The renal and genital system both develop from the intermediate mesoderm, a collection of cell at the back

of the foetal abdominal cavity. Both drain into the same space foetal cloaca Form- Pronephros - cervical region -Mesonephros - below regression

-Metanephros - pelvic region.- final adult kidney start fxn 2nd trimester.

Congenital anomalies of the urinary system are not uncommon in clinical practice. Many of these are asymptomatic and go unsuspected until there is complication resulting in symptom.

1. 2. 3.

-Anomalies of the kidney DIVIDED. Anomalies of structure Anomalies of location Anomalies of renal vessels

A) Bilateral renal agenesis (Potters syndrome) - male > female; - Hx- oligohydramnious, SGA stillborn. - characteristic potters facie with low set or malformed ear, prominent epicanthal fold, bird-like nose, small receding chin. -There are usually associated genital anomalies, pulmonary hypoplasia, and abnormalities of extremities. Not compatible with life. -No known effective management

B) Unilateral renal agenesis -Solitary kidney - Common in male - Asymptomatic - Associated with other system congenital anomalies - Compatible with normal life span C)Supernumerary kidney - rare, with extra kidney - small and dysplastic

Aplasia small with no renal tissue and atretic ureter 2. Hypoplasic small size but structure is normal -Unilateral compatible with normal life but patient should be monitored for life -Bilateral, polyuria with dilute and salt wasting with progressive deterioration in renal failure to end stage. 3. Dysplastic: Mal-development, disorganization and persistence of embryonic element, the smooth muscle and cartilage. It may be normal, small or cystic in size.
1.

The prognosis depend on the amount of functional renal tissue and presence of other anomalies like PUV or VesicoUreteric reflex Unilateral No Asymptomatic Bilateral result in progression deterioration in renal function and End Stage Renal Failure and require renal replacement therapy.

A) Multicystic Dysplastic Kidney -Commonest form of cystic kidney disease. -Kidney tissue is replaced with non communicating cysts of varying sizes. - Usually unilateral but abnormality of the opposite kidney are common - Treatment not necessary, kidney usually involutes and shrinks

B) Familial and hereditary Cystic Dysplasia.

(vi) Infantile Polycystic Kidney disease AR -Born with large kidney and not compatible with life. (vi). Juvenile Nephronopthisis AR -Small kidneys with cysts in the renal medulla of both kidneys. -Pts present with polyuria, polydysia or CRF. -Rx-Renal Replacement therapy.

(v) Adult Polycystic kidney disease AD - Present after the 2nd decade but may present early - An unusual associated with intracranial berry aneurysm - Present with Abdominal pain, haematuria, recurrent febrile episode, hypertension, bilateral abdominal mass. -Deterioration in renal function till the fourth decade of life. Dx- IVU, Renal angiography, CT, MRI or USS

(vii). Oligomeganephronia - Small kidneys with reduce number of nephrons which are abnormally large. -Presentation: Polyuria, Vomiting, dehydration, salt wasting, concentration defect and proteinuria -There is deterioration in renal failure to End Stage Renal Failure

TRX -Correction of electrolytes derangement and treatment of hypertension - Manage Renal Failure

Anomalies of location

A) Horseshore kidney - Infection - Stone formation B)- Ectopic kidney located in the pelvis or abdomen
Anomalies of Uninary tract

(b) Posterior Urethral Valve - obstruction mucosal told on the floor of the posterior urethra - commonest cause of obstruction uropathy in male children
Anomalies of the Bladder - Agenesis, duplication, patent urachus and extrophy
-

(a) Duplicate of the ureters common in girls - It may drain into the Urethra or vagina - Reflux, hydroureter, hydronephrosis, dribbling and recurrent infection enuresis. - Diagun in IVU RX surgical repair

Vesical extrophy. The bladder is exposed due to absence of abdominal wall muscle and skin infection is common. Miscellaneous condition

-This is a triad of absent abdominal muscle, underscended tests and urinary tract dilatation.

Prime-belly syndrome:

The obstruction lesion are mostly congenital but may occasionally be acquired. Complete reversal of the effect of obstruction can be achieved by early treatment but a long standing obstruction lead to changes that are usually irreversible or only partially reversible.
The resultant impairment of renal function is detrimental to normal growth and development and may be life threatening. Obstruction uropathy often present with non special feature and high level of clinical suspicion is necessary for it detect

PATHOPHYSIOLOGY The effect of obstruction anywhere in the urinary tract are predictable with proximal holdup of urine resulting in stasis which leads to dilatation, infection and increase pressure within the obstructed system.
There are reversible in the initial stages but lead to deterioration of renal function if the obstruction persists over a long period of time.

