PURPOSE: To report the results of secondary posterior chamber intraocular lens (PC-IOL) implantation in pediatric cataract eyes with microcornea and/or microphthalmos. METHODS: Retrospective studies were conducted by reviewing the charts of 26 eyes of 15 patients with secondary PC-IOL implantations for microcornea and/or microphthalmos associated with cataract in children between 1999 and 2002. The corneal diameter was 9.5 mm or less at the time of secondary PC-IOL implantation. Preoperative examinations were conducted for bilaterality, corneal diameter, eye abnormalities and systemic abnormalities. Postoperative results were evaluated in terms of visual developments, refractive changes, axial length measurements and the occurrence of complications. The follow-up period was at least one year after secondary PC-IOL implantation. RESULTS: Age at the first diagnosis for cataract with microcornea and/or microphthalmos was 2.7 months on average. Among 15 patients, 8 (53.3%) had a family history. All patients received an initial irrigation and aspiration, posterior capsulectomy, and anterior vitrectomy at 0.8 years (0.1-3.3 years) of age and a secondary PC-IOL implantation surgery at 6.7 years (1.6-17.2 years) of age on average. The postoperative follow-up period was 2.1 years (1.1-4.3 years) on average. The average power of the implanted PC-IOL was +21.2D. Postoperative complications were secondary glaucoma in two eyes, secondary pupillary membrane formation in two eyes. Best corrected visual acuities in 20 eyes in children capable of the vision test at the last follow-up were 20/60 or better in 11 eyes, 20/80 to 20/150 in eight eyes, and 20/200 or worse in one eye. CONCLUSIONS: The secondary PC-IOL implantation in pediatric cataract with microcornea and/or microphthalmos is recommended as a means of improving vision, but must be conducted carefully to avoid possible complications.
Visual Acuity ; Treatment Outcome ; Retrospective Studies ; Refraction, Ocular ; Prosthesis Design ; Microphthalmos/*complications/surgery ; *Lenses, Intraocular ; Lens Implantation, Intraocular/*methods ; Infant, Newborn ; Infant ; Humans ; Follow-Up Studies ; Corneal Diseases/*complications/surgery ; Cornea/*abnormalities/surgery ; Child, Preschool ; Child ; Cataract/*complications ; Adolescent

PURPOSE: To report a case of periorbital infantile capillary hemangioma treated with propranolol. CASE SUMMARY: A 6-month-old girl visited our clinic for a mass on the right upper eyelid, which had been present since birth. A dome-shaped, red-purple colored mass occupying almost the entire right upper eyelid was observed, and overlying branch-shaped telangiectases were also detected. The visual axis of the right eye was partially obscured, due to the ptotic eyelid, and her ability to fix and follow was poor in the right eye. The patient was diagnosed with infantile capillary hemagioma. Because amblyopia in her right eye was suspected, treatment with propranolol was initiated. After 2.5 months of propranolol treatment, the hemangioma decreased in size and volume dramatically, changed from red-purple to light red color, and softened. The ptosis of the right upper eyelid improved, as well as the ability of the right eye to fix and follow. No significant adverse effects (e.g. hypotension, bradycardia, hypoglycemia, bronchospasm, or elevated liver enzymes) were observed, except transient QTc prolongation during cotreatment with ibuprofen, due to an upper respiratory infection. CONCLUSIONS: Propranolol should be considered as a treatment option for periorbital infantile capillary hemangioma. Further clinical studies are required to establish the optimal guidelines and long-term outcome.
Amblyopia ; Axis, Cervical Vertebra ; Bradycardia ; Bronchial Spasm ; Capillaries ; Eye ; Eyelids ; Hemangioma ; Hemangioma, Capillary ; Humans ; Hypoglycemia ; Hypotension ; Ibuprofen ; Infant ; Light ; Liver ; Parturition ; Propranolol ; Telangiectasis

No abstract available.
Radiotherapy* ; Retinoblastoma* ; Rhabdomyosarcoma*

Purpose: To investigate longitudinal changes in foveal retinal sublayer thicknesses during axial elongation. Methods: From February 2013 to September 2014, a prospective cohort was established comprising pediatric patients aged < 13 years exhibiting myopia with a spherical equivalent of less than -0.75 diopters (D). At each visit, the foveal retinal thickness was measured using spectral-domain optical coherence tomography and sublayers were distinguished as follows: 1) total retinal layer, 2) inner retinal layer, 3) retinal nerve fiber layer, and 4) ganglion cell-inner plexiform layer. The average thickness was calculated based on the nine subfields of the Early Treatment Diabetic Retinopathy Study (ETDRS) map and compared between the initial and final visits. Intra-individual correlations were analyzed using a linear mixed-effects model. Results: Twenty-three subjects (46 eyes) were observed for 2.7 ± 1.0 years. During that period, the myopia progressed (spherical equivalent of cycloplegic refraction: from -4.26 ± 2.34 to -6.09 ± 2.64 D; p < 0.001, paired t-test), and the axial length increased from 24.80 ± 1.28 to 25.58 ± 1.38 mm (p < 0.001, paired t-test). Nevertheless, the thicknesses of foveal retinal layers (total retinal layer, inner retinal layer, retinal nerve fiber layer, ganglion cell-inner plexiform layer) showed no significant change in any of the nine ETDRS subfields (all p > 0.05, linear mixed-effects model). Conclusions The foveal retinal sublayers maintained their thicknesses despite axial elongation, indicating that the foveal retinal structure, which is critical to visual acuity, is preserved even in elongated, myopic eyes.

BACKGROUNDS: Pituitary hyperplasia can mimic pituitary adenoma. In MRI, enlarged pituitary gland is enhanced homogenously with upward convexity of the superior margin of the gland .The best definition of hyperplasia in the pituitary hyperplasia seems to be a multiplication of one or more cell types. But definition, etiology and clinical courses of this disease are not clear, METHOD: We reviewed clinical symptoms, MRI, and pathologic findindings in 6 patients with pituitary hyperplasia. RESULT: 1. Major clinical symptoms were headache (100%), visual field defect (84%), polyuria/polydipsia (64%), and irregular mensturation (32%). Other symptoms were amenorrhea (16%) and galactorrhea (16%). 2. Three of five cases showed abnormal responses to combined pituitary function test, 3. MRI findings were pituitary hyperplasia (4), macroadenoma (l), and microadenoma (1). 4. In two operated cases, there was no adenoma. One case showed hyperplasia of lactotroph cells, the other was hyperplasia of gonadotroph cells confirmed by the examination of immunocytochemistry. CONCLUSION: Pituitary hyperplasia should be considered in patients with enlarged pituitary gland without focal mass lesion.
Adenoma ; Amenorrhea ; Female ; Galactorrhea ; Gonadotrophs ; Headache ; Humans ; Hyperplasia* ; Immunohistochemistry ; Lactotrophs ; Magnetic Resonance Imaging ; Pituitary Function Tests ; Pituitary Gland ; Pituitary Neoplasms ; Pregnancy ; Visual Fields

PURPOSE: This study was conducted to investigate the immune responses of children with moderate and severe novel influenza A virus (H1N1) pneumonia, and to compare their clinical and immunological findings with those of control subjects. METHODS: Thirty-two admitted patients with H1N1 pneumonia were enrolled in the study. The clinical profiles, humoral and cell-mediated immune responses of the 16 H1N1 pneumonia patients who were admitted to the pediatric intensive care unit (severe pneumonia group), 16 H1N1 pneumonia patients admitted to the pediatric general ward (moderate pneumonia group) and 13 control subjects (control group) were measured. RESULTS: Total lymphocyte counts were significantly lower in patients with H1N1 pneumonia than in the control group (P=0.02). The number of CD4+ T lymphocytes was significantly lower in the severe pneumonia group (411.5+/-253.5/microL) than in the moderate pneumonia (644.9+/-291.1/microL, P=0.04) and control (902.5+/-461.2/microL, P=0.01) groups. However, the number of CD8+ T lymphocytes was significantly higher in the severe pneumonia group (684.2+/-420.8/microL) than in the moderate pneumonia (319.7+/-176.6/microL, P=0.02) and control (407.2+/-309.3/microL, P=0.03) groups. The CD4+/CD8+ T lymphocytes ratio was significantly lower in the severe pneumonia group (0.86+/-0.24) than in the moderate pneumonia (1.57+/-0.41, P=0.01) and control (1.61+/-0.49, P=0.01) groups. The serum levels of IgG, IgM and IgE were significantly higher in the severe pneumonia group than in the 2 other groups. CONCLUSION: The results of this study suggest that increased humoral immune responses and the differences in the CD4+ and CD8+ T lymphocyte profiles, and imbalance of their ratios may be related to the severity of H1N1 pneumonia in children.
Child ; Humans ; Immunity, Humoral ; Immunoglobulin E ; Immunoglobulin G ; Immunoglobulin M ; Influenza A virus ; Influenza, Human ; Intensive Care Units ; Lymphocyte Count ; Lymphocytes ; Pandemics ; Patients' Rooms ; Pneumonia ; T-Lymphocytes

Cicatrical pemphigoid is regarded as a chronic, autoimmune subepithelial blistering disease of mucous membranes in which lesions often heal with scar formation. Cicatrical pemphigoid typically involves the oral and/or ocular mucosa but may affect the nasal, pharyngeal, laryngeal, esophageal, anogenital regions as well. Immunopathologic studies have shown that patients with cicatrical pemphigoid typically have autoantibodies directed against basement membrane antigens and that they demonstrate continuous deposits of immunoglobulins (IgG, A), complement components and fibrin along the basement membrane. A 69-year-old female was admitted with chief complaints of hoarseness, dyspnea, multiple painful oral ulcer and vesiculobullous skin lesion on upper trunk. During admission, dysphagia and epigastric discomfort was developed. Endoscopic examination revealed multiple erosion and ulcerative lesion with scar formation. Immunopathologic studies showed that linear deposit of IgG, A, C3 along the basement membrane zone. We report a case of cicatrical pemphigoid with esophageal involvement with brief review of literature.
Aged ; Autoantibodies ; Basement Membrane ; Blister ; Cicatrix ; Complement System Proteins ; Deglutition Disorders ; Dyspnea ; Esophagus ; Female ; Fibrin ; Hoarseness ; Humans ; Immunoglobulin G ; Immunoglobulins ; Mucous Membrane ; Oral Ulcer ; Pemphigoid, Bullous* ; Skin ; Ulcer

BACKGROUND: For autologous hematopoietic stem cell transplantation (HSCT), the volume of infused and DMSO contained in graft are the major causes of complications related to infusion. In this study, we evaluated feasibility of cryopreserving peripheral blood stem cell collects (PBSCC) at high cell concentration. METHODS: PBSCC from 40 patients with multiple myeloma or lymphoma were split and cryopreserved at two different concentrations of TNCs; one for standard concentration (SC) (2x108 cells/mL) and the other for high concentration (HC) (3x108 cells/mL). The viability of total nucleated cells and CD34+ cell count were examined before cryopreservation and after thawing. CFU-GM was examined with thawed products. Data were analyzed as two groups between good mobilizer (GM) and poor mobilizer (PM). RESULTS: There were no differences in TNC viability between SC and HC of all patients (P=0.0656) and PM (P=0.9658), however HC of GM showed significantly lower viability than SC (P=0.0314). CD34+ cell viability did not differ between SC and HC. CD34+ cell recovery was decreased in HC of all patients (P=0.459) and GM (P=0.0164), but no differences between SC and HC in PM (P=0.9658). CFU-GM clonogenic efficiency between SC and HC was not different in all patients (P=0.0635) and PM (P=0.8984), but was decreased in HC of GM (P=0.0427). CONCLUSION: Cryopreservation of PBSCC at 3x108 cells/mL seems to have minimal adverse effect on the quality of PBSC after thawing, particularly in PM. This approach may help to reduce infusion related complications while decreasing the cost of processing and storage of PBSCC.
Antigens, CD34 ; Cell Count ; Cell Survival ; Cryopreservation ; Dimethyl Sulfoxide ; Feasibility Studies* ; Granulocyte-Macrophage Progenitor Cells ; Hematopoietic Stem Cell Transplantation ; Humans ; Lymphoma ; Multiple Myeloma ; Peripheral Blood Stem Cell Transplantation ; Stem Cells* ; Transplants