Deterioration Of General Condition Research Articles

An attempt to deconstruct the French "syndrome de glissement"

The "syndrome de glissement" is a French term used to describe end-of-life situations where the elderly person becomes adynamic and any therapeutic intervention seems ineffective until the patient dies. It is close to failure to thrive. Although its use, which is widespread, seems appropriate to briefly describe a complex situation, it has the major disadvantage of leading to an absence of clinical and therapeutic approach, which represents a loss of chance for the patient. In clinical practice, we consider that what is referred to as a sliding syndrome is actually a situation of apathy associated with a deterioration in general condition. A diagnostic approach should therefore look for all organic pathologies that associate deterioration in general condition and apathy, and neuropsychiatric situations such as episodes of major depression that produce such symptoms. Once these diagnoses have been ruled out, the person should be considered for palliative care and benefit from the expertise that goes with this practice. Any elderly person in a situation resembling what is known as a syndrome de glissement should therefore benefit from a rigorous clinical approach, and not be considered beyond any therapeutic resources. It is a matter of dignity and quality of care.

Leadless pacemaker implantation versus conventional transvenous cardiac pacing early after transcatheter aortic valve replacement: a safe and relevant option?

Abstract Background Leadless pacemakers (LPM) are known to have lower postoperative complications than conventional transvenous cardiac pacing especially in frail patients (pts) with many comorbidities. The aim of this study was to assess the "real-life" feasibility and outcomes of LPMs compared to transvenous pacemakers (TPMs) in patients who underwent transcatheter aortic valve replacement (TAVR). Methods This is a retrospective single center study including all consecutive patients who received LPM or TPM (either single or double chamber) implantation early after a TAVR over a one-year period since September 2022 with a follow up period of at least 3 months. Results 71 pts were included (mean age 82,56 ± 7,1 years old). 16 pts underwent LPM implantation (22,5%). Leadless system pacing was preferred in pts with history of active systemic infection 43,8%, complex conventional vascular approach 12,5 %, deterioration of general condition and clinical frailty 18,8% and history of breast cancer in 25 % pts. Mean time between appearance of conductive disorders and pacemaker implantation was longer (2,9± 2,8 vs 1,5 ± 1,3 days; p=0,06) in the LPM group probably due to organizational issues. Procedure duration was significantly shorter in the LPM group (46 ±20,5 vs 69 ± 21,1 min; p = 0,01). There was no statistically significant difference in fluoroscopy time between the 2 groups even though this parameter was longer in the LPM group (6,8 min vs 5,6 min; p = 0,413). Significantly lower thresholds were observed in pts with LPM implantation (0,45 ± 0,33 vs 0,66 ± 0,25 V; p = 0,017) with no statistically significant difference between the 2 groups concerning the per-operative impedance value. Median time from pacemaker implantation to discharge was 3,12 ± 3,26 vs 4,78 ±7,16 days after LPM and TPM implantation respectively (p = 0,195). There were less complications in the LPM group (1,4 % vs 9,9 %; p = 0,471). Complications were: arteriovenous fistula for 1 pt with LPM vs 2 pocket hematomas, 1 pneumothorax, 1 per-operative cardiac arrest and 3 leads dislodgements in pts with TPM. The mortality rate was significantly higher in the LPM group (4,2 % vs 2,8 %; p = 0,038) although no pacemaker related deaths were reported in both groups and no significant difference between the 2 groups was noted concerning rehospitalisations for cardiovascular cause (p= 0,407). Conclusion Early LPM implantation after TAVR is a feasible alternative to conventional pacing with significantly shorter procedure durations and better pacing thresholds. Our experience confirms the global clinical frailty of patients receiving LPM after TAVR but underlines the favorable safety profile of leadless system pacing with a low rate of complications.

