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2.
Vet Q ; 44(1): 1-8, 2024 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-38823415

RESUMEN

Dogs that had splenectomy are predisposed to fatal thrombotic conditions, and thrombocytosis is a risk factor for post-splenectomy hypercoagulability. However, in veterinary medicine, there are no specific therapeutic approaches for managing this hypercoagulability. This study aimed to determine the preventive effect of clopidogrel on post-operative hypercoagulability during the first 2 weeks post-splenectomy in dogs with splenic masses. This study included 12 dogs that had splenectomy. Seven dogs received no treatment (group A), and five were treated with clopidogrel (group B). Clopidogrel was loaded at 10 mg/kg on day 2 and continued at 2 mg/kg until day 14. Blood samples were collected on the day of surgery and 2, 7, and 14 days after splenectomy in both groups. In group B, thromboelastography (TEG) was performed on the same days. In group A, there was significant elevation of platelet counts on days 7 (p = 0.007) and 14 (p = 0.001) compared to day 0. In group B, the platelet counts were significantly elevated on day 7 (p = 0.032) but no significant difference was found on day 14 compared to day 0. Platelet counts on day 14 were significantly higher in group A than in group B (p = 0.03). The lower platelet counts were correlated with alterations in TEG parameters, and no significant differences were found in the K and α-angle values at all postoperative assessment points compared to day 0. Our study suggests that clopidogrel may reduce post-operative thrombocytosis and hypercoagulability in dogs that undergo splenectomy for splenic masses.


Asunto(s)
Clopidogrel , Enfermedades de los Perros , Inhibidores de Agregación Plaquetaria , Esplenectomía , Tromboelastografía , Trombofilia , Animales , Perros , Esplenectomía/veterinaria , Esplenectomía/efectos adversos , Clopidogrel/uso terapéutico , Enfermedades de los Perros/sangre , Enfermedades de los Perros/cirugía , Enfermedades de los Perros/tratamiento farmacológico , Recuento de Plaquetas/veterinaria , Femenino , Masculino , Trombofilia/veterinaria , Trombofilia/tratamiento farmacológico , Inhibidores de Agregación Plaquetaria/uso terapéutico , Inhibidores de Agregación Plaquetaria/farmacología , Tromboelastografía/veterinaria , Complicaciones Posoperatorias/veterinaria , Complicaciones Posoperatorias/prevención & control , Neoplasias del Bazo/veterinaria , Neoplasias del Bazo/cirugía , Neoplasias del Bazo/sangre , Enfermedades del Bazo/veterinaria , Enfermedades del Bazo/cirugía , Enfermedades del Bazo/sangre , Trombocitosis/veterinaria
3.
Asian J Endosc Surg ; 17(3): e13335, 2024 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-38840499

RESUMEN

Laparoscopic fenestration is the preferred treatment for symptomatic splenic cysts because it is curative and spleen-sparing. We report a case of a 25-year-old female who underwent laparoscopic fenestration for a giant splenic cyst using a single-incision plus one-port approach. She presented to our hospital with repeated vomiting. Imaging showed a 23 × 18 cm splenic cyst with no solid components, strongly compressing the stomach to the right side. Laparoscopic fenestration was initiated through a 2.5-cm umbilical incision, and a 5-mm port was added in the left abdomen intraoperatively. The drain placement was deemed necessary to prevent abscess formation and post-operative bleeding because of a thick cyst component and unexpectedly thick wall. Splenic cysts are typically benign and commonly develop in young people, hence, organ preservation and cosmetic results are crucial. Laparoscopic fenestration using single-incision plus one-port is considered to be an appropriate procedure for giant splenic cysts with non-serous contents.


Asunto(s)
Quistes , Laparoscopía , Enfermedades del Bazo , Humanos , Femenino , Laparoscopía/métodos , Quistes/cirugía , Adulto , Enfermedades del Bazo/cirugía , Enfermedades del Bazo/diagnóstico por imagen
4.
Med Trop Sante Int ; 4(1)2024 Mar 31.
Artículo en Francés | MEDLINE | ID: mdl-38846118

RESUMEN

Sarcoidosis is a multisystem inflammatory disease of unknown etiology. The isolated extrapulmonary form is rare. We report the case of hepatosplenic sarcoidosis in a 29-year-old female patient.It is a patient with no notable medical history, who was seen in consultation for repeated epistaxis. Clinical examination noted nodular hepatomegaly associated with signs of portal hypertension and splenomegaly. Sedimentation rate, alkaline phosphatase, serum angiotensin converting enzyme, aminotransferases were high. Histological examination of the spleen and liver biopsy noted granulomatous inflammatory infiltration without cancerous lesion or tonsil stones.This picture is comparable with sarcoidosis, despite the absence of PET scans. The main challenge remains the differential diagnosis with other granulomatoses. Corticosteroid therapy is the first-line treatment, and after splenectomy the patient has achieved clinical and biological stability.


