Henoch-Schönlein Purpura

Henoch-Schönlein purpura is a condition that can occur one to three days after an infection affecting an IgA-secreting mucous membrane. It commonly follows pharyngitis but can also occur after infection of the gastrointestinal tract, bladder, or breast. The condition is characterized by painless macroscopic haematuria, which can be accompanied by microscopic haematuria and proteinuria. The rash is due to a cutaneous vasculitis, and abdominal pain is due to gut vasculitis, which can be severe in some cases, leading to bloody diarrhoea. Arthralgia is also a common symptom.

In adults, the cause of similar symptoms and signs may not necessarily be HSP, but may be any type of vasculitis. However, in children, HSP is the most common cause of vasculitis affecting the kidneys. Patients with hypertension and/or proteinuria should be started on an angiotensin-converting enzyme (ACE) inhibitor, which may control blood pressure and proteinuria. If there is worsening renal function or proteinuria, a renal biopsy should be performed. If the biopsy shows changes of a crescentic glomerulonephritis (GN), then and immunosuppression regimen similar to that used in renal vasculitis should be started, probably with high dose steroids in the first instance, plus or minus cyclophosphamide.

Overall, the prognosis for patients with Henoch-Schönlein purpura with minimal renal involvement is good, as long as there is no protein and only 1+ blood on urine dipstick.

Treatment for Calcium Urinary Tract Stones

Calcium urinary tract stones can be caused by idiopathic hypercalciuria, which is often familial and results in increased gastrointestinal absorption of calcium. The most common type of stone is calcium oxalate. In a patient with normal serum calcium but raised urinary excretion of calcium, the recommended treatment is to increase their daily urinary output to over 2000 ml. This can be achieved by advising the patient to increase their fluid intake. A high protein intake and excessive dairy consumption can also contribute to the formation of urinary stones, so reducing these dietary factors may be helpful in preventing future stones. However, the initial focus should be on increasing urine volume through increased fluid intake.

Urinalysis is the most appropriate initial test for investigating the cause of haematuria, as it is a simple and effective way to confirm that the discolored urine is due to blood. Haematuria can have various causes, including stones, haematological disorders, infection, tumours, trauma, and certain treatments. In this case, the patient’s vigorous exercise routine puts him at risk of rhabdomyolysis, which can be detected by haemoglobin on a urine dipstick test but not by red blood cells on microscopy. Other potential causes of haematuria, such as renal stones or urological malignancy, are unlikely in a young, healthy man without pain. A KUB X-ray can be useful for identifying calcium-containing renal stones, but it is not necessary in this case. A CT scan of the abdomen is not recommended as it would expose the patient to unnecessary radiation and is unlikely to reveal any relevant information. The ESR may be elevated in infectious or inflammatory causes of rhabdomyolysis, but it is not specific to this condition. A renal ultrasound is not helpful in detecting rhabdomyolysis and is mainly used to assess kidney size and exclude hydronephrosis as a cause of acute kidney impairment. Overall, maintaining adequate hydration is the main treatment for haematuria in this case.

Renal Artery Stenosis and its Association with Hypertension

Renal artery stenosis is a condition that can lead to hypertension. It is commonly seen in patients who have underlying vascular diseases and have a poor prognosis, with an 80% mortality rate within five years, especially if they have concurrent coronary disease. The primary cause of renal artery stenosis is atherosclerosis, but it can also be caused by other factors such as fibromuscular dysplasia, vasculitis, and external compression.

One of the typical changes seen in renal artery stenosis is asymmetrical kidneys, with the affected kidney being more than 2 cm smaller than the unaffected kidney. It is important to note that ACE inhibitors are contraindicated in this condition as they inhibit the contraction of the efferent arterioles, which promote glomerular filtration in the disease. Therefore, it is crucial to diagnose and manage renal artery stenosis promptly to prevent further complications.

Nephrotic syndrome is a condition characterized by heavy proteinuria and nephrotic-range protein loss. Two common types of nephrotic syndrome are minimal change nephropathy and membranous nephropathy. Understanding the light microscopy features of these conditions is crucial for accurate diagnosis and treatment.

Minimal change nephropathy is a childhood disorder that can also affect adults. It is characterized by sudden onset of lower limb edema, heavy albuminuria, and normal glomeruli on light microscopy. Effacement of podocyte foot processes is an electron microscopy finding in minimal change nephropathy.

