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Case presentation polycystic kideny in pediatrics presented with convulsion
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CASE PRESENTATI
ON
Yassin M Al-Saleh
Supervised by: Dr.Aziza Al-
Shehab
بسم الله الرحمن الرحيم
)وما اوتيتم من العلم إال قليال(
HISTORY AND EXAMINATION
HISTORY Hussin is 3 year old boy.
chief complaint : abnormal movement .
Patient not known to have any illness before.
he developed subjective fever.
Few hours , generalized tonic clonic movement of the body with uprolling of the eye.
HISTORY CONT. 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.
HISTORY CONT 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
HISTORY CONT Developmentally normal.
Family 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.
EXAMINATION 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.
EXAMINATION 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.
INITIAL IMPRESSION IN ER
Febrile convulsion.
URTI.
HOSPITAL COURSE
PLAN RBS 164 mg/dL
Admitted to the pediatric ward
IV Fluid. Midazolam PRN.
INVESTIGATION Biochemstry: BUN 59 mmol/l Creat 559
μmol/L Na 131
mmol/L K 4.8
mmol/L Ca 1.16
mmol/l Mg 0.51
mmol/l PO3 2.85
mmol/l Alb 38 g/dL
hypocalcemia
Renal failure
REMANING OF INVESTIGATION Blood gas: PH 7.27 HCO3 14.4 pco2 30.4
Complet blood count:
WBC 7.2 )μ L( Hgb 6.5 g/dl Hct 20.1 % Plt 194 )μ L( MCV 84 fL MCH 27 pg/cell
Metabolic acidosis
Normochromic Normocytic anaemia
PTH 2086 pg/l
LFT NORMAL
IN THE WARD Patient given Ca gluconate. Biochem double checked urgently. Patient continue to seize. Blood pressure was 162/79. Patient shifted to PICU
SECOND IMPRESSION Chronic Renal failure. Seizure secondary to 1-hypocalcemia. 2-hypomagnisemia. 3-febrile convulsion. 4-uremia )uremic encephalopathy(.
IN PICU Patient continue to seize despite of
normalizing of calcium and magnesium.
IN PICU Blood pressure noted to be persistently
high high SBP 160-180 Patient managed as hypertensive
encephalopathy . After starting antihypertensive
medication patient settle down
THIRD IMPRESSION
Chronic renal failure
hypertensive encephalopathy
IMAGING ULTRASOUND Patient US: Enlarged both kidney . Lack of cortical differentiation. Multiple small cyst noted suggesting ARPKD. Liver normal.
IMAGING ULTRASOUND Mother US: Normal kidney size. No calical dilatation. Rt kidney echogenicity suggest old
infection.
IMAGING ECHO ECG LVH
ECHO mild left ventricular hypertrophy and interventricular septum.
IMAGING MRI Brain MRI normal. Abdomin MRI: Bilateral large lobulated kidney.
FINAL IMPRESSION
Chronic renal failure with hypertensive
encephalopathyMost likely due to
Autosomal dominant polycystic kidney disease )ADPKD(
MANAGEMENT: IVF. Magnisum sulphate . Ca gluconate Hydralazine. Captopril. Propranolol. Ca carbonate. Calcitiol. Iron. Folic acid.
MANAGEMENT OF HYPERTENSIVE
ENCEPHALOPATHY
HYPERTENSION IN CHILDREN Definition : SBP and/or DBP >95th percentile for
gender, age, and height on > 3 occasions.
CLASSIFICATION OF HYPERTENSION IN CHILDREN
Normal <90th
Prehypertension 90-<95th or if >120-80
Stage 1 hypertension
95th-99th plus 5 mm Hg
Stage 2 hypertension
>99th plus 5 mm Hg
HYPERTENSIVE URGENCY significant elevation in BP without
accompanying end-organ damage.
no absolute level of blood pressure.
HYPERTENSIVE EMERGENCY 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
HYPERTENSIVE ENCEPHALOPATHY 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.
HYPERTENSIVE ENCEPHALOPATHY Rapidity of BP elevation of greater
import than magnitude of the elevation
The actual prevalence may be underestimated.
HYPERTENSIVE ENCEPHALOPATHY
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.
HYPERTENSIVE ENCEPHALOPATHY With severe and abrupt increase in
BP Cerebral vasculture is unable to
constrict to maintain constant blood flow.
Cerebral hyperperfusion and resultant edema.
Rapid rise in BP
Failure of Vasoconstricti
on
hyperperfusion
Edema
Overcoming of the autoregulatory capacity of the cerebral
vasculature.
HYPERTENSIVE ENCEPHALOPATHY 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.
CLINICAL PICTURE ACUTE: seizures coma.
INDOLENT: Headache. Drowsiness. Nausea. Vomiting. blurred vision. transient cortical
blindness. hemiparesis.
papilledema and retinal hemorrhages.
IMAGING 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.
MANAGEMENT
restoration of a normotensive state.
ACUTE MANAGEMENT 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.
ACUTE MANAGEMENT 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.
ACUTE MANAGEMENT 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.
ANTIHYPERTENSIVE DRUGS
DRUGMECHANISM OF ACTION
DOSAGE RANGE SIDE EFFECTS
Labetalol 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
Thiocyanate production, increased ICP hypothyroidism
Nicardipine Ca channel bloker vasodilatation
1–3 μg/kg/min Hypotension, increased ICP
PROGNOSIS Treatment of hypertension often leads to
complete neurologic recovery.
Untreated hypertension can lead to irreversible cerebral infarction, coma, and death.
MESSAGE
Convulsion may be the
first manifestation
of hypertension.
THANK YOU