L5- Diseases of the Kidney and Ureter –P2
L5- Diseases of the Kidney and Ureter –P2
L5- Diseases of the Kidney and Ureter –P2
learning
objectives
Illustration by Smart-Servier Medical Art
Congenital cystic kidney
• Autosomal dominant
• Abdominal mass
• Haematurea
• Infection
• Hypertension
• Uraemia
• Conservative vs transplant
Hydronephrosis
• bacteria reach the kidney either from the blood or from the lower part of
the urinary system.
• More in females
• Progress to septicaemia
• Acute pyelonephritis, common type of kidney infection
Presentation Investigation
• Fever •Midstream urine sample
• Pain •Culture and senstivity
•Ultrasound
• Headache
•Renal function test
• Nausea •Blood culture
• Urgency •Cystoscope
• Frequency •Contrast study
• Dysurea
• uraemia
Treatment
• According to C and S antibiotic is
given eg. Amoxicillin and gentamycin
• It can cause significant morbidity and is a rare cause of mortality in patients with serious
comorbidities or in patients with urinary tract obstruction.
• Recurrent UTI is more common in women, affecting 30–40% in the sexually active age group.
• Asymptomatic colonization or bacteriuria is also common and can be from a UTI by the
absence of symptoms and pyuria (leukocytes in urine).
Classification
• UTI is classified as uncomplicated when it occurs in an immunocompetent host with an
anatomically normal and functional urinary tract.
• UTIs may also be classified on their site of origin as pyelonephritis (kidney), cystitis (bladder),
urethritis or prostatitis.
• While acute pyelonephritis indicates an acute infection of the kidney, chronic pyelonephritis is
only a morphological description of previous infection-related sequelae such as scarring in the
kidney as seen on radiological or nuclear imaging.
Acute pyelonephritis
• This commonly occurs as a result of ascending infection from
organisms in the lower tract, usually caused by Gram-negative
bacteria.
• It is associated with fetal growth retardation and preterm delivery. Therefore, all pregnant women
must be screened in the first trimester for ABU because, untreated, a third of these patients will
develop UTI.
• Lower tract UTI typically occurs in the first trimester whereas pyelonephritis most often presents
in the second or third trimester with acute abdominal pain or premature labor.
• Antibiotic use during pregnancy is tailored to avoid fetal harm and typically includes fosfomycin,
penicillin’s or cephalosporins
Renal and perirenal abscess
• A renal abscess results from an ascending UTI in association with an underlying urinary tract
abnormality such as obstructive uropathy or VUR.
• It is usually caused by common uropathogens such as E. coli and other Gram-negative bacilli.
• Renal abscesses may extend and perforate the renal capsule to form a perirenal abscess.
• Multiple renal abscesses may conglomerate into a solitary suppurative lesion called a renal
carbuncle. This is usually caused by Staphylococcus aureus, which reaches the kidney by
hematogenous spread.
• The clinical presentation may be insidious and non-specifc but patients usually present with
persistent fever, back pain, abdominal pain and costovertebral tenderness.
• Urine examination may be normal if the abscess does not communicate with the collecting
system.
• Treatment with antibiotics without drainage may be effective in carefully selected patients
when the abscess is small (5 cm and in patients not responding to antibiotics. Open surgical
drainage is indicated when percutaneous drainage is inadequate.
Emphysematous pyelonephritis
• This is an acute-onset, rapidly progressive, possibly lethal form of pyelonephritis characterized
by parenchymal necrosis and gas formation, caused by organisms including E. coli, Klebsiella
pneumoniae, Pseudomonas aeruginosa and Proteus mirabilis.
• Most patients have diabetes (up to 90%) and they may have obstruction secondary to calculi or
papillary necrosis.
• Increased glucose levels in those with diabetes may provide a substrate for carbon dioxide
production from fermentation.
• Symptoms are suggestive of pyelonephritis and an abdominal mass may be palpable.
• CECT of the abdomen is diagnostic and shows gas in the renal parenchyma, collecting system
or both, along with other features of infection such as abscess, obstruction and perinephric
stranding.
