Answer: 3. K – oxalate + NaF

Review:

• Bulb for glucose must contain sodium fluoride, so that glycolysis, which starts after about 30 mins after collection, is prevented.
  
• Enolase is a homodimer that is inhibited by fluoride, with formation of the magnesium
  fluorophosphates complex at the active site.
  
• This property of fluoride is used to inhibit
  glycolysis in blood specimens obtained for measurement of glucose concentration. In the absence of fluoride (or any other antiglycolytic agent), the blood glucose concentration decreases ast about 10 mg/dL per hour at 25oC.
  
• The rate of decrease is more rapid in blood from newborn infants owing to the increased metabolic activity of the erythrocytes and in leukemia patients because of the larger numbers of leukocytes.

• But the same chemical being toxic to enzymes, the enzyme dependant tests, like urea, creatinine, serum enzymes, reduces the values markedly and hence should not be used for such tests.
  
• Also the preparation of the anticoagulants should be proper. Even preparation of such bulbs matters : Adding 1 - 2 granules of anticoagulants powder produces macrocoagulation if not properly swirled immediately.
  
• Bulbs prepared by adding dissolved anticoagulant in proper portion and then drying is the best method.
  
• Citrate bulb for prothrombin time contains citrate solution just necessary and sufficient for 2 ml blood. Smaller or larger quantity of blood by itself alters prothrombin time significantly.

Answer: Diarrhea

Review:

• An ileostomy is a stoma that has been constructed by bringing the end of the small intestine (the ileum) out onto the surface of the skin.
  
• Intestinal waste passes out of the ileostomy and is collected in an external bag stuck to the skin.
  
• Ileostomies are usually sited above the groin on the right hand side of the abdomen.
• Diseases of the large intestine which may require surgical removal include:

• 
  
• Crohn's disease
• Ulcerative colitis
• Familial adenomatous polyposis(Total Colonic)
• Hirschprung's Disease
• The most common ileostomy is called a Brooke ileostomy after the English surgeon who developed it.
• Ileostomies need to be emptied frequently because the digested food contains large amounts of water. Shortly after the operation, the ileostomy produces 1-2 qt.(0.9-1.9 l) of fluid per day; after a month or two of adjustment, the volume decreases to 1-2 pt (0.5-0.9) per day.
• Ileostomy patients must learn to watch their fluid and salt intake. They are at greater risk of becoming dehydrated in hot weather, from exercise, or from diarrhea. In some cases they may need extra bananas or orange juice in the diet to keep up the level of potassium in the blood.
• About 40% of ileostomy patients have complications afterward, however; about 15% require minor surgical corrections. Possible complications include:
• skin irritation caused by leakage of digestive fluids onto the skin around the stoma; Irritation is the most common complication of ileostomies
• diarrhea
• the development of abscesses
• gallstones or stones in the urinary tract
• inflammation of the ileum
• odors can often be prevented by a change in diet
• intestinal obstruction
• a section of the bowel pushing out of the body (prolapse)

Answer: Juvenile Polyp

Review:

Juvenile polyps:

• Commonest form of polyp in children
• Can occur throughout large bowel but are most common in the rectum
• Usually present before 12 years
• Present with Prolapsing lump or rectal bleeding
• Not pre-malignant
• Treated by local endoscopic resection

Peutz-Jeghers syndrome:

• Rare familial disorder
• Circumoral pigmentation and intestinal polyps
• Polyps found throughout gut but most common in the small intestine
• Presents in childhood with bleeding, anaemia or intussusception
• Polyps can become malignant

Familial adenomatous polyposis:

• Aetiological factor in 1% of colorectal cancers
• Its is an autosomal dominant
• Due to mutation on long arm of chromosome 5
• Mutation induces proliferation of mucosa throughout GI tract
• Develop colonic polyps in teens or early 20’s
• Untreated progresses to cancer by 30’s
• Screening – by rigid or flexible sigmoidoscopy
• Safe alternative to colonoscopy as rectal sparing rarely seen
• Start late teens and continue until 40 yrs and polyp free.
• Extra-colonic manifestations:
  
