Children with Renal Abnormalities Flashcards

1
Q

What do all sex cells (sperm and oocytes) start out as?

1 - haploid gametes
2 - diploid gametes
3 - diploid zygotes
3 - haploid zygotes

A

1 - haploid gametes

  • half the number of a full diploid cell of chromosomes
  • so 23 in total
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2
Q

What are the 3 layers of the trilaminar germ disk?

A
  • ectoderm (top)
  • mesoderm (middle)
  • endoderm (bottom)
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3
Q

List a few things that the 3 layers of the trilaminar germ disc will go on to develop?

A
  • ectoderm = skin, eyes, brain
  • mesoderm = muscle, RBCs, heart
  • endoderm = GIT tract, lungs, pancreas
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4
Q

Stem cells will develop into a specific tissue following cell signalling, which is facilitated through transmembrane receptors. What are the 4 types of transmembrane receptors?

1 - Ion channels, steroid receptors, enzyme linked receptors, intracellular receptors
2 - Ion channels, GPCR, toll like receptors, intracellular receptors
3 - Ion channels, GPCR, enzyme linked receptors, intracellular receptors
4 - toll like receptors, GPCR, enzyme linked receptors, intracellular receptors

A

3 - Ion channels, GPCR, enzyme linked receptors, intracellular receptors

  • Ion channels (cholinergic nicotinic receptors)
  • GPCR (alpha/beta adrenoreceptors)
  • enzyme linked receptors (insulin receptors)
  • intracellular receptors (steroid receptors)
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5
Q

There are 4 main methods cells are able to communicate with one another. What do each of these mean:

1 - endocrine
2 - autocrine
3 - gap junctions
4 - paracrine

A

1 - endocrine (signals are sent in the blood)
2 - autocrine (self signalling cell)
3 - gap junctions (physical contact)
4 - paracrine (close cells are signalled)

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6
Q

What are transcription factors?

1 - protein able to allow membrane receptors to let in molecules
2 - proteins within cell membranes able to repel pathogens
3 - proteins able to bind and promote DNA
4 - proteins able to bind and inhibit or promote DNA

A

4 - proteins able to bind and inhibit or promote DNA

- they can promote or inhibit a gene from DNA to mRNA

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7
Q

Once a protein has been made following gene coding for that protein, transcription and the protein being put together, it this the way the protein will remain?

A
  • no
  • proteins can be modified in post-translation, but it will affect the proteins shape, activity and interactions in the body
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8
Q

Transcription factors are specific proteins that are able to bind with DNA. They can promote or inhibit a gene from DNA to mRNA. Hox proteins are a group of transcription factors, what is the role of Hox proteins?

A
  • conserved through evolution
  • embryonic development by activating and repressing genes
  • important in body pattern and formation
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9
Q

In addition to transcription factors that can affect if a gene is active or inactive, ultimately determining if a protein is created, can proteins be altered once they have been synthesised?

A
  • yes
  • can undergo methylation, hydroxylation etc…
  • all will change a proteins shape, function and interactions with other proteins
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10
Q

What are WNT or wingless genes?

A
  • vertebrae version of the wingless fly gene

- if the gene is knocked out or mutated then the fly will develop without wings

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11
Q

WNT or wingless genes are genes that have been identified to be crucial in flys. If the gene is knocked out or mutated then the fly will develop without wings. WNT signals bind with frizzled transmembrane cell surface receptors (GPCR) and are involved in what?

A
  • body patterning, cell fate and cell proliferation and migration
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12
Q

WNT or wingless genes are genes that have been identified to be crucial in flys. If the gene is knocked out or mutated then the fly will develop without wings. WNY signals bind with frizzled transmembrane cell surface receptors (GPCR) and are involved in body patterning, cell fate and cell proliferation and migration. If there is a mutation or impaired function of WNT genes, what can this cause in humans?

A
  • limb, eye, genitourinary and bone development disorders

- resulting in congenital abnormalities

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13
Q

What are notch proteins?

A
  • important cell signalling proteins
  • crucial for cell differentiation and maintaining stem cell population (sperm)
  • crucial for T cells, neural, endothelial and cardiocytes
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14
Q

What are hedghog proteins?

