A patient with big kidneys

A 48 year old man is found to have BP 170/95 and a follow-up blood test shows eGFR 55.  The abdomen seems to contain hard masses.  On following this up, this investigation is undertaken:

PKD liver and kidney MRI radiopaedia 5202

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So that’s the diagnosis.  What is the prognosis likely to be?  Is there anything you can do to alter it?

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This is advanced PKD with very large kidneys and not much normal kidney tissue in between the cysts. This patient is likely to be heading for dialysis and/or a renal transplant. It is amazing that liver failure is extremely rare in PKD, despite sometimes massive cyst formation – though there can be physical symptoms from all this extra volume in the abdomen.

PKD accounts for close to 10% of end stage renal disease in most countries.

Management of PKD has been mostly symptomatic until recently, but the first drug to slow down the progressive growth of cysts is now licensed: Tolvaptan, an ADH receptor antagonist.  CKD issues in PKD are the same as for any other patient with renal impairment.

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The image shown is courtesy of Prof Frank Gaillard (Radiopaedia)

A swollen leg

A 39 year old subsistence farmer is referred to the dermatology clinic with massive left leg swelling, present for several years.  His main complaint is of “leaking” and malodour, and he is reluctant to remove the tubifast dressing that he has applied himself.

What is the likely diagnosis?

What advice and treatment would you give?

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He has elephantiasis which is characterised by skin thickening and swelling of the underlying tissue leading to enlargement of his limb.  This is secondary to underlying problems with lymphatic drainage, and in this case the most likely cause is lymphatic filariasis.

The old bandages that he has applied should be removed and he should be shown/advised how to clean to reduce infection.  Emollients may be used to restore and maintain skin suppleness.

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Case contributed by Levie Mwale and Ann Sergeant

A call from a concerned GP

They have been treating an 86yo lady who is resident in a local nursing home.  She has known Stage 3 CKD, hypertension, peripheral vascular disease, eczema, osteoporosis and mild/moderate Alzheimer’s disease (which which she is usually independent in dressing and eating+drinking).  She has suffered from urinary tract infections in the past and these have been associated with worsening in her cognitive state.

Usual medications:  Bendroflumethiazide, Perindopril, Donepezil, Omeprazole, Alendronate (weekly), Calcichew-D3.

She was seen as a home visit 5 days ago due to increased confusion and reduced oral intake.  She appeared clinically dehydrated, pyrexial (38.2) and BP was 100/55.  Urine dipstick showed ++Blood, +Protein, +Nitrites, +Leukocytes.  An MSU was taken and she was started empirically on Trimethoprim.  U&Es taken at the time were available later that evening and showed worsened renal function (see below), at which point the nursing home was phoned and the thiazide diuretic and ACE inhibitor stopped.  She was reviewed this morning, and she was now apyrexial, her cognitive state was improved, and the nursing staff reported that her oral intake was now starting increase.  However despite this reported improvement, her renal function had deteriorated further.

Serial Blood tests     Urea  (mmol/L)  Creatinine(umol/L) Sodium            Potassium

GP 5 months ago             8.7                        133umol/L              136                        4.5
Initial consult                  20.6                        205umol/L              151                        5.4
Today                             22.3                        267umol/L              147                        5.7

Neither the GP nor the patients family were keen on admission, as the as been associated with worsened confusion and agitation in the past, but wonder if this is now necessary?

What would your management plan would be?  Where would the patient be best managed?.

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Now read what the expert wrote

 This lady has acute kidney injury on a background of chronic kidney disease.

Urosepsis can cause renal dysfunction in its own right, particularly in the elderly and those with pre-existing CKD.  In this case the history and the elevation of serum sodium, urea and creatinine in the first set of U&Es suggests that the patient is significantly dehydrated.  It is unusual to see overt hypernatraemia unless some form of disability – in this case reduced cognition- prevents the patient from drinking in response to thirst.

Stopping the ACEi and thiazide was the appropriate course of action.  The use of trimethoprim will partially confound the interpretation of the 2nd set of U&Es, as it competitively inhibits renal tubular creatinine secretion, causing increases in serum creatinine of around 30% in patients with CKD, likely accounting for the apparent worsening in these lab results.

