Case presentation polycystic kideny in pediatrics presented with convulsion

1. Yassin M Al-Saleh
Supervised by:
Dr.Aziza Al-Shehab

4.  Hussin
 chief
is 3 year old boy.
complaint : abnormal movement .
 Patient
 he
not known to have any illness before.
developed subjective fever.
 Few
hours , generalized tonic clonic
movement of the body with uprolling of the
eye.

5.  Patient
taken immediately to private hospital
while convulsing.
 Convulsion last for 15 min aborted by
diazepam .
 Temp :39.1
 Their
impression: Otitis media ,febrile
convulsion.
 Family ask for referral.
 Presented to MCH ER.

6.  No
Hx of URTI ,trauma ,rash, urinary
symptom, drug ingestion or headache.
 Perinatal:
 Product
of near term NSVD
 Stay for 6 days due to jaundice.
 Past
medical Hx: AGE at age of 6 months
 Past surgical: circumcision

7.  Developmentally
 Family
normal.
Hx:
 Consangious marriage
 1 sister 1 brother all healthy
 Mother is 32 yrs K/C of cystic disease in the
kidney .
 Father is 45 yrs healthy.
 No hx of febrile convulsion, seizure
congenital heart disease or chronic disease.

8. 
Patient looks unwell ,sleepy.
Vital sign:
 Oxygen Saturation
98%
 Heart rate
114 bpm
 Respiratory rate
30 bpm
 Tempreature
37 C
 Blood pressure 106/75 (85)

Growth parameter:
 wt:12 kg
<5th.
 Ht:95 cm
50th.
 HC: 49 cm 25-50th.


9. 
CNS: sleepy. GCS 15/15

CVS:s1+s2+o CRT<2 sec.

RS: good air entry no added sound.

Abdomen: soft no oraganomegally.

Musculoskeletal: unremarkable.

ENT: congested throat.

No skin stigmata.

10. Febrile
convulsion.
URTI.

12.  RBS
164 mg/dL
 Admitted
 IV
to the pediatric ward
Fluid.
 Midazolam PRN.

13.  Biochemstry:
 BUN
 Creat
 Na
K
 Ca
 Mg
 PO3
 Alb
59 mmol/l
559 μmol/L
131 mmol/L
4.8 mmol/L
1.16 mmol/l
0.51 mmol/l
2.85 mmol/l
38 g/dL
Renal failure
hypocalcemia

14.  Blood
gas:
 PH
7.27
 HCO3
14.4
 pco2
30.4
Metabolic
acidosis
 Complet
 WBC
 Hgb
 Hct
 Plt
 MCV
 MCH
 PTH
 LFT
2086 pg/l
NORMAL
blood count:
7.2 (μ L)
6.5 g/dl
20.1 %
194 (μ L)
84 fL
27 pg/cell
Normochromic
Normocytic
anaemia

15.  Patient
given Ca gluconate.
 Biochem double checked urgently.
 Patient continue to seize.
 Blood pressure was 162/79.
 Patient shifted to PICU

16.  Chronic
Renal failure.
 Seizure secondary to
1-hypocalcemia.
2-hypomagnisemia.
3-febrile convulsion.
4-uremia (uremic encephalopathy).

17.  Patient
continue to seize despite of
normalizing of calcium and magnesium.

18.  Blood
pressure noted to be persistently high
high SBP 160-180
 Patient managed as hypertensive
encephalopathy .
 After starting antihypertensive medication
patient settle down

19. Chronic renal
failure

hypertensive
encephalopathy

20. Patient US:
 Enlarged both kidney .
 Lack of cortical differentiation.
 Multiple small cyst noted suggesting ARPKD.
 Liver normal.


