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2.
BMC Surg ; 20(1): 257, 2020 Oct 29.
Artigo em Inglês | MEDLINE | ID: mdl-33121468

RESUMO

BACKGROUND: Idiopathic portal hypertension (IPH) generally has a good prognosis and rarely results in liver transplantation. Furthermore, there are few reports of living donor liver transplantation (LDLT) for IPH with extrahepatic portal vein stenosis. CASE PRESENTATION: We report the case of a 51-year-old female patient diagnosed with IPH more than 20 years ago. She suffered severe jaundice, massive ascites, and encephalopathy at the time of her visit to our hospital. The patient's extrahepatic portal vein showed a scar-like stenosis, and the portal flow was completely hepatofugal. Collateral circulation such as the splenorenal shunt was well developed, and multiple splenic artery aneurysms up to 2 cm were observed in the splenic hilum. Her Model for End-Stage Liver Disease score increased to over 40 because of renal dysfunction, requiring temporary dialysis. We performed LDLT using her husband's right lobe graft and splenectomy. The extrahepatic stenotic portal vein was completely resected, and the superficial femoral vein (SFV) graft collected from the recipient's right leg was used for portal reconstruction as an interposition graft. Although the clinical course after LDLT had many complications, the patient was discharged on postoperative day 113 and has been fine for 2 years after LDLT. Histopathologically, the explanted liver had obliterative portal venopathy, nodular regenerative hyperplasia, and incomplete septal cirrhosis. CONCLUSION: This case showed that severe IPH is occasionally associated with extrahepatic portal vein stenosis and can be treated with LDLT with portal vein reconstruction using an interposition graft. It was also suggested that the SFV is a useful choice for the interposition graft.


Assuntos
Aneurisma/cirurgia , Hipertensão Portal/cirurgia , Cirrose Hepática/cirurgia , Transplante de Fígado , Doadores Vivos , Pancitopenia/cirurgia , Veia Porta/cirurgia , Artéria Esplênica/cirurgia , Esplenomegalia/cirurgia , Aneurisma/complicações , Constrição Patológica/complicações , Constrição Patológica/cirurgia , Feminino , Humanos , Hipertensão Portal/complicações , Cirrose Hepática/complicações , Pessoa de Meia-Idade , Pancitopenia/complicações , Veia Porta/patologia , Procedimentos Cirúrgicos Reconstrutivos/métodos , Esplenectomia , Esplenomegalia/complicações , Procedimentos Cirúrgicos Vasculares/métodos
5.
Am J Med Sci ; 359(5): 296-302, 2020 05.
Artigo em Inglês | MEDLINE | ID: mdl-32265009

RESUMO

Thrombotic microangiopathy (TMA) is characterized by microangiopathic hemolytic anemia with thrombocytopenia. In addition to the primary TMA syndromes, microangiopathic hemolytic anemia with thrombocytopenia can be seen in many systemic diseases. Transplant associated TMA (TA-TMA) affects patients following stem cell or solid organ transplant. A 48-year-old male who underwent autologous stem cell transplant for nonsecretory multiple myeloma was admitted to our hospital with worsening anemia, thrombocytopenia, renal dysfunction and hepatosplenomegaly. Initial blood work revealed rare schistocytes and normal lactate dehydrogenase and haptoglobin levels. He underwent an extensive workup looking for an infectious, inflammatory or malignant etiology but a definitive diagnosis could not be reached. Over his prolonged stay at the hospital, he suffered from multiorgan failure and eventually passed away. An autopsy revealed TMA involving all clinically affected organ systems and was deemed to be the cause of his demise. The absence of typical blood work suggestive of hemolysis does not rule out a diagnosis of TA-TMA. Knowledge of this rare disease entity will help physicians identify and treat this life-threatening condition early and effectively.


