Desmopressin Treatment Research Articles

Effect of desmopressin on water and solute circadian rhythms in treatment-naïve children with monosymptomatic enuresis and nocturnal polyuria.

Enuresis has a complex pathophysiology involving nocturnal polyuria, reduced bladder capacity at nighttime, and impaired arousability. Desmopressin has long been used as a treatment. However, approximately 30% of children do not fully respond to it, suggesting the involvement of other factors. Solute handling and osmotic excretion have been studied in refractory patients. Nevertheless, data on the effect of desmopressin on these factors are sparse. We conducted a post hoc analysis of the SLEEP study. We analyzed the circadian rhythm of solute and water excretion before and after desmopressin in 30 children with monosymptomatic enuresis and nocturnal diuresis > 100% of expected bladder capacity by means of a 24-h urine concentration profile (four daytime and four nighttime urine portions at equivalent time intervals). Under desmopressin, nocturnal diuresis (rate) and Na/creatinine ratio were significantly lower compared to day values (p = 0.009, p = 0.021, respectively). Osmolality, Na/creatinine, and osmotic excretion showed a significant day vs. night variance only after desmopressin. Nighttime osmotic and sodium excretion were significantly lower (p = 0.004, p = 0.019, respectively) under treatment, indicating the impact of desmopressin on kidney sodium handling. During desmopressin treatment, nocturnal diuresis (rate) showed strong positive correlation with nighttime Na/creatinine (r = 0.436, p < 0.05) and very strongly with nighttime osmotic excretion (r = 0.875, p < 0.0001). However, no correlation was observed with osmolality under desmopressin treatment. The anti-enuretic and antidiuretic effects of desmopressin therapy are not only related to urinary concentration and nocturnal diuresis but also to the amelioration of circadian rhythms of sodium and solute handling.

7227 Acute Hyponatremia Following Initiation of Antidepressant in Patient on Desmopressin Treatment for AVP Deficiency

Abstract Disclosure: M.E. Horowitz: None. Y. Gao: None. S.B. Abraham: None. P. Kishore: None. Introduction: Intranasal desmopressin (DDAVP) is indicated as antidiuretic replacement therapy in arginine vasopressin (AVP) deficiency. It increases water permeability in the distal nephron, reducing urine output, increasing urine osmolality, and decreasing serum osmolality. Treatment is titrated to normalize serum sodium and urine output (UOP). Initiation of medications affecting sodium balance or fluid status may lead to abnormalities in these markers. One of these is escitalopram, an antidepressant which can cause hyponatremia attributed to inappropriate AVP secretion. We report a case of acute hyponatremia in a patient with previously stable sodium on chronic DDAVP who was started on escitalopram. Clinical Case: A 44-year-old female with history of a craniopharyngioma resection in 2007 complicated by AVP deficiency on chronic DDAVP, bipolar disorder, and depression presented with nausea, vomiting, and dizziness for two days. Patient was found to be hyponatremic to 118 mEq/L (135-145 mEq/L). Sodium checked one month prior was 135 mEq/L, and historically was never less than 130 mEq/L since 2008. DDAVP was prescribed since resection with few dose changes, at this time four 10mcg sprays in each nostril three times daily since 2020. Escitalopram 5mg daily was initiated two months prior. Desmopressin was held in the setting of hyponatremia, which resolved appropriately to 135 mEq/L over the first five days. During this time, the patient complained of large volume diuresis at ∼three liters daily. On day six, sodium acutely increased to 146 mEq/L; subcutaneous (SC) DDAVP was given and sodium normalized. UOP decreased significantly over the next 24 hours, but large volume diuresis resumed thereafter. On day 10, DDAVP SC was given, again decreasing UOP for 24 hours. Psychiatry recommended continuing escitalopram given improvement in psychiatric symptoms since initiation. Upon discharge, the patient was prescribed DDAVP two 10mcg sprays in each nostril nightly, a significant reduction compared to her prior dose. Sodium rechecked one week later remained within normal limits. Clinical Lessons: This effect of combination DDAVP and escitalopram has not been reported previously. Prescribers should be aware of the potential for hyponatremia and the need to adjust DDAVP dose in combined use. To avoid these risks in patients on chronic DDAVP, the side effects of antidepressants and antipsychotics should be reviewed prior to initiation. Presentation: 6/2/2024

Effects of long-term desmopressin treatment for nocturia in older people.

