The Tanned Lady

This Case from another hospital has been anonymised.

This 50 year old female was admitted to a hospital with the following Chief Complaints:

1. Fever
2. Back pain
3. Fatigue
4. Muscle Weakness
   

The history was relatively short being only 8 days which started with fever up to 38 deg C and associated back pain. She attended a nearby Clinic and the doctor prescribed antibiotic therapy although it is not known what diagnosis was established at that time. She took the antibiotics for 3 days but there was no symptomatic improvement.

After 6 days she developed Fatigue and Loss of Appetite. After a further 2 days, she was becoming increasingly weak and could not get out of bed. Her husband needed to help her get to the toilet, and on one occasion she experienced diarrhoea.

At this point, several important questions were asked to elaborate on the history such as what type of fever was it? Continuous or Oscillating / Spiking Fever. The latter is very suggestive of a bacterial / viral infection / abscess whereas continuous low grade fevers can represent non-infective aetiologies once infection has been ruled out. It was a continuous fever, although the patient said it was 'up and down' suggesting it was a spiking fever.

Moreover, where was the back pain? It was in her central back. Then, what type of pain? Was in continuous? Intermittent? Dull? Sharp? Associated with movement? Tearing? Worse on breathing deeply?

The importance of back pain can represent many different aetiologies here especially with fever such as a discitis, vertebral osteomyelitis, paravertebral abscess, myeloma, inflammatory arthropathies, metastatic disease, dissection, chest infection, pulmonary embolism, UTI, renal pain from rhabdomyloysis??....the list is extensive and by no means complete here, and by asking the right questions can lead the doctor to a more accurate idea of what is going on.

The patient had diarrhoea but it was not asked whether the diarrhoea was painful, the colour, the amount, whether there was any associated blood or mucus. This could possibly fit with inflammatory bowel disease which can cause diarrhoea (bloody), fever, fatigue, and arthropathy or a reactive arthritis / Reiter's Syndrome. The diarrhoea of course could be just due to antibiotic therpay!

Was the back pain due to pancreatic cancer invading the retroperitoneum and causing malabsortion and steatorrhoea?? The importance of asking the most minute details about diarrhoea cannot be taken lightly.

Were there night sweats? Weight loss? These questions needed to be asked and in addition, any history of TB exposure, chronic cough, haemoptysis-- TB can cause fever, and if it spread to the spine it may cause back pain too (Pott's disease).

The patient's past history included some congenital hip problems only and the patient had never smoked, did not drink alcohol and had never had a blood transfusion.

She was taking no medications apart from the antibiotic therapy.

No Family History or Detailed Social History was asked which is unfortunate as these may have given some clues.

Physical Examination: She was sleepy and hardly answered questions which may account for the paucity of history.

Temp: 39.1 Deg C, BP 82/40, SpO2 96% ? with Oxygen or Room Air. No Resp Rate or heart rate were recorded which in this situation was very important as the patient was clearly ill.

There were no meningeal signs-- no neck stiffness, No Kernig's Sign. Babinski was not performed.

The rest of the examination was described as normal.

Clearly, from the above examination, it was not entirely clear what the problem was with this patient.

However, the patient was clearly shocked and with a high fever and low blood pressure one has to consider Septic Shock as the primary problem.

Chest Xray revealed a left sided pneumonia. CT Chest again confirmed the pneumonia seen on chest xray.

Blood tests:

WBC 11,200, Platlets 9.4 , CRP 29.3, INR 1.46, D-Dimer 2.7, [FDP not tested]
CK 615
BUN 76, Creat 4.57, K 4.0, Na 128
Hb 10.3, MCV 84.2

AST 97, ALP 1434, gamma-GT 266, Bil 3.4 (Direct 2.4, Indirect 1.0), LDH 295, Alb 2.5

Urine: WBC 10-19. No bacteria. Myoglobin 1632.

ABG: pH 7.5, Low pCO2, HCO3 19.6, PO2 54.4 and BE -0.9

The above results signify severe problems for this patient including:

• Acute [probably Bacterial] Pneumonia with Hypoxaemia and Respiratory Alkalosis
  
• Disseminated Intravascular Coagulation
• Rhabdomylosis
• Acute Renal Failure [probably from combination of Shock, Sepsis and Rhabdomyolysis]
• Severely abnormal liver function [? Shock Liver -- unlikely here as usually ALT and AST are very high in shock liver; this picture looks more obstructive]

The patient was treated with a 3rd generation cephalosporin and this was changed to meropenem by the 13th day.

No organism was identified by blood culture which may be due to previous antibiotic therapy.

She also received gamma-globulin, Anti-Thrombin III for the DIC and under went plasmaphoresis for endotoxin related shock and renal failure on several occasions.

The HDF was stopped when the plasma creatinine reached 0.94 although the bilirubin was now 5.7

Antibiotics were stopped 14 days after admission.

However, she was complaining of itching. She was otherwise saying that she felt well.

The most striking thing that was noticed was her tanned skin which was not the yellow one sees with Jaundice, but a tan from being in the Sun.

However, she said she never went out in the sun and she did not come from a Southern area of Japan. She said her skin had become increasingly tanned since last year BEFORE she was unwell with this acute problem. She otherwise was feeling well.

She had never had Hepatitis B or C infection and as mentioned, she did not drink alcohol.

This suggested, before examination, that the liver problem pre-dated the infection and was chronic in nature.

On examination, she looked well. There was no asterixis [liver flap], slight palmar erythema [red palms], and mild excoriations [scratch marks] on her arms.

She had deep tanning in exposed and non-exposed area of her skin.

JVP was not raised. No lymphadenopathy. Eyes were jaundiced. No liver breath [fetor hepaticus].

Pulse 80/minute, No heaves / Thrills. Heart sounds were normal. Mild peripheral pitting oedema.

Respiratory rate was 18/minute. Chest revealed some mild left basal crepitations [crackles] which did not clear on coughing consistent with resolving pneumonia.

