Haemophagocytic lymphohistiocytosis associated with liver injury in systemic sarcoidosis.

Hepatic involvement in sarcoidosis, though common, is usually asymptomatic. Hepatomegaly and deranged liver function tests are the usual manifestations. However, unexplained hepatomegaly in sarcoidosis not responding to immunosuppressive therapy could indicate an alternative pathology. Haemophagocytic lymphohistiocytosis (HLH), although seldom reported in sarcoidosis, can cause hepatosplenomegaly and cytopenias. HLH occurring concomitantly with hepatic sarcoidosis is extremely rare. We report a patient of systemic sarcoidosis who presented with fever, hepatosplenomegaly and jaundice despite being on steroid therapy. He was subsequently diagnosed with HLH. The clinical response to treatment with pulse steroid and oral cyclosporine was dramatic.

Management outcomes of mesenteric cysts in paediatric age group.

BACKGROUND: Mesenteric cysts are rare intra-abdominal masses and high index of suspicion is required to clinically suspect this pathology and make a definitive diagnosis. They are most commonly located in the ileal mesentery but can be located anywhere along the gastrointestinal tract mesentery and may extend into the retroperitoneum. The rarity of these anomalies and diversity of clinical picture pose challenges in the diagnosis and operative management. The objective of this study is to determine the demographic pattern, clinicopathological diagnosis and management outcome of mesenteric cysts in our hospital. MATERIALS AND METHODS: This retrospective study was conducted on children operated for mesenteric cysts from October 2013 to September 2020. Statistical analysis was performed using Open Epi Version 3.01. RESULTS: Out of 32 children with a diagnosis of mesenteric cyst, 78% were below 5 years of age. Small intestine was affected in 26 cases and large bowel mesentery was the site of origin in six cases. Complete excision of mesenteric cyst was possible in all cases. Bowel resection was required in ten cases (31%) to enable complete excision. Bowel resection was required more often in children operated on emergency than those with elective surgery, and is statistically significant (P = 0.04; confidence interval: 0.05-0.96, odds ratio: 0.23). An uncommon co-existence with ileal atresia and detection of rare chylolymphatic cyst in another infant were also remarkable findings. Histopathology proved the diagnosis in all cases. There was no mortality or recurrence during hospitalisation and follow-up, which ranged from 3 months to 3 years. CONCLUSIONS: Mesenteric cysts present with diverse clinical features and children below 5 years of age are most commonly affected. Complete surgical excision is the optimal treatment and may require bowel resection in significant number of cases, especially those operated during emergency. Histopathology is the gold standard for diagnosis.

Clear cell lesions in pathology: Histomorphologic approach to diagnosis.

There has been remarkable progress in the field of surgical pathology; however, histomorphology has remained the most important and essential tool of the surgical pathologist in everyday practice till now. It is surprising that the hematoxylin-eosin (H and E) stain, introduced more than a century ago, has still remained the gold standard stain for histological examination and diagnosis of human diseases. Besides different findings or clues observed in histopathology sections like inclusions, granules, grooving, globules, halo, or clearing, which would enable the pathologist to provide a precise and accurate diagnosis; observation of clear cells is one of the important findings and clue for reporting. It may also sometimes lead to difficulties and delays in establishing the diagnosis. It can be focal or extensive and primary or rarely it may be secondary. Clear cell changes may be observed in many non-neoplastic, benign, or malignant tumors of diverse origin. Clear cell tumors contain a preponderance of clear cells. It can be seen in almost all the organs of human body and can be classified according to location or biological behavior. Commonly seen clear-cell tumors are usually malignant and common organs involved are female genital tract, urogenital tract, head and neck areas, central nervous system, skin, and rarely in bone and soft tissues. For approach to clear cell lesions, one has to decide if the change is artifactual, a mimic of clear cell tumors, or a clear cell tumor in reality. Once the mimics and artifactual/degenerative changes have been ruled out, a tumor either primarily of clear cell origin or showing secondary change has to be decided. The tumor next is to be diagnosed as benign/malignant and epithelial/mesenchymal based on morphology.

Biopsy Proven Fibrosis in Non-Alcoholic Fatty Liver Disease: An Analysis of Risk Factors.

