• Clinical and Experimental Pediatrics
• /
• v.54 no.6
• /
• pp.234-240
• /
• 2011

Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease of the central nervous system (CNS) that typically presents as a monophasic disorder associated with multifocal neurologic symptoms and encephalopathy. ADEM is considered an autoimmune disorder that is triggered by an environmental stimulus in genetically susceptible individuals. The diagnosis of ADEM is based on clinical and radiological features. Most children with ADEM initially present with fever, meningeal signs, and acute encephalopathy. The level of consciousness ranges from lethargy to frank coma. Deep and subcortical white-matter lesions and gray-matter lesions such as thalami and basal ganglia on magnetic resonance imaging (MRI) are associated with ADEM. In a child who presents with signs of encephalitis, bacterial and viral meningitis or encephalitis must be ruled out. Sequential MRI is required to confirm the diagnosis of ADEM, as relapses with the appearance of new lesions on MRI may suggest either multiphasic ADEM or multiple sclerosis (MS). Pediatric MS, defined as onset of MS before the age of 16, is being increasingly recognized. MS is characterized by recurrent episodes of demyelination in the CNS separated in space and time. The McDonald criteria for diagnosis of MS include evidence from MRI and allow the clinician to make a diagnosis of clinically definite MS on the basis of the interval preceding the development of new white matter lesions, even in the absence of new clinical findings. The most important alternative diagnosis to MS is ADEM. At the initial presentation, the 2 disorders cannot be distinguished with certainty. Therefore, prolonged follow-up is needed to establish a diagnosis.

• Journal of Yeungnam Medical Science
• /
• v.39 no.2
• /
• pp.153-160
• /
• 2022

Multicentric Castleman disease (MCD) is an uncommon systemic lymphoproliferative disorder that may cause multiple organ damage. Castleman disease-associated diffuse parenchymal lung disease (DPLD) has not been well studied. A 32-year-old man was referred to our hospital for progressive generalized weakness, light-headedness, and dyspnea on exertion for more than one year. Laboratory evaluations showed profound anemia, an elevated erythrocyte sedimentation rate, and an increased C-reactive protein level with polyclonal hypergammaglobulinemia. Chest radiography, computed tomography (CT), and positron emission tomography-CT scan demonstrated diffuse lung infiltration with multiple cystic lesions and multiple lymphadenopathy. In addition to these clinical laboratory findings, bone marrow, lung, and lymph node biopsies confirmed the diagnosis of idiopathic MCD (iMCD). Siltuximab, an interleukin-6 inhibitor, and glucocorticoid therapy were initiated. The patient has been tolerating the treatment well and had no disease progression or any complications in 4 years. Herein, we report this case of human herpesvirus-8-negative iMCD-associated DPLD accompanied by multiple cystic lesions, multiple lymphadenopathy, and polyclonal hypergammaglobulinemia with elevated immunoglobulin G (IgG) and IgG4 levels. We recommend a close evaluation of MCD in cases of DPLD with hypergammaglobulinemia.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• v.19 no.4
• /
• pp.276-280
• /
• 2016

Dieulafoy lesions, vascular anomalies typically found along the gastrointestinal tract, have been viewed as rare and obscure causes of sudden intestinal bleeding, especially in pediatric patients. Since their discovery in the late 19th century, the reported incidence has increased. This is due to an increased awareness of, and knowledge about, their presentation and to advanced endoscopic diagnosis and therapy. Our patient was a three-year-old male, without a complex medical history. He presented to the emergency department with acute hematemesis with blood clots and acute anemia requiring blood transfusion. Endoscopy revealed four isolated Dieulafoy lesions along the lesser curvature of the stomach, which were treated with an epinephrine injection. The Dieulafoy lesion, although thought to be rare, should be considered when investigating an acute gastrointestinal bleed. These lesions have been successfully treated endoscopically. Appropriate anticipation and preparation for diagnosis and therapy can lead to optimal outcomes for the pediatric patient.

