We report a rare case of intrathoracic lymphangiomatosis associated with chylothorax in a 3-year-old boy. The patient had been healthy until he had chickenpox followed by continued dyspnea. The biopsy specimen showed proliferating lymphatic channels & spindle cells in the lung tissue. The boy died of respiratory failure despite conservative treatment and surgical treatment with pleurodesis. An autopsy was performed. Pleura and pericardium showed severe adhesion to the right lung parenchyma. The cut surface of lung showed thickened interlobular septum with honeycomb-appearance. Histologically, extensive intercommunicating and anastomosing endothelial-lined lymphatic channels were noted along the visceral and parietal pleura, pericardium, around the great vessels, and in the anterior mediastinum over the thymus. These lymphatic channels infiltrated into the pulmonary parenchyme along the bronchovascular bundles. There were scattered areas of spindle cell proliferation with extravasation of RBCs mimicking Kaposi's sarcoma. Histologic diagnosis and differential diagnosis on biopsy materials can be difficult to establish but awareness of the intrathoracic lymphangiomatosis and its various clinical presentation may be helpful for diagnosis.
Autopsy* ; Biopsy ; Cell Proliferation ; Chickenpox ; Child, Preschool ; Chylothorax ; Diagnosis ; Diagnosis, Differential ; Dyspnea ; Humans ; Lung ; Male ; Mediastinum ; Pericardium ; Pleura ; Pleurodesis ; Respiratory Insufficiency ; Sarcoma, Kaposi ; Thymus Gland

No abstract available.
Hypertrophy*

No abstract available.
Skin*

No abstract available.

The Mayer-Rokitansky-K ster-Hauser syndrome represents a spectrum of m llerian anomalies, including vaginal agenesis with or without renal anomalies, in genotypically and phenotypically normal female subjects with normal endocrine status. We experienced a case of this anomaly which combined with unilateral renal agenesis and pelvic cystic mass in child.
Child* ; Female ; Humans

Topical immunotherapy with diphenylcyclopropenone(DPCP) has been used for the treatment of alopecia areata. Due to the therapeutic principle of producing a contact eczema, itching, erythema and scaling are inevitable or even desired side effects. However, erythema multiforme-like reactions following topical DPCP treatment have been rarely reported with an estimated incidence of 1.2%. We present herein a case of Stevens-Johnson syndrome, the severe form of erythema multiforme as a side effect of topical DPCP. A 57-year old male patient visited us for the treatment of alopecia totalis. After sensitization with 0.2% DPCP in acetone, we applied DPCP on the scalp once a week for three weeks. Following the 3rd challenge of DPCP, iris-shaped lesions, erosions, vesicles, and bullae developed with fever. Also, he had vesicles and erosions in the oral cavity. The patient was treated with systemic antibiotics, steroids, and antihistamines. The cutaneous lesions were cleared with hyperpigmentation, and pronounced hair regrowth was observed.
Acetone ; Alopecia ; Alopecia Areata ; Anti-Bacterial Agents ; Dermatitis, Contact ; Erythema ; Erythema Multiforme ; Fever ; Hair ; Histamine Antagonists ; Humans ; Hyperpigmentation ; Immunotherapy* ; Incidence ; Male ; Middle Aged ; Mouth ; Pruritus ; Scalp ; Steroids ; Stevens-Johnson Syndrome*

