A 32 YEAR OLD FEMALE WITH HYPOKALEMIC PARALYSIS WITH MULTIPLE JOINT PAINS

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CONSENT AND DEIDENTIFICATION : 

The patient and the attenders have been adequately informed about this documentation and privacy of the patient is being entirely conserved. No identifiers shall be revealed through out the piece of work whatsoever

Cheif complaints :

Deformities of both hands since 1 year

Complaints of low grade fever since yesterday night.

Dry cough since yesterday night

HISTORY OF PRESENT ILLNESS:

A 32years old female,who is a daily wage labourer (stopped 1 year back),due to her deformity,came with complaints of deformities of bilateral hands and low grade fever and dry cough since yesterday night.

Patient was apparently asymptomatic 1 year ago,and then she developed mild deformity of right hand(ulnar deviation of fingers)second metacarpalophalengeal joint(initially to start with),not associated with any pain,redness and tenderness and the deformity sequentially progressed to 3rd,4th and 5th MCP in a period of 1 month.She initially thought her deformity is due to prolonged use of her hand in that position while working (plucking weed from field),and later after one month  she developed similar sequence of deformity in other hand(left hand),2nd,3rd,4th and 5th MCP,in a sequence and then after 10 days she started developing pain in multiple joints (large and small of upper limbs and lower limbs)at same time and eventually after pain she developed swelling of all the involved joints not associated with any redness and local rise of temperature,and she developed swelling of her bilateral soles finding her unable to walk,with the above complaints she went to a local hospital for which she was given some medicine and used for 1 month and her pains subsided for that month,and later once she stopped those medication she again developed pains all over the joints,,and she has been abused by her husband these 7 months as she is not earning money,and  sitting idle at home,and so she didn’t used medication for 7 months,and suffered from the same pain for 7 months and 15 days ago,she went to hospital again and was prescribed medication for which her pains got decreased now,and on sunday(3 days ago)at around 3am, while she felt some dragging sensation of the left lower limb she thought of pulling her legs close,but she couldn’t and she couldn’t raise her right upper limb,for which she pinched her  left lower limb and right upperlimb herself and she didn’t felt any sensation and again,around 10am in the morning,she developed similar weakness in left upper limb and right lowerlimb and after an hour she couldn’t even hold her head,and for which she was taken to hospital around 2:00pm ,(her serum potassium was 1.65mEq/L)and was connected to Iv line(medication not known)and she regained her neck power initially(after 30 minutes) and after second infusion she regained power of left upperlimb and right lowerlimb and again after another infusion she regained power of right upperlimb and left lowerlimb and at 10:00am next day ,she regained her full power and she walked on her own,(serum potassium levels 6.8mEq/L) and she developed shortness of breath during the quadriparetic episode and decreased on its own,and she was referred to higher centre for management of hyperkalemia ,and patient came here for management and since yesterday she developed low grade fever with chills and dry cough and Sob on and off (since Sunday).

Restriction of mouth opening since yesterday and feeling of pain in the left cheek and zygomatic prominence when tried to open.

Early morning stiffness+

No deformity of wrist joint and no deformity of thumb.

No history of any nodules over body

Past History:

No similar complaints in the past.

History of dental implantation of upper 4 incisors,due to cosmetic appearance.

History of weight loss (8kgs)in 4years.

Not a known case of Dm,HTN,TB,Asthma,Epilepsy.

PERSONAL HISTORY:

Mixed diet,Normal apetite,Bowel and bladder moments regular,sleep adequate.

Treatment history:Using T.Hydrocholroquine 100mg tablets 

MENSTRUAL HISTORY:

Cycles were regular,3/28 days.

She gave birth to 3 children(2 girls and 1 boy),by Cesarean section, each at a gap of 2 years starting from 20 years.

The age of last child(son)11years.

GENERAL EXAMINATION:

Patient is lean bulit and nourished.

Bilateral cervical lymphadenopathy +

Conjunctiva encroachment over eyes medial side in both eyes+

No pallor,icterus,cyanosis,clubbing,edema.

VITALS: 

Bp:110/70mmHg

PR:84bpm

CVS: S1 and S2 Present 

CNS:HMF intact 

Patient is Afebrile at the time of presentation

CARDIOVASCULAR SYSTEM:

Apex beat felt at 5th intercostal space just lateral midclavicular line

Heart sounds normal 

No parasternal heave

No thrills and murmurs

RESPIRATORY SYSTEM:

Chest symmetrical 

Normal vesicular breath sounds 

No crepts,rhonchi,rales

ABDOMEN:

Soft and non tender

No organomegaly

No visible scars and sinuses .