21.  Mother
US:
 Normal kidney size.
 No calical dilatation.
 Rt kidney echogenicity suggest old infection.

22.  ECG
LVH
 ECHO
mild left ventricular hypertrophy and
interventricular septum.

23.  Brain
MRI normal.
 Abdomin
MRI:
 Bilateral large lobulated kidney.

24. Chronic renal failure
with hypertensive
encephalopathy
Most likely due to
Autosomal dominant
polycystic kidney
disease (ADPKD)

25.  IVF.
 Magnisum
sulphate .
 Ca gluconate
 Hydralazine.
 Captopril.
 Propranolol.
 Ca carbonate.
 Calcitiol.
 Iron.
 Folic acid.

27.  Definition
:
 SBP and/or DBP >95th percentile for gender,
age, and height on > 3 occasions.

29.  significant
elevation in BP without
accompanying end-organ damage.

no absolute level of blood pressure.

30.  Elevation
of both systolic and diastolic BP
with acute end-organ damage

(e.g., cerebral infarction, pulmonary
edema, renal failure, hypertensive
encephalopathy, and cerebral hemorrhage).
 No
absolute level of blood pressure

31. 
is a condition characterized by varying
degrees of headache, nausea, vomiting,
visual disturbances, focal neurologic deficit,
and seizures in the setting of severe systemic
hypertension that is relatively acute in onset.

32.  Rapidity
of BP elevation of greater import
than magnitude of the elevation
 The
actual prevalence may be
underestimated.

33.  commonly
associated with renal disease.
 Examples:
 reflux
nephropathy,
 obstructive uropathy.
 renovascular disease.
 glomerular disease.
 polycystic kidney disease,
 haemolytic-uraemic syndrome.
 Less common :
 coarctation,
 phaeochromocytoma, Wilm’s tumour.

34.  With
severe and abrupt increase in BP
 Cerebral vasculture is unable to constrict
to maintain constant blood flow.
 Cerebral hyperperfusion and resultant
edema.
Overcoming of the autoregulatory
Rapid rise
hyperperfusi
Failure of
in BP
on
capacityVasoconstriction
of the cerebral vasculature.
Edema

35.  previously
normotensive persons develop
hypertensive encephalopathy at lower blood
pressures than do chronically hypertensive
persons.
 typically
start to occur 12–48 hours after a
sudden and sustained increase in blood
pressure.

36.  ACUTE:
 INDOLENT:
 seizures
 Headache.
 coma.
 Drowsiness.
 Nausea.
 Vomiting.
 blurred
vision.
 transient cortical
blindness.
 hemiparesis.
 papilledema
and retinal hemorrhages.

37.  MRI
often shows
increased signal
intensity in the
parito-occipital lobes
on T2 weighted
images.
 a finding termed
reversible posterior
leukoencephalopathy
syndrome.
 Hypertensive
encephalopathy is a
clinical diagnosis.

38.  restoration
of a normotensive state.

39.  Intravenous
administration is often
preferred.
 antihypertensive
agents with minimal
central nervous system side effects should
be chosen.
 BP
reduction should be performed in slow
controlled fashion to prevent ischemic
stroke.

40. a
stepwise reduction in pressure:
 the pressure should be reduced by about
25% over 6-8 hr.
 the remaining over the following 24–48 hr.

41.  Most
antihypertensive agents have not
been fully tested in children .
 the
choice is based upon the preference and
experience of the responsible physician.
 Whichever
drug is prescribed, the aim is to
reduce blood pressure slowly.

42. DRUG
Labetalol
MECHANISM OF
ACTION
DOSAGE RANGE SIDE EFFECTS
α- and β-Blockade 0.2–3.0 mg/kg/hr lower cardiac
output,
bronchospasm
Sodium
Nitroprusside
Dilatation of
arterioles and
venules
0.3–8.0
μg/kg/min
Nicardipine
Ca channel bloker 1–3 μg/kg/min
vasodilatation
Thiocyanate
production,
increased ICP
hypothyroidism
Hypotension,
increased ICP

43.  Treatment
of hypertension often leads to
complete neurologic recovery.
 Untreated
hypertension can lead to
irreversible cerebral infarction, coma, and
death.

44. Convulsion may
be the first
manifestation of
hypertension.