IgG subclasses in symptomatic IgA deficiency.

We evaluated IgG subclass levels in 11 symptomatic patients (ages between 3 and 22; mean 7.5 years) with IgA deficiency, seven with selective IgA deficiency, and four with low IgA levels. All patients had experienced three or more episodes of sinopulmonary infections a year. Combined IgG2-IgG4 deficiency was detected in two patients, IgG2 deficiency in one patient and IgG4 deficiency in two patients. Elevated IgG1 and IgG3 levels were detected in most of the IgG subclass deficient and sufficient patients. It is known that gammaglobulin replacement therapy reduces the frequency of infections significantly in IgG subclass deficiency. Although immunization against IgA is a risk in IgA deficiency, these patients can be treated with gammaglobulins containing low IgA.     

Diagnosis and management of cystic duct leakage after laparoscopic cholecystectomy, report of 3 cases

BACKGROUND： Operative complications after laparoscopic cholecystectomy （LC） vary. Abdominal pain and other symptoms caused by fluid accumulation in the operative area are not uncommon. Cystic duct （CD） leakage is one of the main sources of the fluid. This study was to evaluate the procedures used in the diagnosis and management of CD leakage after LC. METHOD： The clinical materials of 3 patients with CD leakage after LC were studied retrospectively.     

Gastric antral stricture in a patient with chronic granulomatous disease

Chronic granulomatous disease (CGD) is a rare disorder of phagocytic cell oxidative metabolism. Patients have recurrent infections with catalase-positive organisms and granulomatous lesions throughout the body. Gastric antrum can be an occult site of involvement. We describe a four-year old boy with chronic granulomatous disease who was admitted with the complaints of persistent vomiting and weight loss. Gastric antral narrowing was diagnosed according to radiological findings. Treatment with steroid and antibiotics yielded a good clinical response in 15 days with a relief of the obstruction. This case report emphasizes the beneficial effect of this form of therapy in preventing life-threatening obstruction of vital organs in CGD.     

Angiogenesis factor in endometrial carcinoma: A new prognostic indicator?

OBJECTIVE: Tumor angiogenesis is believed to be a prognostic indicator associated with tumor growth and metastasis. Studies of angiogenesis in breast, prostate, and lung cancer, as well as melanoma, have shown that neovascularization correlates with the likelihood of metastasis and recurrences. The purpose of this study was to evaluate microvessel density as a prognostic factor in endometrial cancer. METHODS: Between 1980 and 1991 the tumor registry identified 25 patients with a diagnosis of recurrent endometrial cancer. These patients were matched with 25 patients with nonrecurrent disease for age, stage, grade, and treatment. The histologic slides of the 50 patients were reviewed. The paraffin blocks were obtained, and the area of the deepest myometrial invasion was selected for staining. The microvessels within the invasive cancer were highlighted by means of immunocytochemical staining to detect factor VIII - related antigen. Microvessels were counted by two investigators who were blinded to the patients' clinical status. Survival data were analyzed with Kaplan-Meier survival curves. RESULTS: Microvessel count was related to likelihood of recurrence, although this trend did not reach statistical significance. Patients with tumors of low capillary density had a mean survival time of 123 months. Patients with tumors of high capillary density had a mean survival time of 75 months (p = 002). Among patients with recurrent disease, those with a low capillary count survived a mean of 64 months. Patients with recurrent disease with tumors of high capillary density survived a mean of 45 months (p = 0.002). CONCLUSION: Angiogenesis factor correlates with survival in endometrial carcinoma. (Am J Obstet Gynecol 1996;174:1879-84.)     