Abdominaal Examination was abnormal: The patient had one spider naevus [vascular spider] on the left supraclavicular area. The liver was enlarged about 3 fingers below the costal margin and was mildly tender but smooth to the touch. Splenomegally could not be identified from palpation, but Traub's Space was Dull on percussion consistent with mild splenomegally.

Bowel sounds were normal.

Clinical Impression

The acute problem had clearly resolved.

However, she had obvious Hepatosplenomegally, Deeply Tanned Skin, Signs of Chronic Liver Disease and Liver Biochemistry consistent with an Obstructive Picture and particularly a High Alkaline Phosphatase.

The CT abdomen confirmed the physical findings and ultrasounds showed no dilatation of the Common Bile Duct.

Obviously, the above findings are consistent with Primary Biliary Cirrhosis. A differential diagnosis, if this were a Caucasian patient, would be Haemochromatosis especially because of the Deep Tanning of the skin.

Autoantibodies were performed and ANA was positive 160 (+), Anti-Mitochondrial M2 Antibody 49.4 (+)

Hence, the diagnosis here was likely to be PBC. It would fit the clinical features of this patient in that it is common in females aged between 30-60 years and approx 50% have no symptoms prior to diagnosis. Particular features include pruritis [itchy skin], tanning of the skin[due to increased Melanin deposition NOT BILIRUBIN], and smooth Hepatomegally in advanced stages. Most other causes of chronic liver disease produce a small liver. However, acute hepatitis can produce an enlarged liver.

PBC is associated with other autoimmune phenomena such as Sjogrens syndrome, autoimmune thyroiditis, CREST syndrome and autoimmune arthritides.

Most patients thesedays are identified by picking up blood abnormalities when asymptomatic with a raised ALP being one of the first signs.

Obviously, this patient had advanced disease and it is a surprise that it was not identified sooner than this when it could have been more amenable to therapeutic intervention.

Most patients are commenced on ursodeoxycholic acid as this is the only drug known to alter the natural history of PBC although it does not prevent chronic liver failure or the need for transplantation. Other drugs that have been used in the past include: colchicine and methotrexate although in randomisd trials they have shown no statistical benefit. [please see UpToDate 15.1 for further details]

It suggested that other causes of liver disease should also be ruled out including Haemochromatosis [ferritin level], Wilson's Disease [caeruloplasmin], and alpha-1-antitrypsin deficiency. Note that copper metabolism is abnormal in PBC and Keyser-Fleischer Rings can occur in these subjects.

An ERCP was recommended to rule out Primary Sclerosing Cholangitis.

Moreover, the patient would have needed a liver biopsy to confirm the diagnosis and stage of liver involvement.

Ultimately, the patient would require a liver transplant.

Other things to consider include checking levels of fat-soluble vitamins including A, D, K [ and E, but not clinically relevant]. Vitamin A deficiency can lead to night blindness in severe disease especially in those with raised bilirubin levels. A prolonged clotting time can be indicative of vitamin K deficiency and vitamin D deficiency can lead to osteomalacia. If levels are low, they need to be replaced.

Malabsorption can lead to steatorrhoea and reduced nutritional absorption. In such cases, either fatty acid intake is reduced and / or pancreatic enzyme substitutes are given.

The patient should have also been considered for a diagnostic-therapeutic upper GI endoscopy as the patient has a high risk for oesophageal varices even in the absence of obvious cirrhosis. Treatment consists of banding varices and reducing portal pressure with non-selective beta-blockers and calcium antagonists.

This case, once again, underlines the importance of obtaining a thorough history and physical examination.

Finally, causes of Hepatosplenomegally should be reviewed here and include:

• Liver disease and Elevated Portal Pressure including PBC

• Infections: Malaria, Schistosomiasis, Toxoplasmosis [cats: social history!], CMV, EBV, Acute Hep B, C infection, Weil's Disease [Rat's urine -- Social History!], Brucellosis [food history part of social history!]
  
• Haematological-Neoplasia: CML, CLL, Lymphoma, Extramedullary haematopoiesis of myeloporliferatice disease e.g. polycythema rubra vera
  
• Granulomatous: TB, Sarcoidosis
• Inflammatory: Amyloidosis
• Vascular: Budd-Chiari Syndrome

Causes of Hyperpigmentation should also be reviewed and include:

• Racial
• Sun Related
• Dermatological: severe chronic eczema, freckles, acanthosis nigricans, chloasma [pregnancy related]
• SunTan Supplements e.g. Tanning Creams (がんがろ！！)
• Tattooing, arsenic exposure, gold, argyria.
• Drugs: chloroquine, chlorpromazine, minocycline, amiodarone.
• Topical Agents: benzoyl peroxide, tretinoin, flurouracil
• Berloque hyperpigmentation: phototoxicity from citrus or celery
• Drug Eruptions: Septrin, NSAIDs, Tetracyclines
• Oral Supplements: Beta-carotenaemia
• ACTH related: Primary Hypoadrenalism, ACTH secreting Pituitary adenoma, ACTH secreting tumours e.g. Ca Lung
• GI related: Haemochromatosis, Primary Biliary Cirrhosis, Wilson's Disease, Whipple's Disease, Hyperbilirubinaemia from any cause.
• Infection: Visceral Leishmaniasis

Collapsing Patients and Drugs

This case has been anonymised for patient safety but provides good examples of investigating collapses in elderly patients.

This elderly female patient was recently admitted to another institution due to 1) symptoms of a chest infection and 2) Collapse.

The patient was demented and was unable to provide any history of what happened. The brief history was provided by the care home.

The patient had recently developed a cough, sputum and fever and had been seen by a local community physician who had diagnosed a chest infection for which levofloxacin had been prescribed.

The patient had been getting better and the sputum had stopped although the patient had developed rhinorrhoea and general upper respiratory symptoms for which a further course of levofloxacin had been prescribed.

The patient had been eating her dinner when she suddenly collapsed at the table with her head falling forwards on to the table. Within a few minutes, the patient had regained consciousness. The patient could not remember anything of the event. There was no description of seizure activity. It was not clear whether the patient had bitten her tongue or developed urinary incontinence during the episode.