Desmopressin improves nocturia frequency; however, reports on its long-term efficacy and safety are few, and concerns regarding its effect on body composition exist. We thus investigated the efficacy and safety of long-term desmopressin administration and its effect on body composition. This retrospective study, conducted at Chikugo City Hospital between August 2020 and December 2022, involved 133 men (mean age, 77.7 years) with nocturnal and persistent nocturia, who were administered an initial dose of 50 μg desmopressin. Efficacy endpoints included nocturnal urinary frequency, nocturnal urinary volume, hours of undisturbed sleep, nocturnal polyuria index, initial nocturnal urinary volume, and daily urinary frequency in a frequency-volume chart (3 days), before treatment and at 1, 4, 12, 24, and 52 weeks after desmopressin administration. Additionally, the effects of desmopressin on body composition were examined, including blood-brain natriuretic peptide and a chest radiography, before and 52 weeks after administration. Treatment improved most efficacy endpoint evaluation parameters. Around 87.6% of patients showed improved symptoms after 52 weeks compared with those before treatment (score ≤ 3). The blood-brain natriuretic peptide level rose; however, cardiothoracic ratio was unchanged. Long-term administration of desmopressin is thus effective and safe in older people with nocturnal polyuria, with little effect on body composition.

Development of a Novel Prediction Tool for Response to First-Line Treatments of Monosymptomatic Nocturnal Enuresis: A Randomized, Controlled, International, Multicenter Study (DRYCHILD).

Nocturnal urine volume and bladder reservoir function are key pathogenic factors behind monosymptomatic nocturnal enuresis (MNE). We investigated the predictive value of these together with other demographic and clinical variables for response to first-line treatments in children with MNE. A randomized, controlled, international, multicenter study was conducted in 324 treatment-naïve children (6-14 years old) with primary MNE. The children were randomized to treatment with or without prior consideration of voiding diaries. In the group where treatment choice was based on voiding diaries, children with nocturnal polyuria and normal maximum voided volume (MVV) received desmopressin (dDAVP) treatment, and children with reduced MVV and no nocturnal polyuria received an enuresis alarm. In the other group, treatment with dDAVP or alarm was randomly allocated. A total of 281 children (72% males) were qualified for statistical analysis. The change of responding to treatment was 21% higher in children where treatment was individualized compared to children where treatment was randomly selected (risk ratio = 1.21 [1.02-1.45], P = .032). In children with reduced MVV and no nocturnal polyuria (35% of all children), individualized treatment was associated with a 46% improvement in response compared to random treatment selection (risk ratio = 1.46 [1.14-1.87], P = .003). Furthermore, we developed a clinically relevant prediction model for response to dDAVP treatment (receiver operating characteristic curve 0.85). The present study demonstrates that treatment selection based on voiding diaries improves response to first-line treatment, particularly in specific subtypes. Information from voiding diaries together with clinical and demographic information provides the basis for predicting response. NCT03389412.

Management of the refractory nocturnal enuresis patient to desmopressin in a pediatric population: Desmopressin + oxybutynin vs. desmopressin + imipramine

