No abstract available.

No abstract available.

No abstract available.
Patella*

Giant pigmented nevus is a variety of congenital melanocytic nevi, and may be associated with various abnormalities, such as skeletal malformation, lipoma, vascular nevi, melanosis in the meninges, meningocele, and intracranial arteriovenous malformation. We report a case with congenital giant pigmented nevus and porencephaly. This association should be considered as the spectrum of neurocutaneous syndromes related to congenital giant pigmented nevus.
Intracranial Arteriovenous Malformations ; Lipoma ; Melanosis* ; Meninges ; Meningocele ; Neurocutaneous Syndromes ; Nevus ; Nevus, Pigmented*

BACKGROUND: Cicletanine bydrochloride is a newly developed anti-hypertensive agent. The presence of a furopyridine group characterizes its uncommon chemical structure as an antihypertensive molecule. This clinical trial was performed to confirm the efficacy and safety of cicletanine for the treatment of hypertension as a monotherapy. METHODS: In order to investigate the efficacy and safety of oral cicletanine, a now class of antihypertensive durgs, the furopyridines, on essential hypertension, a single daily dose of 50mg to 100mg cicletanine was administered in 46 hypertensive patients with diastolic blood pressure in the range of 95mmHg-115mmHg. The patients were asked to cut off other anti hypertensive agents for 10 weeks prior to this clinical trial. Blood pressure and heart rate were measured every 4 weeks. The complete blood count, blood chemistry done by SMA-12, serum electrolytes and urinalysis were performed at the 12th week of therapy. RESULTS: 1) Baseline blood pressures after 2 weeks of placebo at sitting and standing positions were 158.7+/-16.1/102.9+/-6.2 and 148.7+/-14.5/102.7+/-6.7mmHg, respectively. The overall slope which represents the tendency of blood pressure decline over the treatment period with cicletanine for all the patients in each position are as follows ; -0.726(SE : 0.150) for sitting systolic blood pressure(BP), -0.390(SE : 0.080) for sitting diastolic BP, -0.214(SE : 0.183) for standing systolic BP and -0.341(SE : 0.139) for standing diastolic BP. 2) The slope of sitting systolic BP line in cicletanine 100mg treated group was significantly stiffer than that of cicletanine 50mg treated group(-0.445 vs -1.021, p=0.0336). 3) There were no significant interval changes in heart rate over the treatment period. 4) There were no significant interval changes in blood chemistry, electrolytes, hematologic findings and urinalysis over the treatment period. 5) Several side effects were observed in six patients(epigastric discomfort in 4, easy fatigue and insomnia in one patient, respectively). CONCLUSION: Treatment with cicletanine was well-tolerated and the incidence of side effects was relatively low. Because of its unique anti-hypertensive mechanism and moderated antihypertensive effects, cicletanine may be well suited in the treatment of hypertension combined with other classes of antihypertensive agents.
Antihypertensive Agents ; Blood Cell Count ; Blood Pressure ; Chemistry ; Electrolytes ; Fatigue ; Heart Rate ; Humans ; Hypertension ; Incidence ; Sleep Initiation and Maintenance Disorders ; Urinalysis

BACKGROUND: The angiotensin-converting enzyme inhibitors have been found to be safe and efficacious in the treatment of essential hypertension. Fosinopril is the first angiotensin-converting enzyme inhibitor from a new class of agents containing phosphorus. This drug is known to be metabolized to almost and equal extent by the hepatic and renal pathways. METHODS: This study was performed to investigate the efficacy and safety of oral fosinopril, a new class of phosporus-containing angiotensin converting enzyme inhibitor, on essential hypertension. A single daily dose of 10mg to 20mg fosinopril was administered in 21 hypertensive patients with diastolic blood pressure in the range of 95mmHg-115mmHg while off all other anti-hypertensive agents for 10 weeks. Blood pressure and heart rate were measured every 4 weeks. The complete blood count, blood chemistry by SMA-12, serum electrolytes and urinalysis were performed at 12th week of therapy. RESULTS: 1) Baseline systolic and diastolic blood pressures after 2 weeks of placebo at sitting position were 158.8+/-15.7 and 99.4+/-6.3mmHg respectively. There was a statistcally significant reduction of blood pressure after 4 week treatment of fosinopril which was maintained up to 12 weeks of follow-up(158.8+/-15.7-99.4+/-6.3mmHg vs 139.3+/-18.2/86.6+/-10.3mmHg, p<0.05). 2) The proportion of responders defined by diastolic blood pressures less than 90mmHg or decline more than 10mmHg at 4, 8 and 12 weeks after treatment with fosinopril were 90.5, 95.2, and 95.2% respectively. 3) THere were no significant changes in blood chemistry, serum electrolytes, hematologic findings and heart rate over the treatment period. 4) Three patients experienced severe non-productive cough that required to discontinue the medication. CONCLUSION: In patients with mild to moderate hypertension, once-daily fosinopril(10mg and 20mg) provided significant anti-hypertensive effects without serious side effects. The 10mg dose was effective in majority of patients and may be considered as a starting dose.
Angiotensin-Converting Enzyme Inhibitors ; Antihypertensive Agents ; Blood Cell Count ; Blood Pressure ; Chemistry ; Cough ; Electrolytes ; Fosinopril* ; Heart Rate ; Humans ; Hypertension ; Peptidyl-Dipeptidase A ; Phosphorus ; Urinalysis