  • Osteomas. epidermoid cysts = Gardener’s Syndrome
  • Gastroduodenal polyps
  • Desmoid tumours
  • Congenital hypertrophy of retinal pigmented epithelium
• Surgical options:
  
  • Panproctocolectomy and ileostomy
  • Restorative panproctocolectomy
  • Subtotal colectomy and ileorectal anastomosis
  • NB will require surveillance of rectal stump

Juvenile polyposis:

• polyps develop throughout the gastrointestinal tract in the first decade or two of life.
• Its autosomal dominant
• 15% to 20% of cases are due to germline mutations in the MADH4 gene (also known as SMAD4/DPC4) which is located on chromosome 18 in band 18q21 1. Mutations in the gene for bone morphogenic protein receptor 1A (BMPR1A), residing on chromosome 10 in band 10q22.3, account for 25% to 40% of cases.
• majority of cases sporadic with no family history of juvenile polyposis.
• People with the disease are at increased risk for developing gastrointestinal cancers.

Answer: 3.Broad Ligament

Reference: BDC, 3rd, Vol2, Pg-320

Answer: 1.Slow induction & recovery

Review:

• First used in 1951 by Cullen on an 81yr old man having an orchidectomy..
• Very close to the ‘ideal agent’
• The very large electron shell of xenon ca be distorted and polarised by nearby molecules, creating a dipole.
• Xenon inhibits the plasma membrane Ca ²⁺ pump, altering excitability. It inhibits the nociceptive responsiveness of spinal dorsal horn neurons.
• MAC = 71%. (With more widespread usage the Russians have noticed the MAC to be closer to 60%)
• Minimal haemodynamic effects.
• Lowest blood/gas partition coefficient = 0,115 of currently available inhalational agents.
• Low oil/water partition co-efficient of 20.
• Rapid induction and recovery regardless of duration of administration
• 4 stages of anaesthesia noted with 70% Xenon/ 30% oxygen.

1. Whole body paraesthesia & hypo-algesia.
2. Euphoria & increased psychomotor activity.
3. Analgesia with partial amnesia (after 3-4min).
4. Surgical anaesthesia with a degree of muscle relaxation.

• Equivalent analgesia when compared with equipotent doses of N ₂ O The analgesia produced by both gases is not reversible by naloxone.
• No occupational/ environmental disadvantages.
• Respiratory:
  
  
  1. Central depression causes a decrease in respiratory rate with a compensatory increase in tidal volume and can progress to apnoea
  2. Diffusion hypoxia is very mild as the blood/gas partition of Nitrogen (0.014) is only 10 times less than that of Xenon (0.14) as opposed to the almost 40 times less than Nitrous Oxide (0.47)

• Cardiovascular:
  
  1. No significant change in contractility, blood pressure and systemic resistance.
  2. Some reports of decrease in heart rate with variability in rhythm.
  3. No sensitisation of the myocardium to adrenaline
  4. May attenuate the myocardial depressant effects of isoflurane.

Summary

• Colourless and odourless gas with no irritation to the respiratory tract. Well tolerated with gas induction
• Low blood/gas and oil/water partition co-efficients allowing rapid induction and eduction
• Produces unconsciousness with analgesia and a degree of muscle relaxation
• MAC of 60-70% allows a reasonable inspired oxygen concentration
• It does cause respiratory depression, to the point of apnoea.
• It is cardiac stable.
• Not metabolised in the body and is eliminated rapidly and completely via the lungs.
• It is non toxic and is not associated with allergic reactions
• Stable in storage, no interaction with anaesthesia circuits or soda lime. Should not be used with rubber anaesthesia circuits as there is a high loss through the rubber
• Non flammable

• "unique drug"- hypnotic (sleep producing), analgesic (pain relieving) and amnesic (short term memory loss)
  
