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A challenging situation - cashmir
#1
Please, have some patience and read through this TRUE story!

SYMPTOMS AND MEDICAL HISTORY

I™ve been quite ailing since January Even before then; I™d some kind of chronic fatigue which I always manage to overcome. And I have been HBsAg positive since my medical school days. Since then, I have always had some kind of a vague right hypochondriac pain over the years. I have had five liver biopsies before. All of which showed normal parenchyma: Three in London, (two of then by the Famous Prof. X) the last was in 1991 in Paris. All along, my liver function test including AFT have remained within normal limits.

Since March 2006, I™ve been quite sick with fatigue, loss of appetite and loss of weight, and discomfort on the right hypochondriac area. I now weight 74kg for a height of 1.74m. I used to weigh 84kg. I can™t specify the period of the loss of 10kg. For the last six weeks, I™ve actually been bed ridden with pain in the epigastrium and in the chest immediately to the right lateral to the epigastrium. I™m nauseated, I often belch. I cannot stand the slightest increase in my Intra-abdorminal pressure. The pain will then become piercing, especially under the ribs and abdomen immediately right lateral to the epigastrium and then, there is a referred sharp pain to my right shoulder, probably resulting from pressure/irritation on the diaphragm. All this get worse with laughing, coughing sneezing belching and yawning, and I yawn very frequently. I usually have to put pressure over the area to reduce pain in any of these events and have stopped drinking cold water to avoid sneezing.

An attempt to do gastroscopy failed. I™m so exhausted and lethargenic that of late, I lie down 16 “ 20 hours daily. I only manage to go to the office once or twice weekly when I™m under pressure.

I wish to add a little more information about me as a person, so that my complains can be seen within the context of a total person. I™ve been hypertensive for over fifteen years and I™m well controlled with 20mg of Nifedipine daily. My routine renal function investiations are within normal limits and I have few cysts on my kidney echo™s bilateral and another cyst in the liver. I think focus should be on the hepatic lesion which could be invasive and potentially lethal.

I suddenly went blind in mid July 1991 at the end of a busy OP-day in the hospital maternity. I was later to be diagnosed as: Bilateral optic nerve atrophy of ? Toxic origin! The toxin was never identified, neither through my personal medical history nor through investigations carried out in Baraqué (Baselona “ Spain).

I received a rather prolonged course of Cortico therapy for the optic nerve atrophy. By 1997 it was evident on X “ ray and CT “ Scan that I had developed bilateral Ischemic necrosis of the femural and humeral heads. In 1999 and 2000 I was performed a total hip replacement surgery (Bilateral hip prosthesis) at the hospital X in Germany (Prof. X). I walk with crutches nevertheless.

All the above gives a rather gloomy scenario. Please let me end on a more light hearted note. I™m 58 years of age, and a just retired Gynaecologist/Obstetrician from a big hospital. My father died at the age of 98 “ 102, estimated from contemporary history. I headed the High Risk pregnancy unit of our maternity for X years with only one maternal mortality. X hospital maternity has 350-450 deliveries per month and the overall maternal mortality including referred cases is in the excess of 600 per 100 thousands life birth!*

I™m a father of three kids.
In my labs, CBC is normal, only Alpha feto protein and creatinine are elevated. Serology also shows hepatitis C antibody positive.

CT/SONO: Pancrease, and spleen normal. Liver show a hypodense cyst with poor differentiable boarder, presenting heterogenic contrast uptake. Generally, normal liver parenchyma, no ascitis
Stomach shows infiltrations in the antrum area.
Kidneys: normal, otherwise, small bilateral cysts.


1. What is the most likely diagnosis?
2. What other diagnostic work up could be contributory towards correct diagnosis?
3. What are the therapeutic options for 1?
4. What medication could be added to the present regiment?


In this respect, I do sincerely hope that with your expertise, you contribute greatly towards helping me. Thanks.


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