Assuntos
Eritrócitos Anormais , Hemólise , Microangiopatias Trombóticas/complicações , Biópsia , Evolução Fatal , Hepatomegalia/complicações , Humanos , Inflamação , Integrina beta3/metabolismo , L-Lactato Desidrogenase/metabolismo , Fígado/patologia , Pulmão/patologia , Masculino , Microcirculação , Pessoa de Meia-Idade , Esplenomegalia/complicações , Transplante de Células-Tronco , Trombocitopenia/complicações , Trombose/metabolismo , Microangiopatias Trombóticas/terapia , Transplante Autólogo
6.
Sci Rep ; 10(1): 6917, 2020 04 24.
Artigo em Inglês | MEDLINE | ID: mdl-32332802

RESUMO

There is convincing evidence from different mouse models that chronic psychosocial stress promotes splenomegaly, basal and lipopolysaccharide (LPS)-induced in vitro splenocyte activation and insensitivity towards glucocorticoids (GC) in in vitro LPS-treated splenocytes. However, we just recently showed, employing the chronic subordinate colony housing (CSC) paradigm, that bite wounds received during stressor exposure drive these stress-induced spleen changes. As skin wounds induced by planned surgery or physical trauma are more adequately reflecting what chronically stressed humans are likely to experience, it was the objective of the present study to investigate whether abdominal surgery prior to stressor exposure also promotes respective stress-induced spleen effects in the absence of any bite wounds. In line with our hypothesis, abdominal surgery prior to CSC induced splenomegaly, increased in vitro cell viability under basal and LPS conditions as well as the delta response to LPS (LPS - basal), and promoted the inability of isolated splenocytes to respond with a decreased cell viability to increasing concentrations of corticosterone following LPS-stimulation in vitro. Together with previous data, these findings demonstrate that physical injury, either in form of received bite wounds during stressor exposure or in form of abdominal surgery prior to stressor exposure, promotes the development of splenic immune activation and GC resistance.


Assuntos
Abdome/cirurgia , Glucocorticoides/farmacologia , Baço/patologia , Estresse Psicológico/complicações , Animais , Doença Crônica , Abrigo para Animais , Masculino , Camundongos Endogâmicos C57BL , Esplenomegalia/complicações
7.
J Pediatr Endocrinol Metab ; 33(3): 437-441, 2020 Mar 26.
Artigo em Inglês | MEDLINE | ID: mdl-32069232

RESUMO

Background Peroxisomal biogenesis disorders (PBDs) include a miscellaneous group of diseases which cause serious multisystem disease. Mutations of 13 different PEX genes lead to PBDs including Zellweger syndrome (ZS). Different types of mutations of PEX1 and PEX10 genes are correlated with broad-range phenotypes of PBDs. Case presentation Patient 1 is a 4-month-old boy who was affected by myoclonic seizures, poor oral feeding since birth. The patient was hypotonic and had hepatosplenomegaly. Patient 2 is a 2-month-old boy who presented with decreased movement, severe hypotonia and failure to thrive. The laboratory studies of the patients revealed increased plasma very-long-chain fatty acids (VLCFAs). The genetic analyses of patient 1 demonstrated the first homozygous missense mutation in the PEX10 gene. A novel homozygous missense mutation was found in the PEX1 gene in patient 2. Conclusions This report highlights that the detected homozygous missense mutations of PEX10 and PEX1 genes and the substitutions of specific amino acids lead to the severe form of PBDs.


Assuntos
Estudos de Associação Genética , Mutação de Sentido Incorreto/genética , Endopeptidase Neutra Reguladora de Fosfato PHEX/genética , Síndrome de Zellweger/genética , ATPases Associadas a Diversas Atividades Celulares/genética , Epilepsias Mioclônicas/etiologia , Epilepsias Mioclônicas/genética , Evolução Fatal , Ácidos Graxos/sangue , Hepatomegalia/complicações , Hepatomegalia/diagnóstico por imagem , Humanos , Lactente , Imagem por Ressonância Magnética , Masculino , Proteínas de Membrana/genética , Peroxinas/genética , Prognóstico , Receptores Citoplasmáticos e Nucleares/genética , Esplenomegalia/complicações , Esplenomegalia/diagnóstico por imagem , Síndrome de Zellweger/diagnóstico por imagem
9.
Niger J Clin Pract ; 22(11): 1617-1620, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31719286

RESUMO

Budd-Chiari syndrome (BCS) is a rare disease characterized by obstruction of hepatic venous outflow tract with diversified etiologies. Sea-blue histiocytosis (SBH) is a kind of storage diseases defined by the deposition of abundant sea-blue histiocytes in various organs and can lead to hepatosplenomegaly, cirrhosis, or even liver failure. The association between BCS and SBH has never been reported before. Here, we report a patient with BCS presenting with hepatosplenomegaly, portal hypertension, and pancytopenia who was later confirmed to also have SBH.


