scholarly journals Clinical Approach to Sodium Homeostasis Disorders in Children with Pituitary-Suprasellar Tumors

2019 ◽  
Vol 110 (3-4) ◽  
pp. 161-171
Author(s):  
Gerdi Tuli ◽  
Patrizia Matarazzo ◽  
Luisa de Sanctis

Children with pituitary-suprasellar tumors are at high risk of developing sodium metabolism disorders since the tumoral mass itself or surgical and medical treatment can damage AVP release circuits. Additional risk factors are represented by the use of hypotonic fluids, the young age, total parenteral nutrition, and obstructive hydrocephalus secondary to tumor pathology. The most frequent hyponatremic disorders related to AVP in these patients are the syndrome of inappropriate ADH secretion and the cerebral/renal salt wasting syndrome, while hypernatremic conditions include central diabetes insipidus (CDI) and adipsic CDI. The main challenge in the management of these patients is to promptly distinguish the AVP release disorder at the base of the sodium imbalance and treat it correctly by avoiding rapid sodium fluctuations. These disorders can coexist or follow each other in a few hours or days; therefore, careful clinical and biochemical monitoring is necessary, especially during surgery, the use of chemotherapeutic agents, or radiotherapy. This monitoring should be performed by experienced healthcare professionals and should be multidisciplinary, including pediatric endocrinologists, neurosurgeons, and oncologists since maintaining sodium homeostasis also plays a prognostic role in terms of disease survival, therapeutic response, hospitalization rate, and mortality. In this review, we analyze the management of sodium homeostasis disorders in children with pituitary-suprasellar tumors and discuss the main challenges in the diagnosis and treatment of these conditions based on literature data and over 30 years of clinical experience at our Department of Pediatric Endocrinology.

2019 ◽  
Author(s):  
Aoife Garrrahy ◽  
Osamah Hakami ◽  
Iona Galloway ◽  
Stephen McNally ◽  
Rory Dwyer ◽  
...  

1999 ◽  
Vol 276 (3) ◽  
pp. G567-G571 ◽  
Author(s):  
Edith Hummler ◽  
Jean-Daniel Horisberger

The epithelial Na+ channel (ENaC) controls the rate-limiting step in the process of transepithelial Na+ reabsorption in the distal nephron, the distal colon, and the airways. Hereditary salt-losing syndromes have been ascribed to loss of function mutations in the α-, β-, or γ-ENaC subunit genes, whereas gain of function mutations (located in the COOH terminus of the β- or γ-subunit) result in hypertension due to Na+ retention (Liddle’s syndrome). In mice, gene-targeting experiments have shown that, in addition to the kidney salt-wasting phenotype, ENaC was essential for lung fluid clearance in newborn mice. Disruption of the α-subunit resulted in a complete abolition of ENaC-mediated Na+ transport, whereas knockout of the β- or γ-subunit had only minor effects on fluid clearance in lung. Disruption of each of the three subunits resulted in a salt-wasting syndrome similar to that observed in humans.


2021 ◽  
Author(s):  
Kohei Haraguchi ◽  
Takahisa Kimata ◽  
Shohei Akagawa ◽  
Sohsaku Yamanouchi ◽  
Kazunari Kaneko

Nephron ◽  
1999 ◽  
Vol 82 (2) ◽  
pp. 100-109 ◽  
Author(s):  
John K. Maesaka ◽  
Sandeep Gupta ◽  
Steven Fishbane

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