Polycystic kidney disease

Polycystic kidney disease (PKD)

Polycystic kidney disease (PKD) is an inherited disorder in which clusters of cysts develop primarily within your kidneys, causing your kidneys to enlarge and lose function over time. Cysts are noncancerous round sacs containing fluid. The cysts vary in size, and they can grow very large. Having many cysts or large cysts can damage your kidneys.

Polycystic kidney disease also can cause cysts to develop in your liver and elsewhere in your body. The disease can cause serious complications, including high blood pressure and kidney failure.PKD varies greatly in its severity, and some complications are preventable. Lifestyle changes and treatments might help reduce damage to your kidneys from complications.

CAUSES

The two main types of polycystic kidney disease, caused by different genetic flaws, are:

• Autosomal dominant polycystic kidney disease (ADPKD). Signs and symptoms of ADPKD often develop between the ages of 30 and 40. In the past, this type was called adult polycystic kidney disease, but children can develop the disorder.
  Only one parent needs to have the disease for it to pass to the children. If one parent has ADPKD, each child has a 50 percent chance of getting the disease. This form accounts for about 90 percent of cases of polycystic kidney disease.
• Autosomal recessive polycystic kidney disease (ARPKD). This type is far less common than is ADPKD. The signs and symptoms often appear shortly after birth. Sometimes, symptoms don't appear until later in childhood or during adolescence.
  Both parents must have abnormal genes to pass on this form of the disease. If both parents carry a gene for this disorder, each child has a 25 percent chance of getting the disease.

Symptoms

Polycystic kidney disease symptoms can include:

• Increased size of your abdomen due to enlarged kidneys
• A feeling of fullness in your abdomen
• Urinary tract or kidney infections

• High blood pressure
• Blood in your urine
• Back or side pain
• Kidney stones
• Kidney failure
• Headache

Prevention

If you have polycystic kidney disease and you're considering having children, a genetic counselor can help you assess your risk of passing the disease to your offspring.

Keeping your kidneys as healthy as possible may help prevent some of the complications of this disease. One of the most important ways you can protect your kidneys is by managing your blood pressure.

Renal agenesis

Renal agenesis

Renal agenesis is a condition in which a newborn is missing one or both kidneys. Unilateral renal agenesis (URA) is the absence of one kidney. Bilateral renal agenesis (BRA) is the absence of both kidneys.

Both types of renal agenesis occur in fewer than 1 percent of births annually. Fewer than 1 in every 1,000 newborns has URA. BRA is much rarer, occurring in about 1 in every 3,000 births.

The kidneys perform functions that are necessary for life. In healthy people, the kidneys:

• produce urine, which removes urea, or liquid waste, from the blood
• keep a balance of sodium, potassium, and other electrolytes in the blood
• supply the hormone erythropoietin, helping red blood cell growth
• produce the hormone renin to help regulate blood pressure
• produce calcitriol, also known as Vitamin D, which helps the body absorb calcium and phosphate from the GI tract

Everyone needs at least part of one kidney to survive. Without either kidney, the body cannot remove waste or water properly. This accumulation of waste and fluid can offset the balance of important chemicals in the blood, and leads to death without treatment.

What are the signs and symptoms of renal agenesis?

Both types of renal agenesis are associated with other birth defects, such as problems with the:

• lungs
• genitals and urinary tract
• stomach and intestines
• heart
• muscles and bones
• eyes and ears

Babies born with URA may have signs and symptoms at birth, in childhood, or not until later in life. Symptoms can include:

• high blood pressure
• poorly working kidney
• urine with protein or blood
• swelling in the face, hands, or legs

Babies born with BRA are very sick and do not usually live. They typically have distinct physical features that include:

• widely separated eyes with skin folds over the eyelids
• ears that are set low
• a nose that is pressed flat and broad
• a small chin
• defects of the arms and legs

This group of defects is known as Potter Syndrome. It occurs as a result of reduced or absent urine production from the fetal kidneys. The urine makes up a large part of the amniotic fluid that surrounds and protects the fetus.

Who is at risk for renal agenesis?

Risk factors for renal agenesis in newborns appears to be multi-factorial. This means that genetic, environmental, and lifestyle factors combine to create a person’s risk.

For example, some early studies have linked maternal diabetes, young maternal age, and alcohol use during pregnancy to renal agenesis. More recently, studies have shown prepregnancy obesity, alcohol use, and smoking to be linked to renal agenesis. Binge drinking, or having more than 4 drinks over 2 hours, during the second month of pregnancy also increases risk.

Environmental factors may also result in kidney defects like renal agenesis. For example, maternal medication use, illegal drug use, or exposure to toxins or poisons during pregnancy may be factors.

