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

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: 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

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 purine antimetabolite 6-Mercaptopurine (6-MP) has been in clinical use for over 30 years and is still a widely used agent in the treatment of childhood acute lymphoblastic leukemia. The bioavailibility, clinical efficacy and toxicity of 6-MP administered orally for maintenance therapy of children with acute lymphoblastic leukemia are highly variable in many studies, as well as at differnt times in same patient. there are many factors affecting the bioavailibility of 6-MP. The most notably factor being that concomitantly administered drugs and foods might contribute to a decrease in the bioavailibity of this drug. In our sociocultural environment milk is a major constituent of child's foods. Cow's milk contains a high concentration of xanthine oxidase, which could potentially transform 6-TM into 6-thioxanthine (6-TX) and 6-thiouric acid (6-TUA) which have no more therapeutic effects. In this study, we evaluated the effect of various milk products on the bioavailability of 6-MP. Incubation at 37degrees C for 30 min raw or pasteurized milk resulted in transformation of a large quantity of clinically relevant concentration of 6-MP into 6-TUA. The concomitant adminstration of folic acid and allopurinol has markedly inhibitory effect on the 6-MP destroying activity of milk at clinically relevant concentrations. These observations may help to optimize modalities of administration of 6-MP for the treartment of patients with childhood leukemia.
6-Mercaptopurine* ; Allopurinol ; Biological Availability* ; Child ; Complement Factor B ; Folic Acid ; Humans ; Leukemia ; Milk* ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Xanthine Oxidase

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*