No abstract available.
Choanal Atresia*

PURPOSE: Among renotropic agents, a growth hormone(GH) independent insulin-like growth factor I(IGF-I) improves renal function without inducing glomerulosclerosis and its potential for treating renal disease is increasing. To elucidate the effect of externally administered IGF-I toward enhancing renal function, the change in renal function and renal nitric oxide synthase(NOS) activity were investigated in the presence of the GH-IGF-I axis. MATERIALS AND METHODS: 100?150g Sprague-Dawley male rats were classified four groups according to experimental methods. The left kidney were removed in the rats of UNx group(uninephrectomy) and UNx+IGF-I group(IGF-I treated after uninephrectomy), and sham operation were done in the other rats of control group and Sham+IGF-I group(IGF-I treated after sham operation). The UNx+IGF-I group and Sham+IGF-I group were treated with 300microgram of recombinant IGF-I(rhIGF-I) daily for 5days after operation. The NOS activity were determined in the contralateral kidney tissues and the glomerular filtration rate(GFR) and effective renal plasma flow(ERPF) were determined in the serum. RESULTS: The GFR, ERPF and NOS activity significantly increased compared to control after uninephrectomy(p<0.05). The GFR, ERPF and NOS activity were significantly different between UNx+IGF-I group and UNx group(p<0.05), but there was no significant difference between Sham+IGF-I group and Sham group after the administration of rhIGF-I. CONCLUSIONS: Externally administered rhIGF-I increases GFR and ERPF via increasing NOS activity. We suggest that it is especially effective in improving the renal function of damaged kidneys.
Animals ; Axis, Cervical Vertebra ; Filtration ; Humans ; Hypertrophy* ; Insulin-Like Growth Factor I* ; Kidney* ; Male ; Nitric Oxide Synthase* ; Nitric Oxide* ; Plasma ; Rats ; Rats, Sprague-Dawley ; Renal Plasma Flow, Effective

No abstract available.
Kidney* ; Neuroectodermal Tumors, Primitive*

PURPOSE: Tacrolimus (FK506) is a new potent immunosuppressive agent which has been used as a primary immunosuppressive agent and rescue therapy for refractory rejection in kidney transplantation. In vitro, on a molecular basis, tacrolimus is 10 to 100 times more potent than cyclosporine. Complications associated with tacrolimus are similar to those seen in cyclosporine, including nephrotoxicity. An early marker of tacrolimus-induced nephropathy is tubular vacuolization, whereas long-term administration of tacrolimus is associated with striped interstitial fibrosis and arteriolar hyalinosis. However, morphological changes and pathogenesis of fibrosis in chronic tacrolimus-induced nephropathy remain poorly understood. Transforming growth factor (TGF)-beta1 has been implicated in the fibrosis of a number of chronic diseases of the kidney and other organs. This study was designed to clarify the ultrastructural changes of tacrolimus-induced nephropathy, and to evaluate the relationship between tacrolimus- induced nephropathy and expression of TGF-beta1. METHODS: Male ICR mice received tacrolimus daily at a dose of 2.5 mg/kg by intraperitoneal route for 12 weeks and sacrified 1, 4, 8, 10, and 12 weeks after the initiation of the study, respectively. The kidneys were removed, the cortex is carefully dissected from the medulla, and the tissues are processed for evaluation by light microscopy, electron microscopy, immunohistochemistry and RT-PCR for RNA analysis. RESULTS: Characteristic histological changes of tacrolimus-induced nephropathy were peritubular capillary and intraglomerular capillary congestions, vacuolizations of the tubular epithelium, pericapillary focal fibrosis, and tubular atrophy. Tacrolimus- treated kidneys had a progressive increase in the expression of TGF-beta1, especially in the glomerular and interstitial capillary endothelial cells and atrophied tubular epithelial cells. TGF-beta1 mRNA is expressed persistently in tacrolimus- treated mice for 12 weeks. CONCLUSION: It can be concluded that TGF-beta1 may be involved in the fibrogenesis in the tacrolimus-induced nephropathy.
Animals ; Atrophy ; Capillaries ; Chronic Disease ; Cyclosporine ; Endothelial Cells ; Epithelial Cells ; Epithelium ; Estrogens, Conjugated (USP) ; Fibrosis ; Humans ; Immunohistochemistry ; Kidney ; Kidney Transplantation ; Male ; Mice ; Mice, Inbred ICR ; Microscopy ; Microscopy, Electron ; RNA ; RNA, Messenger ; Tacrolimus ; Transforming Growth Factor beta1 ; Transforming Growth Factors