Purpose: Acute promyelocytic leukemia (APL) is a rare disease in children and there are some different characteristics between children and adult. We aimed to evaluate incidence, clinical characteristics and treatment outcomes of pediatric APL in Korea. Materials and Methods: Seventy-nine pediatric APL patients diagnosed from January 2009 to December 2016 in 16 tertiary medical centers in Korea were reviewed retrospectively. Results: Of 801 acute myeloid leukemia children, 79 (9.9%) were diagnosed with APL. The median age at diagnosis was 10.6 years (range, 1.3 to 18.0). Male and female ratio was 1:0.93. Thirty patients (38.0%) had white blood cell (WBC) count greater than 10×109/L at diagnosis. All patients received induction therapy consisting of all-trans retinoic acid and chemotherapy. Five patients (6.6%) died during induction chemotherapy and 66 patients (86.8%) achieved complete remission (CR) after induction chemotherapy. The causes of death were three intracranial hemorrhage, one cerebral infarction, and one sepsis. Five patients (7.1%) suffered a relapse during or after maintenance chemotherapy. The estimated 4-year event-free survival and overall survival (OS) rates were 82.1%±4.4%, 89.7%±5.1%, respectively. The 4-year OS was significantly higher in patients with initial WBC < 10×109/L than in those with initial WBC ≥ 10×109/L (p=0.020). Conclusion This study showed that the CR rates and survival outcomes in Korean pediatric APL patients were relatively good. The initial WBC count was the most important prognostic factor and most causes of death were related to serious bleeding in the early stage of treatment.

Background: The fatality rate of patients with coronavirus disease 2019 (COVID-19) varies among countries owing to demographics, patient comorbidities, surge capacity of healthcare systems, and the quality of medical care. We assessed the clinical outcomes of patients with COVID-19 during the first wave of the epidemic in Korea. Methods: Using a modified World Health Organization clinical record form, we obtained clinical data for 3,060 patients with COVID-19 treated at 55 hospitals in Korea. Disease severity scores were defined as: 1) no limitation of daily activities; 2) limitation of daily activities but no need for supplemental oxygen; 3) supplemental oxygen via nasal cannula; 4) supplemental oxygen via facial mask; 5) non-invasive mechanical ventilation; 6) invasive mechanical ventilation; 7) multi-organ failure or extracorporeal membrane oxygenation therapy; and 8) death. Recovery was defined as a severity score of 1 or 2, or discharge and release from isolation. Results: The median age of the patients was 43 years of age; 43.6% were male. The median time from illness onset to admission was 5 days. Of the patients with a disease severity score of 3–4 on admission, 65 (71.5%) of the 91 patients recovered, and 7 (7.7%) died due to illness by day 28. Of the patients with disease severity scores of 5–7, 7 (19.5%) of the 36 patients recovered, and 8 (22.2%) died due to illness by day 28. None of the 1,324 patients who were < 50 years of age died; in contrast, the fatality rate due to illness by day 28 was 0.5% (2/375), 0.9% (2/215), 5.8% (6/104), and 14.0% (7/50) for the patients aged 50–59, 60–69, 70–79, and ≥ 80 years of age, respectively. Conclusion In Korea, almost all patients of < 50 years of age with COVID-19 recovered without supplemental oxygen. In patients of ≥ 50 years of age, the fatality rate increased with age, reaching 14% in patients of ≥ 80 years of age.

Bilateral paramedian thalamic infarction is a rare subtype of stroke caused by occlusion of the artery of Percheron, an uncommon variant originating from one of the posterior cerebral arteries. This type of stroke has several major clinical presentations: altered mental status, behavioral amnestic impairment, aphasia or dysarthria, ocular movement disorders, motor deficits, cerebellar signs, and others. Few cases of bilateral paramedian thalamic infarction-related pseudobulbar palsy characterized by dysarthria, dysphagia, and facial and tongue weakness have been reported. We report here a rare case of acute severe pseudobulbar palsy as a manifestation of bilateral paramedian thalamic infarction.
Aphasia ; Arteries ; Deglutition Disorders ; Dysarthria ; Infarction* ; Movement Disorders ; Posterior Cerebral Artery ; Pseudobulbar Palsy* ; Stroke ; Thalamus ; Tongue

