Migraine equivalents include cyclic vomiting, acute confusional states, and benign paroxysmal vertigo. Cyclic vomiting is characterized by recurrent, sometimes monthly, boults of severe vomiting that may be so intense dehydration and electrolyte abnormalities occur, particularly in an infact. We experienced a 3-year-old girl who showed manifestations of cyclic vomiting and abdominal pain. We report this case with a review of related literatures.
Abdominal Pain ; Child, Preschool ; Dehydration ; Female ; Humans ; Migraine Disorders* ; Vertigo ; Vomiting

Gap junctions, which mediate rapid intercellular communication, consist of connexins, small transmembrane proteins that belong to a large family of proteins found throughout the species. Mutations in the GJB2 gene, encoding Connexin 26, can cause nonsyndromic autosomal recessive or dominant hearing loss with or without skin manifestations. A 3-yr-old Korean female and her mother presented to our clinic with diffuse hyperkeratosis of the palms and soles (May 3, 2007). Skin biopsies from the soles of both patients demonstrated histopathological evidence of palmoplantar keratoderma. The patient and a number of her maternal family members also had congenital hearing loss. The combination of congenital hearing loss and palmoplantar keratoderma, inherited as an autosomal dominant trait, led us to test for a mutation in the GJB2 gene in both patients. The results showed the R75W mutation of the GJB2 gene in both. In conclusion, the simultaneous occurrence of a GJB2 mutation in a mother and daughter suggests that R75W mutation cause autosomal dominant hearing loss presenting with palmoplantar keratoderma. To the best of our knowledge, this is the first report of a GJB2 mutation associated with syndromic autosomal dominant hearing loss and palmoplantar keratoderma in a Korean family.
Adult ; Alleles ; Amino Acid Substitution ; Child, Preschool ; Connexins/*genetics ; DNA Mutational Analysis ; Deafness/complications/*genetics ; Female ; Humans ; Keratoderma, Palmoplantar/complications/*genetics/pathology ; *Mutation ; Pedigree ; Skin/pathology

Pseudohypoparathyroidism (PHP) is a rare clinical type of hypoparathyroidism. The patients with PHP show classic clinical and biochemical features of hypoparathyroidism, but elevated serum level of parathyroid hormone (PTH) and characteristic physical appearances termed 'Albright's hereditary osteodystrophy' (AHO). PHP is classified into types Ia, Ib, Ic and II according to the presence of AHO phenotype and the mechanism of PTH resistance. We experienced a case of PHP in a 12 year-old girl with carpopedal spasm, syncope and partial AHO. She showed very low serum calcium level (1.4 mmol/L), high phosphorus level (3.62 mmol/L) and high immunoreactive PTH level (186.6 ng/L). In the Ellsworth-Howard test, urinary cyclic adenosine monophosphate and phosphorus levels after an exogenous PTH injection remained unchanged. Therefore, we were able to classify the patient as either PHP type Ia or Ic. After the patient had been treated with daily calcium carbonate (1 g), cholecalciferol (250 IU) and calcitriol (0.5 microgram), her neurological signs and symptoms as well as her biochemical abnormalities of hypocalcemia and hyperphosphatemia were improved.
Adenosine Monophosphate ; Calcitriol ; Calcium ; Calcium Carbonate ; Child ; Cholecalciferol ; Female ; Humans ; Hyperphosphatemia ; Hypocalcemia ; Hypoparathyroidism ; Parathyroid Hormone ; Phenotype ; Phosphorus ; Pseudohypoparathyroidism* ; Spasm ; Syncope

