Cystic fibrosis (homozygous) | PGIMER Frequently Asked Medical PG topics

A pt. of cystic fibrosis (homozygous) married a carrier (heterozygous). The chances of developing cystic fibrosis in the offsprings is

A. 50% carriers, 50% affected 
B. 25% carriers, 75% affected 
C. 75% carriers, 25% affected 
D. All are carriers 
E. All are affected

Ans.  A.  50% carriers, 50% affected

• Cystic fibrosis is an autosomal recessive disorder. Imprtant featrure of AR disease: 

- Either sex affected with equalfrequency or severity. 

- Affected person is homozygous or compound heterozygote for a-single gene deftet. 

- Heterczygous carriers of a deft ctive allele are usually clinically normal. 

- Horizontal pattern ofpedigree with a single generation affected. 

• The chance of developing cystic fibrosis in our question is 50%. 

Whole body radiation MCQ | PGIMER MCQs for MD MS Preparations

Whole body radiation is indicated in 

A. Advanced head & neck tumours 
B. BM transplantation 
C. Medulloblastoma 
D. Mycosis fungoides 
E. Histiocytosis-x ]

 Ans. B.  Bone marrow transplantation ; (D) Mycosis fungoides;
When radiation is given in a way to cover thewhole body it is called total body irradiation or whole body irradiation. This done for one of two reasons
(i) To suppress the pt’s immune system and prevent rejection of donor bone marrow after BM transplantation using donor BM from someone other than the patient.
(ii) To kill abnormal cells that escape other therapies such as surgeries, chemotherapy or local irradiation and remain hidden in the body to regrow later.
• It is used in:
- Aplastic anaemia to prevent rejection of donor marrow.
- Beta thalassernia
- CML
-AML&ALL
-NHL
- Neuroblastoma
- Ewing’s sarcoma I unfavourable pediatric sarcoma
• Mycosis fungoides is treated with whole body radiation.
• Histiocytosis X resolves spontaneously in usual cases and it can be treatedwith Chemotherapy.
•Medulloblastoma can be treated with craniospinal radiation.
• Advanced head & neck turnours are treated with local or regional radio therapy. 

Members of southeast Asia of WHO | Medical PG Preparations

Members of southeast Asia of WHO are 

A. Japan 
B. Afganistan 
C. India 
D. Pakistan 
E. Srilanka

Ans. C. India ;(E) Srilanka 

SEARO— The South East Asia Regional Office Headquarter is located in New Delhi (India). It has new eleven members — Bangladesh, Bhutan, lndia, indonesia, Korea, Maldives Islands, Mongolia, Myanmar, Nepal, Srilanka, Thailand. 

The WHO activities in South East Asia Region cover a wide range of subjects such as malaria eradication, tuberculosis control, control of other communicable disease, health laboratory services and production of vaccine, health statistics, public health administration and rural health service, maternal and child health nursing, environmental health, dental health, medical rehabilitation, quality control of drugs and medical education. 

ACE inhibitor induced cough | PGIMER MCQs for MD MS Preparations

ACE inhibitor induced cough is mediated by. 

A. Bradykinin 
B. Substance - P 
C. Prostaglandin 
D. Serotonin 
E. Renin

Ans. A. Bradykinin ; (B) Substance—P ; (C) Prostaglandin

•About 5-20% of patients taking ACE inhibitors develop a non-productive cough. Onset is usually within 1 week of starting the drug but can be delayed up to 6 months. Mechanism is not known with certainty, but may be due to accumulation of bradykinin, substance-P, and / or prostaglandin which are degraded by ACE. 

• Treatment:Cough disappears within 4 days if stoppage. Sometime require cessation of therapy. 

• Thromboxane antagonism reduces ACE inhibitor induced cough. 

Celiac sprue diagnosis | PGIMER MCQs for MD MS Preparations

Celiac sprue is diagnosed by 

A. Intestinal biopsy 
B. Unequivocal response to gluten restriction 
C. Finding of organism 
D. Improvement on dapsome treatment 
E. H/O fat malabsorption

Ans. B. Unequivocal response to Gluten resriction :

• The diagnosis of celiac spure requires the presence of characteristic hisotpatholocic changes on small intestinal biopsy together with a prompt Clinical and histopathological response following 

the institution of gluten-free diet. 

The changes seen on duodenum/jujunal biopsy are restricted to muscosa and includes-

(i) Absence or reduced height of villi, resulting a “flat” appearance. 

(ii) Increased loss of villus cells in association with ted crypt cell proliferation resulting in crypt hyperplasia and loss of villous structure, with consequent villus but not mucosal atrophy. 

(iii) Cuboidal appearance and nuclei that are oriented basally in surface epithelial cells and increased intraepithelial lymphocytes. 

(iv) increased lymphocytes and plasma cells in the lamina propria. 

• Although histopathological features are characteristic celiac sprue but not diagnostic as similar appearance is seen in — Tropical sprue, eosinophilic enteritis, milk-protein intolerance in children and occasionally in lymphoma, bacterial over growth, Crohn’s disease and gastrinoma with acid hypersecretion. 

Pulmanary flow | PGIMER entrance Preparations

Pulmanary flow is decreased in

A. Fallot’s tetralogy
B. Ebstein’s anomaly 
C. Common atria 
D. TGV with intact septum 
E. Postoperative TGV correction

Ans. A. Fallot’s tetralogy ; (B) Ebstein’s anomaly.

• The route of blood flow in complete Transposition of great vessels (TGA) results in two separate circulation and survival depends on mixing that occur at a trial, ventricular or great vessel level. In TGA with intact septum, mixing occurs through foramen ovale and being small, the mixing is poor. Pulmonary plethora is seen. 

• In corrected TGA, route of blood flow is normal. Haemodynamics depend on associated anomalies.

• Normal or Decreased pulmonary blood flow with cyanosis is seen in

- Tricuspid atresia 

- Ebstein’s anomaly with atrial shunt 

- Pulmonary atresia with intact ventricular septum

- Pulmonic stenosis or atresia with VSD 

- Pulmonic stenosis with right to left atrial shunt

- Complete TGA with pulmonic stenosis 

- Double — out left ventricle with pulmonic stenosis 

- Single ventricle with pulmonic stenosis 

- Pulmonary AV fistula 

- Vena cava to Lt atrial communication. 

• Pulmonary blood flow increased in common artria. 

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