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European Urology Focus Jan 2022Neurological disease can affect the rate of urine production and bladder storage function, increasing nocturia severity, with additional risks if mobility or cognition... (Review)
Review
CONTEXT
Neurological disease can affect the rate of urine production and bladder storage function, increasing nocturia severity, with additional risks if mobility or cognition is impaired.
OBJECTIVE
To conduct a systematic review (SR) of nocturia in neurological diseases and achieve expert consensus for management in clinics without neurologist input.
EVIDENCE ACQUISITION
Four databases were searched from January 2000 to April 2020. A total of 6262 titles and abstracts were screened and 43 studies were included for full-text screening. Eleven of these met the inclusion criteria and two studies were identified through other sources. The nominal group technique (NGT) was used to develop consensus in panel comprising experts and public representation.
EVIDENCE SYNTHESIS
Thirteen studies (seven in Parkinson's disease, five in multiple sclerosis) were included, all undertaken in secondary care. Neurological disease severity was incompletely described, and nocturia severity was generally measured subjectively. NGT consensus supported basic neurological assessment, and the use of bladder diaries where neurological impairment permits. Treatments include pelvic-floor muscle training, review of medications, risk mitigation, improving bowel function, therapy for overactive bladder syndrome (if urgency is reported in association with nocturia episodes), treatment of postvoid residual and desmopressin according to licence. Measures to improve mobility and mitigate risk when using the toilet overnight should be considered. Multifactorial issues such as obstructive sleep apnoea and hypoventilation must be considered.
CONCLUSIONS
Nocturia in neurological disease is complex and lacks a robust evidence base, with very little research done in the primary care context. Guidance should be pragmatic, with reduction of risk a key requirement, until a multidisciplinary evidence base can be developed.
PATIENT SUMMARY
People with a neurological disease can suffer severe sleep disturbance because of the need to pass urine several times overnight (called nocturia). We looked at published research and found very little information to help general practitioners in managing this condition. We assembled a group of experts to develop practical approaches for assessing and treating nocturia in neurological disease.
Topics: Consensus; Humans; Nervous System Diseases; Nocturia; Primary Health Care; Severity of Illness Index
PubMed: 35031351
DOI: 10.1016/j.euf.2021.12.012 -
Haemophilia : the Official Journal of... Mar 2022von Willebrand disease (VWD) is the common bleeding disorder with a clinically relevant bleeding prevalence of 1:10,000. von Willebrand disease patients lack both von...
Efficacy of emicizumab in von Willebrand disease (VWD) patients with and without alloantibodies to von Willebrand factor (VWF): Report of two cases and review of literature.
INTRODUCTION
von Willebrand disease (VWD) is the common bleeding disorder with a clinically relevant bleeding prevalence of 1:10,000. von Willebrand disease patients lack both von Willebrand factor (VWF) and factor VIII (FVIII), which are critical for normal haemostasis. The conventional treatment for VWD includes desmopressin and replacement therapy with plasma derived FVIII with VWF concentrates or recombinant VWF. Development of alloantibodies is a rare occurrence, there is a paucity in the literature of treatment modalities in these patients. Not many reports are available in literature on the efficacy of emicizumab in VWD patients with or without alloantibodies to VWF.
AIM
To do systematic review of literature on emicizumab in VWD and report our experience of emicizumab in two patients of VWD METHODS: We used electronic search engines till May 2021 in 'Google scholar' and 'PubMed', to collect the case reports or case series on use of emicizumab for management of VWD. Two of our severe VWD patients were successfully treated with emicizumab. A systematic review was performed and the results discussed.
RESULTS
The electronic search revealed six case reports using emicizumab for treatment of VWD. Two were in vitro studies and four in patients with VWD type 3 disease. In vitro studies and in VWD patients on emicizumab, showed improvement in thrombin generation and fibrin formation. Among four patients, three had alloantibodies to VWD and one was negative. All these patients were treated with emicizumab for 6-12 m. After starting emicizumab, none of them had spontaneous bleeding requiring treatment. During treatment with emicizumab, one patient had trauma-associated soft tissue hematoma, which was treated with rFVIIa and another patient had bleeding following dental exfoliation treated with Humate P. We treated two of our VWD patients one with and one without inhibitors with emicizumab after failure of other therapies. Both the patients showed marked improvement and continued to remain well and free of bleeding episodes. None of the patients had any thrombosis or thrombotic microangiopathy (TMA) during treatment with emicizumab.