Membranous nephropathy can be primary or secondary. Primary membranous nephropathy is driven by phospholipase A2 receptor antibodies and is treated with aggressive immunosuppression. Secondary membranous nephropathy commonly occurs as a result of an underlying malignancy. The classical pattern of histopathology in membranous nephropathy is spike formation of the basement membrane.

It is important to note that other conditions, such as focal segmental glomerulosclerosis, can present with similar symptoms and may require different treatment approaches. Accurate diagnosis and appropriate treatment can improve outcomes for patients with nephrotic syndrome.

The patient’s symptoms of hypochromic, microcytic anemia and gout are consistent with lead poisoning, which can also cause nephropathy and lead to proximal tubular defects. Penicillamine, DMSA, and calcium disodium edetate are effective treatments for lead poisoning. Hypertensive nephropathy is often misdiagnosed as lead nephropathy due to the presence of hypertension, but lead nephropathy causes worse hyperuricemia and gout. Acute interstitial nephritis is typically caused by hypersensitivity reactions to drugs, while chronic interstitial nephritis can be caused by lead poisoning. Pseudohypoaldosteronism is unlikely due to the patient’s mild hypokalemia and reduced GFR. Atenolol is not likely to have caused the kidney disease, but dose adjustment is necessary based on the patient’s creatinine clearance.

Management of Renal Anaemia

This patient is likely suffering from renal associated anaemia due to a deficiency of erythropoietin. According to Renal Association guidance, it is important to ensure that the patient is iron replete before considering an erythropoiesis-stimulating agent. Therefore, the patient’s iron stores should be checked before starting erythropoietin. Although the reticulocyte count may be elevated, it would not change the management of the patient.

While a blood transfusion may increase the haemoglobin levels temporarily, it is not a viable long-term solution for managing renal anaemia. A marrow biopsy may be considered if there is a lack of response to iron and erythropoietin, but it is likely to be normal given the normal white count and platelet count.

In summary, the management of renal anaemia involves ensuring that the patient is iron replete before considering erythropoietin. Blood transfusions are not a long-term solution, and a marrow biopsy may be considered if there is a lack of response to treatment.

Following renal transplantation, Alport syndrome patients may develop anti-GBM disease, with approximately 5 percent of patients experiencing de novo antibody development. The antibodies primarily target the collagen alpha-5(IV) chain, rather than the Goodpasture antigen. While ANCA-associated vasculitis can lead to crescentic glomerulonephritis, there are no indications of this in the current case. Graft dysfunction may be caused by acute rejection, tubulointerstitial nephritis, or CMV infection, but these conditions would produce distinct biopsy findings.

Alport’s syndrome is a genetic disorder that is typically inherited in an X-linked dominant pattern. It is caused by a defect in the gene responsible for producing type IV collagen, which leads to an abnormal glomerular-basement membrane (GBM). The disease is more severe in males, with females rarely developing renal failure. Symptoms usually present in childhood and may include microscopic haematuria, progressive renal failure, bilateral sensorineural deafness, lenticonus, retinitis pigmentosa, and splitting of the lamina densa seen on electron microscopy. In some cases, an Alport’s patient with a failing renal transplant may have anti-GBM antibodies, leading to a Goodpasture’s syndrome-like picture. Diagnosis can be made through molecular genetic testing, renal biopsy, or electron microscopy. In around 85% of cases, the syndrome is inherited in an X-linked dominant pattern, while 10-15% of cases are inherited in an autosomal recessive fashion, with rare autosomal dominant variants existing.

In patients with chronic kidney disease and a ferritin level below 200, it is important to address any iron deficiency before considering erythropoiesis-stimulating agents. Blood transfusions should be avoided if possible to prevent antigen-sensitization in case the patient becomes eligible for a transplant. If iron levels do not improve after transfusion, further investigation for potential sources of blood loss, such as gastrointestinal bleeding, should be considered. An OGD would be appropriate for this purpose, while faecal occult blood testing is only useful for screening and not for investigating iron deficiency anaemia.

Anaemia in Chronic Kidney Disease

Patients with chronic kidney disease (CKD) may develop anaemia due to various factors, with reduced erythropoietin levels being the most significant. This type of anaemia is usually normochromic normocytic and becomes apparent when the GFR is less than 35 ml/min. Other causes of anaemia should be considered if the GFR is greater than 60 ml/min. Anaemia in CKD can lead to the development of left ventricular hypertrophy, which is associated with a three-fold increase in mortality in renal patients.

