ABSTRACT
Mauriac syndrome consists of a triad of poorly controlled diabetes, profound growth retardation and hepatomegaly. We experienced a case of Mauriac syndrome in an 18-year-old girl who had poorly controlled diabetes mellitus, short stature, hepatomegaly and central obesity. Also at the time of examination, she had complications of diabetic cataract and nephropathy. Fourteen years prior to admission, she was diagnosed as diabetes mellitus at a hospital. Thereafter, she had been managed with irregular insulin injection. On physical examination at admission, her height was 135cm(<3 percentile) and her weight was 39kg(<3 percetile). She was short and obese. The liver was 5 cm palpable below the right subcostal margin. Her sexual maturation was Tanner stage I. On ophthalmologic examination, the cataracts were observed on both eyes and diabetic retinopathy was absent. Diabetic nephropathy could not be confirrned by kidney biopsy due to her mother's refusal. We studied the hormonal, radiographic and histological abnormalities. The hormonal study was normal and the bone age was by delayed as much as 10 years. The liver biopsy revealed glycogen accumulation in hepatocyte. She was consistent with Mauriac syndrome. She was managed by strict diabetic control with insulin therapy, diabetic diet and intensive education. She was discharged with well controlled blood glucose. Five months later, growth acceleration and sexual maturation have not been observed, but hepatomegaly subsided. (J Korean Pediatr Soc 2000;43-837-841)
Subject(s)
Adolescent , Female , Humans , Acceleration , Biopsy , Blood Glucose , Cataract , Diabetes Mellitus , Diet, Diabetic , Diabetic Nephropathies , Diabetic Retinopathy , Disulfiram , Education , Glycogen , Hepatocytes , Hepatomegaly , Insulin , Kidney , Liver , Obesity, Abdominal , Physical Examination , Sexual MaturationABSTRACT
Apert syndrome is an uncommon congenital disorder characterized by malformation of the skull in association with symmetrical syndactyly of both hands and feet. This syndrome is autosornal dominant. The original description was presented by Apert in 1906. Since then more than 200 cases have been reported in the world. Recently, we experienced a case of newhorn male infant with congenital anomalies of the skull and extremities. Molecular biologically, he was found to have Ser252Try mutation in the FGFR2 exonIIIa. A brief review of literature was made.
Subject(s)
Humans , Infant , Male , Acrocephalosyndactylia , Congenital, Hereditary, and Neonatal Diseases and Abnormalities , Extremities , Fibroblast Growth Factors , Fibroblasts , Foot , Hand , Receptor, Fibroblast Growth Factor, Type 2 , Receptors, Fibroblast Growth Factor , Skull , SyndactylyABSTRACT
A preduodenal portal vein is a rare anomaly. A left-sided gallbladder is also a rare anatomical variant that may be occasionally combined with a preduodenal portal vein. We report here a patient with multiple biliary stones whose preduodenal portal vein was discovered at operation. The anomaly was a preduodenal portal vein associated with a left-sided gallbladder, which is very rare. This is the first case reported in Korea. An abnormal location of the round ligament to the right side can make the gallbladder appear to be a left-sided gallbladder. Our case belongs to this category. This patient had multiple biliary stones in the intrahepatic duct, the common bile duct, and the gallbladder. We treated him with a cholecystec tomy, choledochotomy, and choledochoscopic stone extraction using a basket, and electrohydraulic lithotripter. All procedures were done using a laparoscopic method. The patient was treated well with no complication.
