We found a case of Ael for the first time in Korea. The patient was 28-year-old woman admitted for the delivery of her first baby. Patient's red cells were typed as O, while the serum typing was A. The red cells were agglutinated by anti-H, but not agglutinated by anti-A1 or anti-AB. Adsorption of anti-A by patient's RBC was confirmed on the adsorption-elution test. In the saliva, only H substance was demonstable. A substance was not demonstrated either in the serum or in the saliva. A transferase was not demonstrated in patient's serum. One of the patient's sister and her daughter, and the patient's son also had same Ael phenotype.
Adsorption ; Adult ; Female ; Humans ; Korea* ; Nuclear Family ; Phenotype ; Saliva ; Siblings ; Transferases

PURPOSE: The growth velocity in patients with central precocious puberty during treatment cannot be predicted. There is a positive correlation in growth among the long bones of the body and the length of the femur and tibia may determine individual height. We want to determine whether the second metacarpal bone can be used as a predictive index for growth velocity during gonadotropin-releasing hormone (GnRH) agonist treatment. METHODS: Thirty-four female children who were diagnosed with precocious puberty at our clinic and treated with GnRH agonist for about 1 year were included in this study. Patients who had growth-related disease, such as growth hormone deficiency and thyroid diseases were excluded. We reviewed their medical records retrospectively. We measured their height and the second metacarpal length from the X-ray film (left hand Anterior-Posterior at the time of their diagnosis and about a year after their GnRH agonist treatment. RESULTS: The age of the subjects was 8.5+/-0.6 years. The growth velocity during treatment was 4.9+/-1.2 cm/yr. There was a positive correlation between height and the second metacarpal length at diagnosis (P = 0.000, r = 0.666) and at one year after treatment (P = 0.000, r = 0.654). There was no correlation between the second metacarpal length at diagnosis and growth velocity during treatment for 1 year. CONCLUSION: We could not find the correlation between the second metacarpal length and growth velocity during GnRH agonist treatment for 1 year. However, the second metacarpal length showed a positive correlation with height before and after treatment. Therefore further study should be done to discovering the mechanisms working during GnRH agonist treatment including bone age, midparental height and so on.
Body Height ; Child ; Female ; Femur ; Gonadotropin-Releasing Hormone ; Growth Hormone ; Hand ; Humans ; Medical Records ; Metacarpal Bones ; Puberty, Precocious ; Retrospective Studies ; Thyroid Diseases ; Tibia ; X-Ray Film

PURPOSE: The growth velocity in patients with central precocious puberty during treatment cannot be predicted. There is a positive correlation in growth among the long bones of the body and the length of the femur and tibia may determine individual height. We want to determine whether the second metacarpal bone can be used as a predictive index for growth velocity during gonadotropin-releasing hormone (GnRH) agonist treatment. METHODS: Thirty-four female children who were diagnosed with precocious puberty at our clinic and treated with GnRH agonist for about 1 year were included in this study. Patients who had growth-related disease, such as growth hormone deficiency and thyroid diseases were excluded. We reviewed their medical records retrospectively. We measured their height and the second metacarpal length from the X-ray film (left hand Anterior-Posterior at the time of their diagnosis and about a year after their GnRH agonist treatment. RESULTS: The age of the subjects was 8.5+/-0.6 years. The growth velocity during treatment was 4.9+/-1.2 cm/yr. There was a positive correlation between height and the second metacarpal length at diagnosis (P = 0.000, r = 0.666) and at one year after treatment (P = 0.000, r = 0.654). There was no correlation between the second metacarpal length at diagnosis and growth velocity during treatment for 1 year. CONCLUSION: We could not find the correlation between the second metacarpal length and growth velocity during GnRH agonist treatment for 1 year. However, the second metacarpal length showed a positive correlation with height before and after treatment. Therefore further study should be done to discovering the mechanisms working during GnRH agonist treatment including bone age, midparental height and so on.
Body Height ; Child ; Female ; Femur ; Gonadotropin-Releasing Hormone ; Growth Hormone ; Hand ; Humans ; Medical Records ; Metacarpal Bones ; Puberty, Precocious ; Retrospective Studies ; Thyroid Diseases ; Tibia ; X-Ray Film