Asunto(s)
Hepatopatías , Sarcoidosis , Enfermedades del Bazo , Humanos , Sarcoidosis/patología , Sarcoidosis/diagnóstico , Femenino , Adulto , Enfermedades del Bazo/patología , Enfermedades del Bazo/cirugía , Enfermedades del Bazo/diagnóstico , Congo , Hepatopatías/patología , Hepatopatías/diagnóstico , Hospitales Universitarios
5.
Medicine (Baltimore) ; 103(23): e38466, 2024 Jun 07.
Artículo en Inglés | MEDLINE | ID: mdl-38847710

RESUMEN

RATIONALE: Sclerosing angiomatoid nodular transformation (SANT) of the spleen is an uncommon benign vascular lesion with an obscure etiology. It predominantly affects middle-aged women and presents with nonspecific clinical signs, making preoperative diagnosis challenging. The definitive diagnosis of SANT relies on pathological examination following splenectomy. This study aims to contribute to the understanding of SANT by presenting a case series and reviewing the literature to highlight the clinical presentation, diagnostic challenges, and treatment outcomes. PATIENT CONCERNS: In this retrospective study, we analyzed the clinical data of 3 patients with confirmed SANT admitted from November 2013 to October 2023. The cases include a 25-year-old male, a 15-year-old female, and a 39-year-old male, each with a splenic mass. DIAGNOSES AND INTERVENTIONS: All of the three cases were treated by laparoscopic splenectomy (LS). Pathological examination confirmed SANT in all cases. OUTCOMES: No recurrence or metastasis was observed during a 10-year follow-up for the first 2 cases, and the third case showed no abnormalities at 2 months postoperatively. Despite its rarity, SANT is a significant condition due to its potential for misdiagnosis and the importance of distinguishing it from malignant lesions. The study underscores the utility of LS as a safe and effective treatment option. LESSONS: SANT is a rare benign tumor of the spleen, and the preoperative diagnosis of whom is challenging. LS is a safe and effective treatment for SANT, with satisfactory surgical outcomes and favorable long-term prognosis on follow-up. The study contributes to the limited body of research on this rare condition and calls for larger studies to validate these findings and improve clinical management.


Asunto(s)
Esplenectomía , Neoplasias del Bazo , Humanos , Masculino , Adulto , Femenino , Esplenectomía/métodos , Adolescente , Neoplasias del Bazo/patología , Neoplasias del Bazo/cirugía , Neoplasias del Bazo/diagnóstico , Bazo/patología , Histiocitoma Fibroso Benigno/patología , Histiocitoma Fibroso Benigno/cirugía , Histiocitoma Fibroso Benigno/diagnóstico , Estudios Retrospectivos , Laparoscopía/métodos , Diagnóstico Diferencial , Enfermedades del Bazo/cirugía , Enfermedades del Bazo/patología , Enfermedades del Bazo/diagnóstico
6.
Chirurgia (Bucur) ; 119(2): 184-190, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-38743831

RESUMEN

Background: Splenectomy has been performed for various indications from haematological diseases to benign cysts and tumours, and for splenic traumatic injuries. However, there has been a steady decline in splenectomies in the last 20 years. The aim of this study is to establish the reasons behind this decline in splenectomy and to analyse them based on indication, type of splenectomy, and manner of approach (open, laparoscopic or robotic). Material and Methods: This is a retrospective study of a single centre experience of all the splenectomies, both total and partial, performed in the Department of General Surgery of Fundeni Clinical Institute (Bucharest) between 2002 and 2023. Only surgeries for primary splenic diseases were selected, splenic resections as part of other major operations were not included. Results: Between 2002 and 2023, 876 splenectomies were performed in the Department of General Surgery of Fundeni Clinical Institute (Bucharest). Most splenectomies (n=245) were performed for immune thrombocytopenic purpura (ITP), followed by benign tumours and cysts (n=136), lymphoma (n=119), hypersplenism due to cirrhosis (n=107) and microspherocytosis (n=95). Other indications included myelodysplastic syndrome (n=39), trauma (n=35), thalassemia (n=22), leukaemia (n=18) and also there were 60 splenectomies that were performed for hypersplenism of unknown cause. There were 795 total splenectomies (TS) and 81 partial splenectomies (PS). There was a decline in the number of splenectomies both TS and PS for all these indications, most notably in the case of ITP, microspherocytosis and hypersplenism due to cirrhosis with no splenectomies performed for these indications since 2020. Conclusion: With the development of new lines of treatment, advances in interventional radiology and in surgery with the spleen parenchyma sparing options, the need for total splenectomy has been greatly reduced which is reflected in the decline in the number of splenectomies performed in the last 20 years in our clinic.