Further questions I would have liked to ask include: pre-syncopal dizziness, palpitations, chest pain, sudden onset of headache and visual aura before the collapse. Also, was the patient coughing prior to the syncope (cough syncope), any history of collapse / dizziness on turning of her head (hypersensitivity of baroreceptor response). Any cardiac history such as dysrhythmias, valvular disease, ischaemic heart disease?

Previous history included hypertension and dementia plus borderline diabetes.

Drugs included amlodipine, topical GTN patch, oral cough medication (opiate based I think), and levofloxacin.

The care home did not mention about angina but the patient was clearly taking medication that would be used for her heart!

No known drug allergies.

From the history alone it was convincing that this was a Cardiac Syncope possibly induced by drugs (combination of calcium blocker, nitrate, opiate derivative) plus there is the possibility of Torsade de Pointes from a Long QT interval induced by Levofloxacin especially on a back ground of probable ischaemic heart disease.

Vitals revealed a BP of 151/57 (large pulse pressure), pulse 70 regular, Resp Rate 20/min, Sats 97% on RA. Patient was afebrile.

Pulse examination revealed a Bounding and Collapsing pulse suggesting possible Aortic Regurgitation. JVP was not raised. There was an obvious Carotid Murmur originating from the heart and a Soft systolic mumur at the Aortic area.

Chest examination was normal with no crackles.

Leg examination revealed mild oedema probably as a result of the Amlodipine, which is a known side effect.

Chest Xray revealed calcification of the rib ends which is a normal sign of aging. There was some mild shadowing behind the heart which was consistent with consolidation with current or recently treated infection.

ECG revealed sinus rhythm but with first degree heart block and a corrected QT of 416ms. There were no acute changes.

Urine revealed 4+ bacteria and 2+ white cells.

Bloods showed a raised CRP and white cell count was normal. CK and CK-MB were normal. There was some mild chronic renal failure consistent with the patient's advanced age.

Clinical Impression:

1) Syncope due to drop in cardiac output possibly from combination of hypotensive drugs and opiate-derivative (which causes venodilatation).

2) Possible Torsade de Pointes from Levofloxacin ( a rare but well described phenomenon)

3) Post-pneumonic changes on chest Xray

4) Current Urinary Tract Infection

Suggestions

It was suggested that the Levofloxacin be stopped and continue with the already started Ceftriaxone. The patient coming from a Care Home might have a multi-drug resistant bacterium and hence, urine culturing and sensitivity would be of prime importance here plus blood cultures. Levofloxacin is an excellent drug for treating UTI, but with the urine being positive suggests to me that the infection was not being effectively treated.

It was also suggested obtaining a cardiac echo to ascertain if there was underlying aortic regurgitation because if moderate-severe, it can be dangerous using nitrate drugs as these can cause sudden drops in cardiac output and collapse.

It would also be of advantage to obtain continuous cardiac monitoring or at the very least a 24 hour Holter monitoring to see if a dysrhythmia can be identified.

It was also suggested that if the above tests proved negative the Carotid Sinus Massage (CSM) could be performed to see if there was any underlying hypersensitivity of the carotids to cause sudden heart block and collapse.

This is done with the patient in a recumbent position wired up to a monitor. The patient must not have any carotid stenosis (otherwise stroke might occur!) and hence, it should be done on the side with no murmur. If a patient has bilateral murmurs, then a Carotid Doppler would need to establish if the carotids have any disease and hence, whether it would be safe to perform CSM. If safe to do so, the carotid on ONE SIDE of the neck is massaged with light pressure for 30 seconds to one minute to see if there is any cardiac slowing. If the patient feels unwell during the procedure such as dizziness, then the procedure should be terminated.

If the patient's heart rate slows, then it should be recorded and printed out if possible.

If this test is normal, then a Dix-Hall-Pike test can be performed which involves cardiac monitoring and manouvers angulating the patient. This can reveal underlying problems causing collapse.

Every Breath You Take- Every Move You Make

Professor Lonny Ashworth, MEd, RRT.

Today we had the pleasure of having Professor Lonny Ashworth from Boise State University in the USA attend the hospital to lecture on how to use ventilators.

His visit was brief, just for 4 hours only, with him previously having been visiting a hospital in Chiba.

Today, he hosted two identical practical sessions whereby junior doctors could try Ventilators for themselves to see how CPAP feels, in additon to Square Wave Forms, Assisted ventilation and SIMV to mention but a few topics discussed and tried.

Each doctor got to be a 'patient' on a ventilator with the other doctors changing the controls.

The ventilators used were very hi-tech, the ones that I am used to seeing in the UK.

The junior doctors appeared to enjoy themselves very much and with the help of a translator, were able to understand well.

Here some junior doctors pretending to look studious but really being comedians!

Looks like SCUBA diving but a great lesson in ventilator control !!

No sleeping or time to be intubated and ventilated ! !

During his lecture, Lonny made a joke and said 'Every breath you take....' and I said 'Every move you make....' and he said --- ah no that's a song!!! Yes, from Sting and the Police ! A great respiratory song.

Some panoramic views of the study session....

Lonny, we really enjoyed your session and you are welcome back anytime!

History and Physical Revisited: a neurological quandry

This case has been anonymised for patient confidentiality.

A male patient of advanced age who presented with a sudden history of collapse. The patient apparently did not lose consciousness and returned to his home. Following this, the patient's conscious level began to decrease, and his breathing became laboured and his family admitted him to hospital.

It was not clear exactly what was described when the patient's breathing became abnormal. For example, did respiratory rate increased or decrease? Did the patient develop asthma-like symptoms, was there any pulmonary oedema, cough, sputum, haemoptysis?

Also, there is no history about what happened before the collapse. Where was the patient? What was he doing? Was there a sudden onset of headache? Were there any other features? Was there seizure activity and if so, was it sustained?