Quality of life, functional outcome, and voice handicap index in partial laryngectomy patients for early glottic cancer

BACKGROUND: In this study, we aim to gather information about the quality of life issues, functional outcomes and voice problems facing early glottic cancer patients treated with the surgical techniques such as laryngofissure cordectomy, fronto-lateral laryngectomy, or cricohyoidopexi. In particular, consistency of life and voice quality issues with the laryngeal tissue excised during surgery is examined. In addition, the effects of arytenoidectomy to the life and voice quality are also studied. METHODS: 29 male patients were enrolled voluntarily in the study. The average age was 53.9 years. Three out of 10 patients with laryngofissure cordectomy also had arytenoidectomy. 11 patients had fronto-lateral laryngectomy with Tucker reconstruction, two of which also had arytenoidectomy. There were eight patients with cricohyoidopexi and bilateral functional neck dissection. Three of these patients also had arytenoidectomy. In bilateral functional neck dissection cases, spinal accessory nerve was preserved and level V of the neck was not dissected. None of the patients had neither radiotherapy nor voice therapy. Cordectomy patients never had a temporary tracheotomy or were connected to a feeding tube. Data was collected for 13 months for the cordectomy group, 14 months for fronto-lateral laryngectomy and cricohyoidopexi groups on average post-operatively. Statistical analysis in this study was carried out using the one-way analysis of variance, and the Post-Hoc group comparisons were made after Bonferroni and Scheffe-procedures.In order to determine the effects of arytenoidectomy, a regression analysis is carried out to see if there are statistical differences in answers given to the survey questions among patients who were arytenoidectomized during their surgeries. RESULTS: There was a statistically significant difference between cordectomy and cricohyoidopexi group in answers to the University of Washington- Quality of Life- Revised survey part 1. (p = 0). A statistically significant difference was also established between cordectomy and fronto-lateral laryngectomy groups, as well as between cordectomy and cricohyoidopexi groups in answers to the University of Washington- Quality of Life- Revised survey part 2. (p = 0,036 and p = 0.009, respectively). Cricohyoidopexi group has given the lowest scores and the cordectomy group has given the highest scores in three survey questions representing the quality of life, performances and new voices. These ranges are also consistent with the laryngeal tissue excised during surgery (cricohyoidopexi > fronto-lateral laryngectomy > cordectomy). There was no statistically significant difference between groups in Performance Status Scale for Head and Neck cancer patients instrument. The difference between the Voice Handicap Index and Voice Handicap Index (functional); Voice Handicap Index (physical) and Voice Handicap Index (emotional) scores in three patient groups was not significant either. All of the patients evaluated that their new voices have similar functional, physical and emotional impact on their life. Decanulation and oral feeding times of cricohyoidopexi and fronto-lateral laryngectomy patients are found to be significantly longer than cordectomy patients. Lastly, the removal of arytenoid does not have any significant adverse effects on the quality of life, the functional outcomes, or the quality of voice. CONCLUSION: In the present study, all patients with early glottic cancer, treated with different surgical technics reported fairly good quality of life outcomes, functional results and voice qualities. This study also finds that the removal of arytenoid does not have any adverse effects on the quality of life and voice from the patients' point of view.     

Early diagnosis of ataxia-telangiectasia using radiosensitivity testing

OBJECTIVES: To utilize radiosensitivity testing to improve early diagnosis of patients with ataxia-telangiectasia (A-T). STUDY DESIGN: We established normal ranges for the colony survival assay (CSA) by testing cells from 104 patients with typical A-T, 29 phenotypic normal patients, and 19 A-T heterozygotes. We also analyzed 61 samples from patients suspected of having A-T and 25 patients with related disorders to compare the CSA with other criteria in the diagnosis of A-T. RESULTS: When cells were irradiated with 1.0 Gy, the mean survival fraction (microSF +/- 1 SD) for patients with A-T was 13.1% +/- 7.2% compared with 50.1% +/- 13.5% for healthy control patients. These data served to define a diagnostic range for the CSA (ie, <21%), a normal range (>36%), and a nondiagnostic intermediate range of 21% to 36%. The mutations of patients with A-T with intermediate radiosensitivity tended to cluster around the functional domains of the ATM gene. CONCLUSIONS: The CSA is a useful adjunctive test for confirming an early clinical diagnosis of A-T. However, CSA is also abnormal in other chromosomal instability and immunodeficiency disorders.     

Hemobilia in a patient with multiple intrahepatic,hepatic artery aneurysms

Hemobilia is a rare form of upper gastrointestinal system (GIS) bleeding. Rupture of the hepatic artery aneurysm into the biliary tract is also an uncommon cause of hemobilia. We report a case of hemobilia, secondary to rupture of one of multiple aneurysms of both right and left hepatic arteries. It was treated by hepatic artery ligation.