Bimonthly test oct 7

 Bimonthly test 

Q1) 57 year old man with jaundice, pedal edema and abdominal distension since three years and bleeding gums since three days"

1. Chronic liver disease causing hypoalbuminemia and or portal hypertension leading to ascitis. 

Cirrhotic ascitic fluid accumulation results from a number of factors broadly defined in terms of hormonal and cytokine dysregulation and related volume overload in the setting of portal hypertension[1].

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3664283/

A. Bipedal edema may be due to hypoalbuminemia leading to decreased albumin in turn decreases plasma oncotic pressure causing lymphedema.

B. Cellulitis in liver cirrhosis is caused by numerous factors. Increased bacterial translocation from the gut due to altered intestinal immunity and bacterial overgrowth in cirrhotics produces Gram-negative bacteraemia, giving an opportunity for distant invasion by these microorganisms [3]. This is substantiated by the culture reports in these patients [6-8]. Moreover, low albumin levels are also a risk factor for skin infection [6, 11]. With advanced liver disease, poor liver function results in increased susceptibility to infection due to defective bactericidal function of immunoglobulin, decreased polymorphonuclear leukocyte activity, complement deficiency, and a reduced number of Kupffer cells [12]. Chronic oedema, congestion and barefoot walking are other risk factors for cellulitis in cirrhotics [6, 10].

Cellulitis in liver cirrhosis – a series of 25 cases from southern India

The pathophysiological basis of pruritus in liver diseases is incompletely understood. Historically, the accumulation of bile salts, bile acids, and bilirubin has been considered to be responsible for cholestatic pruritus. 

Hemorrhagic bullae in cirrhotic patients usually imply a fatal infection and Gram-negative bacteria are the most common pathogen.

Hemorrhagic bullae represent an ominous sign for cirrhotic patients - PubMed

1. hyperammonemia is the main factor responsible for the brain abnormalities in HE. Ammonia influences also other mechanisms leading to development of hepatic encephalopathy such as: 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 leading to asterixis and constructional apraxia (features of hepatic encephalopathy)

Treatment 

Treatment of an hepatic encephalopathy episode includes corrective measures, nutritional intervention, and pharmacological therapy. Moreover, it is necessary to maintain adequate nutrition, with an energy intake of 35–40 kcal/kg/day and protein intake of 1.2–1.5 g/kg/day

The current standard of care for patients with hepatic encephalopathy includes lactulose and rifaximin, which is associated with improvement in mental status;

It acts locally in the gastrointestinal tract, with systemic adverse effects that are similar to placebo.

Rifaximin in the treatment of hepatic encephalopathy

Treatment given is Syp lactulose & Rifaxamine & ursodeoxycholic acid

Lactulose is effective for primary & secondary prevention of overt hepatic encephalopathy in patients with cirrhosis.

Primary prophylaxis of overt hepatic encephalopathy in patients with cirrhosis: an open labeled randomized controlled trial of lactulose versus no lactulose - PubMed

Secondary prophylaxis of hepatic encephalopathy in cirrhosis: an open-label, randomized controlled trial of lactulose, probiotics, and no therapy - PubMed

1. Inj. Augmentin ?  Inj. Piptaz..?Rifaxamine,lactulose, for HE, vit k to prevent bleeding manifestation, thiamine to prevent beriberi  nutritional management. Udivil for hepatic protection.

       

1. Q2)  A 54 year old male with cough,abdominal tightness,pedal edema and diarrhea.

a. May be ATT induced hepatotoxicity as collaborated by raised hepatic enzyme levels, ?Pt was symptomatic,restarting ATT is appropriate, 

There are no guidelines for ATT  in chronic liver disease and cirrhosis .

b. Infiltrates in CXR

      Plural thickening and fibrocavities on HRCT

        sputum + TB with Rifampcin sensitivity 

C. Hypoalbuminemia, alcoholic cirrhosis,portal hypertension 

1. Q3)   47 year old man with bipedal edema since one year and abdominal distension since one month

I will Classify his nephrotic syndrome into primary and secondary

Renal pathology in primary nephrotic syndrome

▪ Minimal change disease

▪ Focal segmental glomerulosclerosis

▪ Membranous nephropathy

▪ Membranoproliferative glomerulonephritis: C3 glomerulonephritis, dense deposit disease, and "idiopathic" immune complex-mediated membranoproliferative glomerulonephritis*

Secondary causes

Infections: HIV, macrophage activation syndrome, polyomavirus, cytomegalovirus

Drugs: Interferon, pamidronate, NSAIDs, lithium, vaccines, venoms, stings

Malignancies: Thymoma, Hodgkin lymphoma (formerly called Hodgkin disease)

Genetic disorders of podocyte proteins (eg, nephrin, podocin, Alport syndrome, etc.)

Other: Sickle cell, myelodysplastic syndromes, hepatitis C, von Gierke disease, Fabry disease, dysautonomia, obesit

Detailed history , basic investigations and Renal biopsy can establish diagnosis.

1. Patients with nephrotic syndrome and a bland sediment are likely to have lupus membranous nephropathy but may also have evidence on biopsy of a proliferative glomerulonephritis or a podocytopathy.

Protein excretion greater than 500 mg/day. Patients with an elevated serum creatinine

potential complications of renal biopsy that may or may not be related to bleeding include:

Pain lasting more than 12 hours in 4 percent; this problem may be due to ureteral obstruction from a blood clot in patients with gross hematuria or to stretching of the renal capsule by a subcapsular hematoma.

Arteriovenous fistulas form in up to 14 percent of cases due to damage to the walls of an adjacent artery and vein. Post-biopsy fistulas are usually clinically silent and resolve spontaneously over one to two years. Symptomatic fistulas, causing hematuria, hypotension, or high-output heart failure, are now rare. The diagnosis can be established by color Doppler ultrasonography or arteriography. Either transcatheter arterial embolization or surgical ligation can be used to close a symptomatic fistula 

Another rare complication is chronic hypertension due to the "Page kidney" .In this setting, pressure-induced ischemia from a large subcapsular hematoma can lead to hypertension due to persistent activation of the renin-angiotensin system.

Perirenal soft tissue infection may occur in 0.2 percent of cases, most often in patients with active parenchymal renal infection .

Rarely, puncture of the liver, pancreas, or spleen may occur, as well as urinoma formation from puncture of the urinary tract .

There wont be any significant improvement that lead him to normal life.