Budd-Chiari syndrome has different presentations and disease severity during adolescence.

There are limited data on clinical profile of adolescent patients with Budd-Chiari syndrome (BCS). We studied clinical, radiological, thrombophilia profile and treatment outcomes in adolescent patients with BCS. METHODS: Forty-three consecutive patients of BCS with onset of symptoms during adolescence (10-19 years) were enrolled in the study. 129 randomly selected adult patients with BCS and 36 children with BCS formed the two control groups. The clinical history, physical examination, laboratory tests, thrombophilic disorders, radiological features and treatment outcomes of adolescents were compared to adults and children. RESULTS: In adolescents, ascites (25/43 vs. 110/129, p = 0.0004) and thrombophilic disorders (16/43 vs. 93/129 p < 0.0001) were less frequent than adults. More adolescents (14/43) presented with hepatomegaly alone without ascites than adults (9/129, p < 0.001) or children (1/36, p = 0.005). Adolescents had lower Clichy scores [3.75 (1.2)] than adults [4.72 (1.3), p < 0.0001) or children [4.43 (1.7), p = 0.041]. JAK-2 V617F mutation was the most common thrombophilic disorder in adolescents (5/43) and more common than children (0/36, p = 0.043). Response to therapy was better in adolescents (74.4%) than children (52.8%, p = 0.038), but similar to adults (63.56%, p = 0.13). CONCLUSION: During adolescence, patients with BCS present less commonly with ascites and may present with hepatomegaly alone. JAK-2 V617F mutation is the most common thrombophilic disorder during adolescence; though thrombophilic disorders are less common in adolescents than adults. Response to therapy is similar to adults, but better than children.

Pregnancy outcomes in women with Budd Chiari Syndrome before onset of symptoms and after treatment.

BACKGROUND & AIMS: Pregnancy is known to have poor outcomes in women with Budd Chiari syndrome. There are no data on fertility and pregnancy outcomes prior to onset of symptoms or the effect of therapy on these parameters. We therefore evaluated reproductive profile of women with Budd Chiari Syndrome before the onset of symptoms and after therapy. PATIENTS AND METHODS: Eighty women with Budd Chiari Syndrome (29 years [20-45]) were enrolled over 8 years. Baseline demographic characteristics, disease severity, thrombophilic disorders and treatment were reviewed. Their obstetric history before symptoms and after therapy was noted and compared. RESULTS: Sixty women conceived at least once (150 pregnancies) before symptom onset and 20 had primary infertility. Eighty-one pregnancies resulted in live births and remaining 69 pregnancies had adverse pregnancy outcomes. Post-intervention, 28 women (15 with primary infertility) attempted conception. Thirteen patients conceived 15 times. More women had live births after successful therapy as compared to presymptomatic period (0/28 vs 5/28 P = .000, Odds ratio5.6; 95% CI: 2.16-14.5). In women with primary infertility, conception (0/15 vs 3/15 P = .007, Odds ratio 5, 95% CI: 1.44-17.27) and proportion of live births (0/15 vs 2/15 P = .002, Odds ratio 7.5, 95% CI: 1.71-32.79) was higher as compared to presymptomatic period. CONCLUSION: Primary infertility is common and pregnancy outcomes are poor before the onset of symptoms in women with Budd-Chiari syndrome. Effective therapy of Budd-Chiari syndrome may improve fertility and pregnancy outcomes.

The Role of Red Cell Distribution Width as a Predictor of Mortality for Critically Ill Patients in an Inner-city Hospital.