Pseudohypoparathyroidism (PHP) is a rare clinical type of hypoparathyroidism. The patients with PHP show classic clinical and biochemical features of hypoparathyroidism, but elevated serum level of parathyroid hormone (PTH) and characteristic physical appearances termed 'Albright's hereditary osteodystrophy' (AHO). PHP is classified into types Ia, Ib, Ic and II according to the presence of AHO phenotype and the mechanism of PTH resistance. We experienced a case of PHP in a 12 year-old girl with carpopedal spasm, syncope and partial AHO. She showed very low serum calcium level (1.4 mmol/L), high phosphorus level (3.62 mmol/L) and high immunoreactive PTH level (186.6 ng/L). In the Ellsworth-Howard test, urinary cyclic adenosine monophosphate and phosphorus levels after an exogenous PTH injection remained unchanged. Therefore, we were able to classify the patient as either PHP type Ia or Ic. After the patient had been treated with daily calcium carbonate (1 g), cholecalciferol (250 IU) and calcitriol (0.5 microgram), her neurological signs and symptoms as well as her biochemical abnormalities of hypocalcemia and hyperphosphatemia were improved.
Adenosine Monophosphate ; Calcitriol ; Calcium ; Calcium Carbonate ; Child ; Cholecalciferol ; Female ; Humans ; Hyperphosphatemia ; Hypocalcemia ; Hypoparathyroidism ; Parathyroid Hormone ; Phenotype ; Phosphorus ; Pseudohypoparathyroidism* ; Spasm ; Syncope

BACKGROUND: Dermatitis occurring after ingestion of the urushiol chicken is quite common in Korea. Although many cases of the systemic contact dermatitis due to urushiol chicken have already been reported, systemic study on its epidemiology is not done yet. OBJECTIVE: To elucidate the demography of the eaters of the urushiol chicken with its general understandings in society and the incidence of the resultant systemic dermatitis. METHODS: By the help of the patients who developed the dermatitis, all the persons who ate it together with the patients were interviewed. RESULTS AND CONCLUSION: l. 171 eaters of the urushiol chicken(125 male and 46 female) were enrolled in this study(M:F= 3.1:1). The age group in 4th decade was the most common in male, the 5th decade in female. The age prevalence of the systemic contact dermatitis corresponded to the age prevalence of the eater. 2. Incidence rate of systemic contact dermatitis among the eaters was 32.2% (55/171). Incidence rate according to each age group were as follows : 0% in 2nd decade, 33.3% in the 3rd decade, 32.4% in 4th decade, 32.8% in 5th decade, 33.3% in 6th decade, 25% in the 7th decade. 3. One third (57/171) of the eaters ate the urushiol chicken to strengthen their internal organs, especially stomach, which they believed to be weak 70 persons (40.9%) ate it at home, 101 persons (59.1%) ate it at a restaurant. 4. Out of 67 persons having past history of allergic contact dermatitis to Rhus tree, 28 persons (41.8%) showed systemic contact dermatitis. Out of 104 persons without past history of allergy to Rhus, 27 persons (26.0%) showed systemic contact dermatitis.
Chickens ; Demography ; Dermatitis ; Dermatitis, Allergic Contact ; Dermatitis, Contact* ; Eating* ; Epidemiologic Studies* ; Epidemiology ; Female ; Humans ; Hypersensitivity ; Incidence ; Korea ; Male ; Prevalence ; Restaurants ; Rhus* ; Stomach ; Trees

Background: This study was performed to identify acute tinnitus and evaluate the efficacy of steroids for noise-induced acute tinnitus by measuring the gap-prepulse inhibition of the acoustic startle (GPIAS) value in an animal model. Methods: Nineteen rats (the noise group [n = 7] and the noise + dexamethasone [DEX] group [n = 12]) were exposed to narrow-band noise centered at 16 kHz from a sound generator for 4 hours. The noise + DEX group received intraperitoneal steroid administration daily for 5 days (1.5 mg/kg/day) after completing noise exposure. Auditory brainstem response and GPIAS value were measured just prior to, and 1 day after noise exposure and on days 1 and 10 days after completing steroid administration. The changes in cochlear structure were evaluated by histological analysis. Results: The threshold shift was checked 1 and 10 days after intraperitoneal steroid injection, and no differences in threshold shift were observed between the two groups in each frequency except for 32 kHz 1 day after steroid injection. The mean GPIAS value in the noise + DEX group (36.4% ± 14.1%) was significantly higher than that in the noise group (16.4% ± 18.8%) 10 days after intraperitoneal steroid administration (P = 0.017). There were no pathological changes associated with noise trauma in the two groups as determined on hematoxylin and eosin and immunohistochemical staining. Conclusion An acute tinnitus model with minimal structural changes by noise exposure was set up, and used to verify tinnitus objectively by measuring the GPIAS value. Steroid therapy for control of tinnitus was validated in this animal model.