CONCLUSION
In conclusion, this review supports the safety and efficacy of emicizumab in type 3 VWD patients with or without alloantibodies. Further large studies are required to confirm the safety and efficacy of emicizumab in VWD.
Topics: Antibodies, Bispecific; Antibodies, Monoclonal, Humanized; Factor VIII; Humans; Isoantibodies; von Willebrand Diseases; von Willebrand Factor
PubMed: 35014121
DOI: 10.1111/hae.14491 -
Explore (New York, N.Y.) 2022The efficacy of different forms of acupuncture for the treatment of nocturnal enuresis in children is not known. (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
The efficacy of different forms of acupuncture for the treatment of nocturnal enuresis in children is not known.
OBJECTIVE
To determine the efficacy of different forms of acupuncture, such as manual acupuncture, laser/electroacupuncture, acupoint injection, and moxibustion, for the treatment of nocturnal enuresis.
METHODS
A literature search was conducted on Medline, EMBASE, Web of Science, CINAHL, PubMed, Physiotherapy Evidence Database, and Scopus from database inception to September 2020. The Cochrane risk of bias tool was utilised to evaluate the risk of bias in each included study. The quality of the evidence was evaluated using the Grading of Recommendations, Assessment, Development, and Evaluation tool.
RESULTS
Thirteen trials (n = 890) were included. Meta-analyses revealed significantly greater numbers of children reporting improved nocturnal enuresis in the moxibustion (p = 0.004), acupoint injection (p = 0.020), and laser acupuncture (p = 0.001) groups than in the control groups. Meta-analyses showed no significant differences in the numbers of children reporting the complete cure of nocturnal enuresis between laser acupuncture and desmopressin (p = 0.57).
CONCLUSIONS
The review identified moxibustion, acupoint injections, and laser acupuncture as effective treatments for nocturnal enuresis in children. However, the evidence for these interventions is limited and of very-low-grade quality. The effects of laser acupuncture compared with desmopressin remain inconclusive.
Topics: Acupuncture Therapy; Child; Deamino Arginine Vasopressin; Humans; Moxibustion; Nocturnal Enuresis; Treatment Outcome
PubMed: 34893441
DOI: 10.1016/j.explore.2021.11.008 -
Archives of Gynecology and Obstetrics Sep 2022Acute Sheehan's syndrome is a rare, but potentially life-threatening, obstetric event that can be complicated by diabetes insipidus. Little information on the diagnosis...
PURPOSE
Acute Sheehan's syndrome is a rare, but potentially life-threatening, obstetric event that can be complicated by diabetes insipidus. Little information on the diagnosis and treatment of Sheehan's syndrome with diabetes insipidus is available. We report on a 28-year-old patient who developed acute Sheehan's syndrome with diabetes insipidus after giving birth, and on a systematic review of similar cases.
METHODS
We performed a systematic review of the literature cataloged in PubMed and Google Scholar using the keywords "Sheehan syndrome" OR "Sheehan's syndrome" AND "diabetes insipidus" to identify relevant case reports published between 1990 and 2021. Eight Reports met the inclusion criteria (English-language abstracts available, onset in the puerperium, information about the day of the onset).
RESULTS
In the present case, postpartum curettage was necessary to remove the residual placenta. The total amount of blood loss was severe (2500 ml). On the second day postpartal, the patient developed polyuria. Laboratory analysis revealed hypernatremia with increased serum osmolality and decreased urinary osmolality. Hormone analysis showed partial hypopituitarism involving the thyroid, corticotropic, and gonadotropic axes. The prolactin level was elevated. Brain magnetic resonance imaging showed pituitary gland infarction. Desmopressin therapy was initiated and resolved the polyuria. Hormone replacement therapy was administered. Four months later, the patient was well, with partial diabetes insipidus. The literature review indicated that this case was typical in terms of symptoms and disease onset. Most reported cases involve hypotension and peripartum hemorrhage, but some patients without hemorrhage also develop Sheehan's syndrome. Elevated prolactin levels are uncommon and associated with poor prognosis in patients with Sheehan's syndrome.
CONCLUSION
Acute Sheehan's syndrome with diabetes insipidus involves nearly all pituitary hormone axes, indicating severe disease. Prolactin elevation could suggest that a case of Sheehan's syndrome is severe.
