A male baby with intrauterine growth retardation had a short neck, small hands and feet, hypospadia, both grade I hydronephrosis, type II atrial septal defect, and moderate valvular pulmonary stenosis. The routine chromosome and banding analyses revealed a 46,XY,rec(8)del(8)(p21)dup(8) (q24.1)inv(8)(p21q24.1)pat chromosome constitution. His mother has normal chromosomes, but the father had 46,XY,inv(8)(p21q24.1). Also his uncle had an inv(8) chromosome constitution. We used lymphocytes and examined 40 mitotic cells. All mitotic cells showed deletion of 8p21-->pter and duplication of 8q24.1-->qter. Because 8p21 involves secretion of macrophage and lymphocyte against cancer cells, long-term follow-up for cancer will be needed.
Chromosome Deletion ; Chromosomes, Human, Pair 8 ; Constitution and Bylaws ; Fathers ; Female ; Fetal Growth Retardation ; Foot ; Hand ; Heart Septal Defects, Atrial ; Humans ; Hydronephrosis ; Hypospadias ; Lymphocytes ; Macrophages ; Male ; Mothers ; Neck ; Pulmonary Valve Stenosis ; Trisomy

PURPOSE: Although eosinophilia is a common laboratory finding in many neonatal intensive care units (ICUs), its causative mechanisms remain obscure. We aimed to determine the causes of eosinophilia in the neonatal ICU environment. METHODS: Serial eosinophil counts were determined weekly for 288 hospitalized, appropriately grown neonates. Infants were divided into four groups according to gestational age, and the incidence and etiologic factors of eosinophilia were retrospectively studied. RESULTS: Absolute eosinophilia (>700/mm3) was documented in 18% (52/288) of neonates. Twenty-two infants (42.3%) exhibited mild eosinophilia (700-999 cells/mm3), 27 (51.9%) exhibited moderate eosinophilia (1,000-2,999 cells/mm3), and 3 (5.8%) exhibited severe eosinophilia (>3,000 cells/mm3). Of the 288 infants studied, 54 suffered sepsis. Thirty of these 54 infants (55.6%) showed eosinophilia, and 22 out of the remaining 234 infants (9%) without sepsis showed eosinophilia, indicating that eosinophilia was more prevalent in the sepsis group (P <0.05). All 5 infants suffering from bronchopulmonary dysplasia showed eosinophilia, and 47 out of the remaining 283 infants (16.7%) without bronchopulmonary dysplasia showed eosinophilia. Thus, eosinophilia was more prevalent in the bronchopulmonary dysplasia group (P <0.05). Furthermore, increased prevalence of eosinophilia was associated with respiratory distress syndrome, ventilator use, blood transfusion, and total parenteral nutrition (P <0.05). CONCLUSION: Our results suggest that eosinophilia is influenced by sepsis and bronchopulmonary dysplasia, although it can also occur idiopathically at birth. Moreover, the potential role of eosinophils in conditions such as wound healing and fibrosis in sepsis or chronic lung disease may be a cause of eosinophilia.
Blood Transfusion ; Bronchopulmonary Dysplasia ; Eosinophilia ; Eosinophils ; Fibrosis ; Gestational Age ; Humans ; Incidence ; Infant ; Infant, Newborn ; Intensive Care Units, Neonatal ; Intensive Care, Neonatal ; Lung Diseases ; Parenteral Nutrition, Total ; Parturition ; Prevalence ; Retrospective Studies ; Sepsis ; Stress, Psychological ; Ventilators, Mechanical ; Wound Healing

In order to find out the effect of blood glucose on the ischemic brain injury, the authors studied the relationship between the blood glucose level and the infarct volume in a focal cerebral ischemia-reperfusion model in a series of 60 adult rats. The experimental animals were divided into 4 groups of 15 rats: rats in group I were allowed free access to food until ischemic insults: rats in group II were fasted for 24 hours prior to ischemic insult: rats in group III were fed but received intraperitoneal injection of 1.7unit/kg of insulin 50 minutes before the onset of ischemia: and rats in group IV were fed and received intraperitoneal injection of 2g/kg of 50% glucose during ischemia. The ischemia was made through unilateral occlusion of the middle cerebral artery(MCA) by inserting a 16mm length of 4-0 nylon surgical thread through the internal carotid artery as well as occlusion of both common carotid arteries(CCA) using nontraumatic aneurysm clips. Reperfusion was induced by pulling the thread that occluded the MCA as well as removing the aneurysm clips from both of the CCAs. Each group was further divided into a(2 hour), b(4 hour), and c(6 hour) subgroups of 5 rats according to the duration of ischemia. All animal were killed 3 hours after reperfusion, and infarct volume determined by triphenyltetrazolium chloride was calculated by a computer image software. The results showed that rats of glucose loaded during ischemia(group IV) developed the highest blood glucose levels during ischemia and post-ischemia and the largest infarct volume among groups. The rats which were fed until ischemic insult(group I) developed higher blood glucose levels and larger infarct volume than those developed in group II and III. The rats of group III developed higher blood glucose levels and larger infarct volume than group II. According to our data, lowering the blood glucose level by fasting or intraperitoneal injection of insulin reduced the infarct volme in model of transient focal cerebral ischemia. These results suggest that maintenance of low level of blood glucose during early phase of cerebral infarction may reduce volume of infarction and neurological sequelae.
Adult ; Aneurysm ; Animals ; Blood Glucose* ; Brain Injuries ; Brain Ischemia ; Carotid Artery, Internal ; Cerebral Infarction ; Fasting ; Glucose ; Humans ; Infarction ; Injections, Intraperitoneal ; Insulin ; Ischemia ; Ischemic Attack, Transient* ; Nylons ; Rats* ; Reperfusion

