APPROACH TO GENITOURINARY SYSTEM, CYSTIC KIDNEY DISEASE AND URINARYTRACT INFECTIONS IN PAEDIATRIC POPULATION

GENITOURINARY SYSTEM AND CYSTIC KIDNEY DISEASE AND UTI (PAEDIATRICS)

1. Evaluation of Genitourinary System, Cystic Kidney Diseases, and Urinary Tract Infections DR. AVINASH KUMAR JHA MBBS(GOLD MEDALIST), DNB(PAEDIATRICS), PGPN(BOSTON)

2. Importance of understanding the genitourinary system Early Diagnosis : Recognizing congenital and acquired genitourinary abnormalities early can prevent complications and improve outcomes. Growth and Development : Proper functioning of the genitourinary system is crucial for normal growth and development. Preventing Chronic Kidney Disease : Early detection and management of genitourinary issues can prevent progression to chronic kidney disease. Preventing Infections : Understanding urinary tract infections and their management is essential to prevent recurrent infections and renal damage. Genetic Counseling : Knowledge of hereditary genitourinary conditions aids in providing genetic counseling and family planning advice. Minimizing Psychological Impact : Addressing and managing urogenital issues can reduce psychological and social impacts on pediatric patients. 7.Guiding Treatment : Accurate assessment and diagnosis guide appropriate treatment plans, including medical and surgical interventions.

3. KEY ROLES OF GUS

4. ANATOMY OF GUS

5. Pediatric Evaluation of the Genitourinary System History Taking: Prenatal and perinatal history. Family history of renal disease. Symptoms: Dysuria, hematuria, frequency, urgency, etc. Physical Examination: Inspection and palpation of the abdomen. Examination of the external genitalia. Blood pressure measurement. Diagnostic Tests and Imaging: Urinalysis, urine culture. Ultrasound, Voiding Cystourethrogram (VCUG), Renal Scintigraphy.

6. EXAMINATION OF GUS

7. Examination of the External Genitalia

8. URINALYSIS Purpose of Urinalysis: To detect and manage urinary tract infections (UTIs). To diagnose and monitor kidney diseases and systemic conditions affecting the kidneys. To screen for metabolic disorders and evaluate hydration status. Visual Inspection: Color: Normal urine color ranges from pale yellow to deep amber, depending on concentration. Abnormal colors (e.g., red, brown, green) can indicate various conditions. Clarity: Normal urine is clear. Cloudiness or turbidity can indicate the presence of cells, bacteria, mucus, or crystals.

9. Dipstick Testing: pH: Normal urine pH ranges from 4.5 to 8. Abnormal pH levels can indicate renal tubular acidosis, infection, or a diet-related issue. Specific Gravity: Normal range is 1.005 to 1.030. It measures urine concentration and can indicate hydration status or kidney function. Protein: Presence of protein (proteinuria) can indicate kidney disease, nephrotic syndrome, or other systemic conditions. Glucose: Presence of glucose (glucosuria) usually indicates diabetes mellitus or a rare kidney condition affecting glucose reabsorption. Ketones: Presence of ketones (ketonuria) indicates fat metabolism, which can occur in diabetes, fasting, or starvation. Blood: Presence of blood (hematuria) can indicate infection, trauma, stones, or glomerular diseases. Leukocyte Esterase: Indicates the presence of white blood cells, suggesting infection or inflammation. Nitrites: Indicates the presence of bacteria that convert nitrates to nitrites, commonly seen in UTIs. Bilirubin and Urobilinogen: Their presence can indicate liver disease or hemolysis.

10. Microscopic Examination: Red Blood Cells (RBCs): Presence can indicate glomerular disease, infection, or trauma. White Blood Cells (WBCs): Presence indicates infection or inflammation. Epithelial Cells: A few are normal, but an increased number can indicate contamination or renal pathology. Casts: Formed in the renal tubules; types include: Hyaline Casts: Can be normal or indicate dehydration. RBC Casts: Indicate glomerulonephritis. WBC Casts: Indicate pyelonephritis or interstitial nephritis. Granular and Waxy Casts: Indicate chronic renal disease. Bacteria and Yeast: Presence indicates infection. Crystals: Certain types can indicate metabolic disorders or predisposition to kidney stones.

