The vas deferens is a long tube that connects the epididymis to the ejaculatory ducts. It acts as a canal through which mature sperm may pass through the penis during ejaculation.

Most men with CF (97-98 percent) are infertile because of a blockage or absence of the vas deferens, known as congenital bilateral absence of the vas deferens (CBAVD). The sperm never makes it into the semen, making it impossible for them to reach and fertilize an egg through intercourse. The absence of sperm in the semen can also contribute to men with CF having thinner ejaculate and lower semen volume.

This is a classical picture of graft versus host disease (GVHD) following bone marrow transplant. Acute GVHD occurs in the first 100 days post transplant with chronic GVHD occurring 100-300 days after transplant. GVHD is graded according to the Seattle system, and each organ involved is scored (skin, liver, and gut).

The standard initial treatment in the acute setting is high-dose methylprednisolone started immediately. If there is no response, a more intensive immunosuppressive agent such as alemtuzumab or antilymphocyte globulin is needed.

The most appropriate treatment in this patient would be to stop warfarin therapy and keep the patient under observation.

The drugs that lead to enhanced potency of warfarin include: disulfiram, trimethoprim-sulphamethoxazole, metronidazole, phenylbutazone, aspirin, heparin, and clofibrate.
Liver disease, thrombocytopenia, hyperthyroidism also increase the oral anticoagulant potency.
If the patient has minor bleeding and the international normalized ratio (INR) is >6.0, warfarin should be stopped; the INR should be rechecked daily and in addition to the stoppage of warfarin, vitamin K 2.5 mg oral or 0.5 mg intravenously should also be administered.
In a patient with INR of 2.0 or 3.0, it takes two or three times longer for that individual’s blood to clot than someone who is not taking any anticoagulants. Most patients on warfarin have an INR goal of 2 to 3.

If there is major bleeding then prothrombin complex concentrates 50 u/kg or fresh-frozen plasma 15 ml/kg may be considered.

Lead poisoning is characterised by abdominal pain, fatigue, constipation, peripheral neuropathy (mainly motor), and blue lines on gum margin in 20% of the adult patients (very rare in children).

For diagnosis, the level of lead in blood is usually considered with levels greater than 10 mcg/dL being significant. Furthermore, the blood film shows microcytic anaemia and basophilic stippling of red blood cells. Urinary coproporphyrin is increased (urinary porphobilinogen and uroporphyrin levels are normal to slightly increased). Raised serum and urine levels of delta-aminolaevulinic acid may also be seen, making it sometimes difficult to differentiate from acute intermittent porphyria.

Congenital CMV infection can occur when a pregnant woman is infected with the cytomegalovirus (CMV) and passes the virus to her unborn child. Infants infected with CMV during pregnancy can exhibit a range of symptoms, including jaundice, seizures, and microcephaly (abnormally small head size). These symptoms are consistent with congenital CMV infection.

Given the mother’s HIV-positive status, the infant may have been at increased risk of acquiring other infections, including CMV, due to potential immunodeficiency.

Normal pressure hydrocephalus is a form of communicating hydrocephalus characterised by enlarged ventricles with normal opening pressure on lumbar puncture. For diagnostic purposes, a triad of urinary incontinence, gait abnormality and dementia is necessary. It is necessary to diagnose the condition early because it is reversible by placing a ventriculoperitoneal shunt. It is most common in patients aged more than 60 years.

Mechanism of action of low molecular weight heparin (LMWH):
It inhibits coagulation by activating antithrombin III. Antithrombin III binds to and inhibits factor Xa. In doing so it prevents activation of the final common path; Xa inactivation means that prothrombin is not activated to thrombin, thereby not converting fibrinogen into fibrin for the formation of a clot.

LMHW is a small fragment of a larger mucopolysaccharide, heparin. Heparin works similarly, by binding antithrombin III and activating it. Heparin also has a binding site for thrombin, so thrombin can interact with antithrombin III and heparin, thus inhibiting coagulation.
Heparin has a faster onset of anticoagulant action as it will inhibit not only Xa but also thrombin, while LMWH acts only on Xa inhibition.

