CIDSCON Clinical case discussion Case 1. Dr Abi Manesh CMC

Transcription

1 CIDSCON Clinical case discussion Case 1 Dr Abi Manesh CMC

2 History 21 year old lady, lab technician from Kerala Completed training, working in a small hospital 2013 had fever, cough & expectoration x 3/12 evaluation revealed consolidations involving the right upper and middle zones repeated sputum AFB smears and cultures were negative Treated empirically with ATT (HRZE) had some improvement of symptoms received 18 months of ATT in total.

3 2016 symptoms recurred evaluation revealed multifocal consolidations involving both the lungs (right more than left) with small cavitations mediastinaladenopathy. Twice BAL and TBLB done elsewhere biopsy revealed chronic granulomatous inflammation Both times and xpertand cultures for MTB are negative.

4 She has been well since childhood though she was treated empirically for TB at 6 years of age. Has one sister. No unusual exposures / travel / pet contact. Does not smoke or consume alcohol.

5 Examination Blood pressure = 90/60 mm/hg Pulse = 108 /min; BMI 13.2 kg/m 2 Respiratory rate = 26/min, JVP not elevated No pallor, icterus, cyanosis, clubbing, lymphadenopathy and pedal edema. Trachea central. Right sided reduced air entry with bilateral scattered crepitationsand bronchial breathing present Rest of examination normal.

6 Investigations Hb 10gm% ; TC 14600cumm (NE:82, LY:11, MO:6, EO:1, BA:0) Blood borne virus screen negative LFT: 0.3/ 0.2/ 8.4/ 2.9/ 35/ 23/114 CRP: 138 mg/dl Blood cultures x 2 negative

7 Chest xray

8 What are the possible differentials and the further treatment plan?

9 Our differentials Young lady with previously no pre-morbid illnesses with slowly progressive multifocal consolidations no other system involvement clinically Unusual organisms with underlying structural lung problems / systemic immune defects like nocardia, aspergillusetc. Rarer pathogens like atypical mycobacteria, actinomycosis MDR TB, though was considered less likely.

10 What is the next step of evaluation you would request for? 1. Sputum anaerobic cultures 2. History of intake of fresh crab and ask senior parasitologist to look for eggs in sputum. 3. Ask for xpert Ultra in the sputum 4. Request for Nitro blue tetrazolium test 5. Consider emperical treatment of MDR TB as extensive evaluation for other causes has been negative.

11 Additional information BAL, TBLB and sputum cultures aspergillus spp. NBT test negative. Lung biopsy here granulomatous inflammation. On further enquiry, her sister was admitted in CMC in 2006 for the treatment of pericardial meliodosis. Consanguinousmarriage

12 How would you proceed further? 1. Aspergillusis a contaminant here as it s a ubiquitous organism do not treat it! 2. 16s sequencing in the lung biopsy tissue to rule out meliodosis 3. CGD is still likely do Dihydrorhodaminetest 4. Request for serum antibodies against aspergillus to rule out ABPA. 5. Request the microbiology lab to maintain the LJ culture at lower temperatures to grow rarer atypical mycobacteria.

13 Final diagnosis DHR strongly positive Chronic granulomatous disease (CGD) with chronic invasive pulmonary aspergillosis.

14 What would be the treatment plan for this patient now?

15 CGD genetically heterogeneous condition characterized by recurrent, life-threatening bacterial and fungal infections and granuloma formation. Infections are generally caused by catalasepositive micro-organisms (most bacterial and all fungal pathogens are catalase positive). Aspergillusspecies, S. aureus, Burkholderiacepacia, Serratiamarcescens, Nocardia species

16

17 Management of CGD Antimicrobial prophylaxis: TMP-SMX DS 1 OD lowered the incidence of bacterial infections from 15.8 per 100 patient-months to 6.9 per 100 patient-months Itraconazole200mg OD as prophylaxis Vaccines: BCG, Live bacterial vaccines CI Live viral vaccines no problem. HSCT: Curative

18 Back to our patient Started on voriconazole Slowly improved, became oxygen independent The sister developed varicellaa week later she developed a disseminated varicella infection with altered sensorium and died in Medical College, Trivandrum.

19 Case 2

20 History 30 yr old female teacher from Bhagalpur, Bihar March 2015 Headache and vomiting since 4 months was started on ATT (emperical) along with steroids April MRI brain done was showing obstructive hydrocephalus June symptoms worsened CT done was showing bilateral ventriculomegalywith more on left side CSF - 80 cells with lymphocytic predominance (96%) protein 120mg/dl; glucose 110mg/dl (plasma180 mg/dl)

21 Underwent decompression of the hydrocephalus in November Immediately following the surgery she developed Left sided facial weakness Left sided weakness of upper limb and lower limb with bladder incontinance. She continued ATT with steroids with no significant improvement. Came to CMC at that point.

22 Examination No meningeal signs Left side power 3/5 Left UMN facial palsy Left plantars upgoing No cerebellar signs Fundii: Papilloedema +; no other lesions.