Desmopressin is well accepted as first-line medical therapy for enuresis. If ineffective, combination therapy of desmopressin+oxybutynin or desmopressin+imipramine has been used. This study assessed the efficacy of adjunct therapy with either imipramine or oxybutynin in the management of enuresis patients who failed desmopressin treatment. A retrospective chart review of our database for patients with enuresis was performed. Patients who were prescribed desmopressin, oxybutynin, and imipramine over 14 years for enuresis were included. Two cohorts of patients were examined; group OXY was treated with desmopressin and oxybutynin, and group IMP received desmopressin and imipramine. Pretreatment measurement of Vancouver Symptom Scores (VSS) were used to compare groups using the VSS question "I wet my bed at night" where 4: every night, 3: 4-5 nights per week, 2: 1-2 nights per week, 1: 3-4 nights per month, and 0: never. International Children's Continence Society (ICCS) criteria for continence success was utilized to determine outcomes. 2521 patients prescribed one of the 3 medications were identified. Among them, 81 patients (mean age: 10.5±2.8 years) received combination therapy. Of which, 55 were male and 26 female. Specifically, 58 were prescribed both desmopressin and imipramine (group IMP), 23 desmopressin and oxybutynin (group OXY), and 4 transitioned from OXY to IMP. Mean pretreatment VSS showed no difference between groups. Both groups experienced minimal drops in wet nights with desmopressin alone. A comparison revealed that group IMP reduced wet nights significantly more than group OXY (VSS wet night score 0.7±1.2 vs. 2.3±1.1 respectively, p<0.0001). Non-intent-to-treat complete response rate was 68% vs 5% (OR=42.5, p<0.001) (IMP vs. OXY respectively). Intent-to-treat response rates were 58%. Although first-line desmopressin treatment for enuresis is effective, it does not work for all patients, and many parents and children desire nighttime dryness. Clinicians have combined desmopressin with oxybutynin or imipramine for improved results, but research comparing these modalities is scarce. Our study suggests that the desmopressin and imipramine combination is superior at reducing nights wet compared to desmopressin and oxybutynin, attributed to imipramine's probable central mechanism rather than its secondary anticholinergic properties. Limitations include a modest sample size, retrospective design, and subjective responses to the Vancouver questionnaire. A combination of desmopressin and imipramine was more effective in reducing wet nights and had a complete response rate that was 42.5 times greater than desmopressin and oxybutynin.

Does Desmopressin Reduce Intraoperative Bleeding in Patients Who Undergo Nasal Surgery? A Systematic Review and Meta-Analysis

Background and Objectives: This study aimed to determine the efficacy of prophylactic desmopressin administered via the intranasal or intravenous route in reducing intraoperative bleeding during nasal surgery. We conducted a meta-analysis of the relevant literature to investigate the role of preoperative desmopressin in minimizing bleeding complications associated with nasal surgery.Methods: We screened the relevant literature published before February 2023. Nine articles that compared the perioperative use of desmopressin (treatment) with a placebo or no treatment (control) were included. The analyzed outcomes were intraoperative bleeding during nasal surgery and postoperative morbidity.Results: The treatment group showed significant improvements in intraoperative bleeding, the surgical field, and surgeon satisfaction compared to the control group. However, the prophylactic use of desmopressin was associated with elevated blood pressure and decreased serum sodium levels compared to the control group. Nonetheless, no significant adverse effects were reported in the included studies. Subgroup analyses comparing the route of administration (intravenous vs. intranasal) and type of surgery (rhinoplasty vs. endoscopic sinus surgery) showed that desmopressin had a beneficial effect on intraoperative bleeding and the surgical field, regardless of the route of administration or type of surgery.Conclusion: The prophylactic use of desmopressin for nasal surgery effectively reduced intraoperative bleeding, improved the surgical field, and increased surgeon satisfaction, with no significant adverse effects. However, caution should be exercised when administering desmopressin as it may cause an elevation in postoperative blood pressure in patients with cardiopulmonary problems.

The Rate of Relapse of Primary Monosymptomatic Nocturnal Enuresis with Desmopressin Treatment in Comparison with Combined Treatment of Desmopressin and Tolterodine in Children Aged 5 to 16 Years

The Rate of Relapse of Primary Monosymptomatic Nocturnal Enuresis with Desmopressin Treatment in Comparison with Combined Treatment of Desmopressin and Tolterodine in Children Aged 5 to 16 Years

Tachyphylaxis and reproducibility of desmopressin response in perioperative persons with nonsevere hemophilia A: implications for clinical practice

