Bart's syndrome was initially described as a genodermatosis characterized by congenital localized absence of the skin with blistering and nail deformities 1-3. However, it is considered as any type of epidermolysis bullosa(EB) with localized congenital absence of the skin on the extremities. A 33-day-old fbmale baby was presented with congenital absence of the skin over the left shin and dorsa of both feet which were covered with the thin, translucent, and brown-red glistening membranes. Blistering of the right calf and left great toe nail deformity were also noted. She was diagnosed as a recessive dystrophic EB by the histopathological, ultra- structural and immunomapping studies.
Blister ; Congenital Abnormalities ; Extremities ; Foot ; Membranes ; Skin ; Toes

Dermatofibroma with granular cells is composed mainly of spindle shaped cells mixed with in-conspicuous foamy cells that contain granular cytoplasm. We present a case of dermatofibroma with granular cells in a 36-year-old male who visited our clinic to evaluate a slow-growing nodule on the medial side of the left lawer leg. He have had the nodule for five years and sometimes felt an itching sensation. Biopsy of the nodule identified a dermal nodular tumor showing cellular spindle cell proliferation admixed with peripheral foamy histiocytes. With the immunohistochemical staining, the granular cell was positive for alpha-1-antitrypsin and CD68, and negative for S-100. A few electron-dense granules were seen in the cytoplasm on the electronmicroscopic study. These results suggested that the granular cells originated from histiocytes.
Adult ; Biopsy ; Cell Proliferation ; Cytoplasm ; Histiocytes ; Histiocytoma, Benign Fibrous* ; Humans ; Leg ; Male ; Pruritus ; Sensation

The role of GABA or glycine as an inhibitory neurotransmitter is well established, and GABAergic or glycinergic neurons appear to play an important role in the mammalian retinas. It has been reported that certain amacrine, bipolar, displaced amacrine and ganglion cells are consistently labeled with anti-GABA or anti-glycine antisera in the mammalian retinae so far, and it has been suggested that colocalization of GABA and glycine within the retinal neurons could be common in the mammalian retina by recent immunecytochemical and electrophysiological studies. This study was conducted to localize GABAergic and glycinergic neurons and to define whether GABA and glycine are colocalized within same retinal neurons of the rat retina by immunocytochemical method using anti-GABA and anti-glycine antisera. The results were as follows : 1. GABAergic neurons of the rat retina were amacrine, interplexiform, bipolar, displaced amacrine and ganglion cells, and processes of GABAergic neurons formed dense networks with distinct two bands in the inner plexiform layer. 2. Glycinergic neurons were amacrine, bipolar, displaced amacrine and ganglion cells,and their processes were evenly distributed as dense networks through whole inner plexiform layer. 3. 28.5% of GABA immunoreactive amacrine cells and 9.8% of GABA immunoreactive bipolar cells located in the inner nuclear layer,and 11.9% of labeled neurons located in the ganglion cell layer showed glycine immunoreactivity in the rat retina. These results demonstrate that GABA and glycine, major inhibitory neurotransmitters, are colocalized within certain amacrine and displaced amacrine cells, and a few bipolar cells, and that neurons synthesizing and utilizing both GABA and glycine as their neurotransmitters may play an unique role in the visual processing in the rat retina.
Amacrine Cells ; Animals ; GABAergic Neurons ; gamma-Aminobutyric Acid* ; Ganglion Cysts ; Glycine* ; Immune Sera ; Neurons* ; Neurotransmitter Agents ; Rats* ; Retina* ; Retinal Neurons

BACKGROUND: Although herpes zoster primarily affects afferent sensory neurons, it may complicated by motor nerve involvement in 0.5% to 5% of cases. However herpes zoster-induced muscle paresis is still under-recognized by dermatologists, and there has been insufficient clinical investigation of these conditions. OBJECTIVE: This study was conducted to investigate the clinical aspects of motor nerve involvement of herpes zoster. METHODS: We retrospectively reviewed the data of 711 patients with herpes zoster who had been admitted to our hospital in the departments of dermatology, ophthalmology, otorhinolaryngology, neurology, neurosurgery and rehabilitation medicine between 2005 and 2010. RESULTS: Among the 711 patients with herpes zoster, 15 patients had motor paresis (2.1%). 11 patients (73.3%) had facial nerve palsy including one patient who had accompanying vagus nerve palsy. Besides, one case of trochlear nerve palsy, one case of hypoglossal nerve palsy, one case of upper limb paresis and one case of lower limb paresis were noted. Associated underlying diseases were observed in nine patients (60.0%). Duration of motor weakness ranged from 1 month to 4 years. Twelve patients (80.0%) achieved complete recovery. CONCLUSION: In our patient population, the incidence of motor involvement of herpes zoster was higher in cranial nerves, especially the facial nerve, than in spinal nerves. Although motor nerves are usually involved in relation to dermatome, cranial nerve palsy may occur regardless of its dermatome because of complicated anatomical association. The majority of cases recovered completely without any complications. Further prospective investigation with a large group of patients is necessary to better understand the epidemiology of motor nerve involvement of herpes zoster.
Cranial Nerve Diseases ; Cranial Nerves ; Dermatology ; Facial Nerve ; Herpes Zoster ; Humans ; Hypoglossal Nerve Diseases ; Incidence ; Lower Extremity ; Neurology ; Neurosurgery ; Ophthalmology ; Otolaryngology ; Paralysis ; Paresis ; Retrospective Studies ; Sensory Receptor Cells ; Spinal Nerves ; Trochlear Nerve Diseases ; Upper Extremity ; Vagus Nerve

Renovascular hypertension is the leading cause of surgically curable arterial hypertension. With the marked advancement in the technique of vascular surgery, renal revascularization is preferred to nephrectomy. It is used for the recovery of impaired function or the prevention of renal failure and the control of hypertension. We report a case of renovascular hypertension with the nonfunction of the left kidney due to complete renal artery obstruction, treated successfully with an aortorenal bypass graft.
Hypertension ; Hypertension, Renovascular* ; Kidney ; Nephrectomy ; Renal Artery Obstruction ; Renal Insufficiency ; Saphenous Vein* ; Transplants*

No abstract available.
46, XY Disorders of Sex Development* ; Humans ; Male*

No abstract available.
Multiple Endocrine Neoplasia*

No abstract available.
Humans ; Insulin Resistance* ; Insulin*

No abstract available.
Animals ; Calcium ; Glucose ; Insulin ; Magnesium ; Pancreas ; Rats