Since the mid-2000s, massive blood transfusion protocols and damage control resuscitation have improved the prognosis of trauma patients. As a part of damage control resuscitation, whole blood transfusion, especially using low titer group O whole blood (LTOWB), has been widely accepted in both military and civilian trauma settings based on its safety and significant advantages in terms of efficiency and efficacy. To implement LTOWB effectively, each institution should establish relevant policies which should simultaneously consider safety and accessibility factors, including titer threshold, blood management, blood supply, and transfusion protocols for LTOWB. These policies will need to be revised through continuous audits and monitoring. Additionally, whole blood and LTOWB may benefit hemorrhagic patients in non-trauma contexts, or in rural and pre-hospital settings. Further supporting evidence for these applications is needed.

Adult onset Still's disease (AOSD) is a systemic autoinflammatory disorder that presents with recurrent fever, extreme fatigue, and joint pain. Pulmonary involvement is not uncommon and, although rare, severe pneumonitis can progress to respiratory failure. Still's disease-associated pneumonitis is generally treated with immunosuppressive agents, but improvement in our understanding of systemic inflammatory processes led us to explore alternative agents. Anakinra is an interleukin-1 receptor antagonist used to treat autoinflammatory disorders resistant to immunosuppressive therapy. Several case reports have demonstrated efficacy of anakinra in treating AOSD, but its relevance in cases complicated with severe pneumonitis has not been examined. Our patient's disease activity was not controlled with systemic steroids and cyclophosphamide. Treatment with anakinra led to a dramatic clinical response. This is the first reported case of AOSD with severe pneumonitis refractory to conventional therapy successfully treated with anakinra.
Arthralgia ; Cyclophosphamide ; Fatigue ; Fever ; Immunosuppressive Agents ; Interleukin 1 Receptor Antagonist Protein* ; Interleukin-1 ; Pneumonia* ; Respiratory Insufficiency ; Steroids ; Still's Disease, Adult-Onset*

PURPOSE: To evaluate the usefulness of echo-planar FLAIR imaging in various cerebral lesions as compared with fast FLAIR imaging. MATERIALS AND METHODS: We obtained consecutive fast FLAIR and echo-planar FLAIR images in 33patients in whom MR imaging had revealed ischemic infarction (n=11), acute infarction (n=5), brain tumor (n=3), other conditions (n=3) or no abnormality (n=11). On both sets of images, percentage contrast and contrast to noise ratio (CNR) were calculated for white matter-gray matter and white matter-lesion. RESILTS: White matter-gray matter percentage contrast and CNR were lower on echo-planar FLAIR imaging than on fast FLAIR imaging (percentage contrast, 19 +/- 2 % vs 28 +/- 3 %, CNR, 2.77 +/- 0. 5 vs 4.86 +/- 0.7). White matter-lesion percentage contrast on echo-planar FLAIR imaging was similar to or greater than that on fast FLAIR imaging; 75 +/- 12 % vs 45 +/- 11 % in ischemic infarction, 80 +/- 12 % vs 78 +/- 11% in acute infarction, and 121 +/- 25 % vs 102 +/- 15 % for tumors. White matter-lesion CNR was similar on both sets of images: 8.3 +/- 0.9 vs 7.9 +/- 0.8 in ischemic infarction, 11 +/- 1.5 vs 9.5 +/- 1.2 in acute infarction, and 24 +/- 4 vs 27 +/- 3 for tumors. Due to high susceptibility to magnetization, echo-planar FLAIR imaging showed image degradation at the interface of the paranasal sinus and adjacent to the temporal bone. CONCLUSION: Echo-planar FLAIR imaging may be a useful pulse sequence in the diagnosis of various cerebral lesions.
Brain Neoplasms ; Diagnosis ; Infarction ; Magnetic Resonance Imaging ; Noise ; Temporal Bone

Obesity-associated focal segmental glomerulosclerosis(OB-FSGS) has been known to progress into advanced renal insufficiency, and its clinicopathological features include obesity, FSGS lesions with glomerulomegaly and, nephrotic-range proteinuria without edema. A 14- year old girl with Prader-Willi syndrome showed nephrotic-range proteinuria without hypoalbuminemia or edema. The renal biopsy revealed focal segmental glomerulosclerosis together with glomerular hypertrophy and an increased mesangial matrix. We report here a case of OB-FSGS as one of the renal problems of Prader-Willi syndrome, and we came to the conclusion that Prader-Willi syndrome is one of the possible disease entities that can lead to renal insufficiency through obesity.
Biopsy ; Child* ; Edema ; Female ; Glomerulosclerosis, Focal Segmental* ; Humans ; Hypertrophy ; Hypoalbuminemia ; Obesity ; Prader-Willi Syndrome* ; Proteinuria ; Renal Insufficiency