With the paramedics attending his home, his GCS was described as 3/15 but on admission to hospital, the GCS had apparently increased to 14/15 and the patient was able to speak and follow commands during examination.

However, following this, his breathing became further laboured with there being identification of hypoxaemia on blood gas analysis and the patient was intubated and ventilated.

The patient had not apparently sustained any head injury but other details of history such as sudden onset headache / chest pain / neck stiff were not elicited.

The previous medical history was that of hypertension only for which he took medication, although on admission, it was not known what medication he was taking.

Vitals signs were slightly abnormal, with the patient being normotensive, afebrile but with a of pulse 100/min and regular.

From what was relayed to me, the CNS examination was relatively unremarkable and the only positive finding on the rest of the physical examination were bilateral crackles at both bases on chest exam.

The patient had had the usual tests including chest Xray and Chest CT which showed old changes which looked like fibrosis, but there was no obvious pneumonia.

CT head showed a very small area of fibrosis near the thalamus on the right side, but this looked old. There was no blood or space occupying lesion (SOL). The MRI scan showed no abnormality apart from some artifact over the left frontal area. The diffusion MRI was otherwise normal.
The vascular scan showed no obvious abnormality.

Bloods revealed a slightly raised BUN of 27 but a normal creatinine. White cell count was slightly raised and CRP was increased to 2.1. CK, CK-MB, Troponin T were all normal. All other usual bloods were normal.

ABG- revealed a normal pH but CO2 and HCO3 were decreased and the SpO2 was approx. 50mmHg consistent with a compensated respiratory alkalosis and hypoxaemia.

The team were concerned that a pneumonia may be causing the chest symptoms but were unable to ascertain the cause for the collapse and coma, especially as the examination and tests had been normal.

My Opinion.......

On hearing this rather brief history, the differentials that come to mind include:

• Subarachnoid haemorrhage -- initial collapse from bleed and then as vascular constriction ensues, and intracranial pressure increases, the patient can become comatosed from ischaemia. Some SAH cannot be picked up on CT and only a Lumbar puncture performed, usually after 18 hours will pick up Xanthochromia.

• Stroke: a sudden onset phenomenon with sudden loss of neurological function either from infarction or bleeding. When considering thrombotic stroke, one should remember that thrombo-emboli can originate from the heart chambers, the valves, myxoma, platelet rich emboli from the carotids and primary thrombosis from atherosclerotic plaques. Paradoxical embolus and stroke can occur from a patent foramen ovale giving a right to left shunt through the atria. Emboli can pass from right to left cardiac circulations and cause stroke! This however is very rare. Patients may regain some level of consciousness but this may deteriorate with cerebral oedema.

• Sudden Cardiovascular Collapse: Loss of cardiac output for any reason such as a dysrhythmia, can lead to collapse and patients may suffer cerebral hypoxia and brain damage or head injury. With the latter, injuries to the temporal area can cause laceration of the middle meningeal artery and tense extradural bleeds and reduced consciousness.

• Dissection: Dissection can lead to acute collapse from sudden loss of valvular competency and / or tamponade, and if there is a proximal dissection, it can occlude the arteries supplying the head and neck leading to stroke.

• Pulmonary Embolism: Large PEs can cause sudden collapse and unconsciousness due to sudden cardiovascular compromise. PE can be chronic in nature and cause fibrotic change. Hypoxaemia from a massive PE can lead to hypoxaemia and reduced conscious level.
  

From the scans and data, there was neither an obvious large stroke nor an SAH. There was no evidence of a dissection or head injury either. The CT chest, which showed contrast imaging of the pulmonary vessels, did not reveal any PE.

Further results revealed a normal CSF examination with a slightly raised protein level only.

Urine results revealed no sugar but two plus of ketones (probably explained by fasting state)
Blood sugar approx 210. Could have this been an undiagnosed DKA with respiratory compensation??? I think unlikely in view of the absence of glucose in the urine.

Examination

Examination of the patient was difficult due sedation from propofol. However, at the end of the bed, the patient was making some unusual movements of adducting his shoulders and turning his wrists slightly inwards. He also had twitches which I thought might be sub-clinical seizure activity.

On examining him, his pupils were approx 4mm bilaterally and responded rapidly to light and were hence intact. Response to pressure on the nailbeds on both hands revealed Extension to Pain. Babinski responses were bilaterally positive as was Achille's Tendon Pinch which also causes plantar extension. Just to ensure that this was not just a withdrawal response, the patient was tested with a noxious stimulus, which in the case was a blunted pin. A normal response should be to plantar flex away from the pin tip when applied to the dorsal aspect of the toe. A positive response is dorsiflexion towards the pin tip. This patient showed bilateral Positive Signs confirming bilateral Upper Motor Neurone impairment.

Chest examination revealed bilateral crackles with them being of higher intensity of the right side.

Clinical Impression

Following this most revealing examination, it showed that the patient actually had developed severe neurological signs. With the signs being Bilateral this suggested that both cerebral hemispheres had probably become involved.

With the patient Extending to pain, this is a decerebrate condition. However, his pupillary responses were spared and when examined by a very astute neurologist, the Doll's Eyes reflex was also normal suggesting a normal midbrain.

Hence, a definite abnormality was identified which could now account for some of the features of the history.

The basic neurological examination helped reveal the problem that no scan could identify.

This underlines the importance of a good history (where possible from the patient / family /friend /bystander) and detailed physical examination.

Please remember that detailed physical examination should be able to reveal gross physical abnormalities. When performing a neurological examination on a conscious or unconscious patient, the Babinski Response can be of paramount importance. There are many other tests for finding upper motor neurone signs such as Hoffman's Sign in the fingers and many other in the feet which can be found in any good neurology textbook.

Don't forget to check for Clonus as more than three beats is signifcant og UMN signs.

Don't forget the Reflexes. It does not take long to do but can reveal the possible level of the problem and determine absent, decreased, normal or increased reflexes, which are all significant of either normal or abnormal neurological states.

Don't forget to do sensory testing in the conscious patient as this again can determine the level of a lesion.