What causes renal agenesis?

Both URA and BRA occur when the uretic bud, also called the kidney bud, fails to develop at an early stage of fetal growth.

The exact cause of renal agenesis in newborns is not known. Most cases of renal agenesis are not inherited from the parents, nor do they result from any behavior by the mother. Some cases, however, are caused by genetic mutations. These mutations are passed on from parents who either have the disorder or are carriers of the mutated gene. Prenatal testing can often help determine if these mutations are present

Diagnosing renal agenesis

Renal agenesis is typically found during routine prenatal ultrasounds. If your doctor identifies BRA in your child, they can use a prenatal MRI to confirm the absence of both kidneys.

Treatment and Outlook

Most newborns with URA have few limitations and live normally. The outlook depends on the health of the remaining kidney and the presence of other abnormalities. To avoid injuring the remaining kidney, they may need to avoid contact sports when they’re older. Once diagnosed, patients of any age with URA need to have their blood pressure, urine, and blood tested annually to check kidney function.

BRA is typically fatal within the first few days of a newborn’s life. Newborns usually die from underdeveloped lungs shortly after birth. However, some newborns with BRA survive. They must have long-term dialysis to do the work of their missing kidneys. Dialysis is a treatment that filters and purifies the blood using a machine. This helps keep your body in balance when the kidneys can’t do their job.

Factors such as lung development and overall health determine the success of this treatment. The goal is to keep these infants alive with dialysis and other treatments until they grow strong enough to have kidney transplants.

Prevention

Since the exact cause of URA and BRA is not known, prevention is not possible. Genetic factors can’t be changed. Prenatal counseling can help prospective parents understand the risks of having a baby with renal agenesis.

Women can lower the risk of renal agenesis by reducing exposure to possible environmental factors before and during pregnancy. These include use of alcohol and certain medications that can affect kidney development.

Early signs of Stroke

Strokes occur due to problems with the blood supply to the brain: either the blood supply is blocked, or a blood vessel within the brain ruptures, causing brain tissue to die. A stroke is a medical emergency, and treatment must be sought as quickly as possible.

Stroke Warning Signs:-

• Sudden numbness or weakness of the face, arm or leg, especially on one side of the body
• Sudden confusion, trouble speaking or understanding
• Sudden trouble seeing in one or both eyes
• Sudden trouble walking, dizziness, loss of balance or coordination
• Sudden, severe headache with no known cause