Aneurogenic pulmonary edema(NPE) following an aneurysmal subarachnoid hemorrhage(SAH) is a rare but devastating complication. The authors analyzed the clinical characteristics, therapeutic problems and management results from our cases in order to throughly evaluate for future therapeutic guide for such patients. There were seven patients diagnosed as NPE out of 546 patients who were admitted within one day following a SAH(1.3%) during the past 13 years. They were relatively young(average 51.3 years), had poor clinical grades on admission and had large amount of SAH. Hypotension and hypoxia on admission were also characteristics. Good results were obtained in 4 of the 7 patients by rapid correction of hypoxia and hypotension with intubation, mechanical ventilation and with positive end-expiratory pressure, diuretics and hypertensive drugs. We conclude that NPE following SAH may be triggered by an acute rise in intracranial pressure followed by a simultaneous decrease of the function of the heart and lungs. Although the patients had shown fulminant clinical state on admission, we recommend aggressive management of these patients because some of these patients can recover without neurological deficits by an aggressive treatment.
Aneurysm* ; Anoxia ; Diuretics ; Heart ; Humans ; Hypotension ; Intracranial Aneurysm ; Intracranial Pressure ; Intubation ; Lung ; Positive-Pressure Respiration ; Pulmonary Edema* ; Respiration, Artificial ; Subarachnoid Hemorrhage*

No abstract available.
Carpal Tunnel Syndrome*

OBJECTIVE: The purpose of this study is to identify correlations between diffusion tensor imaging(DTI) and motor improvement by quantifying and visualizing the corticospinal tract on DTI to predict motor impairment in patients with hypertensive intracerebral hemorrhage(ICH). METHODS: Fifteen normal subjects and 7 patients with hypertensive ICH were examined and the latter were treated surgically. DTI was performed with a 3.0 T MRI. The region of interest(ROI) from the posterior limbs of both internal capsules was measured on a fractional anisotropy(FA) map, and the ratios of ROIs were calculated. Tractography, 3-dimensional DTI was then constructed. Motor impairment was assessed on admission and 2weeks after stroke by the Motricity Index(MI). The FA ratio, tractography and score on MI were analyzed for correlations. RESULTS: The FA ratio from the initial DTI did not show a linear correlation with motor impairment. However, after 2weeks, patients with high FA ratios showed high degrees of motor recovery, regardless of the initial severity, and patients with low FA ratios showed low recovery rates. Otherwise, a relationship between the amount of hematoma and the degree of motor recovery could not be determined. On tractography, injury of the corticospinal tract could be visualized and estimated 3-dimensionally. CONCLUSION: FA ratio analysis and tractography constructed from DTI may be useful in understanding corticospinal tract injury and in predicting the recovery from motor impairment in patients.
Diffusion Tensor Imaging* ; Diffusion* ; Extremities ; Hematoma ; Humans ; Internal Capsule ; Intracranial Hemorrhage, Hypertensive* ; Magnetic Resonance Imaging ; Pyramidal Tracts* ; Stroke