This patient had a repeat scan but again this was normal.


After discussion with the neurologist, it was suggested that the clinical features and a normal set of scans with 24 hours of admission could be accounted for by:

• Diffuse bilateral hypoxic-ischaemic damage of the cerebral hemispheres

• Multiple embolic phenomena

Hence, the history of sudden collapse and waxing-waining consciousness might be accountable from a cardiovascular cause leading to cerebral ischaemia or multiple infarction.

Bread and Butter

Today's case is a time to consider reviewing common murmurs and how to identify a pleural effusion in a patient with heart failure.

This is a real case but I have as always anonymised the case. All pictures are self drawn so please excuse my poor artistic skills!

This elderly patient presented to another hospital with symptoms of fatigue, appetite loss and dyspnoea occurring over 4 days.

She was normally quite well and had just been on a recent trip within Japan. However, the patient began to feel weak and had difficulty walking far, needing to frequently rest. She also began to lose her appetite and prior to admission the patient was noticed to have difficulty breathing although the patient denied being breathless.

When seen, the patient admitted to being only able to walk no more than 100 metres before needing to rest. She rested normally for up to 2 minutes but had no chest pains. The patient had recently needed to prop herself up in bed with pillows to feel comfortable. Again, the patient denied being breathless!

The patient denied having a fever, cough, sputum, haemoptysis. There was no recent history of a common cold. Although the patient's spouse had said the patient had lost her appetite, the patient said there was no problem!

There was no history of any abdominal problem. No Genitourinary problems.

The patient was known to have Sick Sinus Syndrome for which the patient had a biventricular pacemaker, some mild dementia, and type 2 diabetes mellitus for which she took insulin.

Drugs included warfarin, digoxin 125mcg, donezapil and insulin.

There was a history of heavy alcohol use in the past but the patient had stopped drinking several years before and she still smoked 12 cigarettes per day.

When examined, the following findings were seen:

On inspection the patient looked relatively well.

BP 159/95, pulse 76 regular, Afebrile, Sats 97% on room air, Resp rate 30/min.

Hands showed some fine tremor and there were red palms. There was some early clubbing and no Quinke's Sign. There was no liver/ CO2 flap [asterixis]. Sclerae looked yellow. The tongue was blue tinged consistent with central cyanosis.

JVP was pulsatile and raised above 4cm. The Carotid was evident at the neck and pulsatile (Corrigan's Sign). Precordial examination revealed a left parasternal heave consistent with RV hypertrophy. Aortic area revealed a grade 2/6 systolic murmur with no diatolic component audible radiating to the carotids in the neck (aortic systolic murmur; probable aortic stenosis). The Apex revealed a 3/6 mitral pansystolic murmur radiating to the left axilla. Both murmurs increased in loudness on expiration.

Interestingly, there was a murmur in the tricuspid area (right side of the xiphoid process) and with slight radiation over the liver area consistent with tricuspid regurgitation. There was no pulsatility of liver as can be sometimes seen in TR (please see diagram 2).

Respiratory examination revealed 'stony' dullness at the left base. Vocal Resonance was reduced compared to the right side [ask patient to say 'hitotsu' whilst listening over the area of dullness and compare to the normal side; in effusion the sound decreases; in consolidation the sound increases] and the breath sounds were decreased over the area of dullness. These findings were consistent with a pleural effusion. There were also crackles on the surface of the effusion and at the right base (see diagram 3)

Abdominal examination was normal.

Lower limb examination revealed no oedema or evidence of deep vein thrombosis.

Clinical Impression:

1) Congestive Heart Failure

2) Mixed Valvular Disease (Mixed AS and AR, TR and MR)

3) Right Ventricular Hypertrophy

4) Left sided Pleural Effusion

5) Possible thyrotoxicosis / liver disease (tremor and red palms)

6) Possible recent MI (decompensated Heart Failure and possible silent event due to diabetes)

7) Possible alcholic cardiomyopathy (history of heavy alcohol use)

Chest Xray (diagram 1) revealed severe cardiomegally and upper lobe diversion (bat's wings or Butterfly shadow) and fluid in the horizontal fissure on the right. The left costophrenic angle was obscured [please don't refer to it as 'dull'; dull is a sound not the description of an X-ray shadow!! Pictures don't make sounds]

The ventricular pacing leads did not reach the apex suggesting that either the heart was normal size and surrounded by a large pericardial effusion, or the heart has enlarged significantly in the 15 years since the pacemaker was put into the patient.

ECG showed a single pacing spike and a wide QRS complex consistent with being paced.

Bloods showed a normal Neutrophil Count, Normal Haemoglobin, Slightly raised BUN but normal creatinine. Liver Function was normal. INR was 1.29 and therefore subtherapeutic. CK was 66 and troponin T was Negative. CRP was raised at 2.1 and monocytes were mildly raised making me suspicious of a possible viral aetiology for his decompensated cardiovascular status.

Blood gas revealed a respiratory alkalosis and hypoxaemia.

Hence, AMI had been ruled out here but not thyroid disease.

Echocardiogram revealed an ejection fraction of 29%, severely thinned LV wall, hypokinesis, 3rd degree TR and MR. There was 1st degree AR but no Aortic stenosis component. There was no pericardial effusion.

Sometimes the increased cardiac output associated with AR can cause a Flow Murmur that can be easily confused with AS as in this situation.

Hence the diagnosis here from history and physical was not altered significantly by other tests.

It was decided not to attempt removing the pleural effusion on the left as firstly it was relatively small, the patient had not had a long challenge with diuretic therapy, the patient was taking warfarin and the patient's heart was so large there was a risk of puncturing the ventricle.

The treatment suggested was 1) Furosemide 2) ACE inhibitor therapy

ACE-I therapy improves cardiac function by remodelling the myocardium and reduding hypertension, and it is also known to reduce hospital admissions. It is also protective of the patient's kidneys especially as she is diabetic. However, always be cautious with ACE-I / ARB / Aldactone therapy as it can worsen creatinine and cause hyperkalaemia. On starting an ACE-1 /ARB it is acceptable to see a rise in creatinine of up to 30%.