11. Collection Methods: Clean Catch Midstream: Preferred method for older children who can void voluntarily. Catheterization: Used when a clean catch sample is not possible, ensuring minimal contamination. Suprapubic Aspiration: Used in infants or when sterile urine is required for diagnosis. Interpretation and Clinical Correlation: UTIs: Presence of nitrites, leukocyte esterase, bacteria, and WBCs. Glomerulonephritis: Presence of RBCs, RBC casts, and proteinuria. Nephrotic Syndrome: High levels of proteinuria, lipiduria , and possible hyaline casts. Diabetes Mellitus: Presence of glucose and ketones. Metabolic Disorders: Presence of specific crystals (e.g., cystine, oxalate).

12. Imaging Studies in Genitourinary Diseases Ultrasound (USG) Uses: Detecting congenital anomalies (e.g., hydronephrosis, polycystic kidney disease). Assessing renal size, shape, and position. Identifying obstructions, stones, and masses. Evaluating bladder volume and residual urine. Advantages: Safe for repeated use. Provides real-time imaging. Limitations: Operator-dependent. Limited by patient body habitus.

13. Voiding Cystourethrogram (VCUG) Purpose: Evaluates the bladder and urethra during urination. Detects vesicoureteral reflux (VUR) and bladder outlet obstruction. Uses: Identifying reflux of urine from the bladder into the ureters/kidneys. Assessing for posterior urethral valves in boys. Diagnosing recurrent UTIs. Procedure: Involves catheterization and filling the bladder with contrast material. Radiographic images are taken during voiding. Limitations: Invasive. Uses ionizing radiation.

14. Renal Scintigraphy (DMSA Scan) Purpose: Assesses renal cortical function and structure. Uses: Detecting acute pyelonephritis. Evaluating renal scarring. Assessing differential renal function. Procedure: Involves intravenous injection of a radiopharmaceutical agent. Gamma camera captures images of renal uptake. Advantages: Provides functional information. Limitations: Involves exposure to radiation.

15. Magnetic Resonance Urography (MRU) Purpose: Provides detailed images of the urinary tract using magnetic resonance imaging. Uses: Evaluating complex congenital anomalies. Assessing renal parenchyma, collecting systems, and vasculature. Functional assessment using contrast-enhanced techniques. Advantages: No ionizing radiation. Excellent soft tissue contrast. Limitations: Expensive and less available. Requires sedation in younger children.

16. Computed Tomography (CT) Purpose: Provides detailed cross-sectional images using X-rays. Uses: Detecting renal and ureteral stones. Evaluating traumatic injuries. Assessing complex masses and congenital anomalies. Advantages: Rapid and highly detailed. Limitations: High radiation exposure. May require sedation or anesthesia in children.

17. Intravenous Pyelogram (IVP) Purpose: Provides images of the kidneys, ureters, and bladder using contrast dye. Uses: Assessing urinary tract obstructions. Evaluating structural abnormalities. Procedure: Involves intravenous injection of contrast material followed by a series of X-rays. Limitations: Involves ionizing radiation and contrast dye, which can cause allergic reactions.

18. CYSTIC KIDNEY DISEASES Cystic kidney diseases are a group of disorders characterized by the presence of cysts (fluid-filled sacs) in the kidneys. These conditions can be inherited or acquired and vary widely in their presentation and severity.

19. Autosomal Dominant Polycystic Kidney Disease (ADPKD) Genetics: Caused by mutations in the PKD1 or PKD2 genes. Characteristics: Bilateral kidney enlargement with multiple cysts. Cysts can also be found in the liver, pancreas, and other organs. Commonly presents in adulthood but can be detected in childhood. Symptoms: Hypertension, hematuria, flank pain, recurrent urinary tract infections. Progressive renal failure in middle age or later. Diagnosis: Ultrasound, CT scan, MRI. Genetic testing.

