This study was performed to assess the accuracy of pulsed Doppler echocardiographic(PDE) diagnosis of ventricular septal defect(VSD). 119 children with congenital heart disease, aged 9 months to 17 years, admitted to our hospital for cardiac surgery, were studied. These children had VSD(56), ASD(18), PDA(43), PS(1) and Cortriatrium(1). A specific PDE diagnosis of VSD can be made by following the turbulent VSD jet(TVJ) through the septum, and a PDE diagnosis of VSD was made in 53 out of 56 patients(95%) proven by catheterization and operation. There were two false positive PDE diagnosis of VSD by PDE, were proven to be perimembranous VSD at operation, and 3 children who were classified into subarterial infundibular VSD by PDE, were proven to be perimembranous VSD at operation. PDE seems to be a very useful tool in preoperative evaluation of VSD.
Catheterization ; Catheters ; Child ; Diagnosis* ; Echocardiography, Doppler, Pulsed* ; Heart Defects, Congenital ; Heart Septal Defects, Ventricular* ; Humans ; Thoracic Surgery

Ambulatory BP monitoring was performed in a patients with pheochromocytoma before and after removal of the tumor. Before surgery, it did not show any significant diurnal variation. But, after surgery the diurnal variation was restored.
Blood Pressure Monitoring, Ambulatory* ; Blood Pressure* ; Humans ; Pheochromocytoma*

Extramammary Paget's disease is a rare cutaneous malignancy and frequently associated with an underlying adnexal carcinoma and perhaps with underlying internal malignancy. In our case, genital Paget's disease was followed by the discovery of renal cell carcinoma. We report a rare case of extramammary paget´s disease of the penis and scrotum which associated with a renal cell carcinoma.
Carcinoma, Renal Cell* ; Male ; Paget Disease, Extramammary* ; Penis* ; Scrotum*

Angiokeratoma circumscriptum is present at birth or early childhood and is an uncommon dermatosis characterized by papules and small nodules that may coalesce to form plaques. Histopathologically, there are varying degrees of hyperkeratosis, papillomatosis, and irregular acanthosis. In the papillary dermis, greatly dilated capillaries are observed, The acanthotic epidermis encircles the vascular spaces(blood cysts) where, occasionally, organized thrombi may be found. The use of pulsed-dye lasers to treat cutaneous vascular lesions is based on the theory of selective photothermolysis. We report a case of an angiokeratoma circumscriptum in an 48-year-old woman for whom the flashlamp pulsed tunable dye laser proved to be a highly successful means of treatment.
Angiokeratoma* ; Capillaries ; Dermis ; Epidermis ; Female ; Humans ; Lasers, Dye* ; Middle Aged ; Papilloma ; Parturition ; Skin Diseases

Isolated deficiency of 3-methylcrotonyl CoA carboxylase is a rare disorder of the catabolic pathway for leucine and many patients have mild symptoms or no symptom. However, the introduction of tandem mass spectrometry in newborn screening has revealed an unexpectedly high incidence of this disorder. We report an asymptomatic premature infant with isolated 3-methylcrotonyl CoA carboxylase deficiency detected by newborn screening program using tandem mass spectrometry. She was born at preterm, 36 weeks of gestation and her birth weight was 1,912 gm. She was delivered by Cesarian section due to maternal preeclampsia and oligohydramnios. An elevation of 3-hydroxyisovalerylcarnitine in a blood sample obtained at Seven days was detected by tandem mass screening. Massively elevated excretion of 3-hydroxyisovalerate and 3-methylcrotonylglycine was detected in the urine collected at 15 days. L-carnitine(100 mg/kg/day) was administrated orally to correct sencondary carnitine deficiency. Carnitine is conjugated with metabolites, to decrease the potential toxic effects. She is asymptomatic to date, and her growth and development are within normal limits.
Birth Weight ; Carnitine ; Female ; Growth and Development ; Humans ; Incidence ; Infant, Newborn* ; Infant, Premature ; Leucine ; Mass Screening* ; Oligohydramnios ; Pre-Eclampsia ; Pregnancy ; Tandem Mass Spectrometry*

Transient hyperammonemia of the newborn is an overwhelming disease manifestated by hyperammonemic coma in ill premature infant. This recognized metabolic disorder is chiefly characterized by severe hyperammonemia in the postnatal period, a comatous state, absence of abnormal organic aciduria, normal activity of urea cycle enzymes and, usually, complete recovery. The etiology is unknown. Infant had mild respiratory distress that progressed within 48 hours to deep coma requiring ventilatory assistance and had marked hyperammonemia. The degree of neurologic impairment and developmental delay in this disorder depends on the duration of the hyperammonemic coma. So, treatment of hyperammonemia should be initiated promptly and continued vigorously. We report of a preterm infant (34+5 weeks of gestation) presenting with respiratory distress, seizure, coma, and marked elevated plasma ammonia level.
Ammonia ; Coma ; Humans ; Hyperammonemia* ; Infant ; Infant, Newborn* ; Infant, Premature ; Plasma ; Seizures ; Urea