The funny thing was that I have NEVER done a CT scan for someone who presented with pure heart failure as history, examination and a simple chest Xray usually suffice. If an effusion is considered then an ultrasound is sought or even a diagnostic needle aspiration.

The CT showed all the features already found by other methods but two metallic objects were noted which were the pacing leads causing interference of the image on the CT scan!!

Lumbar Punctures-- Advice from B

If you already know how to do a perfect lumbar puncture (LP) procedure then great!! However, many first year doctors both here and in the UK have great difficulty in performing this procedure, in some cases because they have not had much experience and also tuition may not have been optimal.

The following is my way of performing an LP that should hopefully help junior doctors get it right in the end.

Firstly, does the patient actually need a lumbar puncture?? Is meningitis suspected or an SAH?? Does the patient have raised intracranial pressure. You should be checking for raised intracranial pressure in such circumstances by monitoring pulse and blood pressure looking for a Cushing's Reflex (low pulse, high blood pressure) and looking for papilloedema in the fundi and of course any NEW focal neurology.

If uncertain, then CT scanning should be performed to obtain images to try and rule out raised ICP.

If the patient does have raised ICP then you should not do an LP because by decompressing the pressure within the subarachnoid space from below with an LP needle can precipitate cerebellar-brainstem herniation and you then have a decerebrate patient!!

If you have a meninigitis case, then treating with antibiotics is the most important thing you can do, and if you want to get confirmation, then this can be done through obtaining blood cultures, throat and nasal swabs immediately, and PCR of CSF at a later date once signs of pressure have reduced.

So, if the patient does not have raised ICP it is safe to proceed with an LP unless the patient is on warfarin. An INR of less than 1.5 is usually safe for doing proecedures. If raised above this, and it is essential to do an LP, then the INR can be reduced by FFP in the acute setting.

I do not consider aspirin or other anti-platelet agents to be an absolute contra-indication as although they prolong the bleeding time, they do not affect the intrinsic and extrinsic coagulation pathways. More over the risk to benefit ratio tips towards doing the procedure in such scenarios.

When you have decided to do an LP please try and explain the procedure to the patient / family to obtain their permission -- this is called Informed Consent. It is necessary to explain that the procedure may feel uncomfortable although you should be using local anaesthetic in any case. You need to explain the side effects of the procedure including headache and the possibility of bleeding and introducing infection, although in my experience, this is rare. Also explain that the patient will need to usually lie flat for about 4 hours after the procedure.

Set up your tray with sterile drapes, iodine, 1% or 2% lidocaine (10ml), several LP needles, normal sized needles, a 10ml syringe, sterile gloves, a Manometer (for CSF pressure), three sterile tubes for CSF, a marker pen (not on the sterile tray), swabs and dressings.

1) Lie the patient in a Lateral position on either their Right or Left Side with their back parallel to the edge of the bed. Ask them to curl into a Foetus position-- hips and knees flexed with the knees as close to the abdominal wall as is possible. This will open the intervertebral spaces by flexing the spine.

2) Feel the top of the pelvis and then feel down to the lumbar spine. The vertical imaginary line will dissect the L4 vertebra-- this is the Intercristal Plane. Below this is the L4 intervertebral space and above is the L3 space.

3) Wherever is the most easiest place to enter use a marker pen to identify the site you wish to enter.

4) Now WASH YOUR HANDS and then put your sterile gloves on

5) Then put a sterile drape underneath the patient

6) Now, sterilise the skin with iodine in the area you want to put the needle into. Remember to also sterilise a wide field as you may have to go up higher if the space you have chosen does not work. Also, I sterilise the top of the hip and loin area so that I can find the Intercristal Plane and re-establish my land marks during the procedure. However, you may have drapes to allow just access to the designated area so you can re-establish your markings in any case.

7) You must use Local Anaesthetic during the procedure. Use 1% or 2% Lignocaine (lidocaine) and infiltrate the skin and deep muscle with a normal needle. Usually instill about 5 ml and wait until the patient cannot feel the prick of the needle on the skin. REMEMBER to ALWAYS draw back on the syringe before injecting local anaesthetic to look for blood or even CSF, because inappropriate entry into a vein or subarachnoid space can cause patients to have a seizure or cardiac dysrrhythmias.

8) Then, take your LP needle and angle the needle in the head direction and towards the umbilicus NOT perpendicular to the skin, otherwise you will hit bone. If you hit bone, then pull back and re-angle the needle upwards and advance again. If you keep hitting bone then STOP and try at a higher interspace, again have instilled local anaesthetic.

9) Once you advance the needle into the correct space, you will notice that it can go in quite deeply. You should feel some resistance to the needle and then a sudden loss of resistance that is called 'the Give'. You should now be in the subarachnoid space.

10) Now, withdraw the middle stylet of the needle and wait for the CSF. It may not come out immediately but with respiration the increased in CSF pressure should cause CSF drops to come.

11) You should now put on a MANOMETER to check the pressure in cm/mm of Water. A normal pressure is between 10-20cm of water.

12) Now take your samples of CSF, usually 10 drops, into three bottles. Label them up as 1,2 and 3 with number 1 being the first sample and number 3 being the final sample.

13) Normally 1 and 3 are sent for microscopic examination to compare white and red cell components. It is also examined for bacteria.

14) The 2nd bottle is sent for protein and glucose examination.

15) The stylet should then be reintroduced down the barrel of the LP needle and then the whole needle is removed. This is supposed to reduce post-LP headache by aiding sealing off of the dural hole created by the needle.

16) Press firmly over the entry site with a swab until any bleeding / swelling subsides and then apply a sterile dressing.

17) In my experience, the patient should lie flat for several hours although a recent JAMA 2006 article suggests that patients can ambulate soon after such a procedure. If the LP fails to produce CSF then a higher interspace level can be chosen such as the L3/L4 interspace or the L5/S1 space can also be chosen instead.

