Nomimmune hydrops fetalis due to Diamond-Blackfan anemia.

We describe case report of a baby with Diamond-Blackfan anemia, who presented as non-immune hydrops fetalis. The diagnosis was confirmed by measurement of red cell adenosine deaminase activity which is increased in Diamond-Blackfan anemia. At 2 years of age he is dependent on small dose of alternate day steroid to maintain his hemoglobin.

Intussusception due to gastrointestinal stromal tumour of the colon: diagnosis by colonoscopy.

Intussusception of the intestine is rare in adults. We report a case of a 45-year-old male who presented with long-standing pyrexia of unknown origin, abdominal pain, gastrointestinal bleeding and constitutional symptoms. Colonoscopic examination revealed a large invaginated mass suggestive of colonic intussusception due to gastrointestinal stromal tumour of the colon. His symptoms disappeared after surgical removal of the tumour.

Functional outcome and patient satisfaction after ileal pouch anal anastomosis for ulcerative colitis in a developing country.

AIM: To study the long-term outcome and patient satisfaction of patients with an ileal pouch-anal anastomosis (IPAA) for ulcerative colitis in India. PATIENTS AND METHODS: We studied 35 patients who had undergone IPAA for ulcerative colitis between 1985 and 1998 and had intestinal continuity restored for more than 6 months. These patients were asked to answer a detailed questionnaire on their bowel function, urogenital function, etc. A complete haemogram, serum iron studies, liver function tests and D-Xylose absorption test were done. In addition hepatobiliary ultrasound, stool microscopy, pouchoscopy and pouch biopsies were also performed. Patient satisfaction after the procedure was also evaluated. RESULTS: Thirty-five patients (17 men and 18 women) underwent a complete evaluation. The duration after restoration of continuity ranged from 6 months to 164 months (mean 78.6 months). The mean stool frequency was 7.2 stools per 24 hours. Five patients had urgency of stool, 9 had occasional soiling and 1 had major incontinence. Four patients had minimal restriction of social activities and 1 discontinued his employment. All patients were sexually satisfied except one man who had impotence and one woman who had dyspareunia. Fifteen patients had abnormal serum iron studies (Haemoglobin < 9 g/dl in 11). Eleven patients had D-Xylose absorption below normal values. Two patients were found to have gallstones. All pouch biopsies showed chronic inflammation and 1 patient had histological evidence of pouchitis. Eighty-five percent of patients reported that they were very satisfied with the procedure. CONCLUSION: Good functional recovery and acceptance of the procedure over the long term suggests that it is a valid procedure to be recommended for patients with ulcerative colitis in India.

Video assisted thoracoscopic surgery (VATS) in the diagnosis of intrathoracic pathology: initial experience.

We report our experience with the use of video-assisted thoracoscopic surgery [VATS] in the diagnosis of intrathoracic disease of unknown origin. In the last two years, 32 patients (18 males) underwent this procedure for diagnostic purposes. Of them, 18 patients had lung pathology, eight mediastinal and six pleural disease. All attempts at achieving a tissue diagnosis were unrewarding. In all of them, diagnostic thoracotomy was being contemplated to procure tissue for histopathological diagnosis. Diagnostic thoracoscopy was successful in providing tissue diagnosis in all the patients with lung disease; seven of the eight patients with mediastinal pathology and five of the six patients with pleural lesions. Diagnostic thoracoscopy was associated with minimal morbidity, short hospital stay, better cosmetic result and quicker return to work as compared to conventional thoracotomy. We conclude that video-assisted thoracoscopy is an excellent diagnostic tool to confirm tissue diagnosis in patients with undiagnosed chest diseases.

Dumping syndrome in the intra-thoracic stomach.

