BACKGROUND: Congenital heart disease may be complicated by pulmonary hypertension. We assessed whether inhaled nitric oxide would produce selective pulmonary vasodilation in pediatric patients with congenital heart disease and pulmonary hypertension. METHODS: Inhaled low dose (10 20 ppm) nitric oxide was administrated in patients who were at risk of pulmonary hypertension after operations for congenital heart disease. To identify the nitric oxide effects, we evaluated hemodynamic and ABGA data before (T0) and after (T1) inhaled nitric oxide and just before (T2) decreasing concentration of inhaled nitric oxide. RESULTS: Inhaled nitric oxide decreased pulmonary arterial pressure and increased PaO2/FiO2 without decreasing systemic arterial pressure. CONCLUSIONS: Inhaled nitric oxide selectively decreased pulmonary arterial pressure in patients with congenital heart disease complicated by pulmonary artery hypertension.
Arterial Pressure ; Heart Defects, Congenital ; Heart Diseases* ; Heart* ; Hemodynamics ; Humans ; Hypertension ; Hypertension, Pulmonary* ; Nitric Oxide* ; Pulmonary Artery ; Vasodilation

OBJECTIVE: To consider the relation between MRI findings and clinical subtypes of cerebral palsy (CP). METHOD: The subjects comprised 83 patients with CP. We analyzed the brain MRI findings such as periventricular leukomalacia (PVL), brain atrophy, infarction or hemorrhage, basal ganglia lesion, migration anomaly and delayed myelination with consideration of clinical subtypes of CP. RESULTS: Of the 83 MRI findings, 69 abnormalities (83.1%) were the followings; PVL in 47 cases{17 spastic diplegics (SD), 17 spastic quadriplegics (SQ), 5 spastic hemiplegics (SH), 4 atonic or hypotonic quadriplegics, 2 ataxic quadriplegics and2 mixed quadriplegics (MQ)}, brain atrophy in 6 cases (3 SQ, 1 SD, 1 SH and 1 MQ), infarction or hemorrhage in 7 cases (5 SH and 2 SQ), migration anomaly in 2 cases (1 SQ and 1 SH), delayed myelination in 3 cases (2 SQ and 1 SH) and basal ganglia lesion in 4 cases (3 MQ and 1 atonic quadriplegic). 33 cases of 47 PVL and 2 cases of 6 brain atrophy were preterm CPs. There was no difference in severity of CP between preterm and fullterm CPs. CONCLUSION: The results of this study would be helpful in estimating the brain lesions in various clinical subtypes of CP.
Atrophy ; Basal Ganglia ; Basal Ganglia Hemorrhage ; Brain ; Cerebral Palsy* ; Hemorrhage ; Humans ; Infant, Newborn ; Infarction ; Leukomalacia, Periventricular ; Magnetic Resonance Imaging* ; Muscle Spasticity ; Myelin Sheath

Multiple endocrine neoplasia type 1 (MEN1) syndrome includes varying combinations of endocrine and non-endocrine tumors. There are also a considerable number of atypical MEN1 syndrome. In this case, a 68-yr-old woman was referred to the Department of Endocrinology for hypercalcemia. Five years ago, she had diagnosed as primary hyperaldosteronism and now newly diagnosed as parathyroid hyperplasia with laboratory and pathologic findings. Hurthle-cell thyroid cancer was also resected during the parathyroid exploration and small meningioma was found on brain MRI. Her general condition has markedly improved and her adrenal mass and meningioma are being closely observed now. We could find the loss of heterozygosity of the MEN1 locus in parathyroid glands, suggesting a MEN1-related tumor, but not a germline mutation. Considering a variety of phenotypic expression and a limitation of current molecular analysis, periodic follow up will be needed in patients with a MEN1-like phenotype.
Aged ; Base Sequence ; Brain/radionuclide imaging ; Female ; Humans ; Hyperaldosteronism/complications/*diagnosis ; Hyperparathyroidism, Primary/*diagnosis/etiology/pathology ; Loss of Heterozygosity ; Magnetic Resonance Imaging ; Meningeal Neoplasms/complications/*diagnosis/radionuclide imaging ; Meningioma/complications/*diagnosis/radionuclide imaging ; Mutation ; Parathyroid Glands/pathology ; Proto-Oncogene Proteins/genetics/metabolism ; Sequence Analysis, DNA ; Thyroid Neoplasms/complications/*diagnosis/pathology ; Tomography, X-Ray Computed

