Congenital hypopituitarism is a rare disorder with absence or reduction of hormones produced by the pituitary gland. The clinical manifestations are hypoglycemia, prolonged jaundice, hyponatremia, micropenis, underdeveloped clitoris, lethargy, convulsion, pallor, cyanosis, apnea, hypotension, temperature instability etc. in neonates and growth failure, delayed or absent puberty in older infants and children. We experienced a case of congenital hypopituitarism who was a 20-year-old male patient with short stature and delayed puberty. Combined pituitary function test revealed panhypopituitarism and magnetic resonance imaging of brain showed anterior pituitary aplasia and ectopic posterior pituitary gland.
Adolescent ; Apnea ; Brain ; Child ; Clitoris ; Cyanosis ; Female ; Humans ; Hypoglycemia ; Hyponatremia ; Hypopituitarism* ; Hypotension ; Infant ; Infant, Newborn ; Jaundice ; Lethargy ; Magnetic Resonance Imaging ; Male ; Pallor ; Pituitary Function Tests ; Pituitary Gland ; Pituitary Gland, Posterior* ; Puberty ; Puberty, Delayed ; Seizures ; Young Adult

Congenital hypopituitarism is a rare disorder with absence or reduction of hormones produced by the pituitary gland. The clinical manifestations are hypoglycemia, prolonged jaundice, hyponatremia, micropenis, underdeveloped clitoris, lethargy, convulsion, pallor, cyanosis, apnea, hypotension, temperature instability etc. in neonates and growth failure, delayed or absent puberty in older infants and children. We experienced a case of congenital hypopituitarism who was a 20-year-old male patient with short stature and delayed puberty. Combined pituitary function test revealed panhypopituitarism and magnetic resonance imaging of brain showed anterior pituitary aplasia and ectopic posterior pituitary gland.
Adolescent ; Apnea ; Brain ; Child ; Clitoris ; Cyanosis ; Female ; Humans ; Hypoglycemia ; Hyponatremia ; Hypopituitarism* ; Hypotension ; Infant ; Infant, Newborn ; Jaundice ; Lethargy ; Magnetic Resonance Imaging ; Male ; Pallor ; Pituitary Function Tests ; Pituitary Gland ; Pituitary Gland, Posterior* ; Puberty ; Puberty, Delayed ; Seizures ; Young Adult

No abstract available.
Humans ; Outpatients* ; Silicon Dioxide*

PURPOSE: The purpose of this study is to investigate the relative surgical risk and problems in hip hemiarthroplasty for treatment of an unstable intertrochanteric fracture in elderly patients over 80 years old. MATERIALS AND METHODS: Between April 2005 and May 2010, 58 patients whose age was over 80 years were available for inclusion in this study. They were divided into two groups: group 1 included 30 patients with femoral neck fracture and group 2 included 28 patients with intertrochanteric fracture. No significant differences in average age, concomitant disease, and walking ability before development of fracture were noted between the two groups. The following factors, including interval from development of fracture to operation, operation time, amount of blood loss, start time of walking after operation, duration of hospital stay, complications, revision rate, and walking ability were compared between the two groups. RESULTS: Operation time was an average of 85.2 minutes in group 1 and 97.5 minutes in group 2(P=0.03). The amount of bleeding was an average of 483 cc in group 1 and 695 cc in group 2(P=0.006). Similar results for walking start and recovery of walking ability after operation were observed in the two groups. No significant differences were observed in duration of hospital stay, complications, and revision rate. While 25 patients in group 1(83.3%) showed restoration of walking ability after operation to the same level of walking before injury, 19 patients in group 2(67.8%) showed restoration of walking ability postoperatively. CONCLUSION: Even though patients in group 2 showed a longer operation time and a higher amount of blood loss, compared with those in group 1, patients in group 2 had similar surgical risk and complications, compared with those in group 1. Therefore, primary hip hemiarthropalsty could be a good treatment option for intertrochanteric fracture in elderly.
Aged ; Femoral Neck Fractures ; Femur Neck ; Hemiarthroplasty ; Hemorrhage ; Hip ; Hip Fractures ; Humans ; Length of Stay ; Walking

Septo-optic dysplasia, as first described by de Morsier, is a rare developmental anomaly of absent or hypoplastic septum pellucidum, a primitive optic vesicle, and dysplasia of the optic nerve, chiasm, and optic tracts. It is a common cause of hypopituitarism in children and has variable hypothalamic-pituitary dysfunction. We experienced a case of septo-optic dysplasia in 5 month old female baby who presented with developmental delay and seizure. A magnetic resonance imaging of the brain showed absence of the septum pellucidum and the fundoscopic examination disclosed the absence of left optic disc and atrophy of right optic disc.
Atrophy ; Brain ; Child ; Female ; Humans ; Hypopituitarism ; Infant ; Magnetic Resonance Imaging ; Optic Nerve ; Seizures ; Septo-Optic Dysplasia* ; Septum Pellucidum ; Visual Pathways