Topics: Adult; Diabetes Mellitus; Female; Humans; Hypopituitarism; Polyuria; Postpartum Hemorrhage; Postpartum Period; Pregnancy; Prolactin
PubMed: 34779875
DOI: 10.1007/s00404-021-06294-2 -
Seminars in Thrombosis and Hemostasis Apr 2022Mortality after aneurysmal subarachnoid hemorrhage (aSAH) is augmented by rebleeding and delayed cerebral ischemia (DCI). A range of assays evaluating the dynamic...
Mortality after aneurysmal subarachnoid hemorrhage (aSAH) is augmented by rebleeding and delayed cerebral ischemia (DCI). A range of assays evaluating the dynamic process of blood coagulation, from activation of clotting factors to fibrinolysis, has emerged and a comprehensive review of hemostasis and fibrinolysis following aSAH may reveal targets of treatment. We conducted a systematic review of existing literature assessing coagulation and fibrinolysis following aSAH, but prior to treatment. PubMed, Embase, and Web of Science were searched on November 18, 2020, without time boundaries. In total, 45 original studies were eventually incorporated into this systematic review, divided into studies presenting data only from conventional or quantitative assays ( = 22) and studies employing dynamic assays ( = 23). Data from conventional or quantitative assays indicated increased platelet activation, whereas dynamic assays detected platelet dysfunction possibly related to an increased risk of rebleeding. Secondary hemostasis was activated in conventional, quantitative, and dynamic assays and this was related to poor neurological outcome and mortality. Studies systematically investigating fibrinolysis were sparse. Measurements from conventional or quantitative assays, as well as dynamic fibrinolysis assays, revealed conflicting results with normal or increased lysis and changes were not associated with outcome. In conclusion, dynamic assays were able to detect reduced platelet function, not revealed by conventional or quantitative assays. Activation of secondary hemostasis was found in both dynamic and nondynamic assays, while changes in fibrinolysis were not convincingly demonstrable in either dynamic or conventional or quantitative assays. Hence, from a mechanistic point of view, desmopressin to prevent rebleeding and heparin to prevent DCI may hold potential as therapeutic options. As changes in fibrinolysis were not convincingly demonstrated and not related to outcome, the use of tranexamic acid prior to aneurysm closure is not supported by this review.
Topics: Brain Ischemia; Fibrin Clot Lysis Time; Fibrinolysis; Hemostasis; Humans; Subarachnoid Hemorrhage
PubMed: 34261149
DOI: 10.1055/s-0041-1730346 -
Blood Coagulation & Fibrinolysis : An... Sep 2021Unclassified bleeding disorders account for 2.6% of all new bleeding disorder registrations in the UK. The management of the bleeding phenotype associated with these... (Meta-Analysis)
Meta-Analysis
UNLABELLED
Unclassified bleeding disorders account for 2.6% of all new bleeding disorder registrations in the UK. The management of the bleeding phenotype associated with these disorders is poorly described. Systematic review and meta-analysis to determine the bleeding rates associated with tranexamic acid, desmopressin, platelet transfusion, plasma transfusion and recombinant activated factor VII, for patients with unclassified bleeding disorders undergoing surgery, childbirth or with menorrhagia. We searched for randomized controlled trials in MEDLINE, Embase, The Cochrane Central Register of Controlled Trials, PubMed, ISI Web of Science and the Transfusion Evidence Library from inception to 24 February 2020. Wherever appropriate, data were pooled using the metaprop function of STATA. Two studies with 157 participants with unclassified bleeding disorders were identified. The pooled risk of minor bleeding for patients undergoing surgery treated with peri-operative tranexamic acid was 11% (95% confidence interval 3--20%; n = 52; I2 = 0%); the risk for desmopressin and tranexamic acid in combination was 3% (95% confidence interval 0--7%; n = 71; I2 = 0%). There were no instances of major bleeding. In one procedure, 1 of 71 (1.4%), treated with a combination of desmopressin and tranexamic acid, the patient had a line-related deep vein thrombosis. There were too few patients treated to prevent postpartum haemorrhage or for menorrhagia to draw conclusions. The GRADE quality of evidence was very low suggesting considerable uncertainty over the results. However, both tranexamic acid, and the combination of tranexamic and desmopressin have high rates of haemostatic efficacy and have few adverse events.
PROTOCOL REGISTRATION
PROSPERO CRD42020169727.