JOINT EXAMINATION:

Bilateral 2nd,3rd,4th ,5th MCP of hands showing (ulnar deviation)deformity 

Tenderness present over right 3rd PIP,and 2nd and 3rd MCP of right hand.

Thumbs of both hands are spared with no deformity.

Tenderness Present over bilateral temporomandibular joint,restriction of mouth opening +

No wrist joint deformity.

Provisional diagnosis:?AUTO IMMUNE ARTHRITIS.

INVESTIGATIONS:

FINAL DIAGNOSIS:RHEUMATOID ARTHRITIS WITH HYPOKALEMIA SECONDARY TO ? RENAL TUBULAR ACIDOSIS.

TREATMENT:

1)Inj.Neomol 1gm /IV/SOS

2)Tab.Dolo 650mg/po/TID

3)Bp and temperature monitoring 4th hourly 

4)Tab.Methotrexate 7.5mg/PO/every friday

5)Tab.Folic acid 5mg/PO/Every Saturday 

6)Syrup.Potklor 5ml in 1 glass of water PO BD

7)Tab.Nodosis 500mg PO BD

DISCUSSION AROUND THIS PATIENT 

4 days back there’s documentation of HCQ 100mg sir 

Apart from it they said they don’t remember and usually they discarded

Can this be distal RTA sir ?? 

ABG showing bicarb of 8 

Left renal calculus 

RA??

Also urine ph 6

But abg is showing HAGMA

139-(104+8.7)=26

Na 139

K 3.2

Cl 104

Points in favour of distal RTA

Abg showing acidosis 

Autoimmune condition

Hypokalemia

Urine ph >5.5

Renal caliculi

And most importantly a similar case report that I have found

Renal tubular acidosis (RTA) is a disorder of renal acidification characterized by inability to acidify urine to pH <5.5 despite the presence of severe systemic metabolic acidosis and hypokalemia. Hypokalemia leads to acute-onset paralysis and may be a presenting manifestation of RTA. Its association with various autoimmune disease has been reported previously in published reports, but has not been much emphasized. We, hereby, report a case of RTA that presented during the flare of rheumatoid arthritis (RA). A 42-year-old female, a known case of RA for 5 years, presented with persistent joint pain for 1 week and acute-onset quadriparesis for 3 days. Primary investigations revealed hypokalemia with metabolic acidosis. She was managed conservatively with potassium supplements and bicarbonate supplements along with steroids and disease-modifying anti-rheumatic drugs. Such a presentation of renal tubular acidosis in a patient during the flare of rheumatoid arthritis is distinctly rare and previously unreported in published studies.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6152546/

"Definition of bone erosion

Bone erosion is a radiological term and reflects the fact that imaging is used for detection.10 Erosions are visible on plain radiographs as breaks in the cortical bone surface, and are often accompanied by loss of the adjacent trabecular bone."

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4096779/

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8497874/#!po=3.57143

Alkali therapy may be used to correct acidosis in patients with distal or proximal RTA .In patients with distal RTA, alkali therapy also corrects for hypokalemia. Alkali therapy with 1–2 mmol/kg/day NaHCO3 or KHCO3 is normally sufficient to equal daily acid production . however, in patients with nephrolithiasis or nephrocalcinosis, the elevated Na+ load with NaHCO3 therapy may cause increased urine calcium excretion, which can precipitate kidney stone formation. In these patients, K-citrate administration is preferable; this will also increase urine citrate excretion and prevent recurrence of kidney stones . Patients with severe hypokalemia should also receive K+ replacement (i.e., with KCl or K-citrate) to prevent further lowering of serum K+ concentrations and symptomatic hypokalemia . Long-term treatment of distal RTA generally requires a combination of NaHCO3 and KHCO3 . Children with distal RTA require sufficient NaHCO3 or KHCO3 (usually 4–8 mmol/kg/day) to maintain normal serum HCO3– concentrations and prevent growth retardation

https://link.springer.com/article/10.1007/s12325-020-01587-5

One reason is that phase 3 can be more challenging to do in rare disorders due to less sample size.

WARD PATIENT 

ADMITTED ON 08/09/2022

10/09/2022

S: Fever subsided and swelling of multiple Joints present 

O:

Patient is conscious,coherent and cooperative 

BP -  100/60 mm Hg

PR -  82 bpm

RR-   16 cpm

Temp- AFEBRILE 

spo2 - 99%  on RA

CVS-S1,S2 +,no added sounds heard

R/S-BAE+,clear

P/A-soft , non tender

CNS-NAD

A : Autoimmune arthritis with Hypokalaemia secondary to ? Renal tubular acidosis 

P : 

1. INJ. NEOMOL 1GM / IV /SOS 

2.  TAB. PCM 650 MG / PO /SOS 

3. TEMP charting 4th Hourly 

4. TAB. METHOTREXATE 7.5 MG /OD/ ONCE A WEEK (EVERY FRIDAY)

5. TAB. FOLIC ACID 5 MG / ONCE A WEEK  ( EVERY SATURDAY)

Anion gap

143-(12.4+103)=27

Delta anion gap=(pt anion gap-normal anion gap)=27-10=17

Delta bicarb =difference between normal and pt bicarb=25-12.4=12.6

If delta anion gap Is more than delta bicarb It means the patient is having both normal and high anion gap

What about potassium and bicarb in her treatment? 24 hour urinary results?