BACKGROUND: Non-alcoholic fatty liver disease (NAFLD) is emerging as an important cause of liver disease in India. NAFLD is characterized by hepatic steatosis in absence of a significant alcohol use or other known liver disease. Non-alcoholic steatohepatitis (NASH) is a progressive form of NAFLD which deserves particular attention because it is more prone for development of fibrosis. Liver biopsy is the gold standard for diagnosis of NASH by evaluating necroinflammatory activity and stages of fibrosis. The aim of the study was to analyze liver biopsy specimens and identify risk factors associated with fibrosis in patients of NAFLD in eastern coastal India. METHODS: A total of 216 subjects with fatty liver in ultrasonography (USG) were selected for needle biopsy. Those NAFLD cases showing fibrosis in biopsy were analyzed for risk factors association. RESULTS: Definite NASH was diagnosed in 50 (23.14%), borderline NASH in 66 (30.55%) and not NASH in 100 (46.39%) of cases. Those patients with fibrosis (22%) were taken as cases and those without fibrosis (78%) were taken as controls for risk factor analysis. Age > 40 [odds ratio (OR) 2.01 (1.09-4.04)], female gender [OR 2.74 (1.24-6.05)], body mass index (BMI) > 23 [OR 15.36 (4.59-51.37)] and moderate fatty change in USG [OR 1.89 (1.01-3.62)] were observed as risk factors for progression to fibrosis in NAFLD cases. CONCLUSION: Older age, females, obesity and moderate fatty liver on USG are risk factors for development of fibrosis in patients with NAFLD. Patients with these risk factors should be selected for liver biopsy and to be kept for close follow-up.

Trucut Biopsy vs FNAC of Pelvic Tumors-Who Wins the Match?

Preoperative pathologic diagnosis of pelvic tumors is mandatory for proper management of patients like neoadjuvant chemotherapy and interval debulking. Currently there are many minimally invasive methods available which include fine-needle aspiration cytology (FNAC) and trucut biopsy, mostly complimentary to each other. FNAC is a cheap, rapid and sensitive method for diagnosis of pelvic tumors. It can be done as an outpatient procedure without complications. But with it, the tissue architecture cannot be seen. Trucut biopsy on the other hand reveals tissue architecture and can help in grading and subtyping of malignant tumors. Trucut biopsy has to be done under image guidance like ultrasound and computed tomography. Patient is administered local anaesthetic and can be discharged safely after 2 hours. Pathologists familiar with histomorphology can give a correct diagnosis easily. But many times sampling errors may occur; especially in large tumors, resulting only in necrosis, hemorrhage and degenerated tissue bits. Also differentiation of borderline from malignant ovarian tumors is very difficult. In case of mixed tumors one component may be missed. Hard tumors like fibromas and leiomyomas yield scanty material and result in inadequate reporting. With FNAC, the overall accuracy rate is estimated to be around 96.3%. With trucut biopsy, adequacy is from 91 to 95% and accuracy is approximately 98% in different studies. When both methods are combined, the adequacy is 100%, diagnostic accuracy 95.5%, sensitivity 94.9% and specificity 100%. Therefore depending on the clinical diagnosis and the location of tumors, either FNAC and/or trucut biopsy can be chosen.

Apple-Peel Intestinal Atresia Along with Isolated Jejunal Duplication Cyst in a Newborn - An Extremely Rare Case Report and Brief Review.

Apple-peel type of intestinal atresia and non-communicating jejunal duplication cyst are rare congenital malformations. The coexistence is not reported in English literature. A five-day-old female neonate having intestinal obstruction and was found to have both the anomalies during laparotomy and was successfully managed. Being an extremely uncommon association between two congenital anomalies of gastrointestinal tract and surgical emergencies, it is reported with review of relevant literature.

Biochemical Profile of Nonalcoholic Fatty Liver Disease Patients in Eastern India with Histopathological Correlation.