#2473 Renal failure in the emergency department: indications for emergency hemodialysis

Abstract Background and Aims kidney failure is a frequent reason for emergency department (ED) visits. Patients may require emergency hemodialysis and this situation may increases mortality risk. We set out to study the clinical relevance and biological characteristics of patients with renal failure presenting to the emergency department with an indication for emergency hemodialysis. Method A prospective observational study conducted in ED conducted over a 6-month period. Patients were included if they were 18 years or older and suffering from acute or chronic renal insufficiency defined by creatinine clearance (CrCL) calculated by MDRD<60 ml/min. Information is recorded on demographics, clinical profile, clinical presentation, laboratory, causes of for emergency hemodialysis and in-hospital complications. Results Eighty one patients were enrolled, among them 61.7% patients had an indication for emergency hemodialysis. Mean age was 61± 12, sex-ratio=1,7. Sixty seven patients (82.7%) were known to have a chronic kidney failure and 17.3 % were discovered at the emergency department at the same hospitalisation period. Clinical profile (%): Anuric patients (11.1), preserved diuresis (40.7). Clinical presentation (%): deterioration of general condition (35.8), dyspnea and chest pain (21) and fever (17.3). Clinical examination showed a peripheral oxygen saturation between 70 and 100% with a mean of 94.81, a respiratory rhythm mean of 20 cycles/min, a systolic blood pressure mean of 134.61 mmHg and a diastolic blood pressure mean of 70 mmHg. biological exams showed that kalemia was between 2.4 and 8.6 and 45 of those patients had kalemia more or equal to 5.5. Twelve per cent of these patients had hyperkalemia signs on the electrocardiogram. The average were 28 mmol/l with a maximum of 83.3 mmol/l. Creatinine values were between 185 et 2620 μmol/l with a median of 81. The value of Ph was between 7.04 and 7.53 with a median of 7.29 mm Hg. The value of HCO3- value was between 4.6 and 28.4 with a median of 14.2. Causes of emergency hemodialysis were acidosis in 12 cases, severe anemia with blood transfusion in 4 cases, hyperkalemia in 10 cases, acute pulmonary oedema in 5 cases and acute renal failure in 6 cases .the evolution of the patients was marked by improvement of 61.7% of the patients, only 3 of the 81 patients died during the hospitalization. Conclusion This study showed that the causes of emergency dialysis can be avoided by improving care and educating patients to improve the prognosis.

Autoimmune myelofibrosis revealing a systemic lupus erythematosus

Background: Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease, characterised by multi-organ affections. Haematological involvement is a common manifestation of SLE, consisting of autoimmune peripheral cytopenia. Autoimmune myelofibrosis (AIMF) is a rare cause of cytopenia in SLE; it could precede or be concurrent with the diagnosis of SLE. There are few studies that describe this association. Case description: We report a case of AIMF revealing the diagnosis of SLE in a 34-year-old female, presented with episodes of gingival bleeding associated with peripheral inflammatory polyarthralgia, photosensitivity and deterioration of general condition. Clinical examination revealed a soft pitting oedema in the lower limbs. Laboratory investigations showed a pancytopenia, inflammatory biological syndrome, with positive 24-hour proteinuria and anti-native DNA antibodies. A bone marrow biopsy showed diffuse myelofibrosis associated with maturation disorders and no tumour infiltrate. Renal biopsy revealed proliferative glomerulonephritis class III with immune deposits. Conclusion: The association of AIMF with SLE has been rarely reported, and it could be another cause for cytopenia in SLE.

Léiomyosarcome portal : une localisation extrêmement rare !

Leiomyosarcomas of large vessels are rare. It is a malignant tumour and the vast majority of these tumours arose from the inferior vena cava. We report a rare case of portal vein leiomyosarcoma, in a 56-years-old female patient admitted for chronic abdominal pain with abdominal mass in the right hypochondrium all evolving in a context of deterioration in general condition. We performed an abdominopelvic CT scan and then a MRI with contrast agent which objectified a large tissue mass containing areas of necrosis at the level of the duodeno-pancreatic compartment communicating at a large angle with the portal trunk over its entire length from the hepatic hilum to the spleno-mesenteric confluence responsible for a portal cavernoma downstream. This is associated with multiple secondary nodular tissue hepatic lesions. We also noted a respect for the fatty border separating the mass of the duodenal tract and the head of the pancreas, and also the absence of dilation of the pancreatic ducts making a pancreatic origin unlikely. To eliminate a duodenal origin of the mass we performed an upper digestive endoscopy which came back without any abnormality. An ultrasound-guided trans parietal biopsy of a secondary hepatic lesion was done and the pathological result of which speaks of a secondary hepatic lesion of a leiomyosarcoma.

