Melkersson-Rosenthal syndrome(MRS) is a rare disorder, having a symptom triad of recurrent facial palsy, orofacial swelling and fissured tongue(lingua plicata). This disorder is usually recurrent or progressive, and monosymptomatic or oligosymptomatic forms have been reported to be more common than classic forms. Generally, MRS occurs in young adults at the end of the second decade of life and incidence of the disease in childhood is known to be very low. Although the clinical manifestation of MRS in children is similar to that in adults, early diagnosis and management is essential to avoid long-lasting functional disorders and psychological problems. We experienced MRS in a 13 year old boy with a history of recurrent facial palsy. We report this case with review of related literature.
Adolescent ; Adult ; Child ; Early Diagnosis ; Facial Paralysis ; Humans ; Incidence ; Male ; Melkersson-Rosenthal Syndrome* ; Young Adult

Allergic angitis and granulomatosis is a kind of rare systemic vasculitis, with various manifestations of disease of lung, heart, skin, musculoskeletal system, nervous system and hepatobiliary tract. There was no report of a case with manifestation of pleuritis and pericarditis, while several cases had been reported in Korea. So we here report a case of allergic angitis and granulomatosis with manifestations of pleuritis and pericarditis. The case also showed clinical manifestations of hypereosinophilia, asthma, rhinitis, pulmonary infilterates with eosinophilia and nephritis. Open lung biopsy showed arteritis with heavy infilteration of activated eosinophil in lung, pleura and pericardium. The involvement of heart might cause critical complication leading death. The patients who are supposed as allergic angitis and granulomatosis should be examined for the involvement of heart.
Arteritis ; Asthma ; Biopsy ; Eosinophilia ; Eosinophils ; Heart ; Humans ; Korea ; Lung ; Musculoskeletal System ; Nephritis ; Nervous System ; Pericarditis* ; Pericardium ; Pleura ; Pleurisy* ; Rhinitis ; Skin ; Systemic Vasculitis

OBJECTIVE: Recently some reports suggested substance P and CGRP might be important factors for inflammation and hyperalgesia. This study was performed to see whether substance P or CGRP containing nerve fibers might be changed by mustard oil-induced inflammation. METHODS: After injection of mustard oil(5%) into uterine lumen, the uteri were removed and examined with immunohistochemical methods for substance P and CGRP. RESULTS: In the normal uterus, most of the substance P- or CGRP-immunoreactive nerve fibers were observed along the vascular structure and some in the myometrium, only few in the endometrium. Mustard oil did not changed this pattern of nerve fiber distribution but after 48 hrs, the amount of substance P or CGRP immunoreactive nerve fibers were greatly reduced compared with the normal uterus. It is not clear whether the decrease of substance P and CGRP immunoreactive fibers in the uterus was resulted from the depletion of the neuropeptides in the nerve fibers or the retraction of nerve fibers. CONCLUSIONS: These results suggest that the inflammation should cause the change of nerve fibers included in the nociception. This change may attribute the generation of inflammation and inflammatory hyperalgesia.
Animals ; Endometrium ; Female ; Hyperalgesia ; Inflammation* ; Mice ; Mustard Plant ; Myometrium ; Nerve Fibers ; Neuropeptides ; Nociception ; Rats* ; Substance P* ; Uterus*

Granular cell tumors (GCTs) have been reported in various tissues, especially the skin and subcutaneous soft tissue of the head and neck. We report a 60-year-old man who presented with intermittent headache and dizziness for 3 months, but no other neurological symptoms. Magnetic resonance imaging (MRI) showed the presence of a mass in the pituitary stalk, and contrast-enhanced MRI showed nodular enhancement in this region. The lesion was completely excised microscopically via a frontotemporal (pterional) approach. On pathological examination, a final diagnosis of a typical GCT was made.
Diagnosis ; Dizziness ; Granular Cell Tumor* ; Head ; Headache ; Humans ; Magnetic Resonance Imaging ; Middle Aged ; Neck ; Pituitary Gland* ; Pituitary Neoplasms ; Skin

