Clinical Problem Solving

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Clinical Problem Solving

• Discussant Bill Curry
• Presenter Stan Massie
• December 18, 2007

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Case 1

• 78 y.o. WF with shortness of breath
• Started 7 months ago
• Progressive DOE, now cant do 1 flight of stairs
  without stopping
• No orthopnea or PND, no CP
• Has had some cough, intermittent sputum described
  as yellow with occasional streaks of blood
• No F/C/N/V/LE edema or sick contacts

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Case 1

• PMH
• Non Hodgkins Lymphoma
• Dx 1980, s/p resection, recurrences in 86 90
  rx Chemo
• Hepatocellular Carcinoma s/p resection 9 mos ago
• Cirrhosis due to Chronic Hepatitis C
• HTN
• Hypothyroid
• Meds Synthroid, Lyrica, Inderal
• Social Hx No T/E/D, worked in knitting mill for
  1 yr, otherwise no employment history. Married
  with grown children.
• FH no significant illnesses

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Case 1 Physical Exam

• BP 112/73m P-71, R-12, AF
• HEENT-normal
• Chest-sparse bibasilar rales, no wheezes
• Cardiac- RRR no M/G/R
• Abd- normal, no ascites or HSM
• Extr- no edema, no clubbing

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Case 1 Evaluation

• Labs
• Chem 7 normal, Calcium normal
• CBC normal except Hct 48, PLT 96
• LFTs normal, Alb 3.5
• TFTs normal
• CXR lingular predominance of small nodules and
  air bronchograms

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Case 1 Evaluation

• Chest CT nodular lesions worrisome for
  granulomatous disease
• Bronch Mycobacterium Avium (MAC)
• Diagnosis MAC/Lady Windermeres syndrome

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Mycobacterium Avium Complex (MAC)

• Ubiquitous free-living organisms readily
  recovered from natural reservoirs
• Organisms typically acquired from the environment
  
• Prevalence/incidence unknown
• One survey estimated 3000 cases per year in U.S.
  in early 1980s
• Many believe incidence is increasing
• Typically presents as either lung disease or
  disseminated disease (immunocompromised)

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Mycobacterium Avium Complex (MAC)

• Four clinical syndromes of MAC lung disease
• Cavitary lung disease usually seen with
  underlying lung disease, age, alcoholism, and
  male sex
• No predisposing conditions mid-lung field
  nodular bronchiectasis due to MAC with persistent
  cough and purulent sputum
• Adolescents or adults with cystic fibrosis
• Hot tub lung Hypersensitivity-pneumonitis
  syndrome most frequently reported in association
  with hot tub use

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Lady Windermeres Syndrome

• Middle lobe bronchiectasis with MAC
• First described in 1992 in 6 elderly women
  without predisposing factors
• Hypothesis voluntary cough suppression inhibits
  removal of secretions leading to focal
  inflammation
• Syndrome named for Lady Windermeres Fan, a play
  by Oscar Wilde

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Lady Windermeres Syndrome

• Common features
• Cough suppression or chest deformities
• Chronic cough
• Findings localized to lingula and RML
• Diagnosis
• Typical clinical and radiographic features
• Cultures positive for MAC
• Management multiple drugs, 50 failure rate at 1
  year

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The American Journal of Medicine (2007) 120,
515-517
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The American Journal of Medicine (2007) 120,
515-517
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http//www.edmurals.com/pages/snoopy-mural.html
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Case 2

• 30 y.o. AAF with N/V for 3-4 days
• Notes N/V starts about 30 mins afer meals, no
  blood in emesis
• Notes dizziness and malaise during same time
  period.
• Denies abdominal pain, diarrhea, F/C
• Denies dysphagia, CP, or sick contacts
• Reports LMP was 1 month ago

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Case 2

• PMH
• HIV (CD4350 one year ago)
• HTN
• CKD (baseline Cr 2.4)
• Meds ACEI
• SH
• Smokes 1 ppd, denies ETOH.
• Prior IVDU (none in last 1-2 years)
• Currently living in rehab facility
• FH GF had ESRD

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Case 2 Physical Exam

• 168/108, P- 81, AF, Wt-133 lbs.
• Gen WDWN, mental status approp.
• HEENT PERRL, no thrush/petechiae
• Cardiac RRR no M/G/R
• Abd soft, NT/ND, no tenderness
• Rest of exam normal