Assuntos
Síndrome de Budd-Chiari/complicações , Hepatomegalia/diagnóstico por imagem , Hipertensão Portal/complicações , Pancitopenia/complicações , Síndrome do Histiócito Azul-Marinho/diagnóstico , Esplenomegalia/diagnóstico por imagem , Adulto , Síndrome de Budd-Chiari/diagnóstico , Síndrome de Budd-Chiari/etiologia , Hepatomegalia/complicações , Humanos , Masculino , Doenças Raras , Esplenomegalia/complicações , Veia Cava Inferior
10.
Clin Nucl Med ; 44(12): 949-955, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31689275

RESUMO

INTRODUCTION: Autoimmune lymphoproliferative syndrome (ALPS) is a rare immune dysregulatory condition, usually presenting in childhood with massive lymphadenopathy, splenomegaly, and an increased incidence of lymphoma. Methods to differentiate between benign ALPS adenopathy and lymphoma are needed. To this end, we evaluated the usefulness of FDG PET. METHODS: We prospectively evaluated 76 ALPS/ALPS-like patients including FS-7-associated surface antigen (FAS) germline mutation with (n = 4) and without lymphoma (n = 50), FAS-somatic (n = 6), ALPS-unknown (n = 6), and others (n = 10) who underwent FDG PET. Uptakes in 14 nodal sites, liver, and spleen were determined. RESULTS: In 76 ALPS patients, FDG PET showed uptake in multiple nodal sites in all but 1 patient. The highest SUVmax values in FAS mutation without lymphoma, FAS mutation with lymphoma, FAS somatic, ALPS-unknown, and other genetic mutations were a median (range) 9.2 (4.3-25), 16.2 (10.7-37.2), 7.6 (4.6-18.1), 11.5 (4.8-17.2), and 5.5 (0-15.3), respectively. Differences between uptake in the FAS group with and without lymphoma were statistically significant, but overlapped, making discrimination between individuals with/without lymphoma impossible. The spleen:liver uptake ratio was greater than 1 in 82% of patients. CONCLUSIONS: While statistically significant differences were observed in FAS mutation ALPS with and without lymphoma, the significant overlap in FDG uptake and visual appearance in many patients prevents discrimination between patients with and without lymphoma. Similar patterns of FDG biodistribution were noted between the various ALPS subgroups.


Assuntos
Síndrome Linfoproliferativa Autoimune/diagnóstico por imagem , Fluordesoxiglucose F18 , Tomografia por Emissão de Pósitrons , Adolescente , Adulto , Síndrome Linfoproliferativa Autoimune/complicações , Síndrome Linfoproliferativa Autoimune/genética , Síndrome Linfoproliferativa Autoimune/metabolismo , Criança , Pré-Escolar , Feminino , Fluordesoxiglucose F18/farmacocinética , Humanos , Linfoma/complicações , Masculino , Mutação , Esplenomegalia/complicações , Distribuição Tecidual , Adulto Jovem , Receptor fas/genética
11.
Autops. Case Rep ; 9(3): e2019100, July-Sept. 2019. ilus
Artigo em Inglês | LILACS | ID: biblio-1017372

RESUMO

Primary splenic angiosarcoma (PSA) is a rare neoplasm of vascular origin associated with aggressive behavior and poor prognosis. The clinical presentation is usually non-specific and is mostly characterized by a wasting disease with anemia and splenomegaly, mimicking a wide range of entities. The authors present the case of an 80-year-old woman with cardiovascular comorbidities with a 6-month history of weight loss, fatigue, weakness, pallor, and abdominal pain. The physical examination showed massive splenomegaly and pallor. After a thorough evaluation that ruled out lymphoproliferative diseases, the working diagnosis was a myelodysplastic disorder. A few days after discharge, she returned to the emergency room with severe abdominal pain, worsening fatigue, and a remarkable pallor. Point-of-care ultrasound showed free intraperitoneal fluid. Spleen rupture was confirmed by abdominal computed tomography (CT) scan, and an emergency laparotomy with splenectomy was performed. The postoperative period was uneventful, and the patient recovered in a few days. The histopathology confirmed the diagnosis of PSA and the patient was referred to an oncological center. Two months later staging CT demonstrated liver and peritoneal metastases, and despite the chemotherapy she died 6 months after the diagnosis.


