35YR OLD MALE WITH? KARTAGENER SYNDROME

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Here is a case i have seen in : 

35 year old man, working as a cook in Hyderabad presented to our casualty at 3am with the chief complaints of 

Palpitations since 3 months 

Headache since 3 days

Neck pain since 3 days

Giddiness since 1 hour

Vomitings since 1 hour 

He was born in Nalgonda district to a coolie and a homemaker

He is 3rd in birth order. He has an elder sister and brother who are farmers and a younger brother who is a rickshaw driver. He studied until 5th grade and later he moved to Hyderabad and started working in a local restaurant. 

Since 15 years he has been cooking in a small restaurant in Hyderabad and got married 10 years back to a cook. No children. 

7years back he lost his right ring finger and little finger in a roti making machine. 

1 year back he had a history of cough with expectoration and fever and was diagnosed to have Pulmonary kochs for which he was on ATT for 6 months. 

10 days back, one fine morning he noticed swelling of both his lower limbs extending upto his ankles and over the next 3 days he even developed facial puffiness. He started to experience dyspnea on exertion after which he decided to visit a local hospital where he was put on tab lasix twice daily, Tab Omnocortil 5mg twice daily, Tab Acyclovir twice daily and Tab Monocef 200mg twice daily which he was on for 10days. 

Since 3 days he has been experiencing diffuse headache associated with neck pain which has aggravated over the past one day. He visited a local hospital yesterday where in he was given Tab amlong 5mg stat and Tab Telma 40mg stat. He started experiencing giddiness since 1 hour along with 2 episodes of vomitings over the last 1 hour.

PAST HISTORY :

No similar complaints in the past. 

K/c/o TB 1yr back took treatment for 6 months

Elder brother who is 45yr old was also diagnosed with HTN 1month back

No h/o DM, Asthma,  Epilepsy, CAD

PERSONAL HISTORY :

He has no addictions

ON EXAMINATION :

Pt is c/c /c

GENERAL EXAMINATION :

Pallor- absent 

Icterus - absent 

Clubbing - absent 

Cyanosis- absent 

Lympadenopathy - absent

Edema- absent 

VITALS: 

SPO2: 98% ON RA

TEMPERATURE : AFEBRILE(97F) 

BP: 170/100 MMHG 

PR: 72BPM, regular, high bounding pulse

RR: 17 CYCLES /MIN

GRBS: 116mg%

CVS:

Tapping apex + in right 5th ICS

Loud S1 +, S2 +

No murmurs

CNS:

Higher mental functions are normal

Cranial nerves are intact.

Power :5/5 in both UL and LL 

Tone : normal

Reflexes: 

Right UL all the reflexes were 3+

Left UL triceps was 3 

Left Supinator, Biceps 2

Knee reflex couldn’t be elicited

Ankle reflex -

Plantars showed flexion in both the limbs

Gait was normal

No signs of cerebellar lesion 

RS:

BAE +

Lungs were clear on auscultation 

PER ABDOMEN :

Abdomen was soft and non tender on palpation

Bowel sounds +

INVESTIGATIONS:

                      1. Hemogram

                                2. CUE

            3. RFT & LFT

           4. ECG

With limb leads reversal

            5. CXR

            6. MRI

7. TROPONIN - I: 

DIAGNOSIS:

?  KARTAGENER SYNDROME

HYPERTENSIVE URGENCY WITH  DEXTROCARDIA 

POST PULMONARY TB 

TREATMENT :

Day 1:  8/11/2020

1. TAB. TELMA 40MG PO OD

2. TAB. PANTOP  40MG PO OD BBF

3. TAB. ZOFER 4MG PO SOS

4. TAB.  NEUROBION FORTE PO OD (2PM) 

5. TAB. DOLO 650MG PO SOS

6. BP MONITORING 1HRLY

7. PR, TEMP, SPO2 4th HRLY 

8. GRBS 8TH HOURLY

DAY 2: 9/11/2020

Pt is c/c/c

Vitals :

SPO2: 98% ON RA

TEMPERATURE : AFEBRILE(97F) 

BP: 170/120 MMHG 

PR: 78BPM, regular rhythm, high bounding

RR: 17 CYCLES /MIN

GR BS: 116mg%

On Auscultation:  Fine crepts Rt: IAA

                               Lt: IAA, ISA, Interscapular

INVESTIGATIONS :

1. 2D ECHO

 Pt was having crepts and cxr showing bronchiectasis changes and also pt was not having children and pt of situs inversus.pt is suspected to have kartagener syndrome. 

Kartagener's syndrome is a rare, autosomal recessive genetic ciliary disorder comprising the triad of situs inversus, chronic sinusitis, and bronchiectasis. The basic problem lies in the defective movement of cilia, leading to recurrent chest infections, ear/nose/throat symptoms, and infertility.

Treatment: 

1. TAB. TELMA 40MG PO OD

2. TAB. PANTOP 40MG PO OD BBF

3. TAB. ZOFER 4MG PO SOS

4. TAB. NEUROBION FORTE PO OD (2PM) 

5. TAB. DOLO 650MG PO SOS

6. BP MONITORING 1HRLY

7. PR, TEMP, SPO2 4th HRLY 

8. GRB S 8TH HOURLY

9. SALT RESTRICTEDT

DAY 3: 10/11/2020

C/O headache and neck pain

O/E: pt is c/c/c 

 Vitals :

SPO2: 98% ON RA

TEMPERATURE : AFEBRILE(97F) 

BP: 170/120 MMHG 

PR: 78BPM, regular rhythm

RR: 17 CYCLES /MIN

GR BS: 116mg%

On Auscultation: Fine crepts 

                   Lt: IAA, ISA

INVESTIGATIONS: 

RENAL ARTERY DOPPLER

RX:

1. TAB. TELMA 80MG PO OD

2.TAB. CLINIDIPINE 5MG PO H/S

3. TAB. PANTOP 40MG PO OD BBF

4. TAB. ZOFER 4MG PO SOS

5. TAB. NEUROBION FORTE PO OD (2PM) 

6. TAB. DOLO 650MG PO SOS

7. BP MONITORING 1HRLY

8. PR, TEMP, SPO2 2ND HRLY 

9. GRB S 8TH HOURLY

10. SALT RESTRICTED DIET

DAY 4: 11/11/2020

C/O Neck pain

Vitals :

SPO2: 98% ON RA

TEMPERATURE : AFEBRILE(97F) 

BP: 150/110 MMHG 

PR: 88BPM, regular rhythm, Normal volume

RR: 17 CYCLES /MIN

CVS: loud S1,  no murmurs

RX:

1. TAB. TELMA 80MG PO OD

2.TAB. CLINIDIPINE 5MG PO H/S

3. TAB. PANTOP 40MG PO OD BBF

4. TAB. ZOFER 4MG PO SOS

5. TAB. NEUROBION FORTE PO OD (2PM) 

6. TAB. DOLO 650MG PO SOS

7. BP MONITORING 1HRLY

8. PR, TEMP, SPO2 2ND HRLY 

9. GRB S 8TH HOURLY

10.  SALT RESTRICTED DIET

11. VOLINI GEL FOR L/A ON NECK