Cerebral Salt Wasting Complicated by Central Diabetes Insipidus and Growth Hormone Deficiency

Papadimitriou, Dimitrios T; Spiteri, Anne; Attilakos, Achilleas; Papadimitriou, Anastasios

doi:10.1007/s12098-018-2640-1

Cited by 5 publications

(4 citation statements)

References 4 publications

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“…Our results indicate considerable abnormalities in the RAAS and bradykinin systems already at an early stage in a rat model of WS when the primary symptoms have not yet fully developed. RAAS has never been studied in Wolfram syndrome, although, it has been shown that due to severe hydronephrosis and to neurodegeneration, WS patients may have a serious imbalance in natriuretic peptides, which may lead to alterations in RAAS and result in mineralocorticoid deficiency [70,71]. We show for the first time that in Wfs1-deficiency, the expression of Agtr2 and Bdkrb1 receptors is downregulated both in vitro and in vivo.…”

Section: Discussionmentioning

confidence: 82%

The Expression of RAAS Key Receptors, Agtr2 and Bdkrb1, Is Downregulated at an Early Stage in a Rat Model of Wolfram Syndrome

Punapart

Seppa

Jagomäe

et al. 2021

Genes

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Wolfram syndrome (WS) 1 is a rare monogenic neurodegenerative disorder caused by mutations in the gene encoding WFS1. Knowledge of the pathophysiology of WS is incomplete and to date, there is no treatment available. Here, we describe early deviations in the renin-angiotensin-aldosterone system (RAAS) and bradykinin pathway (kallikrein kinin system, KKS) observed in a rat model of WS (Wfs1 KO) and the modulative effect of glucagon-like peptide-1 receptor agonist liraglutide (LIR) and anti-epileptic drug valproate (VPA), which have been proven effective in delaying WS progression in WS animal models. We found that the expression of key receptors of the RAAS and KKS, Agtr2 and Bdkrb1, were drastically downregulated both in vitro and in vivo at an early stage in a rat model of WS. Moreover, in Wfs1, KO serum aldosterone levels were substantially decreased and bradykinin levels increased compared to WT animals. Neither treatment nor their combination affected the gene expression levels seen in the Wfs1 KO animals. However, all the treatments elevated serum aldosterone and decreased bradykinin in the Wfs1 KO rats, as well as increasing angiotensin II levels independent of genotype. Altogether, our results indicate that Wfs1 deficiency might disturb the normal functioning of RAAS and KKS and that LIR and VPA have the ability to modulate these systems.

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Section: Discussionmentioning

confidence: 82%

The Expression of RAAS Key Receptors, Agtr2 and Bdkrb1, Is Downregulated at an Early Stage in a Rat Model of Wolfram Syndrome

Punapart

Seppa

Jagomäe

et al. 2021

Genes

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“…20 Hyponatremia occurring due to salt wasting triggered by ANP, if complicated by SIADH, could prove to be lethal. 21 On the other hand, ANP may contribute to maintain water and electrolyte balance in children affected with pneumonia and SIADH through its diuretic, vasodilatory, and natriuretic effects. 9 While in our study, we could not perform ANP and ADH levels or levels of renin and aldosterone, it is possible that both mechanisms, either together or in different stages of the disease, contribute to hyponatremia in children with LRTI.…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Incidence and Risk Factors for Hyponatremia in Hospitalized Children with Lower Respiratory Tract Infections