20. Autosomal Recessive Polycystic Kidney Disease (ARPKD) Genetics: Caused by mutations in the PKHD1 gene. Characteristics: Enlarged kidneys with numerous small cysts in the collecting ducts. Often associated with congenital hepatic fibrosis. Presents in infancy or early childhood. Symptoms: Potter sequence (pulmonary hypoplasia, oligohydramnios, limb abnormalities). Hypertension, renal insufficiency, hepatomegaly. Diagnosis: Prenatal ultrasound. Postnatal ultrasound, MRI. Genetic testing.

21. Multicystic Dysplastic Kidney (MCDK) Nature: Non-hereditary, congenital disorder. Characteristics: One kidney replaced by multiple cysts of varying sizes, leading to non-functional kidney. The affected kidney usually does not function. Symptoms: Often asymptomatic. May be detected prenatally or in early childhood. Contralateral kidney compensates for the loss of function. Diagnosis: Prenatal ultrasound. Postnatal ultrasound, renal scintigraphy.

22. Multicystic Dysplastic Kidney (MCDK) Pathophysiology: Non-hereditary, disorganized renal parenchyma with varying cyst sizes. Symptoms: Typically unilateral, non-functional affected kidney. Diagnosis: Prenatal or postnatal ultrasound showing a kidney with multiple cysts and no normal renal tissue. Simple Renal Cysts Pathophysiology: Common with aging, usually asymptomatic and benign. Symptoms: Typically asymptomatic but can cause pain or infection. Diagnosis: Ultrasound showing simple cysts with clear fluid, thin walls, no septations.

23. Medullary Sponge Kidney Pathophysiology: Congenital, cystic dilation of collecting ducts. Symptoms: Hematuria, recurrent UTIs, nephrolithiasis. Diagnosis: IVP showing 'brush-like' appearance of renal medulla. Acquired Cystic Kidney Disease (ACKD) Pathophysiology: Develops in chronic kidney disease, particularly long-term dialysis patients. Symptoms: Usually asymptomatic but can cause hematuria, renal tumors, or flank pain. Diagnosis: Ultrasound or CT showing multiple cysts in chronic renal failure patients.

24. URINARY TRACT INFECTIONS(UTIs)

25. Asymptomatic bacteriuria

26. What is significant bacteriuria ? Colony count in the urine culture and methods of collection Method of collection Colony count (per mL) Suprapubic aspiration Any number Urethral catheterization >104 Midstream void >105

27. Risk factors for UTI in young children Poor perineal hygiene and unnecessary use of diapers ; Congenital anomalies of kidney and urinary tract (CAKUT) such as vesicoureteric reflux, pelvic ureteric junction obstruction, obstructive uropathy, abnormal communication between urinary tract and gastrointestinal tract, phimosis in boys, vulval synechiae in girls, and renal stones ; Bowel bladder dysfunction (BBD) presenting with features of urinary urgency, frequency, dysuria, voiding postponement during the toilet training age group, incontinence, and constipation ; Almost 50% children with recurrent UTI and 10% children presenting with a single UTI have an associated urological abnormality ; Fungal UTI is more common in immunocompromised patients, intensive care unit (ICU) setting, prolonged antibiotic usage, and in presence of indwelling catheters

28. Common organisms causing UTI E. coli in >70% cases ; Uncommon organisms—non-E. Coli bacteria and fungi (Urine sample should be processed as soon as possible ideally within 30 minutes of collecting the sample to avoid contamination and incorrect result)

29. Approach to UTI in Children

30. Further follow- up and monitoring: Aim for symptomatic improvement, complete and sustained resolution of fever, and return of normal well being Document normal urine analysis at the end of treatment of current UTI Do not repeat urine culture unless there is a new UTI/breakthrough UTI Periodic monitoring of growth Urine analysis during further febrile episodes in presence of a known risk factor for UTI Blood pressure evaluation once in 6–12 months Assess renal function once a year in a child who had severe complicated UTI or recurrent UTI Watch for proteinuria after successful treatment of UTI, it may be associated with pyelonephritic renal scarring and would need medical intervention

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