No abstract available.
Hyperplasia* ; Islets of Langerhans* ; Nesidioblastosis*

DiGeorge syndrome is the developmental anomalies of the third and fourth pharngeal pouches. Recently, damages or abnormal development of the neural crest is suggested as a possible pathogenetic factor, because neural crest cells play a crucial role in development of pharyngeal pouch derivatives, e.g. thymus and parathyroid glands, as well as the aortic arches and conotruncal part of the heat. Most cases have abnormal findings of chromosome 22 and are sporadic, but familial cases have been described. Typical features of DiGeorge syndrome are congenital heart disease, aplasia or hypoplasia of the thymus and parathyroid glands and facial dysmorphism. The main problems and cause of death are severe congestive heart failure due to cardiac anomlies, hypocalcemic complications or immunocompromised conditions. As these results, most cases were expired at infantal period or early childhood. Recently, we have a case of Digeorge syndrome which was associated with complex cardiovascular anomalies(tetralogy of Fallot, atrial septal defect, right aortic arch, left hemitruncus), severe hypocalcemia, aplasia of thymus and facial dysmorphism.
Aorta, Thoracic ; Cause of Death ; Chromosomes, Human, Pair 22 ; DiGeorge Syndrome* ; Heart Defects, Congenital ; Heart Failure ; Heart Septal Defects, Atrial ; Hot Temperature ; Humans ; Hypocalcemia ; Infant ; Neural Crest ; Parathyroid Glands ; Thymus Gland

With the global spread of severe acute respiratory syndrome coronavirus 2, several vaccines were developed; messenger RNA (mRNA) vaccines have recently been widely used worldwide. However, the incidence of myocarditis following mRNA vaccination is increasing;although the cause of myocarditis has not yet been clearly identified, it is presumed to be caused by a problem in the innate immune system. Immune-mediated thrombocytopenia (ITP) after vaccination is rare but has been reported and is also assumed to occur by the same mechanism. We report the first case of simultaneous myocarditis and ITP after mRNA vaccination. A 38-year-old woman presented with chest pain, mild dyspnea, and sweating after vaccination with mRNA-1273 vaccine (Moderna) 4 days prior to admission.Upon admission to the emergency department, cardiac enzymes were elevated; blood test performed 5 months ago showed normal platelet count, but severe thrombocytopenia was observed upon admission. After administration of intravenous immunoglobulin, the platelet count improved; subsequently, myocarditis was observed on endomyocardial biopsy. Thus, myocarditis and ITP were judged to have occurred simultaneously due to the expression of the innate immune system markers after mRNA vaccination. The patient was discharged on day 6 of admission.

The main mechanism of Takotsubo cardiomyopathy (TCM) is catecholamine-induced acute myocardial stunning. Pheochromocytoma, a catecholamine-secreting tumor, can cause several cardiovascular complications, including hypertensive crisis, myocardial infarction, toxic myocarditis, and TCM. A 29-year-old woman presented to our hospital with general weakness, vomiting, dyspnea, and chest pain. The patient was nullipara, 28 weeks’ gestation, and had a cachexic morphology. Her cardiac enzyme levels were elevated and bedside echocardiography showed apical akinesia, suggesting TCM. The next day, she could not feel the fetal movement, and an emergency cesarean section was performed. After delivery, the patient experienced cardiac arrest and was transferred to the intensive care unit for cardiopulmonary resuscitation (CPR). Spontaneous circulation returned after 28 minutes of CPR, but cardiogenic shock continued, and extracorporeal membrane oxygenation (ECMO) was initiated. On the third day of ECMO maintenance, left ventricular ejection fraction improved and blood pressure stabilized. On the eighth day after ECMO insertion, it was removed. However, complications of the left leg vessels occurred, and several surgeries and interventions were performed. A left adrenal gland mass was found on computed tomography and was removed while repairing the leg vessels. Pheochromocytoma was diagnosed and left adrenalectomy was performed.

