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

The vast majority of foreign bodies (FBs) that enter the stomach pass through the gastrointestinal tract spontaneously. When the FB enters the small bowel-beyond the reach of conventional endoscopy-daily radiographs are needed to ensure its safe passage. However, endoscopic intervention is an appropriate management strategy for a sharp-pointed FB, because sharp FBs have a higher risk of intestinal perforation. We describe here a case in which a 1.5-cm, sharp-pointed screw nail in the proximal jejunum was removed successfully by double-balloon enteroscopy from a 19-year-old-male with autism. This case adds to the growing body of evidence demonstrating the value of therapeutic double-balloon enteroscopy in the field of FB ingestion
Autistic Disorder ; Double-Balloon Enteroscopy* ; Eating ; Foreign Bodies ; Gastrointestinal Tract ; Intestinal Perforation ; Jejunum* ; Stomach

The so-called "Hereditary Multiple Exostoses" disease is characterized by hard, irregular prominences appearing in the metaphyseal region of the bones. Though transmitted as an autosomal dominant trait, skipped generation are reported and presumably represent spontaneous mutations. We experienced one case of hereditary multiple exostoses of 15 years old male patient, whose father and one brother were also affected. A brief review of related literature is also presented.
Adolescent ; Exostoses ; Exostoses, Multiple Hereditary* ; Fathers ; Humans ; Male ; Siblings

BACKGROUND/AIMS: Hypoxemia is often associated with liver cirrhosis without cardiopulmonary diseases. Pulmonary vascular impairments including intrapulmonary shunt have been considered as a major mechanism of hypoxemia. The aim of this study was to determine the incidence and pathophysiologic basis of hypoxemia in cirrhotic patients without respiratory symptoms. METHODS: In fourty three cirrhotic patients without heart and lung diseases, we performed the arterial blood gas analysis and calculated alveolar arterial oxygen gradient (A-aO2). According to the A-aO2, the patients were divided into hypoxemic and normoxemic groups. In each group, a Tc-99m-macroaggregated albumin (Tc-99m-MAA) scan, a contrast-enhanced echocardiography and a pulmonary function test were performed. RESULTS: Twenty-eight of 43 patients (65%) showed hypoxemia. Hypoxemic patients showed significantly more increased shunt fraction of 3.1 1.4% than normoxemic one of 2.1 1.1% in the Tc-99m-MAA scan (p<0.05). However, only two of hypoxemic patients had shunt fraction above physiologic shunt range. By contrast-enhanced echocardiography, an intrapulmonary shunt was confirmed in one patient. There was no significant correlation between the shunt fraction and the hepatic reserve based on the Child-Pugh classification in hypoxemic patients. In the results of pulmonary function test, only DLco decreased more significantly in hypoxemic group than in normoxemic group (58.4 14.2 % vs 75.3 16.5% of predicted, p<0.05). CONCLUSIONS: Hypoxemia is not infrequently observed in cirrhosis, but incidence of significant intrapulmonary shunt is low. Therefore, other mechanism such as diffusion defect may be suggested to play a role in the development of hypoxemia in cirrhotic patients without respiratory symptoms.
Anoxia ; Blood Gas Analysis ; Classification ; Diffusion ; Echocardiography ; Fibrosis ; Heart ; Humans ; Incidence ; Liver Cirrhosis* ; Liver* ; Lung Diseases ; Oxygen ; Respiratory Function Tests ; Technetium Tc 99m Aggregated Albumin

There are many reports which show that contraction of the extraocular muscles results in an increase in intraocular pressure(IOP). Goldmann tonometer was used to record IOP after Tensilon injection in 22 patients, 10 myasthenics and 12 patients with other neurologic disease. There was a significant difference in IOP measured at 30 seconds after injection between two groups; 1-7(mean: 3.4) mmHg increase in myasthenics but no change, or 1-6 (mean: 1.7) mmHg decrease in patients with other neurologic disease. It is concluded that Tensilon tonometry is a valuable, sensitive and objective method in the diagnosis of myasthenia gravis.
Diagnosis* ; Edrophonium* ; Humans ; Manometry* ; Muscles ; Myasthenia Gravis*

Primary biliary cirrhosis is a chronic progressive disease most often affecting women. It is characterized by the progressive destruction of the small intrahepatic bile ducts with portal inflammation leading to hepatic fibrosis and cirrhosis. Autoimmune diseases such as Sjogren's syndrome, rheumatoid arthritis, dermatomyositis, CREST syndrome, and autoimmune thyroiditis may be found in up to 80% of the patients. Primary biliary cirrhosis is also associated with renal diseases such as distal renal tubular acidosis, bacteriuria and glomerulopathy, but there is no case report of primary biliary cirhosis with renal manifestations in Korea. We are reporting a case of primary biliary cirrhosis with multiple renal abscess and IgA nephropathy with a review of the literature.
Abscess* ; Acidosis, Renal Tubular ; Arthritis, Rheumatoid ; Autoimmune Diseases ; Bacteriuria ; Bile Ducts, Intrahepatic ; CREST Syndrome ; Dermatomyositis ; Female ; Fibrosis ; Glomerulonephritis, IGA* ; Humans ; Immunoglobulin A* ; Inflammation ; Korea ; Liver Cirrhosis, Biliary* ; Sjogren's Syndrome ; Thyroiditis, Autoimmune