• Emergency nephrectomy is rarely required and is reserved for patients who do not respond to
the described measures.
Xanthogranulomatous pyelonephritis
• Xanthogranulomatous pyelonephritis (XGP) occurs with severe renal infection in an
obstructed kidney and is usually associated with calculi, causing loss of function and
parenchymal destruction.
• Patients may present with flank pain, fever with chills, persistent bacteriuria and a flank mass.
• It is secondary and caused by hematogenous spread of tubercle bacilli from the thoracic lymph
nodes or the lungs.
• Macrophages react and granulomas are formed. If bacterial multiplication goes unchecked,
caseous necrosis results in the formation of tubercles.
• Multiple tubercles coalesce and rupture into the collecting system, causing intermittent
tuberculous bacilluria and pyuria.
• The disease spreads through the collecting system with ulceration initially.
• When bacterial multiplication is halted by the immune system, sequelae due to fibrosis
appear.
• Tubercular obstructing or destructive lesions in the kidneys and ureters are responsible for
renal function loss.
• The disease gradually involves the bladder musculature, which is replaced by fibrous tissue,
causing a decrease in the size and capacity of the bladder (‘thimble’ bladder).
• Urinary bladder involvement is responsible for urinary frequency, which is the most common
symptom of GUTB.
• Epididymal tuberculosis presents as a painless epididymal nodule, usually involving the tail of
the epididymis, or a chronic discharging sinus in the posterior scrotal wall
• Patients may present with urinary frequency, colicky flank pain, hematuria and, rarely, fever and
constitutional symptoms.
• They may also present with symptoms suggestive of recurrent UTIs and, rarely, calcified
tubercular lesions may be misdiagnosed as urinary tract calculi.
• For microbiological confirmation, at least three consecutive earlymorning specimens of urine are
examined for acid-fast bacilli.
• The gold standard for microbiological diagnosis is urine culture.
• Nucleic acid amplification tests (NAATs) provide rapid diagnosis (within hours).
• When the diagnosis remains uncertain, bladder biopsy, tissue culture and tissue NAATs may be
required.
• Imaging with CTU may also help and can show early signs such as calyceal distortion and
papillary necrosis, hydronephrosis, poor function of renal segments secondary to parenchymal
destruction, fibrosis and chronic obstruction.
• Ureteric strictures and proximal dilatation may also be seen.
• IVU can pick up the earliest signs of disease activity, such as calyceal distortion
• Treatment involves short-course antituberculous therapy (ATT). Rifampicin, isoniazid and
pyrazinamide are used sometimes with ethambutol as first-line drugs.
• The primary aim of therapy is preservation of renal function and avoidance of fibrotic sequelae.
• Ureteric strictures may require double J (DJ) stenting to preserve function until definitive
reconstruction is attempted.
• The choice of reconstructive procedure depends on the type and location of sequelae.
• Open surgical repair is generally superior to balloon dilatation for tubercular ureteric strictures.
• Augmentation enterocystoplasty (usually using ileum) for small-capacity bladder ureteric
reimplantation with or without a Boari fap (bladder tube) for lower ureteric stricture and ileal
replacement of the ureter for multiple long ureteric strictures may be required.
• Nephrectomy is done for major renal lesions with a poorly functioning kidney.
• Urinary infection in childhood and vesicoureteric reflux All children with UTI must be evaluated
for underlying predisposing conditions as recurrent pyelonephritis can cause renal scarring and
loss of renal function.
• UTIs account for 7% of childhood febrile illness. In the age group 1 year, it is more common in
females.
• Structural and functional abnormalities of the urinary tract such as VUR and posterior urethral
valves predispose to UTI.
• Reflux is considered primary when it is due to an incompetent UVJ and secondary when it is
due to increased bladder pressure or outlet obstruction.
• Presenting symptoms in neonates and infants include febrile illness or sepsis and may not be
localized to the urinary tract.
• The method of urine sampling, especially before toilet training, is crucial and may involve
suprapubic aspiration or per urethral catheter collection.
