PURPOSE: Fat embolism syndrome (FES) is systemic manifestation of fat emboli in the circulation seen mostly after long bone fractures. FES is considered a lethal complication of trauma. There are various case reports and series describing FES. Here we describe the clinical characteristics, management in ICU and outcome of these patients in level I trauma center in a span of 6 months. METHODS: In this prospective study, analysis of all the patients with FES admitted in our polytrauma intensive care unit (ICU) of level I trauma center over a period of 6 months (from August 2017 to January 2018) was done. Demographic data, clinical features, management in ICU and outcome were analyzed. RESULTS: We admitted 10 cases of FES. The mean age of patients was 31.2 years. The mean duration from time of injury to onset of symptoms was 56 h. All patients presented with hypoxemia and petechiae but central nervous system symptoms were present in 70% of patients. The mean duration of mechanical ventilation was 11.7 days and the mean length of ICU stay was 14.7 days. There was excellent recovery among patients with no neurological deficit. CONCLUSION FES is considered a lethal complication of trauma but timely management can result in favorable outcome. FES can occur even after fixation of the fracture. Hypoxia is the most common and earliest feature of FES followed by CNS manifestations. Any patient presenting with such symptoms should raise the suspicion of FES and mandate early ICU referral.
Adolescent ; Adult ; Central Nervous System Diseases ; etiology ; Early Diagnosis ; Embolism, Fat ; diagnosis ; etiology ; prevention & control ; Fractures, Bone ; complications ; Humans ; Hypoxia ; etiology ; Intensive Care Units ; statistics & numerical data ; Length of Stay ; statistics & numerical data ; Male ; Patient Outcome Assessment ; Time Factors ; Trauma Centers ; statistics & numerical data ; Young Adult

Any medical diagnosis should take a multimodal approach, especially those involving tumour-like conditions, as entities that mimic neoplasms have overlapping features and may present detrimental outcomes if they are underdiagnosed. These case reports present diagnostic pitfalls resulting from overdependence on a single diagnostic parameter for three musculoskeletal neoplasm mimics: brown tumour (BT) that was mistaken for giant cell tumour (GCT), methicillin-resistant Staphylococcus aureus osteomyelitis mistaken for osteosarcoma and a pseudoaneurysm mistaken for a soft tissue sarcoma. Literature reviews revealed five reports of BT simulating GCT, four reports of osteomyelitis mimicking osteosarcoma and five reports of a pseudoaneurysm imitating a soft tissue sarcoma. Our findings highlight the therapeutic dilemmas that arise with musculoskeletal mimics, as well as the importance of thorough investigation to distinguish mimickers from true neoplasms.
Adult ; Aneurysm, False ; diagnosis ; Biopsy ; Bone Diseases ; diagnosis ; Bone Diseases, Metabolic ; diagnosis ; Bone Neoplasms ; diagnosis ; Cell Proliferation ; Diagnosis, Differential ; Diagnostic Errors ; prevention & control ; Female ; Giant Cell Tumors ; diagnosis ; Humans ; Hyperparathyroidism ; complications ; Leukocytosis ; diagnosis ; Male ; Methicillin-Resistant Staphylococcus aureus ; Middle Aged ; Neoplasms ; diagnosis ; microbiology ; Osteomyelitis ; diagnosis ; microbiology ; Osteosarcoma ; diagnosis ; Sarcoma ; diagnosis ; Soft Tissue Neoplasms ; diagnosis ; Tibia ; pathology

Vitamin D deficiency is common and may contribute to osteopenia, osteoporosis and falls risk in the elderly. Screening for vitamin D deficiency is important in high-risk patients, especially for patients who suffered minimal trauma fractures. Vitamin D deficiency should be treated according to the severity of the deficiency. In high-risk adults, follow-up serum 25-hydroxyvitamin D concentration should be measured 3-4 months after initiating maintenance therapy to confirm that the target level has been achieved. All patients should maintain a calcium intake of at least 1,000 mg for women aged ≤ 50 years and men ≤ 70 years, and 1,300 mg for women > 50 years and men > 70 years.
Aged ; Bone Density ; Bone Diseases, Metabolic ; prevention & control ; Calcium, Dietary ; therapeutic use ; Cholecalciferol ; administration & dosage ; Female ; Hip Fractures ; complications ; epidemiology ; Humans ; Male ; Middle Aged ; Osteoporosis ; prevention & control ; Practice Guidelines as Topic ; Prevalence ; Primary Health Care ; methods ; Risk Factors ; Vitamin D ; analogs & derivatives ; blood ; Vitamin D Deficiency ; diagnosis ; epidemiology

