Case of the Week Tuesday 19/9/2006

Prepared by Porf. Dr. Nabil El-Kady Unit.

Presented by Dr. Alaa Aboud.

Cholestasis.

Click here to download a power point presentation for the case.

STAFF ROUND PRESENTATION

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Female patient, 43 years old, married with 3 offsprings, the youngest is 10 years old.

She was born and still living in Cairo .

She is a housewife.

There is no history of contact with canal water or receiving antischistosomal treatment.

No special habits of medical importance.

Menstrual history:

Menarche at 13 years.

LMP 5 years ago .

She is on contraceptive injections for 5 years.

Complaint

Yellowish discoloration of the sclera 4 months ago.

Three weeks later, the jaundice became worse and the patient was admitted again to El Abassia fever hospital for two weeks with no improvement and the patient then was referred to Ain shamas university where abdominal ultrasonography were done and the patient was told to have liver cirrhosis.

A biopsy from left axillary L.N was taken and the pathology shows non specific inflammation.

The condition started four months ago by jaundice of gradual onset and progressive course, preceded by pruritus, with dark colored urine and normal colored stools, but no fever or rigors. The condition was associated with bleeding tendency in the form of bleeding gums.

The patient was admitted to Embaba fever hospital for 2 weeks and received medications of unknown nature, upon which the patient partially improved.

- No history of abdominal distension, lower limb edema , haematemesis or melena.

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Past history

History of three cesarean sections, last one 10 years ago.

History of Paraumbilical hernia operation seven years ago.

History of blood transfusion during 1 st cesarean section.

History of drug intake during her illness in the form of antibiotics and other drugs not known by the patient.

Family history

Negative consanguinity.

No similar condition in the family.

EXAMINATION

- At the time of admission:

The patient was confused and not well-oriented to time place and persons, lying comfortably in bed

-Pulse: 100/min regular.

-Temp: 37 °C.

-B.P: 60/40 mmHg.

She was admitted to the ICU and resuscitated and after 3 days:

-The patient regained her consciousness.

-Pulse: 80/min

-Temp: the patient was afebrile all through the hospital stay.

-B.P: 110/70 mmHg

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General Examination

Head and neck:

Eyes: Jaundice.

Tongue: No cyanosis, no tremors.

No thyroid swelling.

No palpable lymph nodes.

No xanthomata or xanthelasmas

Upper limbs:

Ecchymotic patches at sites of injection.

No palmar erythema, spider naevi or clubbing.

Lower limbs:

Bilateral pitting oedema up to the middle of the leg.

Heart, chest and neurological examination:

Free

Abdominal examination

Inspection:

Wide subcostal angle, divaricated recti, striae gravidarum.

Transverse lower abdominal scar, about 20 cms, healed by secondary intention.

The umbilicus was surgically removed during herniorraphy.

- No visible or dilated veins.

Palpation:

- Liver: Upper border: dectected in the 5th space MCL.

Lower border: not felt.

- Spleen: not palpable, with dull Traube's area.

Percussion: no ascites detected clinically by shifting dullness.

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DIFFERENTIAL DIAGNOSIS

Intrahepatic causes:-

Common

Viral hepatitis

Drugs

Alcoholic hepatitis ± cirrhosis

Less common

Primary biliary cirrhosis

Chronic hepatitis ± cirrhosis

Metastatic carcinoma

Sepsis

DIFFERENTIAL DIAGNOSIS

Intrahepatic causes:-

Common

Viral hepatitis

Drugs

Alcoholic hepatitis ± cirrhosis

Less common

Primary biliary cirrhosis

Chronic hepatitis ± cirrhosis

Metastatic carcinoma

Sepsis

Extrahepatic causes:-

Common

Common bile duct stone(s)

Pancreatic/periampullary cancer

Less common

Benign biliary stricture

Sclerosing cholangitis

Bile duct carcinoma

Benign pancreatic disease

Extrinsic duct compression

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INVESTIGATIONS

1- Urinalysis: Free except Bile pignents +++

2- CBC with differential:-

RBC : 2.41 millions/ litter

HGB: 8.9 g/dl

HCT: 25.6 %

MCV: 102.1 fl

MCH: 36.7 pg

MCHC: 35.9 g/dl

Platelets: 53x103/ul

WBCs count 14x103/ul

-Basophils 0 %

-Eosinophils 1 %

- staff 4 %

- segemented 62 %

-Lymphocytes 22 %

-Monocytes 11 %

Corrected ESR 1 st hour 48 mmHg/hour

(N.Female5-7mm/hour )

Comment

-Moderate Macrocytic anemia .