Aminotransferase assay is often used as a screening test as well as an endpoint for resolution of disease in nonalcoholic fatty liver disease (NAFLD). Aim of the study was to evaluate the relationship of transaminase level with metabolic variables and histology in NAFLD. Single center observational study was conducted in a gastroenterology clinic at Cuttack in coastal Odisha. Subjects were consecutive patients presenting with functional bowel disease and undergoing abdominal sonography. All participants were evaluated for the presence of metabolic syndrome (MS), insulin resistance, liver function test and lipid profile. Various parameters were compared between NAFLD subjects and controls. 53.5 % of NAFLD had normal serum transaminases, whereas 20.8 % of healthy controls had transaminitis. NAFLD patients had significantly higher BMI, fasting plasma glucose, serum transaminases, serum triglycerides, serum insulin and homeostatic model assessment (HOMA) IR than controls. NAFLD patients who had transaminitis had significantly higher incidence of MS and higher mean HOMA IR than those without. There was no significant difference in histopathological features between NAFLD with and without transaminitis. To conclude, over half of NAFLD subjects do not have transaminitis while transaminitis is present in a fifth of healthy people without fatty liver. Hence serum transaminase should not be used as screening test for NAFLD. NAFLD patients with transaminitis had a higher incidence of MS and insulin resistance than those without. However, there was no significant difference in histopathological features between these two groups.

Pheochromocytoma in a Child without Hypertension: A Contribution to the "Rule of 10s".

Pheochromocytoma (PCC) is a neuroendocrine tumor originating from chromaffin tissue in adrenal medulla. Its diagnosis and treatment are well defined in adults, but experience in children is limited. Children constitute only 10% of reported cases, the average age at presentation being 11 years. The most common presentation is sustained hypertension, which is absent in only 10% of children. We managed a 14-month-old female child with PCC, but she was not hypertensive. We report two unusual features, in this case, an extremely young age at presentation and a childhood case of nonhypertensive PCC contributing for "rule of 10s."

Histological Evaluation of Non-alcoholic Fatty Liver Disease and Its Correlation with Different Noninvasive Scoring Systems with Special Reference to Fibrosis: A Single Center Experience.

BACKGROUND: Although liver biopsy remains the gold standard for the diagnosis of non-alcoholic fatty liver disease [NAFLD], many non-invasive markers of liver fibrosis have recently been proposed and assessed as surrogates of liver biopsy. AIMS AND OBJECTIVE: To evaluate the degree of liver fibrosis by different non-invasive fibrosis scoring systems and to compare each non-invasive fibrosis scoring system with histological fibrosis stage. MATERIALS AND METHODS: The study population consists of consecutive patients with biopsy proven NAFLD. Complete medical history was taken and physical examination was done in all patients along with appropriate biochemical evaluations. NAFLD fibrosis score, BARD score, BAAT score and APRI score were calculated and each score was compared with histological fibrosis staging. RESULTS: The study population consisted of 60 patients having mean age 39.73 years (SD 9.62, range 17-63 years) including 51 (85%) males and 9 (15%) females. On histology fibrosis was present in 68.3% (41/60) patients. Out of 60 patients 41 had fibrosis and among them 17, 22, 2 patients had grade 1, 2, 3 fibrosis respectively and no one had grade 4 fibrosis. 61.67% (37/60) had definite NASH. Comparing the fibrosis of histology with the noninvasive scoring systems, the sensitivity and specificity of NAFLD fibrosis score were 5.56% and 100% respectively. BARD score had 45.83% sensitivity and 80.55% specificity. The sensitivities of BAAT score and APRI score were 0% and 29.16% respectively and the specificities were 100% and 97.22% respectively. CONCLUSION: The noninvasive scoring systems like NFS, BARD, BAAT, and APRI are not sensitive enough to detect fibrosis but highly specific to include fibrosis if scores are more than cut-off values in our cohort, however they cannot replace liver biopsy. Newer more efficient non-invasive scoring systems have to be devised for the Indian NAFLD population.

A 10-year-old child presenting with syndromic paucity of bile ducts (Alagille syndrome): a case report.