BackgroundDesmopressin is frequently used perioperatively in persons with nonsevere hemophilia A. However, increase in factor (F)VIII:C after desmopressin use is interindividually highly variable. Tachyphylaxis has only been reported in test setting for persons with hemophilia A, with a remaining response of approximately 70% after a second dose compared with that after a first dose. ObjectivesTo study tachyphylaxis of FVIII:C response after multiple administration(s) of desmopressin in perioperative persons with nonsevere hemophilia A. MethodsWe studied FVIII:C levels after desmopressin before (day 0 [D0]) and on days 1 (D1) and 2 (D2) after surgery in 26 patients of the DAVID and Little DAVID studies. We studied tachyphylaxis by comparing the responses at D1 and D2 with that at D0. We also assessed the reproducibility of the D0 response in comparison to an earlier performed desmopressin test. ResultsThe median absolute FVIII:C increase was 0.50 IU/mL (0.35-0.74; n = 23) at D0, 0.21 IU/mL (0.14-0.28; n = 17) at D1, and 0.23 IU/mL (0.16-0.30; n = 11) at D2. The median percentage of FVIII increase after the second administration (D1) compared with the first (D0) was 42.9% (29.2%-52.5%; n = 17) and that of the third (D2) compared with the first (D0) was 36.4% (23.7%-46.9%; n = 11). The FVIII:C desmopressin response at D0 was comparable with the desmopressin test response in 74% of the patients. ConclusionTachyphylaxis in the surgical setting was considerably more pronounced than previously reported, with FVIII:C at D1 and D2 of 36% to 43% of the initial response. Our results may have important implications for monitoring repeated desmopressin treatment when used perioperatively.

Children with sleep enuresis: does alarm, desmopressin, or combined treatment impact their sleep profile?

Children with sleep enuresis: does alarm, desmopressin, or combined treatment impact their sleep profile?

Hyponatremia due to preserved non-osmotic arginine vasopressin secretion in adipsic diabetes insipidus: a case report with review of literature.

Adipsic diabetes insipidus (ADI) is characterized by central diabetes insipidus and an impaired thirst response to hyperosmolality, leading to hypernatremia. Hyponatremia observed in patients with ADI has been considered a complication of desmopressin therapy. Herein, we present a case of impaired thirst sensation and arginine vasopressin (AVP) secretion without desmopressin therapy, in which hyponatremia developed due to preserved non-osmotic AVP secretion. A 53-year-old woman with hypopituitarism, receiving hydrocortisone and levothyroxine, experienced hyponatremia three times over 5 months without desmopressin treatment. The first hyponatremic episode (120 mEq/L) was complicated by a urinary tract infection with a plasma AVP level of 33.8 pg/mL. Subsequent hyponatremia episodes occurred after administration of antipsychotic (124 mEq/L) and spontaneously (125 mEq/L) with unsuppressed plasma AVP levels (1.3 and 1.8 pg/mL, respectively). Hypertonic saline infusion did not affect AVP or copeptin levels. Regulating water intake using a sliding scale based on body weight prevented the recurrence of hyponatremia without the use of desmopressin. Except during infection, plasma AVP levels (1.3 ± 0.4 pg/mL) were not significantly correlated with serum sodium levels (rs = -0.04, p = 0.85). In conclusion, we present a unique case of impaired thirst sensation and AVP secretion in which hyponatremia developed without desmopressin therapy. Preserved non-osmotic AVP secretion, possibly stimulated by glucocorticoid deficiency, may contribute to the development of hyponatremia in patients with ADI.

