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GP
by Grace Poole0 views·Updated Dec 30, 2021

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  1. Okay, so let's move on to question two.
  2. So Joseph, a tear a boy is brought into the GP by his dad,
  3. who describes that Jason's urine has been brown in colour for the past day.
  4. On questioning, he mentions that Joseph has been urinating less frequently,
  5. but it doesn't appear to be foul smelling.
  6. He mentions that last week Joseph had a cold with a sore throat and enlarged tonsils.
  7. On examination, you notice mild swelling around Joseph's eyes and in his fingers.
  8. What is the most likely diagnosis that explains Joseph's presentation?
  9. Is it a l Port syndrome?
  10. Be post streptococcal glow Maryland arthritis
  11. See minimal change disease
  12. D I G encephalopathy, or E Goodpasture syndrome?
  13. And I'm going to give you guys 45 seconds for this one.
  14. Okay, so the answer here is B. This is post streptococcal glam era lo nephritis.
  15. So this is one of the annoying ones.
  16. It's either sort of, you know, the diagnosis or you don't.
  17. But don't worry if you got it wrong,
  18. because hopefully by the end of this presentation you will, which is fantastic.
  19. So just going through the question.
  20. So you've got Joseph's a two year old has been brought in.
  21. So we've got his to his urine.
  22. Been brown in colour for the past day on questioning,
  23. you mentioned that Joseph has been urinating less frequently.
  24. So we've got some oligarch area
  25. brown in colour, I'd say. Sounds like he material.
  26. Um, but the urine doesn't appear to be foul smelling,
  27. so that kind of excludes that as a u T i to me.
  28. So at the moment, we know Joseph got Algeria,
  29. he materia We know that last week he had a cold and okay or some sort of infection,
  30. and we know that now he's also got some swelling or oedema
  31. and it seems to be around his eyes and his fingers,
  32. so most likely peripheral oedema.
  33. So what we've got is human Suria, peripheral oedema, Allah,
  34. Gary A and a history of some sort of throat infection a week ago.
  35. So all of this is pretty consistent with
  36. what we call post streptococcal glam Marilyn arthritis,
  37. and we'll go through a bit more.
  38. But the other answers we've got here are al port syndrome.
  39. So our ports and drones as an example of one of our nephritis syndromes,
  40. it usually presents with sort of a sensory in your yeah
  41. sensory neural hearing loss as well as acute kidney injury.
  42. And it's a congenital syndrome is usually caused by deficit in Type four collagen,
  43. which is responsible for sort of both the basement membrane
  44. of the kidney and of the cochlear and the ear.
  45. Now we've got minimal change disease,
  46. which is one of our nephrology attic syndromes,
  47. so you wouldn't necessarily get this key material.
  48. We've got a I G A. Nephew. Apathy.
  49. Now, if you've ever done a question bank or have been through some med school exams,
  50. usually there is always a glimmer really nephritis question,
  51. which requires you to differentiate between post streptococcal,
  52. primarily nephritis and i g a N F.
  53. Tripathi.
  54. So usually post streptococcal glomeruli nephritis happens in Children.
  55. I gene a nephew. Apathy.
  56. It tends to happen in young adults,
  57. but that's not always a differentiating feature.
  58. The main thing is that I Jennifer apathy was
  59. that both caused by sort of streptococcal infections,
  60. I Jennifer apathy tends to happen 1 to 2 days afterwards, symptoms will manifest,
  61. such as the human Suria, such as the Algeria and the peripheral oedema,
  62. whereas post synaptic glomeruli nephritis tends to happen 1 to 2 weeks later.
  63. So that's a really important differentiation in terms of time frame.
  64. Now the last is Goodpasture syndrome,
  65. which is caused by anti Guatemala basement membrane antibodies
  66. and that usually presents a sort of he mocked Isis
  67. as well as he mature in knowledge area as well.
  68. So I feel like Marilyn.
  69. Arthritis is one of the topics that is absolutely hated in renal.
  70. It's loved by nephrologist,
  71. so disliked by medical students that comes up in medical exams a lot
  72. to try and break it down.
  73. There's definitely a renal lecture that will do this a lot better than I will,
  74. but I feel like if you characterise it in terms of paediatrics,
  75. it does make it slightly easier to understand.
  76. And also it means that you only have to learn three of the particular syndromes,
  77. which is fat.
  78. So Kimberly nephritis itis means information.
  79. Neff rose Latin for the kidney.
  80. Angela Murillo refers to the glamorous, which is a part in the kidney,
  81. so what it essentially is is inflammation of the glomeruli.