An increase in the dose of chemotherapy enhances the response of many experimental and clinical cancers, but the extent of chemotherapy dose escalation and repeated use is often limited by myelosuppression. The side effects of chemotherapy including bleeding and infection due to myelosuppression have resulted in delayed therapy and a reduction in the therapeutic dose, therefore it is necessary to overcome myelosuppression especially leukopenia in patients with gynecologic malignancies who recieved chemotherapy. This study is undertaken to investigate the clinical effects of rhG-CSF(recombinant human Granulocyte-colony stimulating factor) in 29 patients with gynecologic malignancy who recieved chemotherapy. It was given at a dose of 100 microgram bid/day subcutaneously until significantly increase of leukocyte count in leukopenic patient. The results showed, the rhG-CSF has significantly increased the number and function of leukocyte. The use of rhG-CSF was effective and useful to treat chemotherapy induced leukopenia and to accelerate the recovery from this complications.
Drug Therapy* ; Hemorrhage ; Humans* ; Leukocyte Count ; Leukocytes ; Leukopenia*

Post-micturition dribble is defined as leakage coming after voiding has been completed. Such leakage is often associated with bending or sitting down, and is seldom associated with any demonstrable abnormality. The diagnosis can be made by videourodynamic study. The patient is instructed to press the bulbar urethra manually in the perineum after micturition and evacuate the residue, and symptomatic relief is almost always obtained. We report three cases of patient with postmicturition dribble.
Diagnosis ; Humans ; Perineum ; Urethra ; Urination

CD80 is mainly expressed on Ag-presenting cells (APCs) as a costimulatory molecule but is also detected on T cells. However, the origin and physiological role of CD80 on CD8⁺ T cells remain unclear. In the present study, we demonstrated that effector and memory CD8⁺ T cells, but not naïve CD8⁺ T cells, displayed CD80 molecules on their surfaces after acute lymphocytic choriomeningitis virus infection. Using adoptive transfer of CD80-knockout (KO) CD8⁺ T cells into a wild type or CD80-KO recipient, we demonstrated that the effector CD8⁺ T cells displayed CD80 by both intrinsic expression and extrinsic acquisition, while memory CD8⁺ T cells displayed CD80 only by extrinsic acquisition. Interestingly, the extrinsic acquisition of CD80 by CD8⁺ T cells was observed only in the lymphoid organs but not in the periphery, indicating the trogocytosis of CD80 molecules via interaction between CD8⁺ T cells and APCs. We compared the recall immune responses by memory CD8⁺ T cells that either extrinsically acquired CD80 or were deficient in CD80, and found that CD80, presented by memory CD8⁺ T cells, played a role in limiting their expansion and IL-2 production upon exposure to secondary challenge. Our study presents the in vivo dynamics of the extrinsic acquisition of CD80 by Ag-specific CD8⁺ T cells and its role in the regulation of recall immune responses in memory CD8+ T cells.
Adoptive Transfer ; Antigens, CD80 ; Interleukin-2 ; Lymphocytic choriomeningitis virus ; Memory ; T-Lymphocytes

Background: Clostridioides difficile infection (CDI) is the most essential cause of antimicrobial-related diarrhea in hospitalized patients. It is also considered as an important hospital-acquired infection. Adequately performing the laboratory diagnostic tests for CDI is critical for the control and treatment of CDI in healthcare facilities. This study focused on the management of the extra-analytical phase to secure quality assurance of the diagnostic tests for CDI. Methods: We analyzed the volume and pattern of requested C. difficile - related tests from patients in 2018. The culture-positive rate was compared with the stool quality. Finally, the clinical characteristics of antibiotic-related diarrhea were compared based on whether toxigenic C. difficile was isolated. Results: Culture plus toxin enzyme immunoassay was the most common pattern; however, it showed low positive rates for toxigenic C. difficile . The culture-positive rates were not different according to the Bristol stool form scale. The significant risk factors for the detection of toxigenic C. difficile in patients with antibiotic-associated diarrhea were inhibitor-combined penicillin or cephalosporin (odds ratio [OR], 5.060; 95% confidence interval [CI], 1.317–19.447; P =0.0183) and extended-spectrum cephalosporin (OR, 16.224; 95% CI, 3.166–83.134; P =0.0008). Conclusions The pre-pre-analytical errors are easy to overlook in routine laboratory procedures. Our findings could provide a good example of the management of the extra-analytical phase to secure quality assurance of the diagnostic tests for CDI.