Personal site of Ph.D Roman G. Myazin

Primary Billiary Cirrhosis

Patient Sh, 60,  (case report 11342/396) entered the gastroenterology department of Volgograd Regional Clinical Hospital №1 on May, 20, 2001 with complaints of permanent poignant billiary pruritis, icteritiousness of dermal integuments and scleras, nausea, weakness, weight loss, periodical shooting pains in the right iliac area. The diagnosis on admission to hospital: «Primary Billiary Liver Cirrhosis, active phase, syndrome of cytolysis and cholestasis».

 From the anamnesis: the patient considers herself to be ill since 1997 when she began to suffer from weakness, malaise, a feeling of heaviness in the upper part of abdomen, skin inch. The patient denies alcohol drinking. Any occupational hazard or any intoxication caused by hepatotropic poisons in the anamnesis were not detected. Common blood analysis showed the increase of ESR up to 50 mm/h. It was detected the increase of total bilirubin level up to 80 mkmol/l due to its cojugated fraction.

Since the beginning of the illness the patient is being observed at the Department of Propedeutics of Internal Diseases of Volgograd State Medical University, undergoing the courses of inpatient treatment at the Gastroenterology Department (GED) of Volgograd Reagional Clinlcal Hospital № 1 twice a year. The patient underwent the treatment with prednisolone, hepatoprotectors, intravenous laser therapy and ozone therapy with short-term periods of clinical improvement.

Objectively: dermal integuments are dry, yellowish-brown, with scratch raws. On the skin of chest and abdomen «vascular stars» are seen. Scleras and mucous membranes are icteritious. Vesicular respiration with no rales. Arterial Blood Preasure (ABP) – 160/90 mmHg, pulse/min – 72, rhythmic. Can be heard systolic murmur at the cardiac apex. Appetite is normal. The abdomen is of usual form and configuration, soft, painless while palpated. The liver is dense, occupies the upper third part of the abdominal cavity. Its lower part is sharp and is situated 6–7 cm higher than the navel level. The spleen is dense, painless, its lower part is palpable 1-2 cm higher than the navel level. The results of fibrogastroscopy showed the absence of esophageal varicose veins dilatation. 

According to the results of the abdominal cavity ultrasound investigation: the liver tissue is of increased echogenicity, diffusely non homogeneous. The anteroposterior size of the right lobe of liver is 17,8 cm, of the left lobe of liver – 8,4, of the caudate lobe of liver – 3,8, v. portae – 1,4 cm. Biliary ducts without pathologic changes. The spleen is 19,5´8,2 cm, of usual echogenicity, diffusely non homogeneous due to microcalcification. V. lienalis – 1,4 cm.

Liver biopsy: in the punctate are visualized separate « emptys» portal tracts in which inflammatory infiltrates don’t have biliary ducts. In some portal tracts can be seen proliferating cholangioles and inflammatory infiltrates round which an excrescence of connective tissue is observed. In periportal hepatocytes can be seen orceinpositive granulomas, containing bile pigment.  Can be seen moderate inflammatory changes of liver parenchyma in the form of piecemeal necrosis of isolated hepatocytes.

Conclusion: the clinical presentation of primary billiary liver cirrhosis of the 2 stage, the phase of cholangioles proliferation and periductal fibrosis.

Common blood analysis: Er – 4,0 ´1012, Hb – 120 g/l, colour index – 0,88, reticulocytes –20 %, Tr – 205´109, Le – 3,8´109, b – 1, e – 1, band neutrophils – 2, s – 69, l – 23, m – 5. ESR – 48 mm/h. Blood clotting time – 4 min. Prothrombin index – 72%. Blood glucose – 4,0 mmol/l. Trace elements of blood plasma: Na – 141 mmol/l, K – 4,8 mmol/l. Whole protein – 76 g/l, albumins – 38 %, a1-globulins – 3 %, a2 – globulins – 10 %, b-globulins – 19 %, g-globulins – 30 %, A/G coefficient – 0,61.

Coagulogramma: plasma recalcification time – 174 sec., trombotest rate – V, prothrombin index – 68 %, plasma fibrinogen – 2 g/l, fibrinogen B – low positive.

Clinical urine analysis: yellow, transparent, reaction – acid, relative density – 1013, glucose – not detected, protein – not detected, Le – 1–4 within eyesight, Er – not detected, plano epithelium cells – a small number, cylinders –not detected.

Biochemical tests: MDA – 34,2 mkmol/l, DC – 1,8 u., Cat – 11,9 mkmol/ml/min, SOD – 0,8 conv.u/ml, GP – 1,8 mkmol/ml/min, SU – 0,7 u., SG -0,9 u., CPl – 53,9 mg%, NAG – 18,56 nmol/ml/min, SDG – 108 mkmol/l/h, TDG – 114 mkmol/l/h, total bilirubin – 71,9 mkmol/l, direct bilirubin – 53,9 mkmol/l, indirect bilirubin – 18 mkmol/l, thymol test – 10 u., ALT – 0,68 mkkat/l, AST – 0,88 mkkat/l.

For the purpose of detoxification the patient underwent a course of Natrii Hypochloriti monotherapy according to the scheme, i.e. the course of 7 intravenous drip infusions into the cubital vein with the speed of 40 drips per minute in the concentration of 200 mg/l as 200 ml injections daily. The patient underwent the procedures successfully.

Edemas and hypocoagulation weren't odserved during the treatment. After the finishing of the course of treatment all above-named investigations were made once again.

It was detected the improvement of LPO indices, liver-specific enzymes and standard liver tests. It was observed an increase of AOP enzymes: MDA – 15,4 mkmol/l, DC – 1,3 u., Cat – 14,3 mkmol/ml/min, SOD -2,0 conv. u./ml, GP – 4,0 mkmol/ml/min, SU - 0 u., SG – 0 u., CPl – 41,25 mg%, NAG – 10,33 nmol/ml/min, SDG - 58 mkmol/l/h, TDG – 60 mkmol/l/h, total bilirubin – 33,7 mkmol/l, direct bilirubin – 23,1 mkmol/l, indirect bilirubin – 10,6 mkmol/l, thymol test – 5 u., ALT – 0,26 mkkat/l, AST – 0,13 mkkat/l. It was detected the improvement of protein synthesis functions of liver with the increase of A/G coefficient up to 0,82. The patient stopped suffering from poignant billiary pruritis and aching pains in the right hypochondrium, the intensity of xanthochromia lowered, sleep improved.

The recurring investigations made 3 months after the finishing of Natrii Hypochloriti treatmant showed a small positive dynamics of clinical-laboratory data.

The investigations made 6 months after the Natrii Hypochlority treatment also showed the stable dynamics of all clinical and laboratory data.

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