Sometimes it is necessary to do a Sitting LP. In this case the patient is sat on the edge of the bed and the doctor performs the LP from behind. The same interspace areas can be entered. This can sometimes be the only way to obtain the CSF.

Of course, if you have difficulty with a lying LP you MUST ask your Senior doctor to help you rather than repeatedly attempting the procedure.

I hope this helps and please let me know your experiences with LP procedures. I may not have covered every single problem with LP procedures here as it is a basic guide, and if you want to ask me a specific questions then please leave an anonymous entry on the blog and I will answer as soon as possible.

Kwashiorkor--no way!!

The following case has been anonymised for patient confidentiality.

A few months ago a female patient was seen at a peripheral hospital in her 30s who was admitted with weight loss over several years. She denied all symptoms including loss of appetite, fevers, night sweats, cough, sputum, abdominal pain and diarrhoea.

However, on direct questioning she admitted to having some recent constipation, easy satiety with little food and leg swelling.

During her admission she was noted to have recurrent hypoglycaemia which occurred when she was taken off of the intravenous glucose infusion. During hypoglycaemia she developed dizziness and pre-syncopal symptoms but no sweating, shaking or hunger.

She was noted to have abnormal liver function but she denied drinking alcohol and had no history of risks for hepatitis B or C infection.

Her periods had stopped suddenly several years before, but before the weight loss, she admitted to only ever having 3 periods a year.

She had been investigated previously with a gastroscopy and colonoscopy to rule out malignancy and all had been normal.

Previous medical history was otherwise unremarkable.

When I reviewed this patient she was extremely low in body weight being only 30kg. She had diffuse muscle wasting and temporalis muscles were wasted.

She had cool hands and her nails showed Koilonychia (spoon nails) consistent with Iron Deficiency Anaemia. Her pulse was regular and approx 60 per minute. Her skin was thin and flakey and there were signs of multiple bruising all over her body. The knuckles of her hands showed callosities and her teeth showed some mild dissolution.

JVP was not raised. There was a normal thyroid on palpation and no evidence of lymph nodes.
Heart sounds were loud but no murmur was evident.

Chest examination revealed intercostal recession and percussion was difficult due to the patient's frailty. Chest sounds were normal with no signs of crackles or wheeze.

Abdominal examination showed a thin abdomen and on percussion the liver edge was below the costal margin by approximately two fingers. The liver was non-tender. There were no signs of chronic liver disease and bowel sounds were increased.

Legs were extremely oedematous and leaking. She had sores on her knees.

Clinical Impression

1) Anorexia nervosa (extremely emaciated, callosities on he hands, dissolving teeth, cool peripheries, oedema, secondary amenorrhoea, hypoglycaemia)

2) Likely multivitamin deficiency including signs of Scurvy (fragility of capillaries leading to multiple bruising)

3) Hypoproteinaemic state

4) Possible early liver cirrhosis due to malnutrition resulting in abnormal liver function and recurrent hypoglycaemia ( impaired gluconeogenesis, glycogenolysis and impaired glucogenesis)

5) Possible Iron Deficiency Anemia as a result of decreased nutritional intake.


Blood results revealed a low insulin and C-peptide level, borderline hypothyroidism, an albumin of 3.1, negative anti-insulin antibodies. Cortisol collection result was above normal with a high ACTH level. FSH and LH levels were decreased. GH level was normal.

Abdominal Echo showed some ascites but a normal pancreas. CT showed no evidence of malignancy. Cardiac echo revealed some mild pericardial effusion.

Suggestions

It was suggested that the insulin and C-peptide be repeated during an episode of hypoglycaemia and for a glucagon level to also be drawn. Moreover, I also suggested that urine sulphonylurea levels be checked to rule out self-administration.

The patient might have been developing cirrhosis due to severe malnutrition and hence, a liver biopsy would be an approprite next step.

In view of the severe low body weight and hypoglycaemia It was also suggested that the patient should have NG or PEG feeding as it might be the only way to ensure that the patient had sufficient and guaranteed calorie intake especially as the patient had shown signs of self-induced vomiting.

In view of the probable multi-vitamin deficiency, the patient would need replacement including ascorbic acid (Vitamin C).

This patient saw nothing wrong with being so under weight and often patients consider that they are over weight when they are fact severely under weight and hence, she might have an abnormal recognition of self-image. A psychiatric opinion would also be helpful although congnitive retraining can be difficult especially in older patients, and from the history of infrequent periods, I suspect that she has had an eating disorder most of her life.

Unfortunately, the secondary amenorrhoea, hypothyroidism and other features of this illness only abate when sufficient weight has been put on.

The patient clearly had anorexia nervosa and from the severe features of the disorder including hypoglycaemia, oedema, ascites and moderate liver dysfunction It was considered that she might have Kwashiorkor.

This disorder is seen in states of severe protein malnutrition and the features are the same as described in this patient. Anorexia nervosa is a recognised cause of Kwashiorkor.

With restoration of proper nutrition the features are reversible except those of liver disease if cirrhosis has developed.

This was an extremely interesting case for me and the history, physical and blood tests gave me the answer!

For thinking of causes of Hypoglycaemia, remember EXPLAIN.

EX- Exogenous e.g. alcohol, insulinl, sulphonylureas
P- Pituitary failure
L- Liver failure
A- Adrenal Failure
I- Immune e.g. insulin receptor antibodies
N- Neoplasia (rare e.g. insulinoma, haemangiopericytoma, retroperitoneal fibrosarcoma)

Always rule out drugs by taking urine screens, insulin levels with C-peptide.

For a more indepth explanation of hypoglycaemia and its investigation, please see a more detailed text.

Nasty Pneumococcus

This case has been anonymised to protect patient confidentiality.

A 60 year office worker presented to his local clinic with a fever and symptoms of a common cold.

He was provided with some antibiotic treatment but over the next few days, his status declined further with headache and pain in the back of his neck. He also developed some intermittent hearing loss that progressed to absolute deafness on the day of his admission.