A 50-year-old man with liver cirrhosis and esophageal varix for 3 years was diagnosed with hematemesis and treated for a bleeding varix. However, bleeding recurred 11 days later, and he developed drowsiness with left hemiparesis. His left upper and lower extremity muscle strengths based on the manual muscle test at the onset were grade 2/5 and 1/5, respectively. The Babinski sign was positive. His serum ammonia level was elevated to 129.9 microg/dL (normal, 20-80 microg/dL). Magnetic resonance imaging revealed restriction on diffusion and T2-hyperintensities with decreased apparent diffusion coefficient values in the bilateral frontoparietooccipital cortex. The effect was more severe in the right hemisphere and right parietooccipital cortices, which were compatible with hepatic encephalopathy. Although the patient's mental status recovered, significant left-sided weakness and sensory deficit persisted even after 6 months. Diffusion tensor tractography (DTT) performed 3 months post-onset showed decreased volume of the right corticospinal tract. We reported a patient with hepatic encephalopathy involving the corticospinal tract by DTT.
Ammonia ; Diffusion Tensor Imaging ; Diffusion* ; Esophageal and Gastric Varices ; Hematemesis ; Hemorrhage ; Hepatic Encephalopathy* ; Humans ; Liver Cirrhosis ; Lower Extremity ; Magnetic Resonance Imaging ; Middle Aged ; Muscle Strength ; Paresis ; Pyramidal Tracts* ; Reflex, Babinski ; Rehabilitation ; Sleep Stages ; Varicose Veins

Polymyalgia rheumatica is an inflammatory disease affecting elderly and involving the shoulder and pelvic girdles. No epidemiological study of polymyalgia rheumatica was conducted in Korea. We retrospectively evaluated patients with polymyalgia rheumatica followed up at the rheumatology clinics of 10 tertiary hospitals. In total 51 patients, 36 patients (70.6%) were female. Age at disease onset was 67.4 yr. Twenty-three patients (45.1%) developed polymyalgia rheumatica in winter. Shoulder girdle ache was observed in 45 patients (90%) and elevated erythrocyte sedimentation rate (> 40 mm/h) in 49 patients (96.1%). Initial steroid dose was 23.3 mg/d prednisolone equivalent. Time to normal erythrocyte sedimentation rate was 4.1 months. Only 8 patients (15.7%) achieved remission. Among 41 patients followed up, 28 patients (68.3%) had flare at least once. Number of flares was 1.5 +/- 1.6. The frequency of flare was significantly lower in patients with remission (P = 0.02). In Korea, polymyalgia rheumatica commonly develops during winter. Initial response to steroid is fairly good, but the prognosis is not benign because remission is rare with frequent relapse requiring long-term steroid treatment.
Aged ; Aged, 80 and over ; Anti-Inflammatory Agents/administration & dosage/*therapeutic use ; Blood Sedimentation ; Cohort Studies ; Female ; Humans ; Male ; Middle Aged ; Polymyalgia Rheumatica/*drug therapy/epidemiology ; Prognosis ; Recurrence ; Republic of Korea/epidemiology ; Retrospective Studies ; Seasons ; Steroids/administration & dosage/*therapeutic use

OBJECTIVE: To determine the relation between postvoid residual (PVR) and the occurrence of urinary tract infection (UTI) in stroke patients. METHOD: One hundred and eighty-eight stroke patients who were admitted to an inpatient rehabilitation unit and who did not have UTI on admission (105 males, 83 females, mean age 67.1 years) were included in this study. The PVR was measured 3 times within 72 hours after admission. Mean PVR, demographic variables, K-MMSE (Korean Mini-Mental State Examination), initial K-MBI (Korean Modified Barthel Index), Foley catheter indwelling time and stroke type were defined and the relation to the occurrence of UTI was analyzed. RESULTS: UTI occurred in 74 patients (39.4%) during admission to the rehabilitation unit. There were significant differences between the UTI and non-UTI groups in K-MMSE, K-MBI, Foley catheter indwelling time (p<0.01). However, age, gender, stroke location and type were not associated. The occurrence of UTI was 4.87 times higher in the patients with a mean PVR over 100 ml than in those with a mean PVR <100 ml. The mean PVR was 106.5 ml in the UTI group, while it was 62.7 ml in the non-UTI group (p<0.01). PVR was not associated with age. CONCLUSION: The UTI rate is higher when the mean PVR is over 100 ml irrespective of gender and age. Close monitoring of PVR and appropriate intervention is needed to reduce the occurrence of UTI in stroke patients.
Catheters ; Female ; Humans ; Inpatients ; Male ; Stroke ; Urinary Tract ; Urinary Tract Infections

OBJECTIVE: To determine abnormal MRI findings in adults hospitalized with acute severe axial LBP. METHOD: Sixty patients with back pain were divided into 3 groups consisting of 1) 23 adults with acute axial severe LBP who could not sit up or stand up for several days, but had not experienced previous back-related diseases or trauma (group A), 2) 19 adults who had been involved in a minor traffic accident, and had mild symptoms but not limited mobility (group B), and 3) 18 adults with LBP with radicular pain (group C)., Various MRI findings were assessed among the above 3 groups and compared as follows: disc herniation (protrusion, extrusion), lumbar disc degeneration (LDD), annular tear, high intensity zone (HIZ), and endplate changes. RESULTS: The MRI findings of A group were as follows: disc herniation (87%), LDD (100%), annular tear (100%), HIZ (61%), and end plate changes (4.4%). The findings of disc herniation, annular tear, HIZ, and LDD were more prevalent in A group than in B group (p<0.01). HIZ findings were more prevalent in A group than in group B or group C (p<0.05). CONCLUSION: Patients with acute severe axial LBP were more likely to have disc herniation, LDD, annular tear, HIZ. Among LBP groups, there was a significant association of HIZ on MRI with acute severe axial LBP.
Accidents, Traffic ; Adult ; Back Pain ; Humans ; Intervertebral Disc Degeneration ; Low Back Pain ; Magnetic Resonance Imaging ; Magnetic Resonance Spectroscopy ; Magnetics ; Magnets