Assuntos
Humanos , Feminino , Idoso de 80 Anos ou mais , Neoplasias Esplênicas/complicações , Neoplasias Esplênicas/patologia , Hemangiossarcoma/patologia , Ruptura Esplênica/etiologia , Esplenomegalia/complicações , Evolução Fatal
12.
Indian J Gastroenterol ; 38(4): 310-316, 2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-31423562

RESUMO

BACKGROUND: Although endoscopy is the standard diagnostic screening test to identify esophageal varices in patients with chronic liver disease (CLD), selective endoscopy in patients who are at higher risk of having varices may be cost-effective in a resource-constrained country. The aim of this prospective study was to identify non-endoscopic parameters that may predict the presence of varices, especially high-risk esophageal varices in children with CLD. METHODS: From January 2016 through March 2018, consecutive children with CLD without a history of variceal bleeding were prospectively included. Esophagogastroduodenoscopy was done in all the children to detect and to grade esophageal varices. Both univariate and multivariate logistic regression analyses were done using SPSS version 22 to identify factors associated with esophageal varices. RESULTS: The mean age of 84 children was 9.7 ± 3.2 years (male 44). Esophageal varices were present in 71.4% of children and 55% of them had large varices. On univariate analysis, low platelet count (< 100,000/mm3) and splenomegaly were found to be associated with the presence of esophageal varices (p = 0.006 and 0.001, respectively) and large varices (p = 0.03 and 0.01, respectively). On multivariate analysis, both low platelet count and splenomegaly were independent predictors for the presence of esophageal varices (respectively, OR 11.21, 95% CI 1.2-96.9; and OR 11.39, 95% CI 3.19-40.59). CONCLUSIONS: Splenomegaly and low platelet count independently predict the presence of any grade of esophageal varices and can be used as screening tests to select children for endoscopy. This strategy may help in relieving medical, social, and economic costs in resource-constrained countries.


Assuntos
Doença Hepática Terminal/complicações , Varizes Esofágicas e Gástricas/diagnóstico , Contagem de Plaquetas/estatística & dados numéricos , Baço/diagnóstico por imagem , Criança , Doença Hepática Terminal/sangue , Doença Hepática Terminal/patologia , Varizes Esofágicas e Gástricas/etiologia , Feminino , Humanos , Modelos Logísticos , Masculino , Análise Multivariada , Valor Preditivo dos Testes , Estudos Prospectivos , Curva ROC , Fatores de Risco , Sensibilidade e Especificidade , Baço/patologia , Esplenomegalia/complicações
13.
J Korean Med Sci ; 34(30): e208, 2019 Aug 05.
Artigo em Inglês | MEDLINE | ID: mdl-31373186

RESUMO

BACKGROUND: Performing transarterial chemoembolization (TACE) is difficult with the occurrence of thrombocytopenia in cirrhotic patients with hepatocellular carcinoma (HCC). We aimed to evaluate the long-term efficacy and safety of partial splenic embolization (PSE) combined with TACE in patients with HCC with severe thrombocytopenia related to splenomegaly. METHODS: We conducted a case-control study consisting of 18 HCC patients with severe thrombocytopenia (< 50 × 109/L) who underwent PSE concurrently with TACE (PSE group) and 72 controls who underwent TACE alone (non-PSE group). RESULTS: Mean platelet counts at 1 month and 1, 3, and 5 years after concurrent PSE and TACE significantly increased compared with baseline (all P < 0.05), whereas the platelet count did not significantly increase after TACE alone. In addition, the platelet count at several time points after treatment in the PSE group was significantly higher than that in the non-PSE group, although the baseline platelet count in the PSE group was significantly lower than that in the non-PSE group. The platelet increase after PSE significantly reduced the need for platelet transfusions (P = 0.040) and enabled the subsequent TACE procedures in time (P = 0.046). The leukocyte counts and hemoglobin concentrations after concurrent PSE and TACE were also significantly increased, without deterioration of Child-Turcotte-Pugh score and unexpected side effects. CONCLUSION: PSE combined with TACE is effective in inducing and maintaining long-term thrombocytopenia improvement which reduces the need for the platelet transfusion and helps to perform initial and serial TACE, and is well-tolerated in patients with HCC and thrombocytopenia. PSE may be a promising treatment option for HCC patients with severe thrombocytopenia associated with splenomegaly who will undergo TACE.