Natural killer (NK) cell neoplasms are a group of rare but highly malignant tumors. We report here one case of NK cell leukemia. A 54-yr-old woman presented with a 2-month history of progressive left neck mass. Based on the positive result of tissue PCR for Mycobacterium tuberculosis, she was at first diagnosed with tuberculous lymphadenopathy. After two weeks, she developed generalized lymphadenopathy, hepatosplenomegaly, fever and anemia. Subsequent evaluation was performed including bone marrow aspiration and biopsy. Peripheral blood smear showed leukoerythroblastic features with 31% blasts. Bone marrow was packed with agranular blastoid cells, which were periodic acid-Schiff (PAS) positive and myeloperoxidase (MPO) negative. Immunophenotyping showed that these cells were positive for CD45 and HLA-DR, whereas negative for CD3, CD5, CD7, CD10, CD13, CD14, CD19, CD20, CD22, CD33, CD34, and CD61. Because of the absence of the markers of T-cell, B-cell, and myeloid lineage-specific antigens, we added CD16/56 for the immunophenotyping and the blasts were positive (94%). The tumor cells of biopsied lymph node were only positive for CD56, consistent with NK cell lymphoma. Epstein-Barr virus (EBV) was not detected by RNA in situ hybridization. Culture for M. tuberculosis was negative. Thus this patient was diagnosed with blastic NK cell lymphoma/leukemia involving bone marrow and lymph node.
Antigens, CD45/metabolism ; Bone Marrow/pathology ; Female ; HLA-DR Antigens/metabolism ; Humans ; Killer Cells, Natural/immunology/*pathology ; Leukemia/*diagnosis/immunology/pathology ; Middle Aged ; Tuberculosis, Lymph Node/diagnosis

Background/Aims: Endoscopic ultrasound-guided fine-needle biopsy (EUS-FNB) is integral to the diagnosis of gastrointestinal (GI) subepithelial tumors (SETs). The impact of different EUS-FNB tissue sampling techniques on specimen adequacy and diagnostic accuracy in SETs has not been fully evaluated. This study aimed to compare the diagnostic outcomes of slow-pull (SP) and standard suction (SS) in patients with GI SETs. Methods: In this retrospective comparative study, 54 patients were enrolled. Medical records were reviewed for location and size of the target lesion, FNB needle type/size, technical order, specimen adequacy, diagnostic yield, and adverse events. The acquisition rate of adequate specimens and diagnostic accuracy were compared according to EUS-FNB techniques. Results: The mean lesion size was 42.6±36.4 mm, and most patients were diagnosed with GI stromal tumor (75.9%). The overall diagnostic accuracies of the SP and SS techniques were 83.3% and 81.5%, respectively (p=0.800). The rates of obtaining adequate core tissue were 79.6% and 75.9%, respectively (p=0.799). No significant clinical factors affected the rate of obtaining adequate core tissue, including lesion location and size, FNB needle size, and final diagnosis. Conclusions SP and SS had comparable diagnostic accuracies and adequate core tissue acquisition for GI SETs via EUS-FNB.

BACKGROUND/AIMS: Determining the cause of suspected biliary stricture is often challenging in clinical practice. We aimed to compare the diagnostic yields of endoscopic ultrasound-guided tissue sampling (EUS-TS) and endoscopic retrograde cholangiopancreatography-guided tissue sampling (ERCP-TS) in patients with suspected biliary stricture at different primary lesions. METHODS: We enrolled patients who underwent same-session EUS- and ERCP-TS for the evaluation of suspected biliary stricture. Forceps biopsy and/or brush cytology of intraductal lesions and fine-needle aspiration for solid mass lesions were performed during ERCP and EUS, respectively. RESULTS: One hundred and twenty-five patients treated at our institution between January 2011 and September 2016, were initially considered for the study. However, 32 patients were excluded due to loss of follow-up (n=8) and ERCP-TS on the pancreatic duct (n=20) or periampullary lesions (n=4). Of the 93 patients included, 86 had a malignant tumor including cholangiocarcinoma (n=39), pancreatic cancer (n=37), and other malignancies (n=10). Seven patients had benign lesions. EUS-TS had higher rate of overall diagnostic accuracy than ERCP-TS (82.8% vs. 60.2%, p=0.001), and this was especially true for patients with a pancreatic lesion (84.4% vs. 51.1%, p=0.003). CONCLUSIONS: EUS-TS was found to be superior to ERCP-TS for evaluating suspected biliary strictures, especially those caused by pancreatic lesions.
Biopsy ; Biopsy, Fine-Needle ; Cholangiocarcinoma ; Cholangiopancreatography, Endoscopic Retrograde ; Constriction, Pathologic ; Diagnosis ; Endosonography ; Follow-Up Studies ; Humans ; Pancreatic Ducts ; Pancreatic Neoplasms ; Surgical Instruments