23 November Temporal horn, trigone and occipital horns of the right lateral ventricle are ma rkedly dilated with subfalcine herniation and midline shift. 2. Ependymal, subependymal ring-like enhancement 3. Peripherally enhancing lesion measuring ~10 x 16 mm in the right choroid plexus

24 Tests CSF: Appearance: clear Glucose 55 mg/dl Protein 58 mg/dl Counts 10 cells 96% lymphocytes CSF fungal, routine, mycobacterial cultures, Xpertnegative. Cryptococcalantigen negative. Hb: 11.2g/dl TC: 7200 cells/cumm N59%, L26%; M9%; E6% ESR 70mm at 1 hr BBVS negative CRP 5.25 mg/l CSF cytology: CSF with microbial contamination -unsatisfactory for evaluation of malignant cells.

25 What are the possible differentials and the further treatment plan?

26 What is the most relevant next investigation to do? 1. CSF Xpert with large volume 2. EITB for NCC in the serum 3. Hydatid antibody in the serum 4. The non improvement is likely secondary to paradoxical reaction with tuberculous meningitis increase the dose of steroids and continue ATT 5. EITB for NCC in the CSF

27 Our differentials were This young lady presents with CNS pressure symptoms with no systemic symptoms. does not have signs of meningeal irritation and has also not responded to ATT or steroids. Both suggest a space occupying lesion rather than a primary chronic meningitis. Since the lesions were predominantly in the ventricle, the possibility of NCC was considered. Other differentials like TB, cryptococcusetcwere considered less likely.

28 EITB -Strongly positive for Cysticerci in serum and CSF (all seven bands were strongly positive)

29 How would you treat this lady? 1. Decompression followed by antiparasitic therapy and steroids 2. Preoperative antiparasitic therapy with steroids followed by surgery 3. Surgery and steroids alone and avoid antiparasitic therapy 4. Steroids alone 5. Antiparasitic therapy alone

30 She underwent a urgent decompression of the right lateral ventricle followed by albendazole and steroids. Her hemiparesis improved completely following the surgery. She is on regular followup.

31 What additional step is mandatory in the management of this patient? 1. Whole body x rays to look for subcutaneous cysticerci. 2. Avoiding pig contact 3. Check for pituitary insufficiency 4. Order a MRI spine as well to be complete 5. Screen the siblings for NCC with a plain CT scan

32 November 2016

33 Case 3

34 History 16 years old student from Nigeria presented with history of non healing ulcers over the left lower limb since January Initial lesion was a painless papule (around 1x1 cm) which became 10x10 cm in over 4 months. There was no history of trauma to the limb or any history of inoculation to the lower limb. There no other systemic symptoms.

35 Evaluated locally and treated with Rifampicin and Clarithromycin for a period of one month which he stopped as his ulcer started worsening. Due to persistent non healing ulcers and active new lesion with purulent discharge came to CMCH, Vellore for further management.

36 O/E MSK: Ulcer over the anterior, medial and lateral aspect of the left lower limb. Tenderness over the calf was present. Left inguinal lymph nodes 1x1 cm Edema of the left lower limb-pitting type Sensory examination normal; no thickened nerves.

37

38

39 Hb 9.1g/dl TC 9400 cumm DC N67 L21 M9 E3 Platelets 6.1lakhs/cumm BBVS negative Biopsy: necrotising granulomatous inflammation with no organisms.

40 SUMMARY 16 Years old student from Nigeria presented with the history of non healing ulcerative lesion with pus discharge in the left lower limb which progressed over 6 months. What are the possible differentials in this setting?

41 Our differentials were 1. Buruli ulcer 2. Cutaneous leishmaniasis 3. Basidiobolomycosis 4. Pyoderma gangreonosum 5. Cutaneous tuberculosis

42 Further tests The intra-op pus sample showed many AFB. The Xpertsent in the tissue was negative and Mycobacterial cultures are awaited. LeishmaniaPCR and fungal c/s from the intraop tissue sample was consistently negative. In view of the epidemiological data, clinical presentation and consistent histopathology Buruliulcer was considered as the likely diagnosis.

43 When to suspect?

44 How would you like to treat this patient? 1. Thorough debridement alone 2. Combination therapy with Rifampicin + Clarithromycin / Streptomycin with adequate debridement. 3. Combination therapy with Rifampicin + Clarithromycin / Streptomycin alone 4. Local heat therapy 5. Steroids Wadagni AC, Barogui YT, Johnson RC, et al. Delayed versus standard assessment for excision surgery in patients with Buruli ulcer in Benin: a randomised controlled trial. Lancet Infect Dis. 2018;18(6):

45 On treatment his lesions tend to worsen and ulcerate further however no new lesions appear Paradoxical worsening Noted in 30% Peaks around week 8 Does not affect outcome Mycolactone is probably responsible Nienhuis WA, Stienstra Y, Abass KM, et al. Paradoxical responses after start of antimicrobial treatment in Mycobacterium ulcerans infection. Clin Infect Dis. 2012;54(4):