The potassium level does not mandate admission, and whilst there has been an improvement in the sodium level, there is still a water deficit and probable dehydration present.  My advice was not to admit to hospital, but to push oral fluids in the nursing home, repeating bloods at the end of the week once the course of trimethoprim had been discontinued- provided function did not continue to decline then this could be safely managed in the community.

 

A call from the hepatology ward

A call from the Hepatology FY1:  She has been asked to ring due to concerns on the morning ward round about worsening renal function – specifically do we think this is hepato-renal syndrome?

56yo with history of alcohol excess, chronic obstructive airways disease and known cirrhosis with and previous oesophageal varices (in banding program).  Admitted 5 days previously with worsening jaundice and mild confusion.  An ascitic tap was performed on day 1 and a Bonano catheter inserted through which 4 litres of ascitic fluid was drained over the following 24 hours, with concurrent administration of 2x bottles of 20% human albumin solution.  Gram stain of the ascitic fluid demonstrated numerous neutrophil polymorphs, and the patient was therefore commenced on piperacillin/tazobactam at a dose of 4.5g tid i.v pending culture results.

Usual Medications:  Levofloxacin (stopped on commencing i.v. antibiotics),  Spironolactone (started in clinic, now withheld), Vitamin B co-strong, Seretide inhaler, Bisoprolol (now withheld), Citalopram.

Serial Blood tests                        Urea                        Creatinine

Clinic 2 months ago                         1.7                        53umol/L
Admission                                         5.5                        76umol/L
Day                                                    6.7                        89umol/L
Day 2                                                 7.1                        103umol/L
Day 3                                                 9.9                        131umol/L

What further information would you seek before giving your opinion?

See more of the history or other available results?

Other blood tests:, Bilirubin 121, Hb 121, WCC 13.2 (Neuts 11.1) Platelets 53, Prothrombin time 21 seconds

Abdominal USS :  shrunken nodular liver consistent with cirrhosis.  15cm Splenomegaly.  11cm kidneys bilaterally, no evidence of obstruction.

Blood pressure has been between 70 and 90mmHg systolic throughout the admission.   There has been a borderline temperature (max 37.8degrees). Urine output has not been completely documented due to poor patient compliance, however a urinary catheter has just been inserted.  Post catheter dipstick shows + haematuria, no proteinuria.

Now consider what advice you would give.

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Now read what the expert wrote

 This patient has significant acute renal dysfunction, on the background of chronic liver disease.  His normal sized kidneys and U&Es from clinic indicate that underlying renal function is normal and the dipstick result is against a primary parenchymal renal disease.

His hepatic impairment is a sensitizing factor for subsequent acute kidney injury.  The precise aetiology for the current dysfunction cannot be stated with certainty, but potentially includes components of

i)              Sepsis induced renal dysfunction (from presumed bacterial peritonitis)

ii)             Hypoperfusion from low BP (liver disease+ (i))

iii)           Over-diuresis & intra-vascular depletion from Spironolactone

iv)           Haemodynamic upset from large volume paracentesis (although replacement regime used was appropriate)

All the above lead to a state of pre-renal failure or established acute tubular injury.  An important differential would be the onset of the hepato-renal syndrome, characterised by oliguria in the face of volume repletion, extreme salt retention, progressive renal impairment, and a very poor prognosis (>90% mortality) in those not suitable for liver transplantation.

The common immediate management pathway for all of the above should be active volume resuscitation aiming to leave the patient intra-vascularly replete (this may be aided by the insertion of a central venous pressure line).  Investigation and treatment of sepsis should be undertaken, and consideration given to the use of laxatives to prevent constipation worsening encephalopathy.  Once the patient is volume replete (and assuming no diuretics are prescribed) then a spot urinary sodium can be measured, with a result <20umol/L  consistent with HRS.  The hepatologists may wish to consider the use of alpha-agonists such as midodrine as a means to support BP in the event of ongoing hypotension.  At this stage renal replacement is not required, however the renal team should stay involved with the patients care, as RRT could be required in the event of ongoing deterioration.   If this is occurring in the context of established HRS then careful discussion with the senior hepatologists would required as to the appropriateness of ongoing invasive and potentially injurious therapies in the setting of a very poor prognosis.

