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Extern Conference 23/8/50 An 8-year-old Thai boy was admitted due to severe progressive headache and severe hypertension. History Present Illness • He had episodes of headache of few times per month for 1 year. The characteristic was a throbbing frontal headache aggravated from exercise and relieved by resting or analgesic drug such as paracetamol. • 2 days prior to admission, his headache became progressively severe. History (2) Present illness • No nausea, vomiting, dyspnea, and orthopnea • Vision was normal. • No fever, cough, or sore throat recently • Normal urination, no dysuria, hematuria, or oliguria • No edema, rash, oral ulcer, anemia, abnormal bleeding, or arthralgia • No history of previous hypertension or urinary tract infection. History (3) • No family history of hypertension or renal disease • Development was normal. • Complete vaccination as scheduled • No history of any drug allergy • No ingestion of any medications Physical Examination Vital signs: T 37.5 o C, P 76 /min, no delayed pulse and equal all extremities, RR 24 / min, Blood pressure Right arm: 170/120 mmHg Left arm: 165/110 mmHg Right leg: 170/105 mmHg Left leg: 170/110 mmHg BW 23 kg (P10-25) Ht 126.5 cm (P 50) BMI 14.37 kg/m2 Physical Examination (2) General appearance: alert and cooperative, not pale, no jaundice, no dyspnea, no orthopnea, no puffy eyelids, no edema, and no cyanosis Skin: no rash, no petechiae , no ecchymoses, no alopecia, no café au lait spots HEENT: normal Physical examination (3) CVS: PMI at left 5th intercostals space, lateral to midclavicular line, no heaving or thrill, normal first and second heart sound, no murmur RS: normal Abdomen: soft, not tender, no palpable mass, liver and spleen not palpable, normal bowel sound , no abdominal bruit Nervous system : Normal No superficial lymphadenopathy Problem list 1. Chronic headache for 1 yr with severe progressive headache for 2 days 2. Severe hypertension 3. Cardiomegaly Hypertension in childhood What is hypertension? • Hypertension=average SBP and/or DBP ≥ 95th percentile for gender, age, and height on ≥ 3 separate occasions • Prehypertension : BP 90th -95th or BP ≥ 120/80 • Normotension : SBP and DBP ≤ 90th by age, gender, and height or BP ≤ 120/80 • Hypertension stage I : SBP and/or DBP 95th -99th • Hypertension stage II : SBP and/or DBP ≥ 99th Clinical manifestations of hypertension • Most of the patients do not have symptom related to hypertension at the presentation. • Chronic headache (10 %) • Hypertensive encephalopathy (6.8%) • Epistaxis (1.4%) • Visual disturbance (1.4 %) [ The Study of persistent hypertension in Thai children etiologies and outcome in J med association Thai 2006 ] Hypertensive Emergency • No specific level of BP • Defined as a blood pressure high enough to cause acute injury to target organs • Children are more prone to hypertensive encephalopathy than adults Complication of hypertensive emergency • The most common complications are - Hypertensive encephalopathy - Cerebral infarction and hemorrhage - Facial palsy - Visual symptoms - Cardiac failure - Renal failure Hypertension etiology • Primary or early onset of essential hypertension • Secondary hypertension The Causes of Secondary Hypertension in Children and Adolescents Cause Acute hypertension Chronic hypertension Etiology Renal Acute glomerulonephritis Congenital defects Tumors of the kidney Acute renal failure Chronic pyelonephritis Hypoplastic kidney Hemolytic-uremic syndrome Hydronephrosis Collagen vascular disease - Pheochromocytoma Primary aldosteronism Hyperthyroidism Neuroblastoma Coarctation of the aorta Renal arteriovenous fistula Renal artery stenosis Neurofibromatosis Takayasu arteritis Tuberous sclerosis Dysautonomia - Endocrine Vascular Neurogenic Renovascular trauma Increased intracranial pressure Guillain-Barré syndrome Metabolic Hypercalcemia Adapted from Daniels SR, Loggie JM: Essential hypertension. Adolesc Med State Art Rev 2:555, 