Summative Assessment
QUESTION 1
60 y/o male with ascites, hypoproteinemia & decreased urine output
https://ashakiran923.blogspot.com/2021/03/60-years-old-male-fever-under-evaluation.html?m=1
a) What is the problem representation of this patient and what is the anatomical localization for his current problem based on the clinical findings? How specific is his dilated superficial abdominal vein in making diagnosis?
Problem representation
Fever since 15 days, high grade, intermittent, not a/w chills & rigors, relieved on medication.
Shortness of breath since 3 days, initially on exertion(Grade II) and gradually progressed to SOB at rest(NYHA Grade IV).
H/o burning micturition +
H/o intermittent facial puffiness +
H/o aphthous ulcers a/w difficulty in swallowing.
Anatomical Localization
Liver - Given his history of chronic alcoholism for 25 years, he is an at-risk candidate for developing portal hypertension secondary to liver cirrhosis, which can present with prominent superficial abdominal veins and ascites.
Kidney - Intermittent facial puffiness, bilateral pedal edema and shortness of breath.
LUTS - burning micturition a/w fever.
Clinical Diagnosis is UTI with cirrhosis of liver causing portal hypertension.
Specificity of dilated superficial Abdominal vein in making diagnosis
"Almost any vein in the abdomen may serve as a potential collateral channel to the systemic circulation. Presence of abnormal collateral vessels appears to be one of the most sensitive (70–83%) and specific signs for the diagnosis of portal hypertension. When blood flow through a vessel or a vascular bed is obstructed due to occlusion, as in EHPVO, or distortion, as in liver cirrhosis, collateral pathways open up as blood bypasses the occlusion or obstruction, always flowing down a pressure gradient from a high pressure to a low-pressure vessel or bed. "
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3940321/
In patients with dilated abdominal wall veins due to cirrhosis, the direction of blood flow is away from the umbilicus, radiating like a star from the umbilicus, whereas in vena caval obstruction, the direction of blood flow is either completely above downward in SVC obstruction or completely below upward IVC obstruction.
b) What is the etiology of the current problem and how would you as a member of the treating team arrive at a diagnosis? What is the cause of his hypoalbuminemia?Why is the SAAG low?
Etiology
Chronic alcoholism being the culprit causing liver cirrhosis, Patients with advanced cirrhosis almost always have hypoalbuminemia caused both by decreased synthesis by the hepatocytes and water and sodium retention that dilutes the content of albumin in the extracellular space which could be causing the pedal edema.
Intermittent facial puffiness, bilateral pedal edema and shortness of breath due to renal damage.
c)Will PT,INR derangement preceed hypoalbuminemia in liver dysfunction??Share reference articles if any.
Yes. PT, INR derangement preceeds hypoalbuminemia.
Significant and severe chronic hepatic impairment is required before a noticeable decrease in plasma albumin is manifest . Hypoalbuminemia is a feature of chronic and advanced hepatic cirrhosis. Most commonly, inadequate synthesis of albumin in the presence of increased catabolism due to significant systemic illness contributes to an overall hypoalbuminemia. Albumin production may be inhibited by pro-inflammatory mediators such as IL-6, IL-1 & TNF.
PT, INR
Generally, Compensated and decompensated cirrhotic, non-septic patients live in either a balanced homeostatic state or, due to the systemic inflammation associated with liver dysfunction, a prothrombotic state.
Clinically this phenomenon is often demonstrated by the prevalence of portal vein thromboses and increased frequency of catheter clotting events during renal replacement therapy. More specifically, serum levels of antithrombin, protein C, and protein S range from 30–65% of normal; this is comparable to levels observed in patients with inherited deficiencies. In addition to decreased production of pro- and anticoagulant factors, cirrhotic patients often live in a chronic consumptive state that further decreases these already-low levels of factors on both sides of the clotting spectrum.
In summary the risk of thrombotic events thus may exceed the risk of hemorrhage, and prophylactic anticoagulant therapy – currently regarded as contraindicated in liver disease – may actually provide therapeutic benefit.
https://www.medscape.com/answers/177354-36077/what-is-the-role-of-prothrombin-time-pt-in-the-evaluation-of-acute-liver-failure
d)What is the etiology of his fever and pancytopenia?
e)Can there be conditions with severe hypoalbuminemia but no pedal edema? Can one have hereditary analbuminemia and yet have minimal edema?