We dint start her yet on those medication sir.She is collecting her 24hr urine.will start them after she stops collection tmrw at 8am

The pathophysiological mechanism of dRTA in relation to autoimmunity remains unclear. Several reports suggest that autoantibodies against the CAII enzyme [36, 57] or the acid–base transporters are involved in the pathogenesis of dRTA in autoimmune disease

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4107275/

24hr urine protein is 195 sir,urinary creatinine 0.6,1300ml volume.Can we still consider SLE and send testing for immunological criteria sir??fever (constitutional domaina) and joint involvement +.atleast one clinical criteria is required and total of 10 points is needed

Chasing that diagnosis won't change our management plan currently

Did we get her echo? Please share the video. 

https://www.nejm.org/doi/full/10.1056/NEJM196207052670103

Urine potassium15.6sir(25-125 mmol/lit is normal)🙆🏼‍♀️it came lower than normal sir😅

Check if it can come low in distal RTA

Yes sir I guess some problem with the recorders mobile @⁨bhavana Intern⁩ ?will record it again and send

Urine anion gap in our patient is positive(228+15.6-221=26) indicating RTA in our patient 

Gastrointestinal bicarbonate losses can be differentiated from RTA by estimating the urine anion gap. Negative urine anion gap indicates increased renal NH4 + production (extrarenal cause for metabolic acidosis), while positive gap suggests RTA

https://www.google.com/url?sa=t&source=web&rct=j&url=https://med.stanford.edu/content/dam/sm/pednephrology/documents/secure/1Renaltubdisorders.pdf&ved=2ahUKEwjs7cTxuoz6AhX6H7cAHU0nCTUQFnoECAcQBg&usg=AOvVaw0DGihPwaEYFM4SkGxaki6r

I am not finding urine potassium seperately being tested in dRTA sir.its urine anion gap they are referring to most of the times

Can you check what is normal Urine K+ range? Standardized values and not this lab value?

For adults, normal urine potassium values are generally 20 mEq/L in a random urine sample and 25 to 125 mEq per day in a 24 hour collection.

https://www.ucsfhealth.org/medical-tests/potassium-urine-test#:~:text=Normal%20Results,of%20potassium%20in%20your%20body.

This is because the urine K+ should have been much lower for that hypokalemia and this is classically "pseudonormal". Same like with normal PTH in hypercalcemia is abnormal

👏🏻👏🏻thank u so much sir

DISCHARGE SUMMARY 

Krishnamma

 *Diagnosis* 

Hypokalemia secondary to Distal renal tubular acidosis with autoimmune arthritis(? Jaccouds arthropathy? Rheumatoid arthritis)

TMJ arthritis 

 *Course in the hospital* 

Patient was admitted with the above complaints and investigations were done showing mild hypokalemia,hyperchloremia,abg showing metabolic acidosis and urine ph:6,USG s/o left renal caliculi,24hr urine potassium 15.6(high/pseudonormal for the degree of hypokalemia) suggesting renal wasting of potassum:features suggestive of distal RTA

X rays s/o autoimmune arthritis?Jaccouds arthropathy? Rheumatoid arthritis .Keeping in mind the association of autoimmune disease (RA)and distal renal tubular acidosis patient is started on Tab Methotrexate 7.5mg once weekly,Tab Folic acid 5mg once weekly,Tab Nodosis 500mg BD,syrup potchlor 5ml+1glass water BD.Patient is adviced to regularly be in follow up with her serum potassium and bicarbonate values .OMFS (TMJ arthritis) and orthopedic referral was taken and advice followed

Advice at discharge

Tab methotrexate 7.5 mg OD weekly once on Fridays

Tab folic acid 5mg once daily every Saturday

Tab Nodosis 500mg PO TID

Syrup potchlor 5ml in 1glass water BD

Tab Flexon MR PO OD *2days

Cold fomentation of pre auricular area

Plan :Cementation of maxillary FDP

Hand surgeon opinion on Tuesday /Friday's for deformed MCP joints(in follow up with dept of orthopaedics)

OMFS review after 1 week in view of TMJ arthritis(In view of Cementation of maxillary FDP)

GM review on 15/9/2022 with serum electrolytes.