• Imaging Science in Dentistry
• /
• v.52 no.4
• /
• pp.421-427
• /
• 2022

McCune-Albright syndrome (MAS) is a rare multisystem disorder characterized by a clinical triad of polyostotic fibrous dysplasia (FD), skin pigmentation, and hyperfunctioning endocrinopathies. A 42-year-old man visited our medical hospital for the treatment of intermittent headaches and was diagnosed with MAS with acromegaly. This patient showed various clinical features of MAS, including pituitary adenoma, polyostotic FD, and hypogonadotropic hypogonadism. The FD lesions showed characteristic radiographic features, such as widespread, sclerotic bony lesions in the cranial bones, mixed radiolucent-radiopaque multilocular lesions in the mandible, and radiolucent lesions in the axial and appendicular skeleton. Over the years, the patient had been hospitalized multiple times due to accidental bony fractures associated with the fragile bony state of FD. This report presents a retrospective description of a case of MAS, with a review of the relevant literature.

• Pediatric Infection and Vaccine
• /
• v.27 no.2
• /
• pp.134-139
• /
• 2020

Staphylococcus epidermidis is a part of the normal skin flora of humans. However, it can cause serious infections in people exposed to foreign bodies or in immunocompromised patients. A 13-year-old boy was hospitalized with fever and myalgia. Painful nodular lesions were detected on the scalp, arms, and legs. Pancytopenia and blasts were present in the peripheral blood. He was diagnosed with acute myeloid leukemia. Magnetic resonance imaging of the whole body showed multiple peripheral rim-enhancing, cyst-like lesions. Ultrasonography showed echogenic nodules inside the cystic lesions in the intramuscular space of the arms and legs. Therefore, cysticercosis was strongly suggested initially. However, an abscess was confirmed on sono-guided biopsy and S. epidermidis was isolated from a microbial culture of the tissue. We report a case of multiple disseminated lesions caused by S. epidermidis in a leukemia patient, initially mistaken for cysticercosis.

• Asian Pacific Journal of Cancer Prevention
• /
• v.16 no.18
• /
• pp.8293-8298
• /
• 2016

Background: Urinary stones are known predisposing factors for upper urinary tract carcinoma (UUTC) which are commonly detected at advanced stage with poor outcome because of rarity and lack of specific criteria for early detection. Aims and objectives: The main aim was to evaluate the impact of age, gender andstone characteristics on risk of developing UUTC in patients with chronic nephrolithiasis. We also discuss the role of aberrant angiogenesis (AA) and immunohistochemical expression of p53, p16INK4a, CK20 and Ki-67 in diagnosis of pelvicalyceal neoplastic (NL) and pre-neoplastic lesions (PNL) in these patients. Materials and Methods: Retrospective analysis of pelvicalyceal urothelial lesions from 88 nephrectomy specimens were carried out in a tertiary care centre from June 2012 to December 2014. Immunohistochemistry (IHC) was performed on 37 selected cases. Computed image analysis was performed to analyse aberrant angiogenesis. Results: All UUTC (5.7%) and metaplastic lesions were found to be associated with stones. Some 60% were pure squamous cell carcinoma and 40% were transitional cell carcinoma. Odd ratios for developing NL and PNL lesions in presence of renal stone, impacted stones, multiple and large stag horn stones were 9.39 (95% CI 1.15-76.39, p value 0.05), 6.28 (95% CI 1.59-24.85, p value 0.000) and 7.4 (95% CI, 2.29-23.94, p value 0.001) respectively. When patient age was ${\geq}55$, the odds ratio for developing NL was 3.43 (95% CI 1.19-9.88, p value 0.019). IHC analysis showed that mean Ki-67 indices were $3.15{\pm}3.63%$ for non-neoplastic lesions, $10.0{\pm}9.45%$ for PNL and $28.0{\pm}18.4%$ for NL. Sensitivity and specificity of CK20, p53, p16INK4a, AA were 76% and 95.9%; 100% and 27.5%; 100% and 26.5%; 92.3 % and 78.8% respectively. Conclusions: Age ${\geq}55years$, large stag horn stones, multiple stones and impacted stones are found to be associated with increased risk of NL and PNL in UUT. For flat lesions, a panel of markers, Ki 67 index >10 and presence of aberrant angiogenesis were more useful than individual markers.

