We have experienced three cases of extrarnsmmary Pagets disease. The first case was a 77-year-old female who showed 8x10cm sized, erythematous plaque on the suprspubic area of one year's duration. The second case was a 60-year-old male who showed erythematous, oozing, crusted, well circumscribed patches on the right scrotum and the pubic area of 8 years' duration. Third case was a 79-year-old male who showed erythernstous to gray, eczemstoid plaques with marginal elevation on the right side of the scrotum, penile root and pubic area of 3 years duration. On histologic exarnination, the third case showed swest gland adenocarcinoma in the dermis.
Adenocarcinoma ; Aged ; Dermis ; Female ; Humans ; Male ; Middle Aged ; Paget Disease, Extramammary* ; Scrotum

BACKGROUND: Pharmacological ACTH test provide information only about the ability of the adrenal gland to respond to unusual stimuli and may not reflect the daily cortisol secretion. There were some controversies in the diagnosis of mild adrenal insufficiency by using a pharmacological dose of ACTH. The purpose of the present study was to assess and compare the effect of low dose 1 pg ACTH and high dose 249 pg ACTH in the evaluation of adrenal function. The intravenous injection of a pharmacological dose(250 pg) of ACTH has been used as a standard test in the initial assessment of adrenal function. So we low dose ACTH to evaluate the adrenal function and compare the result of high dose ACTH. METHOD: Basal serum cortisol sampling was done at 2:00 p.m.. And after 1 pg ACTH iv bolus injection, cortisol sampling was done at 20, 30, 45 and 60 min respectively. On the next day same procedure was repeated at same time, but 249 mg ACTH was given instead of the low dose. Normal adrenal function was diagnosed with the criteria of stimulated serum cortisol concentration over 20 mg/dL. Total 65 patients took part in this study. Three hypopituitarism patients and one Addisons disease were included. Sixty-one patients had the history of glucocorticoid ingestion or had physical findings of cushingoid features with symptoms suggest the adrenal insufficiency. RESULTS: Sixteen patients showed normal response to both low dose and high dose ACTH. Thirty-six patients were not stimulated to both low dose and high dose ACTH. The remaining thirteen patients revealed normal response to high dose ACTH, but not to low dose ACTH. CONCLUSION: It appears that low dose ACTH stimulation test is more sensitive and specific than high dose ACTH and is capable of revealing mild adrenal insufficiency.
Addison Disease ; Adrenal Glands ; Adrenal Insufficiency ; Adrenocorticotropic Hormone* ; Diagnosis ; Eating ; Humans ; Hydrocortisone ; Hypopituitarism ; Injections, Intravenous

Situs inversus totalis is a rare congenital condition with a mirror image of the thoracic and abdominal viscera. Although several cases of laparoscopic surgery for treating situs inversus have been reported, we believe that laparoscopic low anterior resection for the rectal cancer associated with situs inversus has not yet been reported on. We recently experienced a laparoscopic low anterior resection for rectal cancer and this was performed on a patient with situs inversus. The patient was a 66-year-old woman who presented with constipation for about 1 month. Chest X-rays showed dextrocardia, and the abdomen CT scans revealed situs inversus totalis with upper rectal cancer. She was successfully treated with laparoscopic low anterior resection even though the surgeon was not familiar with situs inversus totalis.
Abdomen ; Aged ; Constipation ; Dextrocardia ; Female ; Humans ; Laparoscopy ; Rectal Neoplasms ; Situs Inversus ; Thorax ; Viscera

PURPOSE: To compare the effects of full-time and part-time occlusion therapy in patients who had been diagnosed with anisometropic amblyopia after age eight and have begun treatment. METHODS: We included patients eight years old or older who had been diagnosed with anisometropic amblyopia. They were treated with full-time or part-time occlusion therapy and followed up for at least six months. Treatment was considered successful when visual acuity was increased by two lines or more. RESULTS: There were 26 total patients. There were 14 part-time and 12 full-time occlusion therapy patients in the respective groups. Visual acuity for the amblyopic eyes was significantly improved while the non-amblyopic eyes did not show any significant differences after the treatment. The changes in the visual acuity were significantly larger for the full-time treatment group compared to the part-time treatment group. The full-time occlusion group showed a significant difference in visual acuity of the amblyopic eyes after treatment. Lower visual acuity of an amblyopic eye at the first visit led to a greater improvement in visual acuity after the treatment. CONCLUSIONS: With good compliance, occlusion therapy for anisometropic amblyopia can be successful even if it is initiated after eight years of age.
Aged ; Amblyopia ; Compliance ; Eye ; Humans ; Visual Acuity

