Stuart J. O’Toole

UK guidance on the initial evaluation of an infant or an adolescent with a suspected disorder of sex development

It is paramount that any child or adolescent with a suspected disorder of sex development (DSD) is assessed by an experienced clinician with adequate knowledge about the range of conditions associated with DSD. If there is any doubt, the case should be discussed with the regional team. In most cases, particularly in the case of the newborn, the paediatric endocrinologist within the regional DSD team acts as the first point of contact. The underlying pathophysiology of DSD and the strengths and weaknesses of the tests that can be performed should be discussed with the parents and affected young person and tests undertaken in a timely fashion. This clinician should be part of a multidisciplinary team experienced in management of DSD and should ensure that the affected person and parents are as fully informed as possible and have access to specialist psychological support. Finally, in the field of rare conditions, it is imperative that the clinician shares the experience with others through national and international clinical and research collaboration.

The concordance between serum anti-Mullerian hormone and testosterone concentrations depends on duration of hCG stimulation in boys undergoing investigation of gonadal function

Background  In boys undergoing investigation of gonadal function, the relationship between a single measurement of serum anti‐Mullerian hormone (AMH) and hCG stimulated serum testosterone is unclear.

Urinary Tract Infection: United Kingdom

Urinary tract infection (UTI) is common in childhood. Three percent of boys and eleven percent of girls have had at least one UTI before their 16th birthday. In the first 3 years of life the sex related incidence is approximately equal, but thereafter UTI becomes more common in girls. Correct diagnosis, treatment and subsequent targeted investigation of UTI in children is important because of the associations between UTI, underlying urological abnormalities, subsequent progressive renal damage and associated hypertension.

G13 Continent Catheterisable Conduit

• This procedure is used to provide a catheterisable conduit between the skin surface and bladder.

G14 V-Quadrilateral-Z (VQZ)-plasty for Stoma

• This procedure is used to create the cutaneous opening of a continent catheterisable conduit instead of a flush stoma.

G17 Posterior Urethral Valves

• The valves consist of a leaflet of tissue that originates from inferior aspect of the verumontanum in the posterior urethra (Fig. 1).

F16 Hypospadias Repair: Stage Two

The second stage of a proximal hypospadias repair. Is timed for approximately 6 months after the first stage.

F15 Two-Stage Hypospadias Repair: Stage One

A two-stage repair is done for a hypospadia with a proximal meatus or severe chordee, when a graft is required to create a new urethral plate (Fig. 1).