Pseudohypoaldosteronism type 1 is a genetic renal tubular disease of salt wasting, presenting in young infants. Tubular unresponsiveness to elevated endogenous and exogenous aldosterone is the suggested pathogenetic mechanism. Oral sodium chloride supplementation relieve the clinical symptoms and electrolyte distrubances. We experienced 2 cases of PHA type 1 in 38-day and 45-day old male infants who were presented with failure to thrive, vomiting and/or dehydration. Laboratory data showed hyponatremia, hyperkalemia, hypochloremia and metabolic acidosis. Renal and adrenal functions were normal. Plasma renin activity and plasma aldosterone concentration were markedly elevated. Under the diagnosis of pseudohypoaldosteronism type 1, oral supplementation of NaCl and/or kayexalate improved the clinical states of the patients.
Acidosis ; Aldosterone ; Dehydration ; Diagnosis ; Failure to Thrive ; Humans ; Hyperkalemia ; Hyponatremia ; Infant ; Male ; Plasma ; Pseudohypoaldosteronism* ; Renin ; Sodium Chloride ; Vomiting

A case of myofibroblastoma of the breast in a 55-year-old man is described. Myofibroblastoma is a relatively recently recognized benign stromal tumor, and predominantly occurs in middle aged men. The pateint presented with a nontender firm mass in his right breast. Fine needle aspiration biopsy revealed bland looking stromal cell clusters without epithelial cells. Simple excision was done and the patient discharged uneventfully. The mass was well demarcated, lobulated and sligtly myxoid. Microscopically bipolar elongated spindle cell fascicles with interspersing broad collagen bands are so characteristic. Ultrastructurally the tumor cell show features of fibroblast as well as smooth muscle cell.
Male ; Humans ; Biopsy

Improvement of lichen planus was achieved by 11 weeks of daily oral treatment with griseofulvin. The patient was 59-year-old male, has had hypertension and diabetes mellitus for 9 months, and history of various drug intake to these diseses for 6 months. Four months before first visit, symptome of lichen planus had developed suddenly. Because of treatment failure of oral antihistamine and topical steroid for 6 weeks, we began to use griseofulvin. Praspective studies are needed to better assess the affectiveness of griseafulvin in the treatment of lichen planus.
Diabetes Mellitus ; Griseofulvin* ; Humans ; Hypertension ; Lichen Planus* ; Lichens* ; Male ; Middle Aged ; Treatment Failure

Reye syndrome is a rapidly progressive encephalopathy with hepatic dysfunction, which often begins several days after apparent recovery from a viral illness, especially varicella or influenza A or B. Salicylate use was identified as a major precipitating factor for the development of Reye syndrome. With the recommendation to avoid use of salicylates in children, Reye syndrome has virtually disappeared in recent years. We report a case of Reye syndrome in a 5-month-old infant who had been treated with intravenous immunoglobulin and aspirin under the diagnosis of Kawasaki disease, and showed symptoms of sudden onset of irritability, rigidity, decreased activity, vomiting, poor appetite, lethargy, liver dysfunction without jaundice, coagulopathy, and hyperammonemia.
Appetite ; Aspirin ; Chickenpox ; Child ; Humans ; Hyperammonemia ; Immunoglobulins ; Infant ; Influenza, Human ; Jaundice ; Lethargy ; Liver Diseases ; Mucocutaneous Lymph Node Syndrome ; Precipitating Factors ; Reye Syndrome ; Salicylates ; Vomiting

BACKGROUND: Beta-adrenergic receptor Kinase 1(betaARK1) is a serine/threonine kinase attached, which inhibits the coupling of beta-adrenergic receptor with G-protein. Myocardial betaARK1 level is usually elevated in heart failure and hypertrophy, but it is not known whether the circulating betaARK1 level is related with the degree of cardiac hypertrophy. This study was performed to evaluate the association of the betaARK1 level in circulating mononuclear leukocytes(MNL) in untreated hypertension with left ventricular mass in hypertensive patients. Method: Nineteen non-treated hypertensive patients were included for this study. High blood pressure was confirmed when systolic BP is over 150 mmHg or diastoli BP is over 95 mmHg. Echocardiography was performed to evaluate the degree of hypertrophy by measuring the left ventricular mass index(LVMI) and relative wall thickness(RWT), and test the LV function by measuring the ejection fraction(EF) according to ASE guideline. At the same time, blood was collected from each patient and MNL were isolated by gradient centrifuge with Ficoll-400. Total RNA was purified from MNL and semi-quantitative RT-PCR was performed. After reverse transcription, PCR was done with primers for human betaARK1 and GAPDH as external control. betaARK1 levels were expressed by ratio to GAPDH level and estimated the relations with clinical and Echocardiographic parameters. Result: We studied confirmed 19 hypertensive patients(10 men and 9 women, mean age of 50.6 years). Echocardiographically measured indices(mean+/-SD) were as follows; LVMI(137.3+/-30.6g/m2), PWT(0.53+/-0.09) and EF(54.6+/-8.5%). Ratio of betaARK1 levels to GAPDH was from 0.10 to 0.96 (0.62+/-0.25). betaARK1 levels were correlated with LVMI(correlation coefficient: r=.502, p=.029) and RWT(r=.627, p=.004). But Systolic BP(r=0.009, p=.93), diastolic BP(r=.07, p=.85) or EF(r=.045, p=.84) were not related to level of betaARK1. CONCLUSIONS: The betaARK1 level of circulating MNL was correlated well with the degree of the cardiac hypertrophy estimated by LVMI and RWT. This data suggests that activation of sympatho-adrenal system would exert a major role in developing cardiac hypertrophy and we can expect the decreased responsiveness to catecholamine in the heart of hypertensive patients. betaARK1 in circulating MNL might be used as a predictor or marker for LV hypertrophy in hypertensive patients.
beta-Adrenergic Receptor Kinases ; Cardiomegaly ; Echocardiography ; Female ; GTP-Binding Proteins ; Heart ; Heart Failure ; Humans ; Hypertension ; Hypertrophy ; Leukocytes, Mononuclear* ; Male ; Phosphotransferases ; Polymerase Chain Reaction ; Reverse Transcription ; RNA