Asunto(s)
Laparoscopía , Procedimientos Quirúrgicos Robotizados , Esplenectomía , Enfermedades del Bazo , Humanos , Esplenectomía/métodos , Esplenectomía/estadística & datos numéricos , Estudios Retrospectivos , Laparoscopía/métodos , Rumanía/epidemiología , Procedimientos Quirúrgicos Robotizados/métodos , Resultado del Tratamiento , Enfermedades del Bazo/cirugía , Femenino , Masculino , Adulto , Persona de Mediana Edad , Púrpura Trombocitopénica Idiopática/cirugía , Anciano , Linfoma/cirugía , Hiperesplenismo/cirugía , Hiperesplenismo/etiología , Talasemia/cirugía , Quistes/cirugía
7.
J Ethnopharmacol ; 331: 118276, 2024 Sep 15.
Artículo en Inglés | MEDLINE | ID: mdl-38697408

RESUMEN

ETHNOPHARMACOLOGICAL RELEVANCE: As a classical traditional Chinese medicine formula to invigorating spleen and replenishing qi, Sijunzi decoction (SJZD) is composed of four herbs, which is applied to cure spleen deficiency syndrome (SDS) clinically. The non-polysaccharides (NPSs) of SJZD (SJZD_NPS) are important pharmacodynamic material basis. However, the amelioration mechanism of SJZD_NPS on SDS has not been fully elaborated. Additionally, the contribution of herbs compatibility to efficacy of this formula remains unclear. AIM OF THE STUDY: The aim was to explore the underlying mechanisms of SJZD_NPS on improving SDS, and uncover the scientific connotation in SJZD compatibility. MATERIALS AND METHODS: A strategy integrating incomplete formulae (called "Chai-fang" in Chinese) comparison, pharmacodynamics, gut microbiome, and metabolome was employed to reveal the role of each herb to SJZD compatibility against SDS. Additionally, the underlying mechanism harbored by SJZD_NPS was further explored through targeted metabolomics, network pharmacology, molecular docking, pseudo-sterile model, and metagenomics. RESULTS: SJZD_NPS significantly alleviated diarrhea, disordered secretion of gastrointestinal hormones and neurotransmitters, damage of ileal morphology and intestinal barrier in SDS rats, which was superior to the NPSs of Chai-fang. 16S rRNA gene sequencing and metabolomics analyses revealed that SJZD_NPS effectively restored the disturbed gut microbiota community and abnormal metabolism caused by SDS, showing the most evident recovery. Moreover, SJZD_NPS recalled the levels of partial amino acids, short chain fatty acids and bile acids, which possessed strong binding affinity towards potential targets. The depletion of gut microbiota confirmed that the SDS-amelioration efficacy of SJZD_NPS is dependent on the intact gut microbiome, with the relative abundance of potential probiotics such as Lactobacillus_johnsonii and Lactobacillus_taiwanensis been enriched. CONCLUSION: NPSs in SJZD can improve SDS-induced gastrointestinal-nervous system dysfunction through regulating microbiota-gut-metabolites axis, with four herbs exerting synergistic effects, which indicated the compatibility rationality of SJZD.


Asunto(s)
Medicamentos Herbarios Chinos , Microbioma Gastrointestinal , Enfermedades del Bazo , Animales , Medicamentos Herbarios Chinos/farmacología , Microbioma Gastrointestinal/efectos de los fármacos , Masculino , Ratas , Enfermedades del Bazo/tratamiento farmacológico , Ratas Sprague-Dawley , Metabolómica , Simulación del Acoplamiento Molecular , Bazo/efectos de los fármacos , Bazo/metabolismo , Sinergismo Farmacológico , Modelos Animales de Enfermedad , Multiómica
8.
J Cardiothorac Surg ; 19(1): 220, 2024 Apr 16.
Artículo en Inglés | MEDLINE | ID: mdl-38627803

RESUMEN

BACKGROUND: Splenic abscess is a serious complication associated with infective endocarditis. There is still contradicting evidence regarding the optimal treatment pathway including timing of valve intervention and the approach for managing splenic foci. CASE PRESENTATION: We present a case of a hybrid staged approach in which we successfully performed a laparoscopic splenectomy following percutaneous abscess drainage and a delayed aortic valve replacement. CONCLUSIONS: A multidisciplinary teamwork is fundamental in providing optimal care for patients with distant complications associated with infective endocarditis. Our hybrid approach seems safe and feasible.


