ABSTRACT
Testicular feminization syndrome (TFS) is a genetic disorder due to androgen insensitivity of the target organs. The most common clinical presentation of complete TFS is inguinal hernia in the infant or primary amenorrhea in the adolescence. A 7-year old phenotypically female patient was seen with a complaint of a right inguinal mass. Under the diagnosis of right inguinal hernia, high ligation was performed. Six months later, the patient showed a left inguinal mass. On operation, the mass looked like a testis. The external genitalia were normal female, but a uterus and ovary were not identified. Chromosome study showed a 46, XY karyotype and the levels of serum testosterone and dihydrotestosterone were increased after HCG stimulation. The patient was diagnosed as complete TFS and underwent bilateral gonadectomy 6 months later.
Subject(s)
Adolescent , Child , Female , Humans , Infant , Male , Amenorrhea , Androgen-Insensitivity Syndrome , Diagnosis , Dihydrotestosterone , Genitalia , Hernia, Inguinal , Karyotype , Ligation , Ovary , Testis , Testosterone , UterusABSTRACT
Testicular feminization syndrome (TFS) is a genetic disorder due to androgen insensitivity of the target organs. The most common clinical presentation of complete TFS is inguinal hernia in the infant or primary amenorrhea in the adolescence. A 7-year old phenotypically female patient was seen with a complaint of a right inguinal mass. Under the diagnosis of right inguinal hernia, high ligation was performed. Six months later, the patient showed a left inguinal mass. On operation, the mass looked like a testis. The external genitalia were normal female, but a uterus and ovary were not identified. Chromosome study showed a 46, XY karyotype and the levels of serum testosterone and dihydrotestosterone were increased after HCG stimulation. The patient was diagnosed as complete TFS and underwent bilateral gonadectomy 6 months later.
Subject(s)
Adolescent , Child , Female , Humans , Infant , Male , Amenorrhea , Androgen-Insensitivity Syndrome , Diagnosis , Dihydrotestosterone , Genitalia , Hernia, Inguinal , Karyotype , Ligation , Ovary , Testis , Testosterone , UterusABSTRACT
PURPOSE: Mouse liver nonparenchymal cells play an important role in the development of active apoptosis in graft- infiltrating cytotoxic T lymphocytes, and this apoptosis can be an explanation for liver graft acceptance. We intended to clarify whether immature mouse liver dendritic cells can induce apoptosis in allogeneic activated T cells and determine which mechanism is involved in this phenomenon. METHODS: A radiometric DNA fragmentation test ("JAM" assay) was used to determine whether mouse liver dendritic cells were able to induce activated T-cell apoptosis in vitro. In addition, immunohistochemical staining for Bax and Bcl-2 was examined to clarify whether Bax or Bcl-2 was involved in this apoptosis. RESULTS: Immature mouse liver dendritic cells were quite strong inducers of activated T cell apoptotic death in allogeneic mice in vitro (39.2+/-13.2% at E/T ratio=12.5/1) compared with spleen cells as effectors (4.7+/-13.4% at E/T ratio=12.5/1) (P<0.0001). By using immunohistochemical staining, we also showed that Bax might play some role in this phenonenon, but that Bcl-2 might not. CONCLUSION: Our data indicate that immature mouse liver dendritic cells might have a strong apoptotic activity toward activated T cells in allogeneic mice in vitro through a Bax-involved mechanism.
