We experienced a case of achondroplasia in 34 month old male infant. Diagnosis was suspected by clinical features and radiologic examination. A review of literature was made briefly.
Achondroplasia* ; Child, Preschool ; Diagnosis ; Humans ; Infant ; Male

A 4-day-old patient with Down syndrome (DS) visited out patient department (OPD) because of jaundice and VSD. Peripheral blood smear showed 21% of myeloblast. After 4 weeks of observation, WBC count was 55,100/mm3 (blast 90%). BM aspirate showed AML (M7) and treatment was started with low dose Ara-C (20 mg/m2 for 21 days). After remission, maintenance therapy was done with low dose Ara-C (16 mg/m2 for 21 days), 6-TG (40 mg/m2 for 21 days) and low dose IL-2 (0.5 106U/m2 for 21 days) alternatively for 2 years. The patient remained in complete remission and VSD was corrected at 9 months of age. This case shows that remission can be achieved with low dose Ara-C and it can be maintained thereafter with low dose Ara-C, 6-TG and IL-2. Low dose IL-2 has the advantage of selectively activating immune cells with high affinity receptors, low treatment related morbidity, good compliance which can be injected at OPD. As the patients with DS have defect in IL-2 secretion, IL-2 may have an beneficial effects on treating AML in DS.
Compliance ; Cytarabine ; Down Syndrome* ; Drug Therapy* ; Granulocyte Precursor Cells ; Humans ; Interleukin-2* ; Jaundice ; Leukemia, Myeloid, Acute*

Henoch-Schonlein purpura (HSP) is a leukocytoclastic vasculitis of small vessels with deposition of IgA, commonly resulting in skin, joint, gastrointestinal, and kidney involvement. HSP is an uncommon disorder in adults and accounts for 0.6% to 2% of adult nephropathy. We report a case of HSP with acute renal failure successfully treated with corticosteroid. In this case, the patient presented with vasculitic purpuric rash on lower extremity, arthralgia in the wrist, abdominal pain, hematochezia, oliguria and azotemia. Abdominal CT showed wall thickening of the small and large bowels. Skin biopsy revealed leukocytoclastic vasculitis. Percutaneous renal biopsy showed no crescent formation, but mesangial IgA and C3 deposits were observed by immunofluorescence. The patient was treated with corticosteroid (1mg/kg per day) and hemodialysis. After treatment, renal function improved and purpuric lesion, arthralgia and abdominal pain disappeared. Thus, when adults present with purpuric rash and rapidly progressive glomerulonephritis (RPGN), HSP should be a diagnostic consideration.
Abdominal Pain ; Acute Kidney Injury ; Adult ; Arthralgia ; Azotemia ; Biopsy ; Exanthema ; Fluorescent Antibody Technique ; Gastrointestinal Hemorrhage ; Glomerulonephritis ; Humans ; Immunoglobulin A ; Joints ; Kidney ; Lower Extremity ; Oliguria ; Purpura ; Purpura, Schoenlein-Henoch ; Renal Dialysis ; Skin ; Vasculitis ; Vasculitis, Leukocytoclastic, Cutaneous ; Wrist

A 73-year-old male was admitted for unhealed wound. Eight months ago, the patient had been operated for excision of soft tissue mass on left distal thigh area in previous hospital and after 4 months from first operation, had been reoperated because of recurrence. The pathologic diagnosis of previous operation was simple cyst. In operating finding, the mass invaded the vastus lateralis fascia and had irregular margin and adhesion. We carried out simple excision with retaining 5 cm of free margin from the mass. The pathologic diagnosis of our hospital was malignant fibrous histiocytoma, and then the patient was performed radiation therapy. In 1 year follow-up, there was no significant finding either increasing mass size or metastasis. We misdiagnosed as simple cyst and then performed simple excision, however finally pathologic diagnosis confirmed as malignant fibrous histiocytoma. It is considered to operate a mass that preoperative proper evaluation and diagnosis are required.
Fascia ; Follow-Up Studies ; Histiocytoma, Benign Fibrous ; Histiocytoma, Malignant Fibrous ; Humans ; Male ; Neoplasm Metastasis ; Quadriceps Muscle ; Recurrence ; Thigh

PURPOSE: The purpose of this study is to present the effective design of N-plasty of the iliotibial band and surgical results of its use as a treatment for refractory external snapping hip. MATERIALS AND METHODS: We evaluated 17 patients (24 cases) with external snapping hip who underwent N-plasty between October 2013 and May 2016 and who were followed up for at least 12 months. All patients were male and the mean age was 20.8 years. The mean duration of symptoms prior to surgical intervention was 28.5 months with an average follow up of 24.5 months. Surgery was defined as being successful when patients could carry out their daily activities and exercise without a clicking sensation or pain 6 months after surgery until their last follow-up. Failure was defined when either a clicking sensation or pain was present. The visual analog scale (VAS) and modified Harris hip score (mHHS) were measured and compared preoperatively and at last follow-up. RESULTS: All patients had complete resolution of pain and snapping. The VAS decreased from 6.77 preoperatively to 0.09 postoperatively and mHHS improved from 69.5 to 97.8 after surgery. CONCLUSION: Modified designed N-plasty is considered to be an excellent treatment method facilitating operation reproducibility with maximum elongation effect of the iliotibial band.
Follow-Up Studies ; Hip* ; Humans ; Male ; Methods ; Sensation ; Visual Analog Scale