• no other drug used in clinical practice combines these three important features.
• The anaesthetic state produced is frequently called "dissociative anaesthesia" which implies that the patient is detached from their surroundings
• the patient's eyes often remain open and constantly move from side to side
• the patient may be agitated on recovery from ketamine. This is often called "emergence delirium", during which the patient may be disorientated, restless, and crying
• Ketamine causes a rise in intracranial pressure
• The blood pressure rises by about 25% (on average the systolic pressure rises by 20-30 mmHg) and the heart rate is increased by about 20% - the overall effect is therefore to increase the workload of the heart.
• Ketamine produces some bronchodilation making it a useful anaesthetic drug for patients with asthma
• Muscle tone is often increased
• Ketamine crosses the placenta easily
• not suitable for use in patients with a perforated eye injury or for ophthalmic surgery
• Ketamine may be used as the sole anaesthetic agent for a large number of superficial operations and procedures in both adults and children. Common procedures undertaken with ketamine anaesthesia include minor to intermediate orthopaedic surgery (especially distal arm or lower leg surgery including manipulation of fractures), gynaecological surgery (eg. dilatation and curettage and other minor surgical procedures), drainage of abscesses, debridement of burns, change of dressings and minor dental procedures, as well as a variety of examinations under anaesthesia

Answer: 3. Pylorrizidine alkaloids

Review:

Pyrrolizidine alkaloids:

• Pyrrolizidine alkaloids (PAs) are hepatotoxins found in many species of plants throughout the world.
  
• Most cases of pyrrolizidine alkaloid toxicity result in moderate to severe liver damage.
• Gastrointestinal symptoms are usually the first sign of intoxication, and consist predominantly of abdominal pain with vomiting and the development of ascites.
  
• Death may ensue from 2 weeks to more than 2 years after poisoning, but patients may recover almost completely if the alkaloid intake is discontinued and the liver damage has not been too severe.

Sanguinarine

• Epidemic dropsy is a form of edema due to intoxication with Argemone mexicana (Mexican prickly poppy).
• In Northern India, epidemic dropsy occurs as a food adulterant disease where use of mustard oil as cooking medium is common.
  
• When mustard oil is adulterated deliberately (as in most cases) or accidental contamination with argemone oil, proteinuria (specifically loss of albumin) occurs, with a resultant edema as would occur in nephrotic syndrome.
  
• Other symptoms are headache, nausea, loose bowels, erythema and breathlessness.
• In severe cases fatalities are reported due to congestive heart failure.
• The toxic effects of argemone oil have been attributed to the presence of benzophenanthridine alkaloids, sanguinarine and dihydrosanguinarine.
  
• It has been reported that the sanguinarine alkaloid content in argemone oil varies from 0.44% to 0.50%.

Aflatoxins

• Aflatoxins are naturally occurring mycotoxins that are produced by many species of Aspergillus, a fungus, most notably Aspergillus flavus and Aspergillus parasiticus.
• After entering the body, aflatoxins are metabolized by the liver to an reactive intermediate, aflatoxin M1, an epoxide.
• High-level aflatoxin exposure produces an acute necrosis, cirrhosis, and carcinoma of the liver exhibited by hemorrhage, acute liver damage, edema, alteration in digestion, and absorption and/or metabolism of nutrients
• Children, however, are particularly affected by aflatoxin exposure which leads to stunted growth and delayed development. Chronic exposure also leads to a high risk of developing liver cancer, as the metabolite Aflatoxin M1 can intercalate into DNA and alkylate the bases through its epoxide moiety.

Ergot

• Ergot is the common name of a fungus in the genus Claviceps.
• Ergotism is the effect of long-term ergot poisoning, classically due to the ingestion of the alkaloids produced by the Claviceps purpurea fungus which infects rye and other cereals, and more recently by the action of a number of ergoline-based drugs.
  