BACKGROUND: The purpose of this article was to identify the risk factors related to development of hemodynamically significant cardiac arrythmias in patients with mechanical ventilation. METHODS AND RESULTS: Holter recording and echocardiogram were performed after 30 minutes of ventilator initiation in patients on mechanical ventilation(MV) owing to respiratory failure(RF) from various reasons. From 68 patients, hemodynamically significant cardiac arrythmias were detected in 18 patients(26.5%). Initial mean arterial pressure, maximal heart rate, and initial pH were identified as risk factors for hemodynamically significant cardiac arrythmias. Additionally, the patients with pressure-controlled ventilation as an initial ventilatory mode developed hemodynamically significant cardiac arrythmias less frequently than the patients with other modes(15.8% vs. 40%, p=0.03). In multivariate analysis, initial mean arterial pressure(<70mmHg, odds ratio[OR]:5.5;95% confidence interval[CI]:1.2 to 24.2, p=0.026), maximal heart rate(>120/min, OR:19.7;95% CI:2.0 to 190.0, p=0.01), and pressure-controlled ventilation(OR:0.13,95% CI:0.03 to 0.55, p=0.006) were associated with the development of hemodynamically significant cardiac arrhythmias. CONCLUSION: Theses findings suggest that during the early stages of mechanical ventilation with acute respiratory failure, hemodynamically significant cardiac arrhythmias are directly associated with tachycardia(>120/min), initial MAP(<70mmHg), and, inversely, the initial use of pressure-controlled ventilation.
Arrhythmias, Cardiac* ; Arterial Pressure ; Heart ; Heart Rate ; Humans ; Hydrogen-Ion Concentration ; Multivariate Analysis ; Respiration, Artificial* ; Respiratory Insufficiency* ; Risk Factors* ; Ventilation ; Ventilators, Mechanical

BACKGROUND: Dendritic cells (DC) are professional antigen-presenting cells in the immune system and can induce T cell response against virus infections, microbial pathogens, and tumors. Therefore, immunization using DC loaded with tumor-associated antigens (TAAs) is a powerful method of inducing anti-tumor immunity. For induction of effective antitumor immunity, antigens should be efficiently introduced into DC and presented on MHC class I molecules at high levels to activate antigen-specific CD8+ T cells. We have been exploring methods for loading exogenous antigens into APC with high efficiency of Ag presentation. In this study, we tested the effect of the cationic liposome (Lipofectin) for transferring and loading exogenous model antigen (OVA protein) into BM-DC. METHODS: Bone marrow-derived DC (BM-DC) were incubated with OVA-Lipofectin complexes and then co-cultured with B3Z cells. B3Z activation, which is expressed as the amount of beta-galactosidase induced by TCR stimulation, was determined by an enzymatic assay using beta-gal assay system. C57BL/6 mice were immunized with OVA-pulsed DC to monitor the in vivo vaccination effect. After vaccination, mice were inoculated with EG7-OVA tumor cells. RESULTS: BM-DC pulsed with OVA-Lipofectin complexes showed more efficient presentation of OVA-peptide on MHC class I molecules than soluble OVA-pulsed DC. OVA-Lipofectin complexes-pulsed DC pretreated with an inhibitor of MHC class I-mediated antigen presentation, brefeldin A, showed reduced ability in presenting OVA peptide on their surface MHC class I molecules. Finally, immunization of OVA-Lipofectin complexes-pulsed DC protected mice against subsequent tumor challenge. CONCLUSION: Our data provide evidence that antigen-loading into DC using Lipofectin can promote MHC class I-restricted antigen presentation. Therefore, antigen-loading into DC using Lipofectin can be one of several useful tools for achieving efficient induction of antigen-specific immunity in DC-based immunotherapy.
Animals ; Antigen Presentation ; Antigen-Presenting Cells ; beta-Galactosidase ; Brefeldin A ; Dendritic Cells* ; Enzyme Assays ; Immune System ; Immunization ; Immunotherapy ; Liposomes ; Mice ; Ovum ; T-Lymphocytes ; Vaccination

No abstract available.

Granulocytic sarcoma is an uncommon extramedullary localized tumor composed of granulocytic precursor cells. The majority of cases have been reported in association with acute myeloid leukemia(AML) especially in children, but infrequently may occur in patients with myeloproliferative disorders or myelodysplastic syndromes. Most common sites of involvement were bone, soft tissue, lymph nodes and skin, but gastrointestinal tract, testis, central nervous system are also involved rarely. We present an unusual case of granulocytic sarcoma of the intracranium and the retro-orbital soft tissue occurring in a patient with concurrent myelodysplastic syndrome, diagnosed by brain magnetic resonance images and ultrasound guided needle biopsy.
Biopsy, Needle ; Brain ; Central Nervous System ; Child ; Gastrointestinal Tract ; Granulocyte Precursor Cells ; Humans ; Lymph Nodes ; Myelodysplastic Syndromes* ; Myeloproliferative Disorders ; Sarcoma, Myeloid* ; Skin ; Testis ; Ultrasonography

No abstract available.
Teratoma*