BACKGROUND: Alagille syndrome, a rare genetic disorder with autosomal dominant transmission, manifests with five major features: paucity of interlobular bile ducts, characteristic facies, posterior embryotoxon, vertebral defects, and peripheral pulmonary stenosis. Globally, only 500 cases have so far been reported, with only five cases reported in the Indian subcontinent. Rarely, Alagille syndrome also presents with skin manifestations and early-onset chronic liver disease, which was found in our case. We believe that we report what could be the first case of Alagille syndrome presenting with café au lait spots, as no such published case report could be found in the literature. CASE PRESENTATION: We report an unusual case of childhood cholestatic jaundice with neonatal onset of jaundice. A 10-year-old boy from the Indian subcontinent presented with obstructive jaundice from early infancy. He also had recurrent fractures of his upper limb bones, intermittent bleeding from his nose, productive cough, decreased night vision, hyperpigmented spots over his skin, and progressive enlargement of his abdomen. Histological examination of a liver biopsy specimen revealed a paucity of bile ducts and changes suggestive of chronic liver disease. Our patient was diagnosed with Alagille syndrome and managed conservatively but died 1 year after the final diagnosis. CONCLUSIONS: This particular syndromic form of paucity of bile duct disorder has been rarely reported in the Indian literature so far. Our case is notable because the child had café au lait spots and very early onset of chronic liver disease, which is quite rare in Alagille syndrome. We believe this to be the first case report on Alagille syndrome manifesting with café au lait syndrome and such early onset of chronic liver disease.

Risk Factors Associated With Non-Alcoholic Fatty Liver Disease in Indians: A Case-Control Study.

BACKGROUND/AIMS: NAFLD has today emerged as the leading cause of liver disorder. There is scanty data on risk factors associated with NAFLD emanating from India. The present study was conducted to identify the risk factors associated with NAFLD. METHODS: 464 consecutive NAFLD patients and 181 control patients were subjected to detailed questionnaire regarding their lifestyle and dietary risk factors. Anthropometric measurements were obtained and biochemical assays were done. Comparison of different variables was made between NAFLD patients and controls using principal component analysis (PCA). RESULTS: NAFLD patients had higher BMI [26.25 ± 3.80 vs 21.46 ± 3.08 kg/m(2), P = 0.000], waist-hip ratio [0.96 ± 0.12 vs 0.90 ± 0.08, P = 0.000] and waist-height ratio [0.57 ± 0.09 vs 0.50 ± 0.06, P = 0.000] compared to controls. Fasting blood sugar [101.88 ± 31.57 vs 90.87 ± 10.74 mg/dl] and triglyceride levels [196.16 ± 102.66 vs 133.20 ± 58.37 mg/dl] were significantly higher in NAFLD group. HOMA-IR was also higher in NAFLD group [2.53 ± 2.57 vs 1.16 ± 0.58, P = 0.000]. Majority (90.2%) of NAFLD patients were sedentary. Family history of metabolic syndrome (MS) was positively correlated with NAFLD. Dietary risk factors associated with NAFLD were non-vegetarian diet [35% vs 23%, P = 0.002], fried food [35% vs 9%, P = 0.000], spicy foods [51% vs 15%, P = 0.001] and tea [55% vs 39%, P = 0.001]. Diabetes, hypertension, snoring and sleep apnoea syndrome were common factors in NAFLD. On multivariate PCA, waist/height ratio and BMI were significantly higher in the NAFLD patients. CONCLUSION: The risk factors associated with NAFLD are sedentary lifestyle, obesity family history of MS, consumption of meat/fish, spicy foods, fried foods and tea. Other risk factors associated with NAFLD included snoring and MS.

Nonalcoholic fatty liver disease (NAFLD) without insulin resistance: Is it different?

BACKGROUND AND AIMS: Nonalcoholic fatty liver disease (NAFLD) is considered the hepatic manifestation of insulin resistance [IR]. However, a significant proportion of NAFLD patients are devoid of IR. Is NAFLD sans IR a different entity? The aim of the study was to compare the anthropometric, metabolic, biochemical, ultrasonography, and histological profile of NAFLD patients with and without IR. METHODS: Retrospective analyses of 336 NAFLD patients diagnosed during the last two years was done. Patients without IR were compared with those with IR. RESULTS: Out of 336 patients, 153 [45.53%] were without IR. Although age, gender, BMI and transaminase levels were comparable, significantly higher proportion of patients in non-IR group were non-obese [43.14% vs. 25/14%; P=0.0005], and had mild fatty change on ultrasonography; [78.43% vs. 67.21%; P=0.022]. Higher proportion of them had elevated transaminases; [67.97% vs. 56.83%; P=0.036]. Serum triglyceride [178.52±78.78 vs. 204.86±94.72 mg/dl; P=0.02], FBG [85.39±13.80 vs. 98.93±31.56 mg/dl; P=0.00], PGBG [123.76±36.77 vs. 148.07±64.67m g/dl; P=0.00], and serum insulin [6.33±2.18 vs. 15.39±12.56 µIU/ml; P=0.00] were significantly lower in patients without IR. Although there was no difference in histology, interestingly fibrosis was seen in one third of patients despite absence of IR. CONCLUSION: Nearly half of our NAFLD population was without IR; one third of them had significant fibrosis. NAFLD is probably a heterogeneous disease and IR is not the sole factor responsible for NAFLD; further studies are needed to find out other possible etiological factors.