A call from the orthopaedic ward

A 68yo retired hospital cook had been admitted electively for total knee replacement 4 days previously. There was a background of longstanding rheumatoid arthritis, treated hypertension and CKD Stage 3 for which she no longer attends the renal clinic.

Serial Creatinines

Pre admission clinic               151umol/L

Day 1 post op                         176umol/L

Day 2 post op                          275umol/L

Day 3 post op                          329umol/L

Today (Day 4) post op             319umol/L

Renal dysfunction was first noted on Day 2 post operatively, and she has received a total of 7.5L i.v fluids since then.  A urinary catheter was inserted on Day 3, with hourly urine volumes initially 20-30mls/hr, but currently running at 50-100ml/hr.  Yesterday (Day 3) oxygen saturations were recorded at 87% and the patient placed on supplemental O2 which has raised the sats to 93-95%.  The FY1 states that there are crackles audible bi-basally on auscultation of the chest, but little evidence of dependent oedema.  The surgical registrar felt that the JVP was elevated and favoured administering IV furosemide.

The specific question posed by the senior surgical team is whether there is a role for the administration of i.v furosemide in this patient’s case given her reduced oxygen saturations and cumulative positive fluid balance, and what additional measures should be undertaken to ensure recovery of renal function.

What other tests might you request in this patient.  Is there anything else you would like to know about the patient’s management?  How would you answer their question regarding furosemide?

What further information would you seek before giving your opinion?

See more of the history or other available results?

Medications:  Bendroflumethiazide 2.5mg , Hydroxychloroquine, Losartan 50mg, Paracetamol 1g qds, Tramadol 50mg qds, Morphine 5-10mg prn.  (Losartan was administered up to and including post-op Day 2).

Chest X-ray : pleural thickening R base (present in 2010), prominent hilar vessels.

Renal tract USS : Kidneys Left 9.5cm Right 9cm, no evidence of obstruction, catheter seen in bladder.

Now consider what advice you would give.

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Now read what the expert wrote

On further questioning, it emerged that there had been relative hypotension (systolics 80-100mmHg) persisting from at least the immediate post-operative period to around Day 3.  For much of this time anti-hypertensives had continued to be administered, including the Angiotensin II receptor antagonist Losartan.  Hence the combination of pre-existing CKD, surgery, low BP (particularly in a patient with background hypertension and Losartan administration would offer a multifactorial set of renal insults to account for her acute on chronic renal failure.

Importantly, BP was now better, the potential nephrotoxic drug had been stopped, and urine output had risen with serum creatinine stabilized/fallen marginally over the last 24 hours.  With this combination (and a safe K+) there was no indication for renal replacement, and indeed if further insults could be avoided she was very likely to continue to recover.

Whilst my instinct over the phone was not therefore to administer furosemide, (and given in the surgical wards there are not middle grade physicians in routine attendence) I offered to review the patients fluid status myself.

Meeting the patient, she was lying flat in bed on 2L/min supplemental oxygen, had a respiratory rate of 10/minute and had difficulty staying awake whilst being examined.  Pupils were constricted.  T37.6.  BP 110/80.  HR 80.  Sats 94% on oxygen.  Dry crackles were present in both bases, more marked on the right side.  JVP was +3cm.  Sputum pot contained thick green sputum.

My impression/advice was therefore:

i)              Likely hospital acquired chest sepsis.  Start HAP antibiotics

ii)             Opiate toxicity due to declining renal function and drug accumulation.  Stop tramadol & morphine.  If strong opiate required consider fentanyl/alfentanyl due to safer pharmacokinetics in renal impairment

iii)           Low oxygen sats reflect (i) and (ii) rather than pulmonary oedema.  No indication for diuretics at present- fluid status appears appropriate.  Bibasal chest signs could represent atelectasis, but possibility of rheumatoid lung disease should also be considered.

iv)           Withold losartan at present.  There is no contraindication to re-introduction when renal function is normalised, but this would be best done after discharge with monitoring in primary care.