Tuberculous optochiasmatic arachnoiditis (OCA) is a rare complication of tuberculous meningitis. We describe a 47-year-old female with tuberculous OCA confused with ethambutol-associated optic neuropathy. She was on anti-tuberculous treatment (i.e., isoniazid, rifampin, ethambutol, and pyrazinamide) for two months due to tuberculous meningitis. Visual impairment occurred during treatment, and ethambutol was changed to levofloxacin because of concern for ethambutol-associated optic neuropathy. Her visual impairment did not improve three months after anti-tuberculous treatment that excluded ethambutol, and she was referred to our hospital. Brain MRI showed enhancement of the optic chiasm and bilateral optic tract, and fundoscopy revealed bilateral optic nerve atrophy, suggesting tuberculous OCA. Her visual acuity was partially improved after anti-tuberculous treatment. Tuberculous OCA should be considered in addition to ethambutol-associated optic neuropathy for a patient with tuberculous meningitis who presents with visual impairment.
Arachnoid ; Arachnoiditis ; Atrophy ; Brain ; Ethambutol ; Female ; Humans ; Isoniazid ; Middle Aged ; Ofloxacin ; Optic Chiasm ; Optic Nerve ; Optic Nerve Diseases ; Rifampin ; Tuberculosis, Meningeal ; Vision Disorders ; Visual Acuity ; Visual Pathways

STUDY DESIGN: Experimental animal study OBJECTIVES: To examine the ability of human umbilical cord blood (hUCB) stem cells to target a zone of injury and to determine the efficacy of hUCB cells to ameliorate the behavioral deficits after a hUCB cell infusion in paralyzed rats. SUMMARY OF LITERTURE: Many groups have investigated the use of stem cells as potential treatments for a CNS injury. hUCB cells have recently been reported to alleviate the behavioral consequences of a stroke injury. MATERIALS AND METHODS: Thirty Sprague Dawley rats were divided into 6 groups (Gr) (Gr 1. SCI (spinal cord injury) + hUCB delivered at one day postinjury, Gr 2. SCI + hUCB delivered at 3 days postinjury, Gr 3. SCI + hUCB delivered at 5 days postinjury, Gr 4. laminectomy + hUCB, Gr 5. SCI only, Gr 6. Laminectomy only). SCI was produced by compressing the spinal cord to the level of the 8-9th thoracic spine for 1 minute with an aneurysm clip that was calibrated to a closing pressure of 50 gms. The hUCB cells (0.5 ml, 1.5x106) were administered intravenously to the rats. The rat was assessed behaviorally at one, two and three weeks using the BBB behavioral scale. Four weeks after the injury, the animals were sacrificed and the hUCB positiveresponse neural cells (mouse anti-human mitochondria monoclonal antibody=MAB 1273) at the injury level observed using optical and fluorescent microscopy. RESULTS: MAB 1273 positive cells were observed in groups 1, 2 and 3 but not in groups 4, 5 and 6. In particular, there were 870 cells distributed over an area of 1.2 mm(2) in group 3. Group 3 showed the most significant recovery over time in the open field exam, and the most improvement in another tests of incline, leg extension, and toe spread compared with group 1 (p<0.01). CONCLUSION: After infusing the hUCB stem cells to SCI rats, it was confirmed that hUCB cells migrate to an injured area and ameliorate the behavioral deficits. A hUCB infusion 5 days after the injury produced best results in terms of the number of cells and motor recovery.
Aneurysm ; Animals ; Fetal Blood* ; Humans* ; Laminectomy ; Leg ; Microscopy ; Mitochondria ; Rats* ; Rats, Sprague-Dawley ; Spinal Cord ; Spinal Cord Injuries ; Spine ; Stem Cells ; Stroke ; Toes ; Umbilical Cord*