On admission he was unable to hear but was able to express his pain.

Examination on admission revealed a temperature of 36.4 degrees, pulse 90, BP 128/70, respiratory rate of 20/min, SpO2 of 96% on 6L oxygen

CV examination was normal.

Resp examination showed reduced excursion of the chest on the Left side, dullness and reduced breath sounds. Crackles were audible only at the Left base.

Abdominal examinatiom was reported as normal except for a slightly palpable liver edge which was apparently tender.

CNS examination was reported as all normal including No Neck Stiffness and Negative Kernigs signs.

However, he had complete deafness albeit that Weber's and Rinne's tests had not been attempted to determine if it was conductive or sensorineural deafness, although with such a rapid history and bilateral hearing loss, it is likely to have been sensorineural hearing loss.

Bloods revealed WCC 12, Differential low neutrophils and low Lymphocytes. CRP was over 30. BUN 16 creat 0.76. Liver function showed slightly raised bilirubin of 1.9, AST 90, ALT 80, Alb 26. INR 1.3. FDP / D-Dimer had not been done.

Lumbar Puncture showed an opening pressure of 50cm H2O, WCC of 9, 8 were Lymphocytes and 1 was a PMN. Protein was over 150 and Glucose of ZERO. Diplococci were seen on gram stain.

Blood cultures grew Streptococcus pneumoniae.

CXR revealed a severe left upper lobe pneumonia with there being some smaller right sided perihilar consolidation as well.

CT head showed dilated lateral ventricles but no cerebral matter effacement.

When reviewed, the patient's family were able to provide a wealth of information to fill in the history, which I have included above. He had recently visited an onsen but he had had no foreign travel. He had been a smoker of 3-4 cigarettes per day, but he had stopped 6 months previously.

On examination, the patient appeared severely unwell and he appeared in pain.

His eyes were screwed up and he was cold and sweaty despite being on a warm ward.

CV and Resp exams were as above BUT Respiratory Rate was increased to 30.

Abdominal exam revealed a large, tense bladder which was stony dull to percussion and a dull liver edge on percussion. Traub's Space was also dull suggestive of splenic enlargement although no spleen was palpable.

CNS examination revealed spontaneous eye opening, localisation to pain but no speech (GCS= 10/15). Pupils were consticted to approx 3mm equally but fundoscopy was not possible due to the pupils being small, continuous eye motion, intermittent eye closure of the patient and cataracts.

The patient clearly had neck stiffness as he winced on having his neck turned and a similar situation occurred on testing Kernigs making it Positive.

Reflexes were increased in the upper limbs but completely absent in the lower limbs although the patient appeared to have power to withdraw in both upper and lower limbs. Plantar reflexes: left foot normal, right foot equivocal. Other ways to elicit the plantar reflex such as achilles tendon pinching and flicking the medial malleous were Negative.

Skin revealed no rash.

Clinical Impression:

Having considered the clinical case and physical findings with lab data the following diagnoses were considered:

1) Pneumococcal Pneumonia

2) Pneumococcal Meningitis with Secondary Communicating Hydrocephalus due to probable Arachnoid Villi blockage in the Superior Sagittal Sinus from the infection. The possibility also exists of superior sagittal sinus thrombosis.

3) Bilateral nerve deafness due to pneumococcal meinigitis and hydrocephalus.

4) Liver dysfunction from sepsis / DIC / ? underlying liver disease

5) Pneumococcal Septicaemia and possible DIC

6) Urinary Outflow Obstruction possibly due to severe illness / autonomic dysfunction / Reduced level of Higher Function due to meningitis and hydrocephalus

Suggestions

It was suggested that the antibiotic therapy be continued with Ceftriaxone 4g/day and Vancomycin, although it was not entirely convincing that intravenous Erythromycin that was also commenced would be effective especially as this antibiotic is Bacterostatic and Not directly Bacterocidal.

The patient was also given Dexamethasone which again is the correct treatment in such circumstances.

It was advised on passing a Foley urinary catheter and measuring hourly urinary output especially as he had a systemic illness.

The patient clearly had developed a System Inflammatory Response Syndrome (SIRS).

It was also advised that the neurosurgical team should be contacted for advice as the patient might require a ventriculoperitoneal shunt if the raised pressure continued to be a problem.

In view of the raised INR and abnormal liver tests and with DIC having not been excluded it would not have been safe to consider heparin for possible superior sagittal sinus thrombosis and this would have need an MRI scan first.

I suppose the lessons to be learnt here are:

1) Get as much history as you can from family if the patient can't communicate

2) Don't rely on observations taken on admission as they can change and deteriorate; DO YOUR OWN OBSERVATIONS TOO WHEN YOU REVIEW THE PATIENT.

3) Look at your patient's eyes to see if they are in pain when examining as in this case the neck stiffness and Kernig’s that were 'negative' were in fact Positive.

4) Always consider catheterising the severely sick patient as it is essential to relieve bladder pressure and monitor urine output as it is a very sensitive way to determine deterioration in renal function

5) Always consider putting Meningitis patients on an HDU/ICU for intensive nursing care

6) Always think of using Steroids in such cases; the evidence these days suggests using steroids.

7) Remember that patients can develop DIC, so obtaining FDPs, D-Dimer and a Blood Smear looking for MicroAngiopathic Haemolytic Anaemia (MAHA) may provide useful clues.

Update: The organism was later found to be a Penicillin Sensitive Streptococcus pneumoniae.

It was then suggested to stop the Vancomycin in view of the organism being a PSSP and also worsening renal function. It was also suggested to switch from ceftriaxone to Benzylpenicillin- the best therapy in such instances.

Moreover, the patient's conscious level decreased perhaps in part due to hypernatraemia that developed but also from a possible superior sagittal sinus thrombosis. MRI and MRV were suggested imaging to be obtained.

DIC was excluded in the end, and in fact, the liver function initially worsened before beginning to improve.

The patient did eventually make an adequate recovery but required a VP shunt due to worsening pressure and underwent physiotherapy.