Pleomorphic Carcinoma of Pulmonary Location: Case Report and Review of the Literature

Pulmonary pleomorphic carcinoma is a rare primary lung tumor, part of the group of non-small cell cancers. We report the case of a 54-year-old patient, a chronic smoker who presented with hemoptysis evolving in a context of deterioration in general condition. Chest imaging revealeda left upper lobar tissue density mass with some hypodensity. The diagnosis of pleomorphic lung carcinoma was made based on histological data from bronchial biopsies, and classified as stage IVb according to the TNM classification (8th edition). Palliative chemotherapy was then offered to the patient. The evolution was marked by the death of the patient 2 months after diagnosis. Through this observation, we wish to shed light on this rare and particular histological type but just as aggressive as other pulmonary neoplasias.

A rare case of bleeding in diverticular disease of the small intestine

The article describes a clinical case of bleeding from diverticula of the small intestine in patient E. 1957, who entered the State Budgetary Institution RO OKB on 10/03/2023. with clinical picture gastrointestinal bleeding of unspecified etiology. In the result of a diagnostic study, collection of anamnestic data and clinical picture, a diagnosis was made: gastrointestinal intestinal bleeding of unspecified origin. After FGDS and no visible sources of gastric bleeding, and lack of positive effect from conservative therapy, increasing hypotension, deterioration of general condition with hemoglobin 82 g/l. a decision was made to perform a blood transfusion and combined hemostatic therapy. Conservative treatment turned out to be ineffective, and therefore it was accepted decision on emergency surgical treatment. Urgent patient a diagnostic laparotomy was performed. During revision of the abdominal organs cavity, the source of bleeding was identified. A section of the small intestine was resected at a distance of 15 cm from the ligament of Treitz over a distance of 70 cm with multiple diverticula. An interintestinal side to side anastomosis. In the postoperative period, data for relapse. There is no gastrointestinal bleeding. On day 9 the patient was discharged under the supervision of a surgeon at the place of residence in satisfactory condition.

Diffuse Lymphocytic Infiltration Syndrome (DILS) as a Mode of Revelation of HIV: On a Case

Diffuse Lymphocyte Infiltration Syndrome (DILS) is a rare condition observed in HIV patients, characterized by infiltration of CD8+ T lymphocytes in several organs. In this article, a case of revelation of HIV infection is presented, diagnosed following bilateral parotidomegaly associated with DILS. The patient, aged 60, presented a progressive deterioration in general condition, significant asthenia, weight loss, and local symptoms including bilateral parotid swelling, cervical lymphadenopathy, and intermittent fever. Investigations revealed HIV-1 seropositivity, lymphocytosis with high T-CD8+ levels, salivary gland enlargement on imaging, and benign lymphoepithelial lesions on parotid biopsy. The diagnostic criteria for DILS were met, highlighting the importance of salivary gland involvement in this syndrome. Treatment with HAART resulted in significant regression of symptoms. In summary, DILS represents a multisystem complication of HIV, requiring appropriate management including antiretroviral treatment to improve patient prognosis.

Tuberculosis or Sarcoidosis or the Both? A Case Report of Concomitant Tuberculosis and Sarcoidosis

Tuberculosis and sarcoidosis are two chronic granulomatous diseases that are similar in many aspects, although different. We report a case of 38-year-old female patient who presented a progressive shortness of breath and recurrent epistaxis associated to a night sweats and fatigue with a deterioration of general condition, in which a CT-scan showed a multiple voluminous lymphadenopathy involving all the mediastinum territories, without parenchymal abnormalities. During investigations bronchoscopy showed mucosal hypertrophy and granulations located on the nasopharyngeal walls. The biopsy of the lip and nasal’s lesion showed a non-caseating granuloma and ACE (angiotensin converting enzyme) was high, which considered sufficient to retain the diagnosis of sarcoidosis, but the blood Quantiferon-TB Gold in Tube test was positive and the excision biopsy of cervical lymphadenopathy, revealed a necrotizing granulomatous inflammation suggestive of tuberculosis. The association between sarcoidosis and tuberculosis was discussed. the patient was treated with antituberculosis drugs during six months. The evolution was marked by a progressive regression of lymph nodes. There was no significant improvement of rhinological symptoms with local corticoids. Consequently, the corticosertoide treatement in form oral was introduced during 1 month. The prednisolone was gradually tapered off after 1 month due to the complete disappearance of lesions. The case described is suggestive of a coexistent systemic sarcoid manifestation and tuberculosis, which is an underrecognized entity in the medical literature.