BACKGROUND: Inhaled nitric oxide (NO) therapy causes selective pulmonary vasodilation in patients with pulmonary hypertension. However, attempts to discontinue inhaled NO may be complicated by abrupt life-threatening rebound pulmonary hypertension (RPH). The purpose of this study was to determine the risk factors to develop RPH and to present the adequate weaning methods. METHODS: We studied 19 consecutive children who were treated with inhaled NO because of pulmonary hypertension after surgery for congenital heart disease. We compared the dose of NO at the time of start and withdrawal, the duration of weaning and treatment, hemodynamic data, and blood gas analysis before inhaled nitric oxide withdrawal, between patients without (group I, n = 13) and with RPH (group II, n = 6). RESULTS: Compared with group I, group II patients were older in age (1204 1688 versus 546 1654 days, P < 0.05), had a lower NO concentration just before withdrawal (3 +/- 1.6 versus 5 +/- 2.6 ppm, P <0.05), a shorter duration of NO weaning period (4 +/- 3.3 versus 15 +/- 13.4 hours, P < 0.05) and received NO therapy for a shorter duration (26 +/- 11.6 versus 57 +/- 46.0 hours, P < 0.05). CONCLUSIONS: We recommend a progressive withdrawal of inhaled nitric oxide to avoid life-threatening RPH observed in the sudden discontinuation.
Blood Gas Analysis ; Child ; Heart Defects, Congenital* ; Hemodynamics ; Humans ; Hypertension, Pulmonary* ; Nitric Oxide* ; Risk Factors ; Vasodilation ; Weaning

In diabetic patients who were being treated with hemodialysis, coma and other neurological deficits did not occur in spite of extremely elevated serum glucose levels. In this report, we compared diabetic patients with renal failure in severe hyperglycemia with nonketotic hyperosmolar coma patients with normal renal function to know what affect mental changes. Mental changes were not present in diabetic patients with renal failure. These patients with renal failure showed more severe hyperglycemia, but corrected serum sodium concentration and calculated effective serum osmolality were low. So it is suggested that corrected serum sodium concentration and effective serum osmolality are more important factor affecting mental changes than high blood glucose levels in diabetic patients with renal failure, due to absence of osmotic diuresis.
Blood Glucose ; Coma ; Diuresis ; Humans ; Hyperglycemia* ; Osmolar Concentration ; Renal Dialysis ; Renal Insufficiency* ; Sodium

Tuberculous optochiasmatic arachnoiditis (OCA) is a rare complication of tuberculous meningitis. We describe a 47-year-old female with tuberculous OCA confused with ethambutol-associated optic neuropathy. She was on anti-tuberculous treatment (i.e., isoniazid, rifampin, ethambutol, and pyrazinamide) for two months due to tuberculous meningitis. Visual impairment occurred during treatment, and ethambutol was changed to levofloxacin because of concern for ethambutol-associated optic neuropathy. Her visual impairment did not improve three months after anti-tuberculous treatment that excluded ethambutol, and she was referred to our hospital. Brain MRI showed enhancement of the optic chiasm and bilateral optic tract, and fundoscopy revealed bilateral optic nerve atrophy, suggesting tuberculous OCA. Her visual acuity was partially improved after anti-tuberculous treatment. Tuberculous OCA should be considered in addition to ethambutol-associated optic neuropathy for a patient with tuberculous meningitis who presents with visual impairment.
Arachnoid ; Arachnoiditis ; Atrophy ; Brain ; Ethambutol ; Female ; Humans ; Isoniazid ; Middle Aged ; Ofloxacin ; Optic Chiasm ; Optic Nerve ; Optic Nerve Diseases ; Rifampin ; Tuberculosis, Meningeal ; Vision Disorders ; Visual Acuity ; Visual Pathways

Cytomegalovirus (CMV) infection is an important cause of morbidity and mortality among transplant recipients. The first line standard therapy for CMV pneumonia is treatment with a combination of ganciclovir and immunoglobulin. Nevertheless, the mortality of CMV pneumonia is 30~70%. Leflunomide has been recently reported to have novel anti-CMV activity by inhibiting viron assembly. It is also cheaper and is more easily given orally as compared to ganciclovir. We report here on an allogenic stem cell transplant recipient who developed CMV pneumonia that was refractory to ganciclovir and immunoglobulin. The patient was successfully treated with a combination of leflunomide and ganciclovir.
Biological Agents ; Cytomegalovirus ; Ganciclovir ; Humans ; Immunoglobulins ; Isoxazoles ; Pneumonia ; Stem Cell Transplantation ; Stem Cells