Asunto(s)
Embolia , Endocarditis Bacteriana , Endocarditis , Enfermedades del Bazo , Humanos , Enfermedades del Bazo/cirugía , Enfermedades del Bazo/complicaciones , Absceso/etiología , Absceso/cirugía , Válvula Aórtica/cirugía , Endocarditis/complicaciones , Endocarditis/cirugía , Endocarditis Bacteriana/complicaciones , Endocarditis Bacteriana/cirugía , Embolia/complicaciones
9.
Sci Rep ; 14(1): 7917, 2024 04 04.
Artículo en Inglés | MEDLINE | ID: mdl-38575738

RESUMEN

Contained vascular injuries (CVI) of spleen include pseudoaneurysms (PSA) and arterio-venous fistulae (AV-fistulae), and their reported prevalence varies. Our purpose was to assess the prevalence of early splenic CVI seen on admission CT in patients with splenic trauma admitted to a single level 1 trauma center in 2013-2021, and its detection in different CT protocols. A retrospective, single-center longitudinal cohort study. Nine-year data (2013-2021) of all patients with suspected or manifest abdominal trauma were retrieved. All patients, > 15 years with an ICD code for splenic trauma (S36.0XX) were included. CT and angiographic examinations were identified. Reports and images were reviewed. Splenic CVI CT criterion was a focal collection of vascular contrast that decreases in attenuation with delayed imaging. Number of CVIs and treatment was based on medical records and/or available angioembolization data. Of 2805 patients with abdominal trauma, 313 patients (313/2805; 11.2%) fulfilled the study entry criteria. 256 patients (256/313; 81.8%) had a CT examination. Sixteen patients had splenectomy before CT, and the final study group included 240 patients (240/313; 76.7%). Median New Injury Severity Score (NISS) was 27 and 87.5% of patients had NISS > 15. Splenic CVI was found in 20 patients, which yields a prevalence of 8.3% (20/240; 95% CI 5.2-12.6%). In those cases with both late arterial and venous phase images available, CVI was seen in 14.5% of cases (18/124, 95% CI 8.6-22.0%). None of the patients with CVI died within 30 days of the injury. The prevalence of early splenic CVI in patients with a splenic trauma was 8.3-14.5% (95% CI 5.2-22.0%). Our data suggests that both arterial and venous phase are needed for CT diagnosis. The 30-day outcome in terms of mortality was good.


Asunto(s)
Traumatismos Abdominales , Embolización Terapéutica , Enfermedades del Bazo , Lesiones del Sistema Vascular , Heridas no Penetrantes , Humanos , Lesiones del Sistema Vascular/diagnóstico por imagen , Lesiones del Sistema Vascular/epidemiología , Estudios Retrospectivos , Estudios Longitudinales , Prevalencia , Traumatismos Abdominales/diagnóstico por imagen , Traumatismos Abdominales/epidemiología , Traumatismos Abdominales/terapia , Heridas no Penetrantes/terapia
10.
Int J Surg ; 110(4): 2381-2388, 2024 Apr 01.
Artículo en Inglés | MEDLINE | ID: mdl-38668664

RESUMEN

BACKGROUND: A colosplenic fistula (CsF) is an extremely rare complication. Its diagnosis and management remain poorly understood, owing to its infrequent incidence. Our objective was to systematically review the etiology, clinical features, diagnosis, management, and prognosis to help clinicians gain a better understanding of this unusual complication and provide aid if it is to be encountered. METHODS: A systematic review of studies reporting CsF diagnosis in Ovid MEDLINE, Ovid EMBASE, Scopus, Web of Science, and Wiley Cochrane Library from 1946 to June 2022. Additionally, a retrospective review of four cases at our institution were included. Cases were evaluated for patient characteristics (age, sex, and comorbidities), CsF characteristics including causes, symptoms at presentation, diagnosis approach, management approach, pathology findings, intraoperative complications, postoperative complications, 30-day mortality, and prognosis were collected. RESULTS: Thirty patients with CsFs were analyzed, including four cases at our institution and 26 single-case reports. Most of the patients were male (70%), with a median age of 56 years. The most common etiologies were colonic lymphoma (30%) and colorectal carcinoma (17%). Computed tomography (CT) was commonly used for diagnosis (90%). Approximately 87% of patients underwent a surgical intervention, most commonly segmental resection (81%) of the affected colon and splenectomy (77%). Nineteen patients were initially managed surgically, and 12 patients were initially managed nonoperatively. However, 11 of the nonoperative patients ultimately required surgery due to unresolved symptoms. The rate of postoperative complications was (17%). Symptoms resolved with surgical intervention in 25 (83%) patients. Only one patient (3%) had had postoperative mortality. CONCLUSIONS: Our review of 30 cases worldwide is the largest in literature. CsFs are predominantly complications of neoplastic processes. CsF may be successfully and safely treated with splenectomy and resection of the affected colon, with a low rate of postoperative complications.