Subject(s)
Humans , Common Bile Duct , Gallbladder , Korea , Portal Vein , Round Ligament of Uterus , Round LigamentsABSTRACT
BACKGROUND: The use of choledochoscopy has been increasing lately in open and laparoscopic surgery for bile duct stones. Intraoperative choledochoscopy is useful for assessing the biliary trees and stones and for assisting in the removal of bile duct stones. However, large or impacted bile duct stones are difficult to remove using choledochoscopy alone. Application of electrohydraulic lithotripsy (EHL) seems to be suited for these difficult cases. METHODS: Twenty-six (26) patients with bile duct stones were treated with intraoperative cho ledochoscopic EHL to remove stones which could not be retrieved using stone forceps, a basket, saline flushing, or a Fogarty catheter. We divided the 26 cases into two groups: 12 cases of laparoscopic surgery and 14 cases of open surgery. These cases were further divided into two groups: common bile duct stones and intrahepatic duct stones RESULTS: The indications of EHL in laparoscopic surgery for common bile duct stones were large stones in 2 cases and impacted stones in 4 cases. Eighteen (18) patients with intrahepatic duct stones were treated with EHL for impacted stones. Stone clearence rate was 88.5%, and complications occurred in 3 cases (11.5%). One case of a biliary fistula was managed conservatively, and two cases of mucosal bleeding of the bile duct were spontaneously controlled. CONCLUSION: Intraoperative choledochoscopic electrohydraulic lithotripsy is a safe and effective method for removing large or impacted bile duct stones.
Subject(s)
Humans , Bile Ducts , Bile , Biliary Fistula , Catheters , Common Bile Duct , Flushing , Hemorrhage , Laparoscopy , Lithotripsy , Surgical InstrumentsABSTRACT
A case of a papillary thyroid carcinoma presenting as a cystic lateral neck mass is reported. A 73-year-old woman presented with a painless swelling of the right side of the neck which had been noticed for 60 years. The soft tissue mass was clinically and sonographically thought to be branchial cleft cyst. However, excisional biopsy showed it to be a cystic lymph-node metastasis from a papillary thyroid carcinoma. Two weeks later, a total thyroidectomy was performed, but no malignant lesion was found in the thyroid. This case of a metastatic papillary thyroid carcinoma to the lateral neck node mimiced a branchial cleft cyst. In old age, a lateral neck cyst being a cystic metastasis, as well as a benign cyst, should be considered so a careful preoperative evaluation is neccessary.
Subject(s)
Aged , Female , Humans , Biopsy , Branchioma , Neck , Neoplasm Metastasis , Thyroid Gland , Thyroid Neoplasms , ThyroidectomyABSTRACT
A case of a papillary thyroid carcinoma presenting as a cystic lateral neck mass is reported. A 73-year-old woman presented with a painless swelling of the right side of the neck which had been noticed for 60 years. The soft tissue mass was clinically and sonographically thought to be branchial cleft cyst. However, excisional biopsy showed it to be a cystic lymph-node metastasis from a papillary thyroid carcinoma. Two weeks later, a total thyroidectomy was performed, but no malignant lesion was found in the thyroid. This case of a metastatic papillary thyroid carcinoma to the lateral neck node mimiced a branchial cleft cyst. In old age, a lateral neck cyst being a cystic metastasis, as well as a benign cyst, should be considered so a careful preoperative evaluation is neccessary.
Subject(s)
Aged , Female , Humans , Biopsy , Branchioma , Neck , Neoplasm Metastasis , Thyroid Gland , Thyroid Neoplasms , ThyroidectomyABSTRACT
BACKGROUND: The current health care system demands provision of quality patient care in a cost-effective manner. A clinical path defines an optimal sequencing and timing of intervention by a health care team. This path facilitates the streamlining of this process. Implementation of clinical paths may decrease hospital cost without increasing complications in acute appendicitis patients. METHODS: A prospective evaluation of a clinical pathway for acute appendicitis (during March 1999) was conducted and the results were compared with those for control patients (during Feb 1999). Pregnant patients or patients with chronic disease were excluded. The patients with acute appendicitis were classified into three groups: A-type for acute focal and suppurative appendicitis, B-type for gangrenous appendicitis, and C-type for perforative appendicitis. RESULTS: The data for 40 patients with a clinical pathway were compared to those for 30 control patients. The mean age was 25.3 11.7 years in the pathway group versus 39.3 15.8 years in the control group. The mean hospital duration were 4.5 days for the pathway with A-type appendicitis versus 5 days for the control patients (p<0.05) and the mean hospital cost was 85.73% of that for the control group (p<0.05). In B- and C-type, the hospital duration and the cost were not different. The satisfaction rates were increased in all the types of pathway patients. The complication rates for in all the pathways were no different from those for the control patients. CONCLUSION: The clinical pathway with A-type appendicitis decreased the duration of hospitalization and the cost without adversely affecting the diagnosis or the therapy. The clinical paths were useful as means to minimize cost while increasing patient satisfaction.