The main mechanism of Takotsubo cardiomyopathy (TCM) is catecholamine-induced acute myocardial stunning. Pheochromocytoma, a catecholamine-secreting tumor, can cause several cardiovascular complications, including hypertensive crisis, myocardial infarction, toxic myocarditis, and TCM. A 29-year-old woman presented to our hospital with general weakness, vomiting, dyspnea, and chest pain. The patient was nullipara, 28 weeks’ gestation, and had a cachexic morphology. Her cardiac enzyme levels were elevated and bedside echocardiography showed apical akinesia, suggesting TCM. The next day, she could not feel the fetal movement, and an emergency cesarean section was performed. After delivery, the patient experienced cardiac arrest and was transferred to the intensive care unit for cardiopulmonary resuscitation (CPR). Spontaneous circulation returned after 28 minutes of CPR, but cardiogenic shock continued, and extracorporeal membrane oxygenation (ECMO) was initiated. On the third day of ECMO maintenance, left ventricular ejection fraction improved and blood pressure stabilized. On the eighth day after ECMO insertion, it was removed. However, complications of the left leg vessels occurred, and several surgeries and interventions were performed. A left adrenal gland mass was found on computed tomography and was removed while repairing the leg vessels. Pheochromocytoma was diagnosed and left adrenalectomy was performed.

The main mechanism of Takotsubo cardiomyopathy (TCM) is catecholamine-induced acute myocardial stunning. Pheochromocytoma, a catecholamine-secreting tumor, can cause several cardiovascular complications, including hypertensive crisis, myocardial infarction, toxic myocarditis, and TCM. A 29-year-old woman presented to our hospital with general weakness, vomiting, dyspnea, and chest pain. The patient was nullipara, 28 weeks’ gestation, and had a cachexic morphology. Her cardiac enzyme levels were elevated and bedside echocardiography showed apical akinesia, suggesting TCM. The next day, she could not feel the fetal movement, and an emergency cesarean section was performed. After delivery, the patient experienced cardiac arrest and was transferred to the intensive care unit for cardiopulmonary resuscitation (CPR). Spontaneous circulation returned after 28 minutes of CPR, but cardiogenic shock continued, and extracorporeal membrane oxygenation (ECMO) was initiated. On the third day of ECMO maintenance, left ventricular ejection fraction improved and blood pressure stabilized. On the eighth day after ECMO insertion, it was removed. However, complications of the left leg vessels occurred, and several surgeries and interventions were performed. A left adrenal gland mass was found on computed tomography and was removed while repairing the leg vessels. Pheochromocytoma was diagnosed and left adrenalectomy was performed.

The main mechanism of Takotsubo cardiomyopathy (TCM) is catecholamine-induced acute myocardial stunning. Pheochromocytoma, a catecholamine-secreting tumor, can cause several cardiovascular complications, including hypertensive crisis, myocardial infarction, toxic myocarditis, and TCM. A 29-year-old woman presented to our hospital with general weakness, vomiting, dyspnea, and chest pain. The patient was nullipara, 28 weeks’ gestation, and had a cachexic morphology. Her cardiac enzyme levels were elevated and bedside echocardiography showed apical akinesia, suggesting TCM. The next day, she could not feel the fetal movement, and an emergency cesarean section was performed. After delivery, the patient experienced cardiac arrest and was transferred to the intensive care unit for cardiopulmonary resuscitation (CPR). Spontaneous circulation returned after 28 minutes of CPR, but cardiogenic shock continued, and extracorporeal membrane oxygenation (ECMO) was initiated. On the third day of ECMO maintenance, left ventricular ejection fraction improved and blood pressure stabilized. On the eighth day after ECMO insertion, it was removed. However, complications of the left leg vessels occurred, and several surgeries and interventions were performed. A left adrenal gland mass was found on computed tomography and was removed while repairing the leg vessels. Pheochromocytoma was diagnosed and left adrenalectomy was performed.

