Colorectal carcinoma up to the second decade of life: An 8‑year experience in a tertiary care center.

AIMS AND OBJECTIVES: To evaluate the demographic pattern, incidence, and histological characteristics of colorectal carcinomas (CRCs) in very young adults diagnosed in the center. MATERIALS AND METHODS: We retrieved and reviewed slides and data pertaining to all the cases of CRCs and “segregated into decade wise age‑groups” from the archives of Department of Pathology. Patients with age ≤20 years diagnosed during the last 8 years (2006–2013) were further evaluated. RESULTS: Totally, 590 cases of CRCs diagnosed over last 8‑year period, of which 4.2% (25 cases) presented in the study group (age ≤20 years) with a mean age of 17 years. About 50% of the tumors were either signet ring cell, mucin‑secreting or poorly differentiated carcinomas. Four cases occurred in a background of familial adenomatous polyposis (FAP), three of which showed high‑grade dysplasia, while in one case, carcinoma‑in‑situ was diagnosed. In all but two cases, rectum was the site of involvement except FAP cases in which colorectal location was noted. CRCs show a sharp rise in earlier age onset (≤40 years) and an increasing trend was followed in patients between age groups third, fourth, and fifth decades of life over the last 8 years. CONCLUSION: Colorectal carcinomas show an increasing trend in young age (≤40 years). This change may be attributed to dietary, lifestyle changes, and newer genetic alterations in developing countries. In very young age group (≤20 years), a higher grade and stage at the time of diagnosis and predominantly rectal involvements are the distinct features.

Non-diarrheal celiac disease: a report of 31 cases from northern India.

BACKGROUND AND AIMS: Non-diarrheal presentation of celiac disease (CD) is being increasingly recognized. Data on this form of CD from India are limited. METHODS: Consecutive patients with CD presenting to a tertiary referral center in northern India over a 3-year period were studied, with special emphasis on non-diarrheal celiac disease (NDCD). Diagnosis was based on the presence of autoantibodies typical of CD (IgA anti-tissue transglutaminase antibodies and/ or IgA endomysial antibodies), abnormal duodenal biopsy and response to gluten-free diet (GFD). Clinical, hematological and histological responses were assessed over a one-year period after instituting GFD. RESULTS: Of 86 patients with CD, 31 (16 children, 15 adult) had NDCD. Mean (SD) age of these children (12 boys) and adults (4 male) was 10.2 (4.2) y and 35.3 (12.0) y, respectively. Failure to thrive was the most common (11/16) presentation in children, as was refractory anemia in adults (10/15). Malabsorption was found in 8 adults (54%) and 10 children (64%) with NCCD. The duration from onset of symptoms to diagnosis was 2.9 (1.5) y in children and 3.3 (0.3) y in adults. There was significant improvement in body weight (children--baseline 18.9 [5.8] Kg, follow up 27.4 [12.4] Kg; adults--baseline 47.6 [18.2] Kg, follow up 54.9 [5.1] Kg) and hemoglobin (children--8.1 [2.0] g/dL to 11.2 [1.4] g/dL; adults--7.3 [2.3] g/dL to 9.7 [1.7] g/dL) in both groups after one year of GFD; duodenal biopsy also improved, with a majority of patients attaining normal to IIIa Marsh grading. Five adults and all children had evidence of metabolic bone disease at presentation, which did not revert completely with GFD. Eight adults and nine children showed dietary transgression 6 weeks after starting GFD. CONCLUSION: NDCD ac-counted for nearly one-third of all cases with CD at our center, with 'failure to thrive' and refractory anemia being the most common presentations in children and adults, respectively.

Non-resolving liver abscess with Echinococcus cross-reactivity in a non-endemic region.

A 40-year-old woman presented with high fever with chills and rigors. Imaging studies revealed multiple liver abscesses with hepatosplenomegaly and gall-stones. Ultrasound-guided aspirate revealed pus that was negative on Gram and acid-fast staining and for amebic trophozoites. ELISA for echinococcus was strongly positive, but she did not respond to albendazole therapy. At surgery, Fasciola hepatica was detected and she responded well to bithinol postoperative.

Removal of self-expandable metallic wallstent--a case report.

Self-expandable metallic stents (SEMS) have become the preferred palliative treatment for patients with malignant biliary obstruction; endoscopic stent placement is less invasive than surgery and is rapidly effective. A longer duration of patency makes SEMS superior to plastic stents for palliation of patients with malignant stricture. Few cases of removal of an uncovered SEMS are reported in case of a blocked stent. We report the first case report of removal of covered biliary SEMS from India.

Forme fruste choledochal cyst.

Two girls presenting with features of pancreatitis were diagnosed to have minimal dilatation of extra hepatic biliary duct (EHBD) associated with pancreatico-biliary maljunction (PBMJ). A high degree of suspicion is required to diagnose this condition that has been termed Forme fruste choledochal cyst (FFCC). Both did well with pancreatico-biliary disconnection procedure and reconstruction of biliary channel using enteric conduit.

Reversible cholestatic hepatitis due to carbamazepine in an adolescent.

We report a 13-year-old boy who developed fever, rash and hepatitis with cholestasis (on biochemistry and liver histology) after 10 weeks' use of carbamazepine. Recovery of liver biochemistry occurred 4 months after discontinuing the drug.

Diaphragm disease of duodenum following long-term NSAIDs use: endoscopic management.