Asunto(s)
Enfermedades del Bazo , Humanos , Enfermedades del Bazo/cirugía , Enfermedades del Bazo/diagnóstico , Enfermedades del Bazo/terapia , Masculino , Femenino , Persona de Mediana Edad , Fístula Intestinal/cirugía , Fístula Intestinal/diagnóstico , Esplenectomía , Adulto , Anciano , Complicaciones Posoperatorias , Enfermedades del Colon/cirugía , Enfermedades del Colon/diagnóstico , Enfermedades del Colon/terapia , Tomografía Computarizada por Rayos X
11.
J Med Case Rep ; 18(1): 181, 2024 Apr 14.
Artículo en Inglés | MEDLINE | ID: mdl-38615066

RESUMEN

BACKGROUND: Wandering spleen (or ectopic spleen) refers to a hyper-mobile spleen resulting in its displacement from the normal anatomical position to usually in the lower abdominal or pelvic cavity. While ultrasound is often the first radiological modality used, Computed Tomography (CT) shows a clear picture and aides to reach a diagnosis. In circumstances where appropriate imaging modalities are not available, or the operator is inexperienced, diagnosis of wandering spleen can be missed. CASE PRESENTATION: A 22-nulligravida unmarried Sindhi female had presented to the Emergency Room (ER) with a 5-day history of intermittent severe lower abdominal pain. An ultrasound at a local practitioner had suggested an ovarian cyst. Ultrasound-pelvis and later CT scan at our facility reported an enlarged wandering spleen with torsion of its pedicle and infarction. Exploratory laparotomy with splenectomy was done. An enlarged wandering spleen was found with torsion of the splenic vein and thrombosed arterial supply from omentum wrapped over the mass. The patient developed thrombocytosis post-surgery but otherwise did well and was discharged after 2 days. CONCLUSION: Splenic torsion secondary to a wandering spleen can be challenging to diagnose, especially in resource limited settings where ultrasound might be the only modality available. Timely diagnosis and proper intervention are key to saving the life and the spleen.


Asunto(s)
Quistes Ováricos , Enfermedades del Bazo , Ectopía del Bazo , Femenino , Humanos , Ectopía del Bazo/diagnóstico , Ectopía del Bazo/diagnóstico por imagen , Enfermedades del Bazo/diagnóstico por imagen , Enfermedades del Bazo/cirugía , Esplenomegalia , Quistes Ováricos/diagnóstico por imagen , Quistes Ováricos/cirugía
12.
J Am Vet Med Assoc ; 262(6): 1-3, 2024 Jun 01.
Artículo en Inglés | MEDLINE | ID: mdl-38579752

RESUMEN

OBJECTIVE: To describe the clinical presentation of a Thoroughbred filly with acute hemoperitoneum from a splenic source immediately after racing. ANIMAL: A 3-year-old Thoroughbred filly used for racing and that had raced shortly before presentation to the hospital. CLINICAL PRESENTATION, PROGRESSION, AND PROCEDURES: On presentation, the filly was quiet, alert, and responsive with a heart rate of 76 beats/min, pale mucous membranes, and absent borborygmi. All other physical examination parameters were within normal limits. Abdominal ultrasound was performed and revealed echogenic free abdominal fluid and a splenic hematoma. Abdominocentesis yielded sanguinous fluid with a PCV of 35%. The next day, repeat ultrasound revealed the splenic hematoma with capsular separation. TREATMENT AND OUTCOME: The filly was treated overnight with isotonic crystalloid fluids and aminocaproic acid (40 mg/kg, IV, slow bolus over 30 minutes followed by 20 mg/kg, IV, q 6 h), potassium penicillin (22,000 IU/kg, IV, q 6 h), gentamicin (6.6 mg/kg, IV, q 24 h), and omeprazole (4 mg/kg, PO, q 24 h). The lowest PCV obtained from the filly was 36 hours after presentation. The filly stabilized with medical treatment and was discharged to a farm for further recuperation. CLINICAL RELEVANCE: There are no published reports detailing hemoperitoneum of splenic origin in Thoroughbreds immediately after racing. Hemoperitoneum of splenic origin is not common in horses, with most cases of hemoperitoneum being secondary to acute trauma, neoplasia, parturition, or postoperative complications. While uncommon, this case raises awareness to another differential for a colicky horse immediately after racing.