-Mild PMN Leucocytosis.

-Mild monocytosis.

Liver biochemical profile

-Total bilirubin: 34.50 mg/dl ( 0.1-1.O )

-Direct bilirubin: 19.28 mg/dl ( 0.0-0.3 )

-AST: 391 u/l ( 0-37 )

-ALT: 151 u/l (0-41 )

-ALP: 179 u/l (35-104 )

-GGT: 87 u/l (8-61)

- Total proteins: 7.8 g/dl (6.6-8.7 )

- Albumin: 2.8 g/dl ( 3.4-5.2 )

- Globulins: 5 g/dl (3.2-3.5 )

- A/G Ratio: 0.6 (1.1-2.5 )

Serum Protein Electrophoresis

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Conclusion:

Hypoalbuminemia.

Decreased alpha2 & beta globulins.

Polyclonal hypergammaglobulinemia & inverted A/G Ratio.

- PT: 14.0sec

- PC: 77%(75-120%)

- INR: 1.18

Renal Functions:

Normal electrolytes

Serum sodium: 138 mmol/l (132-145)

Serum potassium: 3.8 mmol/l(3.6-5.5)

Kidney function

Urea: 21mg/dl (7-50)

Creatinine: 0.52 mg/dl(o.3-1.2)

Blood sugar

Fasting glucose: 192 mg/dl (60-110)

2 hours pp glucose: 187 mg/dl (70-140)

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Abdominal Ultrasonography

Liver : mildly enlarged in size, coarse echopattern, wavy surface and mildly attenuated hepatic veins. No focal lesions or IHBR dilatation. Portal vein is not dilated (12.5mm).

Gall bladder : contracted. CBD is not dilated (6mm) and it could be traced distally as far as the pancreatic head.

Spleen: mildly enlarged in size (14.2cm), homogenous echopattern.

Kidneys : both are of average size, but with increased parenchymal echogenicity (Grade I), no typical calculi or back pressure changes.

Pancreas and midline structures : Free.

Others : no ascites.

Few lymph nodes are noted in the upper abdomen in the peri-pancreatic region, most are oval in shape and some show central echogenic line, most measure 2cm in average and one is larger measuring 3cm.

Conclusion:

Liver Cirrhosis.

Mild splenomegaly.

No evidence of biliary tree obstruction or pancreatic masses.

Abdominal lymphadenopathy.

Bilateral mild renal parenchymal affection.

Hepatitis markers

HCV antibody: non reactive

HB s Ag: negative.

HB s Ab : negative.

HB c IgM: negative.

HB c Total: negative.

HAV IgM : negative.

Autoimmune profile:

ANA: positive speckled (titer 1/20)

ASMA: negative

AMA: negative

Anti LKM-1: negative

APA: negative

CMV IgM: negative (0.309)

CMV IgG: positive (above 250)

EBV IgG: 11 U/ml(Neg Up to 5)

EBV IgM: 0 U/ml(Neg UP to 10)

Anti - HIV I&II antibodies: negative.

Metabolic Screening:

Alpha one antitrypsin: 1.73 g/l

(0.9-2 g/l)

Ceruloplasmin: 0.292 g/l

(0.2-0.6 g/l)

Iron: 143 ug/dl

(37-145ug/dl)

TIBC: 290ug/dl

(274-385)

Transferrin: 200mg/dl

(160-370)

Blood culture: repeatedly negative.

Urine culture for Leptospira: negative.

Upper Endoscopy

Oesophagus: No masses,ulcers or varices.

Stomach: Mucosa of fundus is hyperaemic and edematous.The mucosa of body and the antrum is free.