BACKGROUND: Red cell distribution width (RDW) is a measure of the variation in the red blood cell volume that is usually recorded as a part of the standard complete blood cell count. Recent studies have demonstrated the prognostic value of RDW in many different clinical settings. The objective of this research study is to investigate the independent association of RDW with 30-day mortality in Intensive Care Unit (ICU) patients. METHODS: One hundred and fifty-six patients admitted to the ICU of our hospital between July 2009 and June 2011 were included in our study. Out of 156 patients, 124 survived the hospital stay. The data on patient's demographics, interventions done in ICU, and their comorbidities were collected. Baseline variables and the RDW value were compared between survivors and nonsurvivors. The cutoff point for RDW used for the comparison was 15.75. Both univariable and multivariable analyses were done. P < 0.05 was considered statistically significant. RESULTS: In the univariable analysis of the study between survivors and nonsurvivors, the median RDW was 17.20 for nonsurvivors, implying statistical significance (P = 0.007). In multivariable analysis, RDW remained significantly associated with inpatient mortality. The receiver operating characteristic is 0.656 (P = 0.007), with an optimal cutoff of 15.75 for RDW. At the cutoff of RDW, i.e., 15.75, the sensitivity and specificity for inpatient mortality was 71% and 89%, respectively. CONCLUSION: In critically ill ICU patients, RDW is an independent predictor of 30-day mortality. Taking into consideration the fact that RDW is routinely measured in complete blood count with no additional cost, this can serve as an "inexpensive prognostic marker" in critically ill patients.

Cirrhotic cardiomyopathy is less prevalent in patients with Budd-Chiari syndrome than cirrhosis of liver.

BACKGROUND AND AIM: Cirrhotic cardiomyopathy (CCM) is associated with high mortality after transjugular intrahepatic portosystemic shunt (TIPS) and liver transplantation in patients with cirrhosis. There is no data about the prevalence or impact of CCM in Budd-Chiari syndrome (BCS). We assessed the prevalence of CCM in patients with BCS and its impact on outcome after radiological intervention. METHODS: Thirty-three consecutive patients with BCS (15 men) and 33 controls with hepatitis B-related cirrhosis (18 men, matched for Child-Pugh score) were evaluated with baseline electrocardiography (ECG), echocardiography (ECHO) and dobutamine stress ECHO, and ECG (DSE). The two groups were compared for prevalence of CCM. Patients with BCS with and without CCM were assessed for development of heart failure, duration of intensive care unit (ICU) stay, and in-hospital mortality immediately after radiological intervention. RESULTS: Fewer patients with BCS had CCM (7/21 vs. 21/33; p = 0.001, OR-0.16, CI [0.05-0.5]), diastolic dysfunction (DD) (0/33 vs. 6/33; p = 0.01, OR-0.06, CI [0.00-1.1]), and prolonged QTc interval (5/33 vs.17/33; p = 0.001, OR-0.16, CI [0.05-0.5]) despite correction for age. Patients with BCS had lower end-systolic and end-diastolic volumes of left and right ventricles. None of the 19 patients (five with CCM) with BCS undergoing radiological intervention (12 TIPS, 4 inferior vena cava, and 3 hepatic vein stenting) developed heart failure or had prolonged ICU stay. There was no in-hospital mortality. CONCLUSION: Patients with BCS have lower frequency of CCM as compared to patients with cirrhosis. CCM may not adversely affect outcomes after radiological interventions.

A rare case report of bilateral testicular masses as an initial manifestation of systemic sarcoidosis.

Sarcoidosis is an idiopathic, systemic disease that rarely involves the genitourinary tract. Here, we present a case of a 40-year-old male presented with bilateral scrotal swelling. The scrotal ultrasound showed multiple echogenic masses bilaterally ranging between 3 mm and 15 mm involving both testicles. Enlarged retroperitoneal lymph nodes were detected on the abdominal computed tomography (CT). Surgical exploration of the testes with a frozen section analysis of the left testicular mass was carried out, and it revealed noncaseating granulomas. CT scan of the chest revealed the classic bilateral hilar and mediastinal lymphadenopathy with reticulonodular infiltrates. The final pathological diagnosis was systemic sarcoidosis with bilateral testicular involvement. Treatment with high-dose corticosteroids resulted in complete resolution of the testicular mass and a significant decrease in the size of the hilar, mediastinal, and retroperitoneal lymphadenopathy.

Hairy Cell Leukemia Presenting with Duodenal Involvement Duodenum: A Case Report.