Cause

1. Posterior Urethral valve in boys 2. Phimosis 3. Meatal stenosis 4. Stricture 5. Renal calculi 6. Neuropathic bladder 7. Ureterocele 8. Megaureter 9. Pelviureteric junction obstruction

POSTERIOR URETHRAL VALVES PUV are the most common cause of Obstruction Uropathy in boys. Dilatertion of posterior urethra and hypertrophy of the urinary bladder follow.

Hx -dribbling of urine, weak stream, recurrent UTT, straining during micturition Recurrent UTT, FTT, CRF Dx -VCUG VUR and bilateral hydroureter and nephrosis may be present. Laboratory tests- evaluate RF.

The condition is characterized by an anatomical obstruction of the flow of urine from the renal pelvis into the ureter. Hx -flank mass upper abdomen or pain UTI. Gross haematuria , stone formation and deterioration RF

Pelviretene Junction (PUJ) obstruction

(Dietls Crisis)

DX: Ultrasandography and DTPA renogram

RX: Pyeloplasty

ENURESIS:

Enuresis is defined as the involuntary passing of urine after the age of 5 years or inability to achieve bladder control resulting in early complete evacuation of the bladder at a wrong place and time at least twice a month after the fifth year of life. As a rule the bed will be soaking wet as against incontinence, which is loss of urine without normal emptying of the bladder.
Bladder control is usually attained between the age of one and five years. Up to the eleventh year enuresis is twice as common in boys as it is in girls but there after the incidence is similar or slightly higher in girls.

Classification

- Enuresis may be diurnal, nocturnal or both - Primary or Secondary - Primary Enuresis: when the child has never been dry - Secondary Enuresis: occurs when after being dry for at least six month and started bed wetting.

AETIOLOGY The cause of enuresis is always functional, in contrast to the cause of incontinence which may be organic or functional.
These are multi factional and identification of the cause help in the choice of management

(1) MATURATIONAL DELAYA delayed in the development of fine and gross motor skill may occur in some normal children especially boys. exaggerated with stressful life events and anxiety. (2) FAMILY HISTORY AND GENETICS The risk of enuresis is as high as 40 percent in child with positive history in one parent and 70 percent if both parent had enuresis. The mode of inheritance appears to be autosomal dominant with reduced penetrance modulated by environmental factor and these genes.

(3) SLEEP FACTOR Deep sleepers and poor wake up signals from the full bladder. Instead of complete arousal, the switch only gets the child to light sleep. (4) LOW BLADDER CAPACITY A low function bladder capacity has been compared with estimated bladder capacity. Calculated using the age based formula. (5) ADH: Lack of Circadian rhythm or impaired response of the kidney to anti diuretic Hormone may be a cause of enuresis especially nocturnal enuresis.

Investigation:

Based on history and initial examination, children with uncomplicated enuresis require no further evaluation. Good Hx Family history (70 percent) Anxiety, depression and stressful life events Social disadvantage Other developmental problems

USS and VCUG is the reserve for patient with suspected neurological or urological dysfunction. Invasive procedure like Uroflowmetry, with or without pelvic floor and abdominal muscle EMG, cystometry and other urodynamic study is reserved for patient with voiding disorder.

Adequate clinical assessment is essential to determine the type and seventy of the condition and the appropriate mode of treatment.
The choice of treatment will depend upon the main concerns expressed by the family. A combination of a behaviour management programme and medication is most effective.

A) Treatment of organic causes Rx UTI, DM DI (B) Voiding Training (C) Behavioural Therapy (D) Motivational therapy (E) Pharmaco therapy (F) Psychotherapy

This disorder comprises a group of tubular transport defects characterized by inability to appropriately acidify the urine with resultant metabolic acidosis. Pathophysiology The underlying abnormality consists of an impairment of bicarbonate reabsorption(Type II)or excretion of H+ ions(Type I) or a combination of both; and exists as three types in children. The plasma bicarbonate level is low, and the children have a metabolic acidosis with normal anion gap in all the type.

Type I

(Distal RTA)

Type II

Proximal RTA

This is an important cause of severe rachitic deformities, failure to thrive and hypokalaemia complication in children; there is also weakness, polyuria and nephrocalcinosis. Lab funding: The urine is alkaline (pH > 5.5) despite system acidosis. (Blood pH < 7.2) Hyper calcuria and hypocitraturia are present; serum KT is normal or low. RX: Correct the acidosis with NaCO3 2-3 mEq/kg/day

Isolated type II RTA is very rare, so it is often seen as a component of primary or secondary Fanconi syndrome.