PURPOSE: Epidemiological data indicate that obesity is a risk factor in asthma, however effects related to obesity and adipokines on airway inflammation and bronchial hyper-responsiveness (BHR) have not yet been demonstrated in the human airway. The aim of this study was to investigate the relationship between serum adipokine levels and BHR to mannitol in asthmatic children. METHODS: Serum adipokine levels were measured and pulmonary function tests were perfomed: baseline, postbronchodilator inhalation, methacholine inhalation, and mannitol inhalation. The response to mannitol was expressed as the dose causing a 15% decrease in forced expiratory volume in one second (FEV1) (PD15), and as the response-dose ratio (RDR) (% fall in FEV1/cumulative dose). RESULTS: Sixty-nine prepubertal children between the ages of 6 and 10 years were participated in the study. They comprised asthmatic children (n=40) and healthy (n=29). Twenty-two subjects (55.5%) with asthma had a positive mannitol bronchial provocation test (BPT) result. The body mass index (BMI) was higher in those asthmatics with positive mannitol BPTs than in asthmatics with negative mannitol BPTs and in the control group (19.30 kg/m2 vs. 17.60 kg/m2 vs. 17.93 kg/m2, P=0.035, P=0.046). Serum leptin levels were also significantly higher in asthmatics with positive mannitol BPTs than in asthmatics with negative mannitol BPTs and in the control group (10.58 ng/mL vs. 5.49 ng/mL vs. 6.75 ng/mL, P=0.002, P=0.016). Leptin values were significantly associated with a PD15 (r=-0.498, P=0.022) and RDR to mannitol (r=0.346, P=0.033) in asthmatic children after adjustment for BMI. CONCLUSION: Serum leptin levels were significantly associated with BHR to mannitol in asthmatic children.
Adipokines ; Asthma ; Body Mass Index ; Bronchial Provocation Tests ; Child* ; Forced Expiratory Volume ; Humans ; Inflammation ; Inhalation ; Leptin* ; Mannitol* ; Methacholine Chloride ; Obesity ; Respiratory Function Tests ; Risk Factors

PURPOSE: Recently, component-resolved diagnosis (CRD) using microarray technology has been introduced to the field of clinical allergy. This study was aimed to investigate the clinical usefulness of microarray-based IgE detection for diagnosing clinical raw fruit allergy in birch pollen-sensitized children. METHODS: Thirty-one children with allergic disease who had been sensitized to pollen were studied. A pollen-sensitized patient was defined as having an allergen-specific history with concomitant positive skin-prick tests (SPTs) to natural allergen extracts or positive allergen-specific IgE. All subjects underwent SPTs for pollen and fruit. In all subjects, specific IgE to pollen and fruit were measured by ImmunoCAP. Specific IgE antibodies to allergen components were determined by a customized allergen microarray (ISAC). RESULTS: Thirteen of the 31 patients (41.9%) had a history of fruit hypersensitivity with positive SPTs. Measuring IgE to allergen components by ISAC, all the 13 patients with fruit hypersensitivity were positive to at least one of Mal d 1, Pru p 1, Pru p 3, Act d 8, and Act d 2 compared to 12 of the 13 patients (92.3%) who had at least 1 positive IgE to fruits (apple, peach, and kiwi) using ImmunoCAP. The sensitivity of ISAC microarray was 100.0% for the diagnosis of fruit hypersensitivity, but its specificity was 27.7% (5/18). The sensitivity of ImmunoCAP was 92.3%, and its specificity was 83.3%. CONCLUSION: The sensitivity of allergen components tested using microarray for the diagnosis of clinical fruit hypersensitivity in children with pollen allergy was high; however, its specificity was low.
Antibodies ; Betula* ; Child* ; Diagnosis* ; Fruit* ; Humans ; Hypersensitivity* ; Immunoglobulin E ; Pollen* ; Prunus persica ; Rhinitis, Allergic, Seasonal

PURPOSE: Dysregulated cysteinyl leukotriene (CysLT) synthesis is prominent in exercise-induced bronchoconstriction (EIB). Secreted phospholipase A2 (sPLA2) plays a key regulatory role in the biosynthesis of CysLTs. We previously found that serum leptin levels correlate with (EIB) in children with asthma. The aim of this study was to address the relationship between plasma sPLA2/leptin levels and EIB. METHODS: Sixty-seven prepubertal children between the ages of 6 and 10 years were included in the study. They were asthmatics with EIB (n=25), asthmatics without EIB (n=21), and healthy subjects (n=21). We measured the plasma sPLA2 and leptin levels. We also performed pulmonary function tests at baseline, after bronchodilator inhalation, and after exercise. RESULTS: The sPLA2 and leptin levels were significantly higher in asthmatics with EIB than in those without and control subjects. In addition, sPLA2 levels were significantly correlated with body mass index (Speraman correlation coefficient r=0.343, P=0.023) and leptin levels (partial correlation coefficient r=318, P=0.033). The maximum decrease in % forced expiratory volume in 1 second after exercise was significantly correlated with both PLA2 levels (r=0.301, P=0.041) and leptin levels (r=0.346, P=0.018). CONCLUSION: The sPLA2 and leptin levels were significantly higher in asthmatics with EIB than in asthmatics without EIB and control subjects. In addition, sPLA2 levels were significantly correlated with leptin levels and EIB in asthmatic children.
Asthma ; Body Mass Index ; Bronchoconstriction* ; Child* ; Forced Expiratory Volume ; Humans ; Inhalation ; Leptin* ; Phospholipases A2* ; Plasma* ; Respiratory Function Tests