Compared to heparin, LMWHs have a longer half-life, so dosing is more predictable and can be less frequent, most commonly once per day.

Dosage and uses:
LMWH is administered via subcutaneous injection. This has long-term implications on the choice of anticoagulant for prophylaxis, for example, in orthopaedic patients recovering from joint replacement surgery, or in the treatment of DVT/PE.

Adverse effects:
The main risk of LMWH will be bleeding. The specific antidote for heparin-induced bleeding is protamine sulphate.
Less commonly it can cause:
Heparin-induced thrombocytopenia (HIT)
Osteoporosis and spontaneous fractures
Hypoaldosteronism
Hypersensitivity reactions

Tuberculous lymphadenitis is a chronic, specific granulomatous inflammation of the lymph node with caseation necrosis, caused by infection with Mycobacterium tuberculosis or a related bacteria. The characteristic morphological element is the tuberculous granuloma (caseating tubercle).

Because of the patient’s presentation with flank pain that radiates to the groin, we are suspecting renal colic. We should follow guidelines for acute renal management and prescribe IM diclofenac for immediate relief of pain.

Annual incidence of DVT in the general population is about 1 per 1000 adults. So the most suitable answer is about 2 per 1000.

Lymphedema is most commonly the result of removal or damage to lymph nodes.

The following are conditions commonly associated with pyoderma gangrenosum:
Inflammatory bowel disease:
– Ulcerative colitis
– Crohn’s disease
Arthritides:
– Rheumatoid arthritis
– Seronegative arthritis
Haematological disease:
– Myelocytic leukaemia[8]
– Hairy cell leukaemia
– Myelofibrosis
– Myeloid metaplasia
– Monoclonal gammopathy
Autoinflammatory disease:
– Pyogenic sterile arthritis, pyoderma gangrenosum, and acne syndrome (PAPA syndrome)
– Granulomatosis with polyangiitis

Lidocaine is an example of class IB antiarrhythmics.

The Vaughan-Williams classification of antiarrhythmics
I: Membrane stabilizing agents
IA: Quinidine, Procainamide, Disopyramide
IB: Lidocaine, Mexiletine
IC: Propafenone, Flecainide
II: β blockers – Propranolol, Esmolol
III: Agents widening AP – Amiodarone, Dronedarone, Dofetilide, Ibutilide, Sotalol
IV: Calcium channel blockers – Verapamil, Diltiazem
V: Miscellaneous – Digoxin, adenosine, magnesium

Tumour marker CA 15-3 is mostly associated with breast cancer.

Tumour markers can be divided into:
1. Monoclonal antibodies
CA 125: Ovarian cancer, primary peritoneal cancer
CA 19-9: Pancreatic cancer
CA 15-3: Breast cancer

2. Tumour specific antigens
Prostate specific antigen (PSA): Prostatic carcinoma
Alpha-feto protein (AFP): Hepatocellular carcinoma, teratoma
Carcinoembryonic antigen (CEA): Colorectal cancer
S-100: Melanoma, schwannomas
Bombesin: Small cell lung carcinoma, gastric cancer

3. Enzymes
Alkaline phosphatase (ALP)
Neuron specific enolase (NSE)

4. Hormones
Calcitonin
Antidiuretic hormone (ADH)
Human chorionic gonadotropin (hCG)

Vestibular damage is a neurological side effect of streptomycin.

Streptomycin is an aminoglycoside bactericidal antibiotic. It is used in the treatment of tularaemia and resistant mycobacterial infections.
The most common neurological side-effect is vestibular damage leading to vertigo and vomiting.
Cochlear damage is less frequent and results in deafness.
Other side-effects include rashes, angioneurotic oedema, and nephrotoxicity.

The given clinical scenario warrants urgent admission and psychiatric evaluation as the mother seems to be suffering from puerperal psychosis.

