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PaediatricRenalandUrology Topicswe’regoingtocover Renal Urology Nephrotic Syndrome Urinary Tract Infections Nephritic Syndrome Undescended testes (cryptorchidism) Henoch-Schonlein Purpura Testicular Torsion Haemolytic Uraemic Syndrome Hypospadias Polycystic Kidney Disease Hydrocele Wilm’s TumourPaediatricRenalNephroticSyndrome What is it? Investigations: • Leaky basement membrane in the • Bloods inc. glomerulus • Permeable to protein à leaks into the • FBC,U&E,LFTs, urine Clotting,Cholesterol and lipids,+ HbA1c • Most commonly caused by minimal (can be caused by change disease diabetes) Gold Standard = renal biopsy and microscopy Clinical Features Management Triad of: • High dose prednisolone (usually 4 1.Proteinuria (3+ urine dip) weeks) 2.Hypoalbuminaemia (<25) • 80% children respond 3.Oedema • Some are steroid resistant and Urine – frothy may need ACEi & immunosuppression Child’s Appearance – pale with • Diuretics generalised oedema • Diet à low saltNephriticSyndrome Investigations: • Urine dip • Early morning urine for What is it? albumin:creatinine ratio • Hallmark = microscopic • Autoantibodies,ESR, complements haematuria • Most commonly = post- • Clotting infection (Group A strep) • ASOT (anti-streptolysin O antibody titres - looks for • IgA nephropathy à post URTI strep) Management • Supportive Clinical • Fluids & electrolyte monitoring Features • Haematuria • Proteinuria • Rapidly progressive • Oliguria glomerulonephritis = the rapid • Oedema deterioration of renal function • Crescent formation on renal • HTN biopsy • SteroidsHenoch-SchonleinPurpura- Investigations: What is it? • BP IgAVasculitis (autoimmune) • Urine dip Can be triggered by infection, • U&Es vaccinations • Albumin • FBC = normal platelets, important when considering differentials of Clinical Features a purpuric rash • Purpuric rash – raised,over extensor surfaces of limbs and buttocks • Joint Pains – especially Management knees and ankles Simple analgesia (no NSAIDs if impaired • Abdo Pain renal function) May need discussion with Paediatric NephrologyNephritis–IgANephropathy(Berger’ sDisease) What is it? • IgAVasculitis related to HSP • Deposit IgA into nephrons à inflammation Investigations: Renal biopsy = GOLD STANDARD Clinical Features 1. Reduced GFR IgA deposits + glomerular 2. Haematuria mesangial proliferation 3. Proteinuria Management Mostly supportive Steroids + immunosuppression to slow disease HaemolyticUraemicSyndrome=MedicalEmergency Clinical Features • Gastroenteritis – often bloody diarrhoea • HUS often starts 5 days after diarrhoea onset • Reduced urine output • Abdo pain What is it? • Haematuria Another triad: • Lethargy/ confusion/ irritability 1. Haemolytic Anaemia- destruction of rbc • Oedema (microantiopathic,non-immune,coombs • HTN • Petechiae negative) 2. Thrombocytopaenia 3. AKI Management Basically = lots of clots in small blood • Urgent referral to Paeds Nephrology vessels for dialysis if needed • Fluid balance Often caused by E.Coli 0157 diarrhoea à • May need a blood transfusion and produces Shiga toxin antihypertensives PolycysticKidneyDisease What is it? • In children,most commonly autosomal recessive polycystic kidney disease • PKHD1 gene chromosome 6 Complications: • Renal failure Clinical Features • Liver failure & fibrosis à • Presents in neonates portal hypertension à • Normally picked up on USS varices • Chronic lung disease antenatally • Cystic enlargement of renal collecting ducts Management • Oligohydramnios à pulmonary Dialysis hypoplasia as a result à Potter Multi-specialty involvement Syndrome High mortality (only approx.33% survive • Congenital Liver fibrosis to adulthood) WilmsT umour(Nephroblastoma) What is it? Investigations: • Renal tumour • Children <5 yrs • Urine dip • Accounts for 5% all cancer in children • Blood tests • Abdo USS • Can be bilateral • May require CT/MRI for • Pathophysiology poorly understood staging • GOLD STANDARD = renal biopsy Management Clinical Features • Initially provide supportive care • Abdominal mass • Definitive management = • Abdo pain nephrectomy • Weight loss/ fever/ lethargy • May need adjuvant chemotherapy • HTN • 85% cure rate • HaematuriaPaediatric Urology UrinaryT ractInfectionsinChildren What is it? • An infection anywhere Investigations: in the urinary tract • Clean catch urine from the urethra all the • Urine dip - send for MC&S way up to the kidneys • Cystitis = inflammation • Recurrent UTIs – USS of the bladder • For any child <6m with UTI • Or recurrent/ atypical UTIs • Do within 6 weeks Clinical Features Babies: Management • Fever/lethargy/irritability • Admit any child <3 months + fever for • Poor feeding IV abx! (will need a full septic screen and cover for meningitis) • Vomiting • Any child with features of • Frequency sepsis/pyelonephritis – admit for IV Children: abx • Abdo pain • Otherwise PO abx okay (usually • Dysuria trimethoprim,nitrofurantoin,cefalexin, • Incontinence amoxicillin) DMSAScans Vesico-Ureteric Reflux What is it? • Injection of Dimercaptosuccinic Acid What is it?: (radioactive) + gamma camera • Reflux of urine into the ureters from assessment to see renal take up the bladder • Assesses for renal scarring from • Patients are more likely to develop previous infections pyelpnephritis Management Should be done 4-6 months after • Micturating atypical or recurrent infections Cystourethrogra m to diagnose • Definitive Mx = surgery under Paediatric urology • Conservative Mx: avoiding constipation and full bladder • Prophylactic abx UndescendedT estes(Cryptorchidism) What is it? • Testes that have not descended into Management the scrotum • Initially watch an wait • Testes develop in the abdomen and • Most descend within first 3-6 months migrate into the scrotum and by 1 year • Refer to Paeds Urology if not • May be palpable in the inguinal canal descended by 6 months • Can increase risk of • Orchidopexy done at age 6-12 months • Torsion • Testicular cancer • Infertility Risk Factors • Prematurity • Low birth rate • SGA • FHx • Maternal smoking Hypospadias What is it? • Opening of the urethra (meatus) is on the ventral aspect of the penis • 90% of cases more towards the glans, but can be more proximally Clinical Features Found during NIPE assessment of the newborn (congenital) May have difficulty with urination, sexual dysfunction,cosmetic and psychological concerns in later life Management • Referral to Paediatric urology • Avoid circumcision until seen by a urologist • Surgery at 3-4 months TesticularT orsion Investigations: If history and What is it? examination suspicious – go • Spermatic cord twists within the tunica vaginalis immediately to theatre for scrotal • Surgical emergency exploration • Peaks in neonatal period and then Testicular USS – can assess blood flow adolescents 12-25yr Examination: • RF:Age,FHx,Previous torsion, Absent cremasteric reflex Undescended testes High position of testis in comparison to opposite side Horizontal lie of testis May be swollen and very tender Clinical Features • Severe unilateral testicular pain Management • Usually sudden-onset • Aim for surgery within 4-6 hours of • May have associated onset of symptoms nausea and vomiting • If viable testis – untwist and perform • May experience referred bilateral orchidopexy abdo pain • If non-viable testis – orchidectomy and contralateral orchidopexyAny questions? Thankyou!