We report our experience with endoscopic management of 3 men (aged 62, 63 and 65 years) with duodenal diaphragm disease following NSAID use for 5-15 years. In the first patient a 24 F through-the-scope balloon dilatation was attempted but failed; he subsequently underwent gastro-jejunostomy. The other two patients subsequently underwent radial incisions of the web with mixed cutting and coagulation current using a standard 5 F sphincterotome.

Symptomatic volvulus due to mid gut malrotation in an adult.

Anomalies of rotation and fixation of gut occur most commonly in the neonatal period and usually become symptomatic in infancy. We report an adult patient with symptomatic volvulus due to mid gut malrotation.

Primary biliary cirrhosis: an Indian experience.

Primary biliary cirrhosis (PBC) is believed to be rare in India. We analyzed our data pertaining to patients with PBC seen in a tertiary referral center over a 5-year period. The diagnosis of PBC was based on liver biochemistry, histology and antimitochondrial antibodies, in the absence of biliary obstruction. Five patients, all women, were diagnosed to have PBC. Pruritus, jaundice and fatigue were the most common initial symptoms. Hepatomegaly was seen in 4 of 5 patients. Associated autoimmune diseases were present in 2 patients. All patients presented with mild hyperbilirubinemia (< or = 6 mg/dL) with disproportionately raised serum alkaline phosphatase level. AMA was positive in 4 patients. Liver biopsy showed stage III-IV disease in 3 of 4 patients. The clinical presentation and course of PBC in India are similar to the experience in the West.

Paraganglionoma of extrahepatic biliary tract causing obstructive jaundice.

We report a young woman with paraganglionoma arising from the extrahepatic bile duct presenting with acute obstructive jaundice. The patient underwent excision of the gall bladder and extrahepatic bile duct with the tumor, and Roux-en-Y hepaticojejunostomy. She is asymptomatic 9 months later, with normal biochemical investigations and imaging.

Intestinal lymphangiectasia: presentation in pregnancy and association with herpes zoster and alopecia.

We report a woman with intestinal lymphangiectasia whose symptoms were wrongly attributed to pregnancy; the diagnosis was made in the postpartum period. She also developed alopecia and herpes zoster.

Frequency, microbial spectrum and outcome of spontaneous bacterial peritonitis in north India.

AIM: To determine the prevalence, microbial spectrum and outcome of spontaneous bacterial peritonitis (SBP) and its variants in hospitalized cirrhotics. STUDY DESIGN: Prospective cohort study at a tertiary referral center in North India. METHODS: Over a four-month period, 70 consecutive adult patients with decompensated cirrhosis were screened for the presence of SBP or its variants. Ascitic fluid culture was done by direct inoculation of blood culture bottles at the bedside. Blood, urine and other fluids were cultured during hospital stay when clinically indicated. Ascitic fluid total leukocyte count and culture were repeated at any time during hospital stay if the patient showed clinical signs of deterioration. Patients with SBP and culture-negative neutrocytic ascites (CNNA) were treated empirically on the basis of ascitic fluid leukocyte count. RESULTS: Twenty-one of 70 (30%) patients with cirrhosis were diagnosed to have SBP or its variants CNNA and monomicrobial bacterascites (MBA). Ninety-five percent of the patients who developed this complication were in Child-Pugh class C. A causative organism was isolated in 62% of these patients. Gram-negative bucilli accounted for 6 of 10 patients with SBP whereas all cases of MBA were due to infection with Gram-positive cocei. A third of patients with SBP/CNNA had evidence of extra-abdominal focus of infection with the same organism. All episodes of SBP/CNNA were initially treated with either ciprofloxacin (12 patients) or a combination of third generation cephalosporin, cefotaxime and an aminoglycoside, gentamicin (n = 6). Fourteen patients (67%) recovered whereas 6 patients died during hospital stay. CONCLUSION: SBP is a common complication of decompensated liver disease in North India and is associated with significant in-hospital mortality. Ciprofloxacin is an effective drug for initial treatment of SBP/CNNA. Synchronous extra-peritoneal focus of infection is a frequent occurrence in these patients.

Immunoproliferative small intestinal disease: a frequently missed diagnosis.

Immunoproliferative small intestinal disease (IPSID) is a poorly recognized cause of malabsorption syndrome in India. Clinicopathological features of five patients with IPSID seen over a two-year period are described. Our data suggest that IPSID is commonly misdiagnosed as intestinal tuberculosis due to lack of awareness and reluctance to obtain small bowel biopsies. Empirical institution of anti-tubercular chemotherapy not only leads to delayed diagnosis but also possibly alters the natural history of the disease, resulting in an intermediate phase of amelioration followed by a terminal phase of lymphomatous transformation. The disease is therefore usually diagnosed at an advanced stage and hence is associated with a relatively poor outcome.

Clinical and endoscopic features of hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease) in India.

Hereditary hemorrhagic telangiectasis (HHT) is an autosomal dominant disease characterized by recurrent epistaxis and telangiectasia of the skin and mucous membranes. Most reports of HHT are from Europe and N. America. In this report of 7 patients from India we postulate that increased skin pigmentation in Asians and Negroids masks the cutaneous manifestations of the disease but without any discernible effect on mucosal lesions. The median hemoglobin value in these patients was 4 g/dl. Endoscopic lesions in the stomach or duodenum were detected in six patients. Most patients in our report (5/7) presented with a chronic iron deficiency anaemia. The treatment of HHT is mainly supportive although our preliminary data shows that low dose ethinyl estradiol therapy decreases transfusion requirement in these patients.