  82. Listen, the kidney and inflammation usually happens due to some kind of damage.
  83. Now, in terms of like Marilyn arthritis,
  84. So that's just a broad description of basically damage to the glamorous.
  85. We tend to divide the syndromes into to
  86. either never frenetic syndrome or near frenetic syndrome.
  87. And they're divided based on where the damage is in the glamorous colas,
  88. so focusing first on a frenetic syndrome.
  89. If there is damage to the Glamorama capillary,
  90. it usually is an athletic syndrome
  91. and the main sort of characterising feature in
  92. terms of symptomology is he mature area.
  93. The other features you might get is a small amount of protein urea,
  94. but not a significant.
  95. You might get Ali G area, which is reduced urine, hypertension and oedema.
  96. But the main main thing I want you to remember is human Suria.
  97. And in paediatrics, there are 22 types of athletic syndrome to be aware of.
  98. One of them is post streptococcal glomeruli nephritis,
  99. and we'll go through in a bit more detail as well.
  100. But that was the one that we had in our question,
  101. and the other is al port syndrome,
  102. which I mentioned is where you get hearing loss associated with renal disease.
  103. And you said that that's due to a deficit in type four collagen
  104. now the other is Neff Robotic syndrome.
  105. So this is where you actually get damaged
  106. to the filtration barrier of the Gulf amaryllis.
  107. Now what happens is when you get damage to the filtration barrier,
  108. the gaps between the sort of Potter sites get bigger and the policies
  109. of structures that are sort of responsible for creating this filtration barrier.
  110. So if the gaps are bigger, that means that more can leak through.
  111. So that means proteins, which is larger.
  112. Molecules that would normally be filtered can fit through these new, bigger gaps.
  113. So you get protein your ear
  114. now because you've got protein urea and thinking about these gaps being bigger.
  115. This means that albumin can fit through the gaps as well.
  116. Albumin is quite a large molecule.
  117. Normally, it can't fit through the filtration barrier.
  118. However, obviously with damage, wider gap albumin goes into the urine.
  119. So that means in the circulation you get a hypo album anaemia.
  120. Now, albumin is usually got osmotic properties,
  121. so that means if you get low albumin, you get more fluid accumulating.
  122. So you get this sort of peripheral oedema as well,
  123. and I always forget this is a cause of peripheral oedema in adults and Children.
  124. It's easy to think of sort of heart failure as a cause of peripheral oedema.
  125. But high pay album anaemia anaemia is really big ones you have to exclude as well.
  126. And the most common type of necrotic syndrome that happens in Children is minimal.
  127. Change disease,
  128. so minimal change disease.
  129. And you can see this sad licking child on the right usually tends to be idiopathic.
  130. However,
  131. it can be induced by medications the particular
  132. medication that's been listed as Reform Person,
  133. which is used in the management of TV.
  134. And it is associate with with Hodgkin's lymphoma as well.
  135. And the reason it's called minimal change disease is because actually,
  136. you don't get any changes on light microscopy,
  137. which is the first line if you take a biopsy
  138. to sort of investigate what might be causing the glow.
  139. Maryland arthritis,
  140. however, on electron microscopy,
  141. which is obviously has a high resonance and shows more detail,
  142. you can see the effacement of Potter sites
  143. so sometimes in questions,
  144. they might be quite nice to you and say that unlike microscopy,
  145. so they'll give you the story of a child
  146. that presents with sort of this peripheral a Diemer.
  147. They've got protein in their urine on a urine dipstick.
  148. However, unlike microscopy a box he was shown and they couldn't see anything.
  149. Then you're thinking minimal change disease.
  150. But the easy thing And they do put in questions about management of this
  151. is it can be managed just with a short course of prednisone alone.
  152. And if that's an effective, you might escalate to cyclophosphamide,
  153. which is actually sort of a chemotherapy agent or anti immune agent.
  154. Uh huh.
  155. So the next sort of niggling Merlin athletic syndrome to talk about is,
  156. um post streptococcal blue Maryland nephritis.
  157. So what you have? What happens if you have a group B group A beta hemolytic politic?
  158. Streptococcus infection.
  159. Now that usually tends to cause sort of tonsillitis,
  160. So usually it tends to be sort of these upper,
  161. then lower respiratory tract symptoms.
  162. Now those symptoms get better.
  163. But two weeks later, because, remember,
  164. we said the main difference between post streptococcal glomeruli nephritis
  165. and I Jennifer apathy is the timing of symptoms,
  166. so I g a tends to be about two days later.