Post-partum mental health problems can range from the ‘baby-blues’ to puerperal psychosis.

The Edinburgh Postnatal Depression Scale may be used to screen for depression:
A 10-item questionnaire, with a maximum score of 30
indicates how the mother has felt over the previous week
score > 13 indicates a ‘depressive illness of varying severity’
sensitivity and specificity > 90%. It includes a question about self-harm

Postpartum psychosis has a complex multifactorial origin. Risk factors include a history of bipolar disorder, history of postpartum psychosis in a previous pregnancy, a family history of psychosis or bipolar disorder, a history of schizoaffective disorder or schizophrenia and discontinuation of psychiatric medications during pregnancy.
Incidence rate: 2.6 per 1000 births.
Onset usually within the first 2-3 weeks following birth
Symptoms of puerperal psychosis include confusion, lack of touch with reality, disorganized thought pattern and behaviour, odd effect, sleep disturbances, delusions, paranoia, appetite disturbances, a noticeable change in the level of functioning from baseline, hallucinations and suicidal or homicidal ideation.
The safety of the patient and new-born is of utmost importance, and thus, immediate hospitalization is warranted if there is a risk of harm to either one.
Cognitive-behavioural therapy may be beneficial. Certain SSRIs such as sertraline and paroxetine may be used if symptoms are severe – whilst they are secreted in breast milk it is not thought to be harmful to the infant
There is around a 20% risk of recurrence following future pregnancies

The most likely diagnosis with anaemia, raised erythrocyte sedimentation rate (ESR), hypercalcaemia, renal impairment, and raised total protein with low albumin is multiple myeloma (MM). Protein electrophoresis will confirm the presence of monoclonal band of paraprotein. Of note, a radioisotope bone scan is not a good test for picking up the lytic lesions of MM.

Diagnosis of MM is based on the confirmation of (a) one major criterion and one minor criterion or (b) three minor criteria in an individual who has signs or symptoms of multiple myeloma.

Major criteria:
1. >30% plasma cells on bone marrow biopsy
2. Monoclonal band of paraprotein on electrophoresis: >35g/L for IgG, 20g/L for IgA, or >1g of light chains excreted in the urine per day

Minor criteria:
1. 10–30% plasma cells on bone marrow biopsy
2. Abnormal monoclonal band but levels less than listed above
3. Lytic bone lesions observed radiographically
4. Immunosuppression

Venesection should be done (essentially blood-letting) to decrease the too-high ferratin level. IV Desterrioxamine would have the opposite effect. ASA does not have to do with ferritin. You do not just watch this and recheck. And you do not want to make the patient anaemic.

Low body weight. Excessively low body weight — about 10 percent under normal weight — interrupts many hormonal functions in your body, potentially halting ovulation.
In Polycystic ovaries there is excess weight gain.

Given she is not showing signs of systemic illness, you do not need to treat for an acute flair (which would be steroids), but you need to put her on maintenance medication. Oral ASA would be the best option for this, it is first line. You cannot give rectal ASA because the location of her disease is in the ascending colon and the enema will not reach.

Immune thrombocytopenic purpura (ITP) refers to thrombocytopaenia occurring in the absence of toxic exposure or other diseases associated with low platelets and involves IgG-type antibodies. It is characterised by normal or increased marrow megakaryocytes, shortened platelet survival, and the absence of splenomegaly. Autoimmune haemolytic anaemia (AIHA) occurs commonly in association with ITP. Leukemic transformation, however, does not occur in ITP.

In neonatal ITP, IgG antibodies are passively transferred across the placenta. The infant platelet count may be normal at birth but decreases within 12–24 hours. It is rarely severe enough to induce bleeding diathesis in the infant.

Pulmonary mycobacterium avium complex (MAC) infection in immunocompetent hosts generally manifests as cough, sputum production, weight loss, fever, lethargy, and night sweats. The onset of symptoms is insidious.
In patients who may have pulmonary infection with MAC, diagnostic testing includes acid-fast bacillus (AFB) staining and culture of sputum specimens.