OBJECTIVES: In the third trimester hypertensive pregnancies, we would like to evaluate effects of white coat hypertension, severity of hypertension and diurnal variation of blood pressure on the fetal outcome by using 24- hour ambulatory blood pressure monitoring. METHODS: Hypertensives(n=50) and normotensives (n=14) in the third trimester of the pregnancy underwent 24-hour ambulatory blood pressure monitoring. We excluded hypertensives(n=5) who became pre-eclampsia patients. Hypertensives(n=45) were classified as white coat hypertensives(n=14, mean ambulatory blood pressure <139/87mmHg) and sustained hypertensives(n=31). Sustained hypertensives(n=31) were divided as moderate to severe hypertensives(n=5, systolic blood pressure >160 mmHg or diastolic blood pressure >100mmHg) and mild hypertensives(n=26). Sustained hypertensives were also divided into two groups which had diurnal variation of blood pressure or not. To exclude effects of hypertension severity, effects of diurnal variation were evaluated in hypertensives with similar mean arterial blood pressure. Gestational age, body weight, body weight for gestational age were used as parameters of the fetal outcome. RESULTS: 1) The prevalence of white coat hypertension was 28%(14/50). 2) There were no significant differences in the fetal outcome between normotensives(n=14) and white coat hypertensives(n=14). 3) Body weight of fetus and body weight for gestational age in moderate to severe hypertensives(n=5) were less than those of mild hypertensives(n=26), but gestational age was not significantly different between two groups. 4) Body weight of fetus and body weight for gestational age in sustained hypertensives without diurnal variation(n=10) were less than those with diurnal variation(n=8), but gestational age was not significantly different between two groups. 5) All hypertensives who became pre-eclampsia (n=5) were severe hypertensives and had no diurnal variation of blood pressure. CONCLUSION: White coat hypertension in the third trimester was quite often and did not affect on the fetal outcome. The more severe hypertension and/or absence of diurnal variation of blood pressure caused poor fetal outcome. Patients who became pre-eclampsia were severe hypertensives and had no diurnal variation of blood pressure. Ambulatory blood pressure monitoring may have several roles in the antenatal management of hypertenison.
Arterial Pressure ; Blood Pressure ; Blood Pressure Monitoring, Ambulatory* ; Body Weight ; Female ; Fetus ; Gestational Age ; Humans ; Hypertension ; Pre-Eclampsia ; Pregnancy Trimester, Third ; Pregnancy* ; Prevalence ; White Coat Hypertension

BACKGROUND AND OBJECTIVES: The overriding influence of physical activity and the diurnal variation during ambulatory blood pressure monitoring (ABPM) has been well demonstrated. We prospectively evaluated the usefulness of electronic activity monitoring for deriving the actual physical activity and the diurnal variation of ABPM. SUBJECTS AND METHODS: 24-hour ABPM with using an electronic activity monitor was performed on ten normotensive volunteers and fifteen hypertensive subjects. To interpret the ABPM results of the fifteen hypertensive subjects, we obtained the actual awake/sleep periods of every subject with using an electronic activity monitor. RESULTS: The activity values obtained from the ten normotensive volunteers correlated well with the values of the blood pressure (BP) and the heart rate. In the hypertensives, the nocturnal mean BP derived by the actual period was significantly lower than that derived by the arbitrary period (130+/-16/81+/-13 mmHg versus 124+/-13/77+/-12 mmHg, respectively, p<0.05). The nocturnal BP fall derived by the actual period was significantly larger than that derived by the arbitrary period (14.9+/-8.7/11.6+/-7.5 mmHg versus 21.1+/-8.6/16.2+/-7.4 mmHg, respectively, p<0.01). Four among the 7 non-dippers determined by the arbitrary period were re-classified as dippers when the actual period was used. One among the 13 hypertensives, as determined by the arbitrary period, was also re-classified as a non-hypertensive. CONCLUSION: The electronic activity monitor was able to determine the actual activity level. The interpretation of ABPM may be altered by the use of the electronic activity monitor. These results suggest that the accuracy and reproducibility of the 24-hour ABPM will be improved by using an electronic activity monitor.
Blood Pressure ; Blood Pressure Monitoring, Ambulatory* ; Heart Rate ; Motor Activity ; Prospective Studies ; Volunteers

Coronary artery fistulae are usually identified during invasive coronary angiographies. However, in this case, we made the early detection of coronary artery fistulae during non-invasive transthoracic echocardiography, by demonstrating diastolic multiple abnormal color Doppler flows on the entire left ventricular walls including left ventricular free wall, interventricular septum and apex, which were mimicking firecracker on the whole left ventricle. Fistulous communication from the coronary artery to the left ventricle is rare. Moreover, a case of multiple coronary fistulae emptying into the left ventricle through the entire left ventricular walls including left ventricular free wall, interventricular septum and apex is uncommon. We report a case of a 31-year-old woman who was diagnosed with multiple fistula communicating with entire left ventricular wall.
Adult ; Coronary Angiography ; Coronary Vessels ; Echocardiography ; Female ; Fistula ; Heart Ventricles ; Humans