CLINICAL FEATURES Failure to thrive, vomiting and muscle weakness Lab Findings - Blood pH and bicarbonate level are low - The urine pH may fall below 5.5 - Normal Ca+ and citrate excretion Diagnosis is made by calculation the fractional excretion of bicarbonate.

Treatment: 2-5mEq/kg/day of Bicarbonate and restriction of sodium intake.

CAUSES OF

TYPE I RTA (DISTAL)

CAUSES OF TYPE II RTA (PROXIMAL)

Isolated defect -Primary: genetic: -Primary: Genetic, Sporadic -Secondary (rare): Drug like sporadic carbonic anhydrase, inhibitor -Secondary (acetazolamide) Multiple tubular dysfunctions #SLE, sjogren syndrome (Fanconi syndrome) #Sickle cell anaemia -Primary: genetic, sporadic -Secondary: cystineosis, lower #Obstructive Uropathy, syndrome, wilsons disease, #reflux nephropathy tyrosinemia, galactosemia, fructosaemia mitochondrial #Nephrocalcinosis, disorders, Vitamin D #Amphotericin, B. toxicity dependent rickets, drugs e.g lead, ifosfamide, #Prematurity and LBW aminoglycosides, cisplatin, Amyloidosis, renal transplantation

Mineralocorticoid deficiency
Aldosterone deficiency without renal disease Addisons disease Isolated aldosterone deficiency Aldosterone deficiency in chronic kidney dx Interstitial nephritis, nephrocalcinosis Aldosterone resistance.

This is spectrum of disorders with similar clinical features of polyuria and polydipsia in the absence of osmotic diuresis as result of deficiency of Anti diuretic hormones(ADH) or tubular unresponsiveness to the hormones.
CENTRAL (CDI) NEPHROGENIC DIABETES INSIPIDUS (NDI)

Secondary to a complete defect in ADH secretion in the posterior pituitary gland resulting in the inability of concentrating urine appropriately despite normal renal function. Pathoplysiology Increase urine output, hypernatraemia and dehydration

Clinical Features (Cont.) - There is a significant risk of hypernatraemia dehydration

LAB FINDINGS - Urine S.G <1.010, low urine osmolarity - Normal to high serum sodium concentration - Water deprivation Test: Positive i.e. persistence of dilute urine with osmolarity less than that of plasma - A rise in serum sodium to > 145mEq/l - A rise of serum osmolarity to > 290 MOSM/kg - Weight loss of 3% - 5% - Other: skill radiograph, CT, MRT

Clinical Features Polyuria, polydipsia, weight loss, growth failure - patient generally prefer water to other fluid - There may be neurologic or visual complaints - Feature may not be obvious in the infant who demonstrates instability, frequent feed requirement, unexplained fever, constipation, failure to thrive and delayed development.

Treatment

Desmopressin (dDAVP) is given intranasally once or twice daily

There distal tubular unresponsiveness to ADH resulting in inability to concentrate the urine hyposthenuria and polyuria Aetiology

Secondary

Primary

Rare X-linked recessive disorder with profound effect in males although females may be mildly affected. In most families, the defect is caused by a mutation in the vasopressin receptor. Autosomal dominant and recessive DI has also been described in which aquaporin (The renal water channel) on chromosome 12q is defective

More common and often less severe than primary Obstruction Uropathy Chronic renal failure Sickle cell disease Drug toxicity CLINICAL FEATURE Present in the first week of life in the severe form Polyuria, polydipsia, failure to thrive Chronic dehydration: the degree of dehydration may be underestimated because the child continues to make urine. Fever, instability Poor feeding are also common

LABORATORY FINDINGS Hypernatraemia, hyperchloraemia Urine Osmolarity < 200 mOsm/kg in the present of serum osmolarity > 300 mOSm/kg ADH level are normal, and there is no response to exogenously administered vasopressin

Treatment Cont.