Subject(s)
Animals , Mice , Apoptosis , Dendritic Cells , DNA Fragmentation , Liver , Spleen , T-Lymphocytes , T-Lymphocytes, Cytotoxic , TransplantsABSTRACT
PURPOSE: Pylorus-preserving pancreatoduodenectomy (PPPD) is an alternative surgical procedure for periampullary lesions. Early delayed gastric emptying is the most common and frustrating complication in the immediate postoperative period after PPPD and late delayed gastric emptying has been reported in some long-term follow-up studies. We evaluated the incidence of early delayed gastric emptying and analyzed temporal changes in gastrointestinal function after PPPD. METHODS: The incidence of early delayed gastric emptying was retrospectively evaluated from the medical records of 15 patients who underwent PPPD. Gastric emptying tests (GETs) using 99mTc-DTPA scan were performed on 11 of the patients every three months until 1 year, where possible. RESULTS: The incidence of early delayed gastric emptying was 6.7%. Five of the eight patients (62.5%) and six of the eight (75%) who underwent scintigraphy at 3 months and 6 months respectively, showed delayed gastric emptying. But at 12 months, all of the four patients who underwent GETs showed normal gastric emptyings. CONCLUSION: The incidence of early delayed gastric emptying after PPPD was 6.7%. Though there were few symptoms in long-term follow-up study using 99mTc-DTPA scan, delayed gastric emptying was frequently observed 3 to 9 months after PPPD. However, gastric emptying might be normalized in almost all patients around 1 year after PPPD.
Subject(s)
Humans , Follow-Up Studies , Gastric Emptying , Incidence , Medical Records , Pancreaticoduodenectomy , Postoperative Period , Radionuclide Imaging , Retrospective StudiesABSTRACT
PURPOSE: Anomalies in the thyroglossal duct are the most common midline, or paramedian cervical lesions of congenital origin. Presenting as a thyroglossal duct cyst (TGDC) or a thyroglossal duct sinus (TGDS), they are found in all age groups. This clinical study was intended to elucidate the clinical characteristics, and the outcomes, of surgical treatment of these anomalies. METHODS: Between January 1992 and May 2002, 91 patients underwent surgery for a TGDC or a TGDS at 4 hospitals affiliated to Hallym University. The demographics of the patients, the clinical characteristics of the lesions, and the outcomes of the treatments were retrospectively evaluated. These characteristics were evaluated according to age groups; younger than 15 (38 patients) and older than 15 (53 patients) years. RESULTS: The male to female ratio was 1.67: 1 (It is better to give the actual figures.), with the first decade showing the highest incidence in the age distribution (33%). Painless masses (83.6%) were the most common presenting symptom, with most symptoms having been manifest for less than 1 year (67%). TGDCs were 90% and TGDSs were 10%. There were 62 cases of infrahyoid and 18 of suprahyoid lesions, with 80 cases on the midline and 11 on the paramedian within 2 cm from the midline. Of the paramedian lesions, 2 cases were in the younger group and 9 were in the older group. This tendency of laterality in the location by age group showed no statistical significance (p=0.172). All the patients underwent a Sistrunk operation, and one experienced a recurrence. CONCLUSION: This study justified the Sistrunk operation as the treatment of choice for anomalies of the thyroglossal duct. The lateral lesions occurred in the old age groups more frequently. A careful approach is needed with paramedian cervial lesions in adults to avoid the loss of the proper treatment of possible hidden congenital lesions.
Subject(s)
Adult , Female , Humans , Male , Age Distribution , Demography , Incidence , Recurrence , Retrospective Studies , Thyroglossal CystABSTRACT
PURPOSE: Choledochal cyst is a rare disease associated with an anomalous junction of the pancreaticobiliary ductal system (AJPBDS). We intended to demonstrate the characteristics of adult-onset choledochal cyst retrospectively. METHODS: ERCP findings of nineteen adult cases of choledochal cyst were compared with those of nine normal persons, in respect to the length and diameter of the common channel, and the angle of the pancreaticobiliary ductal junction. RESULTS: Patients of adult-onset choledochal cyst displayed a long, ectatic common channel and a larger angle of the pancreaticobiliary junction (p<0.05). All patients except one had AJPBDS. Thirteen patients were classified as Todani type Ia, one as type Ic and five as type IVa. Fourteen patients were classified as choledochopancreatic (C-P) type, two as pancreaticocholedochal (P-C) type, and three were unclassified by Kimura's classification. Sixteen patients were compatible with cystic type and three with cylindrical type by Okada's classification. Among the three cylindrical type patients two of them were classified as P-C type and one as C-P type. Histologically cystic type patients showed mild glandular or fibrotic patterns, however cylindrical type patients tended to show the severe glandular pattern. CONCLUSION: Adult-onset choledochal cyst was closely related with AJPBDS, although the reflux of pancreatic juice due to AJPBDS would not be the only cause to determine the morphology of adult-onset choledochal cyst. The meaning of this tendency toward a severe glandular pattern in cylindrical type patients requires elucidation.