Bone Diseases, Metabolic ; diagnosis ; etiology ; therapy ; Chronic Disease ; Humans ; Liver Diseases ; complications ; diagnosis ; therapy

Congenital adrenal insufficiency is caused by specific genetic mutations. Early suspicion and definite diagnosis are crucial because the disease can precipitate a life-threatening hypovolemic shock without prompt treatment. This study was designed to understand the clinical manifestations including growth patterns and to find the usefulness of ACTH stimulation test. Sixteen patients with confirmed genotyping were subdivided into three groups according to the genetic study results: congenital adrenal hyperplasia due to 21-hydroxylase deficiency (CAH, n=11), congenital lipoid adrenal hyperplasia (n=3) and X-linked adrenal hypoplasia congenita (n=2). Bone age advancement was prominent in patients with CAH especially after 60 months of chronologic age (n=6, 67%). They were diagnosed in older ages in group with bone age advancement (P<0.05). Comorbid conditions such as obesity, mental retardation, and central precocious puberty were also prominent in this group. In conclusion, this study showed the importance of understanding the clinical symptoms as well as genetic analysis for early diagnosis and management of congenital adrenal insufficiency. ACTH stimulation test played an important role to support the diagnosis and serum 17-hydroxyprogesterone levels were significantly elevated in all of the CAH patients. The test will be important for monitoring growth and puberty during follow up of patients with congenital adrenal insufficiency.
17-alpha-Hydroxyprogesterone/blood ; 46, XY Disorders of Sex Development/drug therapy/*genetics ; Adolescent ; Adrenal Hyperplasia, Congenital/drug therapy/*genetics ; Adrenal Insufficiency/*congenital/diagnosis/drug therapy/genetics ; Adrenocorticotropic Hormone/*metabolism ; Bone Development/genetics ; Child ; Child, Preschool ; DAX-1 Orphan Nuclear Receptor/genetics ; Female ; Genetic Diseases, X-Linked/drug therapy/*genetics ; Genotype ; Glucocorticoids/therapeutic use ; Humans ; Intellectual Disability/complications ; Male ; Mineralocorticoids/therapeutic use ; Obesity/complications ; Phosphoproteins/genetics ; Puberty, Precocious/complications ; Retrospective Studies ; Steroid 21-Hydroxylase/genetics

Behcet's disease is a multisystemic inflammatory disease characterized with recurrent oral ulcer, genital ulcer, and multiple organ involvement. Aplastic anemia is one of the rarest complications of Behcet's disease. There were only several reports about Behcet's disease associated myelodysplatic syndrome worldwide. Moreover, aplastic anemia in intestinal Behcet's disease was rarely reported. Here, we present a case of aplastic anemia with trisomy 8 and trisomy 9 in intestinal Behcet's disease and a review of the literatures. To the authors' knowledge, this is the first case ever reported in Korea.
Adult ; Anemia, Aplastic/complications/*diagnosis ; Behcet Syndrome/complications/*diagnosis/genetics ; Bone Marrow/pathology ; Chromosomes, Human, Pair 8 ; Chromosomes, Human, Pair 9 ; Female ; Humans ; Intestinal Diseases/complications/*diagnosis/genetics ; Karyotyping ; Tomography, X-Ray Computed ; *Trisomy

Child ; Edema ; diagnostic imaging ; etiology ; Emphysema ; diagnostic imaging ; etiology ; Ethmoid Bone ; diagnostic imaging ; injuries ; Eyelid Diseases ; diagnostic imaging ; etiology ; Humans ; Male ; Orbit ; diagnostic imaging ; Orbital Diseases ; diagnosis ; diagnostic imaging ; pathology ; Radiography ; Skull ; diagnostic imaging ; pathology ; Skull Fractures ; complications ; diagnostic imaging