• Investigative Magnetic Resonance Imaging
• /
• v.20 no.1
• /
• pp.66-70
• /
• 2016

Introduction: Distant metastases of mucoepidermoid carcinoma (MEC) are reported with the most common sites being the soft tissue of skin, lung, liver, and bone. We report here a very rare case of MEC with multiple metastases to the kidney, adrenal gland, skull and gluteus maximus muscle. Case report: A 63-year-old male patient presented with left-sided headache. Radiologic evaluations including CT and MRI showed ill-defined soft tissue lesion involving the left infratemporal fossa and left sphenoid sinus, and multiple enlarged lymph nodes in neck and mediastinum. PET-CT demonstrated multiple hypermetabolic lesions in and around the left kidney, left adrenal gland, right ischium, right gluteus maximus and skull base. These lesions were confirmed as MEC with multiple metastases through biopsy. Discussion: Only one case of metastasis to the skull has been previously reported, and moreover, there has not been a case of metastatic MEC to the kidney, adrenal gland and gluteus maximus muscle so far in the medical literature. It is important to acknowledge the possibility of every unusual MEC metastases, since the presence of metastasis has statistically significant influence on the survival of MEC.

• Journal of the Korean Orthopaedic Association
• /
• v.55 no.4
• /
• pp.338-342
• /
• 2020

Periosteal chondroma is a rare, slow-growing, benign cartilaginous tumor that develops between the periosteum and cortex, but there are no reports of multiple periosteal chondroma of the toes. A 19-year-old male presented with a palpable mass of the right fourth toe with tenderness for one year. A radiology examination revealed multiple, radio-lucent lesions with mild cortical irregularity. The magnetic resonance imaging findings were chondrogenic tumors with multiple, well-defined T1-low and T2-high signal enhanced lesions involved in the fourth proximal, middle, and distal phalanges. The tumors were removed by a surgical resection and curettage. Histologically, the tumors were proven to be periosteal chondroma.

• Journal of Chest Surgery
• /
• v.13 no.4
• /
• pp.507-511
• /
• 1980

Most cases of plasma-cell granuloma occur in adult skeleton system. They usually present as a single lesion, but multiple lesions are not uncommon. Roentgenographically, the lesions are lytic, sclerotic or mixed, but in the majority there is some degree of sclerosis. No specific etiologic agent is known, but it is postulated that the reaction may be associated with a variety of organisms of low-grade virulence. Certain organisms have long been known to produce predominantly plasmacytic infiltrate, notably spirochetes, mycobacteria and some exotic viruses. It is most important, however, that this entity, plasma-cell granuloma, be distinguished from multiple myeloma so those potentially life-threatening chemotherapeutic agents are not prescribed. Histologically, aggregates of chronic inflammatory cells are intermingled with the meets of plasma cells, and note the markedly thickened bone trabeculae in surrounding bone. We experienced a case of plasma-cell granuloma of right 7th. rib, occurring in 32 year old male and reported it with review of literatures.

• Maxillofacial Plastic and Reconstructive Surgery
• /
• v.35 no.6
• /
• pp.421-426
• /
• 2013

Gardner syndrome, an autosomal dominant inherited condition, is a subtype of familial adenomatous polyposis. It causes lesions in bones, skin, and teeth, as well as multiple gastrointestinal polyps, which, if left untreated, become malignant. Because patients with colorectal cancer have a low survival rate, early diagnosis and treatment of Gardner syndrome is critical. Therefore, the characteristic lesions of Gardner disease that appear on the face, jaws, and oral cavity must be understood; these can be evaluated by oral and maxillofacial clinicians. This report describes a case that was diagnosed and treated earlier with the help of a routine oral and maxillofacial examination and has had a seemingly good prognosis so far.