Immunoglobulin-G4 Related Disease with Multiple Organ Involvement

Dear Editor, Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory disease affecting multiple organ systems. The clinical findings may range due to the affected organ. The main distinguishing histopathological features of IgG4-RD are lymphoplasmacytic infiltration, storiform fibrosis, obliterative phlebitis, and mild or moderate tissue eosinophilia. Rarely, it may affect the lungs, hearts, pituitary, meninges, skin, prostate, breast, and thyroid gland [1–3]. In this article, we present a case diagnosed with IgG4-related disease involving multiple organs, including the pituitary, lymphatic system, kidney, and heart, and the patient responded well to immunosuppressive treatment. A fifty-five-year-old female patient applied with complaints of fatigue, polydipsia, polyuria, widespread body pain, and 20 kg weight loss in the last year. After excluding other possibilities, with a prediagnosis of diabetes insipidus (DI) pituitary MRI was performed which showed an increase in size and heterogeneous patchy contrast enhancement in the adenohypophysis (Fig. 1a). Based on the current clinical and imaging findings, the patient's laboratory results were evaluated, and central DI was diagnosed. In computerized thorax tomography, multiple lymph nodes in the mediastinum were detected, the largest of which was 21x17 mm. Tissue sampling was performed with the guidance of EBUS, pathological examination showed no diagnostic findings. Transesophageal Echocardiography (TEE) revealed an appearance consistent with a 10-15 mm thick thrombus surrounding the left atrium wall and narrowing the cavity. Increased thickness narrowing the left atrial lumen was reported in thorax computed tomography (CT) (Fig. 2). Further, a cardiac MRI was performed and reported to be consistent with lymphoproliferative-inflammatory involvement rather than thrombus. Abdominopelvic CT was performed and a lesion of 54x28 mm in size, less contrast enhancing than the surrounding parenchyma, in the middle part posterior of the left kidney was detected (Fig. 3). Histopathological findings were consistent with inflammatory processes, and no findings in favor of a neoplastic lymphoproliferative process were detected in the samples. Since the patient was presented with pituitary involvement, mediastinal lymphadenopathy, renal and cardiac mass, the IgG4 level was ordered and resulted as 299 mg/dl (3-201). IgG4 staining could not be performed in the current biopsy specimen; for confirming the diagnosis of IgG4-related disease, a re-biopsy was performed on the kidney mass. Histopathological findings were consistent with IGG4-RD (Fig. 4). Due to multisystemic involvement, the patient received 0.6 mg/kg/day oral corticosteroid and mycophenolate mofetil 3x1000 mg/day. The pituitary MRI that was performed in the first month of treatment was normal (Fig. 1b). Desmopressin treatment was stopped. Also, control TEE in the first-month follow-up visit showed a significant reduction in the mass image in the left atrium. After three months of follow-up, there was a significant improvement in the patient's symptoms and acute phase response. The corticosteroid was tapered and maintenance treatment with mycophenolate mofetil was continued. IgG4-related disease is rare and difficult to diagnose, though its presentation may be in a wide variety of clinical features. It is crucial to make an early diagnosis and start treatment early in these patients to prevent morbidity and mortality. In cases with mass lesions and especially with multiple organ involvement, as in our case, IgG4-related disease should be kept in mind, and IgG4 staining should always be kept in mind. Kind Regards

Arginine vasopressin regulates the renal Na+-Cl- and Na+-K+-Cl- cotransporters through with-no-lysine kinase 4 and inhibitor 1 phosphorylation.

Vasopressin regulates water homeostasis via the V2 receptor in the kidney at least in part through protein kinase A (PKA) activation. Vasopressin, through an unknown pathway, upregulates the activity and phosphorylation of Na+-Cl- cotransporter (NCC) and Na+-K+-2Cl- cotransporter 2 (NKCC2) by Ste20-related proline/alanine-rich kinase (SPAK) and oxidative stress-responsive kinase 1 (OSR1), which are regulated by the with-no-lysine kinase (WNK) family. Phosphorylation of WNK4 at PKA consensus motifs may be involved. Inhibitor 1 (I1), a protein phosphatase 1 (PP1) inhibitor, may also play a role. In human embryonic kidney (HEK)-293 cells, we assessed the phosphorylation of WNK4, SPAK, NCC, or NKCC2 in response to forskolin or desmopressin. WNK4 and cotransporter phosphorylation were studied in desmopressin-infused WNK4-/- mice and in tubule suspensions. In HEK-293 cells, only wild-type WNK4 but not WNK1, WNK3, or a WNK4 mutant lacking PKA phosphorylation motifs could upregulate SPAK or cotransporter phosphorylation in response to forskolin or desmopressin. I1 transfection maximized SPAK phosphorylation in response to forskolin in the presence of WNK4 but not of mutant WNK4 lacking PP1 regulation. We observed direct PP1 regulation of NKCC2 dephosphorylation but not of NCC or SPAK in the absence of WNK4. WNK4-/- mice with desmopressin treatment did not increase SPAK/OSR1, NCC, or NKCC2 phosphorylation. In stimulated tubule suspensions from WNK4-/- mice, upregulation of pNKCC2 was reduced, whereas upregulation of SPAK phosphorylation was absent. These findings suggest that WNK4 is a central node in which kinase and phosphatase signaling converge to connect cAMP signaling to the SPAK/OSR1-NCC/NKCC2 pathway.NEW & NOTEWORTHY With-no-lysine kinases regulate the phosphorylation and activity of the Na+-Cl- and Na+-K+-2Cl- cotransporters. This pathway is modulated by arginine vasopressin (AVP). However, the link between AVP and WNK signaling remains unknown. Here, we show that AVP activates WNK4 through increased phosphorylation at putative protein kinase A-regulated sites and decreases its dephosphorylation by protein phosphatase 1. This work increases our understanding of the signaling pathways mediating AVP actions in the kidney.