Assuntos
Carcinoma Hepatocelular/terapia , Embolização Terapêutica/métodos , Neoplasias Hepáticas/terapia , Trombocitopenia/diagnóstico , Adulto , Idoso , Carcinoma Hepatocelular/complicações , Carcinoma Hepatocelular/mortalidade , Estudos de Casos e Controles , Quimioembolização Terapêutica , Feminino , Hemoglobinas/análise , Humanos , Contagem de Leucócitos , Neoplasias Hepáticas/complicações , Neoplasias Hepáticas/mortalidade , Masculino , Pessoa de Meia-Idade , Contagem de Plaquetas , Estudos Retrospectivos , Artéria Esplênica/cirurgia , Esplenomegalia/complicações , Esplenomegalia/diagnóstico , Taxa de Sobrevida , Trombocitopenia/complicações
15.
Ann Hematol ; 98(8): 1933-1936, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31201513

RESUMO

Ruxolitinib is the only commercially available JAK1/2 inhibitor approved for the treatment of myelofibrosis-related splenomegaly and symptoms. During treatment, as rare conditions, leukocytosis and/or thrombocytosis could develop and the management of these situations is not well established. We report here 53 myelofibrosis patients that received a combination of hydroxyurea and ruxolitinib because of uncontrolled myeloproliferation. Both drugs were administered outside clinical trials. At 48 weeks, a significant reduction in leucocyte and platelet counts was observed (p = 0.02 and p = 0.04, respectively). Additionally, the spleen volume decreased from a median value of 10 cm below the left costal margin (range, 0-10) to 6 cm (range, 0-15). The rate of spleen response increased from 14% at the start of the combination to 45% after 48 weeks. The safety profile of the combination was consistent with that observed with ruxolitinib single agent. These data require further confirmation in large cohorts of patients prospectively assessed.


Assuntos
Plaquetas/efeitos dos fármacos , Hidroxiureia/uso terapêutico , Leucócitos/efeitos dos fármacos , Mielofibrose Primária/tratamento farmacológico , Pirazóis/uso terapêutico , Esplenomegalia/tratamento farmacológico , Idoso , Idoso de 80 Anos ou mais , Plaquetas/patologia , Contagem de Células , Proliferação de Células/efeitos dos fármacos , Esquema de Medicação , Quimioterapia Combinada , Feminino , Humanos , Leucócitos/patologia , Masculino , Pessoa de Meia-Idade , Segurança do Paciente , Mielofibrose Primária/complicações , Mielofibrose Primária/mortalidade , Mielofibrose Primária/patologia , Estudos Retrospectivos , Esplenomegalia/complicações , Esplenomegalia/mortalidade , Esplenomegalia/patologia , Análise de Sobrevida , Resultado do Tratamento
16.
BMC Infect Dis ; 19(1): 439, 2019 May 20.
Artigo em Inglês | MEDLINE | ID: mdl-31109292

RESUMO

BACKGROUND: Brucellosis has extensive clinical spectrum, clinicians have insufficient understanding of the disease, and the misdiagnosis rate is still high. By collecting and analyzing the clinical characteristics of patients with brucellosis in Heilongjiang Province to provide guidance and reference for clinicians to make timely diagnosis and treatment. METHODS: The demographic and epidemiological characteristics, clinical features, complications, laboratory findings were retrospectively evaluated in 850 brucellosis patients admitted in the Department of Infectious Diseases of the First Affiliated Hospital of Harbin Medical University and the Second Hospital of Daqing from 2012 to 2017. RESULTS: Of the 850 patients, the most common clinical manifestations were fever (93.3%), joint pain (69.8%), sweating (45.2%), fatigue (38.6%), and splenomegaly (34.0%). Peripheral arthritis, spondylitis and epididymal-orchitis were the common complications. Of the 398 patients who were followed up and completed treatment, 22 (5.5%) had relapse. CONCLUSIONS: Brucellosis is a multisystem disease with diverse clinical manifestations. In areas where brucellosis is endemic, the possibility of the disease should be considered in patients with unexplained fever and joints pain. In addition, the high rate of relapse is mainly due to the misdiagnosis of complications, so local CT or MRI examination is necessary for patients with joint pain and low back pain. Timely diagnosis, early detection of complications are essential to improve the prognosis and reduce relapse.