Yes. Some scenarios where hypoalbuminemia persists without pedal edema are listed here.
Yes, one can have hereditary analbuminemia and yet have minimal edema.
https://www.frontiersin.org/articles/10.3389/fgene.2019.00336/full and answer the question.
By contrast, CAA is better tolerated in adulthood. Clinically, in addition to the low level of albumin, the patients almost always have hyperlipidemia, but they usually also have mild oedema, reduced blood pressure and fatigue. The fairly mild symptoms in adulthood are due to compensatory increment of other plasma proteins. The condition is rare; clinically, only about 90 cases have been detected worldwide. Among these, 53 have been studied by sequence analysis of the ALB gene, allowing the identification of 27 different loss of function (LoF) pathogenic variants. These include a variant in the start codon, frame-shift/insertions, frame-shift/deletions, nonsense variants, and variants affecting splicing. Most are unique, peculiar for each affected family, but one, a frame-shift deletion called Kayseri, has been found to cause about one third of the known cases allowing to presume a founder effect.
f) What is the efficacy of each of the drugs listed in his current treatment plan?
Nitrofurantoin efficacy
https://academic.oup.com/jac/article/70/9/2456/721364
Long-Term Efficacy and Safety of Tamsulosin for Benign Prostatic Hyperplasia
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1477608/
1.α1-Adrenergic receptor antagonists are used by 80% of physicians as the first agent to treat patients with benign prostatic hyperplasia (BPH) presenting with lower urinary tract symptoms (LUTS); 27 of 30 clinical trials have confirmed that α-blockers are effective for BPH treatment.
2.Tamsulosin's α1A subtype adrenergic receptor selectivity is considered to be responsible for its low cardiovascular side effects and lack of interaction with antihypertensives.
3.A 4-year extension, multicenter, open-label, phase IIIB clinical study evaluated long-term efficacy, safety, and tolerability of tamsulosin for up to 6 years; the study found a consistent statistically significant improvement in AUA symptom scores over 6 years, and most patients showed improvement during the first year that was sustained over 6 years.
4.The low incidence of acute urinary retention in patients treated with tamsulosin for up to 6 years suggests that tamsulosin may reduce the risk of AUR in patients with BPH.
5.Response to treatment and incidence of adverse events (eg, rhinitis and abnormal ejaculation) in patients with hypertension, diabetes, or nonhypertensive cardiovascular disease did not differ significantly from that in those without.
6.Abnormal ejaculation is an important side effect of tamsulosin, but it resulted in few discontinuations during treatment. It may not always be deemed important by patients, and was not linked to complaints of decreased libido, impotence, or other changes in sexual function.
QUESTION 2
45year old female with abdominal distension
https://navyamallempalli.blogspot.com/2021/02/dr_6.html
a). What is the problem representation of this patient and what is the anatomical localization for her current problem based on the clinical findings?
PROBLEM REPRESENTATION:
1.Abdominal distension since 2years
2.shortness of breath
3.pedal edema since 2 months
4.cachexia_malnourishment
Anatomical localisation:
1.abdominal distension : causes: fluid,fetus,flatus,fat,feaces
Here in this case:
O/E: flankfullness and fluid thrill is present __indicates distension is because of fluid_ASCITIS
In this case the pattern is ascitis followed by pedal edema _clinically it indicates problem is in liver.
2.shortness of breath and dull note in right side _IAA,ISA,IMA and decreased breath sounds on right side indicates pleural effusion .most likely in this case pleural effusion is due to her refractory ascitis _HEPATIC HYDROTHORAX.
3.Cachexia _ is due to her loss of appetite which in turn because of massive ascitis .
Overall the major problem in this case refractory ascitis since 2 years.
b) What is the etiology of her refractory ascites and pleural effusion? and how would you as a member of the treating team arrive at a diagnosis?
Refractory ascites is defined as ascites that does not recede or that recurs shortly after therapeutic paracentesis, despite sodium restriction and diuretic treatment. To date, there is no approved medical therapy specifically for refractory ascitis.