Subject(s)
Humans , Appendicitis , Chronic Disease , Critical Pathways , Delivery of Health Care , Diagnosis , Diagnosis-Related Groups , Hospital Costs , Hospitalization , Patient Care , Patient Care Team , Patient Satisfaction , Prospective StudiesABSTRACT
BACKGROUND: Lowering the wound infection rate is very important to decrease morbidity, mortality, and the cost of therapy. We wanted to know the bacteriologic pathogen of surgical infection, to verify the role and proper selection of prophylactic antibiotics, and to figure out the risk factors of surgical infection in the biliary area. METHODS: A prospective study was done for 109 patients who underwent biliary operations, excluding laparoscopic cholecystectomy from Mar. 1997 to Feb. 1998. Bacteriologic data were collected from 96 patients among them. Wound infection was defined as a purulent discharge from the wound. Ordinary bacterial cultures were taken from the peritoneal cavity (liver bed) and the wound subcutaneous area. Items of investigations were age, sex, degree of obesity, operation time, ASA classification, incision, suture materials, transfusion, used antibiotics, serum albumin, serum glucose, and hemoglobin. Statistical analysis were done with the Mann-Whitney-Wilcoxon-rank test, the Students t-test, the Chi-square test, and Fishers exact test. RESULTS: Wound infections occurred in 5 cases (4.8%). Culture positivity from the wounds was 38.5% (37/96), and that than the peritoneal cavity were 40.6% (39/96). The bacteria isolated from the peritoneal cavity were mainly gram-negative rods (E. coli, Klebsiella, Enterobacter, Pseudomonas, Serratiae, Morganella); gram-positive cocci were also isolated (Enterococcus, Streptococcus). The most common bacteria isolated from the wound was gram-positive Enterococcus; gram-negative rods were also common (Klebsiella, E. coli, Enterobacter). Of the investigated risk factors, a positive culture and biliary tract infection were statistically significant risk factors of wound infection (p<0.05). CONCLUSION: Taking a bacterial culture from the wound and from the peritoneal cavity is important because it directly correlates with the degree of contamination and defines a high risk of infection. Prophylactic antibiotic administration with 3rd generation cephalosporin and aminoglycoside is justified in view of the organisms isolated from the culture.