Assuntos
Brucelose/diagnóstico , Adolescente , Adulto , Idoso , Artralgia/complicações , Brucelose/complicações , Brucelose/epidemiologia , Criança , Pré-Escolar , China/epidemiologia , Fadiga/complicações , Feminino , Febre/complicações , Humanos , Lactente , Recém-Nascido , Masculino , Pessoa de Meia-Idade , Recidiva , Estudos Retrospectivos , Esplenomegalia/complicações , Adulto Jovem
17.
Clin Nucl Med ; 44(4): e313-e314, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30789396

RESUMO

We present the case of a 71-year-old man with Gleason 3 + 3 = 6 pT2N0MxR0 adenocarcinoma of the prostate who presented with rising PSA level 16 years after radical prostatectomy. PSMA-targeted F-DCFPyL PET/CT was performed, which demonstrated recurrent disease in the prostatectomy bed as well as splenomegaly and mild-diffuse bone marrow activation, consistent with the patient's history of hereditary spherocytosis. We briefly review the clinical characteristics of hereditary spherocytosis, its appearance on molecular imaging studies, the normal biodistribution of F-DCFPyL, and the PSMA-RADS scoring system for characterizing findings on PSMA-targeted PET imaging.


Assuntos
Antígenos de Superfície/metabolismo , Medula Óssea/patologia , Glutamato Carboxipeptidase II/metabolismo , Lisina/análogos & derivados , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons , Esferocitose Hereditária/complicações , Esferocitose Hereditária/diagnóstico por imagem , Ureia/análogos & derivados , Idoso , Humanos , Masculino , Prostatectomia , Neoplasias da Próstata/complicações , Neoplasias da Próstata/cirurgia , Esferocitose Hereditária/metabolismo , Esplenomegalia/complicações
20.
J Bone Miner Metab ; 37(2): 378-383, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-29948344

RESUMO

Data from exome sequencing show that a proportion of individuals in whom a genetic disorder is suspected turn out to have not one, but two to four distinct ones. This may require an evolution in our diagnostic attitude towards individuals with complex disorders. We report a patient with splenomegaly, pneumopathy, bone changes and fronto-temporal dementia (FTD). "Sea-blue histiocytes" in his bone marrow pointed to a lysosomal storage disease. Homozygosity for a pathogenic mutation in the SMPD1 gene confirmed Niemann-Pick disease type B (NPD-B). Mild cognitive impairment and abnormal brain FDG PET were consistent with FTD. We initially tried to fit the skeletal and neurologic phenotype into the NPD-B diagnosis. However, additional studies revealed a pathogenic mutation in the SQSTM1 gene. Thus, our patient had two distinct diseases; NPD-B, and Paget's disease of bone with FTD. The subsequent finding of a mutation in SQSTM1 gene ended our struggle to explain the combination of findings by a singular "unifying" diagnosis and allowed us to make specific therapeutic decisions. SQSTM1 mutations have been reported in association with FTD, possibly because of defective autophagy. Bisphosphonates may be beneficial for PDB, but since they are known to inhibit acid sphingomyelinase activity, we refrained from using them in this patient. While the principle of looking for unifying diagnosis remains valid, physicians should consider the possibility of co-existing multiple diagnoses when clinical features are difficult to explain by a single one. Accurate diagnostic work-up can guide genetic counseling but also lead to better medical management.


Assuntos
Osso e Ossos/patologia , Demência Frontotemporal/complicações , Hepatomegalia/complicações , Doença de Niemann-Pick Tipo B/complicações , Osteíte Deformante/complicações , Proteína Sequestossoma-1/genética , Esplenomegalia/complicações , Medula Óssea/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Doença de Niemann-Pick Tipo B/diagnóstico por imagem , Osteíte Deformante/diagnóstico por imagem , Tomografia Computadorizada por Raios X
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