Pyoderma gangrenosum is an uncommon, chronic, recurrent cutaneous ulcerative disease. It is difficult to diagnosis and is frequently associated with systemic diseases. There is no specific therapy, nor effective uniform therapy. Systemic treatment with corticosteroids or cyclosporine, alone or together, appeared to be effective in many cases. However, prolonged therapy is associated with significant side effects, and in refractory cases, immunomodulatory therapy is needed. We report a case of pyoderma gangrenosum, which developed in a 41-years-old woman, who showed no symptoms of having an underlying systemic disease. The conduction was successfully treated with prednisolone, cyclosporine and dapsone.
Adrenal Cortex Hormones ; Cyclosporine* ; Dapsone* ; Diagnosis ; Female ; Humans ; Immunomodulation ; Prednisolone* ; Pyoderma Gangrenosum* ; Pyoderma* ; Ulcer

Pyoderma gangrenosum is an uncommon, chronic, recurrent cutaneous ulcerative disease. It is difficult to diagnosis and is frequently associated with systemic diseases. There is no specific therapy, nor effective uniform therapy. Systemic treatment with corticosteroids or cyclosporine, alone or together, appeared to be effective in many cases. However, prolonged therapy is associated with significant side effects, and in refractory cases, immunomodulatory therapy is needed. We report a case of pyoderma gangrenosum, which developed in a 41-years-old woman, who showed no symptoms of having an underlying systemic disease. The conduction was successfully treated with prednisolone, cyclosporine and dapsone.
Adrenal Cortex Hormones ; Cyclosporine* ; Dapsone* ; Diagnosis ; Female ; Humans ; Immunomodulation ; Prednisolone* ; Pyoderma Gangrenosum* ; Pyoderma* ; Ulcer

Congenital bronchoesophageal fistula (BEF), congenital communication between esophagus and bronchus, is a rare malformation. Benign BEF shows only nonspecific symptoms that may delay a correct diagnosis and proper treatment. This is a case of a 3-year-old boy presenting congenital BEF with imperforate anus and atrial septal defect. He had recurrent pneumonia in the right lower lobe since he was 5 months old. A barium swallow examination showed a communicating fistula between the lower esophagus and the right lower lobe bronchus. Computed tomography scan supported to delineate the course of the fistula. He underwent resection surgery of the fistula and lobectomy of the right lower lobe. He recovered without any postoperative complications.
Anus, Imperforate ; Barium ; Bronchi ; Child ; Esophagus ; Fistula ; Heart Septal Defects, Atrial ; Humans ; Pneumonia ; Postoperative Complications

BACKGROUND: The purpose of this study was to survey the nasal colonization of methicillin-resistant Staphylococcus aureus (MRSA) among the patients admitted in a medical intensive care unit (MICU) and analyze risk factors associated with the colonization. METHODS: The study was carried out on patients admitted into the MICU in a 1,250-bed tertiary care university hospital from January through December 2006. Nasal surveillance cultures were obtained from patients within 24 hours of admission to the unit. Data were analyzed retrospectively by the review of medical records. RESULTS: A total of 312 patients were screened with active nasal cultures; 36 patients (11.6%) were positive for MRSA. Of these, 22 (7.1%) were positive in the nasal cultures only and 14 (4.5%) were positive in the cultures of other specimens (13, sputum; 1, joint fluid) in addition to the nasal swabs. Among the risk factors for MRSA nasal colonization were sex (man), route of admission (from other ICUs or wards), a history of ICU admission during the recent 12 months, and prolonged hospital days in ICU. CONCLUSION: MRSA nasal carrier rate was found higher in this study than in those reported in the literature. Most of the patients colonized with MRSA in the nostril were not colonized with the organism elsewhere in the body. Whether or not active surveillance for MRSA should be performed would depend on the nasal colonization rate of the patients at the time of admission to the ICU.
Colon* ; Humans ; Intensive Care Units* ; Critical Care* ; Joints ; Medical Records ; Methicillin Resistance* ; Methicillin-Resistant Staphylococcus aureus* ; Retrospective Studies ; Risk Factors ; Sputum ; Tertiary Healthcare