  167. Post streptococcal glomeruli nephritis tend to be about two weeks later.
  168. You get this sort of Coca Cola coloured urine, which is the human Suria.
  169. You get protein, Yuria, a Deamer and ah, Liguria as well
  170. and the main things to be aware of.
  171. So we've spoken a little bit about the glow. Marilyn. Arthritis is.
  172. I've done it very, very basic overview.
  173. If it's a topic, you feel like you could do some more work on.
  174. Definitely go check out the renal lectures done on study Hub.
  175. But in terms of paediatrics, the main ones to remember
  176. our post streptococcal we briefly mentioned Al port syndrome and minimal change.
  177. Disease is a necrotic syndrome as well.
  178. So just moving on to our last topic,
  179. we're going to talk a little bit about NAFO blasphemers or Wilms tumours.
  180. So Wilms tumours are essentially tumours of the kidney,
  181. and they are the most common type of renal tur and Children,
  182. and they tend to happen between two and five years of age.
  183. Now what happens is you get a mutation of the W T one gene
  184. in the kidney.
  185. The W T. One gene is on chromosome 11, and it's actually a tumour suppressor gene.
  186. So if you get damaged, two wt one,
  187. both valuables you lose its you are suppressing properties,
  188. which means you get uncontrolled proliferation of something called meta and F.
  189. Rick Blast email cells.
  190. Now the metaphoric busty more cells are almost like
  191. the stem cells of the janitorial janitor urinary system,
  192. so they're responsible for producing sort of the epithelial cells,
  193. the stromal cells that all make up the kidney.
  194. So if you get uncontrolled proliferation of them,
  195. you get a really big mix of cells that form this nephew blast statement.
  196. As I said, they could be glamorous cells. They could be epithelial cells.
  197. They could be stromal cells,
  198. so usually this mutation can be sporadic,
  199. but sometimes it can occur within a syndrome.
  200. And one of the syndromes that can occur with is Wagar syndrome. So W. A G R,
  201. and that stands for basically a mix.
  202. If mix of symptoms, usually you get a Wilms tumour or the nephew blast Omer
  203. an arrhythmia, which is a failure of the Irish to develop,
  204. and they're both caused by mutation in WT one.
  205. Now W. T.
  206. One can commonly associate with pack six,
  207. and that's what forms the basis of this syndrome.
  208. So you get Guo anomalies or other structural anomalies,
  209. and you can get intellectual disability as well,
  210. which was formerly known as mental retardation when the syndrome was discovered.
  211. So a combination of this just forms Wagar,
  212. which is W T one and pack six mutation together.
  213. But the main thing for you guys to remember is how it presents.
  214. So in a question, it will be that a child's got a unilateral,
  215. palpable abdominal flank mass, and that will be the Wilms tumour.
  216. And you can see in the scan just how big these Wilms tumours can get.
  217. I mean, that one is absolutely huge
  218. now associated with that, you can get hypertension.
  219. And that's because you're getting an increase in the number
  220. of cells that are responsible for secretions within the kidney.
  221. Because, remember, the kidney secretes Renan in responses to changes in pressure.
  222. So because we've got these metaphoric blast animal cells that
  223. can form any cell that forms at the kidney,
  224. you get an increase in Renan, which leads to hypertension.
  225. So this would be an example of secondary hypertension,
  226. which is just high blood pressure due to an underlying cause.
  227. Now, if the tumour is actually causing damage to the kidney,
  228. you can sometimes get painless.
  229. You mature as well.
  230. So it's a combination of all these things.
  231. Okay, So just to summarise what we've spoken about,
  232. we've mentioned first Humility Curie mix syndrome,
  233. which we said it typically occurs due to E. Coli.
  234. So will be diarrhoea for several days and then later will
  235. develop this Coca Cola urine associated with ah Liguria as well.
  236. We said the most important thing that this is knowing the organism that causes it,
  237. which is E.
  238. Coli 015787. And just keep saying that yourself.
  239. Now we went through the glass Maryland arthritis. We recap what
  240. Marilyn arthritis was.
  241. We covered the three most common ones in paediatrics, which is al port syndrome,
  242. which is hearing loss renal symptoms.
  243. Minimal change disease, which is an example of our necrotic syndromes,
  244. which is a tree odd of Hypo album anaemia
  245. protein, Yuria and peripheral oedema.
  246. And we said about post streptococcal syndrome as well,
  247. which symptoms arise 1 to 2 weeks after infection.
  248. Then we spoke at the bottom about nafo blast, um, as a Wilms tumours.
  249. We said that their mutation in wt one they present, usually with abdominal mass,
  250. painless you materia and hypertension as well
  251. and