Management of Head and Neck Cellulitis in a Patient Immunocompetent

Head and neck cellulitis are severe infections, sometimes necrotizing, whose diagnosis is difficult at an early stage. We report the clinical observation of a 60-year-old patient who presented to the emergency room for a right laterocervical swelling associated with a deterioration in general condition. The head and neck CT scan revealed abscessed right cervical cellulitis. The patient underwent drainage and debridement in the operating room with wide necrosectomy and placement of a Delbet blade, associated with dual antibiotic therapy. The evolution was marked by an improvement in the patient's clinical condition. The aim of this work was to highlight the importance of emergency medical-surgical care, without forgetting the treatment of the portal of entry. The progression in the absence of treatment is towards sometimes fatal complications. The prognosis of these infections depends on the precocity of the treatment, its initial quality and the terrain.

The Prevalence of Depression and Anxiety Symptoms and Their Association With Respiratory Diseases.

Patients with respiratory diseases face adverse situations such as symptom management, general condition deterioration, and a hostile perception of the hospital environment, favoring the appearance of anxiety and depression. A total of 317 patients hospitalized for a disease of pulmonary origin were analyzed and divided into the following subgroups: infectious, oncological, acute, and chronic diseases. Patients over 18 years of agewith preserved cognitive capacity were included in the study. The Hospital Anxiety and Depression Scale (HADS) was applied to them on the second or fourth day of their hospital stay and five days after the first evaluation. Multiple linear regression models were carried out to analyze the association between anxiety and depression measured over two different periods. The models present the statistically significant variables with a 95% confidence level. The patients presented with anxiety in 74.4% of cases, mainly those with acute respiratory diseases (42.4%) and neoplastic diseases (27.5%). A total of 69.5% presented with depression, with symptoms more significant in those with chronic and oncological pulmonary diseases and those with no job. Patients with at least one comorbidity presented with anxiety in 53.9%of cases and depression in 52.1%of cases. Linear regression models were carried out and showed that anxiety was 1.75 and 1.84 times more frequent in patients with chronic diseases compared to those with infectious pathologies in the first and second reviews, respectively. The linear regression model also showed a higher frequency of depressive symptoms in patients with chronic conditions (1.62 times) compared to the group with infectious and contagious pathologies, and prolonged hospital stays were associated with depressive symptoms 1.37 times more than short stays. Anxiety and depression are frequent disorders in patients with respiratory diseases, negatively affecting the prognosis. Routine mental health screening and multidisciplinary management are essential in this population.

Mortality in the Pediatric Emergency Department of the Mother and Child Academic Hospital in N'djamena

Background: Child mortality is a significant concern for healthcare systems in developing countries. Objective: To contribute to reducing mortality in the Paediatric Emergency Department of the Mother and Child Academic Hospital in N’Djamena. Methods: A cross-sectional analytical study was conducted between June 2019 and May 2020. Children aged one month to fifteen years hospitalised were the study population. Those who died were included. The variables studied were age, sex, and parents’ socio-economic and educational level, time to consultation and access to care, length of hospitalisation, symptoms on admission, history, diagnosis, treatment and outcome. The statistical test used was the KHI2 Results: Of 13972 hospitalised children aged between 1 month and 15 years, 362 (3.44%) died. The children were 56.4% boys (n=204) and 43.6% girls (n=158), with a sex ratio 1.29 and an average age of 24 months. Five-year-olds accounted for 71% (n=257). They were from urban areas, 83.7% (n=303) and of low socio-economic status 57.6% (n=171). Deaths occurred during on-call hours (night shift) 63.8% (n= 231) and within 24 hours 66.5% (n= 241). Malaria 37.1% (n= 43), acute respiratory infections 25% (n= 29) and severe acute malnutrition 12.9% (n= 15) were the causes of death. Impaired general condition, respiratory distress, grade II coma, severe anaemia and lack of blood transfusion were associated with death. Conclusion: Mortality is still high, often due to preventable diseases, and we need to step up communication to change behaviour. Primary prevention involves improving people's living conditions.