PURPOSE: To evaluate the effect of oral sildenafil therapy in neonates with persistent pulmonary hypertension of the newborn (PPHN) METHODS: We conducted a retrospective review of 32 neonates > or =35 weeks' gestation and fraction of inspired oxygen (FiO2) 1.0 with PPHN. The first dose (0.5 mg/kg) of oral sildenafil was started and 1 mg/kg was given every 6 hour thereafter. Mean airway pressure (MAP), FiO2, oxygenation index (OI), mean arterial blood pressure (MBP) were documented before and 6, 12, 24, and 48 hours after sildenafil. For adverse effects, gastrointestinal symptoms, brain ultrasound, funduscopy and auditory brainstem response results were evaluated. RESULTS: The underlying diseases of PPHN (n=32) were meconium aspiration syndrome (n=9), respiratory distress syndrome (n=8), pneumonia (n=3), and idiopathic (n=12). Thirty-one neonates survived; 3 neonates were transferred for inhaled nitric oxide (iNO) and all of them survived. In 28 infants, FiO2 and OI improved significantly by 6 hours and MAP improved significantly by 48 hours after initiation of sildenafil. There were no clinically significant adverse effects of sildenafil. CONCLUSION: Sildenafil may be an effective and safe agent for near-term and term neonates with PPHN, providing significant improvement in oxygenation, and thus may be especially useful in the treatment of PPHN in hospitals without iNO.
Arterial Pressure ; Brain ; Evoked Potentials, Auditory, Brain Stem ; Humans ; Hypertension, Pulmonary ; Infant ; Infant, Newborn ; Meconium Aspiration Syndrome ; Nitric Oxide ; Oxygen ; Piperazines ; Pneumonia ; Pregnancy ; Purines ; Retrospective Studies ; Sulfones ; Sildenafil Citrate

Congenital myotonic dystrophy type 1 (DM1) presents severe generalized weakness, hypotonia, and respiratory compromise after delivery with high mortality and poor prognosis. We presented a congenital DM1 of premature twins in the 30th week of gestation. These twins were conceived by in vitro fertilization (IVF). Both babies presented apnea and hypotonia and had characteristic facial appearance. They were diagnosed DM1 by genetic method. They were complicated by chylothorax and expired at 100 and 215 days of age, respectively. Mother was diagnosed DM1 during the evaluation of babies. This is the first report on congenital DM1 which accompanied the chylothorax. More investigation on the association with chylothorax and congenital DM1 is recommended. With a case of severe neonatal hypotonia, congenital DM1 should be differentiated in any gestational age. Finally, since DM1 is a cause of infertility, we should consider DM1 in infertility clinic with detailed history and physical examination.
Adult ; Apnea/etiology ; Blotting, Southern ; Chylothorax/complications ; Female ; Fertilization in Vitro ; Humans ; Infant, Newborn ; Infant, Premature ; Microsatellite Repeats/genetics ; Muscle Hypotonia/etiology ; Myotonic Dystrophy/complications/*diagnosis/radiography ; Twins

Radial tunnel syndrome is a rare disorder and it's symptoms sometimes so closely overlap those lateral epicondylitis, causing difficulties to differentiate. A 39-year-old man was presented who had a 2.5-year history of right elbow and forearm pain which was unseccesfully treated as 'tennis elbow'. Clinically, severe tender point over the forearm was relieved after a local anesthetic injection. Axial STIR(short tau inversion recovery) image showed high signal intensity at the origin of the extensor carpi radialis brevis, which was so minimal that it was not comparable to clinical symptoms. But radial nerve was revealed normal. So we decided that the symptoms were caused not by lateral epicondylitis but rather by radial tunnel sybdrome and an operation was performed. In the operative field, the most proximal part of the superficial head of the supinator muscle was tendinous and formed a fibrous arch, which was resected. After the operation, the right arm pain was relieved. This is a case diagnosed as lateral epicondylitis which showed no improvement under conservative treatment, but improved after a local anesthetic injection on the tender point. When a case with no correlations between lateral epicondylitis degree in MRI and clinical symptoms, one should take the possibility of radial tunnel syndrome into consideration.
Adult ; Arm ; Elbow ; Forearm ; Head ; Humans ; Magnetic Resonance Imaging ; Radial Nerve

Pseudo-inflammatory tumors are also known as plasma cell granuloma, inflammatory pseudo-tumor and inflammatory myofibroblastic tumor, and these tumors are a group of highly variable proliferations of myofibroblastic cells that are associated with a prominent inflammatory infiltrate. This tumor is known to most commonly occur in the lungs, bladder and gastrointestinal system with only a few cases having been reported in the skin. A previously healthy 26-year-old man presented with a 6-year history of an intermittently pruritic lesion on his back. On the histologic examination, there were spindle cells in fascicles and a mixed inflammatory cellular infiltrate of plasma cells and lymphocytes. A diagnosis of inflammatory fibroblastic tumor was made and the nodule was surgically removed. We report here on an additional case of this rare cutaneous entity, and it is probably the first such report from Korea.
Adult ; Fibroblasts ; Granuloma, Plasma Cell ; Humans ; Korea ; Lung ; Lymphocytes ; Myofibroblasts ; Plasma Cells ; Skin ; Urinary Bladder