Subject(s)
Humans , Anti-Bacterial Agents , Bacteria , Biliary Tract , Blood Glucose , Cholecystectomy, Laparoscopic , Classification , Enterobacter , Enterococcus , Gram-Positive Cocci , Klebsiella , Mortality , Obesity , Peritoneal Cavity , Prospective Studies , Pseudomonas , Risk Factors , Serratia , Serum Albumin , Sutures , Wound Infection , Wounds and InjuriesABSTRACT
BACKGROUND: The authors experienced cholangiocarcinomas associated with intrahepatic duct stones, so we reviewed and summarized the clinical features of these patients to shed light on the pathogenetic implication of hepatolithiasis on the development of cancer and to emphasize the role of hepatic resection in the management of hepatolithiasis. METHODS: We performed 54 hepatic resections in 106 hepatolithiasis patients (51%) during 5 years from Sep. 1993 to Jun. 1998. The medical records of these patients were retrospectively reviewed. Clinical and demographic findings were analyzed and compared with those for all the hepatolithiasis cases. Statistical analysis was done with the chi-square test and Student t-test. RESULTS: Eight patients out of 106 intrahepatic duct stone patients were associated with a cholangiocarcinoma (7.5%). The mean age was 59.5 13.4 years, and the male to female ratio was 1:3. Chief complaints were right upper quadrant pain (87.5%), fever (50%), jaundice (25%), indigestion (25%), and weight loss (12.5%). Clinically, 4 cases were presented as acute cholangitis, and 1 case was pancreatitis. The locations of the tumors were the right lobe in 2 cases, the left lobe in 3 cases, and the hilar portion in 3 cases. Preoperative diagnosis was clinically possible only in one case. Intraoperative diagnoses were possible in 5 cases with clinical suspicion of cancer and were confirmed by using frozen biopsy. Three cases were incidentally diagnosed as cancer by postoperative pathological reports. We missed the presence of cancer in these cases due to associated hepatic abscesses. The operations performed were a right lobectomy in 1 case, an extended right lobectomy in 1 case, a left lobectomy in 2 cases, a left lateral segmentectomy in 2 cases, and an extrahepatic bile duct resection with hepaticojejunostomy in 1 case. A palliative hepaticojejunostomy was done in 1 case, and the patient died 3 months later. Another patients survived for over 2 years after operations. CONCLUSION: Aggressive hepatic resection for hepatolithiasis is recommended in view of highly coincident hidden cancer and the difficulty in diagnosis that cancer. Molecular and genetic investigations are needed to verify the etiological correlation between hepatolithiasis and cholangiocarcinomas.
Subject(s)
Female , Humans , Male , Bile Ducts, Extrahepatic , Biopsy , Cholangiocarcinoma , Cholangitis , Diagnosis , Dyspepsia , Fever , Jaundice , Liver Abscess , Mastectomy, Segmental , Medical Records , Pancreatitis , Retrospective Studies , Weight LossABSTRACT
Bezoars are large conglomerates of vegetable fiber and hair, or concretions of various substances located in the stomach or the intestinal tract. They are classified as trichobezoars, phytobezoars, trichophytobezoars or concretions. The most common site for a bezoar is the stomach, but small-bowel bezoars are occasionally reported. A gastric bezoar can be treated by endoscopic removal or chemical dissolution. Small bowel bezoars are usually discovered due to intestinal obstruction, and surgical removal is the standard treatment method. We report the case of a patient who suffered from an intestinal obstruction due to a phytobezoar about 5 4 3 cm3 size.
Subject(s)
Humans , Bezoars , Hair , Intestinal Obstruction , Stomach , VegetablesABSTRACT
The pregnancy-associated breast cancer is one of the most common malignacy developed during pregnant period and occupies 1-2% of the all breast cancer pateints. It is easy to miss appropriate diagnosis due to difficulty of physical examination of the breast and limitation of proper diagnostic modality. The pregnancy-associated breast cancer has been reported to portend an extremely poor outcome. But recent studies have found no difference in survival between general breast cancer and pregnancy-associated breast cancer. The treatment of choice is the modified radical mastectomy and adjuvant chemotherapy if needed. The authors report two patients of breast cancer diagnosed and treated during pregnancy with review of literature.