Topics: Antifibrinolytic Agents; Blood Component Transfusion; Deamino Arginine Vasopressin; Disease Management; Female; Hemorrhage; Hemorrhagic Disorders; Hemostatics; Humans; Menorrhagia; Postoperative Hemorrhage; Postpartum Hemorrhage; Pregnancy; Tranexamic Acid
PubMed: 33973892
DOI: 10.1097/MBC.0000000000001045 -
Blood Reviews Sep 2021Currently, there is no consensus on the optimal management to prevent postpartum hemorrhage (PPH) in hemophilia carriers. We aimed to evaluate peripartum management...
Currently, there is no consensus on the optimal management to prevent postpartum hemorrhage (PPH) in hemophilia carriers. We aimed to evaluate peripartum management strategies in relation to maternal and neonatal bleeding outcomes by performing an extensive database search up to August 2020. Seventeen case-reports/series and 11 cohort studies were identified of overall 'poor' quality describing 502 deliveries. The PPH incidence in the individual patient data was 63%; 44% for those women receiving prophylaxis to correct coagulation and 77% for those without (OR 0.23, CI 0.09-0.58) and in cohort data 20.3% (26.8% (11/41) vs. 19.4% (55/284) (OR: 1.53, 95% CI: 0.72-3.24), respectively. Peripartum management strategies mostly consisted of clotting factor concentrates, rarely of desmopressin or plasma. Tranexamic acid appears promising in preventing secondary PPH, but was not used consistently. Neonatal bleeding was described in 6 affected male neonates, mostly after instrumental delivery or emergency CS, but insufficient information was provided to reliably investigate neonatal outcome in relation to management. The high PPH risk seems apparent, at most mildly attenuated by prophylactic treatment. Prospective cohort studies are needed to determine the optimal perinatal management in hemophilia.
Topics: Antifibrinolytic Agents; Blood Coagulation Factors; Delivery, Obstetric; Female; Hemophilia A; Hemorrhage; Humans; Infant, Newborn; Peripartum Period; Postpartum Hemorrhage; Pregnancy; Pregnancy Complications, Hematologic; Tranexamic Acid
PubMed: 33775466
DOI: 10.1016/j.blre.2021.100826 -
Journal of Clinical Neuroscience :... Apr 2021The purpose of this study was to perform a systematic review and meta-analysis on the effect of desmopressin on hematoma expansion (HE) in antiplatelet-associated... (Meta-Analysis)
Meta-Analysis
The purpose of this study was to perform a systematic review and meta-analysis on the effect of desmopressin on hematoma expansion (HE) in antiplatelet-associated intracerebral hemorrhage (AA-ICH). Secondary outcomes examined were the rate of thrombotic complications and neurologic outcome. Three databases were searched (Pubmed, Scopus, and Cochrane) for randomized clinical trials and controlled studies comparing desmopressin versus controls in adult patients with AA-ICH. The Mantel-Haenszel method was applied to calculate an overall effect estimate for each outcome by combining stratum-specific risk ratio (RR). Risk of bias was computed using the Newcastle-Ottawa Scale. The protocol was registered in PROSPERO (42020190234). Three retrospective controlled studies involving 263 patients were included in the meta-analysis. Compared to controls, desmopressin was associated with a non-significant reduction in HE (19.1% vs. 30%; RR:0.61; 95%CI, 0.27-1.39; P = 0.24), a similar rate of thrombotic events (5.5% vs. 9.9%; RR:0.47; 95%CI, 0.17-1.31; P = 0.15), and significantly worse neurologic outcome (mRS ≥ 4) (66.3% vs. 50%; RR:1.36; 95%CI, 1.08-1.7; P = 0.008). Qualitative analysis of included studies for each outcome revealed low to moderate risk of bias. The available literature does not support the routine use of desmopressin in the setting of AA-ICH. Until larger prospective trials are performed, the administration of desmopressin should be judiciously considered on a case-by-case basis.
Topics: Cerebral Hemorrhage; Deamino Arginine Vasopressin; Hematoma; Hemostatics; Humans; Platelet Aggregation Inhibitors; Prospective Studies; Randomized Controlled Trials as Topic; Retrospective Studies; Treatment Outcome
PubMed: 33775314
DOI: 10.1016/j.jocn.2021.01.017 -
International Journal of Gynaecology... Sep 2021Hermansky-Pudlak syndrome (HPS) is a rare autosomal-recessive disorder with clinical manifestations of bleeding diathesis, multi-organ disease and variable... (Review)
Review
BACKGROUND
Hermansky-Pudlak syndrome (HPS) is a rare autosomal-recessive disorder with clinical manifestations of bleeding diathesis, multi-organ disease and variable oculocutaneous albinism (OCA). In women, it can cause life-threatening obstetric and gynecological (OB/GYN) bleeding.
