Medicine E Log

BIMONTHLY ASSESSMENT :(6/10/20).

Link to online assessment questions .

http://medicinedepartment.blogspot.com/2020/09/medicine-paper-for-october-2020-first.html?m=1

CASE 1 :

https://swathibogari158.blogspot.com/2020/09/chronic-decompensated-liver-disease.html

This is a case admitted in my unit , so patient is a 57 yr old male with poor lifestyle habits and obesity and chronic alocholic .

It's a case of

a) Decompensated liver disease secondary to chronic ethanol ingestion ,with portal hypertension (due to portal fibrosis ) and

b) Bicytopenia due to hypersplenism secondary to portal hypertension and also reduced synthesis of thrombopoietin .

c) With hepatic encephelopathy and

D) Bilateral elephantiasis due to chronic lymphatic obstruction and bilateral cellulitis and ulcerations

1) Reason for ascitis :

Liver Cirrhosis is characterized by diffuse fibrosis of liver parenchyma resulting in structurally abnormal liver nodules .

The natural history of cirrhotic liver disease progresses from a compensated to a decompensated phase. Ascitis is the main complication of cirrhosis,3 and the mean time period to its development is approximately 10 years.

Ascites is defined as the presence of excessive fluid in the peritoneal cavity.

Portal hypertension ----splanchanic vasodilation---decreased renal blood flow ----RAAS system activation------Sodium water retention.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3860926/#:~:text=Ascites%20is%20defined%20as%20the,resulting%20in%20renal%20sodium%20retention.

2) Patient developed bipedal lymphedema due to chronic lymphatic obstruction due to lymphatic fibrosis secondary to chronic pedal edema and lichenification of skin and recurrent cellulitis causing inflammation of subcutaneous tissues and lymphatic system

Due to lymphatic blockade ,there was decreased lymphatic drainage and swelling of bilateral lower limbs .

Patients with chronic lymphatic obstruction and venous stasis are at more risk of developing recurrent cellulitis .

Cellulitis is a subcutaneous skin infection caused mostly by beta hemolytic streptococcus , whenever there is breach in skin epithelial barrier.

So as pt was having swollen legs ,there were fissures on skin , could be the reason for his recurrent cellulitis .

Also the patient is immunocompromised and has hypoalbunemia and Anemia and coagulation abnormalities which will delay wound healing .

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6452002/?report=reader

3) Asterixis and Constructional apraxia are due to hepatic encephelopathy due to accumulation of ammonia .

The only treatment for this was to remove organic waste from bowel ,by giving lactulose syrups and enema , preventing worsening of encephelopathy.

Pt was passing 3-4 episodes of stools per day.

Ammonia is a by-product of protein catabolism..which goes to liver and urea is synthesized hence ammonia and nitrogen is eliminated. (urea cycle).

Role of ammonia in hepatic encephelopathy:

Impaired blood-brain barrier, changes in neurotransmission, proinflammatory cytokines, oxidative stress, abnormalities in GABA-ergic or benzodiazepine pathways, impaired energy metabolism of the brain, and impaired cerebral blood flow .

In Liver cirrhosis, the activity of urea cycle enzymes and glutamine synthetase in the brain decreases .As the brain is not equipped with an effective urea cycle, the ammonia is removed mainly in the process of glutamine (Gln) synthesis from glutamate (Glu) and ammonia, with the participation of glutamine synthetase localized almost exclusively in astrocytes . Astrocyte swelling secondary to Gln osmotic effects consequently leads to the brain edema.

https://www.hindawi.com/journals/grp/2012/642108/

SIBO in cirrhosis patients was observed at a very high frequency, and SIBO showed a high correlation with bacterial translocation, suggesting that SIBO could be a major risk factor of bacterial translocation, especially in ascitic patients.

https://link.springer.com/article/10.1007/s10620-009-0870-9

4) Although not all treatment modalities applied for this patient had efficacy ,but we had to do something for the constant deterioration of his state and few medications like udiliv ,hepamerz would work like a placebo also for the patient.