Lymphocytic Infundibuloneurohypophysitis Diagnosis Owing to Positive Anti-rabphilin-3A Antibody Test Result in an 8-year-old Boy with Early-onset Central Diabetes İnsipidus.

Childhood-onset lymphocytic infundibuloneurohypophysitis (LINH) has rarely been reported. Pathological evaluation via pituitary biopsy is necessary for a definitive diagnosis of LINH. However, pituitary biopsy is a highly invasive procedure. Recently, anti-rabphilin-3A antibody (RPH3A-Ab) has been reported as a promising diagnostic marker for LINH in adults; however, there are few such reports in the pediatric population. We report the case of an 8-year-old boy with central diabetes insipidus (CDI) who was clinically diagnosed with LINH based on RPH3A-Ab positivity. He was diagnosed with CDI using a water deprivation test combined with desmopressin administration. Serum and cerebrospinal fluid tumor markers were negative, and T1-weighted magnetic resonance imaging (MRI) revealed the absence of high signal intensity in the posterior pituitary gland and an enlarged pituitary stalk. Anterior pituitary function tests revealed no abnormalities. No pituitary biopsy was performed because of its invasive nature, and desmopressin treatment was initiated. Three months after CDI onset, the patient tested positive for RPH3A-Ab. MRI performed 9 months after CDI onset revealed amelioration of the pituitary stalk enlargement, and the clinical course corroborated our diagnosis of LINH. RPH3A-Ab may be useful as an early diagnostic tool for LINH in the pediatric population.

Efficacy and safety of desmopressin in nocturia and nocturnal polyuria control of neurological patients: A systematic review and meta-analysis.

Evidence on the efficacy of desmopressin in nocturia in patients with neurological diseases is still very limited except for multiple sclerosis (MS). Our aim was to evaluate the efficacy and safety of desmopressin treatment on nocturia in patients with underlying neurological diseases. Studies were identified by electronic search of PubMed, Embase, Cochrane, CINAHL, and Google Scholar databases. Studies were considered if they provided information on the effectiveness and safety of desmopressin (1-desamino-8-d-arginine vasopressin, or DDAVP) in the treatment of nocturia and their participants had acquired neurological pathology. Two researchers independently extracted the articles using specified datasets, such as quality-of-study indicators. Statistical meta-analysis was carried out using Review Manager (RevMan) 5.4 statistical software (Cochrane Collaboration). Of a total of 1042 articles in the initial search, 14 studies were included. Most of the published papers were related to MS (n = 7), two were on spinal cord injury,and other conditions were neural tube defect,myelodysplasia, Parkinson's disease, stroke, and multiple system atrophy. Overall, a total of 200 patients (mostly females) were enrolled. Thirteen studies evaluated the intranasal formulation of desmopressin and one study evaluated oral desmopressin. A significant decrease in nocturia episodes was reported in seven studies evaluating this topic. An increase in the maximum hours of uninterrupted sleep was reported in the three studies in which this outcome was assessed. A significant reduction in the volume of nocturnal incontinence was found in one study. Three studies were eligible to include in the meta-analysis. The results showed that desmopressin compared to placebo, significantly reduced nighttime urination (mean difference: -0.75, 95% CI: -1.10 to-0.41; p < 0.00001). The rate of adverse events ranged from 0% to 68.42%. The critical appraisal results for all trials showed that most of the studies had low or moderate quality. Our results emphasized desmopressin's safety and efficacy in reducing nocturia episodes, with transient adverse effects on neurological patients. However, the data were achieved from low or medium-quality trials, and further well-designed randomized controlled trials are needed.