Subject(s)
Adult , Humans , Cholangiopancreatography, Endoscopic Retrograde , Choledochal Cyst , Classification , Pancreatic Juice , Rare Diseases , Retrospective StudiesABSTRACT
Despite of advances in perinatal management and treatment modalities, congenital diaphragmatic hernia (CDH) remains a frustrating problem. Although the sheep has been the most reliable experimental animal of fetal surgery in CDH, the rabbits has some possible advantages over sheep; lower cost, smaller body size, year-round availability, high number of fetuses per pregnancy, and short gestational period. The purpose of this study is to evaluate the feasibility of the animal model of CDH in rabbits. Twenty seven pregnant New Zealand rabbits underwent hysterotomy and fetal operation on gestational day 24 or 25. Two fetuses of each pregnant rabbit were operated. In the fetus of one end of bicornuate uterus, left diaphragmatic hernia was created by excision of fetal diaphragm through open thoracotomy (DH Group). In the fetus of the other end of bicornuate uterus, left diaphragmatic hernia was created and tracheal ligation by small-sized Surgiclip(R) (USSC, Norwalk, Conn., USA) was performed through cervical incision (TL Group). Among twenty seven pregnant rabbits, twelve were born alive with diaphragmatic hernia and eight were born alive with diaphragmatic hernia & tracheal ligation. The most commonly herniated abdominal organ was the left lobe of liver. In DH group, the lungs were hypoplastic with a decrease in lung weight/body weight ratio, a reduction in number of alveoli, an increase of vascular medial wall thickness of pulmonary arteries. The alveoli were immature with thick alveolar septum and increased interstitium. In TL group, the alveoli were more mature than that of DH group and no significant difference from control group was existed. Thus this study demonstrates that (1)Diaphragmatic hernia can be created in rabbits by fetal surgery, (2)Tracheal ligation can be performed in rabbits by fetal surgery, (3)Experimental diaphragmatic hernia results in pulmonary hypoplasia, (4)Concurrent tracheal ligation prevents pulmonary hypoplasia that resulted from diaphragmatic hernia. The above mentioned results show that pregnant New Zealand rabbit is appropriate as the animal model of CDH.
Subject(s)
Animals , Pregnancy , Rabbits , Body Size , Diaphragm , Fetus , Hernia , Hernia, Diaphragmatic , Hysterotomy , Ligation , Liver , Lung , Models, Animal , Pulmonary Artery , Sheep , Thoracotomy , UterusABSTRACT
Kimura's disease is a rare chronic inflammatory and proliferative condition of unknown etiology. It has been thought to be a part of large spectrum of the entity known as angiolymphoid hyperplasia with eosinophilia (ALHE). However, recently the difference is emphasized between the two entities by some authors. It usually presents subcutaneous or dermal mass in the head and neck region. This condition can be mistaken for a malignant tumor. It is defined pathologically as hyperplastic lymphoid follicles, eosinophilic infiltration and vascular proliferation. Authors have experienced three cases of Kimura's disease occurring in the submandibular, axillary and inguinal regions since 1993. There were two males and one female. Two patients presented peripheral eosinophilia. All patients underwent surgical excision. Two patients were managed with oral prednisone postoperatively.