Even though it is a rare complication to have hepatic cyst infection in adult polycystic kidney disease, the patient is expected to be complicated with sepsis or acute cholangitis and die if not treated properly. The typical clinical manifestations of hepatic cyst infection are fever and right upper quadrant abdominal pain. Liver enzyme and serum bilirubin levels may be elevated. The clinical outcome of hepatic cyst infection is better when antibiotic and drainage are performed simultaneously than only antibiotic is used. We experienced a case of infected hepatic cyst from a 58-year-old male patient receiving hemodialysis with autosomal dominant polycystic kidney disease. Due to his persistent fever and leukocytosis during intravenous antibiotic therapy, we performed percutaneous drainage of hepatic cyst. Because the drainage fluid presented leukocytosis and deep yellow color, we diagnosed the case as a hepatic cyst infection. We applied both percutaneous drainage of infected liver cyst and antibiotic and we observed clinical improvement.
Abdominal Pain ; Adult* ; Bilirubin ; Cholangitis ; Drainage* ; Fever ; Humans ; Leukocytosis ; Liver ; Male ; Middle Aged ; Polycystic Kidney Diseases ; Polycystic Kidney, Autosomal Dominant* ; Renal Dialysis ; Sepsis

PURPOSE: Primary focal and segmental glomerulosclerosis(FSGS) is a clinicopathological entity defined by the segmental sclerosis involving glomeruli in a focal distribution with poor prognosis. Approximately 50% to 70% of adults with FSGS manifests nephrotic syndrome, and the others show mild to moderate proteinuria. In this study, we intended to figure out the clinical course, treatments and possible prognostic factors of FSGS in adults. METHODS: We retrospectively reviewed the clinical characteristics of 54 adult patients diagnosed as primary focal segmental glomerulo-sclerosis(FSGS) by renal biopsy in Chungnam National University Hospital. from Dec. 1989 to Jan. 2000. RESULTS: Mean age of 54 patients was 36.8+/-24.6 (15-75) years. Male to female ratio of them was 1.08 : 1. Thirty one patients(57%) of them were manifested as nephrotic syndrome and their male to female ratio was 1.4 : 1. In all of 54 primary FSGS patients, generalized edema(59%), microscopic hematuria (44%), hypertension(39%) and azotemia(15%) were noted as clinical manifestations at the time of diagnosis. Median duration of follow-up was 48.7(6-123) months. Except generalized edema, serum cholesterol and albumin level, and 24 hour urine protein excretion, there was no significant difference in hypertension, hematuria, serum creatinine, duration of follow- up and progression to chronic renal failure between 31 nephrotic and 23 non-nephrotic patients. Sixteen (52%) of 31 nephrotic patients with primary FSGS showed complete remission. Seven(22%) of them showed partial remission and eight(26%) of them showed no response after 8 weeks of first steroid treatment. No response group of primary FSGS nephrotic patients progressed significantly more to chronic renal failure than remission group including complete or partial remission patients. No response groups showed significantly higher serum creatinine, lower creatinine clearance, higher degree of glomerular global sclerosis and interstitial fibrosis than remission group at the time of diagnosis. Eleven patients(8 nephrotic and 3 non-nephrotic patients) of 54 primary FSGS patients progressed to CRF during follow-up period, and they showed significantly lower creatinine clearance and higher degree of global glomerular sclerosis at the time of diagnosis than normal renal function maintaining group. CONCLUSION: It is speculated that initial renal function and degree of global glomerular sclerosis at the time of diagnosis in primary FSGS patients, and additively response to initial steroid therapy and the degree of interstitial fibrosis at the time of diagnosis in nephrotic primary FSGS patients are thought to be significant long-term prognostic factors.
Adult* ; Biopsy ; Cholesterol ; Chungcheongnam-do ; Creatinine ; Diagnosis ; Edema ; Female ; Fibrosis ; Follow-Up Studies ; Hematuria ; Humans ; Hypertension ; Kidney Failure, Chronic ; Male ; Nephrotic Syndrome ; Prognosis ; Proteinuria ; Retrospective Studies ; Sclerosis

Comamonas testosteroni has rarely been implicated as a human pathogen. We here present a case of peritonitis due to this organism in a patient on continuous ambulatory peritoneal dialysis (CAPD). A 32-year-old woman was admitted with abdominal pain and cloudy peritoneal effluent. Empirical intraperitoneal (IP) treatment with cefazolin and ceftazidime was started. The culture was positive for C. testosteroni and antibiotic was changed to ceftazidime IP. Four days after the ceftazidime treatment, the patient became asymptomatic. The follow-up culture from peritoneal effluent at 7th day was negative. This treatment was maintained for 21 days. After that, culture negative peritonitis occurred twice for 2 months in this patient, so CAPD catheter had to be removed. This is the first reported case of CAPD peritonitis caused by C. testosteroni. It is important for clinicians to recognize that CAPD peritonitis is caused by this organism which has been largely overlooked as a potential pathogen.
Abdominal Pain ; Adult ; Catheters ; Cefazolin ; Ceftazidime ; Comamonas ; Comamonas testosteroni ; Female ; Follow-Up Studies ; Humans ; Peritoneal Dialysis ; Peritoneal Dialysis, Continuous Ambulatory ; Peritonitis