AimTreatment for the Syndrome of Inappropriate Antidiuretic Hormone secretion (SIADH) includes fluid restriction to adjust fluid intake to the diminished water excretion. This is potentially hazardous in infants, as fluid and caloric intake are coupled. Antagonists for the type 2 vasopressin receptor have demonstrated efficacy in adult patients with SIADH, but evidence in children is lacking. Moreover, the current unavailability of a liquid preparation makes the administration of these drugs challenging especially in small children. We aimed to review our experience from 2 recent cases. MethodsRetrospective review of clinical data of 2 patients diagnosed with SIADH in infancy and treated with the oral vasopressin receptor antagonist tolvaptan. ResultsPersistent hyponatraemia was noted in both patients in the first month of life and eventually led to a diagnosis of SIADH. Initial salt supplementation in one patient resulted in severe hypertension, treated with four anti-hypertensive drugs. Tolvaptan was commenced at 2 and 4 months of age, respectively and was associated with normalisation of plasma sodium values and blood pressure without the need for anti-hypertensive treatment. There was transient hypernatraemia in one patient, which normalised with dose reduction. Tolvaptan was administered by crushing the tablet and mixing in water. ConclusionTolvaptan was effective for the treatment of SIADH in both infants and could be administered orally. 3 Key notes: SIADH is difficult to treat with fluid restriction in infants due to the coupling of fluid and caloric intake. Tolvaptan, an oral vasopressin receptor antagonist was effectively used in 2 infants reported here.

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Puberty is defined as the time when the onset of sexual maturity occurs and the reproductive organs become functional, and it is therefore the time when a child becomes an adult capable of reproduction. Although the terms puberty and adolescence are commonly used interchangeably, the term puberty tends to be used for the physical, and adolescence for the psychological and social changes.

Introduction: Autosomal recessive forms of pseudohypoaldosteronism are caused by genetic defects in the epithelial sodium channel. Little is known about the long-term outcome and medication needs during childhood and adolescence. Objective: This study reports a single centre experience of children affected with this ultra-rare condition over a 37 year period. Methods: We report the clinical presentation, growth, neuro-development, associated conditions, mortality and medication dosing and administration for 12 affected children from 8 families. Results: All children presented within the first 2 weeks of life with life threatening, severe hyperkalaemia and hyponatraemia. All parents were consanguineous and of South Asian, Middle Eastern or African ethnic origin. Eight children had homozygous mutations in the SCNN1A and SCNN1G genes encoding the epithelial sodium channel subunits alpha and gamma, respectively, including one novel mutation. Three children died (25%) and two (16%) have severe neurological impairment post cardiac arrest secondary to hyperkalaemia. One affected female had a successful pregnancy at the age of 28 years. Conclusion: Despite high mortality and morbidity in this condition, survival with normal physical and neurological outcome is possible, justifying intensive management to prevent electrolyte imbalance.