Case presentation polycystic kideny

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.

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