FMGE(MCI Screening) MARCH 2012 paper

FMGE(MCI Screening) MARCH 2012 paper

1. True abt 1 hypothyroidism A/E ------- Low TSH 
2. Mickel diverticulum DxOC ???.....Tc 99 
3. Patient of CRF on dialysis, cause of death???...... 
4. HPV 8 asso. Cancer is ???.... Kaposi sarcoma 
5. MC tumor of heart???.... Secondries / myxoma* 
6. Rynaud's phenomenon???..... White blue-red 
7. Prostate can MC arise from ?------ peripheral zone 
8. Reed Sternberg cell seen in ?------ lymphoma 
9. True abt acromegaly A/E ?------ muscle hypertrophy 
10. MC complication of blood Tx ?------ febrile reaction 
11. Classical Triad of massive blood Tx ?------ Acidosis , hypothermia , coagulopathy 
12. All increase in shock A/E ?------ insulin 
13. Pulsus bifuriance is seen in A/E ?------ Mitral valve prolapsed* 
14. Zink deficiency known as ?------ acrodermatitis enetrohepatica 
15. Typhoid ulce perforate on ?------ 3 wk (Not sure of this Que) 
16. Absolute leucocytosis and rise TLC seen with ?------ diptherial infection* 
17. MC tumor of new born male is ?------ 
18. True abt cluster headache A/E ?------ Female have 3 times more risk than male 
19. Intentional tremor seen in ?------ cerebellar damage 
20. True abt hypothyroidism A/E ?------ Tachycardia 
21. ST elevation seen in A/E ?------ hypo Ca++ / hyper K+ 
22. Patient of ectopic cushing release of cortisol not true ?------ lemon stick app*/hypokalemia 
23. Schistocyte is seen in ?------ microangiopathic hemolytic anemia 
24. True abt Osteromalacia A/E ?------ higher serum Ca level 
25. Not true for osteoporosis ?------ rise SAP {serum alkaline phosphatase} 
26. True abt polycythema vera A/E ?------ increase level of Erythropoetin 
27. True abt FRC ?------ increase with age 
28. MAC infection occure when CD 4+ cell count ?------ less than <50 nbsp="" or="" span="" versa="" vise="">
29. Shrunken lung disease seen with which of following ?------ SLE 
30. Puddle sign use for Dx of ?------ ascites 
31. Last cardiac marker to rise in MI ?------ LDH 
32. Liver biopsy C/I in ?------ Liver abscess 
33. Amebic liver abscess dx by ?------USG 
34. Rise in glucocorticoids lead to which of this condition?------ decrease inflammatory condition 
35. True for A/E at high altitude ?------ rupture of capillary/alveoli** 
36. Normal healthy person on alcohol consumption produce sever vomiting with persistant bleed in vomiting what is Dx ?------ mallory weiss syndrome 
37. Scleroderma induce hypertension  ACE inhibitor** 
38. To prevent gas gangrene ?------ hyperbaric O2 therapy / proper debridement 
39. Gold stand DxOC for Celiac disease  biopsy 
40. False about turner syndrome A/E ?------ female height less than 140 cm/ only seen in female 
41. Individual with fasting glucose level 104 gm% and and 75 gm oral glucose measure after 2 hr reading is 111 gm % what is Dx ?------ he is normal / has impaired glucose tolerance 
42. Renal papillary necrosis cause by A/E  choronic tobacco use 
43. Most common cause of Addison di 
44. Tremor start in alcohol withdrawal within ?------ 12 hr/ 12-24 hr/ 48-72 hr 
45. Patient with subnephrotic protinuria , low serum C3 complement level what is Dx?------ membrabous GN/ lupus nephritis 
46. True abt Achalasia cardia A/E ?------ LES pressure decrease in menometory 
47. Patient with FVC is 90% and FEV1/FVC is 86% , O2 saturation on exercise drop from 91% to 86 % dx ?------ Primary Pulmonary hypertension 
48. TB joint/synovium, synovial fluid finding of ?------ increase protein, decrease sugar......incerease lymphocyte 
49. All of following show shrunken kidney except ?------ 
50. Patient with blood in urine with low serum albumin ..(other symptoms were given) , what is propable diagnosis for this ?------ FSGN/Minimal change disease/MPGN/ 
(in med Que some other related subs question also added) 
Surgery 
1. Osler shellen regimen use for?------ Appendicular lump 
2. FANC use for A/E ?------follicular ca of thyroid 
3. In which breast cancer would u like to o biopsy of opposite breast ?------ lobular carcinoma 
4. Which of following has high risk of invasive breast cancer?------ apocrine metastasis* 
5. Splenic angle asso with A/E ?------ ileo-cecal junction* 
6. MC tumor of appendix ?------Argentaffinoma 
7. Undesending testis is ?------ All of above (sterility/ torsion/ infertility) 
8. Newborn present with Congenial inguinal hernia what is next best step ?------ conservative/ operate immediately / wait till 3 year than sx/ 
9. Marjoline ulcer ?------ squamous cell carcinoma 
10. Palmer surface in Burns - 1% 
11. Fluid used in burn managemanet - Ringer Lactate 
12. Escerectomy is require for which type of burn ?------ deep burn (subcutaneous ) 
13. Circumcision protect from ?------ all of above 
14. Which of following prevent risk for carinoma of penis ..... circumcision 