Subject(s)
Female , Humans , Male , Angiolymphoid Hyperplasia with Eosinophilia , Eosinophilia , Eosinophils , Head , Neck , PrednisoneABSTRACT
The infectious complications after renal transplantation remain as an important causes of both mortality and graft loss. The lung is the most common target for post-transplant infection. We analyzed the causative agents, treatments and outcomes of post-transplant pulmonary infections. From 1990 to 1998, 192 renal allografts were performed at Hallym University Hospital. Seven cases (3.6%) of 5 males and 2 females developed serious pulmonary infections and required hospitalization. The mean age was 42.6 years. The onsets of pulmonary infections were from a month to 6 months in 3 cases, from 6 months to a year in one case and after a year in 3 cases. Triple therapy regimen was used in 4 cases as an immunosuppression therapy. Acute rejections developed in 6 cases. Causative organisms were Cytomegalovirus in 2 cases, Mycobacteria in 2 cases, Aspergillus in a case, Nocardia in a case and unknown in a case. Despite appropriate antibiotics, four patients did not respond to the treatment and died. The early recognition of infection and appropriate therapy is important to reduce fatal consequence.
Subject(s)
Female , Humans , Male , Allografts , Anti-Bacterial Agents , Aspergillus , Cytomegalovirus , Hospitalization , Immunosuppression Therapy , Kidney Transplantation , Kidney , Lung , Mortality , Nocardia , TransplantsABSTRACT
One fullterm infant whose clinical feature initially was that of necrotizing enterocolitis eventually developed intussusception. The symptomatology of these two conditions is strikingly similar, and when they coexist, recognition of a complicating intussusception is difficult. The pathogenic relationship between necrotizing enterocolitis and intussusception remains obscure. When a presumptive diagnosis of necrotizing enterocolitis is made but the infant's clinical course varies from that expected, other diagnoses, such as intussusception, must be considered.
Subject(s)
Humans , Infant , Diagnosis , Enterocolitis, Necrotizing , IntussusceptionABSTRACT
Venous anomalies, including portal vein thrombosis (PVT) and prior portosystemic shunts, are not uncommon in orthotopic liver transplantation (OLT) and require vascular reconstruction. PVT has been considered as a contraindication to OLT because of surgical complexity and increased postoperative morbidity and mortailty rates. Postoperative chylous ascites occur following disruption of abdominal lymphatics after retroperitoneal dissection. Chyloperitoeum after OLT is very rare. We report a case of piggyback OLT in a cirrhotic patient with portal vein thrombosis. A thromboendarterectomy was attempted first, and a venous jump graft was required between the donor portal vein and the infrapancreatic superior mesenteric vein. An aortic conduit was used for the hepatic arterial reconstruction. Retroperitoneal dissection was inevitable. Chylous ascites developed after the operation and were managed successfully with a low-at diet. In conclusion, we suggest that the presence of PVT is not a contraindication for OLT. Chylous ascites are rare after OLT, and the treatment of choice is a conservative one based on diet control.
Subject(s)
Humans , Chylous Ascites , Diet , Endarterectomy , Liver Transplantation , Liver , Mesenteric Veins , Portal Vein , Portasystemic Shunt, Surgical , Tissue Donors , Transplants , Venous ThrombosisABSTRACT
Renal transplantation has been adopted as the treatment of choices for most patients with a end stage renal disease. This is the presentation of our experience of renal transplantation at Hallym University Hospital. From March 1984 to July 1998, 207 cases of renal transplantations were performed, 15 cases out of which were lost during follow-up period and excluded from the study. The mean age of recipients was 37.1 years. The male to female ratio was 1.5:1. Eighty nine renal allografts (46.4%) were from living unrelated donors, 59 (30.7%) from living donors and 44 (22.9%) from cadaveric donors. As an immunosuppression theray, a triple regimen was used in 104 cases (54.2), a double regimen in 86 (44.8%) and a single regimen in one (0.5%). The overall rejection developed in 87 cases (45.3%). The rejection rate was 59.3% in renal allografts from living related donors, 37.1% from living unrelated donors and 43.2% from cadavaric donors. The OKT3 and the antilymphocyte globulin (ALG) were used for steroid resistent rejection in 8 and 2 cases, respectively. Post-transplant diabetes mellitus were noticed in 22 cases (11.5). The most common complication was infection (28.1%), and 11 recipeints (5.7%) died during follw-up period. Infection was the leading cause of mortality. The overall graft survival was 92.2% at 1 year span and 83.1% at 3 year span. The overall patient survival was 95.6% at 1 year span and 92.5% at 3 year span.