CONTEXT: A rare case of adult hairy cell leukemia (HCL) with duodenal involvement is presented. CASE REPORT: The patient was a 48-year-old man, who had a history of hairy cell leukemia. Three days after completion of 2-chlorodeoxyadenosine (CDA) chemotherapy, the patient started experiencing abdominal pain. An extensive gastroenterological workup culminated in the patient getting an esophagogastroduodenoscopy (EGD) that revealed duodenal inflammation and biopsies were taken. The duodenal biopsy was positive for chronic inflammatory infiltrate, primarily consisting of atypical lymphocytes and plasma cells with tartrate-resistant acid phosphatase (TRAP) positivity, and hence a diagnosis of duodenal involvement with HCL was made. Repeat bone marrow biopsy done 2 weeks after finishing chemotherapy revealed residual disease. At the 3-month follow-up, the patient was asymptomatic with a normocellular marrow and no residual disease. Repeat abdomen computerized tomography (CT) scan at completion of therapy showed resolution of duodenal thickening and spleen size of 12 cm. Currently, patient is in clinical remission for 6 years with 4-6 monthly follow-up visits and continues to do well. CONCLUSION: This case is presented to highlight the first case report of HCL with duodenal involvement that was successfully treated with CDA.

Hepatic vein obstruction is the most common type of hepatic venous outflow obstruction regardless of socioeconomic status.

BACKGROUND: Data regarding role of socioeconomic status (SES) as etiology and site of involvement of veins in hepatic venous outflow tract obstruction (HVOTO) is scarce and only described from Nepal. We prospectively evaluated the role of SES in patients with HVOTO. METHODS: 70 consecutive patients (41 females; mean age 29, range 3-65 years) with HVOTO were studied. Their clinical history, socioeconomic factors (income, education, and occupation), birth history, dietary factors, living standards, baseline characteristics, liver function, and clinical parameters were evaluated. RESULTS: Of the 70 patients analyzed, 48 (68.5%) had hepatic vein (HV) obstruction, 7 (10%) had isolated inferior vena cava (IVC) obstruction, and 15 (21.5%) had combined HV and IVC obstruction. Of the 10 patients belonging to the upper SES, 7 had isolated HV obstruction, and 3 had combined IVC and HV obstruction; no patient had isolated IVC obstruction. Of the 60 patients belonging to the lower SES, 41(68.4%) had HV obstruction, and 19 (31.6%) had IVC involvement with (n=12) or without (n=7) HV involvement. HV/IVC involvement did not correlate with setting of delivery (hospital vs. home), birth weight, birth complications, immunization in childhood, ventilation in house, water storage facilities, history of diarrhea, or diet. Patients with HVOTO living in a mud house had IVC obstruction more commonly than HV (6/22 vs. 4/48; P=0.04). CONCLUSION: Isolated hepatic vein obstruction is the most common site of obstruction in patients with HVOTO in India, even among those belonging to low SES. Patients with HVOTO living in a mud house have IVC obstruction more commonly. Other socioeconomic factors studied do not appear to correlate with the site of obstruction.

Autoimmune Hepatitis with Distal Renal Tubular Acidosis and Small Bowel Partial Malrotation.

Renal tubular acidosis (RTA) is not uncommon in patient with chronic autoimmune hepatitis (AIH), but usually remains latent. Here, we report a case of renal tubular acidosis RTA who presented with AIH. She was also diagnosed to have partial bowel malrotation. A 9-year-old girl, a case of distal RTA, presented with jaundice, abdominal distension and altered sensorium. She was diagnosed to be AIH, which was successfully treated with steroids and azathioprine. Coexistent midgut partial malrotation with volvulus was diagnosed during the treatment. She was treated successfully with anti-tuberculous treatment for cervical lymphadenitis. Autoimmune hepatitis should not be ruled out in each case of RTA presenting with jaundice. HOW TO CITE THIS ARTICLE: Modi TK, Parikh H, Sadalge A, Gupte A, Bhatt P, Shukla A. Autoimmune Hepatitis with Distal Renal Tubular Acidosis and Small Bowel Partial Malrotation. Euroasian J Hepato-Gastroenterol 2015;5(2):107-109.