There are several causes of anaemia in renal failure, including reduced erythropoietin levels, reduced erythropoiesis due to toxic effects of uraemia on bone marrow, reduced absorption of iron, anorexia/nausea due to uraemia, reduced red cell survival (especially in haemodialysis), blood loss due to capillary fragility and poor platelet function, and stress ulceration leading to chronic blood loss.

To manage anaemia in CKD, the 2011 NICE guidelines suggest a target haemoglobin of 10-12 g/dl. Determination and optimisation of iron status should be carried out before the administration of erythropoiesis-stimulating agents (ESA). Oral iron should be offered for patients who are not on ESAs or haemodialysis. If target Hb levels are not reached within three months, patients should be switched to IV iron. Patients on ESAs or haemodialysis generally require IV iron. ESAs such as erythropoietin and darbepoetin should be used in those who are likely to benefit in terms of quality of life and physical function.

Due to excessive consumption of NSAIDs, this individual has developed tubulointerstitial nephritis. Although an autoimmune vasculitis is a possibility, the presence of respiratory symptoms would be typical of Churg-Strauss syndrome.

Acute interstitial nephritis is a condition that is responsible for a quarter of all drug-induced acute kidney injuries. The most common cause of this condition is drugs, particularly antibiotics such as penicillin and rifampicin, as well as NSAIDs, allopurinol, and furosemide. Systemic diseases like SLE, sarcoidosis, and Sjögren’s syndrome, as well as infections like Hanta virus and staphylococci, can also cause acute interstitial nephritis. The histology of this condition shows marked interstitial oedema and interstitial infiltrate in the connective tissue between renal tubules. Symptoms of acute interstitial nephritis include fever, rash, arthralgia, eosinophilia, mild renal impairment, and hypertension. Sterile pyuria and white cell casts are common findings in investigations.

Tubulointerstitial nephritis with uveitis (TINU) is a condition that typically affects young females. Symptoms of TINU include fever, weight loss, and painful, red eyes. Urinalysis is positive for leukocytes and protein.

The patient has severe hyperkalaemia and needs immediate treatment to stabilize the myocardium. Calcium gluconate should be administered first, followed by definitive treatment such as haemodialysis. Holding measures like insulin/dextrose infusions may not be necessary if dialysis can be organized quickly. Nebulized salbutamol may be effective in lowering serum potassium, but it may not work for all patients. Sodium bicarbonate is not recommended for acute hyperkalaemia treatment due to potential side effects. The patient also has metabolic acidosis related to renal failure.

The patient’s chronic use of analgesics for their lower back pain has resulted in tubulo-interstitial nephritis and papillary necrosis. This can cause the papillae to slough off and potentially obstruct the urinary tract, leading to acute kidney injury or chronic renal failure. Additionally, a low sodium level may be present due to a salt-losing nephropathy commonly associated with this condition.

Understanding Papillary Necrosis: Causes and Features

Papillary necrosis is a medical condition that affects the kidneys, specifically the renal papillae. This condition is characterized by the death of the cells in the renal papillae, which can lead to various complications. There are several causes of papillary necrosis, including chronic analgesia use, sickle cell disease, tuberculosis, acute pyelonephritis, and diabetes mellitus.

One of the most common causes of papillary necrosis is the chronic use of analgesics, particularly nonsteroidal anti-inflammatory drugs (NSAIDs). Sickle cell disease, which affects the shape of red blood cells, can also lead to papillary necrosis. Tuberculosis and acute pyelonephritis, which are both bacterial infections, can also cause this condition. Finally, diabetes mellitus, a chronic metabolic disorder, can also lead to papillary necrosis.

The features of papillary necrosis include fever, loin pain, and hematuria. These symptoms can be indicative of other medical conditions, so it is important to undergo diagnostic tests to confirm the presence of papillary necrosis. One such test is an intravenous urogram (IVU), which can show papillary necrosis with renal scarring, also known as cup and spill.

Intervention for Proximal Renal Tract Infection

When faced with a situation of significant risk from continued proximal renal tract infection, intervention to relieve the obstruction is required. Out of the available options, ureteroscopy and stone removal is the best choice as it carries the lowest potential burden of morbidity. However, this procedure is only considered for selected patients without stent placement. