We experienced one case of severe pituitary dwarfism in a 10 years old female girl. Magnetic resonance image (MRI) revealed transection of the pituitary stalk stalk with the formation of high intensity ectopic posterior lobe located at the median eminence and agenesis of an anterior lobe of pituitary gland. The serum growth Hormone (GH) response to clonidine and L-dopa revealed severe GH deficiency. The patient had responses to TRH, normal TSH and partial prolactin response, respectively. There was not response LH and FSH to GnRH. The morning cortisol concentration and serum T4 concentration were decreased below the normal range. These findings and no hyperprolactinemia suggested the presence of a vascular connection between the pituitary gland and hypothalamus, which is not visible on MRI. Sofar, the primary cause of idiopathic pituitary dwarfism in many patients is injury to hypothalamus by perinatal insults. In this patient, there was no history of perinatal insults and postnatal head trauma but transection of the pituitary stalk. We report a case of severe pituitary dwarfism due to agenesis with brief review of related litereature.
Child ; Clonidine ; Craniocerebral Trauma ; Dwarfism, Pituitary* ; Female ; Gonadotropin-Releasing Hormone ; Growth Hormone ; Humans ; Hydrocortisone ; Hyperprolactinemia ; Hypothalamus ; Levodopa ; Magnetic Resonance Imaging ; Median Eminence ; Pituitary Gland* ; Pituitary Gland, Anterior* ; Prolactin ; Reference Values

No abstract available.
Child* ; Humans ; Immunoglobulins ; Infant, Newborn* ; Lactoglobulins ; Milk Proteins* ; Milk* ; Mothers* ; Postpartum Period*

No abstract available.

Twenty-eight cases of initial psoriasis were studied with histochemical methods in an attempt to obtain changes of acid mucopolysaccharides and mast cells. Early psoriatic lesions (pin head sized) and clinically uninvolved skin at distances of 2-4cm from the psoriatic lesions were excised respectively. The results are as follows; 1. Dermal changes were more prominent than the epidermal changes in almost all specimens in early psoriatic lesions. The main features of the dermal changes were perivascular inflammatory infiltrate which were consisted mainly of lymphoid cells. 2. Inflammatory infiltrate in the region of epidermal a.lteration and papillary edema is par ticulary striking. R. Among the 26 cases, the amount of acid mucopalysaccharides of initial lesion was lesser in 14 cases, greater in 4 cases and same in 8 cases as compared with clinically normal skin. 4. The number of mast cells of initial psoriatic lesion as compared with clinically normal skin was increased(p<0. 01).
Dermis* ; Edema ; Glycosaminoglycans ; Head ; Lymphocytes ; Mast Cells* ; Psoriasis* ; Skin ; Strikes, Employee

PURPOSE: To compare the image quality between swept-source optical coherence tomography (SS-OCT) and spectral domain optical coherence tomography (SD-OCT), especially in eyes with media opacity. METHODS: Forty eyes without media opacity and 60 eyes with media opacity (30 eyes with cataract, 20 eyes with vitreous opacity, and 10 eyes with corneal opacity) were included in this study. SD-OCT and SS-OCT 6 x 6 macular scans were taken by a single operator. For image quality analysis, a total of 200 OCT images were subjectively graded by two trained retina specialists and measured quantitatively using the image quality factor (QF) built into the OCT devices. RESULTS: Compared to conventional SD-OCT, SS-OCT had statistically significantly better subjective and objective grades in the normal group, as well as each of the media opacity groups (p-value < 0.001). In both the subjective and objective grades, there was no significant difference according to the types of media opacity (QF: p = 0.188, subject grading scale [SGS]: p = 0.635) and the degree of media opacity (Group I: 20 < or = QF < 50, Group II: 0 < or = QF < 20; QF: p = 0.088, SGS: p = 0.051) in the superiority of image quality of SS-OCT to SD-OCT. CONCLUSIONS: In this media opacity patient population, swept-source OCT is a superior diagnostic tool when compared with SD-OCT in both objective and subjective assessments, even in the ocular media opacity. This result may be useful in diagnosis and progression detection of retinal disease in media opacity eyes.
Cataract ; Diagnosis ; Humans ; Retina ; Retinal Diseases ; Specialization ; Tomography, Optical Coherence*

We report a case of hypereosinophilia causing multiple areas of cerebral infarcts. A 52-year-old Korean man presented with dysarthria and weakness in both arms. A brain MRI revealed multiple acute infarcts in the distal border zone with focal intracerebral hemorrhage, whereas a cerebral angiogram was not remarkable. The eosinophil count was 5,500/microLiter and was accompanied by elevated cardiac enzyme levels. The pattern of cerebral infarcts and laboratory results suggest a thromboembolic infarction associated with hypereosinophilia.
Cerebral Hemorrhage/diagnosis/*etiology ; Cerebral Infarction/diagnosis/*etiology ; Diagnosis, Differential ; Eosinophilia/*complications/drug therapy ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged

Mitochondrial dysfunction represents a biochemically and clinically diverse group of conditions that can affect any organs with high energy requirement such as brain and muscle being particularly vulnerable. Pyruvate dehydrogenase complex (PDHC) deficiency is one type of mitochondrial dysfuntion that is anesthetically associated with lactic acidosis, muscle hypotonia, malignant hyperthermia, and postoperative respiratory failure. We report a case of general anesthetic management during ventriculoperitoneal shunt in a pediatric patient with PDHC deficiency and its possible considerations.
Acidosis, Lactic ; Brain ; Humans ; Malignant Hyperthermia ; Muscle Hypotonia ; Muscles ; Pyruvate Dehydrogenase Complex ; Pyruvic Acid ; Respiratory Insufficiency ; Ventriculoperitoneal Shunt

Mitochondrial dysfunction represents a biochemically and clinically diverse group of conditions that can affect any organs with high energy requirement such as brain and muscle being particularly vulnerable. Pyruvate dehydrogenase complex (PDHC) deficiency is one type of mitochondrial dysfuntion that is anesthetically associated with lactic acidosis, muscle hypotonia, malignant hyperthermia, and postoperative respiratory failure. We report a case of general anesthetic management during ventriculoperitoneal shunt in a pediatric patient with PDHC deficiency and its possible considerations.
Acidosis, Lactic ; Brain ; Humans ; Malignant Hyperthermia ; Muscle Hypotonia ; Muscles ; Pyruvate Dehydrogenase Complex ; Pyruvic Acid ; Respiratory Insufficiency ; Ventriculoperitoneal Shunt

Dystrophic epidermolysis bullosa(DEB) is a rare, chronic non-inflamrnatory bullous disease of hereditary trait, which easily produces bullae in response to even minor traurna. We report two cases which developed at birth and infancy, respectively. Bullae, atrophic scars on the knees, ankles and elbows, as well as milia of both hands and loss of nails were seen in both cases. Histologically, cleavage occurs beneath the basal lamina and anchoring fibrils are rudirnentary on electron microscopy. We present two cases of DEB that seem to be dominant types considering the presence of family history with clinical, histological and electronmicroscopic findings.
Ankle ; Basement Membrane ; Cicatrix ; Elbow ; Epidermolysis Bullosa Dystrophica* ; Hand ; Humans ; Knee ; Microscopy, Electron ; Parturition

Cornelia de Lange syndrome (CdLS) is a relatively uncommon, multiple malformation syndrome involving neurodevelopmental, craniofacial, cardiac, musculoskeletal and gastrointestinal systems. Anesthetic management of a patient with CdLS may pose a serious problem mainly from difficult tracheal intubation, aspiration complications and cardiac malformation. We report our successful experience in anesthetic management of a 15-month-old male patient with CdLS who underwent orchiopexy for bilateral cryptochidism.
De Lange Syndrome ; Humans ; Infant ; Intubation ; Male ; Orchiopexy