compared with their sibling controls. Wives of male survivors had no apparent excess risk for problem pregnancies. The families had a number of severe reproductive problems and major birth defects, such as primary amenorrhea in two survivors, bicornuate uterus in two survivors and one control, and mental retardation in one male survivor and a male control. The son of a female survivor died after bilateral Wilms' tumors. Birth defects in the offspring of female survivors are compatible either with intrauterine constraint, possibly due to radiation-induced fibrosis or with the complex of malformations associated with Wilms' tumor. Female survivors of Wilms' tumor appear to be at increased risk for a variety of reproductive problems, from sterility to fetal loss, early delivery, and birth defects in offspring. Furthermore, relatives of survivors of Wilms' tumor may be at risk of having associated birth defects, with clinically significant consequences.     

Diagnosis of 21-hydroxylase deficiency in newborn infants by GC-MS of urinary steroids

In a study using gas chromatography-mass spectrometry (GC-MS) on urine specimens from 16 normal infants and 16 infants with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency (aged 1 day to 4 weeks), the major steroids recognized in all infants were: 16 alpha-hydroxy-dehydroepiandrosterone, 16 beta-hydroxy-dehydroepiandrosterone, 16-oxo-androstenediol, androstenetriol, 15 beta,17 alpha-dihydroxy-pregnenolone and 16 alpha-hydroxy-pregnenolone. Pregnanetriol was detectable in three normal infants (aged 3, 6 and 15 days) but the levels seen in 15 CAH patients were in a higher range. Pregnanetriolone, 5 beta-17-hydroxy-pregnanolone and 15 beta,17 alpha-dihydroxy-pregnanolone were present in the urine of 15 CAH patients, but were not detectable in any of the normal infants. The older the patient, the higher the level was of each of these four steroids. The results indicate that, even on day 1, patients with CAH due to 21-hydroxylase deficiency may be positively identified using GC-MS of urine specimens. This does not preclude the possibility that a minority of patients with CAH, most likely those with mild 21-hydroxylase deficiency, may not exhibit the characteristic GC-MS findings on day 1, as seen in one of the 16 CAH patients.