BACKGROUND AND PURPOSE: The receptor for advanced glycation end products (RAGE) may contribute to the development of diabetic neuropathy. To assess its relevance in humans, this study examined the expression of RAGE in the skin biopsy samples of patients with diabetes mellitus, and investigated its correlation with intraepidermal nerve-fiber density (IENFD) and clinical measures of neuropathy severity. METHODS: Forty-four patients who either had type 2 diabetes or were prediabetes underwent clinical evaluation and a 3-mm skin punch biopsy. The clinical severity of their neuropathy was assessed using the Michigan Diabetic Neuropathy Score. IENFD was measured along with immunohistochemical staining for RAGE in 29 skin biopsy samples. The expression of RAGE was also quantified by real-time reverse-transcription PCR in the remaining 15 patients. RESULTS: RAGE was localized mostly in the dermal and subcutaneous vascular endothelia. The staining was more intense in patients with a lower IENFD (p=0.004). The quantity of RAGE mRNA was significantly higher in patients with severe neuropathy than in those with no or mild neuropathy (p=0.003). The up-regulation of RAGE was related to dyslipidemia and diabetic nephropathy. There was a trend toward decreased sural nerve action-potential amplitude and slowed peroneal motor-nerve conduction with increasing RAGE expression. CONCLUSIONS: The findings of this study demonstrate up-regulation of RAGE in skin biopsy samples from patients with diabetic neuropathy, supporting a pathogenic role of RAGE in the development of diabetic neuropathy.
Biopsy* ; Diabetes Mellitus ; Diabetic Nephropathies ; Diabetic Neuropathies* ; Dyslipidemias ; Glycosylation End Products, Advanced* ; Humans ; Michigan ; Polymerase Chain Reaction ; Prediabetic State ; Rage ; RNA, Messenger ; Skin* ; Sural Nerve ; Up-Regulation* ; Advanced Glycosylation End Product-Specific Receptor

The major pathogenesis of acute respiratory distress syndrome (ARDS) is an inflammatory process that results from a diversity of injuries to the body. Due to the various cytokines and vasoactive peptides released from the endothelium, the vascular permeability is increased; the migration of inflammatory cells and the leakage of plasma proteins then occur and edema develops in the alveolus. There is a hypothesis that the impairment of alveolar recruitment in ARDS is caused by a defect of the surfactant system and the resultant increase of alveolar surface tension. This has been studied in pediatric patients in ARDS; after the administration of surfactant, hypoxia, respiratory symptoms and survival chances were improved. To alleviate the major pathogenic mechanism in this disease, that is to say, inflammation of the lung, steroids have been used and studied as another treatment modality for ARDS, and it has been concluded that the administration of low dose methylprednisolone may improve patients' symptoms and survival rates. We report here on a case of a young infant admitted with ARDS, who, after the intratracheal administration of 120 mg/kg surfactant, on PaO(2)/FiO(2) was elevated. Subsequent low doses of methylprednisolone were given, and the symptoms did not recur, and no fibrotic change was shown during the follow-up period of 2 months.
Anoxia ; Blood Proteins ; Capillary Permeability ; Cytokines ; Edema ; Endothelium ; Follow-Up Studies ; Humans ; Infant ; Inflammation ; Lung ; Methylprednisolone* ; Peptides ; Respiratory Distress Syndrome, Adult* ; Steroids ; Surface Tension ; Survival Rate

Neonatal lupus erythematosus (NLE) is a rare disease characterized by typical clinical features and the transplacental passage of maternal autoantibodies, particularly anti-SSA/Ro. The major clinical manifestations are cutaneous lupus lesions, congenital heart block, hematological disorders, and hepatobiliary diseases. We report a case of NLE presenting with multiple round and oval target-like erythematous skin lesions and abnormal liver function, born to a clinically asymptomatic mother whose diagnosis was made retrospectively only after her newborn's diagnosis. Both the infant and the mother were positive for the anti-SSA/Ro and anti-SSB/La antibodies.
Antibodies ; Autoantibodies ; Heart Block ; Humans ; Infant ; Liver ; Lupus Erythematosus, Systemic ; Mothers ; Rare Diseases ; Retrospective Studies ; Skin

