115 - miscellaneous 7 bits

A 40-year-old farmer presented to Casualty with a 24-hour history of fever and increasing confusion. On examination he was febrile 39.5ºC. A generalized erythematous rash, covering most of his body, was observed. He also had a paronychial infection of his right index finger, with lymphangitis extending caudally and with axillary lymphadenopathy. His heart rate was measured at 120 beats per minute with a blood pressure of 80/60 mmHg. What is the most likely diagnosis?

a-Hantavirus infection
b-Leptospirosis
c-Orf
d-Staphylococcal toxic shock syndrome
e-Stevens-Johnson syndrome

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d is the answer .

The history is typical of staphylococcal toxic shock syndrome (TSS) – shock, fever, confusion and rash. The primary source of infection in this case is the paronychia of his right index finger. Hantavirus infections (viral zoonoses transmitted via rodents) typically have two distinct presentations: either as a haemorrhagic fever with renal failure or as an acute pulmonary syndrome. The former manifestation is commonest in the far east and eastern Europe, while the latter is the predominat form in the southwestern United States and South America. Leptospirosis does not typically cause a rash and is often associated with jaundice; leptospirosis would not explain the lesion on his finger. Orf, a zoonotic infection caused by a pox virus, presents with painless ulcerated lesions on the hands of farmers, but does not fully explain this clinical picture. Stevens-Johnson syndrome typically starts with an erythema multiforme-type rash that spreads widely and involves the buccal mucosa and conjunctivae; often caused by antibiotic therapy, but does not fit the clinical picture presented here.

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A publication reports the outcome of a new statin therapy in a placebo controlled primary prevention of ischaemic heart disease in a diabetic population. 1000 patients were randomised to receive the new therapy and 1000 allocated to placebo. The study was completed over a five year period. In the placebo group there were 150 myocardial infarcts and in the group treated with the new statin there were 100 myocardial infarcts. What is the number needed to treat to prevent one MI over the course of this study?

10
20
30
40
50

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20 is the correct answer

You treat 1000 patients for 5 years with the new statin and prevent 50 MIs. Thus the Number Needed to Treat (NNT) to prevent 1 MI is 20 (1000/50). Again, this statistical figure provides us with a lot of information regarding the efficacy of a treatment rather then purely statistically significant data. As it stands, you may expect to prevent an infarct by treating as few as 20 patients over 5 years from this data. Furthermore, cost economic data can be calculated from such results by factoring in how much the drug costs against all the costs of treating and rehabilitating a patient with an MI.

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Percutaneous transluminal coronary angioplasty (PTCA):

True / False
has proven benefit in symptom relief - ---true
The treatment of choice for significant left main stenosis----false
is contraindicated in unstable angina-- false
produces endothelial disruption in the treated vessel ---true
General anaesthesia should be used.--- false

Percutaneous transluminal coronary angioplasty is mainly for symptom relief rather than for its prognostic benefits. CABG is the treatment of choice for left main stem stenosis. Almost always done under local anaesthesia.

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A 26-year-old woman with a history of epilepsy presents with numerous episodes of tonic-clonic seizures over the last four hours. She has not regained consciousness at any point during this time according to an observer.

1. What is the diagnosis?

Status epilepticus

2.List TWO urgent investigations that you would perform.

(max 4) Glucose (2), blood gases (2), U&E (2), Calcium (2), FBC (0), ECG (1), anticonvulsant levels (2), toxicology screen (2), lumbar puncture (1), culture blood/ urine (2), EEG (2), CT (2), pregnancy test (2)

3. List TWO intravenous therapies you would consider for this patient.

(max 4) lorazepam (2), diazepam (2), phenytoin (2), dexamethasone (2), Magnesium (2), (thiamine (0), fluids (1),

Status epilepticus is a medical emergency, where two or more seizures follow each other without regaining consciousness, or when one seizure lasts for 30 minutes or longer. There is a risk of cardiorespiratory failure and death if grand mal seizures are involved. In known epileptics, several factors such as abrupt withdrawal of treatment, alcohol abuse or poor compliance may precipitate status epilepticus. However, more than half of cases occur in people with no history of epilepsy. Of these, many are due to eclampsia in pregnant women.

