Incidence and Predictors of Cerebral Salt Wasting Syndrome in Stroke Patients – Results of a Prospective Observational Study

BackgroundCerebral salt wasting syndrome (CSWS) and Syndrome of Inappropriate Anti Diuretic Hormone secretion (SIADH) are the most common aetiological factors for developing hyponatremia following stroke. The differentiation of these two entities is crucial as the treatment options are completely different. Hence the knowledge on predictors of CSWS is important to make a more accurate diagnosis of CSWS. MethodsTwo hundred and fourty six patients with confirmed stroke were prospectively observed throughout the hospital stay in a tertiary referral center in Sri Lanka to identify the possible predictors of CSWS. Hyponatremia was defined as serum Na+ level less than 131mmo/l. Serum osmolality, urine osmolality, urinary Na+, serum cortisol and thyroid function tests were performed on all the hyponatremic patients. Differentiation of the CSWS and SIADH was based on physical examination findings and laboratory parameters. ResultsThe incidence of hyponatremia in our study population was 19.1% (95% Confidence Interval 14.39-24.58). The majority of patients (24, 51%) were attributed to CSWS. SIADH group comprised of 17 (36.2%) patients and 6 (12.7%) patients had other undetermined causes. There was a significant statistical difference between the aetiologies of hyponatremia and laboratory investigations like urinary Na+, urinary osmolality and serum osmolality. Demographic characteristics, comorbidities, imaging findings and clinical parameters like systolic blood pressure, diastolic blood pressure, on admission GCS were considered in the multivariable logistic regression model and the overall model was not significant. Conclusion The incidence of CSWS is higher than the incidence of SIADH. The demographic characteristics, comorbidities, imaging and clinical parameters like blood pressure, on admission GCS could not predict the occurrence of CSWS

Carboplatin-induced Renal Salt Wasting Syndrome in Paediatric Patients with Intracranial Germ Cell Tumours and Concomitant Diabetes Insipidus

We report the first case series of 14 children with intracranial germ cell tumour (iGCT) and concomitant central diabetes insipidus (DI), who developed hyponatremia secondary to renal salt wasting syndrome (RSWS) following the administration of carboplatin. Clinicians prescribing platinum-based chemotherapy for this group of patients should be alert to the risk of RSWS. Regular monitoring should be performed as hyponatraemia can be asymptomatic until it is severe.

Disorders of Sex Development of Adrenal Origin

Disorders of Sex Development (DSD) are anomalies occurring in the process of fetal sexual differentiation that result in a discordance between the chromosomal sex and the sex of the gonads and/or the internal and/or external genitalia. Congenital disorders affecting adrenal function may be associated with DSD in both 46,XX and 46,XY individuals, but the pathogenic mechanisms differ. While in 46,XX cases, the adrenal steroidogenic disorder is responsible for the genital anomalies, in 46,XY patients DSD results from the associated testicular dysfunction. Primary adrenal insufficiency, characterized by a reduction in cortisol secretion and overproduction of ACTH, is the rule. In addition, patients may exhibit aldosterone deficiency leading to salt-wasting crises that may be life-threatening. The trophic effect of ACTH provokes congenital adrenal hyperplasia (CAH). Adrenal steroidogenic defects leading to 46,XX DSD are 21-hydroxylase deficiency, by far the most prevalent, and 11β-hydroxylase deficiency. Lipoid Congenital Adrenal Hyperplasia due to StAR defects, and cytochrome P450scc and P450c17 deficiencies cause DSD in 46,XY newborns. Mutations in SF1 may also result in combined adrenal and testicular failure leading to DSD in 46,XY individuals. Finally, impaired activities of 3βHSD2 or POR may lead to DSD in both 46,XX and 46,XY individuals. The pathophysiology, clinical presentation and management of the above-mentioned disorders are critically reviewed, with a special focus on the latest biomarkers and therapeutic development.

Devastating Salt-wasting Crisis in a Four-month-old Male Child with Congenital Adrenal Hyperplasia, Highlighting the Essence of Neonatal Screening

Classic Congenital Adrenal Hyperplasia in males usually presents late until before puberty, but rarely at birth. In cases of undiagnosed CAH, severe deficiency of cortisol may lead to life threatening situation in an infant with acute salt losing crisis. Screening for CAH at birth can lead to better outcomes.