OBJECTIVE
To summarize OB/GYN presentations, outcomes, and management strategies in women with HPS.
SEARCH STRATEGY
Main databases (MEDLINE, EMBASE, Cochrane, PubMed, Web of Science Core Collection and Google Scholar) were searched from inception until June 30, 2020.
SELECTION CRITERIA
Case reports/series of women with confirmed HPS.
DATA COLLECTION AND ANALYSIS
A systematic review using PRISMA guidelines. Methodological quality assessment performed using adapted Newcastle Ottawa scale.
MAIN RESULTS
A total 29 pregnancies in 15 women and 2 gynecological patients were identified. Heavy menstrual bleeding (HMB), the most common bleeding symptom, was reported in 8/15 (53%) of women. HMB and post-partum hemorrhage (PPH) led to diagnosis of HPS in 5/17 (29%) women. Primary PPH was reported in 12/27 (44%) of viable pregnancies; half were major PPH. In 17 pregnancies with known HPS diagnosis, 9 had hemostatic cover with desmopressin and 8 with platelet transfusion. Major PPH occurred in 3/9 (33%) pregnancies covered with desmopressin compared with none in the platelet group.
CONCLUSION
Diagnosis of HPS should be considered in women with OCA presenting with HMB or PPH. Hemostatic management options include desmopressin and platelet transfusion. Management should be multidisciplinary with close collaboration between OB/GYN and hematology teams.
Topics: Female; Hemorrhage; Hemorrhagic Disorders; Hemostatics; Hermanski-Pudlak Syndrome; Humans; Obstetrics; Pregnancy
PubMed: 33521972
DOI: 10.1002/ijgo.13632 -
Clinical Neurology and Neurosurgery Jan 2021The transsphenoidal approach presents unique challenges in young, with scanty literature. This study compares the outcome of pituitary tumors among young in our center... (Comparative Study)
Comparative Study Meta-Analysis
PURPOSE
The transsphenoidal approach presents unique challenges in young, with scanty literature. This study compares the outcome of pituitary tumors among young in our center between endoscopic(EES) and microscopic(MTS) transsphenoidal surgery, with a meta-analysis.
METHODS
Patients within 20 years were studied for their surgical approach to a favorable outcome of endocrine remission (ER) (functioning) or Gross/Near-Total resection (nonfunctioning), besides the need for retreatment. Relevant studies were pooled and analyzed according to PRISMA guidelines.
RESULTS
Out of 64 young patients with pituitary tumors, 48 underwent transsphenoidal surgery using MTS(33) or EES(15). Of these, 21, 14, 5, and 8 had Cushing's, somatotropinomas, prolactinomas, and non-secreting tumors, respectively. Mean symptom duration was 28months, with weight gain(50 %) and visual complaints(29 %) most prevalent. Hypogonadism(21 %) was the most frequent endocrinopathy. The mean tumor volume was 3.8 cm. Over mean follow-up of 4.4years, favorable outcome was significantly higher after EES than MTS(78.6 % vs. 46.7 %)(odds ratio 4.18, p = 0.05). EES's better outcome was homogeneous across subgroups of age and tumor type, with no significant subgroup difference. Symptom duration was significantly higher among those who required retreatment(p = 0.05), while ER had a non-significant association with tumor volume(p = 0.07). Overall, 40 %, 27 %, 17 %, and 8% were on hydrocortisone, thyroxine, sex hormone, and desmopressin, respectively, at follow-up with no significant difference between EES and MTS. In pooled analysis of literature, both favorable outcome(74 % vs. 48 %,p = 0.02) and retreatment rate(8% vs. 37 %,p = 0.004) were significantly better with EES than MTS.
CONCLUSION
Among young patients with pituitary tumors, the favorable outcome and retreatment rates are better with endonasal endoscopy and associated with symptom duration and tumor volume.
Topics: Adenoma; Humans; Microsurgery; Nasal Cavity; Neuroendoscopy; Pituitary Neoplasms; Sphenoid Bone; Tumor Burden
PubMed: 33338824
DOI: 10.1016/j.clineuro.2020.106411