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A 20-year-old man presented to hospital two days after returning from visiting his family in Bangladesh. Within a day of his return to the UK, he suddenly developed profuse watery diarrhoea. He says there had been an outbreak of diarrhoea in his family's village in the week before his return.

Stool culture revealed a growth of Vibrio cholerae.

Which one of the following blood types is associated with the greatest susceptibilty to severe cholera

Blood Group A
Blood Group AB
Blood Group B
Blood Group O
Rhesus -ve

ANSWER IS D - blood group O

"Individuals with blood group O are more susceptible than other individuals to severe cholera, although the mechanism underlying this association is unknown.

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The following conditions tend to improve spontaneously:

True / False
Breast milk jaundice- TRUE
Erythema toxicum - TRUE
Napkin candidiasis - FALSE
Ranula - TRUE
Stork mark - TRUE

Napkin candidiasis requires treatment with nystatin or miconazole cream. Breast milk jaundice is prolonged unconjugated hyperbilirubinaemia in the absence of identifiable disease in a breast-fed infant, and rarely requires treatment. Erythema toxicum and stork marks fade with time. A ranula (little frog in Latin) is a small gum cyst that does not require treatment.

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most appropriate treatment for the following cases who present with earache.

1-A mother is concerned regarding the incessant screaming of her 24 hour old female infant. On examination, the baby has a temperature of 37.8C and has an inflamed right tympanic membrane.

---- The correct answer is Cefotaxime I.V

----- The neonate has features suggestive of otitis media with an inflamed tympanic membrane. In newborns, gram-negative enteric bacilli, particularly Escherichia coli, and Staphylococcus aureus cause -------------------suppurative otitis media. The most appropriate treatment for the neonate is IV cefotaxime to ensure appropriate coverage of these organisms that may be resistant to penicillins.

2-A 12-year-old boy presents with earache following an air journey a week ago. On examination the drum is not inflamed, but fluid is noted in the middle ear.

---The correct answer is Paracetamol

The 12-year-old boy with earache has features of serous otitis media and requires only simple analgesia with paracetamol.

3-A 3-year-old boy is brought to clinic with a history of tugging at his ear. He has a temperature of 36.7C and examination reveals a red inflamed ear canal but the eardrum cannot be visualized due to pain.

--------The correct answer is Topical gentamicin

The 3-year-old child has otitis externa with ear pain and redness of the canal preventing visualization of the eardrum. He requires topical antibiotic therapy which should be adequate together with analgesia. ----------risk factors associated with otitis externa include swimming and cleaning of the ears with cotton wool buds.

4- A 3-year-old girl presents with a two day history of fever, nausea and vomiting. On examination she has a temperature of 39.5C and her left tympanic membrane appears red and inflamed with fluid behind it.

---- The correct answer is Amoxicillin I.V

The 3-year-old with fever, vomiting and nausea again has otitis media and should be treated with parenteral antibiotics. Amoxicillin is regarded as the 1st line treatment for this condition.

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Which of the following is correct concerning cystic fibrosis


a-Is an autosomal recessive condition
b-Has a gene frequency in the population of 1:22
c-the mutation responsible for CF is found on chromosome 7
d-The commonest pathogens responsible for respiratory disease are Pseudomonas and Staph. aureus .
e-Infection with Burkholderia Cepacia is highly transmissible.

all are correct .

Cystic fibrosis is an autosomal recessive condition, with the most common mutation being deltaF508 located on chromosome 7 (Gene map locus 7q31.2). It is one of the commonest mutations in the western world with a gene frequency of approximately 1 in 22. It is associated with meconium ileus, pancreatic dysfunction, recurrent respiratory tract infections and infertility in males to name but a few. Recurrent and chronic respiratory tract infections in patients with cystic fibrosis results in progressive lung damage and is the primary cause of morbidity and mortality. Infections are usually caused by Gram-negative organisms especially the pseudomonads including Pseudomonas aeruginosa. Burkholderia cepacia, has emerged as a pathogen in patients with CF that may lead to rapid deterioration of lung function. Infection with B. cepacia may be highly transmissible in CF patients and epidemics have been described in a number of CF centres. Staph. aureus is also an important pathogen in children.

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In a 2-year-old boy, which of the following conditions are likely to require surgery?