Cryptococcal lymphadenitis in HIV: a chance diagnosis by FNAC.

Cryptococcosis is a life threatening, opportunistic fungal disease in human immunodeficiency virus-infected individual. Lymph node involvement as a presenting feature in Cryptococcosis is not a common manifestation. A prompt diagnosis is of utmost importance in this disseminated form of cryptococcosis. There are very few reports, however, of cryptococcal lymphadenitis as a presenting feature. We report here a case of cryptococcal lymphadenitis that was diagnosed by fine-needle aspiration cytology of the involved cervical lymph node.

Profile of patients with incidentally detected nonalcoholic fatty liver disease (IDNAFLD) in coastal eastern India.

BACKGROUND AND AIM: Non-alcoholic fatty liver disease (NAFLD) has emerged as the commonest hepatic problem in both developed and developing countries. This study was conducted to delineate the clinical profile of incidentally detected NAFLD patients. METHODS: A retrospective analysis of clinical, biochemical and histological parameters was performed for 632 patients in whom fatty liver was diagnosed by ultrasonography during 2006-2010. Every patient's clinical presentation, history, liver function tests, blood sugar, lipid profile, ultrasonogram, histology and other relevant investigations were analyzed. Patients referred for transaminitis or overt chronic liver disease were excluded. RESULTS: Out of 632 consecutive NAFLD patients, 484 (76.6%) were males and 148 (23.4%) were females. Mean age of our patients was 42.44 +/- 10.18 years. Their mean body mass index (BMI) was 26.14 +/- 3.38 kg/m2. Obesity (BMI > 25), diabetes mellitus, hypertension, hyperlipidemia, metabolic syndrome and insulin resistance were present in 61.7%, 15.2%, 19.8%, 62.0%, 40.0%, and 54.4% patients, respectively. SGOT and SGPT were elevated in 38.29% and 55.1% patients, respectively. The common primary diseases for which these patients sought consultation were non-ulcer dyspepsia (NUD) (54.5%) and irritable bowel syndrome (IBS) (29.4%). Of the 64 patients who underwent liver biopsy, one-fourth had definite NASH while one-third patients had fibrosis. CONCLUSION: In India, incidentally detected NAFLD (IDNAFLD) patients are predominantly middle aged males, most of whom are not lean. Most of these patients seek consultation for functional bowel disease.

Adenoid cystic carcinoma in the uterine cervix: a case report.

BACKGROUND: Adenoid cystic carcinoma, a variant of adenocarcinoma of the uterine cervix, is an extremely rare neoplasm. This report describes a case of primary adenoid cystic carcinoma of the uterine cervix and includes the cytopathologic and histopathologic findings. CASE: A 55-year-old woman presented with postmenopausal bleeding for 2 months. Speculum examination showed a firm mass in the uterine cervix. A Pap smear was obtained, which showed small epithelial cells with hyperchromatic nuclei and coarse chromatin dispersed and adhered around hyaline stromal globular material, along with a few atypical squamous cells. The diagnosis of adenoid cystic carcinoma was confirmed by the histopathologic examination of the biopsy specimen as well as the excised specimen, which characteristically revealed the presence of a cribriform pattern of the tumor cells. CONCLUSION: We reported a case of a rare and unusual variant of adenocarcinoma of the uterine cervix by considering the characteristic cytologic features of the globules of pale, translucent material surrounded by halos of cells in cribriform adenoid cystic carcinoma.

Intravitreal live adult Brugian filariasis.

Human ocular infestation by live filarial worm is a rare occurrence and has been reported mostly form South-East Asia. It involves the eyelids, conjunctiva, cornea, anterior chamber and uvea. No case of intravitreal Brugian microfilaria / adult worm has been found by Medline search. Here we report a case of live intravitreal adult Brugian filaria, where the parasite was successfully removed by pars plana vitrectomy. Identification of the worm was done by light microscopy and confirmed by immuno chromatographic test.