Asunto(s)
Hematoma , Hemoperitoneo , Enfermedades de los Caballos , Animales , Caballos , Hematoma/veterinaria , Hematoma/etiología , Hemoperitoneo/veterinaria , Hemoperitoneo/etiología , Enfermedades de los Caballos/etiología , Femenino , Enfermedades del Bazo/veterinaria , Carrera
13.
Br J Haematol ; 204(5): 1672-1686, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-38600782

RESUMEN

Guidelines for the prevention and treatment of infection in patients with an absent or dysfunctional spleen were published by the British Committee for Standards in Haematology in 1996 and updated in 2002 and 2011. With advances in vaccinations and changes in patterns of infection, the guidelines required updating. Key aspects included in this guideline are the identification of patients at risk of infection, patient education and information and immunisation schedules. This guideline does not address the non-infective complications of splenectomy or functional hyposplenism (FH). This replaces previous guidelines and significantly revises the recommendations related to immunisation. Patients at risk include those who have undergone surgical removal of the spleen, including partial splenectomy and splenic embolisation, and those with medical conditions that predispose to FH. Immunisations should include those against Streptococcus pneumoniae (pneumococcus), Neisseria meningitidis (meningococcus) and influenza. Haemophilus influenzae type b (Hib) is part of the infant immunisation schedule and is no longer required for older hyposplenic patients. Treatment of suspected or proven infections should be based on local protocols and consider relevant anti-microbial resistance patterns. The education of patients and their medical practitioners is essential, particularly in relation to the risk of serious infection and its prevention. Further research is required to establish the effectiveness of vaccinations in hyposplenic patients; infective episodes should be regularly audited. There is no single group ideally placed to conduct audits into complications arising from hyposplenism, highlighting a need for a national registry, as has proved very successful in Australia or alternatively, the establishment of appropriate multidisciplinary networks.


Asunto(s)
Esplenectomía , Humanos , Esplenectomía/efectos adversos , Bazo , Enfermedades del Bazo/terapia , Vacunación
14.
Ann Hematol ; 103(5): 1765-1774, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-38509388

RESUMEN

Gaucher disease (GD) is an autosomal recessive ailment resulting from glucocerebrosidase deficiency caused by a mutation in the GBA1 gene, leading to multi-organ problems in the liver, spleen, and bone marrow. In China, GD is extremely uncommon and has a lower incidence rate than worldwide. In this study, we report the case of an adult male with an enlarged spleen for 13 years who presented with abdominal distension, severe loss of appetite and weight, reduction of the three-line due to hypersplenism, frequent nosebleeds, and bloody stools. Regrettably, the unexpected discovery of splenic pathology suggestive of splenic Gaucher disease was only made after a splenectomy due to a lack of knowledge about rare disorders. Our patient's delayed diagnosis may have been due to the department where he was originally treated, but it highlights the need for multidisciplinary consultation in splenomegaly of unknown etiology. We then investigated the patient's clinical phenotypes and gene mutation features using genetically phenotypical analysis. The analysis of the GBA1 gene sequence indicated that the patient carried a compound heterozygous mutation consisting of two potentially disease-causing mutations: c.907C > A (p. Leu303Ile) and c.1448 T > C (p. Leu483Pro). While previous research has linked the p. Leu483Pro mutation site to neurologic GD phenotypes (GD2 and GD3), the patients in this investigation were identified as having non-neuronopathic GD1. The other mutation, p. Leu303Ile, is a new GD-related mutation not indexed in PubMed that enriches the GBA1 gene mutation spectrum. Biosignature analysis has shown that both mutations alter the protein's three-dimensional structure, which may be a pathogenic mechanism for GD1 in this patient.