1991. Hypernatremia The Causes of Secondary Hypertension in Children and Adolescents (2) Cause Acute hypertension Chronic hypertension Etiology Metabolic Hypercalcemia - - Cocaine Nonsteroidal Anabolic steroids Phencyclidine anti-inflammatory drugs Corticosteroids Amphetamines Oral contraceptives Alcohol Burns Leg traction Heavy metal poisons - Hypernatremia Drugs Others Adapted from Daniels SR, Loggie JM: Essential hypertension. Adolesc Med State Art Rev 2:555, 1991. Etiology of Secondary Hypertension in Pediatrics • • • • • 78% 12% 2% 0.5% 7.5% renal parenchymal renovascular coarctation of the aorta pheochromocytoma others Ronald Portman,MD professor and director,division of pediatric nephrology and hypertension, University of Texus Houston, 2003 Cause of the persistent hypertension according to age group in Thailand Age 6-12 yr Cause No. of patient(%) lupus nephritis 36.1 chronic renal failure 22.2 idiopathic nephrotic syndrome 5.6 IgA nephropathy 2.8 renovascular disase 13.9 drug induced 11.1 coarctation of aorta 2.8 Essential hypertension 2.8 unknown 2.8 [ The Study of persistent hypertension in Thai children etiologies and outcome in J med association Thai 2006 ] How to approach hypertension Diagnostic work up & Evaluation for target organ damage The Causes of Secondary Hypertension as Suggested by History History Suggests Known urinary tract infection; recurrent abdominal or flank pain with frequency, urgency, dysuria; secondary enuresis Renal disease Joint pains, rash, fever, edema Renal disease, vasculitis Complicated neonatal course, umbilical artery catheter Renal artery stenosis Renal trauma Renal artery stenosis Drug use (e.g., sympathomimetics, anabolic steroids, oral contraceptives, illicit drugs) Drug-induced hypertension Aberrant course or timing of secondary sexual characteristics; virilization Adrenal disorder Muscle cramping, constipation, weakness Hyperaldosteronism (primary or secondary) Excessive sweating, episodes of pallor and flushing Pheochromocytoma Adapted from Hiner LB, Falkner B: Renovascular hypertension in children. Pediatr Clin North Am 40:128-129, 1993. The Causes of Secondary Hypertension as Suggested by Physical Examination Physical finding Possible secondary cause Blood pressure >140/100 at any age Multiple secondary causes Leg < arm blood pressure Coarctation of the aorta Poor growth Chronic renal disease Short stature, features of Turner syndrome Coarctation of the aorta Multiple café-au-lait spots or neurofibromas Renal artery stenosis, pheochromocytoma Decreased or delayed pulse in leg Coarctation of the aorta Vascular bruits Over large vessels Arteritis Over upper abdomen, flank Renal artery stenosis Flank or upper quadrant mass Renal malformation, renal or adrenal tumor Excessive virilization or secondary sex characteristics inappropriate for age Adrenal disorder Extremities Adapted from Hiner LB, Falkner B: Renovascular hypertension in children. Pediatr Clin North Am 40:128-129, 1993. Edema Renal disease Excessive sweating Pheochromocytoma Adapted from Hiner LB, Falkner B: Renovascular hypertension in children. Pediatr Clin North Am 40:128-129, 1993. Investigation The first line • • • • Urinalysis Urine culture BUN , creatinine 24 hr. urine for vanillymandelic acid • Renal ultrasound including Doppler study of renal ateries • • • • • • Complete blood count Electrolyte Calcium, Phosphate Chest x- ray EKG Retinal examination Investigation The second line • urine catecholamine • plasma renin and aldosterone • CT angiography • ESR and ANA Investigation in this patient Investigation • CBC : Hb 12.1 g/dl Hct 35.3 % WBC 3890 cell/mm3 ( N 46%, L 39%, Mo 9%) Plt 187,000 cell/mm3 • UA : pH 6, Sp.gr. 1.015, protein 2+ ,ketone negative WBC 0-1 cell/HP , RBC 0-1 cell/HP, no dysmorphic RBC, no cast • Urine protein/creatine : 0.8 • 24 hr. urine protein : 9 mg/kg/day Investigation • Blood chemistry : BUN 13.0 mg/dl, Cr 0.7 mg/dl, Na 137 mmol/L, K 3.1 mmol/L, Cl 102 mmol/L, HCO3 22 mmol/L • Lipid profile : Chol 203 mg/dl, TG 68 mg/dl, HDL 66 mg/dl, LDL 123.4 mg/dl • EKG : LVH by voltage criteria • Fundoscopic examination : atherosclerosis grade II BE • CXR : Cardiomegaly, CT ratio 0.53, no pulmonary infiltration. Investigation • Echocardiogram : no coarctation of aorta, no irregularity or aneurysmal dilatation of abdominal aorta. concentric LVH without LVOT obstruction. good LV systolic function. no structural heart disease. Investigation • • • • Throat swab culture : normal flora Anti-streptolysin O : 576 IU/ml AntiDNAse B : 79.1 U/ml C3 : 105 mg/dl • U/S : Bilateral hydronephrosis and hydroureter UVJ, possibly related with stricture at UVJ from megaureter or stenosis related with ectopic ureter. VCUG : bilateral vesicoureteric reflux grade 5, suspected bilateral primary megaureter • Tc-99m MAG3 : hydronephrosis and hydroureter both kidneys with no evidence of obstruction. Mild impair function of left kidney. Investigation • Tc-99m DMSA : multiple small renal infarction (function right: left = 51%: 49%) Renal parenchymal disease • Acute glomerulonephritis • • Lupus nephritis • Acute or chronic renal failure • • Nephrotic syndrome • • IgA nephropathy • Henoch-Schonlein nephritis Coarse renal scarring (reflux nephropathy, obstructive uropathy, neuropathic bladder) Polycystic kidney disease Hemolytic uraemic syndrome. VESICOURETERAL REFLUX VESICOURETERAL REFLUX • The retrograde passage of urine from the bladder into the upper urinary tract • Incidence : 1 % of children • 2 categories : primary and secondary • Screening with a radionuclide cystogram of all sibling < 3 year and any sibling with a UTI is appropriate. older sibling may undergo renal U/S and if an abnormality is found, VCUG is recommended The length of the submucosal segment of the distal ureter is an important factor in determining the effectiveness of the ureteral valvular mechanism in preventing VUR. Clinical manifestation • Prenatal presentation : hydronephrosis via U/S (80% are male) • Postnatal presentation : UTI • In other children, VCUG is performed during evaluation for pathology of urinary tract International system of radiographic grading of VUR Treatment • Goal of treatment are to prevent complication • Surgery for severe VUR • ATB prophylaxis for mild to moderate VUR Treatment of hypertension Nonpharmacologic treatment • • • • • dietary salt restriction mineral supplementation weight control regular exercise life style modification Indications for Antihypertensive drugs • • • • Symptomatic hypertension Secondary hypertension Hypertensive target-organ damage Persistent hypertension despite nonpharmacologic measure • DM? Antihypertensive drugs • ACEI and Ca channel blocker are commonly prescribed in children • Diuretics are usually adjunct therapy. • Need regular long term follow up with special attention to target organ injury and underlying disease Antihypertensive drugs for hypertensive emergency Most useful • Esmolol : IV 100-150 ug/kg/min • Hydralazine : IV or IM 0.2-0.6 mg/kg/dose • Labetalol : IV 0.2-1.0 mg/kg/dose • Nicardipine : IV 1-3 mg/kg/min • Sodium nitroprusside : IV 0.53-10 ug/kg/min Management in this patient Management • Antihypertensive drug : Enalapril (5 mg) ½ tab oral bid Adalat CR (30 mg) 1 tab oral OD Atenolol (50 mg) ½ tab oral bid pc • ATB prophylaxis : Bactrim (80 mg of TMP) 1 tab oral hs • At ward , BP 100-130 / 80-90 mmHg , UA : protein 2+ , wc 0-1/HP Home medications: • • • • Bactrim (80 mg of TMP) 1 tab oral hs Enalapril (5 mg) ½ tab oral bid Adalat CR (30 mg) 1 tab oral OD Atenolol (50 mg) ½ tab oral bid pc Progression Progress Note Surgery: bilateral re-implantation (Cohen Cross trigone) After surgery : no anti-hypertensive medications (BP 117/80 mmHg) Medications: – Bactrim (80 mg of TMP) ½ tab oral bid – Paracetamol (500) ½ tab oral prn for pain q 4-6 hrs VCUG 1 month after surgery Tc-99m DMSA : no significant change of bilateral renal cortex compare to the previous study Special thank ศ.พญ. อัจฉรา สั มบุณณานนท์ Thank you