Central diabetes insipidus (DI) is a disease caused by insufficient release of antidiuretic hormone. Central DI with lung cancer is very rare. Most of them are caused by the pituitary metastasis, and rarely, by the paraneoplastic syndromes. Central DI is diagnosed by the water deprivation test. The treatment consists of surgical resection, radiotherapy and administration of desmopressin. We report an unusual case of central DI with non-small cell lung cancer. The diagnosis was confirmed by water deprivation test. After the administration of desmopressin, the urine osmolarity was increased. The patient's symptoms and urine osmolarity were improved by intranasal desmopressin.
Carcinoma, Non-Small-Cell Lung* ; Deamino Arginine Vasopressin ; Diabetes Insipidus, Neurogenic* ; Diagnosis ; Humans ; Lung Neoplasms ; Neoplasm Metastasis ; Osmolar Concentration ; Paraneoplastic Syndromes ; Radiotherapy ; Water Deprivation

Primary venous thrombosis caused by deficiency or qualitative abnormality of antithrombin III, protein C and protein S is usually inherited as an autosomal dominant trait. Usually, deep vein thrombosis or pulmonary thromboembolism is developed by such abnormalities, however, mesenteric vein thrombosis is rarely reported. A 27-year-old man with previous history of deep vein thrombosis underwent segmental resection of jejunum due to mesenteric vein thrombosis complicated by necrosis of jejunum. Postoperative investigation disclosed combined deficiency of antithrombin III and protein C. His son also showed deficiency of antithrombin III. Postoperatively, he is on life-long warfarin therapy without experiencing recurrence of venous thrombosis.
Adult ; Antithrombin III* ; Humans ; Jejunum ; Mesenteric Veins ; Necrosis ; Protein C* ; Protein S ; Pulmonary Embolism ; Recurrence ; Thrombosis ; Venous Thrombosis* ; Warfarin

BACKGROUND: IPF is characterized by chronic, fibrosing inflammatory lung disease of unknown etiology. Typical symptoms of IPF are exertional dyspnea with nonproductive cough. Why patients with typical IPF have dry cough rather than productive cough, is unknown. IP-10 plays an important regulatory role in leukocyte trafficking into the lung. The present study investigated the effect of IP-10 in the pathogenesis of dry cough rather than productive cough in IPF patients. METHODS: IP-10 concentration was measured by ELISA from BALF of IPF patients. To evaluate the role of IP-10 in mucin expression, the expression of the MUC5AC mucin gene was measured in NCI-H292 cells, a human pulmonary mucoepidermoid carcinoma cell line, after stimulation by TNF-alpha with or without IP-10 pretreatment. EGFR-MAPK expression was also examined as a possible mechanism. RESULTS: IP-10 levels were significantly higher in the BALF of IPF patients compared to healthy controls. IP-10 pretreatment reduced TNF-alpha induced MUC5AC mucin expression by inhibiting the EGFR-MAPK signal transduction pathway in NCI-H292 cells. CONCLUSION: These findings suggest that little mucus production in IPF patients might be attributable to IP-10 overproduction, which inhibits the EGFR-MAPK signal transduction pathway required for MUC5AC mucin gene expression.
Carcinoma, Mucoepidermoid ; Cell Line ; Cough ; Dyspnea ; Enzyme-Linked Immunosorbent Assay ; Gene Expression ; Humans ; Idiopathic Pulmonary Fibrosis ; Leukocytes ; Lung ; Lung Diseases ; Mucins ; Mucus ; Receptor, Epidermal Growth Factor ; Signal Transduction ; Sputum ; Tumor Necrosis Factor-alpha

BACKGROUND: Argyria is a rare irreversible cutaneous pigmentation disorder caused by prolonged exposure to silver. Herein, we report a case of generalized argyria that developed after chronic ingestion of soluble silver-nano particles and presented with muscle weakness. CASE PRESENTATION: A 74-year-old woman visited our emergency room, complaining of fever and mental deterioration. She was diagnosed with acute pyelonephritis and recovered after antibiotic therapy. At presentation, diffuse slate gray-bluish pigmented patches were noticed on her face and nails. Two months prior to visiting our hospital, she was diagnosed with inflammatory myopathy and given steroid therapy at another hospital. We performed a nerve conduction study that revealed polyneuropathy. In skin biopsies from pigmented areas of the forehead and nose, the histopathologic results showed brown-black granules in basement membranes of sweat gland epithelia, which are diagnostic findings of argyria. We reviewed pathology slides obtained from the left thigh muscles and found markedly degenerated myofibers with disorganization of myofibrils without inflammatory reactions, consistent with unspecified myopathy, rather than inflammatory myopathy. The patient was diagnosed with generalized argyria with polyneuropathy and myopathy and transferred to a rehabilitation institution after being tapered off of steroids. CONCLUSIONS: Clinicians should be aware of clinical manifestations of argyria and consider it in differential diagnosis when they examine patients who present with skin pigmentation and muscle weakness.
Aged ; Argyria* ; Basement Membrane ; Biopsy ; Diagnosis, Differential ; Eating ; Emergency Service, Hospital ; Female ; Fever ; Forehead ; Humans ; Muscle Weakness* ; Muscles ; Muscular Diseases ; Myofibrils ; Myositis ; Neural Conduction ; Nose ; Pathology ; Pigmentation Disorders ; Polyneuropathies ; Pyelonephritis ; Rehabilitation ; Silver ; Skin ; Skin Pigmentation ; Steroids ; Sweat Glands ; Thigh