A
  • family of proteins crucial for body patterning roles
  • able to bind and signal cells to perform different functions
  • how it affects the cell depends on proliferation stage of the cell, dosage of hedghog receptors and cell type
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15
Q

Hedgehog proteins are a family of proteins crucial for body patterning roles and are able to bind and signal cells to perform different functions. How hedgehog affects the cell depends on proliferation stage of the cell, dosage of hedgehog receptors and cell type. If there is any mutation of defect in the genes encoding these proteins, what can this cause in humans?

A
  • sonic hedgehog homologue is most important
  • important in neural, bone, limb and kidney development; muscle, patterning; and lung branching
  • important for special sense organs
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16
Q

What are Fibroblast growth factors (FGF)?

A
  • cell signalling proteins involved in modulating adult tissues
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17
Q

Fibroblast growth factors (FGF) are cell signalling proteins involved in modulating adult tissues. What are the main roles they are involved in?

A
  • limb and neural development
  • angiogenesis
  • mesoderm induction
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18
Q

What is transforming growth factor β (TGF-β)?

A
  • highly pleiotropic protein called a cytokine

- important in wound healing, angiogenesis, immunoregulation, cancer and DNA transcription

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19
Q

Which part of the trilaminar germ disc does the urinary tract derive from?

1 - ectoderm
2 - mesoderm
3 - endoderm

A

2 - mesoderm

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20
Q

The urinary tract derives from the mesoderm. A condensation of cells forms in the abdomen either side of the aorta, which will then go on to develop the urinary tract and reproductive system. What is this called?

1 - urogenital ridge
2 - urogenital cord
3 - mesonephric duct
4 - para-mesonephric duct

A

1 - urogenital ridge

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21
Q

The urinary tract derives from the mesoderm. A condensation of cells forms in the abdomen either side of the aorta, which will then go on to develop the urinary tract and reproductive system, called the urogenital ridge. This will contain 2 main parts, what are they called?

1 - nephrogenic cord and the para-mesonephric duct
2 - gonadal ridge and mesonephric duct
3 - para-mesonephric duct and gonadal ridge
4 - nephrogenic cord and the gonadal ridge

A

4 - nephrogenic cord and the gonadal ridge

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22
Q

There are 3 structures involved in kidney development that grow from the intermediate mesoderm in an anterior to posterior sequence. Label the image below with the 3 structures, using the labels below:

  • metanephros
  • pronephros
  • mesonephros
A

1 - pronephros
2 - mesonephros
3 - metanephros

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23
Q

There are 3 structures involved in kidney development that grow from the intermediate mesoderm in an anterior to posterior sequence

  • pronephros
  • mesonephros
  • metanephros

Of the 3 structures, which one will ultimately go on to develop the upper urinary system and what happens to the other 2 structures?

A
  • metanephros = upper urinary system (kidneys)
  • pronephros = week 3 forms primitive non functional kidneys before disappearing due to evolution
  • mesonephros = disappears due to evolution
24
Q

From the 3 structures below that are involved in kidney development, as below, which structure at week 4 forms the first functional form of the kidneys?

  • pronephros
  • mesonephros
  • metanephros
A
  • mesonephros

- develops caudally (towards the tail) to the pronepheros

25
Q

At week 4 the first functional form of the kidneys forms from the mesonephros. There are ducts that are formed called mesonephric ducts (Wolffian ducts), epithelialined tubes that form in the intermediate mesoderm and extend caudally to the cloaca (last part of the primitive digestive tract). As the embryo continues to develop, what will these mesonephric ducts develop into?

1 - urethra
2 - bowmans capsules
3 - blood vessels
4 - kidneys

A

2 - bowmans capsules

  • renal corpuscles develop from mesonephric tubules into Bowmans capsule
  • capillaries from the dorsal aorta form the glomerulus
  • lateral end of the mesonephric duct discharges into where the bladder will form
26
Q

The mesonephros starts to produce urine at what week?