The inhibitory effect of rice(Oryza sartiva L., illpumbyeo) against mutagenicity induced by tryptophan pyrolysates were investigated using Salmonella typhimurium reversion assay. Both methanol extracts of obtained from brown and white rice were found to possess strong activites of inhibiting the mutagenicities of 3-amino-1,4-dimethyl-5H-pyriod[4,3-b]indol(Trp-P-1) and 3-amino-1-methyl-5H-pyrido[4,3-b]indol(Trp-P-2) on Salmonella typhimurium reversion assay. As the concentration of methanol extract increased, inhibitory effect on mutagenicity increased but reached at steady state as inhibition rate of 90% when the concentration was above 10mg/plate. There was no significant difference(p>0.05) in inhibitory effect of methanol extracts between brown and white rice against tryptophan pyrolysates.
Methanol ; Salmonella typhimurium ; Tryptophan*

Most familial adenomatous polyposis (FAP) patients undergo total colectomy, but duodenal polyposis develops in up to 90% of patients with FAP and a 4% to 18% risk of duodenal and ampullary cancer remains. Laparoscopic pancreas-preserving near total duodenectomy is thought to be a potential option and can be an effective approach to preserve the pancreas. A 48-year-old male patient, who underwent laparoscopic total colectomy with end ileostomy because of FAP with colorectal cancer, was diagnosed with a 20 mm-sized duodenal adenoma in the second to the third portion. The operation was performed on December 27, 2021. Near total duodenectomy was done and type II Billroth gastrojejunostomy was done. Laparoscopic pancreas-sparing duodenectomy is shown to be safe, with favorable short-term oncologic outcome compared to laparoscopic pancreatoduodenectomy in terms of less blood loss, faster recovery time, and much less total cost.

Endometrial cancer is increasing in South Korea. In young women, endometrial cancer can be treated by progestins for preserving fertility. We experienced a successful case of twin pregnancy after conservative therapy of endometrial cancer with Megestrol acetate. Ovulation induction and intrauterine insemination was done. A brief review of related literature was done.
Endometrial Neoplasms* ; Female ; Fertility ; Humans ; Insemination ; Korea ; Megestrol Acetate ; Ovulation Induction ; Pregnancy ; Pregnancy, Twin* ; Progestins ; Twins*

Left atrial myxoma is well-known mimicker of systemic vasculitis. In general, however, these patients showed some symptoms and signs of cardiovascular disease. We experienced a patient with left atrial myxoma who first presented as vasculitis without any symptoms and signs suggesting cardiovascular disorders. A 45 year-old lady showed tender skin nodules, livedo reticuiaris-like skin lesions, severe constitutional symptoms, multiple cerebral infarctions, and elevated ESR and globulin. Skin biopsy findings were thought to be consistent with polyarteritis nodosa. Echocardiography was performed as a routine test for evaluating suspected vasculitis patients and it revealed left atrial myxoma. However, we were not able to detect any auscultation abnormality, even after echocardiography, After echocardiography, we found the tumor emboli in skin biopy specimen with deeper section. This case demonstrated the importance of suspecting the possibility of left atrial myxoma when performing diagnostic work-up for vasculitis.
Auscultation ; Biopsy ; Cardiovascular Diseases ; Cerebral Infarction ; Echocardiography ; Humans ; Middle Aged ; Myxoma ; Polyarteritis Nodosa ; Skin ; Systemic Vasculitis ; Vasculitis

A 43-year-old female presented with persistent headache and dizziness which had first occurred two years earlier. The physical and neurological findings at admission were unremarkable, though plain radiography revealed the presence of a dense calcified mass in the left frontal area, and CT showed that a homogeneous high-density nodule was attached to the inner surface of the left frontal skull. The hard bony mass found and excised during surgery was shown at histopathologic examination to be a subdural osteoma. We describe the clinicopathologic findings of this entity and discuss the radiological features which suggest its subdural location.
Adult ; Case Report ; Female ; Frontal Bone/*pathology/*radiography/surgery ; Human ; Osteoma/*pathology/*radiography/surgery ; Skull Neoplasms/*pathology/*radiography/surgery ; Subdural Space/pathology/radiography/surgery ; Tomography, X-Ray Computed