PURPOSE: The purpose of this study is to present the effective design of N-plasty of the iliotibial band and surgical results of its use as a treatment for refractory external snapping hip. MATERIALS AND METHODS: We evaluated 17 patients (24 cases) with external snapping hip who underwent N-plasty between October 2013 and May 2016 and who were followed up for at least 12 months. All patients were male and the mean age was 20.8 years. The mean duration of symptoms prior to surgical intervention was 28.5 months with an average follow up of 24.5 months. Surgery was defined as being successful when patients could carry out their daily activities and exercise without a clicking sensation or pain 6 months after surgery until their last follow-up. Failure was defined when either a clicking sensation or pain was present. The visual analog scale (VAS) and modified Harris hip score (mHHS) were measured and compared preoperatively and at last follow-up. RESULTS: All patients had complete resolution of pain and snapping. The VAS decreased from 6.77 preoperatively to 0.09 postoperatively and mHHS improved from 69.5 to 97.8 after surgery. CONCLUSION: Modified designed N-plasty is considered to be an excellent treatment method facilitating operation reproducibility with maximum elongation effect of the iliotibial band.
Follow-Up Studies ; Hip* ; Humans ; Male ; Methods ; Sensation ; Visual Analog Scale

PURPOSE: The aim of this study was to evaluate the usefulness of a method for positioning the femoral stem in neutral position refer to the measured value of "lateral distance" in pre-operative templating and the femoral stem position post-operatively. MATERIALS AND METHODS: A total of 81 hips in 76 patients underwent cementless total hip arthroplasty between March 2008 and April 2012 using the C2 stem (Lima Corporate S.P.A, Udine, Italy). We measured the perpendicular distance from the lateral outermost part of the greater trochanter to the lateral outermost part of the femoral stem (lateral distance, LD) in pre-operative templating. We aimed to insert the femoral stem in neutral position by comparing LD measured intra-operatively. We evaluated the position of the femoral stem on post-operative plain radiography. We compared the result of post-operative re-measurement of the LD with that of pre-operative measurement. RESULTS: In all of the 81 cases, the femoral stem was inserted in neutral position, defined as the femoral stem position within 3degreesvalgus or varus. The average angle of the inserted femoral stem was 1.20degrees(0.003-2.85degrees). LD measured in preoperative templating was 15.6 mm(10-21.5 mm), and it showed no correlation ratio with LD; LD re-measured post-operatively was 15.9 mm(10.3-23.5 mm) (P=0.781). CONCLUSION: Measuring LD pre-operatively and using the value intra-operatively is an effective method for insertion of the femoral stem in neutral position.
Arthroplasty ; Femur ; Hip ; Humans

Human listeriosis is a relatively rare but serious disease with mortality rate 20~40%. Listeria monocytogenes affects patients with decreased cell mediated immunity such as the elderly, transplant recipients, cancer patients, renal failure, diabetes, HIV, pregnant women and unborn child, neonate. We experienced a case of listeriosis in twin pregnant woman at 35th weeks without preterm labor. The pregnant woman present sepsis with fever at first time. Sepsis progress to MODS (multiple organ dysfunction syndrome) and combined endocarditis. We reported it with brief of literatures.
Aged ; Child ; Endocarditis ; Female ; Fever ; HIV ; Humans ; Immunity, Cellular ; Infant, Newborn ; Listeria ; Listeria monocytogenes ; Listeriosis ; Multiple Organ Failure ; Obstetric Labor, Premature ; Pregnancy ; Pregnancy Trimester, Third ; Pregnancy, Twin ; Pregnant Women ; Renal Insufficiency ; Sepsis

Internal impingement syndrome is characterized by the posterior shoulder pain when the arm is abducted and external rotated, and articular partial rotator cuff tear with posterosuperior labral fraying in throwing athletes. Osteochondral lesion of humeral head as an associated lesion is reported in some cases but, not considered to be a main origin of the symptoms. We found the similar features of osteochondral lesion on humeral head in three cases of internal impingement syndrome irrespective of conservative treatment for over three months and report good results obtained from arthroscopic debridement and microfracturing for these lesions with a review of the literatures.
Arm ; Athletes ; Cartilage Diseases ; Debridement ; Humans ; Humeral Head* ; Rotator Cuff ; Shoulder Pain ; Shoulder*

Leptomeningeal metastasis occurs in approximately 1% of patients with non-small cell lung cancer and this is an extremely serious complication. Without treatment, the median survival of patients is 4~6 weeks. The treatment options currently available are limited and achieve only modest results. Gefitinib was recently approved for the treatment of advanced/refractory non-small cell lung cancer. In addition, there have been case reports showing activity of gefitinib against brain metastasis in non-small cell lung cancer patients. However, there is limited data on the ability of gefitinib to cross the blood-brain barrier. We report the case of a patient with leptomeningeal metastasis from adenocarcinoma of the lung that had a dramatic response to gefitinib treatment.
Adenocarcinoma ; Blood-Brain Barrier ; Brain ; Carcinoma, Non-Small-Cell Lung ; Humans ; Lung ; Neoplasm Metastasis ; Quinazolines