Bhargava

Patra

Amonkar

et al. 2022

J Pediatr Intensive Care

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Lower respiratory tract infection (LRTI) is an important cause of hospitalization in pediatric population. Hyponatremia is frequently present in children hospitalized with pneumonia and bronchiolitis and is associated with poorer outcomes. We conducted a study to identify the risk factors for hyponatremia in hospitalized children with LRTI and to evaluate the relationship between serum sodium levels and severity of respiratory distress. A total of 147 patients between 2 months and 12 years of age, admitted with the diagnosis of LRTI at the Mahatma Gandhi Mission Hospital and Medical College, a tertiary hospital in Navi Mumbai, Maharashtra, India, between December 2017 and November 2019, were studied prospectively. Patients with any other comorbidities were excluded. The severity of respiratory distress at presentation was graded using the Clinical Respiratory score (CRS). Serum electrolytes were sent within 1 hour of hospital admission. Median age of enrolled patients was 14 months. Overall, 23 (16%) children had mild respiratory distress, 97 (66%) had moderate respiratory distress, and 26 (18%) had severe distress. The incidence of hyponatremia in children admitted with LRTI was 66% and majority of them (70%) had mild hyponatremia. The incidence, as well as severity of hyponatremia differed significantly with etiology (p < 0.05). An inverse correlation was found between serum sodium levels and CRS at presentation (r = − 0.26, p = 0.001). In multivariate analyses, parenchymal disease was an independent risk factor for development of hyponatremia (adjusted odds ratio [aOR] = 5.64 (2.52–12.59), p < 0.001). Incidence and severity of hyponatremia in children with LRTI increased with severity of respiratory distress and presence of parenchymal disease.

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“…Diagnosis of postoperative CDI can be further complicated by pre-existing unrecognized partial CDI with acute worsening following surgery, or coincident electrolyte abnormalities induced by syndrome of inappropriate diuretic hormone, cerebral salt wasting, or growth hormone deficiency alone or in varying combinations. 18 A recent publication offered a possible postoperative definition for adult CDI: urine production >300 mL/h for 3 consecutive hours, urine specific gravity <1.005, and at least one of the following: excessive thirst, serum osmolality >300 mOsm/kg, or serum sodium >145 nmol/L. This did not include urine osmolality as a part of the definition, again highlighting significant variation in working clinical definitions for CDI in the perioperative setting.…”

Section: Discussionmentioning

confidence: 99%

Diagnosis and Management of New-Onset Central Diabetes Insipidus in Critically Ill Children Varies between Pediatric Critical Care Medicine and Pediatric Endocrinology Clinicians

Hunter

Guzman

Winters

et al. 2022

J Pediatr Intensive Care

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The diagnosis and management of central diabetes insipidus in critically ill children is not standardized. Our objective was to characterize differences between Pediatric Critical Care Medicine (PCCM) and Pediatric Endocrinology (PE) clinicians in the diagnosis and management of new-onset CDI in the pediatric intensive care unit. We also sought to characterize knowledge gaps among general pediatrics (GP) residents. This is a scenario-based survey to assess patterns of diagnosis and management of new-onset CDI that was distributed to PCCM, PE, and GP clinicians who work in a quaternary care urban children's hospital. Of 275 PCCM, PE, and GP clinicians surveyed, 158 (57%) responded. More PCCM than PE clinicians relied on serum sodium levels (96 vs. 75%, p <0.01) and more PE than PCCM clinicians relied on serum osmolality (91 vs. 40%, p < .001) for diagnosis. Fewer PCCM than PE clinicians favored restricting IV fluids to two-thirds maintenance rate (4 vs. 37%, p <0.001). More PCCM than PE clinicians favored a starting dose of 0.5 milli-units/kg/h for IV vasopressin infusion (76 vs. 53%, p = 0.048). More PCCM clinicians than PE clinicians favored titrating the IV vasopressin infusion every 20 minutes (24 vs. 2%, p = 0.02), whereas more PE clinicians than PCCM clinicians favored titration every 60 minutes (38 vs. 14%, p = 0.03). GP residents earlier in training had greater self-reported gaps in knowledge. We observed substantial variability in the diagnosis and management of new-onset CDI in critically ill children among PCCM, PE, and GP clinicians. There is a need for greater standardization in care of these patients.

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Cerebral Salt Wasting Complicated by Central Diabetes Insipidus and Growth Hormone Deficiency

Cited by 5 publications

References 4 publications

The Expression of RAAS Key Receptors, Agtr2 and Bdkrb1, Is Downregulated at an Early Stage in a Rat Model of Wolfram Syndrome

The Expression of RAAS Key Receptors, Agtr2 and Bdkrb1, Is Downregulated at an Early Stage in a Rat Model of Wolfram Syndrome

Incidence and Risk Factors for Hyponatremia in Hospitalized Children with Lower Respiratory Tract Infections

Diagnosis and Management of New-Onset Central Diabetes Insipidus in Critically Ill Children Varies between Pediatric Critical Care Medicine and Pediatric Endocrinology Clinicians

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