15. True abt obstructive jaundice A/E ?------ enlarge gall baldder 
16. Most silent Renal stone ?------ Staghorn stone (Not sure for this Que)
OBG (25 ques) 
1. H.mole MC metastasis to ?------ Lung 
2. Rxoc for Chorioncarcinoma ?------ chemotherapy 
3. Which of these infection not tx congenital ?------ adenovirus 
4. During Suction and Evacuation uterus perforate what would u do?------ immediate leprotomy* 
5. Percentage of Infertility for couple than unexplained ?------ 
6. Risk of rupture of LSCS ?------ 1-4 % 
7. OCP protect against A/E ?------ Cancer cervix 
8. External cephalic version is C/T for A/E ?------ sever breech 
9. Pre-natal cause of death of infant ?------ Birth injury * 
10. MCC of 1st Trimester abortion ?------ chromosomal anomaly 
11. Longest fetal diameter ?------ mentovertical 
12. At time of ovulation, true statement is ?------ 1st polar body release 
13. On USG nuchal transulence is seen in A/E ?------ fetal infection/down syndrome/ cardiac defect/ 
14. Late decleration of fetus is seen with ?------ fetal anoxia 
15. MC type of twins ?------ Dichorionic diamniotic (dizygotic twin) monochorinic diamniotic (monozygotic twin)* 
16. True abt placental separation A/E ?------ decrease in fundal height 
17. test use for sperm dx motility or morphology?------ (not sure for motlity) 
18. Correct sequence of lochia. ?------ Rubra ,serosa-alba 
19. Fetal hemetopoetic in liver start at ?------ 12 week (something of fetal hematopoetic Question not sure this one.) 
20. Uterine artery is brach of ?------ internal ileac artery 
21. Cu T 380 A life span if ?------ 10 year 
22. Acyclical vaginal bleeding knowns as ?------ meterorrhagia 
23. Failure rate of sterilization by pomeroy method ?------ 0.4 
OPTHALMOLOGY (25 questions were ask in paper) 1. Max. Visual impaire seen with??? post. Sub scapular cataract 
2. 1st line Rx for sever OAG???...... pilocarpine* latanoprost* 
3. Trabeculotomy mostly sefull for ???...….ACG (or vise versa) 
4. Keratometer measure???......corneal curvature 
5. Recurrent corneal erosion seen with???........Corneal dystrophy 
6. Drug use for dendritic ulcer A/E???........corticosteriod 
7. Which CN paly cause Exposure keratitis???......CN 7{facial nerve} 
8. Coroiditis mostly asso with???.....RA 
9. Micropsia, metamorphopsia, seen with which of following???.... post uveitis 
10. Cobble stone papille seen in???........Spring catarrh{vernal keratocunj.} 
11. Drug use for vernal keratoconjuctivitis???.......Olonpatadine 
12. Post.stypyloma seen in ???......Degenerative mypoia 
13. Excessive accommodation produce???…………. Peudomyopia 
14. Neovascularization 1st occur at???.......disk…..retina…coroid….iris??? 
15. Altitudinal hemianopia seen with???........AION 
16. Tobacco amblyopia treated by???……..cobalamine (B12) 
17. 6th CN palsy eye deviate toward right side..which is ture???....head turn to right 
18. Visual defect with pituitary tumor is???.......Bitemporal hemoanpia 
19. M/C/C of vitreous hemorrhage in young adult???......Trauma 
20. Rhegmatogous retinal detachment seen A/E???...... DM 
21. Orbital cellulitis M/C asso with… Ethmoiditis (not sure this Q was in paper) 
22. Viual aquity of an eye is best described as
PSM 1. Millennium goal achieved by???.......2015 
2. Which of these has poor source of Vit D???.......Fish fat,egg.olive oil. 
3. Acceptable noise in Hospital (In db)???......40-60 db/15-30db/35-50db 
4. Weight gain in Pregnancy for indian women???.....12kg*/10 kg *( 11 kg is not in Option) 
5. Residual chlorine is measure by???.....Chloroscope 
6. 6 month old infant present with breathing rate of 40/min Dx will be???......pneumonia other option are /sever, /mild,/no pneumonia 
7. IMR does not include???Perinatal mortality 
8. Direct cause of Maternal death is???...... Hemorrhage 
9. M/C/C of MMR is???.....Hemorrhage (yes this Que is asked twice ) 
10. Female MPW can Dx A/E ??? .Chronic Renal failure 
11. How much meal in gm is given in mid day meal program????........50 ,/ 100,/ 150,/ none??? (75 gm is not in option) 
12. Cafeteria approach use for ???........ family planning programe 
13. carrier state not possible -tetnus 
14. Disease Tx by mite???.....scrub typhus 
15. One community block for???.......100 village and 100000 population 
16. In normal distribution Mean + 2SD will cover ???.......95.4 
17. True abt breast milk A/E???..... max production at 12 month of lactation 
18. Indicator of water contamination???......E.coli 
19. Reverse cold chain use for ???.........Transport of polio stool sample from PHC to CHC 
20. Measles vaccine post exposure can be use up to ???.....3 days (not sure this Q was in paper) 
21. Soiling Index use for ???.....Air pollution 
22. Sanitary line should be away minimum of ???.......10 m,100m, 
