PURPOSE: The purpose of this study was to describe the clinical outcome after pancreatcetmy and its relationship with pathological appearances and clinical features in patients with persistent hyperinsulinemic hypoglycemia of infancy(PHHI). METHODS: Medical records of 10 patients(9 males and 1 female, mean age:40.4+/-1.5 months) who were diagnosed as PHHI and underwent pancreatectomy from 1988 to 2000 were reviewed. Clincal and biochemical data were recorded. Subjects were classified arbitrarily into early-onset or late-onset group according to age of onset. Pathologic appearance of pancreas was divided into 2 forms:diffuse or focal. The former had a focal pancreatic adenomatous hyperplasia and the latter was characterized by increased number of betacells with similar distribution seen in normal neonates. RESULTS: One patient had focal, and nine had diffuse lesions. After near-total pancreatectomy, 4 patients(40.0%) showed complete response, 4(40.0%) had persistent hypoglycemia, and 2(20.0%) developed diabetes mellitus. As neurological sequelae, 6 patients(60.0%) had persistent seizures, and 6(60.0%) had delayed motor and speech development. No clinical or biochemical factors related to postoperative outcome were found. CONCLUSION: This data indicate that early diagnosis of patients who present with hypoglycemic symptoms in infancy, especially early in life, and development of more effective therapy are warranted, because there is no clinical or biochemical factor predicting final outcome after near-total pancreatectomy and only 40% of patients with PHHI remained euglycemic after surgery with possible severe neurological sequelae.
Age of Onset ; Congenital Hyperinsulinism* ; Diabetes Mellitus ; Early Diagnosis ; Female ; Humans ; Hyperplasia ; Hypoglycemia ; Infant, Newborn ; Male ; Medical Records ; Pancreas ; Pancreatectomy* ; Seizures

Glycogen storage diseases(GSD) are inherited disorders affecting glycogen metabolism and type I GSD is due to the absence or deficiency of glucose-6-phosphatase(G6Pase) enzyme in the liver, kidney, and intestinal mucosa. The defect leads to inadequate hepatic conversion of G6P to glucose and thus make affected individuals susceptible to fasting hypoglycemia, and the accumulation of glycogen occurs in the liver and other organs. Type Ia is the most common form of GSD and clinically growth retardation may manifest of GSD itself rather than growth hormone deficiency(GHD), but we experienced a case of type I GSD with GHD in a 14-year-o1d male. The height was 125 cm, compatible with 50 th percentile of height of 8 years of age. He has doll-like face with fat cheek, relatively thin extremities, and metabolic acidosis, hyperuricemia, hypoglycemia, hyperlipidemia. GH stimulation test with clonidine and L-dopa revealed that the patient had decreased GH secretion. After laboratory work up including liver biopsy, he was diagnosed as type I GSD. Hypoglycemia was managed with frequent feeding with high starch diet(uncooked cornstarch). Metabolic acidosis and hyperuricemia were treated with sodium bicarbonate, allopurinol and probenecid. The patient is being followed at out-patient clinic with clinical improvement after of diet therapy and GH administration.
Acidosis ; Allopurinol ; Biopsy ; Cheek ; Clonidine ; Diet Therapy ; Extremities ; Glucose ; Glycogen Storage Disease* ; Glycogen* ; Growth Hormone* ; Humans ; Hyperlipidemias ; Hyperuricemia ; Hypoglycemia ; Intestinal Mucosa ; Kidney ; Levodopa ; Liver ; Male ; Metabolism ; Outpatients ; Probenecid ; Sodium Bicarbonate ; Starch

BACKGROUND: Our study aimed to make a comparative analysis of clinical outcomes of arthroscopic decompression for rotator cuff calcific tendinitis by location of calcific deposits and by its size. METHODS: We enrolled a total of 38 patients, comprising 39 affected shoulders, who underwent arthroscopic decompression for calcific tendinitis. As our clinical scores, we evaluated the UCLA, the ASES, and the VAS scores and analyzed them by calcific location, by calcific deposit size, by the presence or absence of calcific remnants, and by whether concomitant cuff repair was performed. RESULTS: The clinical scores of those whose calcific deposit had an area greater than 77.0 mm2 and of those whose calcific deposit had an area smaller than 77.0 mm2 did not significantly differ (p=0.21 in ASES; p=0.19 in UCLA; p=0.17 in VAS). Nor did the clinical scores significantly differ with respect to the location of calcification (p=0.23). Further, the clinical scores did not significantly differ between those who had calcific remnants and those who did not and between those who received additional cuff repair and those who did not. CONCLUSIONS: We found that the clinical outcomes after arthroscopic decompression of calcific tendinitis were not significantly associated with the cuff tendon in which the calcium deposits are found; the location of the calcium deposits in the supraspinatus tendon (if found in this tendon); the size of calcific deposits; the presence of calcific remnants; and concomitant cuff repairs.
Calcium ; Decompression ; Humans ; Rotator Cuff ; Shoulder* ; Tendinopathy* ; Tendons

No abstract available.
Bowen's Disease ; Foot

No abstract available.
Cellulitis* ; Dermatitis, Allergic Contact*

A 48 year-old male with back pain and foot drop had been treated. Studies for myelomatosis were all negative. A plain lumbar spin X-ray showed an erosion of the left pedicle of the first lumbar vertebra. A myelography via lumbar and cisternal routes revealed total block with feather appearance at the upper level of the L2 and the lower level of the T12 vertebra. Pathologic evaluation confirmed the diagnosis of solitary myeloma of the bone. After undergoing almost total excision, followed by radiation, the patient was still doing well.
Animals ; Back Pain ; Diagnosis ; Feathers ; Foot ; Humans ; Male ; Middle Aged ; Multiple Myeloma ; Myelography ; Spine*