Antibiotics were indicated for the cellulitis and ulcer part ,and also pt was having fever spikes.

Challenge was constant derangement of coagulation profile with aptt and pt greater than 1 min and inr of 3.6 inspite of giving 3-4 FFps per day

.FFP contains all coagulation factors except platelets. FFP contains fibrinogen (400 to 900 mg/unit), albumin, protein C, protein S, antithrombin, tissue factor pathway inhibitor. It is free of erythrocytes and leukocytes. FFP corrects coagulopathy by replacing or supplying plasma proteins in patients who are deficient in or have defective plasma proteins. A standard dose of 10 to 20 mL/kg (4 to 6 units in adults) will raise factor levels by approximately 20%. An increase of roughly 10% of several factors is enough to effect hemostasis.

Even hemoglobin dint increase inspite of 2 prbc transfusion due to constant GI bleed ( manifested as black stools ) and bleeding from ulcers .

Rifaximin is a nonabsorbable antibiotic, which reduces the production of ammonia by gut bacteria and, to some extent, other toxic derivatives from the gut. Clinical trials show that these effects improve episodes of hepatic encephalopathy. A large randomized trial found that rifaximin prevents recurrent episodes of hepatic encephalopathy. Most patients were treated concurrently with lactulose.

Although there is no proven efficacy for it..but giving the drug wouldn't harm the patient much.

HEPAMERZ SYRUP :

Eighty patients were randomized to two treatment groups, L-ornithine-L-aspartate (20 g/d) or placebo, both dissolved in 250 mL of 5% dextrose water and infused intravenously for four hours a day for five consecutive days with 0.5 g/kg dietary protein intake at the end of daily treatment period. Outcome variables were postprandial blood ammonia and mental state grade. Adverse reactions and mortality were also determined.

Results: Both treatment groups were comparable regarding age, gender, etiology of cirrhosis, Child-Pugh class, mental state grade and blood ammonia at baseline. Although, improvement occurred in both groups, there was a greater improvement in L-ornithine-L-aspartate group with regard to both variables. Four patients in the placebo group and 2 in L-ornithine-L-aspartate group died.

https://pubmed.ncbi.nlm.nih.gov/18983791/

-DIURETICS , LACTULOSE - these two were the main treatment required.

Although udiliv and protein powder wasn't required. High protein intake can actually worsen hepatic encephelopathy.

CASE 2:

https://sainiharika469.blogspot.com/2020/09/hello-everyone.html?m=1

1) Patient wasn't having Att induced hepatitis ,as ATT was started recently(3 days ) and also hepatitis includes elevation of ALT AND AST enzyme 2-3 fold the normal value , but in this patient the values are just on higher end . ALT which is specific for liver is not even elalevated.

Here the patient has hyperbilirubinemia mostly bcoz of chronic liver disease or Cholelithiasis or because of Budd - Chiari syndrome .

So patient can be continued on ATT , as there was no acute hepatitis after intiation of therapy or the liver enzymes were normal. But dose modifications would be necessary according to weight and would recommend starting low doses in view of impaired hepatic metabolism of drugs and BP monitoring should be done as we are using rifampicin.

2) As Patient was having sputum positive for AFB

And also with cxr and ct showing consolidation ofright middle lobe and fibrocavitatory changes and pleural thickening secondary to chronic fibrosis .

Pt even had clinical history of low grade fever and weight loss..so there is possibility of pulmonary koch.

3)Cause of ascitis :

Ascitis analysis revealed high saag (1.2 mg/dl)

Indicating portal htn and ascitic protein is low ,so possibilities are cirrhosis ,Budd Chiari syndrome .

This patient is having thrombosis in hepatic vein so he might be having ? Budd- Chiari syndrome .

BCS should be suspected in patients with: (1) Abrupt onset of ascites and painful hepatomegaly; (2) Massive ascites with relatively preserved liver functions; (3) Sinusoidal dilation in liver biopsy without heart disease; (4) Fulminant hepatic failure associated with hepatomegaly and ascites; (5) Unexplained chronic liver disease; (6) Liver disease with thrombogenic disorder.

