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.
Skin*

No abstract available.
Hypertrophy*

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

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

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

BACKGROUND: Contact immunotherapy using diphenylcyclopropenone (DPCP) has been used in the treatment of alopecia and warts. DPCP seemed to be a promising agent for viral disease including molluscum contagiosum (MCI). OBJECTIVE: The purpose of this study was to evaluate the effect of DPCP immunotherapy on MC in children. METHODS: Twenty three patients with multiple lesion of MC were treated with DPCP immunotherapy. RESULTS: Twelve patients (52.2%) showed cure, and eleven patients (47.8%) showed treatment failure. No severe side effects were seen. CONCLUSION: DPCP immunotherapy may be an effective treatment in children with MC without serious side effects.
Alopecia ; Child ; Humans ; Immunotherapy* ; Molluscum Contagiosum* ; Treatment Failure ; Virus Diseases ; Warts

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

The author analyzed 352 patients who had been admitted to P.M.C. with clinical signs and symptoms of occlussive stoke from 1973 to 1980. Cerebral angiography was performed on 250 patients and 126 patients of them were confirmed to have angiographical evidence of occlusion. We've analyzed these 126 patients in clinical point of view. As presented above, positive rate of angiography was 50%. The site of lesion was most common at MCA which was somewhat different from other reports, and the side of predelection was left. In majority of cases, the occlusive stroke was occurred in age group between 40 to 60, and man was about 3 times as common as woman. Risk factors, in our series, were hypertension, cardiac problems, diabetes and so on which were similar to other reports. Hypertension, the most common risk factor, correlated well to the peak age incidence but there was not any intimate relationship with the level of serum cholesterol. The outcome of medically treated group(112 patients) was somewhat better than surgery(14 patients). Among 12 of EIAB, TIA noted the best result compared to RIND and SIE. Meticulous selection of patient using CT scanning and measurement of rCBF would promote the benefit of surgery.
Angiography ; Cerebral Angiography ; Cholesterol ; Female ; Humans ; Hypertension ; Incidence ; Risk Factors ; Stroke ; Tomography, X-Ray Computed