AN INVESTIGATION OF THE RELATIONSHIP BETWEEN THE QUALITY OF LIFE OF THE CHILD PATIENT AND THE PARENT AFTER DESMOPRESSIN TREATMENT IN CHILDREN WITH PRIMARY NOCTURNAL ENURESIS

Our study aimed to assess the quality of life of both children with monosymptomatic enuresis and their parents during the enuresis period and through the process of cessation with desmopressin treatment. Materials and methods: The study assessed 78 children aged 6-18 with Monosymptomatic Nocturnal Enuresis (MEN) treated with desmopressin. Comprehensive evaluations, including physical exams and lab tests, were conducted. The surveys on the impact of enuresis on daily life and emotional well-being were evaluated. Results: The study observed 78 patients, averaging 10.6 years old, primarily treated for urinary incontinence with desmopressin. Patient demographics indicated 68% had education below high school, 74% were Turkish, and 26% Syrian. Familial data revealed an average of 3.87 siblings per patient. Key findings included a significant reduction in the number of incontinence days per week post-treatment (from 5.09 days to 2.59 days). Quality of Life (QOL) scores for both patients and parents showed significant improvement after treatment. The mean QOL for patients increased from 20 to 26.8, while for parents, it rose from 13 to 24.6. Overall, desmopressin demonstrated a positive impact on urinary incontinence and the associated quality of life. Conclusions: With treatment, there is a noticeable improvement in psychological, social, and emotional aspects among children with MEN and their parents. Therefore, it is essential to treat children experiencing MEN.

Clinical observation on the efficacy of Tongdu Tuina manipulation in the treatment of primary enuresis in children.

The objective was to explore the efficacy of Tongdu Tuina manipulation in the treatment of primary single-symptom enuresis in children. A total of 102 children aged 5-16 with primary single-symptom enuresis were included in this study and randomly assigned to the Tuina group, the medication group and the control group, with 34 children in each group. The Tongdu Tuina group included manipulation of the Guanyuan, Qihai, Zhongji, Mingmen, kidney, Baihui, Sishencong and bladder acupoints, five times a week, the medication group was treated with 0.1 mg desmopressin acetate every night, and in the control group, the patients were given foods with high water content and underwent water deprivation 2 h before bedtime every night. The intervention time of each group was 1 month. The participants were followed up on Day 1 following treatment, as well as half a month, 1 month and 3 months after the implementation of the intervention measures, and the effective rate, the incidence of enuresis per week and the recurrence rate were calculated. As a result baseline demographic characteristics were comparable among 102 patients. Overall, 32 patients in the Tongdu Tuina group, 30 patients in the medication group and 34 patients in the control group completed the intervention. After half a month of treatment, there was no significant difference in the therapeutic efficacy among the three groups (P = 0.158), but each treatment could effectively reduce the frequency of weekly enuresis. The frequency of weekly enuresis in the Tongdu Tuina group was 3.8 ± 1.1 times, while that in the medication group was 4.0 ± 2.0 times. The frequency of weekly enuresis in the control group was 4.7 ± 1.8 times, and the difference was statistically significant (P = 0.016). After 1 month of treatment, the effective rates of the Tongdu Tuina group and the medication group were significantly increased (87.5% vs 83.33%, P < 0.0001), which was not the case with the control group. The frequency of enuresis was 1.9 ± 2.1 times per week in the Tongdu Tuina group, 2.4 ± 1.8 times per week in the medication group and 4.0 ± 0.9 times per week in the control group after 1 month of treatment. The difference between the three groups was statistically significant (P = 0.021), and there was a difference between the Tongdu Tuina group and the medication group (P < 0.0001). There was no significant difference between recurrence rate and the incidence of adverse events (P = 0.837, P = 0.856). In conclusion, both Tuina manipulation and desmopressin treatment can effectively improve children's primary single-symptom enuresis with safety. However, Tongdu Tuina therapy may be superior to desmopressin treatment.