True / False
Bow legs - FALSE
In-toing - FALSE
Knock knees - FALSE
Metatarsus varus - FALSE
Tibial torsion - FALSE

None of these conditions are likely to require surgery, though it may be needed in the occasional extreme case of bow legs e.g. that associated with long-standing rickets.

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A 3 month old boy presents with a 3cm cavernous haemangioma behind the right ear.

Which is the best advice to give the parents?


A.He should be referred to the Plastic Surgeons for surgical removal.
B.He should receive 2 mg/kg of prednisolone for 4 months to shrink it down
C.He should receive alpha interferon injections
D.He should receive intralesional triamcinolone injections
E.It is likely to enlarge to about a year of age, then disappear over the next 5-8 years.

ANSWER IS E .

These lesions usually regress spontaneously. Interventions are used where the lesions affect facial features or vital structures (e.g. larynx, vision).

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For each scenario choose the most appropriate first-line therapy.

AAmoxicillin PO
BBudesonide via nebuliser
CCefuroxime IV
DCo-amoxiclav IV
EDexamethasone PO
FFibreoptic bronchoscopy
GIntubation
HOxygen via facemask
ISalbutamol via nebuliser
JSalbutamol via spacer

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A 13 month old boy is awoken from sleep with breathing difficulty and presents to A&E. He has had coryza for 2 days. On examination he is crying, has marked stridor and a barking cough. Oxygen saturations are 95%.

The correct answer is Dexamethasone PO

The 13 month old boy has viral croup, which is best treated with oral dexamethasone.

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An 18 month old boy develops stridor and cough. This came on suddenly whilst he was playing in the kitchen. Oxygen saturations are 93%.

The correct answer is Fibreoptic bronchoscopy

The 18 month old boy has probably inhaled a foreign body. This may be radio-opaque on chest X-ray or cause a ball-valve effect (hyperinflation on one side of lung fields on chest X-ray). Fibreoptic bronchoscopy should be done to remove it.

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A 14 month old boy presents with cough and wheeze. He has had a cold for 2 days. On examination he has a respiratory rate of 60/min, with marked recession and audible wheeze. Oxygen saturations are 89%.

The correct answer is Salbutamol via spacer

The 14 month old has moderately severe respiratory distress due to virus-induced wheeze or possibly early asthma. He should be tried on salbutamol via spacer. Nebulised salbutamol may be required if he deteriorates.

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Theme:Cleft Palate

AAmnion Rupture Sequence
BBranchio - Oculo - Facial Syndrome
CDeletion 4p Syndrome
DEEC Syndrome
EFetal Hydantoin Syndrome
FFetal Valproate Syndrome
GOrofacial Digital Syndrome
HPatau Syndrome
IPierre Robin Syndrome
JTrisomy 13

Which syndrome is most likely to be associated with the presence of cleft lip and palate together with the following descriptions?

Epicanthic folds, a short nose with anteverted nostrils and a long philtrum.

Fetal Valproate Syndrome.
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Micrognathia, glossoptosis, airway obstruction.

Pierre Robin syndrome
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Thin wiry hair, fair skin and microdontia.

EEC Syndrome-Ectrodactyly, ectodermal dysplasia and cleft lip/palate syndrome.

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Fetal Valproate syndrome patients have characteristic features including epicanthic folds, a low nasal bridge with anteverted nostrils and a long philtrum with a thin vermilion border. There is a risk of congenital heart disease. Some patients have learning difficulties. Pierre Robin syndrome is the sequence of micrognathia, glossoptosis and cleft palate. Airway obstruction may lead to hypoxia, apnoeas, cor pulmonale and failure to thrive. Patients need to be monitored carefully. EEC syndrome is the combination of ectodermal dysplasia, ectrodactyly and cleft palate. Some patients have developmental delay. Once diagnosed it is necessary to carry out a renal ultrasound, as urinary tract abnormalities are common, especially renal agenesis, hydronephrosis and ureteric duplication.

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A 3-year-old girl presents with eye prominence, which has been worsening over the past 3 weeks. She has generally been tiring easily. Full term normal delivery with no neonatal complications. Immunisations up to date. There is no FH/SH of note.

On examination she has proptosis of the right eye, with bilateral bruised appearance of the eyelids. She looks a little pale and quiet, but alert and cooperative.

