No abstract

Nephrogenic diabetes insipidus (NDI) is characterized by the inability to concentrate urine that results in polyuria and polydipsia, despite having normal or elevated plasma concentrations of arginine vasopressin (AVP). In this study, we review the clinical aspects and diagnosis of NDI, the various etiologies, current treatment options and potential future developments. NDI has different clinical manifestations and approaches according to the etiology. Hereditary forms of NDI are mainly caused by mutations in the genes that encode key proteins in the AVP signaling pathway, while acquired causes are normally associated with specific drug exposure, especially lithium, and hydroelectrolytic disorders. Clinical manifestations of the disease vary according to the degree of dehydration and hyperosmolality, being worse when renal water losses cannot be properly compensated by fluid intake. Regarding the diagnosis of NDI, it is important to consider the symptoms of the patient and the diagnostic tests, including the water deprivation test and the baseline plasma copeptin measurement, a stable surrogate biomarker of AVP release. Without proper treatment, patients may developcomplications leading to high morbidity and mortality, such as severe dehydration and hypernatremia. In that sense, the treatment of NDI consists in decreasing the urine output, while allowing appropriate fluid balance, normonatremia, and ensuring an acceptable quality of life. Therefore, therapeutic options include nonpharmacological interventions, including sufficient water intake and a low-sodium diet, and pharmacological treatment. The main medications used for NDI are thiazide diuretics, nonsteroidal anti-inflammatory drugs (NSAIDs), and amiloride, used isolated or in combination.

We studied the glomerular hemodynamics and activity of the tubuloglomerular feedback system (TGFS) in Wistar rats with persistent hypertension 60 days after removal of the clipped kidney in the Goldblatt (two-kidney, one clip) hypertension model. Ten hypertensive rats (HBP) were compared with 12 normotensive ones (NBP). Micropuncture studies revealed that values for the single nephron glomerular filtration rate (SNGFR), glomerular plasma flow (QA), and afferent oncotic pressure (PAR.A) were similar in both groups, whereas glomerular capillary pressure (PGC) and effective filtration pressure (EFP) were higher in the HBP group (p less than 0.05). A slight but insignificant increase in afferent resistance was present in the HBP group. A positive correlation was found between mean arterial pressure and stop flow pressure (SFP) (r = 0.64, p less than 0.05) but not with SNGFR, suggesting a reduction in the ultrafiltration coefficient in hypertensive rats. This was further supported by studies of the activity of the TGFS, which demonstrated that interrupting flow to the macula densa was followed by a smaller increment in SNGFR in HBP, in spite of a similar rise in SFP. The mechanism responsible for decreasing glomerular permeability is unknown but could be related to structural changes in glomerular capillary or to an increase in intrarenal angiotensin II, as has been demonstrated previously in this model. It is suggested that these adaptations occurring in the kidney exposed to hypertension can contribute to the maintenance of elevated arterial pressure after removing the stenotic kidney.

Objectives We report a case of an infant with nephrogenic diabetes insipidus (NDI) diagnosed by the measurement of serum copeptin. There is only one study that previously evaluated the use of copeptin measurement in a pediatric patient. Case presentation We present a 10-month-old child with polyuria–polydipsia syndrome (PPS) and hypernatremia that could not support water restriction due to increased risk of dehydration and worsening of his condition. Therefore, plasma measurement of copeptin allowed the diagnosis of NDI. Conclusions The water deprivation test (WDT) is considered the gold standard for diagnosis in PPS. However, WDT has serious limitations regarding its interpretation. Furthermore, the WDT can cause dehydration and hypernatremia, especially in young children. Therefore, the measurement of plasma copeptin seems to be a promising method to perform an earlier, safer, and accurate investigation of PPS. Up to now, our study is the second to report the usefulness of copeptin in children.

BACKGROUNDSystemic lupus erythematosus (SLE) is a chronic and inflammatory disease and its control depends on adequate management of comorbidities, adoption of a healthy lifestyle and good adherence to treatment. The Waiting Room Project is a patient educational strategy that stimulates patients' autonomy in the management of the disease by teaching important aspects of self-care and treatment, through the elaboration and distribution of booklets. METHODSThis extension project, created in 2011, produced 17 booklets about different aspects of the disease and comorbidities, such as smoking, hypertension, diabetes, obesity, physical activity, sun protection, healthy food, oral health, vaccination, pregnancy and osteoporosis. In the years 2020/2021, the project focused on SLE treatment and scientific information about COVID-19. The booklets were written in lay language by medical students and rheumatology professors. The students have also been responsible for the graphic design of the booklets and the illustration and organization of the addressed topics in order to provide more accessible and understandable information. The material is handed out to the patients, while they wait for their medical appointment, by the students and care team, who explain the main topics and answer any questions that may come up. A total of 700 lupus patients, currently under treatment at the university hospital, are involved.