Real-World Outcomes of Newly Diagnosed Multiple Myeloma (NDMM) Patients Treated before the Anti-CD38 Antibodies Era in France, the Emmy Cohort 2017 to 2020

Background In randomized clinical trials (RCTs), patients (pts) with comorbidities or a very aggressive presentation of myeloma may be excluded. The results of RCTs may not be generalizable to pts in the real-world setting. Therefore, RCT data need to be backed up by results from real-world studies. Methods EMMY is a non-interventional, prospective study conducted in 73 IFM (Intergroupe Francophone du Myélome) sponsor sites in France. Any patient initiating treatment for multiple myeloma (MM) over a 3-month (mo) period, from October to December, is included, since 2017. It is a dynamic cohort with 900 pts on average, enrolled each year. Here, we present the results of NDMM pts for the first 4 years of inclusion (2017 to 2020). Pts with NDMM who receive front-line therapy with an autologous stem cell transplant (ASCT group) are described separately from non-transplant eligible pts (NTE group). Results 1597 pts with NDMM were included in EMMY over the 2017-2020 period. Of these, 1036 were in the NTE group (64.9%) and 561 in the ASCT group (35.1%). Data are presented with a median follow-up of 23.7 mo (IQR 12.3 - 39.0). ASCT group These pts had a median age of 60.6 years (range 23.4 - 78.5). 5.2 % were older than 70 years at ASCT. In routine practice, cytogenetic tests were carried out for 75.7% of pts, 19.6% of them harboured high-risk (HR) anomalies of t(4;14) or del(17p). The period 2017-2020 was marked by a shift in induction treatment from Velcade Thalidomide dexamethasone (VTd) (n = 163, 29.1% of pts who received ASCT) to Velcade Revlimid dexamethasone (VRd) (n = 309, 55.1%). Fifty-three (9.5%) pts received a double transplantation which was stable over time. 22.8 % of pts with HR anomalies received a double transplant. NTE group The 1036 NTE pts had a median age of 74.9 years (range 33.8 - 97.8), and more than 30.4% (n = 315) were aged ≥ 80 years. Among these pts, 65.9% were considered frail, with 33.6% having an Eastern Cooperative Oncology Group-Performance Status (ECOG-PS) ≥ 2 and almost half (47.1%) had comorbidities. Significant renal impairment (glomerular filtration rate [GFR] < 30 mL/min) was observed in 118 (13%) pts at treatment initiation. A subgroup of 126 younger pts (age < 65 years) was considered ineligible for transplantation. Regarding cytogenetics 56.4% of the pts were tested in regular practice (mean age 73.3 years). NTE pts mostly received three main combinations: VRd (28.4% of pts, n = 294), Velcade Melphalan prednisone (VMp) (21.9% of pts, n = 21.9%) and Revlimid dexamethansone (Rd) (21.1%, n = 219). During the study period, VMp use decreased from 36.9% to 6.5% of pts, VRd use increased from 10.6% to 48.5% and Rd use remained constant, at around 20%. Other bortezomib-based regimens, including Velcade dexamethasone (Vd) and Velcade Cyclophosphamide dexamethasone (VCd), were used steadily, in approximately 12% and 7% of pts, respectively, over the period. Survival data Median progression-free survival (mPFS) was 25.8 mo (95% CI: 23.5-29.1) in the overall NDMM population, 46.5 mo (95% CI: 37.8-50.6) in pts who received ASCT and 18.7 mo (95% CI: 16.3-20.8) in NTE pts ( P < 0.0001). (Figure 1). The median PFS2 (mPFS2) was estimated at 50.6 mo (95% CI: 47.6-NA) in all pts with NDMM. mPFS2 was 36.4 mo (95% CI: 34.7-39.9) in NTE pts; it was not reached in the ASCT group. The median overall survival (OS) had not yet been reached. The estimated OS rate was 72.9% (95% CI: 69.8-75.9) at 48 mo for the total NDMM population. (Figure 2). AE leading to discontinuation. 139 pts experienced an adverse event (AE) leading to treatment discontinuation (6.8 % of ASCT pts and 14.5% in the NTE group). This occurred at a median of 4.3 mo after treatment initiation and was mainly reported with the most used agents (lenalidomide, bortezomib or melphalan). The most frequently reported AEs were neuropathy (n = 14; 10.1%), digestive disorders (n = 13; 9.4%), rash (n = 12; 8.6%), deterioration of general condition (n = 12; 8.6%), cardiovascular AEs (n = 10; 7.2%) and acute renal failure (n = 10; 7.2%). Conclusion The 2017-2020 period for pts with NDMM in France was marked by the expansion of the use of lenalidomide in first line for the majority of pts. A major difference in PFS and OS is still observed between ASCT and NTE pts. A shorter PFS than in IFM2009 trial (PFS 50 mo) was observed in EMMY ASCT pts.