Desmopressin in nonsevere hemophilia A: patient perspectives on use and efficacy

BackgroundDesmopressin increases plasma factor VIII and von Willebrand factor levels in persons with nonsevere hemophilia A. Patients’ perspectives on desmopressin are relevant to increase and optimize its suboptimal use. However, patients’ views on desmopressin are not reported. ObjectivesTo evaluate the perspectives of persons with nonsevere hemophilia A on desmopressin use, barriers for its use, side effects, and their knowledge about desmopressin’s efficacy and side effects. MethodsPersons with nonsevere hemophilia A were included in a cross-sectional, national, multicenter study. Questionnaires were filled out by adult patients and children aged ≥12 years themselves. Caretakers filled out questionnaires for children aged <12 years. ResultsIn total, 706 persons with nonsevere hemophilia A were included (544 mild, 162 moderate, [age range, 0–88 years]). Of 508 patients, 234 (50%) patients reported previous desmopressin use. Desmopressin was considered as at least moderately effective in 171 of 187 (90%) patients. Intranasal administration was the modality of choice for 138 of 182 (76%) patients. Flushing was the most reported side effect in 54 of 206 (26%) adults and 7 of 22 (32%) children. The most frequently reported advantage and disadvantage were the convenience of intranasal, out-of-hospital administration by 56% (126/227) and side effects in 18% (41/227), respectively. Patients’ self-perceived knowledge was unsatisfactory or unknown in 28% (63/225). ConclusionOverall, desmopressin was most often used intranasally and considered effective, with flushing as the most common side effect. The most mentioned advantage was the convenience of intranasal administration and disadvantage was side effects. More information and education on desmopressin could answer unmet needs in patients with current or future desmopressin treatment.

Central diabetes insipidus: a late sequela of BNT162b2 SARS-CoV-2 mRNA vaccine?

BackgroundThe development of an effective vaccine is a powerful tool to contain the global spread of coronavirus disease 2019 (COVID-19). Still, it raises potential safety concerns about the subsequent enhancement of associated immunopathology. Increasing evidence shows that the endocrine system, including the hypophysis, may be involved in COVID-19. Moreover, occasional but increasing reports of endocrine disorders involving the thyroid have been reported after the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccine. Among them, a few cases encompass the pituitary. Here we report a rare case of central diabetes insipidus following SARS-CoV-2 vaccination.Case presentationWe report a 59-year-old female patient with a 25-year history of Crohn's disease in long-term remission, who presented with sudden onset of polyuria eight weeks after administration of an mRNA SARS-CoV-2 vaccination. Laboratory evaluation was consistent with isolated central diabetes insipidus. Magnetic resonance imaging displayed involvement of the infundibulum and the posterior hypophysis. Eighteen months after the vaccination, she is still under desmopressin treatment and had stable pituitary stalk thickening on magnetic resonance imaging. Although Crohn's disease-associated hypophysitis has been reported, it is scarce. In the absence of other recognizable causes of hypophysitis, we believe the involvement of the hypophysis in our patient may have been triggered by the SARS-CoV-2 vaccine.ConclusionsWe report a rare case of central diabetes insipidus potentially associated with SARS-CoV-2 mRNA vaccination. Further studies are needed to understand better the mechanisms underlying autoimmune endocrinopathies development in the context of COVID-19 infection and SARS-CoV-2 vaccination.

Predictive factors of full response to desmopressin treatment in children with primary monosymptomatic enuresis nocturna

Predictive factors of full response to desmopressin treatment in children with primary monosymptomatic enuresis nocturna

Rhinoplasty on Patients with von Willebrand's Disease

Von Willebrand's disease (VWD) is the most common hereditary bleeding disorder. Due to its frequency, occasionally, patients with this disease apply for rhinoplasty. Classically, desmopressin is used in emergent cases, and it is highly effective. However, the lack of long-term treatment and possible side effects limit the desmopressin treatment. Because the fibrinolytic activity in the nasal mucosa is high, antifibrinolytics such as tranexamic acid are beneficial in all types of VWD. An 18-year-old patient with Type 1 VWD who was operated without facing any complications is presented. Treatment involved 4 days of treatment starting the day before the surgery and healing process was uneventful. Patients with a diagnosis of VWD can be operated successfully with preoperative tranexamic acid treatment and using vasoconstrictor agents and avascular surgery planes. In Type 1 VWD or low von Willebrand's factor cases, tranexamic acid is a safe and successful treatment alternative due to its long-term use, local and systemic applicability, and regional affinity.