PURPOSE: We evaluated an outbreak of Serratia marcescens infections in 24 neonates in a neonatal intensive care unit(NICU). METHODS: From January to August, 2004 a nosocomial outbreak of S. marcescens occurred in our NICU. We describe the clinical characteristics of the outbreak and analyse the risk factors for infections with S. marcescens. After the outbreak stopped, 7 isolates from blood were typed using rapid amplified polymorphic DNA analysis(RAPD). RESULTS: S. marcescens was isolated from 24 neonates, 19 infected and 5 colonized. Seven out of nineteen neonates had bacteremia, 4 had ventilator associated pneumonia, 4 had purulent conjunctivitis, 2 had UTI, 1 had meningitis and 1 had a wound infection. Three neonates died due to S. marcescens infection, 2 of 3 had ventilator associated pneumonia, 1 had meningitis complicated with abscess. The mortality rate of S. marcescens infection was 15.8%. Factors associated with S. marcescens infections were previous antibiotic therapy, indwelling catheter and use of ventilators. The isolated strains were resistant to most antibiotics, but frequently sensitive to imipenem, bactrim and amikacin. RAPD typing results show that at least 3 epidemic strains were related with this outbreak. But one genotype was predominant type in this outbreak. The control measures were instituted and the outbreak stopped within 2 months. CONCLUSION: S. marcescens can cause rapidly spreading outbreaks associated with fatal infections in neonates. If S. marcescens is isolated from clinical specimens, meticulous infection control measures and epidemiologic investigations should be done at an early stage of the outbreak.
Abscess ; Amikacin ; Anti-Bacterial Agents ; Bacteremia ; Catheters, Indwelling ; Colon ; Conjunctivitis, Bacterial ; Disease Outbreaks ; DNA ; Genotype ; Humans ; Imipenem ; Infant ; Infant, Newborn* ; Infection Control ; Intensive Care, Neonatal ; Meningitis ; Mortality ; Pneumonia, Ventilator-Associated ; Risk Factors ; Serratia marcescens* ; Serratia* ; Trimethoprim, Sulfamethoxazole Drug Combination ; Ventilators, Mechanical ; Wound Infection

Transient peripheral eosinophilia occurs in several disorders, such as allergic diseases, cancer, and parasitic in fections. However, in most cases, their persence is not accompanied by tissue destruction or organ dysfunc tion. In certain disease states, eosinophils can accumulate in any organ in the body and cause tissue destruction as a result of the eosinophil infiltration or the toxic effects of the degranulated proinflammatory products. Idiopathic hypereosinopilic syndrome is a rare disorder characterized by persistent eosinophilia of an unknown origin, usually associated with a dysfunction of organs such as the heart, lung, skin, and nervous system. Idiopathic hypereosinophilic syndrome usually has an indolent course over a period of several months. However, in some cases, they have grave symptoms if vital organs such as heart and lung are infiltrated. Here we report a case of idiopathic hypereosinophilic syndrome presenting acute pulmonary edema involving the heart, bone marrow, and lung with a review of the relevant literatures.
Bone Marrow ; Eosinophilia ; Eosinophils ; Heart ; Hypereosinophilic Syndrome* ; Lung ; Nervous System ; Pulmonary Edema* ; Skin

A multiple endocrine neoplasia type 2B(MEN2B) is the most distinct and aggressive form of the MEN type 2 variants. We report a case of a 24-years-old woman with MEN2B. The patient had previously undergone a Duhamel's operation due to a megacolon at 6 years old, minor surgery to remove small tumors on the lip at 8 years old, and a bilateral osteotomy of the femur, due to coxa valga, at 15 years old. She underwent a total thyroidectomy and neck dissection, due to a growing thyroid nodule, despite thyroxine treatment, at 19 years old. The pathology revealed a medullary thyroid carcinoma. There was no history of MEN 2B in her family. She had prominent lips, multiple oral mucosal masses, and marfanoid habitus. During the subsequent follow-up, a positron emission tomogram was taken due to a persistently high level of serum calcitonin, despite repeated neck dissections, which revealed a mass in the right adrenal gland. Adrenomedullary function tests showed high levels of urinary catecholamine metabolites, and a genetic analysis of the peripheral leukocyte showed a codon 918 mutation (Met918Thr) at exon 16 of the RET proto-oncogene. The patient underwent a right adrenalectomy and the pathology revealed a pheoch-romocytoma.
Adolescent ; Adrenal Glands ; Adrenalectomy ; Calcitonin ; Child ; Codon ; Coxa Valga ; Electrons ; Exons ; Female ; Femur ; Follow-Up Studies ; Humans ; Leukocytes ; Lip ; Male ; Megacolon ; Multiple Endocrine Neoplasia Type 2b* ; Multiple Endocrine Neoplasia* ; Neck Dissection ; Osteotomy ; Pathology ; Pheochromocytoma ; Proto-Oncogenes* ; Surgical Procedures, Minor ; Thyroid Neoplasms ; Thyroid Nodule ; Thyroidectomy ; Thyroxine ; Young Adult