Ja Batch

Use of bisphosphonate therapy for osteoporosis in childhood and adolescence

Abstract:  Congenital and acquired forms of osteoporosis in childhood and adolescence can result in morbidity from fracture and pain in childhood, and place an individual at significant risk for problems in adult life. A range of therapies exist for the prevention and treatment of osteoporosis, including optimization of daily calcium intake, adequate vitamin D status, weight‐bearing exercise, treatment with sex steroids where delayed puberty is a problem and, more recently, use of bisphosphonate therapy. Intravenous pamidronate therapy (a bisphosphonate) has been shown to reduce fractures and improve bone density in children with osteogenesis imperfecta, and might prove to be of benefit in other osteoporotic conditions in childhood. However, a number of issues regarding the optimal use of bisphosphonate therapy in children and adolescents remain to be resolved, including total annual dose and frequency and duration of administration. Bisphosphonate therapy should, therefore, be used only in the context of a well‐run clinical programme with specialist knowledge in the management of osteopenic disorders in childhood.

Hepatic glycogenosis: Reversible hepatomegaly in type 1 diabetes

Objective: To describe the aetiology, clinical features and appropriate treatment for hepatic glycogenosis in poorly controlled type 1 diabetes.

Adrenal 21‐hydroxylase deficiency in childhood: 25 years' experience

Objective: To review past and present management of congenital adrenal hyperplasia at a single centre, as a guide to best practice.

Cataracts in insulin‐dependent diabetes mellitus: Sixteen years’ experience in children and adolescents

To report on cases of diabetic cataracts in a paediatric and adolescent population.

Cushing's syndrome in childhood and adolescence

Objective: To review the diagnosis, management and outcome of Cushings syndrome in children and adolescents.

Increased serum creatinine associated with severe primary hypothyroidism

A case of myxoedema due to Hashimotos thyroiditis associated with a significant increase in serum creatinine is reported. Thyroid hormone replacement therapy resulted in normalization of the serum biochemistry within 1 month.

CYP11β1 (11-beta-hydroxylase) deficiency in congenital adrenal hyperplasia

Objective: To review experience of CYP11β1 deficiency (previously known as 11β‐hydroxylase) at the Royal Childrens Hospital, Melbourne, Victoria.

Three cases of congenital adrenal hypoplasia: A cause of salt-wasting and mortality in the neonatal period

Three infants with congenital adrenal hypoplasia are described. The two surviving infants were detected and successfully treated in the neonatal period due to a suggestive family history (Case 1) and antenatal maternal oestriol screening (Case 2). The modes of inheritance, diverse clinical presentation, associated conditions, diagnostic work‐up and pathology of congenital adrenal hypoplasia in these three infants is discussed.

Potential adverse endocrine effects of inhaled corticosteroids

Abstract: Evidence exists for a potential role for inhaled corticosteroids, particularly when used in high dose to cause growth impairment, delayed maturation and adrenal suppression in children and adolescents with asthma. The functional significance of biochemical adrenal suppression remains uncertain. Similarly, there is as yet insufficient evidence to determine whether inhaled corticosteroids may adversely affect bone mineral density in children and adolescents with asthma.