Thirty seven patients who survived at least 6 months after oesophagectomy and cervical oesophagogastrostomy for benign and malignant diseases of the oesophagus were evaluated for dumping syndrome. Twenty two of these patients had a pyloroplasty and fifteen had no drainage procedure. All these patients were subjected to a dumping provocation test. Evidence of dumping was noted in four patients, all having a pyloroplasty. Clinically, these four patients had mild symptoms which improved with dietary adjustment. We conclude that dumping does occur following thoracic transposition of the stomach and seems to occur more in patients with pyloroplasty than those without it.

Splenic abscess--case report and review of literature.

Isolated splenic abscess is a rarity and remains a diagnostic dilemma. The presentation is nonspecific and diagnosis is often delayed. We present a case which had roentgenographic signs suggestive of splenic suppuration at admission. Of particular interest was the isolation of Salmonella paratyphi in this patient. Antibiotic therapy alone is insufficient and splenectomy remains the treatment of choice. The literature on splenic abscess is briefly reviewed.

Inhibition of bacterial respiration by a low-molecular weight lectin, scyllin, from Scylla serrata crab hemolymph.

Interaction of plant and/or invertebrate lectins with mammalian cells and different microorganisms is well known. In the present study, we have demonstrated that scyllin, a low molecular weight (MW 4000) lectin from the edible crab Scylla serrata hemolymph, purified by GalNAc-Sepharon affinity column followed by Mono-Q ion exchanger in FPLC exhibits antimicrobial activity against Bacillus cereus and Escherichia coli by inhibiting endogenous respiration as well as exogenous glucose oxidation. In both the cases oxygen consumption has been measured in an oxygraph. Scyllin has produced 50% inhibition of endogenous respiration at a concentration of 110 micrograms/ml and 125 micrograms/ml in B. cereus and E. coli respectively. It also reduced the exogenous glucose oxidation by 50% at a concentration of 12 micrograms/ml and 80 micrograms/ml respectively in B. cereus and E. coli. From the above study the mechanism of bacterial growth inhibitory property of scyllin is suggested though the other studies such as inhibition of nucleic acid biosynthesis, cell wall biosynthesis etc. to evaluate its total mode of inhibitory action are not yet obtained.

Short segment oesophago-cardiomyotomy for achalasia cardia.

Over a seven year period, 25 patients of achalasia cardia underwent a transabdominal short segment oesophago-cardiomyotomy. There was no operative mortality. Two patients had a mucosal tear, detected intraoperatively and promptly repaired. All patients were regularly followed up (range 1 to 7 years). Clinical results were excellent in 76%, good in 20% and fair in 4%. No patient developed reflux or required reoperation for residual dysphagia. We conclude that a transabdominal short segment oesophago-cardiomyotomy performed carefully is a safe and effective procedure in the treatment of achalasia.

The role of estimation of the ratio of preoperative serum thyroglobulin to the thyroid mass in predicting the behaviour of well differentiated thyroid cancers.

Although serum thyroglobulin (STg) is a useful tumour marker to detect the recurrence of tumour in well differentiated thyroid carcinoma, it has as yet not been reported to be of value in predicting the behaviour of thyroid cancer. In the present study of 20 patients, the measurement of preoperative Stg/thyroid mass has been utilised to find out Tg synthesizing capacity of the tumour. This ratio was significantly higher in the patients with follicular variety than in papillary thyroid carcinoma. It was significantly higher in the metastasis group than in the group of patients without metastasis. The patients with functioning metastasis had a higher than average value of this 'ratio' than those with non functioning metastasis, though the difference was not statistically significant. Despite the limitation of a small number of patients included in this study, it is possible to highlight the possible utility of preoperative Stg estimation as a tumour marker in categorization of the patients of carcinoma of the thyroid gland.

Structural and functional aspects of the thoracic stomach.

The acid secretory and emptying function of the thoracic stomach was evaluated in 10 patients, following total oesophagectomy and gastric pull-up for oesophageal cancer. All patients had a Heinecke Mickulicz pyloroplasty. Endoscopic features and histological review of endoscopic biopsies were also studied in these patients. Acid secretion was higher in those with prolonged emptying. This was however, not statistically significant. Gastritis was more severe in those with prolonged emptying. Endoscopic diagnosis of gastritis was found unreliable while histopathology of endoscopic biopsy gave definite evidence of gastritis.