Pituitary apoplexy is a clinical syndrome caused by an acute ischemic or hemorrhagic vascular accident. Although pituitary apoplexy is an emergency, it is often overlooked because it is rare and presents with various signs and symptoms, including those of neurologic and endocrine disorders. We describe a case of pituitary apoplexy misdiagnosed as aseptic meningitis accompanied by acute-onset headache and nausea, followed by fever. Subsequently, it was revealed as pituitary apoplexy by brain magnetic resonance imaging (MRI) performed when the clinical course worsened and ophthalmoplegia developed. We suggest that pituitary apoplexy be included in the differential diagnosis of patients presenting with headache or signs of meningeal irritation; when there is no clinical improvement, prompt steroid treatment should be started, while considering neurological decompression to prevent neurological sequelae.
Brain ; Decompression ; Diagnosis, Differential ; Emergencies ; Fever ; Headache ; Humans ; Hypopituitarism ; Magnetic Resonance Imaging ; Meningitis ; Meningitis, Aseptic ; Nausea ; Ophthalmoplegia ; Pituitary Apoplexy

The purpose of this study was to analyze left ventricular (LV) torsion and untwisting, and to evaluate the correlation between torsion and other components of LV contraction in children with dilated cardiomyopathy (DCM). Segmental and global rotation, rotational rate (Vrot) were measured at three levels of LV using the twodimensional (2D) speckle tracking imaging (STI) method in 10 DCM patients (range 0.6-15 yr, median 6.5 yr, 3 females) and 17 age- and sex-matched normal controls. Global torsion was decreased in DCM (peak global torsion; 10.9+/-4.6degrees vs. 0.3+/-2.1degrees, p<0.001). Loss of LV torsion occurred mainly by the diminution of counterclockwise apical rotation and was augmented by somewhat less reduction in clockwise basal rotation. In DCM, the normal counterclockwise apical rotation was not observed, and the apical rotation about the central axis was clockwise or slightly counterclockwise (peak apical rotation; 5.9+/-4.1degrees vs. -0.9+/-3.1degrees, p<0.001). Systolic counterclockwise Vrot and early diastolic clockwise Vrot at the apical level were decreased or abolished. In DCM, decreased systolic torsion and loss of early diastolic recoil contribute to LV systolic and diastolic dysfunction. The STI method may facilitate the serial evaluation of the LV torsional behavior in clinical settings and give new biomechanical concepts for better management of patients with DCM.
Adolescent ; Cardiomyopathy, Dilated/pathology/*physiopathology ; Child ; Child, Preschool ; Echocardiography, Doppler/methods ; Female ; Humans ; Infant ; Male ; Reproducibility of Results ; Ventricular Dysfunction, Left/pathology/*physiopathology

Recent literatures suggest that pulmonary embolus secondary to renal cell cancer may be more common than previously suspected. A 63-year-old man, who suffered a massive tumor embolism to the right main pulmonary artery after surgery for renal cell carcinoma with vena caval and renal vein invasion, was treated by emergency pulmonary embolectomy using cardiopulmonary bypass. Renal cell carcinoma occasionally extends into the inferior vena cava as a tumor thrombus. In such patients, removal of the tumor thrombus from the inferior vena cava has to be performed in addition to radical nephrectomy. However, the massive pulmonary tumor embolism is a major potential hazard during radical surgical resection. To prevent intraoperative pulmonary embolisms, scheduled use of cardiopulmonary bypass with the cooperation of cardiovascular surgeons is recommended in addition to the standard proximal vena caval occlusion technique of clipping and clamping.
Carcinoma, Renal Cell* ; Cardiopulmonary Bypass ; Constriction ; Embolectomy ; Embolism ; Emergencies ; Humans ; Middle Aged ; Neoplastic Cells, Circulating* ; Nephrectomy ; Pulmonary Artery ; Pulmonary Embolism ; Renal Veins ; Thrombosis ; Vena Cava, Inferior

No abstract available.
Sweet Syndrome*