The diagnostic criteria of refractory ascites consist of ascites that cannot be mobilized with early recurrence within 4 weeks of abdominal paracentesis and lack of response to maximal doses of diuretic (spironolactone 400 mg/d and furosemide 160 mg/d) for at least 1 week.
In this case ,ascitis recurs shortly after therapuetic paracentesis despite sodium restriction and diuretic treatment._refractory ascitis.
CAUSES OF REFRACTORY ASCITIS:
1.Non compliance to treatment
2.Tumor_hepatoma
3.Renal failure
4.spotaneous bacterial peritonitis
5.Portal vein thrombosis
6.NSAIDS
7.Infection
8.GI Bledding
On ascitic fluid analysis: It is high SAAG and low protein ,it probably due to portal hypertension.
she is on diuretics,frequent therapeutic paracentesis
Her RFT was normal _no renal failure,
Noincreased cell counts in ascitic fluid, no features of infection _rule out SBP
No H/O 7se of NSAIDS
On CECT abdomen_ no tumors identified in liver
No symptoms of GI bleeding:melena,hemetemesis.
So in this case most probably ascitis due to portal hypertension :
DD: cirrhosis of liver
Schistosomiasis
Portal vein outflow obstruction.
Cirrhosis is ruled out clinically no symptoms like jaundice,no coagulation disorders,no other signs of liver cell failure radiologically no altered echotexture,no shrunken liver,no nodules .
Stool microscopy was done to rule out schistosomiasis.
Portal vein thrombosis evaluation done by triple phase cect followed byMR Venogram.
On triple phase CECT :Focal short segment of intrahepatic segment of IVC just below the diaphragm with normal hepaticveins And multiple osteosclerotic lesions in pelvis,ribs,vertebral bodies __sarcoidosis /metastasis.
MR Vengram_ suggested severe narrowing of intrahepatic portion of IVC___Chronic buud chiari syndrome.
PLEURAL EFFUSION:
When pleural and Ascitic tap done on same time sent for analysis _both looks like almost same _both are transudates:so, pleural effusion is most likely due to Hepatic hydrothorax.
DD: 1.sarcoidosis:
2.tuberculosis: pleural fluid and sputum for AFB and CBNAAT was negative.clinically no h/o TB.
3.Malignancy: pleural fliuid cytology was normal ,no dysplastic cells
c) Approach to a patient with ascites?Clinically is there any way to differentiate pre hepatic, post hepatic and hepatic causes?
d)Causes of budd chiari syndrome?Why did the patient undergo bone biopsy?
Hypercoagulable states:
Inherited : Anti thrombin deficiency, Protein c deficiency,protein S deficiency,Factor v leiden mutation, prothrombin mutation
Acquired :Myeloproliferative disorders,PNH,Anti phospholipid syndrome,cancer,pregnancy,ocp use
Uncommon causes: HCC,RCC,Adrenl carcinoma
Misc: Aspergillosis,behcet syndrome,IVC webs,trauma,IBD,systemic sarcoidosis.
IN THIS CASE BONE BIOPSY DONE FROM POSTERIOR ILIAC CREST ON LEFT SIDE TO EVALUATE THE OSTEOSCLEROTIC LESIONS IN PELVIS , RIBS, VERTEBRAE
Reason for biopsy
Primary Budd-Chiari syndrome is characterized by a blocked hepatic venous outflow tract at various levels from small hepatic veins to inferior vena cava, resulting from thrombosis or its fibrous sequellae. This rare disease affects mainly young adults. Multiple risk factors have been identified and are often combined in the same patient. Myeloproliferative diseases of atypical presentation account for nearly 50% of patients; their diagnosis can be made by showing the V617F mutation in Janus tyrosine kinase-2 gene of peripheral blood granulocytes and, should this mutation be absent, by showing clusters of dystrophic megacaryocytes at bone marrow biopsy.
d) Management strategies for refractory ascites and Budd chiari syndrome? Share the potential advantages and disadvantages of Peritoneal dialysis catheter placement in refractory ascites?
Management of BCS:
1.Diagnosis of BCS made by clinical features, laboratory and radiological investigations , management of underlying cause
2.Anti coagulant therapy with LMWH and then warfarin aim for INR 2_3
3.Treat the complications of portal hypertension with spirinolactone ,furosemide,and gastroscopy for variceal screening and band ligation
4.consider angioplasty /stenting for venous obstruction
5.consider TIPS if no improvement with anticoagulation , angioplasty, stenting.