Pulmonary alveolar proteinosis is a rare disorder in which lipoproteinaceous material accumulates within the alveoli. We report a case of pulmonary alveolar proteinosis in a 41 year old female patient. She complained of a dry cough in the preceding 6 months. She presented symptoms of mild hypoxemia and diffuse infiltration at both lower lung fields. A milky fluid was obtained by bronchoalveolar lavage. We confirmed by light microscopic examinations of the lung tissues obtained by transbronchial lung biopsy. Through several follow-ups, the patients symptoms were mild.
Adult ; Anoxia ; Biopsy ; Bronchoalveolar Lavage ; Cough ; Female ; Follow-Up Studies ; Humans ; Lung ; Pulmonary Alveolar Proteinosis

Holoprosencephaly is a rare CNS developmental defect with midline cleavage of embryonal forebrain during the fourth week of fetal development characterized with s single ventricle. Holoprosencephaly is divided into three types by the degree of the brain cleavage : alobar, semilobar, and lobar. Depending on the degree of defected regions, there exist variable clinical symptoms and signs such as stillbirth, hypotonia, apnea, convulsion, delayed development and visual disturbance. It was frequently associated with the deformity of the brain as well as the midline facial structures. Advances in neuroimaging over the past decades have led us to a better understanding of the pathogenesis and the variability of holoprosencephaly. As a result, a lot of cases of holoprosencephaly with various clinical and pathologic findings have been reported. We experienced a case of isolated interhemisphere fusion of frontal lobes with intact septum pellucidum in a child with febrile status epilepticus. Is it a variant of holoprosencephaly?
Apnea ; Brain ; Child ; Congenital Abnormalities ; Fetal Development ; Frontal Lobe* ; Holoprosencephaly* ; Humans ; Muscle Hypotonia ; Neuroimaging ; Prosencephalon ; Seizures ; Septum Pellucidum* ; Status Epilepticus ; Stillbirth

Objective: Orthostatic hypotension (OH) is one of the most common autonomic dysfunctions in Parkinson’s disease (PD) patients. However, many patients with OH are asymptomatic. Conversely, orthostatic dizziness (OD) is not always associated with OH. We investigated the effects of positional changes on cerebral perfusion in patients with PD and OH. Methods: We enrolled 42 patients, comprising 31 PD patients and 11 healthy controls. All the subjects underwent the following clinical assessments: the OH questionnaire, head-up tilt test (HUTT) with transcranial Doppler (TCD), near-infrared spectroscopy, measurement of the change in oxygenated hemoglobin (ΔHboxy) during the squat-to-stand test (SST), measurement of the time derivative of total hemoglobin (DHbtot), and time taken to reach the peak (peak time [PT]) of DHbtot after restanding. Results: The mean flow velocity change (ΔMFV) in the TCD during the HUTT failed to differentiate between the PD-OH(+) and PD-OH(-) groups. The change in oxygenated hemoglobin ΔHboxy was greater in the PD-OH(+) group, which persisted for 9 min until the end of the HUTT only in the left hemisphere. During SST, PT was significantly delayed in the left hemisphere in PD-OH(+) patients. Conclusion Although TCD demonstrated no significant difference in ΔMFV, the parameters measured by near-infrared spectroscopy, such as ΔHboxy during HUTT and PT during the SST, significantly increased ΔHboxy or delayed PT in the left hemisphere of PD-OH(+). Positional changes have a detrimental effect on cerebral hemodynamics in patients with PD and OH, especially in the left hemisphere.