Prognostic significance of argyrophilic nucleolar organizer regions (AGNOR) in oesophageal cancer.

AgNOR (Argyrophilic nucleolar organiser region) has been shown in recent times, to have value in knowing the prognosis of carcinoma oesophagus. We have evaluated the significance of AgNOR in oesophageal cancer with reference to prognosis following treatment. Fifty patients of histologically proven squamous cell carcinoma of the oesophagus were studied. Following oesophagectomy the specimens removed were evaluated for AgNOR number. Of the resected specimens, 25 (50%) had an AgNOR count < or = 3.0, 18 (36%) had an AgNOR count of more than 3 per nucleus and in the remaining 7 cases, AgNOR number was not quantifiable due to the total absence of tumour tissue in the postoperative specimen due to preoperative radiotherapy. When followed up for an average period of 25 months (3 to 47 months), it was seen that patients with AgNOR count of < or = 3.0 per nucleous had a similar mean survival (30.39 +/- 3.29 months) as those with counts > 3.0 per nucleus (27.80 + 3.33 months). The survival in the seven patients in whom no tumour was present following preoperative radiotherapy, was 30.30 +/- 2.42 months. An analysis was done for the presence of change in the AgNOR count before and after radiotherapy in twenty eight case of carcinoma oesophagus treated with preoperative radiotherapy. It was found that the counts on an average were lower in patients after radiotherapy (2.89 +/- 1.04 per nucleus), than before radiotherapy (3.17 +/- 9.69). This was found in 24 cases, while the remaining 4 cases showed no change in count after radiotherapy. This suggested that radiotherapy caused a reduction in AgNOR counts. Mean survival in those with decreased count after radiotherapy was 33.65 (+/- 3.35) months. Since the AgNOR counting is a simple method and can be applied to paraffin embedded section, estimation of the AgNOR number may help in determination of prognosis in patients with oesophageal carcinoma. Preoperative radiotherapy seems to decrease AgNOR count with improved survival. These observations need however, to be reproduced with a larger sample size.

Spontaneous hemoperitoneum due to rupture of hepatocellular carcinoma.

Spontaneous rupture with hemoperitoneum is one of the atypical modes of presentation of hepatocellular carcinoma (HCC), common in the orient (8-14% cases) but reported only once from India. Another case is being reported here. Hemostasis can be achieved by transcatheter hepatic artery ligation, intralesional alcohol injection, microwave application, curettage with suturing or gauze packing. Resectional surgery, preferably at a second stage, has the best long term outcome. Prognosis remains poor with few long term survivors.

Fine needle aspiration cytology, sonography and radionuclide scanning in solitary thyroid nodule.

One hundred and ninety three consecutive patients with solitary thyroid nodule (STN), with a mean age of 36.0 +/- 12.8 years and male to female ratio of 5.6:1, were studied. Ninety five percent of patients came from iodine deficient regions. Seventy two percent presented for local neck swelling, 12.4% for hyperthyroid state and in 7.7% STN was discovered incidentally. Scintigraphically, 77.7% of nodules were cold, 12.4% hot and 8.5% warm. Sonographic evaluation did not reveal any characteristic echotexture diagnostic of malignancy, but detected clinically nonpalpable accessory nodules in 20.6% of patients. Fine needle aspiration cytology was positive for malignancy in 6.2% of patients. Features of follicular and Hurthle cell neoplasm were seen in 12.9% of aspirates. Eighty eight (45.6%) STN were resected surgically. Histologically, there was one false positive and one false negative aspirate and a case of parathyroid adenoma on aspiration proved to be parathyroid carcinoma on histology.

Intussuscepting ileal hemangioma with perforation.

A rare case of solitary intussuscepting capillary hemangioma in the distal ileum with perforation and peritonitis is presented. The diagnosis was made only at surgery for presumed acute intestinal obstruction.