There are numerous studies about the transformation of renal pathology during lupus nephritis progression. A number of researchers suggest that patients with previous proliferative glomerulonephritis may not need to repeat renal biopsy in relation to treatment strategies. However, the pathology of renal biopsy could offer important information to clinicians about the progression of disease. Here, we report a rare case of the convertion of ISN/RPS classification from a proliferative lesion to a wholly non-proliferative lesion. A 40-year-old female was admitted complaining of generalized edema for 1 month. At the age of 33 she had been diagnosed as SLE with proliferative lupus nephritis. The renal remission was induced with corticosteroid pulse therapy and 12 cycles of intravenous cyclophosphamide treatment. The repeated renal biopsy revealed class V lupus nephritis compared with referential biopsy of class IV-G. A better prognosis is expected with lower activity and a lower chronicity index. Repeat renal biopsy may give useful information relating to the prognosis of nephritis.
Adult ; Biopsy ; Cyclophosphamide ; Edema ; Female ; Glomerulonephritis ; Humans ; Lupus Nephritis ; Nephritis ; Prognosis

Systemic lupus erythematosus (SLE) is an autoimmune disease with various manifestations, while its autoantibodies and immune reactions involve multiple organs. Neuropsychiatric involvement in SLE is known to be common, however, peripheral neuropathy is relatively rare. Guillain-Barre syndrome is clinically defined as an acute demyelinating peripheral neuropathy causing weakness and numbness in the legs and arms. We describe a case of Guillain-Barre syndrome with antiphospholipid syndrome and lupus nephritis. The patient was admitted with fever and diarrhea. He developed progressive weakness of the upper and lower extremities and dysarthria with characteristic nerve conduction patterns compatible with Guillain-Barre syndrome. He also had proteinuria and gangrene of the hand and toe with antiphospholipid antibody. He received intravenous immunoglobulin and plasmapheresis for progressive neuropathy, intravenous high dose steroid to control activity of SLE, and anticoagulation for antiphospholipid syndrome. Neuropsychiatric manifestation of SLE is related to lupus activity closely, so it is important to control lupus activity.
Antibodies, Antiphospholipid ; Antiphospholipid Syndrome ; Arm ; Autoantibodies ; Autoimmune Diseases ; Diarrhea ; Dysarthria ; Fever ; Gangrene ; Guillain-Barre Syndrome ; Hand ; Humans ; Hypesthesia ; Immunoglobulins ; Leg ; Lower Extremity ; Lupus Erythematosus, Systemic ; Lupus Nephritis ; Neural Conduction ; Peripheral Nervous System Diseases ; Plasmapheresis ; Proteinuria ; Toes

OBJECTIVE: To examine the usefulness of the Scale for the Assessment and Rating of Ataxia (SARA) in ataxic stroke patients. METHOD: This was a retrospective study of 54 patients following their first ataxic stroke. The data used in the analysis comprised ambulation status on admission and scores on the SARA, the Korean version of the Modified Barthel Index (K-MBI) and the Berg Balance Scale (BBS). The subjects were divided into four groups by gait status and into five groups by level of dependency in activities of daily living (ADLs) based on their K-MBI scores. Data were subjected to a ROC curve analysis to obtain cutoff values on the SARA for individual gait status and levels of activity dependency. The correlations between the SARA, K-MBI and BBS scores were also computed. RESULTS: There was significant correlation between the SARA and the K-MBI scores (p<0.001), and this correlation (r=-0.792) was higher than that found between the BBS and the K-MBI scores (r=0.710). The SARA scores of upper extremity ataxia categories were significantly related to the K-MBI scores of upper extremity related function (p<0.001). The SARA scores were also significantly correlated negatively with ambulation status (p<0.001) and positively with ADL dependency (p<0.001). In the ROC analysis, patients with less than 5.5 points on the SARA had minimal dependency in ADL, while those with more than 23 points showed total dependency. CONCLUSION: SARA corresponds well with gait status and ADL dependency in ataxic stroke patients and is considered to be a useful functional measure in that patient group.
Activities of Daily Living ; Ataxia ; Dependency (Psychology) ; Gait ; Humans ; Retrospective Studies ; ROC Curve ; Stroke ; Upper Extremity ; Walking