• A bacterial count of 50 000 colony-forming units per milliliter is generally considered a positive
culture result in children, although a lower count from a suprapubic aspirate in a symptomatic
child is significant.
• The most important complication of UTI in a child is renal scarring secondary to renal
parenchymal inflammation.
• US should be performed in all children, and children with recurrent UTIs or a first time UTI with
pyelonephritis should be evaluated further.
• VCUG is the investigation of choice to diagnose reflux and should be performed in high-risk
children. 99mTc dimercaptosuccinic acid (DMSA) radionuclide cortical scan is the best modality
to detect parenchymal lesions.
• VUR is present in approximately 30% of children with UTI and in up to 90% of children with
renal scarring.
• Renal scarring may cause hypertension in up to 20% and is an important cause of renal
failure.
• The grades of VUR are summarized in Figure 82.9, with grades I–III generally resolving
spontaneously.
Treatment
• Radical nephrectomy
• Immunotherapy
• Tyrosin kinase inhibitors
Tumours of the kidneys and ureters
Upper tract urothelial cancer
• Primary urothelial neoplasms of the renal pelvis and ureter are rare.
• risk factors are tobacco consumption, occupations in the dye, petrochemicals and rubber
industries, analgesic abuse, high arsenic content in drinking water, exposure to
cyclophosphamide and the presence of chronic inflammation.
• Chronic inflammatory conditions are also associated with squamous cell carcinoma.
Presentation
• Patients commonly present with gross hematuria, with or without flank pain and occasionally
clot colic.
• Patients with known bladder tumors should always be screened for upper tract tumors.
• Very few present with advanced constitutional symptoms and a palpable mass.
Renal oncocytoma
• This derives its name from its cellular appearance on histopathology, where uniformly
highly granular eosinophilic cytoplasm owing to abundant mitochondria (oncocyte) is seen.
• It accounts for around 5% of renal tumors.
• It appears as an enhancing mass on cross-sectional imaging and is difficult to differentiate
from RCC.
Renal angiomyolipoma
• Angiomyolipoma comprises a composite mix of fat tissue with dysmorphic blood vessels
and smooth muscle.
Others include
• diuretics,
• occupational exposure to petrochemicals and dyes and
• ARCD in patients on long-term hemodialysis.
Clinical presentation
• The classic triad of flank pain, hematuria and a palpable mass is now uncommon as most
renal masses are detected incidentally. Symptoms and signs may be non-specific.
• Patients may have constitutional symptoms such as fever, malaise and weight loss in
advanced disease. Advanced disease can present with bilateral lower limb oedema or recent-
onset non-reducing right-sided varicocele owing to thrombus in the IVC.
• Paraneoplastic syndromes (PNSs) are found in up to one third of patients with RCC.
• The most common PNS is an elevated erythrocyte sedimentation rate (ESR) followed by
hypertension, anemia and hypercalcemia.
• The most common site of metastasis is the lung and is classically described as cannon ball
metastases. Metastases may occasionally present as pathological fractures.
Pathology
• ccRCC is histologically an adenocarcinoma arising from the proximal renal tubular epithelium.
• They are slow growing and bulge out of the renal contour.
• The tumor can spread directly, invading the perinephric tissue through the capsule or at times
directly extending into the renal vein as a tumor thrombus.
o Increased alkaline phosphatase should prompt further investigation to rule out liver and
skeletal metastases.
o LDH is useful in risk stratification of metastatic disease.
• The tumor has mixed elements derived from the embryonic nephrogenic tissue, namely
blastemal or undifferentiated tissue, epithelial tubules and stroma.
• The typical presentation is a child aged between 1 and 4 years of either gender with a
large,15 palpable abdominal mass that may cross the midline.
• It may also be associated with hematuria, hypertension, fever and weight loss.
• US can confirm the renal origin and solid nature of the mass.
• Further definitive imaging with either CECT or MRI is necessary to stage the disease.
• The tumors usually infiltrate the kidneys and normal renal parenchyma is compressed at the
periphery around the tumor (claw sign).
• A CT of the chest should be obtained as the lung is the most common site of distant metastasis.