Blunt trauma of the chest and abdomen frequently result in cardiac injury. We experienced a 47 year-old male patient of myocardial contusion with aortic insufficiency after blunt chest trauma. On the secondd day after admission, the patient developed sudden hypoxemia and wide pulse pressure. A pulmonary arterial catheter was inserted and initial cardiac output and pulmonary capillary wedge pressure was 3.56 L/min/m(2) and 32 mmHg, respectively. There was akinesia of the anterior septum, anterior wall, inferior wall and inferior septum with aortic regurgitation(I/IV), and the ejection fraction was 25% on echocardiogram. Myocardial contusion with valvular injury was suspected. Dobutamine infusion was started and after five days the pulmonary capillary wedge pressure was decreased to 14 mmHg. The ejection fraction was increased to 69% in spite of decreasing dobutamine but aortic regurgitation(II/VI) remained. Therefore echocardiogram and pulmonary artery catheterization will be helpful to diagnose suspected cardiac contusion and for better outcome.
Abdomen ; Anoxia ; Blood Pressure ; Cardiac Output ; Catheterization, Swan-Ganz ; Catheters ; Contusions ; Dobutamine ; Heart ; Humans ; Male ; Middle Aged ; Pulmonary Wedge Pressure ; Thorax*

Peripartum cardiomyopathy is a myocardial disease associated with pregnancy and leading to heart failure. A twenty-eight years old primipara was scheduled for a cesarean section due to a twin pregnancy. She had no previous cardiac or other systemic illness. But periodic episode of sudden hypertension, tachycardia, hypoxemia(PaO2/FiO2=58) and pulmonary edema were developed in the perioperative period. After being transferred to the intensive care unit, mechanical ventilation with positive end expiratory pressure was applied for six days and pulmonary infiltration subsided. However, two days after extubation, acute pulmonary edema redeveloped in both lung fields in spite of fluid restriction. In echocardiogram demonstrated global hypokinesia of the left ventricle with an ejection fraction of 31%. Dobutamine infusion was started and one day after infusion the pulmonary edema subsided. In conclusion echocardiogram would be helpful for diagnosis of peripartum cardiomyopathy and adequate cardiac support with assisted ventilation will improve prognosis.
Cardiomyopathies* ; Cesarean Section ; Diagnosis ; Dobutamine ; Edema ; Female ; Heart Failure ; Heart Ventricles ; Hypertension ; Hypokinesia ; Intensive Care Units ; Lung ; Perioperative Period ; Peripartum Period* ; Positive-Pressure Respiration ; Pregnancy ; Pregnancy, Twin ; Prognosis ; Pulmonary Edema* ; Respiration, Artificial ; Tachycardia ; Ventilation

Chronic fatigue of the respiratory muscles has contributed to the decreased ventilatory capacity and reduced excercise tolerance of individuals with COPD, especially in kyphoscoliosis. Nasal mask BiPAP has been shown to be useful for the patient with nocturnal muscle fatigue and COPD. A 35-year-old man with severe kyphoscoliosis was admitted to ICU due to acute respiratory failure. He had been diagnosed of COPD and had been intubated with mechanical ventilatory support for 7 times. This time he was intubated with ventilatory support, too, in ICU and readmitted to the ICU for severe hypoxemia and hypercarbia from general ward. Thereafter he refused the intubation. Nasal mask BiPAP ventilatory support system was applied and IPAP, EPAP level being adjusted to the 12, 4 cmH2O under monitoring vital signs and arterial blood gas analysis. His condition was improved and discharged home with support of nasal mask BiPAP system after 33 day-stay in the ICU.
Adult ; Anoxia ; Blood Gas Analysis ; Fatigue ; Humans ; Intubation ; Masks* ; Muscle Fatigue ; Patients' Rooms ; Pulmonary Disease, Chronic Obstructive* ; Respiratory Insufficiency ; Respiratory Muscles ; Vital Signs

BACKGROUND: The APACHE II scoring system has been regarded as a useful tool in the assessment of the severity of injury and prognosis for acutely ill patients. Recently, there have been many reports that multiple organ failure(MOF) score is the better predictor of the mortality of critically ill patients than any other scoring system. The purpose of this study was to compare APACHE II score and MOF score for mortality prediction in critically ill patients. METHODS: 163 critically ill patients were studied. We analyzed the correlation between the mortality rate and the scores that were produced by APACHE II and MOF scoring system within the first 24 hours in the ICU. We analyzed the correlation between each score and the number of days of ICU stay. We also calculated the mortality rate according to the number of organ failure. RESULTS: 1) The APACHE II score and MOF score of the survivors(n=129) were 9 6 and 1 1, respectively and those of nonsurvivors(n=34) were 16 7 and 5 2(mean SD), respectively. 2) The r2 was 0.62 between APACHE II score and mortality rate, and 0.77 between MOF score and mortality rate. 3) The r2 was 0.06 between APACHE II score and ICU stay, 0.01 between MOF score and ICU stay. 4) The mortality rates were 0, 2, 20, 64, 73, 75 and 100 % in 0, 1, 2, 3, 4, 5 and 6 organ failures, respectively. CONCLUSIONS: The MOF score was more sensitive predictor of the mortality of critically ill patients than the APACHE II score.
APACHE* ; Critical Illness* ; Humans ; Critical Care ; Mortality* ; Multiple Organ Failure* ; Prognosis