Asunto(s)
Enfermedad de Gaucher , Enfermedades del Bazo , Adulto , Humanos , Masculino , Enfermedad de Gaucher/complicaciones , Enfermedad de Gaucher/genética , Enfermedad de Gaucher/cirugía , Esplenectomía , Médula Ósea , Fenotipo , Esplenomegalia/genética , Mutación , Glucosilceramidasa/genética
16.
J Emerg Med ; 66(4): e523-e525, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-38461136

RESUMEN

BACKGROUND: Sickle cell trait (SCT), the heterozygous form of sickle cell disease, is generally thought of as a benign condition. However, it is possible for those with SCT to have serious complications, especially when they are exposed to high altitudes where oxygen levels are low. CASE REPORT: We present a case of a 41-year-old man with a history of SCT who developed severe epigastric pain and nearly lost consciousness while traveling on a commercial airplane. His twin brother, who also has SCT, had a similar episode in the past and required a splenectomy. A splenic subcapsular hematoma was found in a computed tomography scan of the abdomen and pelvis with intravenous contrast. He was admitted and managed conservatively until his symptoms resolved. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Though SCT is prevalent in our population, the complications that can arise, such as altitude-associated splenic syndrome, have likely not been thoroughly investigated. Physicians should add this condition to their differential if they practice at locations near airports or in areas of higher altitude and if their patients have a past medical history of SCT.


Asunto(s)
Viaje en Avión , Rasgo Drepanocítico , Enfermedades del Bazo , Infarto del Bazo , Masculino , Humanos , Adulto , Altitud , Infarto del Bazo/complicaciones , Infarto del Bazo/diagnóstico , Enfermedades del Bazo/etiología , Rasgo Drepanocítico/complicaciones , Rasgo Drepanocítico/diagnóstico , Hematoma/complicaciones
17.
BMJ Case Rep ; 17(3)2024 Mar 25.
Artículo en Inglés | MEDLINE | ID: mdl-38531552

RESUMEN

Purpura fulminans (PF) is a life-threatening complication of septic shock that can occur due to disseminated infections with Streptococcus pneumoniae The spleen is an important organ in the immunisation process against encapsulated bacteria. Patients with asplenia, either functional or anatomical, are therefore at increased risk of developing serious infections and complications, such as PF, if infected with such bacteria.This case report presents a woman in her late 40s with unacknowledged functional asplenia who was admitted to the hospital with signs of an acute disseminated infection causing septic shock, signs of disseminated intravascular coagulation and infectious PF. A few days after admission, the blood cultures showed growth of S. pneumoniae With early sepsis treatment, the patient survived although with some complications. Clinical presentation, investigations, differential diagnosis, treatment and outcome are presented. Treatment and early recognition of PF are presented and discussed. Relevant recognition and preventative treatment strategies for patients with asplenia are also reviewed and discussed.This case demonstrates the importance of early recognition and treatment of PF in septic patients and the importance of preventive treatment strategies for patients with asplenia to avoid serious infections and complications.


Asunto(s)
Bacteriemia , Infecciones Neumocócicas , Púrpura Fulminante , Sepsis , Choque Séptico , Enfermedades del Bazo , Femenino , Humanos , Púrpura Fulminante/diagnóstico , Choque Séptico/complicaciones , Infecciones Neumocócicas/microbiología , Streptococcus pneumoniae , Sepsis/complicaciones , Bacteriemia/complicaciones , Enfermedades del Bazo/complicaciones
18.
Clin J Gastroenterol ; 17(3): 580-586, 2024 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-38457070

RESUMEN

Xanthogranulomatous inflammation is a chronic inflammatory reaction microscopically characterized by aggregation of foamy histiocytes, fibrous tissue, and infiltration of various inflammatory cells. In contrast to xanthogranulomatous inflammation in the gallbladder or kidney, xanthogranulomatous pancreatitis is rare. We herein present a case of xanthogranulomatous pancreatitis in a patient who underwent distal pancreatectomy with splenectomy under preoperative suspicion of a pancreatic pseudocyst or pancreatic tumor. A 77-year-old woman with a 1 month history of epigastric pain, anorexia, and general fatigue was admitted to our hospital. Contrast-enhanced computed tomography revealed a cystic mass with ill-defined margins at the pancreatic tail together with a splenic abscess. Contrast-enhanced endoscopic ultrasound detected a hyperechoic cystic lesion at the tail of the pancreas with heterogeneous internal echogenicity, and part of the intra-cystic content was enhanced by the contrast agent. Endoscopic retrograde cholangiopancreatography showed a cystic lesion at the tail of the pancreas that continued into the main pancreatic duct, and the main pancreatic duct was slightly narrowed downstream of the cystic lesion. Pancreatic juice cytology revealed suspicious cells, leading to the possibility of intraductal papillary mucinous carcinoma. Distal pancreatectomy with splenectomy was performed, and the histopathological diagnosis was xanthogranulomatous pancreatitis with no malignant findings.