Objective: Orthostatic hypotension (OH) is one of the most common autonomic dysfunctions in Parkinson’s disease (PD) patients. However, many patients with OH are asymptomatic. Conversely, orthostatic dizziness (OD) is not always associated with OH. We investigated the effects of positional changes on cerebral perfusion in patients with PD and OH. Methods: We enrolled 42 patients, comprising 31 PD patients and 11 healthy controls. All the subjects underwent the following clinical assessments: the OH questionnaire, head-up tilt test (HUTT) with transcranial Doppler (TCD), near-infrared spectroscopy, measurement of the change in oxygenated hemoglobin (ΔHboxy) during the squat-to-stand test (SST), measurement of the time derivative of total hemoglobin (DHbtot), and time taken to reach the peak (peak time [PT]) of DHbtot after restanding. Results: The mean flow velocity change (ΔMFV) in the TCD during the HUTT failed to differentiate between the PD-OH(+) and PD-OH(-) groups. The change in oxygenated hemoglobin ΔHboxy was greater in the PD-OH(+) group, which persisted for 9 min until the end of the HUTT only in the left hemisphere. During SST, PT was significantly delayed in the left hemisphere in PD-OH(+) patients. Conclusion Although TCD demonstrated no significant difference in ΔMFV, the parameters measured by near-infrared spectroscopy, such as ΔHboxy during HUTT and PT during the SST, significantly increased ΔHboxy or delayed PT in the left hemisphere of PD-OH(+). Positional changes have a detrimental effect on cerebral hemodynamics in patients with PD and OH, especially in the left hemisphere.

Objective: Orthostatic hypotension (OH) is one of the most common autonomic dysfunctions in Parkinson’s disease (PD) patients. However, many patients with OH are asymptomatic. Conversely, orthostatic dizziness (OD) is not always associated with OH. We investigated the effects of positional changes on cerebral perfusion in patients with PD and OH. Methods: We enrolled 42 patients, comprising 31 PD patients and 11 healthy controls. All the subjects underwent the following clinical assessments: the OH questionnaire, head-up tilt test (HUTT) with transcranial Doppler (TCD), near-infrared spectroscopy, measurement of the change in oxygenated hemoglobin (ΔHboxy) during the squat-to-stand test (SST), measurement of the time derivative of total hemoglobin (DHbtot), and time taken to reach the peak (peak time [PT]) of DHbtot after restanding. Results: The mean flow velocity change (ΔMFV) in the TCD during the HUTT failed to differentiate between the PD-OH(+) and PD-OH(-) groups. The change in oxygenated hemoglobin ΔHboxy was greater in the PD-OH(+) group, which persisted for 9 min until the end of the HUTT only in the left hemisphere. During SST, PT was significantly delayed in the left hemisphere in PD-OH(+) patients. Conclusion Although TCD demonstrated no significant difference in ΔMFV, the parameters measured by near-infrared spectroscopy, such as ΔHboxy during HUTT and PT during the SST, significantly increased ΔHboxy or delayed PT in the left hemisphere of PD-OH(+). Positional changes have a detrimental effect on cerebral hemodynamics in patients with PD and OH, especially in the left hemisphere.

Objective: Orthostatic hypotension (OH) is one of the most common autonomic dysfunctions in Parkinson’s disease (PD) patients. However, many patients with OH are asymptomatic. Conversely, orthostatic dizziness (OD) is not always associated with OH. We investigated the effects of positional changes on cerebral perfusion in patients with PD and OH. Methods: We enrolled 42 patients, comprising 31 PD patients and 11 healthy controls. All the subjects underwent the following clinical assessments: the OH questionnaire, head-up tilt test (HUTT) with transcranial Doppler (TCD), near-infrared spectroscopy, measurement of the change in oxygenated hemoglobin (ΔHboxy) during the squat-to-stand test (SST), measurement of the time derivative of total hemoglobin (DHbtot), and time taken to reach the peak (peak time [PT]) of DHbtot after restanding. Results: The mean flow velocity change (ΔMFV) in the TCD during the HUTT failed to differentiate between the PD-OH(+) and PD-OH(-) groups. The change in oxygenated hemoglobin ΔHboxy was greater in the PD-OH(+) group, which persisted for 9 min until the end of the HUTT only in the left hemisphere. During SST, PT was significantly delayed in the left hemisphere in PD-OH(+) patients. Conclusion Although TCD demonstrated no significant difference in ΔMFV, the parameters measured by near-infrared spectroscopy, such as ΔHboxy during HUTT and PT during the SST, significantly increased ΔHboxy or delayed PT in the left hemisphere of PD-OH(+). Positional changes have a detrimental effect on cerebral hemodynamics in patients with PD and OH, especially in the left hemisphere.