1 - week 3
2 - week 4
3 - week 5
4 - week 6

A

4 - week 6

27
Q

The ureteric bud develops from the mesonepheric duct, which will go on to form the adult kidney is formed from the mesonepheros, it grows into the surrounding intermediate mesoderm inducing cells in that region to form what over the ureteric bud?

1 - metanepheros cap at week 12
2 - metanephric cap at week 5
3 - metanephric cap at week 12
4 - metanepheros cap at week 5

A
  • metanephric cap formed from the metanephros

- forms at week 5

28
Q

The metanephros will ultimately form the kidneys that is attached to the mesonepheric tubule in the form of the uteric bud. The ureteric bud and metanephric cap then begin to develop what?

A
  • ureteric bud forms collecting tubules and the ureter

- cells of the metanephric cap form nephrons that then link to the collecting tubules

29
Q

As the ureteric bud forms collecting tubules and the cells of the metanephric cap form nephrons that then link to the collecting tubules, blood capillaries begin to form through angiogenesis in the Bowmans capsule from what blood vessel?

1 - dorsal aorta
2 - anterior aorta
3 - posterior aorta
4 - superior aorta

A

1 - dorsal aorta

30
Q

As the ureteric bud forms collecting tubules and the cells of the metanephric cap form nephrons that then link to the collecting tubules, blood capillaries begin to form through angiogenesis from the dorsal aorta into the Bowmans capsule. As the kidneys continue to develop, what will these capillaries then become?

A
  • glomerulus
31
Q

When do functional renal units begin producing urine normally?

1 - week 12
2 - week 16
3 - week 20
4 - week 28

A

1 - week 12

32
Q

Which cavity do the kidneys develop in during embryology?

A
  • within the pelvic cavity

- then migrate to the lumbar region until they reach T12-L3

33
Q

As the kidneys migrate from the pelvic cavity to the lumbar region of the abdominal cavity, where do the kidneys receive their blood supply from?

A
  • common iliac artery or the aorta

- these will eventually become the renal arteries from the aorta

34
Q

What is the cloaca?

A
  • a common chamber into which some or all of the digestive, urinary, and reproductive tracts discharge their contents during embryological development
35
Q

The cloaca is a common chamber into which some or all of the digestive, urinary, and reproductive tracts discharge their contents during embryological development. The cloaca will develop by week 4 and be separated by urorectal septum and form what 2 structures?

A

1 - urogenital sinus

2 - dorsal anal canal

36
Q

The cloaca is a common chamber into which some or all of the digestive, urinary, and reproductive tracts discharge their contents during embryological development. The cloaca will develop by week 4 and be separated by urorectal septum and form the urogenital sinus and the dorsal anal canal. The urogenital sinus can further develop into 3 structures. What are these structures?

A

1 - top part is the biggest and becomes the bladder
2- middle part forms the urethra in the female pelvis and the prostatic and membranous
urethra in the male
3 - lowest part forms the penile urethra in the male and the vestibule in the female

37
Q

What kidney pathology can we see in the image below?

1 - supernumerary kidneys
2 - polycystic kidney disease (PKD)
3 - horse shoe shaped kidney
4 - kidney tumour

A

1 - supernumerary kidneys

- additional kidney is present, often accompanied by an additional ureter

38
Q

Supernumerary kidneys is when an additional kidney is present, often accompanied by an additional ureter. What is the most common cause of this in renal development?

1 - incomplete development of the metanepheros
2 - failure of urogenital ridge to develop
3 - incomplete division of the ureteric bud
4 - incomplete development of the mesonepheros ducts

A

3 - incomplete division of the ureteric bud

39
Q

What are kidney cysts?

A
  • round fluid filled pouches that form on or in the kidneys

- commonly caused when developing nephron fails to connect to collecting tubule

40
Q

Kidney cysts are round fluid filled pouches that form on or in the kidneys. Why do kidney cysts form?

A
  • developing nephrons fail to connect to a collecting tubule in development,
  • collecting ducts fail to develop and fluid fills the space
41
Q

Kidney cysts are round fluid filled pouches that form on or in the kidneys. What is the term used when someone is born with lots of cysts on their kidneys?