23. Which is not the element of health care system??? 
24. Triad of Epidemiology ???.......- Agent , Host, Environment ,… distribution, determination, 
25. A case of Filariasis A/E??? - Oncocerca Volvulus /Brugiya Malayi/ Timori/ / W. Bancrofti 
26. BMI defined as???..... Wt/Ht2 X 100 (yes there is 100 given in option that's why many student make mistake ) 
27. Total fertility -----gross reproductive rate 
28. Chronic S/E of newer Drug is access in which pahse of clinical trial ? phase 4 
29. kata thermometer-cooling power of air (not sure this Q was in paper) 
Radiology 
1. Radioisotopes use for thyroid destruction???........I131 
2. Pencil and cup deformities/apperance seen in??? 
3. Adder head app seen in???......Ureterocele 
4. Mickel diverticulum DxOC ???.....Tc 99 (not sure this Q was in paper) 
5. Dxoc for interstitial lung disease?------ HRCT 
6. mc radiosensitive tumor -seminoma 
7. Bone in bone is seen in ?------ osteopetrosis* 
8. Three cup urine test use for Dx ?------ 
PHYSIO 
1. T Cells develop in - Thymus 
2. Normal plasma osmolarity?------ 280-290mosmol/L 
3. LtB4 is made by which pathway?------ ----LOX 
4. Hering –Breuer reflex is ?------prevent further inflation of lung 
dermatology 
1. Acantholytic cell seen in ?------phemphigus valgaris 
Anat 
1. Urogenital diaphram is made of A/E?------ 
Anasthesia 
1. False about propofol ?------ 
2. Local anesthetic mechanism of action ?------ block Na+ channel 
FSM 
1. Diatoms are seen in which type of death - Drowning 
2. Last muscle to under go …………?------ 
Psychi 
1. A child always fights in school, has deciplinry problems in school - Conduct Disorder * 
2. Death of near one lead to A/E ?------Anger* 
3. True abt bulimia nervosa A/E ?------ menorrhegia
Pharma(25-30 questions) 1. Drug use oral for Pseudomemranous colitis ?------ vancomycin 
2. Which of following is nephrotoxic and ototoxic ?------ vancomycin 
3. Jet black tongue is seen in ?------ cocaine abuse 
4. True abt Atropine A/E ?------ bronoconstriction 
5. Busulphan S/E is ?------ pulmonary fiberosis 
6. Anti cancer drug most commonly asso with vomiting ?------cisplatin 
7. Mechanism of action of sildenafil ?------ 
8. Infant present with salivation, lacrimation , diarrhea what is most likely poisoing ?------ organophosphate 
9. Antidote of cyanide poisoing. ?------sodium thiosulphate 
10. Drug used in management of NSAID induced Ulcer ?------PPI 
11. For Anti diarrheal drugs which is true?------ 
12. True chronic S/E of of following drugs A/E?------ amiodarone with phosphate deposition in tissue 
13. True abt phynylephrine?------ 
14. Drug use for parkinson disease?------ Promethazine 
15. Drug use for prostate cancer ?------ Finasteride 
16. Thiazide diuretic can lead to ?------ hypercalcemia 
17. Drug act by inhibiting RNA sequence ?------ 
18. Drug cause Retro peritoneal fibrosis?------ Bromocriptin 
19. Which one is action of B-agoinst A/E?------ 
Orthopedics 
1. Ant Shoulder dislocation - Axillary Nerve Injury 
2. Nerve damage in # of Shaft of Humorous ???.......Radial nerve 
3. Cock up splint seen in???..... Radial nerve damage 
4. In # of tibia which nerve damage ???.... common peroneal nerve* 
5. Ulner nerve damage lead to ???........claw finger hand 
6. True abt Neurapraxia is ???...... Physiological nerve conduction block 
7. Avascular necrosis seen in - Talus 
8. Tendon reflex carry by – type 1a fiber (something typical question was there not sure this one.) 
ENT 
1. Eustesian tube open at ?------ behind ferior meatus 
2. Rhynophyma is ?------ hypertrophy of …………* 
3. Bacterial abscess from ear infection cause by ?------ strep pneumococcus* 
4. Pneumatic bone is ?------ frontal sinus 
5. Safety muscle of larynx is?------ post cricoarytenoid 
6. Caloric test stimulates ?------ lateral semicircular canal 
7. For Tunnig fork test frequency use is ?------ 512 Hzs
Microbiology 1. gas gangrene is cause by ?------ Clostridium welchi 
2. Botulinum toxin ?------ presynaptic action 
3. Pseudomembraeous colitis(rather than pseudo they… wrote something else ) cause by ?------ Clostridium defficile 
4. Dermatophytones cause infection of ?------ All of above (hair/ nail/skin) 
5. Paule bannel test use for dx of ?------ infectious mononeucliosis 
6. Lag phase of bacterial life cycle represent ?------ 
7. Which is precipitation test ?------ Weil-felix reaction 
8. Negri body is seen in ?------ Rabies 
Biochem (about 15-17 question) 1. B1 deficiency is Dx by ?------ 
2. Insulin stimulate which enzyme ?------ Glycogen synthase 
3. Alpha KGD and PHD deficiency asso with ?------ beriberi 
4. Which of this is collagen synthesis defect ?------ all four option are collagen defect (scurvy/ osteogenic imprefecta/ other two) 
5. AA is use for Purine synthesis ?------ gycine** 
6. Precursor of Dopamine?------ tysosine