Growth hormone treatment for Turner syndrome in Australia reveals that younger age and increased dose interact to improve response

Objective To investigate response to growth hormone (GH) in the first, second and third years of treatment in the total clinical cohort of Turner syndrome (TS) patients in Australia. Context Short stature is the most common clinical manifestation of TS. GH treatment improves growth. Design Response was measured for each year of treatment. Stepwise multiple regression analyses were used to identify factors that significantly influenced response. Patients Prepubertal TS patients who completed 1 year (n = 176), 2 year (n = 148), or 3 year (n = 117) of treatment and were currently receiving GH. Measurements Change in TS specific Height Standard Deviation Score (ΔTSZ) was the main response variable used. Major influencing variables considered included dose, starting age and height, BMI, bone age delay, karyotype, parental height, and interactions between dose and starting age or height. Results Response was greatest in first year and declined thereafter (median ΔTSZ: 1st year = +0·705, 2nd year = +0·439, 3rd year = +0·377) despite the median dose increasing [1st year = 5·5 mg/m²/week (0·23 mg/kg/week), 2nd year = 6·4(0·24), 3rd year = 7·2(0·26)]. An Age*Dose interaction was identified influencing first, second year, and total ΔTSZ. The ΔTSZ over 3 years was significantly influenced by first‐year dose. Dose increments only attenuated the general decline in response. An acceptable first‐year response (ΔTSZ > 1·01) was achieved by only 17·6% of patients. Conclusions Growth response is greatest and most influenced by dose in the first year. Dose in first year is a major factor contributing to total response. A starting Age*Dose interaction effect was observed such that young girls on a high dose respond disproportionately better. Optimal GH treatment of short stature in TS thus requires early initiation with the highest safe dose in the first year.     

Preventing choking and suffocation in children

Choking, suffocation and strangulation cause serious unintentional injuries in children and are leading causes of unintentional death in infants and toddlers. Nearly all choking, suffocation and strangulation deaths and injuries are preventable. The present statement reviews definitions, epidemiology and effective prevention strategies for these injuries. Recommendations that combine approaches for improving safety, including research, surveillance, legislation and standards, product design and education, are made. Paediatric health care providers should be encouraging parents and other caregivers to learn cardiopulmonary resuscitation (CPR) and choking first aid, as well as offering anticipatory, age-appropriate guidance to prevent these injuries at regular health visits.     

Community-associated methicillin-resistant Staphylococcus aureus in Indigenous communities in Canada

Community-associated methicillin-resistant Staphylococcus aureus (CA-MRSA) infections have emerged as a significant issue in some Indigenous communities (including First Nations, Inuit and Métis) in Canada. Primarily associated with skin and soft-tissue infections, this organism can also result in significant morbidity and mortality. Canadian and American guidelines for managing CA-MRSA infections have been published. The specific epidemiology, microbiology and susceptibility patterns, and the social/environmental circumstances of CA-MRSA infections in Indigenous communities need to be considered for strategies to reduce transmission. While reducing household crowding and improving in-home potable water supply are optimal strategies to reduce the impact of this illness, implementing Canadian guidelines along with increased prevention strategies are recommended as interim measures.     

A survey of 22 individuals with Prader-Willi Syndrome in New South Wales

Abstract Twenty-two individuals with Prader-Willi Syndrome in New South Wales were surveyed. The results show that males were diagnosed at a significantly earlier age than females and suggest a recent trend towards earlier diagnosis. The advantages of early diagnosis are discussed. In those in whom cytogenetic studies had been performed, 47% were found to have a deletion involving chromosome 15q11–13. Profound neonatal hypotonia had been present in all cases. Obesity became apparent between 1.5 and 10 years (mean = 3.8 years). Facial dysmorphism was reported in 83% and acromicria in 100%. Sixty-two per cent of subjects were regarded as less pigmented than first degree relatives. Cognitive assessments were performed on nine subjects. Two (22%) were functioning in the normal range of intelligence. Behaviour problems, both food-related and non-food-related, were present in the majority and placed considerable stress on the family caring for the individual with Prader-Willi Syndrome.     

Brain stem encephalitis due to herpes simplex virus

Abstract A 6 year old child is described with infection due to herpes simplex virus type 1 causing brain stem encephalitis. The diagnosis was established by enzyme immunosorbent assays of the cerebrospinal fluid and serum which demonstrated antibody responses to herpes simplex virus. Recovery occurred and the importance of early use of acyclovir in achieving a good outcome is emphasized.     

Infection with Chlamydia trachomatis and Neisseria gonorrhoeae in women with lower abdominal pain admitted to a gynaecology unit

Summary. One hundred and sixty-five women admitted to a gynaecology unit with lower abdominal pain were screened for infection with Neisseria gonorrhoeae and Chlamydia trachomatis by members of a department of genitourinary medicine. C. trachomatis alone was detected in 21 patients. N. gonorrhoeae alone was isolated from five patients, and dual infection was present in six patients, giving a total of 32 (19%) patients in whom a sexually transmitted disease (STD) was diagnosed. The combination of an endocervical swab placed in Amies transport medium for gonococcal isolation and an endocervical slide for immunofluorescent detection of chlamydiae proved to be a simple and accurate method of screening for STD. Asaresultof contact tracing, 16 sexual partners of women in whom STD was detected were examined. Three cases of gonocoecal and ninc cases of non-gonococcal urethritis were diagnosed. None of the sexual partners had Symptoms suggestive of genitourinary infection.     

Haemodynamic changes associated with caesarean section under epidural anaesthesia

Summary. Serial haemodynamic investigations were performed in 15 women delivered by elective caesarean section under epidural anaesthesia at 38–40 weeks gestation. Cardiac output was measured by Doppler and cross-sectional echocardiography at the aortic valve. No haemodynamic changes were demonstrable after attainment of surgical anaesthesia (T5 or above). Stroke volume increased 13% after delivery of the placenta and remained elevated until the end of the operation at which time cardiac output was 11% above pre-operative values. Stroke volume and cardiac output fell during the first postoperative day. Heart rate remained elevated at pre-operative values for 48 h after delivery. There was a fall in heart rate and cardiac output between the second and the sixth days after delivery. By 2 weeks after delivery cardiac output was 28% lower than pre-operative values. Compared with pre-operative values, diastolic blood pressure was lower on the first and second postnatal days.