1 - supernumerary kidneys
2 - polycystic kidney disease (PKD)
3 - horse shoe shaped kidney
4 - kidney tumour

A

2 - polycystic kidney disease (PKD)

42
Q

Polycystic kidney disease (PKD) is a term used to describe when someone is born with lots of cysts on their kidneys. Is this a dominant or recessive disease?

A
  • can be both
43
Q

Polycystic kidney disease (PKD) is a term used to describe when someone is born with lots of cysts on their kidneys and can be dominant or recessive disease. Is the dominant or recessive form more progressive?

A
  • recessive PKD is more progressive and can cause renal failure in children
44
Q

What is renal agenesis?

A
  • a = missing
  • genesis = development of
  • lack of development of a kidney during embryological development
45
Q

Renal agenesis is a lack of development of a kidney during embryological development. What is the most common cause of this?

A
  • imbalance of fluid in the amniotic sac
  • if urine is not produced by developing kidneys amniotic fluid drops
  • drop in amniotic fluid is called oligohydramnios
46
Q

Renal agenesis is a lack of development of a kidney during embryological development, leaving a child with one kidney. Does this always causes symptoms for the child?

A
  • no
  • can be symptom free as other kidney compensates
  • BUT if no kidneys develop this is lethal
47
Q

As the kidneys ascend from the pelvic cavity up to the abdominal artery the renal arteries will eventually form. Accessory arteries can also form. What are the 2 most common supplementary arteries that can form?

1 - superior polar and medial polar renal artery
2 - medial polar and inferior polar renal artery
3 - lateral polar and inferior polar renal artery
4 - superior polar and inferior polar renal artery

A

4 - superior polar and inferior polar renal artery

- not dangerous as long as kidneys receive sufficient blood supply

48
Q

As the kidneys move from the pelvic cavity to the abdominal cavity to the lumbar region they can rotate, presenting as an abnormal finding, depicted in the image below. This can cause a number of problems, one of which is where the kidneys face each other and become connected. What is this called?

1 - supernumerary kidneys
2 - polycystic kidney disease (PKD)
3 - horse shoe kidney
4 - kidney tumour

A

3 - horse shoe kidney

- generally does not cause symptoms, but can increase risk of UTIs

49
Q

As the kidneys move from the pelvic cavity to the abdominal cavity to the lumbar region they can rotate, presenting as an abnormal finding. This can cause a number of problems, one of which is where the kidneys face the wrong way and get trapped on what blood vessel?

1 - inferior mesenteric artery
2 - superior mesenteric artery
3 - medial mesenteric artery
4 - abdominal aorta

A

1 - inferior mesenteric artery

50
Q

The proximal tubules are most metabolically active and lead to reabsorption what % of the filtrate?

1 - 30 to 90% of the glomerular filtrate.
2 - 10 to 90% of the glomerular filtrate.
3- 60 to 90% of the glomerular filtrate.
4 - 100% of the glomerular filtrate.

A

3 - 60 to 90% of the glomerular filtrate.

51
Q

The proximal tubules are most metabolically active and lead to reabsorption what % of the Na+ and Cl-?

1 - 20%
2 - 40%
3 - 55%
4 - 70%

A

4 - 70%

52
Q

The proximal tubules are most metabolically active and lead to reabsorption what % of H+ is excreted?

1 - 20%
2 - 40%
3 - 55%
4 - 90%

A

4 - 90%

53
Q

If there is no need to assess renal function through blood biochemistry, how do renal problems generally get detected?

A
  • random finding on ultrasound, CT or MRI
54
Q

Aldosterone and anti-diuretic hormone both do what in the kidneys?

A
  • aldosterone = increase Na+ and H20 reabsorption
  • ADH = H2O reabsorption
  • K+ is excreted in both
55
Q

Bladder defects may occur, such as exstrophy. What is exstrophy?

1 - bladder contents leak into abomen
2 - skin of lower abdomen is not formed properly and bladder appears outside the skin
3 - bladder does not form
4 - urethra does not form

A

2 - skin of lower abdomen is not formed properly and bladder appears outside the skin