Prognostic factors and survival in adult acute leukemia in Burkina Faso

Acute leukemia is both a diagnostic and therapeutic emergency. Our study aimed to describe the prognostic factors and survival of adults with acute leukemia in Burkina Faso. Cross-sectional descriptive study with retrospective data collection covering a period of 4.5 years (2018-2022) in two university hospitals in Burkina Faso. Were included all patients over 18 years hospitalized for acute leukemia in these sites with a usable medical record. A total of 42 cases were collected, of which 45% suffered from acute lymphoblastic leukemia and 43% from acute myeloid leukemia. In 12% of cases, acute leukemia was not classified. The average age was 35 ± 15 years, with extremes of 19 and 72 years. 12% of the patients presented an age of poor prognosis. Comorbidities were present in 14% of patients. The deterioration in general condition was fairly constant with 95% of patients at WHO stages 3 and 4. All patients presented with bone marrow failure syndrome and tumor syndrome was found in 45%. Anemia and thrombocytopenia were present in almost all cases. Hyperleukocytosis at diagnosis was present in 28 patients (67%); among them 18 patients (64%) had leukocytes greater than 50 G/L. Death in hospital was found in 38% of patients and loss of sight in 31%. The median survival was 3 months. Survival was 30% at 6 months and 0% at 12 months. Acute leukemias are in our practice conditions of poor prognosis with a fairly short survival.

Randomised Controlled Trial on Prophylaxis of Mastitis-Metritis-Agalactia Syndrome in Swine using Caulophyllum Logoplex and Lachesis Logoplex.

Mastitis-metritis-agalactia (MMA) syndrome occurs in the first days post-partum and causes piglet losses mainly due to malnutrition. One possibility for prophylaxis of MMA is via homeopathy. In this veterinary study, the effectiveness of a prophylactic administration of homeopathic remedies for the prevention of the occurrence of MMA in swine was evaluated. In a randomised and blinded study, 60 sows were examined. Sows were randomly distributed in two groups: the experimental group (CL/LL) received a prophylactic administration of the complex homeopathic remedies Caulophyllum Logoplex and Lachesis Logoplex, and the placebo group was administered a sodium chloride (NaCl) solution in the same injection scheme as the experimental group. Clinical signs of MMA, behavioural changes, as well as production parameters, were recorded beginning with the day of farrowing until 5 days post-partum. The treatment group showed no significant effect on the occurrence of MMA in sows (CL/LL: 56.67% MMA positive sows; NaCl: 53.53% MMA positive sows). Treatment group had also no significant effect on health parameters (vaginal discharge, raised rectal temperature, shortage of milk) or behavioural parameters (impaired feeding behaviour and impaired general condition). For the production parameter average weight gain, statistically significant effects in the treatment group were detected. Prophylaxis with the homeopathic remedies Caulophyllum Logoplex and Lachesis Logoplex showed neither an improvement in MMA prevention nor an improvement in health parameters or behavioural traits in the present herd of sows.

Duodenal Adenocarcinoma: 4 Cases Report

Duodenal adenocarcinoma being the most common tumor of the small intestine: 33 to 48%, it remains very rare representing only 0.5% of malignant tumors of the digestive tract. Our retrospective study focused on four cases during the period 2017-2022 with an average age of 53.25 years and a sex ratio of 1M/3F. The clinical signs were varied with a predominance of cholestatic jaundice and a deterioration in general condition. The diagnosis was confirmed thanks to the endoscopy and the anatomopathological study of the biopsies carried out in all our patients. All our patients benefited from a surgical treatment, curative or palliative, which consisted of three cephalic pancreaticoduodenectomy and in one case a gastro-jejunal anastomosis. The post-operative follow-up was simple in two patients, one of our patients presented with gastroparesis which progressed well under medical treatment. In addition, we deplore a death which presented a hemoperitoneum related to a hemostasis disorder.