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Evaluation

• Labs
• CBC 3.5 217
  29
• Chem 7 137 107 56
  5.0 21
  9.9
• UA 3 Protein

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Course

• Patient admitted and hydrated.
• Renal function failed to improve and she was
  started on dialysis.
• Records showed that she had been evaluated a year
  ago and found to have 15gm protein on 24 urine,
  but unfortunately lost to follow up
• Presumptive Diagnosis HIVAN

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HIV associated Nephropathy

• Presence of proteinuria or renal dysfunction
  associated with worse outcomes in HIV pts
  (regardless of histology)
• Collapsing Focal Glomerulosclerosis is the
  primary lesion of HIVAN (found on 60 of
  biopsies)
• HIV patients can have other reasons for renal
  dysfunction

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Normal Glomerulus
UptoDate Online Version 15.3
Collapsing FGS
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HIV associated Nephropathy

• HIV associated FGS occurs in 2-10 of all HIV
  pts. (decreased incidence in HAART era)
• Mostly affects african americans
• Usually presents with nephrotic syndrome, bland
  urinary sediment and normal blood pressures

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HIV associated Nephropathy

• In the past, pts typically progressed to ESRD in
  1-4 months
• No therapy yet proven effective, but HAART
  appears to be effective in preliminary studies.
  ACEI/ARB also recommended.

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http//www.funnyphotos.net.au/christmas-cartoons/
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Case 3

• 31 y.o. M presents to SDC with rash
• Started 2 weeks ago in perineal area
• Tried OTC steroid, antibiotic and antifungal
  creams without benefit
• Pruritic, worsens with sweating
• Now has spots on thighs and lower abdomen
• No change in soaps/deodorants etc.
• No F/C, joint pains, urinary complaints or change
  in bowel/bladder function

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Case 3

• PMH Neck fracture s/p fixation, chronic neck
  pain
• Meds Lortab, Mobic
• SH monogamous, has 3 children. No IVDU.
• FH noncontributory

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Case 3 Physical Exam

• Healthy appearing male, VSS AF
• Skin
• Diffuse papular rash, lesions all of similar
  morphology involving thighs, lower abdomen and
  inner surfaces of arms and axillae. Some papular
  lesions between fingers.
• Perineal area with weeping, erythema with
  satellite lesions and some confluent areas

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Case 3 Evaluation

• Patient presumptively diagnosed with Scabies
• Empiric treatment with Permethrin prescribed.
  One week later his symptoms and rash had
  completely resolved.

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Scabies

• Infestation of the skin by the mite Sarcoptes
  scabiei
• Intensely pruritic eruption in a characteristic
  distribution
• Worldwide may affect 300 million
• Transmitted person to person, associated with
  crowding
• Highest incidence in winter months (crowding,
  longer survival of parasite)

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Scabies Characteristic Lesions

• Typical lesion small, erythematous, nondescript
  papule, often excoriated and tipped with blood
  crusts
• Pathognomonic lesion the burrow is a thin,
  grayish, reddish, or brownish line that is 2 to
  15 mm long (may not be seen).

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Interdigital lesions of scabies
The essential lesion is a small, erythematous,
nondescript papule.
UpToDate. Online Version 15.3
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Scabies Burrow
http//www.emedicine.com/derm/images/Large/3219REV
D3219ScabiesBurrwow_2.jpg
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Characteristic distribution of lesions in adults
with classic scabies.
American Family Physician Jan 15 2004 69(2).
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Scabies Diagnosis

• Generally made from the history and the
  distribution of lesions
• Suspect in patients with
• Widespread itching, worse at night
• Out of proportion to visible skin changes
• Sparing of the head (except infants and young
  children)
• Family involvement

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Scabies Treatment

• Permethrin 5 cream applied to entire body
  surface from the neck down, including beneath the
  nails
• Household and close personal contacts must be
  treated simultaneously

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Sarcoptes scabiei and eggs
UpToDate. Online Version 15.3
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Selected References

• MAC/Lady Windermeres Syndrome
• Gangat N and Johnson P. A Wilde Diagnosis. Am
  Journal of Medicine. 2007120515-517.
• UpToDate Online v 15.3. Epidemiology of
  nontuberculous mycobacterial infections.
• HIV Associated Nephropathy
• UpToDate Online v 15.3. Collapsing FGS and other
  renal diseases associated with HIV infection.
• Scabies
• Flinders, DC and Schweinitz P. Pediculosis and
  Scabies. Am Fam Physician. Jan 15 2004 69(2).
• UpToDate Online v 15.3. Scabies.