Bimonthly assessment

 1) What is the reason for this patient's ascites? 

Cirrhosis of liver is the cause of ascites in this patient

 1 Chronic alcoholism since 40 years

180g of alcohol per day for 25years increases the risk of cirrhosis by 25 times 

 2. Truncal obesity 

3 B/L pedal edema

4.black stools

5.icterus

4.asterexsis

5.raised bilirubin levels

6.alterted echotexture on usg 

All these localise the pathology to liver

Cause of ascites in cirrhosis 

1.portal Hypertension:increased portal vein hydrostatic pressure causing extravasation of fluid from plasma  into peritoneal cavity

2.decreased synthetic function of liver causing hypoalbuminemia leading to decreased oncotic pressure causing extravasation of fluid

3.splanchnic vasodilation and hyperdynamic circulation :it reduces systemic arterial blood pressure, activates renin angiotensin aldosterone system with development of secondary hyperaldosteronism..Failure of liver to metabolize aldosterone intensifies secondary hyperaldosteronism. This can lead to sodium and fluid retention and expansion of extracellular fluid volume. 

4.percolation of hepatic lymph into peritoneal cavity as hepatic lymph flow exceeds thoracic duct capacity. The excess lymph oozes freely from the surface of cirrhotic liver into peritoneal cavity

2) Why did the patient develop bipedal lymphedema? What was the reason for the recurrent blebs and ulcerations and cellulitis in his lower limbs?  

1. It can be due to filariasis infection caused by filarial worms

2.lymphedema tarda:primary lymph edema in adults occurring in patients with weak immunity

 "Lymphedema tarda is a congenital disease characterized by underdevelopment of lymphatic pathways. It manifests as accumulation of lymph in the interstitial spaces of the skin. Wound healing is significantly impaired. Ulcerations which occur during the course of the disease heal slowly or not at all. The cause of this impairment is the continued lymphatic drainage as well as a regional oxygen deficiency in the diseased tissue. 

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4286225/#:~:text=Lymphedema%20tarda%20is%20a%20congenital,Wound%20healing%20is%20significantly%20impaired.

3.The ulceration, belbs can be due to lymphedema. He punctured the blebs himself and has done multiple self practising measures.Due to lymphedema the healing becomes delayed and also as no proper dressings, no aseptic conditions maintained, multiple self medications, infections of the ulcers have further delayed the healing

3) What was the reason for his asterixis and constructional apraxia and what was done by the treating team to address that?  

A. Asterixis:flapping tremors: is a motor disturbance marked by intermittent lapses of an assured posture, as a result of intermittency of sustained contraction of group of muscles. Usually manifests as a bilateral flapping tremor at wrist, MCP joint, hip joints, sometimes tongue, foot and any skeletal muscle

Mechanism:remains unknown, probably due to interruption of the posture pathway in the rostral reticular formation and abnormal joint proprioception

Causes:hepatic encephalopathy,renal failure, metabolic encephalopathy, co2 toxicity, Wilson disease, electrolyte abnormalities, drugs(phenytoin flap) 

In this patient it is due to hepatic encephalopathy (and there was also increased sleep during day times which is the earliest signs of hepatic encephalopathy and also deranged PT, Inr, Albumin pointing towards hepatic encephalopathy) 

In hepatic encephalopathy (due to cirrhosis of liver ) damage occurs to brain cells due to the impaired metabolism of ammonia is predominantly related to the development of asterixis in hepatic encephalopathy 

B. Constructional apraxia is that the patient is unable to write or draw or copy images(this is also a sign of hepatic encephalopathy) 

C. The treating team has given lactulose to wash out the Bowel contents which is the primary source of ammonia 

Rifaximin (non absorbable derivative of rifampicin so it acts in gut lumen mainly) is given:it is used as an adjunctive to sterilize the gut for intestinal ammonia producing organisms

4) What was the efficacy of each treatment intervention used for this patient? Identify the over and under diagnosis and over and under treatment issues in the management of this patient. 

1.high protein diet (2eggs / day) for decreased albumin synthesis

2.Air or water bed to prevent pressure bed sores in the dependent areas

3.Fluid restriction <1.5litres/day so as to decrease of fluid dissemination into the extra vascular space

Salt restriction <2.4gms/day to prevent retention of water due to osmotic gradient as sodium causes retention

4. Inj augmentin 1.2gm IV/BD to prevent secondary bacterial infections 

5.Syp lactulose 15ml/PO/BD for hepatic encephalopathy 

6. Tab lasilactone (20/50)mg BD ( combination of furosemide and aldactone to decrease pedal oedema
If SBP <90mmhg - to avoid excessive loss of fluid

7. Inj vit k 10mg IM/ STAT ( as vitamin K causes coagulation to further prevent bleeding manifestions

 

8. Tab udiliv 300mg/Po :this comes under the overtreatment issues in this patient. 

9.syp hepameiz 15 ml/PO/OD, it contains L ornithine L aspartate 

https://www.elsevier.es/en-revista-annals-hepatology-16-articulo-efficacy-oral-l-ornithine-l-aspartate-in-cirrhotic-S1665268119319891

This is a Mexican study on LOLA and lactulose in patients with hepatic encephalopathy 

The ultimate therapy for hepatic encephalopathy is orthotopic liver transplantation. However, this approach is not always available in many Latin American or Asian Pacific countries were liver transplant programs are undeveloped.