Subject(s)
Humans , Pregnancy , Breast Neoplasms , Breast , Chemotherapy, Adjuvant , Diagnosis , Mastectomy, Modified Radical , Physical ExaminationABSTRACT
BACKGROUND: E-cadherin, one of the best known cell adhesion molecules plays an important role in embryonic cell development and architectural maintenance of adult tissues. This molecule is a calcium- dependent glycoprotein and is expressed in most normal epithelial tissues. Selective loss of E-cadherin has been observed in many carcinomas of various origins, and this phenomenon is thought to be an induction point for depression of cell adhesiveness and for metastasis. This study was conducted to evaluate the significance of E-cadherin expression as a prognostic factor in breast cancer and to examine the relationship between E-cadherin expression and clinicopathologic factors. METHODS: Surgical specimens were obtained from 73 patients with breast carcinoma from September 1993 to August 1996 in the Department of Surgery, Mok-Dong Hospital, Ewha Womans University. The immunohistochemical staining was performed by using the paraffin embedded tissues. These results were compared with previously known important clinicopathologic characteristics such as age, tumor location, chief complaint, mass size, tumor stage, lymph node invasion, mitosis, nuclear and histologic grades. RESULTS: The expression status of E-cadherin was normal in 41 cases (56%), reduced in 23 cases (32%), lost in 9 cases (12%). When compared with clinicopathologic variables, loss of E-cadherin expression was found in high nuclear and histologic grade, high stage, and severe lymph node invasion cases. However, there was no significant difference in survival rate according to E-cadherin expression. CONCLUSION: This study suggests that E-cadherin expression may be related with the histologic and nuclear grades, the stage and lymph node invasiveness, so it can be used as predictive factor for metastasis.
Subject(s)
Adult , Female , Humans , Adhesiveness , Breast Neoplasms , Breast , Cadherins , Cell Adhesion Molecules , Depression , Glycoproteins , Lymph Nodes , Mitosis , Neoplasm Metastasis , Paraffin , Survival RateABSTRACT
BACKGROUND: Laparoscopic cholecystectomy has substituted open cholecystectomy for cholelithiasis during the last decades. In the past, biliary surgeons have performed the intraoperative bile culture during open cholecystectomy and have used the culture results as a guideline of postoperative antibiotic therapy. The prophylactic antibiotic treatment for cholecystectomy have been needed in the elderly population and the high risk patients, but the effect of antibiotics in laparoscopic cholecystectomy has not been confirmed. The authors performed bile culture during laparoscopic cholecystectomies to evaluate the relationship between the bile culture results and the postoperative infectious complications. METHODS: We analyzed the 57 patients who had undergone laparoscopic cholecystectomy, 53 for cholelithiasis, 4 for gallbladder polyp and an intraoperative bile culture for 6 months period. RESULTS: Positive culture results were found in 12 cholelithiasis patients (22.6%). The most common pathogen was E.coli (8.8%), enterococcus (7.0%); others were Pseudomonas, Strenotrophomonas maltophilia, Klebsiela, ect. The results of the bile culture in 4 patients with gallbladder polyps were negative. Nine patients had severe inflammation of gallbladder and four of them had positive culture results. Risk factors, such as old age and hyperbilirubinemia were significant variables. CONCLUSIONS: Intraoperative bile culture is an easy and simple procedure in a laparoscopic cholecystectomy and offers much informations to the surgeon about biliary bacteriology and postoperative management. The rate of wound infection is very low in laparoscopic cholecystectomy and the necessity of prophylactic antibiotics in laparoscopic cholecystectomy is less than it is in open surgery. Prophylactic antibiotics during laparoscopic cholecystectomy should be reserved for only high-risk patients.
Subject(s)
Aged , Humans , Anti-Bacterial Agents , Bacteriology , Bile , Cholecystectomy , Cholecystectomy, Laparoscopic , Cholelithiasis , Enterococcus , Gallbladder , Hyperbilirubinemia , Inflammation , Polyps , Pseudomonas , Risk Factors , Wound InfectionABSTRACT
The pregnancy-associated breast cancer is one of the most common malignacy developing during the period of pregnancy and occupies 1-2% of the all breast cancer patients. It is not easy to make an appropriate diagnosis due to difficulty of physical examination of the breast and limitation of proper diagnostic modalities. The pregnancy-associated breast cancer had been reported to portend an extremely poor outcome. But recent studies have found no difference in survival between general breast cancer and pregnancy-associated brest cancer. The treatment of choice is the modified radical mastectomy and adjuvant chemotherapy of needed. The authors report two patients of breast cancer diagnosed and treated during pregnancy with review of literature.