6.TIPS fails /no improvement and acute liver failure consider liver transplantation.
7.Monitor chronic BCS for HCC by 6 monthly USG and alpha fetoprotein.
Treatment of Budd-Chiari syndrome is most successful if the disorder is diagnosed early. There are also various methods of treatment. The use of high doses of the corticosteroid drug, prednisone, may also be administered. Drugs that hinder blood clotting (anticoagulants) such as heparin may be beneficial for treatment of individuals with Budd-Chiari syndrome.
Surgical widening (dilation) of affected veins (angioplasty) may ease high pressure in the vessel walls. In some cases, Budd-Chiari syndrome may be treated surgically by diverting blood flow from one vein to another (shunting). In other cases, a blocked vein may be cleared out and then a slender rod (stent) may be inserted into the vein to maintain blood flow. In serious cases of Budd-Chiari syndrome, liver transplantation may be necessary.
Management of Refractory Ascites
e) What is the efficacy of each of the drugs listed in his current treatment plan
Lasilactone
https://www.medicines.org.uk/emc/product/907/smpc
Warfarin and LMWH:
https://emedicine.medscape.com/article/184430-medication
f)What is the current outcome?and what could be the etiology of her current outcome?
Patient expired on14th of march preceded h/o vomiting and nausea
Cause of death was not confirmed, may be because of dislodgement of thrombus leading to pulmonary embolism.
QUESTION 3
55year old male with SOB and abdominal distension,orthopnea
https://jayanth1802.blogspot.com/2021/02/55-year-old-farmer-with-sob-abdominal.html?m=1
a). What is the problem representation of this patient and what is the anatomical localization for his current problem based on the clinical findings?
-Based on the clinical findings the problem in this patient is with his heart.
Based on the detailed history and clinical findings the provisional diagnosis would be- k/c/o cor pulmonale with severe pulmonary arterial hypertension grade III with gross ascitis with acute kidney injury.
b) What is the etiology of his ascites? and how would you as a member of the treating team arrive at a diagnosis?Chart out the sequence of events!
-The reason for ascitis in this patient could be RHF. RHF leads to congestive hepatomegaly and thereby derranging the synthetic funtion of the liver to produce albumin. And hypoalbuminemia leads to fluid accumulation.
AKI can also lead to ascitis due to increased salt and water retention.
COPD---->RHF----> congestive hepatomegaly----> reduced protein synthesis----> ascitis due to fluid accumulation.
Decreased urine output--->AKI---> Salt and water retention ----> ascitis.
c)What is the efficacy of each of the drugs listed in his treatment plan?
d)What are his current outcomes ?
The patient can go in cardiopulmonary arrest.
QUESTION 4
Please go through the thesis presentation below and answer the questions below by also discussing them with the presenter
https://youtu.be/QlPrb1BSHGE
a)What was the research question in the above thesis presentation?
-Role of SAAG in diagnosing the etiology of ascitis.
b) What was the researcher's hypothesis?
c)What is the current available sensitivity and specificity of SAAG in diagnosis of etiology of ascites
When using the SAAG cut-off value 11 g/L, its sensitivity and specificity were 100.00% and 85.19% with an accuracy of 94.37%.
https://diagnosticpathology.biomedcentral.com/articles/10.1186/1746-1596-8-143#:~:text=When%20using%20the%20SAAG%20cut,%25%2C%20P%3C0.01).
QUESTION 5
Journal club questions on Ascites theme
a) Please identify the study design and outcomes in the article linked here https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6644216/ as well as the thesis linked here https://chandanavishwanatham19.blogspot.com/2021/03/of-thesis-clinical-profileevaluationdia.html
b) Please download the CASP diagnostic study checklist here https://casp-uk.net/casp-tools-checklists/ to evaluate the paper here https://www.hindawi.com/journals/ijh/2019/8546010/ and share your learning points on critical appraisal of the paper.
c) Please evaluate this randomized controlled trial on different techniques at ascitic tap here: https://pubmed.ncbi.nlm.nih.gov/28233752/ also after downloading the randomized controlled trial CASP checklist here https://casp-uk.net/casp-tools-checklists/