What is the most likely diagnosis?

Buphthalmos
Cruzon's disease
Grave's disease
Neuroblastoma
Orbital cellulitis

ans is neuroblastoma .

The appearance is of proptosis and periorbital ecchymoses, and is characteristic of metastatic neuroblastoma. This tumour of the sympathetic ganglia or chain is protean in presentations, and may secrete catecholamines producing hypertension. Most present with a large abdominal mass, which may embarrass breathing in infants. About 30% present with thoracic or cervical mass.

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Theme:Skin tumors

ABasal cell epithelioma
BDermatofibroma
CEpidermoid cyst
DEruptive vellus hair cysts
EInfantile digital fibroma
FJuvenile xanthogranuloma
GLipoma
HMilium
IMucosal neuroma syndrome
JNaevoid basal cell carcinoma
KPilar cyst
LPilomatricoma
MSteatocystoma multiplex
NSyringoma
OTrichoepithelioma

Select the term that best matches each case.

An 11-year-old girl presents with 3 small flesh-coloured dome-shaped nodules on the face and neck.

The correct answer is Epidermoid cyst.
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A 14-year-old boy presents with multiple cystic nodules on the sternum and axillae adherent to the skin and about 3mm across. When squeezed they drain cheesy material.

The correct answer is Steatocystoma multiplex

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A 16-year-old girl presents with multiple soft yellow-brown papules on the face and around the eyes.

The correct answer is Syringoma.

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The 11-year-old girl has epidermoid cysts. These may arise from sebaceous follicles, and may become infected with Staphylococcus aureus. Multiple cysts may be present in Gardner syndrome.

The 14-year-old boy has steatocytoma multiplex. This typically affects late teens in sebaceous areas.

The 16-year-old has syringoma, which are derived from intraepidermal sweat glands and are of cosmetic significance only. They may be too numerous to make removal impractical.

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Technetium-labelled 2,3-dimercaptosuccinic acid scans are used to detect renal scars in those who have had previous UTI or pyelonephritis. It is more sensitive than IVU in scar detection, and avoids the use of IV contrast. Within a few weeks of UTI photopaenic areas may be seen that are transient. Most clinicians therefore delay scanning till 6-8 weeks after an acute infection. Those with scars need long-term monitoring of blood pressure.

This scan shows scars predominantly affecting the right upper and lower poles, but the right midzone is also affected. This child will need regular follow-up of renal function and blood pressure.

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Which of the following is/are true concerning MRI?

True / False
It involves ionizing radiation - FALSE
Has no recognised side effects on the foetus - TRUE
The pregnant mother should be turned to her left side during scanning - TRUE
Tissue with high hydrogen concentrations are difficult to distinguish - FALSE
Blood vessels appear white on scanning - FALSE

Water is a molecule composed of hydrogen and oxygen atoms. The nuclei of the hydrogen atoms are able to act as microscopic compass needles. When the body is exposed to a strong magnetic field, the nuclei of the hydrogen atoms are directed into order - stand "to attention". When submitted to pulses of radio waves, the energy content of the nuclei changes. After the pulse, a resonance wave is emitted when the nuclei return to their previous state. The small differences in the oscillations of the nuclei are detected. By advanced computer processing, it is possible to build up a three-dimensional image that reflects the chemical structure of the tissue, including differences in the water content and in movements of the water molecules. This results in a very detailed image of tissues and organs in the investigated area of the body. In this manner, pathological changes can be documented.

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Theme:Organelles

AEndoplasmic reticulum
BGolgi apparatus
CLysosomes
DMicrotubules
EMitochondria
FNucleolus
GPlasma membrane
HRibosomes

Select the most appropriate organelle that matches the following descriptions

Contains enzymes capable of digesting cells and cellular material

LYSOSOMES
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'Reads' the mRNA and builds protein

RIBOSOMES
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Modification of lipids and proteins with storage of material prior to export out of the cell.

GOLGI APPARATUS
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The knowledge of cellular organelles is a pre-requisite for the understanding of the function of the cell. Different tissues having different quantities of organelles to correspond with their differing functions; e.g. muscle is packed with mitochondria, the organelle associated with energy production from Krebs' cycle. White cells have numerous lysosomes, which store enzymes that are capable of digesting cellular materials. The Golgi apparatus receives material from the endoplasmic reticulum (protein having been synthesized by ribosomes from mRNAs), alters and stores it prior to export from the cell.