Subject(s)
Female , Humans , Male , Allografts , Antilymphocyte Serum , Cadaver , Diabetes Mellitus , Follow-Up Studies , Graft Survival , Immunosuppression Therapy , Kidney Failure, Chronic , Kidney Transplantation , Kidney , Living Donors , Mortality , Muromonab-CD3 , Tissue Donors , Unrelated DonorsABSTRACT
Organ procurements were performed in 9 brain-dead patients at our hospital since 1995. They were 7 males and 2 females. The causes of brain death were trauma in 7 patients and CVA in 2 patients. When brain death was confirmed, hypernatremia over 145 mEq/L was showed in 6 and hypothermia in all. Blood transfusion was done in 6 patients for correction of anemia preoperatively. The time intervals from brain death confirmation to organ procurement were from 1 hour to 2 days. Multi-organ harvests were done in 4 patient, 60 year-old female and 2 patients who received the cardioversion before harvest were given up to procure liver and heart preoperatively. And 2 patients were not procured heart and liver because of pathologic findings of intraoperative frozen biopsy. Among 22 recipients, 1 kidney recipient died postoperatively. We conclude that the intensive care should be performed in the brain dead donor for multi-organ harvest and better results of organ transplantations. Beside the organ procurement and transplantation team, another intensive care team for brain dead donor should be composed if possible.
Subject(s)
Female , Humans , Male , Middle Aged , Anemia , Biopsy , Blood Transfusion , Brain Death , Critical Care , Electric Countershock , Heart , Hypernatremia , Hypothermia , Kidney , Liver , Organ Transplantation , Tissue and Organ Procurement , Tissue Donors , Transplantation , TransplantsABSTRACT
We present a case of orthotopic liver transplantation in 63 year-old female patient with liver cirrhosis, who is the record of the oldest recipient in Korea. The donor was 20 year-old male patient with subarachnoidal hemorrhage. The operation time was 12 hours. The duration of cold ischemic time and anhepatic phase were 8 hours and 85 minutes respectively. After operation the patient resumed clear consciousness. The ventilator was weaned and removed. On the third postoperative day, reoperation was required due to hemoperitoneum. Splenectomy was performed for a tearing in the hilum. After reoperation, acute renal failure and sepsis developed. Continous arteriovenous hemofiltration was continued for 3 weeks. The patient was discharged on postoperative 59th day. The patient is doing well with normal liver function. No episode of acute rejection occurred until now. More aggressive approach has been feasible in organ transplantation for older patients by advanced knowledge of perioperative management. We conclude that age over 60 years should not be a barrier to liver transplantation.
Subject(s)
Female , Humans , Male , Middle Aged , Young Adult , Acute Kidney Injury , Cold Ischemia , Consciousness , Hemofiltration , Hemoperitoneum , Hemorrhage , Korea , Liver Cirrhosis , Liver Transplantation , Liver , Organ Transplantation , Reoperation , Sepsis , Splenectomy , Tissue Donors , Transplants , Ventilators, MechanicalABSTRACT
The Budd-Chiari syndrome is no longer a rare disease in the world, but there has been small cases reported until now in Korea. There are three forms of Budd-Chiari syndrome, among them membranous web in the inferior vena cava is the most common form in the oriental area. Authors have expereinced the Budd-Chiari syndrome, 49 year old female patient, caused by membranous web associated with the long thrombus in the inferior vena cava. She underwent side-to-side portacaval shunt and cavoatrial shunt using 19 mm diameter, 25 cm length polytetrafluoroetylene graft. Postoperative major morbidities were ARF and pneumonia. She has improved clinically and pathologically after operation. It is concluded that combined portacaval shunt and cavoatrial shunt has been effective to relieve the symptoms of Budd-Chiari syndrome caused by membranous web and inferior vena cava thrombotic occlusion.
Subject(s)
Female , Humans , Middle Aged , Budd-Chiari Syndrome , Korea , Pneumonia , Portacaval Shunt, Surgical , Rare Diseases , Thrombosis , Transplants , Vena Cava, InferiorABSTRACT
No abstract available.