A young man with severe hypertension secondary to renal failure and a strong family history of organ damage due to vascular causes

He was 3 yrs old when in 1992 his paternal grandfather expired due to stroke.. His paternal grand mother also died due to stroke.. His elder aunt’s death was dramatically heralded when she vomited on the weaving machine and collapsed which presumably was due to vascular pathology mostly brain. In1990 father had a headche for which he went to an RMP and found to have elevated BP which he neglected and took symptomatic medication untill 2000 when he fainted while attending work.he was started on medication but eventually gave up for magneto therapy which he presumed to be better than medication.Father’s sister while cleaning the backyard fell unconsciousand died which was also presumed to be due to a vascular pathology.

One day in 2007 *SUDDENLY* his father noticed he had lost vision in one of his eyes, he was taken to a physician and found to have a BP of 230/110.. He was restarted on medication. In a weeks time father vision recovered which again hints out at a transient vascular pathology... In 2017 father had a chest pain and managed medically by a cardiologist for coronary artery disease. The same yr he had gone to a hospital in bangalore and found to have renal failure and was started on dialysis.The pt cousin sister expired at very young age due to complications of CKD and Anemia diagnosed very late. 

2016 pt had visited a dentist for his tooth ache and found to have a BP of170/130.. After 2 months still his BP was the same after which he was adviced telmisartan 40mg.In the mean time he was receiving unknown medication for his ringworm infection.

In Dec 2018 pt experienced headache and vomitings went to a hospital adviced admission and given IV anti htns as he had an alarming BP valueof 240/130..He was discharged the next day after BP came down to170/90. sr creat found to be 3.5 was adviced amlodipine and torsemide.may 2019 when pt started experiencing headache pain abdomen blood stained vomitings SOB pt taken to hospital and creat found to be 6.15 and HB 6.0 he was adviced dialysis and blood transfusion. august 2019 when pt experienced SOB and chest pain. He was taken to a PG hospital in Kolkata where he was admitted in Pulmonology for his SOB.. 15 days he was evaluated and came to know it was his renal problem that had to be solved first and was discharged and sent to nephro opd.

The above history was taken from here : https://harrisonmedicine99.blogspot.com/2021/02/case-of-29-year-old-male.html first recorded by one of our students. 

They arrived at our college on 16th sept 2019 making a train journey of more than 1000 kms with a lot of hope after having heard about us from one of the patients in their pulomonology ward in IPGMER Kolkata.  

On presentation in opd we saw thin built poorly nourished young male with evident pallor.

This video shows his deidentified clinical  images from that time along with a discussion centered around him : https://youtu.be/xvE5b8Xk3vM

On examination: 

BP-150/90, (on antihypertensives) 

PR-110,

RR-26

Inspection: a visible apical impulse

Palpation: unequal chest expansion

Percussion: dull note in rt MA AA IMA IAA ISA

Auscultation: decreased breath sounds in rt MA AA IMA IAA ISA

16/9/2020:

Pleural tap done showed exudative picture with lymphocytic pleocytosis.. he had dialysis i/v/o his increased urea and creat and just when we had put the central IJV line on rt side he started to bleed..Bleeding was thought to be due to platelet dysfunction in uremia hence we carried out a dialysis by placing a Rt femoral central line and 1 SDP transfusion. bleeding stopped after a stitch. 

Pt was continued  on the following anti hypertensive medications :

TAB.Nifedipine 5mg QID to 10mg TID

INJ.LASIX

TAB.CLONIDINE

18th sep-Added INJ.CEFTAZIDIME,

INJ.AMIKACIN

19th Oct -?Catheter Catheter induced sepsis Added INJ.CETRIAXZONE for 5days(Tlc- 26000 came down to 8000)

2nd Nov - pericardial rub

4th Nov- initiation of ATT

7th Nov- added PRAZOSIN

10th Nov- added TELMI 40mg

11nov -spironolactone to NTG and labetolol iv infusion

13th Nov- onset of pulmonary edema and hypoxia and got intubated

14th Nov- ICD placed

15th Nov- extubated and ATT stopped

After brainstorming for the cause behind flash pulmonary edema and reviewing literature (more here : https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5015508/#!po=0.943396), conclusive decision was made that Rifampicin, as a potent ezyme inducer, was causing failure of anti-htn medication thus ATT stopped.

21st Nov- ATT reinitiated with 

T.ETHAMBUTOL

T.PYRAZINAMIDE

T.LEVOFLOXACIN

FROM JAN TO MARCH2020

T.NICARDIA 10MG QID

T.TELMA AM 40/5 H/S

T.LASIX 20MG TID

T.ALDACTONE 25MGOD

T.PRAZOSIN 2.5MG TID

T.OROFER XT LATER 

INJ.ERYTHROPOIETIN 2000IU/SC

ONTUESDAY,THURSDAY ,SATURDAYS:

T.LABETOLOL 100MG TID

T.PYRAZINAMIDE 1500MGOD

T.ETHAMBUTOL 1000MG OD

T.LEVOFLOX 500MG OD

ALTERNATE DAY HEMODIALYSIS

Advice at Discharge during May 2020: 

T.NICARDIA 10MG QID

T.TELMA AM 40/5 H/S

T.LASIX 20MG TID

T.ALDACTONE 25MGOD

T.PRAZOSIN 2.5MG TID

T.OROFER XT

ON TUESDAY,THURSDAY,SATURDAYS:

T.LABETOLOL 100MG TID

T.PYRAZINAMIDE 1500MG OD

T.ETHAMBUTOL 1000MGOD

T.LEVOFLOX 500MG OD

Follow Up REVIEW TO GENERAL MEDICINE OPD AFTER FITNESS

X Ray

We shared his problem for local inputs at that time with our local ecosystem and the PPTs from that time are available here : http://classworkdecjan.blogspot.com/2020/01/29-m-having-esrd-and-need-kidney.html

Eventually with Dialysis and modified  antitubercular therapy (excluding the enzyme inducers that played havoc with his BP), his pleural effusion subsided although the mystery of the cause for his exudative pleural effusion remains to this day whether it was tubercular or even because of his uremia. 

He continued regular dialysis sessions near his home in West Bengal soon after he left our hospital staying with us from September 2019 to May 2020 through the initial phase of the pandemic. Our elective students led by Avinash helped to raise funds for him through online resourcing that went to pay for his hospitalization for six months. 

He returned on April 2021 around one year later for his renal transplant which was successful without any acute rejection and he was regularly on the following maintenance  medications in the next 10 months  :

1. Tab Wysolone 7.5 mg bid 

3.Tab tacrolimus 3mg-x-2 mg 

4.Tab mycofenolate 750 mg / Tid 

but after 10 months on one of his routine check ups he found his creatinine became 3.2 mg per dl and we quickly asked him to attend our hospital.

Rest of the investigations and raw case records after admission are here : 

https://drkulkarnimd.blogspot.com/2022/02/30m-with-post-renal-transplantation.html?m=1

https://sanjay129gm.blogspot.com/2022/02/30-year-old-renal-transplant-recipient.html?m=1