But enzymes are not elevated and also there is no congestive hepatomegaly..so we can ruleout budd chiari syndrome.

But as patient is chronic alcoholic ..so it is alocholic liver disease and ascitis can be due to portal hypertension .

4) Efficacy of treatment :

-Diuretics and lactulose can be treatment modalities for chronic liver disease ,

If pulmonary koch is suspected ,then there would be no requirement for piptaz .

-Insulin would be a choice of treatment for controlling sugars ,as this patient is in sepsis ,sugars wouldn't be that high and glimeperide can cause recurrent hypoglycemia .

Hypoglycemia can also occur due to interaction between isoniazid and glimeperide. Isoniazid is a cytochrome inhibitor so will lead to delayed metabolism of glimeperide and hyper insulinemia.

https://pubmed.ncbi.nlm.nih.gov/23595176/

-Hyponatremia is asymptomatic ,as patient was conscious , oriented,no seizures .

Wouldn't require much evaluation and treatment.

CASE 3 :

https://sushma29.blogspot.com/2020/09/ascites-secondary-to-nephrotic-syndrome.html?m=1

46 yr old man with nephrotic syndrome :

1)Further approach would be first by finding out cause for his nephrotic syndrome ,which could be

A) Primary (idiopathic) forms:

-Minimal change disease - but seen in children so ruled out .

-Focal segmental glomerulosclerosis- seen in adults so a possibility

-Membranous nephropathy - Most commonly seen in adults ,so a possibility.( Anti PLA2R antibodies)

B) Secondary forms :

-Diabetic nephropathy- pt not a known diabetic

-Amyloid nephropathy: can be associated with multiple myeloma (AL amyloidosis) or chronic inflammatory disease such as rheumatoid arthritis (AA amyloidosis) - not seen in our pt.

-Lupus nephritis. - not a possibility

- Hiv hbsag , were negative . No features of STD .

- Malignancy - lung , adenocarcinoma of colon , stomach , prostate carcinoma ,non hodgkins lymphoma all these are secondary causes of nephrotic syndrome - which could be a possibility in this patient owing to chronic weight loss and muscle wasting and loss of appetite .

So only way to look for diagnosis is :

-First doing a renal biopsy ,

- ANA ,C3,C4 levels would tell us an autoimmune cause - but they are not sensitive and not recommended much . Biopsy would be a better option.

-Can search for primary focus of malignancy ,

By doing HRCT ,Ct abdomen , endoscopy/colonoscopy - All these are very difficult to do in a patient who has financial issues..but sometimes ,it's of benefit to the patient if a malignancy is detected at an early stage .

2) pros of getting renal biopsy would clearly tell us if it's FSGS ,MGN ,MPGN pattern.

Cons - Bleeding , infections risk..but cons are minimal if carefully done.

Although biopsy would only help to know the diagnosis , so that immunosuppressants or further management would be easier .

Could even explain the prognosis to the patients better with diagnosis..as their main concern was to relieve pedal edema and abdominal distension..which would require diuretic therapy frequently.

ARBS have been started for the patient to decrease proteinuria.

Although the histopathology diagnosis is made , patient can progress to CKD still ,if it's primary nephrotic syndrome ,but can be beneficial to certain extent by starting on immunosuppressants.

If it's secondary to malignancy then treatment of the primary would resolve his nephrotic syndrome.

Role of immunosuppressants :

6-month cyclical regimen of alternating alkylating agents (cyclophosphamide or chlorambucil) plus intravenous pulse and oral corticosteroids was effective for achieving remission in patients with mn and nephrotic syndrome [30-32]. however, cumulative risks associated with this approach include opportunistic infection, reactivation of viral hepatitis, alopecia, gonadal damage (aspermatogenesis, ovulation failure), hemorrhagic cystitis (cyclophosphamide only), neoplasia (myelodysplastic syndrome; acute myelogenous leukemia.