A 56 year old farmer with lip ulceration

A 56 year old farmer who lives in a village near Blantyre, Malawi, presents with painful oral ulceration which is making eating difficult.  On further questioning he also admits that he has pain on micturition as he has painful penile ulceration. You also see that he has inflamed conjunctivae.

He started antiretroviral drugs for newly diagnosed HIV infection 1 week ago (lamivudine, nevirapine and stavudine).

What diagnoses would you consider?

How would you manage him?

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It is important to examine the whole of the patient’s skin and mucous membranes to determine the extent of disease, and in this case this established that there was mucous membrane involvement only.

The most likely diagnosis is Stevens-Johnson Syndrome (SJS), an idiosyncratic drug eruption, with nevirapine being the most likely cause.  Other diagnoses to consider include immunobullous disease such as mucous membrane pemphigoid or pemphigus vulgaris, or inflammatory disease such as erosive lichen planus – these are rare conditions which follow a chronic course.  Although infections (eg. mucocutaneous herpes simplex virus infection) should be considered the involvement of all mucous membranes would make this very unusual.

It is important to stop the offending drug quickly, and so nevirapine was stopped and the patient was admitted to hospital.

Other measures are supportive: analgesia, mouth and eye care, antiseptics for skin/genital involvement, may require IV hydration/nasogastric feeding, may require catheterisation.  Long term complications include scarring of genitals (which may lead to phimosis) and scarring of eyes which in the worst cases may lead to blindness – treatment aims to avoid these complications.

In this case the following treatments were used: paracetemol was adequate for pain control, saline mouth washes, tetracycline eye drops, gentian violet as antiseptic to penile ulceration.  He was regularly assessed for signs of infection.  He was able to tolerate oral intake and did not require a catheter.  He was referred to the antiretroviral clinic to institute another drug to complete his HAART regime.  He developed no new lesions and his ulceration gradually improved to allow discharge from hospital.

Further info

Case contributed by Levie Mwale and Ann Sergeant

A 19 year old albino with a skin lesion

A 19 year old lady from Blantyre, Malawi, attends with a scaly lesion which has been present on her right cheek for 3 months.

  1. Can you describe what you see?
  2. What is the diagnosis?
  3. What is your management?

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This is an albino woman with a small (approx 1 cm) superficial looking red scaly lesion on her right lateral cheek.  She has some lentigines (the brown marks) and coarse wrinkled neck skin.  If palpated the skin over the red lesion feels rough.

This is an actinic keratosis (actinic/solar – sunlight (UV) induced, keratosis – scaly/horny growth).  Considered to be pre-malignant, actinic keratoses are most often seen in exposed skin in fair skinned individuals who have had excessive UV light.

An actinic keratosis may follow 1 of 3 paths: it may regress, it may persist unchanged, or it may progress to invasive squamous cell carcinoma. The actual percentage that progress to invasive squamous cell carcinoma remains unknown, and estimates have varied from as low as 0.1% to as high as 10%.  Generally, thicker lesions are more likely to progress.

She should be advised to avoid further damaging sun exposure by wearing a wide-brimmed hat, clothing which covers the skin and a high factor sunscreen on exposed sites.  The actinic keratosis can be treated in several ways including medical management with creams and surgical management.  As she has several further actinic keratoses on her neck and other cheek (not seen in the photo above) and as other treatment modalities are not available in the clinic she is treated with imiquimod.  This is an expensive treatment, but had been donated to the clinic from an overseas organisation.

Further info

A man with a foot ulcer

A 42 year old man attends the dermatology clinic in Queen Elizabeth Central Hospital, Blantyre in 2012 with an ulcer on the sole of his right foot.  He says that it has been present since 2004 but that he underwent surgery 1 and a half years ago.  He was told it was a squamous cell carcinoma.  It initially healed, but since then the skin has broken down and the ulcer is enlarging in size.  He went back to see the surgeons and they have sent him on to dermatology to get dressings.

