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*

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

No abstract available.

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

The pulsed laser lithotripsy has been used in the treatment of urinary calculi. We treated 153 patients of stones, via the pulsed dye laser(Technomed Pulsolith) with 7.5 and 6.5 F. rigid ureteroscope between January 1992 and January 1995. Stones were in bladder(4 cases), upper ureter(17), mid ureter(31) and lower ureter(101). The laser lithotripsies were applied for impacted stones and relatively large stones(more than 6 mm) The success rates according to location were 47%(8/17) in upper ureter, 77%(24/31)in middle ureter, 95%(96/101)in lower ureter and 100%(4/4) in bladder and mean success rate was 86% (132/153). There were 21 failures due to upward migration(9 cases), poor visual field(7), laser resistant stone(3) and malfunction of laser(2). Complications were showed in 28 cases that were gross hematuria(16 cases), ureteral perforation(6), fever(4) and ileus(2), but most of them might be related to ureteroscopy rather than laser, and all of them were resolved with conservative management. So laser lithotripsy is safe and effective method of middle and lower ureteral calculi, and upward migration of stones, the major cause of failure may be resolved by use of extracorporeal shock wave lithotripsy and flexible ureteroscope.
Humans ; Lithotripsy ; Lithotripsy, Laser* ; Shock ; Ureter ; Ureteral Calculi ; Ureteroscopes ; Ureteroscopy ; Urinary Bladder ; Urinary Calculi

PURPOSE: The pharmacologic effect of atropine on HPS can be considered to control pyloric muscle spasm. Therefore, we studied the effects of intravenous atropine sulfate on the clinical course of HPS, and periodically observed the ultrasonographic appearance of the pyloric muscles after atropine treatment. METHODS:From April 1998 to May 1999, 14 infants who were diagnosed with HPS were treated with intravenous atropine sulfate. Intravenous atropine sulfate was administered at an initial dose of 0.04mg/kg/day, which was divided into 8 equal doses. The daily dose was increased by 0.01 mg/kg/day until vomiting was controlled for an entire day while infants received unrestricted oral feeding. Ultrasonographic examinations were performed during hospitalization and repeated at least every 2 months until normalization of pyloric muscles was confirmed. RESULTS: Intravenous atropine was effective in 12 of 14 infants with HPS and the conditions of 9 of them improved. Two infants who were not free from vomiting despite a week of intravenous atropine sulfate treatment underwent pyloromyotomy. A series of ultrasonographic examinations were done after vomiting had improved with intravenous atropine sulfate. The ultrasonographic findings showed good passage of gastric contents through pyloric canals despite thickening of the pyloric muscles. CONCLUSION: Intravenous administration of atropine sulfate is an effective therapy for HPS and can be an alternative to pyloromyotomy. (J Korean Pediatr Soc 2000;43:763-768)
Administration, Intravenous ; Atropine* ; Hospitalization ; Humans ; Infant ; Muscles ; Pyloric Stenosis, Hypertrophic* ; Spasm ; Vomiting

Granuloma annulare is a common, benign, inflamatory, usually self-limited dermatosis. Disseminated perforating granuloma annulare is a rare variant of granuloma annulare. A 23-month-old female patient had asymptomatic, multiple umbilicated papules on the face, limbs including palms and soles, buttock and trunk for about 5 months. The new lesions have developed with mild fever or symptoms of upper respiratory infection. Histopathological examination revealed transepidermal elimination of mucinous degenerated collagen fibers and surrounding palisading lymphohistiocytic granuloma. After she was treated with prednisolone and antibiotics, the lesions slightly resolved, but thereafter, new lesions have developed frequently with mild fever. We herein reported a rare case of disseminated perforating granuloma annulare in the youngest patient yet reported.
Anti-Bacterial Agents ; Buttocks ; Child* ; Collagen ; Extremities ; Female ; Fever ; Granuloma Annulare* ; Granuloma* ; Humans ; Infant ; Mucins ; Prednisolone ; Skin Diseases