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Fetal Varicella Zoster syndrome is characterised by:

True / False
Skin Loss - TRUE
Limb aplasia - TRUE
IUGR - TRUE
Macrocephaly- FALSE
Atrioventricular canal defects - FALSE

Fetal varicella zoster syndrome is charcaterised by areas of segmental skin loss or scarring, limb hypo/aplasia, growth retardation, microcephaly and ophthalmic abnormalities. Cardiac abnormalities are not a classic feature of the condition.

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Theme:Treatment of menorrhagia

ADilatation and curettage
BMirena coil
CTranexamic acid
DProstaglandin analogues
EGnRH analogues
FTotal abdominal hysterectomy (TAH)
GMefenamic acid
HTrans-cervical resection of the endometrium
ICombined oral contraceptive pill (COCP)
JDepot progestagen injection

For each case of menorrhagia below, choose the SINGLE most appropriate treatment from the above list of options. Each option may be used once, more than once, or not at all.

A 32-year-old woman presents with heavy, prolonged but painless periods. She has had problems with breast pains with previous use of the oral contraceptive pill. There is no obvious pelvic pathology on transvaginal ultrasound.

TRENAXAMIC ACID

Tranexamic acid is the best choice in view of the painless menorrhagia.

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A 43-year-old with frequent heavy periods causing anaemia and multiple pelvic adhesions requesting definitive treatment

The correct answer is Trans-cervical resection of the endometrium

The pelvic adhesions here would make hysterectomy a very difficult procedure. Hence TCRE may be the most appropriate option here. Transcervical resection of the endometrium is the most commonly used method of ablation for menorrhagia. The uterus is distended with glycine fluid at constant pressure to permit resectoscopic visualisation of the target area

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A 19-year-old female presents heavy, painful and irregular menstrual periods. She has had a number of sexual partners.

The correct answer is Combined oral contraceptive pill (COCP)

The COCP may be the most appropriate choice in this scenario as the female is young and appears to need contraception. THe pill would be expected to regulate menses and reduce pain.

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A 38-year-old multiparous female smoker presents with occasional heavy periods requiring contraception.

The correct answer is Mirena coil

The OCP would not be the most appropriate choice here due to the potential risks. However the Mirena coil would be ideal in this scenario reducing and stopping periods in the majority and offering adequate contraception.

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A 22-year-old female presents with a one year history of painful and heavy periods. She has a history of chronic active hepatitis and is also trying to conceive.

MEFENAMIC ACID

Mefenamic acid would the most appropriate treatment as it reduces pain and bleeding. Tranexamic acid would only be expected to reduce blood los rather than alleviate pain.

Dilatation and curettage is not a treatment for menorrhagia . Tranexamic acid is more effective than non steroidal anti inflammatory agents (NSAIDs) and progestagens in treating menorrhagia, but unlike NSAIDs such as mefenamic acid does not reduce pain. Trans cervical resection of the endometrium removes fertility but is useful as an alternative to hysterectomy in those who are unsuitable or unwilling for it, providing the uterine cavity is not distorted or very enlarged. In the case of the patient with multiple pelvic adhesions this may be a more appropriate choice than hysterectomy. The COCP should not be used in liver disease, but progestagens may be; neither in someone trying for a pregnancy. The mirena coil is a good treatment for menorrhagia in a multiparous woman and of course is also contraceptive.

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Advice to a patient wishing to suppress lactation should include:

True / False
Wear a well supported bra - TRUE
Express milk regularly from both breasts - FALSE
Cabergoline as one single dose - FALSE
Treatment with metoclopramide - FALSE
Application of cabbage leaves to breasts - TRUE

Breast milk expression will promote lactation via a positive feedback mechanism. Cabergoline may be used to treat galactorrhoea in hyperprolactinaemia secondary to prolactinoma. More than one dose is required to maintain suppression of lactation. Metoclopramide causes hyperprolactinaemia, and therefore does not suppress lactation. Cabbage leaves have been shown to suppress lactation.

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