Achalasia of the Esophagus at Donka National Hospital: A Case Report

Background: Esophageal achalasia is an esophageal motility disorder of unknown etiology that results in impaired lower esophageal sphincter relaxation and loss of esophageal peristalsis. The aim of our study was to show our experience in the management of esophageal achalasia in a case treated by laparotomy in our service. Case Report: 21-year-old patient, student, admitted to our department for progressive dysphagia to solids accompanied by regurgitation, epigastric pain, nocturnal cough and weight loss, evolving for 5 years. ATCD of pulmonary tuberculosis. The physical examination noted a deterioration in general condition, stage (I) of the WHO; there was hypo coloration of the integuments and conjunctivae, TA=120/80mmHg, Pulse=70/min. The abdomen was symmetrical with presence of sluggish skin folds, supple and painless without a palpable mass, the digital rectal examination did not present any particularity. The thorax was symmetrical with a FR=20 c/mn. The biology showed a normo chrome and normocytic anemia of 8 g/l Group O+. The esogastroduodenal transit was in favor of an amputation (tight stenosis) extended on the cardia with megaesophagus upstream. We performed a cardiomyotomy according to Heller associated with a hemivalve according to Toupet. We performed a followed by preesophageal and subhepatic drainage. The postoperative course was simple, marked by a resumption of food on D6 postoperatively. The control test carried out with gastrographine 10 days after the surgical intervention noted a regression of the signs with passage of the product of contrast in the small intestine. The patient seen again 6 months after the operation showed no particularities. Conclusion: Achalasia is a chronic esophageal motility disorder characterized by the inability of the lower esophageal sphincter to relax. Heller cardiomyotomy by laparotomy associated with Toupet-type fundoplication retains its place in its management in our exercise context.

Deprescribing in primary care without deterioration of health-related outcomes: A real-life, quality improvement project.

Medication reviews focusing on deprescribing can reduce potentially inappropriate medication; however, evidence regarding effects on health-related outcomes is sparse. In a real-life quality improvement project using a newly developed chronic care model, we investigated how a general practitioner-led medication review intervention focusing on deprescribing affected health-related outcomes. We performed a before-after intervention study including care home residents and community-dwelling patients affiliated with a large Danish general practice. The primary outcomes were changes in self-reported health status, general condition and functional level from baseline to 3-4 months follow-up. Of the 105 included patients, 87 completed the follow-up. From baseline to follow-up, 255 medication changes were made, of which 83% were deprescribing. Mean self-reported health status increased (0.55 [95% CI: 0.22 to 0.87]); the proportion with general condition rated as 'average or above' was stable (0.06 [95% CI: -0.02 to 0.14]); and the proportion with functional level 'without any disability' was stable (-0.05 [95% CI: -0.09 to 0.001]). In conclusion, this general practitioner-led medication review intervention was associated with deprescribing and increased self-reported health status without the deterioration of general condition or functional level in real-life primary care patients. The results should be interpreted carefully given the small sample size and lack of control group.