3) Administration of hydrochlorothiazide at a dose of 2 4 mg/kg/day. (Encourage proximal tubular Na+ and water re-absorption) 4) Amiloride 20mg/m2 has an additive effect. Differential Diagnosis Psychogenic water drinking Impaired thirst mechanism Diabetes mellitus

Treatment

1) Maintenance of adequate fluid intake 2) Salt (sodium) restriction: 1mEq/kg/day

What is a normal BP in children...... normogram (Age in years X 3) + 100= SBP 95th C Blood pressure increase with age, Ht & gender The increase in blood pressure with age is largely due to increase in height and weight. A normal value of blood pressure for age is < 90th percentile.
-Pre-hypertension BP -values between 90 95th percentiles - A systolic or diastolic BP values > 95th percentile should be repeated on at least 2 more occasions to confirm the diagnosis of hypertension.

All children with elevated systolic or diastolic BP valves > 95th percentiles need evaluation. BP> 99th percentile is define as severe hypertension and need more prompt evaluation and treatment. MEASUREMENT OF BLOOD PRESSURE This is done by direct intra-arterial measurement through non invasive method of (1) Oscillometry (2) Mercury sphygmomanometery (3) Ambulatory Blood Pressure Monitoring (ABPM).

SECONDARY CAUSES cont.

A Renal parenchymal disease - Chronic GN, Reflux nephropathy, obstructive Uropathy - Polycystic kidney disease, renal dysplasia B Renal Tumors - Wilms tumor, (Nephroblastoma) hemangiopericytoma C Renovascular Disease - Idiopathic Aortoarteritis (Takayasus disease) - Renal artery stenosis, renal artery thrombosis

(1) SECONDARY CAUSES

D Cardiovascular disease - Coarctation of aorta E Endocrine disease - Pheochromocytoma, neuroblastoma, primary hyperaldosteronism - Cushing syndrome, congenital adrenal hyperplasia

(2)

Essential hypertension especially in adolescent

PRIMARY CAUSES

-Asymptomatic - headache, abdominal pain, nausea; vomiting and weight loss may be present. Severe HBP- sensorial impairment, visual disturbance, focal neurological deficits, seizure, and heart failure, which may be the presently feature. These occur at lower blood pressure compare to adult and in acute situation with rapid increase in the blood pressure. In sick neonate blood pressure measurement is pertinent since hypertension manifest with feature suggesting sepsis, intracranial haemorrhage or cardiopulmonary instability.

(A) Preliminary Investigation - Urinalysis: cell, cast, protein, 24hrs protein excretion - Urine culture - Blood, urea creatinine, electrolytes, Bicarbonate, uric acid, calcium, fasting cholesterol, triglycerides - Chest x-ray film - ECG, ECHO, Adbominopelvic ultrasound - Funds examination - 99m TC DMSA scan

(B) Additional Investigation Depend on suspected underlying cause Glomerulonephritis

Serum C3, C4, ASO Autoantibodies (ANA, AntidsDNA, ANCA) Renal biopsy

Reflux Nephropathy

Micturating cystourethrogram Dimercaptosuccinic (DMSA) renal scan Intravenous Urogram

Renovascular disease

Phenochromocytoma

Dopplar ultrasound Captopril primed isotype scan (DTPA or MAG-3) Renal angiography or digital subtraction angiography Renin sampling from renal veins and interior vena cava Urine and plasma catecholamines Plasma calcitonin, parathormone I-meta lodobenzlguanidine (MIBG) scan, CT, MRI Arterriography and caval catecholamine sampling.

Coarctation of the aorta Other endocrine causes

Echocardiogran, angiography. Urine steroid profile Plasma aldosterone, cortisol, DOC Dexemethasome/ACTH test. Peripheral plasma renin and aldosterone Spot urine catecholamines

 2o HBP commonest in children and the treatments depend on the cause and degree of hypertension. Surgical treatment is included in coarctation of aorta and most form of renal vascular hypertension such as renal artery stenosis, renal aneurysm are often cured by revascularization surgical procedure and open or percutaneous transluminal angioplasty.

Children with symptomatic and/ or secondary hypertension, and those with features of target organ damages require therapy with antihypertensive medication. Persistent HBP despite adequate nonpharmacilogic measure in essentials HBP will also need antihypertensive.

C)Calcium Antagonists D)Diuretic: E)Adrenoceptor Blocker OTHER DRUG Centrally acting vasodilatorsHydralazine, Minoxidil,Clonidine Methyldops.
The goal for anti-HBP reduction of pressure below the 90th percents and initial therapy should be with single drug. Like calcium, channel blocker, one of the initial medications is ineffective in lowering the blood pressure. It is necessary to use a drug from a different class.

Five classes of drug are most suitable in children in the management of HBP. A)ACE inhibitors and reception blocker B)B-Adrenoceptor blockser