Asunto(s)
Pancreatectomía , Pancreatitis , Enfermedades del Bazo , Tomografía Computarizada por Rayos X , Xantomatosis , Humanos , Anciano , Femenino , Enfermedades del Bazo/cirugía , Enfermedades del Bazo/diagnóstico por imagen , Enfermedades del Bazo/patología , Enfermedades del Bazo/complicaciones , Xantomatosis/cirugía , Xantomatosis/complicaciones , Xantomatosis/patología , Pancreatitis/cirugía , Pancreatitis/complicaciones , Absceso/cirugía , Absceso/diagnóstico por imagen , Esplenectomía , Granuloma/cirugía , Granuloma/patología , Granuloma/diagnóstico por imagen , Colangiopancreatografia Retrógrada Endoscópica , Endosonografía
19.
J Med Case Rep ; 18(1): 136, 2024 Mar 07.
Artículo en Inglés | MEDLINE | ID: mdl-38449048

RESUMEN

BACKGROUND: Gastrosplenic fistula is a rare and potentially fatal complication of various conditions. Lymphoma is the most common cause. It can occur spontaneously or after chemotherapy. Gastrosplenic fistula diagnosis can be confused with a splenic abscess because of the presence of air into the mass. The computed tomography identification of the fistulous tract is the key to a right diagnosis. Treatment modalities include surgical resection, chemotherapy, or a combination of both. CASE PRESENTATION: Here we report two patients with gastrosplenic fistula due to diffuse large B cell lymphoma. The first patient was a 54-year-old Caucasian woman with an enormous primary splenic diffuse large B cell lymphoma leading to the development of a spontaneous fistula in the stomach. The second patient was a 48-year-old Caucasian male patient with an enormous splenic diffuse large B cell lymphoma complicated by fistula after chemotherapy. Both patients died of septic shock several days after surgery. CONCLUSION: Gastrosplenic fistula is a rare complication with a poor-prognosis, for which surgery is currently the preferred treatment.


Asunto(s)
Absceso Abdominal , Fístula , Linfoma de Células B Grandes Difuso , Enfermedades del Bazo , Femenino , Humanos , Masculino , Persona de Mediana Edad , Enfermedades del Bazo/diagnóstico por imagen , Enfermedades del Bazo/etiología , Enfermedades del Bazo/terapia , Linfoma de Células B Grandes Difuso/complicaciones , Linfoma de Células B Grandes Difuso/diagnóstico por imagen , Linfoma de Células B Grandes Difuso/terapia , Confusión
20.
Indian J Pathol Microbiol ; 67(1): 102-106, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38358197

RESUMEN

Background: Splenic sclerosing angiomatoid nodular transformation (SANT) is a rare benign nodular lesion in the red medulla of the spleen. In the past, SANT has not been consistently recognized as the name for this condition and was often misdiagnosed for other conditions. In recent years, SANT has been acknowledged by most scholars as multiple reports have been published. Aim: To assess the clinicopathological features of SANT to identify the histological characteristics of SANT to improve diagnosis and clinical treatment. Materials and Methods: We assessed 25 cases of SANT diagnosed at Zhongshan Hospital affiliated with Fudan University from September 2014 to October 2021, including 14 men and 11 women, aged 24-62 years old. Results: Fourteen cases were complicated with benign tumors of the liver, pancreas, kidney, uterus, and prostate. One case was complicated with renal clear cell carcinoma, and one was complicated with hepatocellular carcinoma. The gross neoplasm is multinodular and well defined. Histologically, angiomatoid nodules are composed of fattened, round, or irregular blood vessels, with or without red blood cells in the lumen, with unequal red blood cell extravasation, and fibrocytes around the nodules. The hemangiomatous nodules were positive for CD31 and CD34, while the vascular wall smooth muscle cells and fibrocytes around the nodules were positive for SMA. Conclusion: The diagnosis of SANT requires a combination of immunohistochemical and histological features, and early splenectomy is crucial for treatment.


Asunto(s)
Hemangioma , Histiocitoma Fibroso Benigno , Enfermedades del Bazo , Neoplasias del Bazo , Masculino , Humanos , Femenino , Adulto Joven , Adulto , Persona de Mediana Edad , Enfermedades del Bazo/diagnóstico , Enfermedades del Bazo/cirugía , Enfermedades del Bazo/patología , Esplenectomía , Hemangioma/diagnóstico , Hemangioma/patología , Neoplasias del Bazo/diagnóstico , Neoplasias del Bazo/cirugía , Neoplasias del Bazo/patología
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