#5816 RAVULIZUMAB “DE NOVO” IN PEDIATRIC PATIENTS WITH ATYPICAL HEMOLITIC UREMIC SYNDROME: FIRST WORLDWIDE CASES

Abstract Background and Aims Ravulizumab is a long-acting C5 inhibitor that has recently demonstrated its effectiveness in adult and pediatric patients for the control of hemolytic uremic syndrome compared to eculizumab, allowing average annual infusion times of up to 70% less. There is still little evidence in the literature of naïve treatment with this drug in pediatrics. Method We present the first two pediatric cases worldwide with the use of de novo Ravulizumab: the first one in the onset of the disease and the second one, post-kidney transplant Results 13-year-old girl referred from another country with 1 week history of acute gastroenteritis with bloody stools, vomiting and compromise of consciousness. Deterioration of general condition, with laboratory tests compatible with thrombotic microangiopathy (TMA), evolves to anuria and convulsive episode requiring invasive mechanical ventilation, corticosteroid boluses, 6 plasmapheresis sessions and 4 intermittent hemodialysis. One Eculizumab dose (600 mg) was administered in the center of origin and STEC was isolated. At admission presented compromised renal function (AKI III), hemolytic anemia, thrombocytopenia, normal ADAMTS-13, negative direct Coombs and decreased complement. Brain MRI study shows images compatible with multiple foci of necrosis. Due to the persistent requirement of renal replacement therapy and persistent TMA, Ravulizumab was started with a loading dose (2400 mg) and a second dose after 2 weeks. The need for renal replacement therapy ceased with improvement of hemolysis and renal function. Genetic study showed mutation of uncertain significance in heterozygosis in exon 6 (c.881_883, p.Ala292del) with risk polymorphism for HUSa (deletion CFHR3-CFHR1) Case 2: 7-year-old girl (stage 5 CKD) in chronic hemodialysis 3 times/week secondary to aHUS (CD46 mutation) was admitted for kidney transplant from a living donor (father), performing hemodialysis session before surgical intervention. Low-intermediate immunological risk (PRA 0%, 9 HLA matches) and high CMV infectious risk (valganciclovir prophylaxis). Induction treatment: Basiliximab, tacrolimus, mycophenolate and steroids. First dose of Ravulizumab was infused the day before transplantation (900mg), well tolerated. 36 hours after presented acute pulmonary edema as well as a drop in hemoglobin (4g/dL). With normal laboratory hemolysis parameters, urgent abdominal-pelvic CT was performed due to suspicion of bleeding, confirming an active bleeding. Surgical reintervention was decided due to ureteral bleeding and the tunnel was redone with good results. The patient has had a favorable evolution of renal function, with a normal value of creatinine at discharge (0.43 mg/dl). Protein/Creatinine urine ratio (iPr/Cr) increases to a maximum of 8 mg/mg. Negative DSA levels but the option of renal biopsy was assessed and ruled out due to a decrease in proteinuria (iPr/Cr 0.51 mg/mg). She received second dose of ravulizumab after 2 weeks, remain stable with no data on recurrence of her underlying disease today. Conclusión: In the two patients, the initial treatment with Ravulizumab was satisfactory, both in the acute phase of the disease and in the immediate post-transplantation. In the first case we observed a functional recovery from the first dose with no notable adverse effects up today, as in the post-transplant patient, maintaining a good control of TMA despite more spaced dosing (8 weeks). The inclusion of this drug in the therapeutic arsenal opens a new safe treatment route in pediatric patients with HUSa

Epidemiological Aspects of Cirrhosis at the Sominé Dolo Hospital in Mopti

Introduction: Due to its frequency and its complications, it constitutes a major public health problem in the world, particularly in Africa. According to the WHO, 350 million people suffer from chronic liver disease in the world, and Africa has 60 million with a quarter of deaths per year. The objective of our study was to describe the epidemiological aspects of cirrhosis at the Sominé DOLO Regional Hospital in Mopti, the first in the region. Methods and Patients: This is a descriptive cross-sectional study extending from January 1, 2021 to December 2021, carried out in the medical department of the Sominé Dolo hospital in Mopti. The diagnosis of cirrhosis was retained in the face of arguments: clinical: oedemato-ascites syndrome, portal hypertension syndrome, hepatocellular insufficiency syndrome; biologicals of hepatocellular insufficiency syndrome; ultrasound suggestive of cirrhosis (hepatic atrophy, hepatomegaly, irregular contours, heterogeneous echostructure, portal trunk greater than 15 mm in diameter, maximum portal blood flow velocity less than 15 cm/s , superior splenic vein 10 mm, splenomegaly and ascites); endoscopic (esophageal varices, cardio-tuberosity varices, antral vascular ectasia, portal hypertension gastropathy. Despite these clinical, biological and imaging arguments, the diagnosis of certainty remains the liver biopsy puncture. Result: The analysis concerned 46 patients out of the 865 hospitalized patients, ie a frequency of 5.31%. The average age was 47.5 with extremes of 21 and 70 years. The most represented age group was 46 – 65 years old. The patients were divided into 34 men (73.9%) and 12 women (26.1%) i.e. a sex ratio of 2.8. Farmers/herders and housewives accounted for 76.1% (35/46) of the patients and the majority were rural (89.1%). The clinical signs found on hospitalization were: abdominal pain (82.6%), impaired general condition syndrome (80.4%), ascites (76.1%), OMI (58.7%), Hepatomegaly (52.2%), CVC (43.8%), jaundice (32.6), ...