• It is also known as ergotoxicosis, ergot poisoning and St Anthony's fire.
• Convulsive Symptoms: painful seizures and spasms, diarrhea, paresthesias, itching, headaches, nausea and vomiting. Usually the gastrointestinal effects precede CNS effects. As well as seizures there can be hallucinations and mental effects including mania or psychosis. The convulsive symptoms are caused by clavine alkaloids.
• Gangrenous symptoms: The dry gangrene is a result of vasoconstriction induced by the ergotamine-ergocristine alkaloids of the fungus. It affects the more poorly vascularized distal structures, such as the fingers and toes. Symptoms include desquamation, weak peripheral pulse, loss of peripheral sensation, edema and ultimately the death and loss of affected tissues.

Answer: 1. B Cells

Review:

Post-transplant lymphoproliferative disease (PTLD) is an unusual entity that has many of the features of immune system malignancy.


It is characterized by uncontrolled proliferation of B cells in a context of post transplant immunosuppression. In some situations, reducing the immunosuppression can reverse this proliferation. PTLD has emerged as a significant complication of solid organ transplantation. This entity was rarely reported until the mid 1980s, when the incidence began rising suddenly. PTLD presents significant problems for the clinician because it is difficult to predict and has high morbidity and mortality rates because of the difficulty in prevention and potential for graft loss.

Answer: Masochism

Review:

Sexual asphyxia is a paraphilia which is a sub-category of sexual masochism. Also known by terms such as asphyxiophilia, autoerotic asphyxia or Hypoxyphilia, this potentially lethal sexual practice refers to sexual arousal that is produced while reducing the oxygen supply to the brain.

1. The essential feature of sexual masochism is the feeling of sexual arousal or excitement resulting from receiving pain, suffering, or humiliation. The pain, suffering, or humiliation is real and not imagined and can be physical or psychological in nature. A person with a diagnosis of sexual masochism is sometimes called a masochist.

2.Voyeurism is a psychosexual disorder in which a person derives sexual pleasure and gratification from looking at the naked bodies and genital organs or observing the sexual acts of others. The voyeur is usually hidden from view of others. Voyeurism is a form of paraphilia.

3.The essential feature of sexual sadism is a feeling of sexual excitement resulting from administering pain, suffering, or humiliation to another person. The pain, suffering, or humiliation inflicted on the other is real; it is not imagined and may be either physical or psychological in nature. A person with a diagnosis of sexual sadism is sometimes called a sadist. The name of the disorder is derived from the proper name of the Marquis Donatien de Sade (1740-1814), a French aristocrat who became notorious for writing novels around the theme of inflicting pain as a source of sexual pleasure.

4. Fetishism is a form of paraphilia, a disorder that is characterized by recurrent intense sexual urges and sexually arousing fantasies generally involving non-human objects, the suffering or humiliation of oneself or one's partner (not merely simulated), or children or other non-consenting persons. The essential feature of fetishism is recurrent intense sexual urges and sexually arousing fantasies involving specific objects. While any object may become a fetish, the distinguishing feature is its connection with sex or sexual gratification. A diagnosis of fetishism is made only if an individual has acted on these urges, is markedly distressed by them, or if the fetish object is required for gratification.

Answer: Renal Function (?)

Review:

Schwartz formula: This employs the serum creatinine, the child's height and a constant to estimate the creatinine clearance.

k - for height in some units and creatine in some other units, the constant is 0.33-0.45 in infants, 0.55 in children or adolescent girls, or 0.70 in adolescent boys.


Use height in the estimate, as height is proportional to muscle mass.
The constants used in the equations differ, likely related to the different assays to measure creatinine.

Ref: http://www.kidney.org/professionals/kdoqi/guidelines_ckd/p5_lab_g4.htm

Answer: Ataxia Telangiectasia

Review:

BRCA1 and BRCA2 are major genes related to hereditary breast cancer. People who have inherited certain mutations in these genes have a high risk of developing breast cancer, ovarian cancer, and several other types of cancer over their lifetimes. Men with BRCA1 mutations have an increased risk of developing prostate cancer. Mutations in the BRCA2 gene are associated with an increased chance of developing male breast cancer and cancers of the prostate, pancreas, gallbladder, bile duct, and gastrointestinal tract.