We report a case of pure red cell aplasia (PRCA), which was initially suspected as a result of bone marrow involvement of diffuse large B cell lymphoma. Persistent anemia without an obvious cause was observed in a 47-yr-old man diagnosed with relapsed diffuse large B cell lymphoma. The bone marrow study showed only erythroid hypoplasia without the evidence of bone marrow involvement with lymphoma cells, thus PRCA was suggested. However, parvovirus infection was excluded as a potential cause of PRCA because of negative IgM anti-parvovirus B19 antibody and negative parvovirus PCR in the serum. Latent Epstein-Barr virus (EBV) infection of bone marrow was suggested by in situ hybridization with EBV-encoded small RNA (EBER) that showed a strong positive expression in bone marrow cells. Thus, PRCA was thought to be associated with latent EBV infection in bone marrow cells. Although the finding of unexplained anemia is a possible predictor of bone marrow involvement with lymphoma cells, PRCA as a result of a viral infection including EBV should be considered in lymphoma patients. This is the first report of the occurrence of PRCA associated with latent EBV infection in a patient with non-Hodgkin's lymphoma.
Adult ; Bone Marrow/pathology ; Bone Marrow Diseases/*complications/diagnosis/pathology ; Bone Marrow Neoplasms/pathology ; Diagnosis, Differential ; Epstein-Barr Virus Infections/*complications/diagnosis ; Humans ; Lymphoma, Large B-Cell, Diffuse/*complications/pathology ; Male ; Red-Cell Aplasia, Pure/diagnosis/*etiology

BACKGROUND: Fibrin-ring granuloma (FRG), which can be found in bone marrow or the liver, is a subtype of epithelioid granuloma characterized by a central fat vacuole and annular peripheral fibrinoid materials. FRG has been proven to be associated with many etiologies such as several infectious organisms (Coxiella burnett; Epstein-Barr Virus, EBV; cytomegalovirus, CMV; and hepatitis A virus), allopurinol induced hepatitis, Hodgkin's lymphoma, and peripheral T-cell lymphoma. METHODS: We retrospectively reviewed 24 patients diagnosed with FRG by bone marrow biopsy at a single institute between 1995 and 2004. We reviewed clinical symptoms and laboratory findings of the patients, classified them by etiology, and compared prognosis of each group. RESULTS: The most common cause of FRG was acute or chronic EBV infection. Chronic or acute EBV infection was associated with 41.4% of patients (10/24). Of the remaining patients, 33.3% (8/24) were leukemia or lymphoma patients after chemotherapy, 4.2% (1/24) was a patient with hepatic failure, and 20.8% (5/24) were diagnosed as fever of unknown origin. The most common symptom and clinical finding were fever and cytopenia. EBV-associated group comprised chronic active EBV infection, EBV-associated hemophagocytic histiocytosis, acute EBV infection, EBV-associated lymphoproliferative disease, and Langerhans' cell histiocytosis. The EBV-associated group showed a lower survival probability compared with the non-EBV group (P<0.05). CONCLUSIONS: Patients with bone marrow fibrin ring granuloma accompanied by fever require an active workup to find out the cause of infectious agents including EBV infection particularly due to their poor prognosis.
Adolescent ; Adult ; Aged ; Bone Marrow Diseases/diagnosis/*etiology/pathology ; Child ; Child, Preschool ; Epstein-Barr Virus Infections/*complications/diagnosis ; Female ; Fibrin/analysis ; Granuloma/diagnosis/*etiology/pathology ; Herpesvirus 4, Human/immunology/isolation & purification ; Humans ; In Situ Hybridization ; Male ; Middle Aged ; Polymerase Chain Reaction ; Prognosis ; Q Fever/diagnosis ; Retrospective Studies ; Survival Rate

Aspergillosis is a rare cause of spondylitis. Moreover, early diagnosis by MR imaging and adequate treatment can prevent the serious complications of fungal infection. To our knowledge, the MR findings of multilevel aspergillus spondylitis in the cervico-thoraco-lumbar spine have not been previously described. Here, we report the MR findings of aspergillus spondylitis involving the cervical, thoracic, and lumbar spine in a liver transplant recipient.
Aspergillosis/*diagnosis ; Aspergillus/isolation & purification ; Bone Transplantation ; Cervical Vertebrae/microbiology/pathology/surgery ; Humans ; *Immunocompromised Host ; Liver Transplantation ; Lumbar Vertebrae/microbiology/pathology/surgery ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Postoperative Complications/diagnosis/microbiology/surgery ; Rare Diseases ; Spondylitis/*microbiology/surgery ; Thoracic Vertebrae/microbiology/pathology/surgery