In conclusion, L-Ornithine-L-Aspartate administration is safe and effective in reducing hyperammonemic hepatic encephalopathy in Mexican patients with hepatic cirrhosis.

10.IVF 1 NS slowly at 30ml/hr to maintain hydration

11.Inj thiamine 100mg in 100mlNS /IV/TID as thiamine deficiency's occur in chronic alcoholics

12.protein x powder in glass of milk TID for protein supplementation and muscle wasting which commonly occurs in cirrhosis patients 

13.2FFP and 1PRBC transfusion to support coagulation pathways and anemia 

14. ASD DONE for wound infections and ulcer

Case 2

1) Why were his antitubercular therapy stopped soon after his current admission? Was he symptomatic for ATT induced hepatitis? Was the method planned for restarting antitubercular therapy after a gap of few days appropriate? What evidence is this approach supported by? 

His ATT was stopped as his SGOT levels were raised and he showed symptoms like unexplained fatigue, abdominal pain whcih point towards a Drug Induced Liver Injury. He also has risk factors like : above the age of 35, malnutrition and alcoholic. Also monitoring hepatotoxicity post ATT is difficult in patients of Chronic Liver disease.

According to American Thoracic Society,Guidelines for re-introducing ATT are:

1. After AST returns to less than two times the normal upper limit, Rifampicin can be restarted with or without ethambutol.
2. After 3-7 days, Isoniazid maybe added, subsequently after checking AST levels.
3. If symptoms recur, then the last drug added should be stopped

2) What were the investigational findings confirming the diagnosis of pulmonary TB in this man? 

Findings pointing towards pulmonary TB in this patient are:

• Fever which spikes at night 
• Generalized weakness
• Sputum was found positive.
• Increased Neutrophil count points to an Active Pulmonary tuberculosis.
• Thromocytopenia
• Hazy opacities seen on Chest X ray.
• CT shows fibrocavitary lesion in right lung.
• Patient looks emaciated and has anemia which signify the severity of tuberculosis.

3) What was the cause of his ascites?

• ALCOHOLIC CIRRHOSIS OF LIVER >> PORTAL HYPERTENSION
• LATE BUDD-CHIARI SYNDROME
• HYPOALBUMINEMIA

4) What are the efficacy of each intervention mentioned in his treatment plan and identify the over and under diagnosis as well as over and under treatment issues in it. 

A. High protein diet 4eggs daily for protein supplementation 

B. ORS sachets in 1 litre of water to compensate electrolytes lost due to diarrhoea 

C. Inj PIPTAZ 4.5gm for antibiotic cover

D. Vit k 10 mg Iv OD for 5 days to prevent forthcoming bleeding manifestations as his PT INR APTT are elevated 

E. IVF - 1 DNS @50ml/hr for hydration

F. Nebulisation with salbutamol and mucomist 12th hourly for cough and crepts

G. Inj thiamine 100 mg in 100 ml NS IV TID. for chronic alcoholism.

Case 3

1) What will be your further approach toward managing this patient of nephrotic syndrome? How will you establish the cause for his nephrotic syndrome?

The patient has albuminuria, hypoalbuminemia, hyperlipidemia making the diagnosis as nephrotic syndrome 

A.Diagnostic uncertainty :We should know the cause of his nephrotic syndrome and treat the primary cause

B. Anti neutrophil antibody can be done to rule out autoimmune disease like sjogrens syndrome, SLE

C. Should rule out malignancies like carcinomas of lung, colon, lymphomas can also present with nephrotic syndrome (supported by his history of weight loss) 

D. It also could be primary idiopathic FSGS

E.Furthwr approach/ Treatment 

Primary cause has to be treated

Ace inhibitors and ARBs should have been used as they reduce proteinuria and slower the rate of progression of renal failure

Prolonged bed rest should have been avoided as it increases the predisposition to venous thrombosis and thrombo embolism(loss of antithrombin in urine increases risk of thrombosis in nephrotic syndrome) 

Sepsis is major cause of death in nephrotic syndrome. Increased susceptibility to pneumococcal infections (loss of immunoglobulin in urine), pneumococcal vaccine should have been given, early detection and aggressive treatment of infections should be done

Lasilactone and lasix have been used and paracentesis has been done to decrease ascites and pedal edema

Prednisolone could have been started even if renal biopsy is not available because if it is Minimal change disease and FSGS anyhow patient needs to be started on steroids, Just in case of membranous glomerulonephritis steroids are of no much use, but can be tried and according to the response may be we can later change the treatment plan

2) What are the pros and cons of getting a renal biopsy for him? Will it really meet his actual requirements that can put him on the road to recovery?

Pros

1.we can know the type of nephrotic syndrome

2.can decide the treatment plan as I have mentioned above MCD good response to steroids(95%), FSGS (20-30% patients respond), if membranous glomerulopathy the response to steroids is less and other drugs like cyclophosphamide, cyclosporine, chlorambucil can be used in combination with steroids

3.prognosis of the patient can be known by renal biopsy

MCD excellent prognosis, although it may show remission and relapses

FSGS 50% progress to end stage renal failure. 

Membranous glomerulopathy :spontaneous remission may occur in 40%,3 to 40% may develop remissions and relapses, 10 to 20% may develop progressive renal failure 

Referance:textbook of medicine by archith baloor and ramdas nayak

The authors have given a lot of information in an organised manner which made it easy to study and understand

Cons

• Invasive procedure.
• Expensive.
• For most of the etiologies of nephortic syndrome in adults, there is no cure and only supportive treatment so it might not impact the treatment.