Reducing unnecessary neonatal testing in infants of mothers with thyroid disease.

AIM: To examine the numbers of asymptomatic infants <8 weeks who had appropriate thyroid function tests (TFTs) in addition to the newborn screening test, because of maternal thyroid disease, before and after the implementation of an updated institutional guideline and staff education. METHODS: A medical record audit of infants <8 weeks born at a metropolitan teaching hospital, who had TFTs between 1 July 2017 and 31 October 2017 was performed as part of a quality improvement project. Records were reviewed to determine the indication for testing and whether this complied with the current 2011 institutional guideline. A multidisciplinary staff education package was developed to coincide with the publication of an updated guideline in August 2018. Staff education and resources were provided throughout July 2018. A post-intervention audit was repeated between 1 August 2018 and 1 December 2018, assessing compliance with the 2018 guideline. RESULTS: In the baseline period, 40 of 457 infants born had TFTs performed, of which 26 of 40 (65%) were for maternal thyroid disease. Of these 10 of 26 (38%) met the 2011 criteria for testing; 1 of 26 (4%) met the updated 2018 criteria. In the post-intervention period, 14 of 412 infants born had TFTs of which 5 of 14 (36%) were tested due to maternal thyroid disease and all were compliant with the new guideline. CONCLUSIONS: Baseline audit revealed unnecessary neonatal thyroid function testing of healthy babies. Implementation of an updated guideline and a brief, targeted education package successfully increased awareness of the updated recommendations, reduced unnecessary testing and led to improved practice.

Sydney Diabetes centre's experience of the Australian Government's roll out of subsidised continuous glucose monitoring for children with type 1 diabetes mellitus.

AIM: To determine patient/carer expectations of continuous glucose monitoring (CGM) and short-term satisfaction, to assess the efficacy of CGM in improving: fear of hypoglycaemia and glycaemic control (HbA1c , ketosis, hypoglycaemia) and to determine time requirements of diabetes clinic staff in commencing and administering CGM. METHODS: We assessed CGM-naïve patients starting on CGM at a Sydney Diabetes Centre following the introduction of a nationwide government subsidy for CGM. A standardised questionnaire was administered collecting demographic and glycaemic information in addition to Likert scale assessment of expectations and satisfaction. Clinic staff reported time dedicated to CGM education, commencement and follow-up. RESULTS: A total of 55 patients or parents/carers completed baseline questionnaires, with 37 completing a 3-month follow-up questionnaire. There were high expectations of CGM prior to commencement and high satisfaction ratings on follow-up. CGM improved fear of hypoglycaemia, and total daily insulin dose increased after commencement of CGM. There was a trend towards lower HbA1c that was not statistically significant and no statistically significant reduction in ketosis or hypoglycaemia. Comments were mostly positive, with some concern raised regarding technical issues and a lack of subsidy after 21 years of age. Staff time requirements were substantial, with an estimated average of 7.7 h per patient per year. CONCLUSIONS: Patients and families have high expectations of CGM, and satisfaction levels are high in the short term. Total insulin delivery increased after CGM commencement. Time requirements by staff are substantial but are worthwhile if families' overall satisfaction levels are high.

Evaluation of bone mineral density and morphology using pQCT in children after spinal cord injury.

OBJECTIVE: To evaluate the effects of spinal cord injury (SCI) on bone density and morphology in children using peripheral quantitative computer tomography (pQCT). DESIGN: Retrospective cohort study of 19 paediatric patients with SCI (9 paraplegics and 10 tetraplegics). RESULTS: There was significant reduction in tibial metaphysial volumetric bone mineral density (vBMD), diaphysial cortical cross-sectional area (CSA), cortical thickness and polar strength-strain index. There was a significant loss of calf muscle CSA. Those who were able to stand had greater trabecular vBMD, tibial cortical thickness and tibial muscle CSA Z-scores. Lower limb fractures did not occur if tibial trabecular vBMD was greater than 100 mg/cm³. Tibial geometry following SCI was more circular compared to controls. CONCLUSIONS: pQCT provides a valuable insight into the regional changes in bone and muscle development in children following SCI. Residual muscle function with the ability to weight bear provides a significant benefit to bone development.

Generalized arterial calcification of infancy: treatment with bisphosphonates.

BACKGROUND: A baby girl developed respiratory distress immediately after birth and required supplemental oxygen. She was born at term via lower-segment cesarean section. The parents were nonconsanguineous, and antenatal ultrasonography during the pregnancy at 18 and 32 weeks of gestation did not reveal any abnormalities. On examination at birth, no pulses were palpable; however, the baby's blood pressure was normal and no remarkable abnormalities were detected. Ultrasonography revealed widespread arterial calcification. INVESTIGATIONS: Chest and abdominal radiography at birth and serial abdominal, renal and cardiac ultrasonography at follow-up examinations; dual-energy X-ray absorptiometry during treatment with bisphosphonates; genetic screening. DIAGNOSIS: Generalized arterial calcification of infancy, secondary to compound, heterozygous mutations in the ENPP1 gene, pL661V and pE668K of the paternal chromosome, and pN792S in the maternal chromosome. MANAGEMENT: Low-dose disodium pamidronate (0.1 mg/kg per week for 4 weeks), which commenced on the seventh day after birth and was changed to oral risedronate sodium (1 mg/kg per week as a single dose) at 4 weeks of age. Complete resolution of arterial calcification was seen by 3 months of age. At 12 months, ergocalciferol was added at a dose of 5,000 U daily for 6 weeks, followed by 200 U daily owing to the patient's vitamin D deficiency and elevated parathyroid hormone level. Treatment with bisphosphonates is ongoing, but is planned to be discontinued at 3 years of age. The child has remained healthy and developmentally normal.

'Red as a beet and blind as a bat' Anticholinergic delirium in adolescents: lessons for the paediatrician.

Anticholinergic syndrome has been widely documented in the literature but is uncommon in paediatric medicine. Teenage boys are most at risk of self-induced anticholinergic syndrome through intentional ingestion of plants. We report on a 14 year old boy who presented to our hospital with clinical signs of anticholinergic toxicity and who was discharged 36 hours after admission with no major residual effects. Classical anticholinergic syndrome should be readily diagnosed by the experienced clinician ('hot as a hare, red as a beet, dry as a bone, blind as a bat and mad as a hatter'). Acute presentations should be treated with benzodiazepines and supportive care. Treatment of the delirium with haloperidol may be harmful. Lack of familiarity with anticholinergic syndrome may also delay the diagnosis or result in potentially harmful treatments. A high index of suspicion is often required in the paediatric setting due to infrequent acute hospital presentation.