  1. Can you describe what you see?
  2. What diagnoses would you consider? Is there any information that would be ideal to have that would help point you towards the most likely diagnosis?
  3. Would you like to examine any other part of his body?
  4. What would you do next?

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This is a deep, irregulary shaped ulcer on the mid-sole of the right foot.  There is no cm scale in the photo, but it must be at least 5 cm in diameter.  The ulcer looks relatively clean.  The base of the ulcer comprises of  red fleshy tissue.  It is not possible to see bone or tendons.

In this case the most likely explanation is recurrence of squamous cell carcinoma (SCC). It would be helpful to get his pathology report from his operation 18 months ago to confirm that it was SCC and to see whether it was completely excised at the time.

The differential diagnoses includes a neuropathic ulcer (this is less likely as the ulcer does not correspond to the area of sole with maximal pressure, and he has no history of neuropathy) and infection (which in this geographical setting may include fungi and mycobacteria: but note there is little discharge, swelling or surrounding inflammation).

You would want to examine his ipsilateral inguinal and femoral lymph nodes to check for signs of metastasis and if they are palpable a fine needle aspirate (or lymph node biopsy) should be sent to pathology.

A biopsy of the base of the ulcer for pathology would confirm or refute the diagnosis of recurrence of SCC.

A surgical opinion as to whether this is operable should be sought.

This case was contributed by Levie Mwale and Ann Sergeant

An unwell lady with a history of breast cancer

A 53 year old female presents to the Queen Elizabeth Hospital, Blantyre with a 2 day history of drowsiness, diarrhoea and vomiting.

Her past medical history includes hypertension, diabetes and recently diagnosed breast cancer.

On examination she is dehydrated, BP 95/60, pulse 120/min, temp 380C, O2 sats 90% on air and Glasgow Coma Score of 14/15 (E4V4M6).

  1. What other history is required?
  2. What diagnoses would you consider?
  3. What immediate tests are indicated?
  4. What treatment would you start?


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Is she on chemotherapy for her breast cancer? If so you would worry about the possibility of neutropenic sepsis.  Immediate tests would include a blood sugar level (quick to do and vital in anyone with a history drowsiness – and we know she has diabetes), urgent full blood count (FBC) and blood cultures.  Less urgent tests include a chest X ray, urine dipstick (send for microscopy and culture if indicated) and urea and electrolytes.

Intravenous (IV) fluids,  IV broad spectrum antibiotics and oxygen should be started as soon as possible.

FBC results: Hb 66, WCC 0.8, Plt 34.

This case was contributed by Ewan Brown and Leo Masamba.

 



A 4 year old boy with patchy hair loss

A healthy 4 year old boy is brought to the Dermatology clinic at Queen Elizabeth Central Hospital, Blantyre by his parents.  He has been scratching his head, and they have recently noticed that he has developed several small scaly areas with associated hair loss.

What is your diagnosis and how would you treat him?

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The diagnosis is tinea capitis (scalp “ringworm”).  This is a cutaneous fungal infection which invades the hair shaft and is often due to Trichophyton tonsurans (caught from other children) or Microsporum canis (caught from kittens or puppies). Without treatment it gets better spontaneously at puberty.  After puberty it only occurs in patients with underlying immunosuppression.

Treatment of choice is oral griseofulvin 15-20mg/kg/day for 6 weeks.  Additional application of a topical antifungal, during the early stages of treatment, may reduce the risk of transmission.  Terbinafine is an unlicensed alternative, but may not be as effective for infections with M. canis.   The only oral antifungal available in the department is ketoconazole, so he is prescribed this and topical Whitfield’s ointment.  His parents then tell you that his elder sister was treated with the same treatment successfully earlier this year.

Other siblings with infection should also be treated and if the family pet’s fur is falling out it must be treated too.

The other differential diagnosis to consider in this case is alopecia areata but the presence of scale distinguishes these diagnoses.

Further info

 

This case contributed by Levie Mwale and Ann Sergeant