27 THE ANTISEPTIC Vol. 114 • December 2017

Case RepoRt

Case report

A 45 year old female, educated upto 12 standard, resident of Hajipur, Bihar, presented with history of breathlessness on exertion for 5 years and generalized itching for 3 months.

She was well till 5 years back when she developed breathlessness on exertion, progressive from NYHA grade 2-3, associated with chest pain and syncopal attacks. She was investigated at her native place and told that she had a heart disease. She took treatment from a cardiologist till 2005. When she did not have enough relief, she started taking ayurvedic drugs which she continued till presenting at our centre. She also complained of severe, generalized itching, present throughout the day all over the body for 3 months. There was no history of jaundice, altered sensorium, upper gastrointestinal bleeding or ascites during the course of illness.

She gave history of jaundice 15 years back which was not investigated.

On examination, she had pulse of 98/min, low volume and blood pressure of 98/50 mmhg. Grade 2 clubbing and palmar erythema was noted. She had vesicular lesions

abstRaCt

A middle aged female with known heart disease presented with itching and breathlessness. She had persistently deranged liver function tests. She was investigated and a rare combination of overlap syndrome and primary pulmonary hypertension was established.

over the genitals, extremities and abdomen. Cardiovascular examination revealed parasternal heave, loud P2, 4th heart sound and pansystolic murmur in tricuspid area. Abdominal examination showed a 5 cms, tender, non pulsatile liver. Clinical diagnosis of pulmonary hypertension with congestive hepatopathy was made.

A review of her previous investigations revealed that she had persistently elevated liver enzymes, two to four fold times, since 2009. After starting the ayurvedic drugs, the blood investigations had revealed elevated serum bilirubin and serum transaminases. So a possibility of drug induced liver injury was also thought of.

Arterial blood gas analysis was normal and she had no orthodeoxia. Electrocardiogram revealed right axis deviation, right atrial enlargement and right ventricular hypertrophy. Chest roentgenogram showed a prominent pulmonary bay with right atrial and ventricular enlargement. 2-D-Echocardiography showed dilated RA, RV, with PAP of 67 mm Hg, Mild pulmonary regurgitation and severe tricuspid regurgitation, LVEF was 65%. The patient was not willing for right heart catheterisation and hence, it could not be done.

Hemoglobin was 11.4 gm%, TLC 6700 and platelet count was normal. Blood sugar level, renal parameters and thyroid profile

were within normal limits. Liver profile showed the following results- serum bilirubin 2.34 mg%, ALT 96 , AST 112 mg%, alkaline phosphatase 178 IU/ml, gamma glutamyl transferase 105 U/ml, total proteins 7.6 gm%, serum albumin 3.0 gm% and INR 1.4.

Serum ACE levels were normal and alpha fetoprotein was 4.5 ng/ml. Ultrasonography of the abdomen showed features of cirrhosis of liver with splenomegaly and absence of free fluid. Upper GI endoscopy demonstrated grade 1 esophageal varices with mild portal hypertensive gastropathy. HRCT of the chest revealed no pulmonary lesion or enlarged lymph nodes and CT pulmonary angiography was normal.

HIV 1 and 2 and viral markers were negative. Anti mitochondrial antibodies and anti smooth muscle antibodies were strongly positive. Dermatology opinion was taken for the vesicular eruptions. It was diagnosed as scabies.

Liver biopsy demonstrated hepatocytes with mild nuclear polyploidy with al tered architecture. There was hepatocyte swelling and pyknosis. Dense lymphoplasmacytic infiltration with active and interface hepatitis was observed. Nodule formation and rosettes were demonstrated. Reticulin stain showed evidence of fibrosis.

Porto Pulmonary Hypertension with Overlap SyndromeMayank Jain

Dr. Mayank Jain, M.D, D.N.B.,Consultant Gastroenterologist,Choithram Hospital and Research Centre, Indore.Gleneagles Global Health City, Chennai - 600 100

Specially Contributed to "The Antiseptic" Vol. 114 No. 12 & P : 27 - 28