Subject(s)
Humans , Pregnancy , Breast Neoplasms , Breast , Chemotherapy, Adjuvant , Diagnosis , Mastectomy, Modified Radical , Physical ExaminationABSTRACT
Because of the two merits of nonoperative management of blunt abdominal trauma, 1) avoidance of operative morbidity and 2) better treatment of associated injuries, the use of nonoperative management has been extended, but the indications for such treatment have not been sufficiently found. One hundred two(102) cases admitted due to hemoperitoneum, 44 involving surgery and 58 conservative managment, were analyzed for age, sex, cause of injury, injured organ, injury grade, transfusion amount, and shock on admission. The major causes of injury in the nonoperative and the operative groups are as follows : 23 cases of auto-pedestrian accidents and 15 cases of in-car accidents in the nonoperative group and 19 cases of auto-pedestrian accidents in the operative group. In terms of the injured organ, liver trauma was the most frequent, and spleen trauma was next. The difference in the transfusion amount between the two groups was statistically significant; 8.1 units in the nonoperative group and 13 units in the operative group. In conclusion, 1) nonoperative management can be considered as a first choice in children with blunt abdominal trauma and stable vital signs; 2) patients with hemodynamically stable liver injury with AAST OIS grade 4 and isolated splenic injury AAST OIS grade 4 are candidates for nonoperative management; and 3) nonoperative management through emergency care without transfusion can be considered in cases with stable vital signs.
Subject(s)
Child , Humans , Emergency Medical Services , Hemoperitoneum , Liver , Shock , Spleen , Vital SignsABSTRACT
Abdominal actinomycosis is very rare infectious disease and seldom reported as a cause of abdominal mass. This disease is diagnosed by pathologic findings and culture result but it is very difficult to differentiate with other granulomatous illammatory disease, diverticular disease, malignancy, etc. The radiologic findings were non-specific and CT scan revealed the inflammatory mass with multiple small abscesses and fibrous change. The treatment of choice for actinomycosis is medical treatment with penicillin but surgical intervention may be needed when complication such as obstruction, fistula formation, abscess formation develop. We report a patient with abdominal actinomycosis that presented with transverse colonic obstruction and severe abdominal wall inflammation. This patient had no past operative history but got intrauterine contraceptive devices during last 10 years. We treated this patient by surgical resection and antibiotic therapy.
Subject(s)
Humans , Abdominal Wall , Abscess , Actinomycosis , Colon , Colon, Transverse , Communicable Diseases , Fistula , Inflammation , Intrauterine Devices , Penicillins , Tomography, X-Ray ComputedABSTRACT
Clinical survey was performed on 413 cases of twin pregnancies and their 816 twin babies who were born at IlSin Christian Hospital during 5years from January 1987 to December 1991. The result of study was as follows: 1) there was 413 twin pregnancies among 40,711 deliveries, so the incidence of twins was 1:98.6. Average male-female sex ratio of twins was 1.3:1, same sex pairs rate was 86% and different sex ratio was 14%. 2) The incidence of relation with maternal age was highest between 25 years old and 29 years old but the ratio of twin delivery to total delivery was 0.9% so, was not higher than other age group. 3) The incidence of relation with maternal parity was highest at first pregnancy but the ratio of twin delivery to total delivery was highest at second pregnancies. 4) Normal birth weight infants were 45.7%, low birth weight infants were 54.3% and very low birth weight infants was 9.3%. Fullt-erm infants were 63.7% and prematurity were 35.4%. 5) The incidence of feto-fetal transfusion was 7.3%. 6) Perinatal death rate was 76.3 and was more higher in second babies. The most common cause of perinatal death was prematurity, followed by respiratory complication, congenital anomaly, infection and asphyxia. Still-births were 8 cases.