Melanoma (an aggressive form of skin cancer) and lymphoma (a cancer of immune system cells) also are more common among people who have BRCA2 mutations.
Inherited changes in several other genes, including ATM, CHEK2, and RAD51, have been associated with an increased risk of breast cancer. Although some research suggests that certain versions of the AR gene also may be associated with breast cancer risk, several other studies have not shown this connection.
Other gene mutations are acquired during a person's lifetime and are present only in certain cells. These changes, which are called somatic mutations, are not inherited. Somatic mutations in the ERBB2 (also called Her-2/neu), DIRAS3 (formerly ARHI), and TP53 genes have been identified in some cases of breast cancer.


The risk for breast cancer is increased with Li-Fraumeni syndrome (LFS), a genetic autosomal dominant cancer syndrome. A diagnosis of LFS is made when all of the following characteristics are present in a family:

• sarcoma <45>and
  
  
• a first degree relative with cancer <45>and
  
  
• a first or second degree relative with cancer <45>

The majority of LFS (about 70 percent) is caused by mutations in a gene on chromosome 17 known as p53. Mutations in p53 confer an increased risk for early onset breast cancer, childhood sarcoma, osteosarcoma, brain tumors, leukemia, and adrenocortical carcinoma. Persons with a p53 mutation have a 50 percent chance of developing one of the associated cancers by age 50. For women, the risk of breast cancer specifically is 49 percent by age 44 with a lifetime risk of 60 percent.


(Ref: http://cancer.stanford.edu/information/geneticsAndCancer/types/lifrmni/)


Overall, the risk of an Ataxia Telangiectasia patient developing any cancer is 37-fold higher than individuals in the general population. The risk of developing lymphoid tumors, however, the most frequently diagnosed cancers in A-T patients, is 100-fold higher than in the general population; A-T patients have about a 10 percent risk of developing lymphoma or leukemia. Cancers also occur in the stomach, brain, ovary, skin, liver, larynx, parotid gland, and breast.

(Ref: http://www.cancer.gov/cancertopics/factsheet/ataxiaqa)

Inhibition of complement activation

a) Capsules with sialic acid residues that prohibit alternative pathway activation

b) Group B and Group A streptococcal C5a-ase

2. Presence of antiphagocytic factors

a) Capsular polysaccharide

b) M-proteins

3. Genetic variation of surface proteins

a) Pili of GC

4. Inhibit phagolysosome fusion- mycobacteria

5. Scavenge reactive oxygen species- glycolipid of M. leprae

6. Block killing- hemolysins of L monocytogenes

DEVELOPMENT: It is mesodermal -- not derived from gut (i.e. non - gut)

• It grows within the two layers of peritoneum going to the posterior wall -- within the two folds defining the dorsal mesogastrium.
• As the stomach rotates, the spleen is moved to the left of the stomach (lateral to stomach)
• The dorsal mesogastrium in this region becomes the gastrosplenic ligament.
• Posterior part of mesogastrium adheres to the posterior wall, and the left kidney will then lie directly deep to it. This portion of the mesentery becomes the splenorenal ligament.
  

LOCATION: Upper left quadrant, left hypochondriac region, articulated with ribs 9-11 (laterally).

EXTERNAL MORPHOLOGY: It has three grooves (surfaces)

• Renal Surface
• Gastric Surface
• Colic Surface: Anterior / Inferior extremity.
• Hilus: Contains the Splenic artery and vein, near the splenorenal ligament.
  

RELATIONSHIPS:

• Kidney is deep to it, connected by splenorenal ligament.
• Stomach is medial to it, connected by gastrosplenic ligament.
  

VASCULAR / LYMPH SUPPLY:

Splenic Artery and Splenic Vein come into the hilus.