PURPOSE: To evaluate the flow resistance though the tube in a glaucoma drainage implant using various tube ligation methods. METHODS: To measure the flow resistance, the following tube ligation methods were designed and tested: Type 1: no ligation of Ahmed valve tube. Type 2: ligation of Ahmed valve tube with 8-0 Vicryl. Type 3: ligation of Molteno tube and a 6-0 Vicryl as an intra-luminal stent with 8-0 Vicryl. Type 4: ligation of Ahmed valve tube and three strands of 8-0 nylon as extra-luminal stents with 8-0 Vicryl. Type 5: ligation of Ahmed valve tube and a 6-0 Prolene as an extra-luminal stent with 8-0 Vicryl followed by removal of the Prolene for partial ligation. RESULTS: The pressure was maintained under 0.143 mm Hg in type 1. In type 2, the pressure increased to 6.688 mm Hg and dropped to approximately 6.384 mm Hg. In type 3, the pressure was maintained at 5.396 mm Hg which decreased to 3.572 mm Hg after stent removal. In type 4, the pressure was maintained at 5.700 mm Hg which dropped to 5.472 mm Hg after the 1st stent removal, to 5.016 mm Hg after the 2nd stent removal and to 4.180 mm Hg after the 3rd stent removal. In type 5, the pressure increased to 6.384 mm Hg and decreased to 5.624 mm Hg. CONCLUSIONS: The tube ligation along with extra-luminal stents followed by staged removal may provide prevention of hypotony and staged control of intraocular pressure after a glaucoma implant operation.
Drainage ; Glaucoma ; Glaucoma Drainage Implants ; Intraocular Pressure ; Ligation ; Nylons ; Polyglactin 910 ; Polypropylenes ; Stents

PURPOSE: To compare the changes in angle parameters after laser peripheral iridotomy (LPI) alone versus LPI and ALPI (argon laser peripheral iridoplasty) in primary angle closure patients by using anterior segment optical coherence tomography (AS-OCT). METHODS: A total of 25 eyes from 17 patients with narrow angles were enrolled in this present study. Eleven eyes in the LPI treatment group and 14 eyes in the LPI and ALPI combined treatment group were evaluated using AS-OCT. The anterior chamber depth (ACD), angle opening distance at 500 microm (AOD 500) and 750 microm (AOD 750), angle recess area at 500 microm (ARA 500) and 750 microm (ARA 750), trabecular-iris space area at 500 microm (TISA 500) and 750 microm (TISA 750), trabecular-iris angle (TIA) were measured. The pre- and post-treatment parameters were compared in each group. The parameter changes after laser treatment were also compared. RESULTS: AOD 500, AOD 750, ARA 500, ARA 750, TISA 500 and TISA 750 except ACD significantly increased following LPI treatment (p = 0.013, p = 0.010, p = 0.008, p = 0.003, p = 0.006, p = 0.003, p = 0.013, respectively, Wilcoxon signed rank test) and LPI and ALPI combined therapy (p = 0.001, p = 0.001, p = 0.001, p = 0.001, p = 0.001, p = 0.001, p = 0.001, respectively, Wilcoxon signed rank test). The AOD 500 difference, TISA 500 difference, and TISA 750 difference were significantly greater after LPI and ALPI combined therapy than after LPI treatment alone (p = 0.112, p = 0.147, p = 0.049, p = 0.037, respectively, Mann-Whitney U-test). CONCLUSIONS: The results from the present study showed LPI alone or LPI and ALPI combined therapy significantly widened the anterior chamber angle and combined therapy showed greater effect than LPI alone.
Anterior Chamber ; Argon ; Eye ; Humans ; Tomography, Optical Coherence

Lichen nitidus (LN) is an uncommon chronic inflammatory skin disease composed of numerous, tiny, shiny, flesh-colored papules that are predominantly observed on the chest, abdomen, glans penis and upper extremities. The distribution of LN is most often localized, but in some cases it can become generalized. Because LN tends to be asymptomatic and presents spontaneous resolution within several years, it usually does not require treatment except in symptomatic, persistent and generalized cases. We describe a 28-yr-old man and a 7-yr-old boy with generalized LN where both cases improved with narrow-band ultraviolet B (NB-UVB) phototherapy plus topical steroid ointment. Both patients noted improvement within the first three treatments and showed almost complete resolution after 18 and 20 treatments, respectively. NB-UVB phototherapy may be an effective alternative therapy for the treatment of generalized LN, even for those patients in their childhood.
*Ultraviolet Therapy ; PUVA Therapy ; Male ; Lichen Nitidus/pathology/*radiotherapy ; Humans ; Child ; Adult

BACKGROUND: In the acute phase of myocardial infarction, the hemostatic mechanism is known to be activated. However, it remains unclear whether increased activity of the hemostatic mechanism is only a marker of the acute thrombotic episode or precedes its appearance. It is also inapparent whether a hypercoagulable state persist for a prolonged period after the apparent resolution of these disorders. METHODS: In a group of 23 patients with acute myocardial infarction who received fibrinolytic therapy with urokinase(group A) or tPA(group B), the plasma level of fibrinogen, antithrombin compared to those of the 10 normal controls. RESULTS: The plasme level of fibrinogen was significantly decreased in both group A and B before and 4 to 24 hours after thrombolytic therapy compared to that of normal controls. But it was increased 7 to 14 days after thrombolytic therapy. In a few of the patients, the plasma level of FDP and D-dimer were positive before thrombolytic therapy and in the most patients they were positive 4 hours after thrombolytic therapy. The plasma level of AT-III was significantly increased in both group A and B before thrombolytic therapy compared with that of normal controls, but, after thrombolytic therapy, there was no significant change in its level. CONCLUSIONS: In the patients with acute myocardial infarction, the thrombolysis occurred before thrombolytic therapy and it lasted for 24 hours after thrombolytic therapy.
Antithrombin III* ; Fibrinogen ; Humans ; Myocardial Infarction* ; Plasma ; Thrombolytic Therapy*