The ATS/IDSA guidelines include clinical, radiographic, and bacteriologic criteria to establish a diagnosis of nontuberculous mycobacterial lung disease.

Clinical criteria are as follows:

Pulmonary signs and symptoms such as cough, fatigue, weight loss; less commonly, fever and weight loss; dyspnoea

Appropriate exclusion of other diseases (e.g., carcinoma, tuberculosis).

At least 3 sputum specimens, preferably early-morning samples taken on different days, should be collected for AFB staining and culture. Sputum AFB stains are positive for MAC in most patients with pulmonary MAC infection. Mycobacterial cultures grow MAC in about 1-2 weeks, depending on the culture technique and bacterial burden.

Salbutamol stimulates beta-2 adrenergic receptors, which are the predominant receptors in bronchial smooth muscle (beta-2 receptors are also present in the heart in a concentration between 10% and 50%).

Stimulation of beta-2 receptors leads to the activation of enzyme adenyl cyclase that forms cyclic AMP (adenosine-mono-phosphate) from ATP (adenosine-tri-phosphate). This increase of cyclic AMP relaxes bronchial smooth muscle and decrease airway resistance by lowering intracellular ionic calcium concentrations. Salbutamol relaxes the smooth muscles of airways, from trachea to terminal bronchioles.

Increased cyclic AMP concentrations also inhibits the release of bronchoconstrictor mediators such as histamine and leukotriene from the mast cells in the airway.

Severe acute respiratory syndrome (SARS) is a viral respiratory illness caused by a coronavirus called SARS-associated coronavirus (SARS-CoV). SARS was first reported in Asia in February 2003.
In general, SARS begins with a high fever (temperature greater than 38.0°C). Other symptoms may include headache, an overall feeling of discomfort, and body aches. Some people also have mild respiratory symptoms at the outset. About 10 to 20 percent of patients have diarrhoea. After 2 to 7 days, SARS patients may develop a dry cough. Most patients develop pneumonia. 

Leptin, a 167–amino acid hormone, was discovered in 1994 and is secreted mainly by adipocytes. Plasma leptin levels are significantly correlated with body mass index (BMI) and the total amount of body fat.

A Mongolian spot can be present in new born babies which usually appears over the back and fades with time. There is nothing to worry about.

The patient’s presentation with slurred speech, intention tremor and past pointing, as well as ataxia and nystagmus, paired with a history of vertigo suggest the cerebellum as the site of cerebrovascular accident (CVA) or stroke.

Narcolepsy is a rare condition characterised by excessive daytime sleepiness, sleep paralysis, hypnagogic hallucinations, and cataplexy (sudden collapse triggered by emotion such as laughing or crying). There is no cure for narcolepsy. Treatment options include stimulants, such as methylphenidate (Ritalin) or modafinil (Provigil), antidepressants, such as fluoxetine (Prozac), citalopram (Celexa), paroxetine (Paxil), sertraline (Zoloft) and sodium oxybate (Xyrem). Modafinil has replaced methylphenidate and amphetamine as the first-line treatment of excessive daytime sleepiness (EDS).

Fundoscopy findings of cotton wool spots and retinal tears, accompanied by a history of blurred vision, are characteristic of retinitis. Cytomegalovirus is known to cause retinitis.

Paget’s accelerates the remodelling process with old bone breaking down more quickly which disrupts the normal growth process. New bone development eventually adjusts to the faster pace and speeds up on its own. The pace is too fast for healthy bone growth, and the regrowth ends up softer and weaker than normal. The effect of this accelerated process causes bone pain, fractures, and deformities. Since osteosarcoma is a type of bone cancer linked to abnormal bone growth, this makes it a rare but possible complication of Paget’s disease although the chances of developing osteosarcoma are minimal. Fractures are painful and restrict movement whereas this patients pain is gradually developing. Pain associated with osteoarthritis is usually mild to moderate and worsens as the day progresses.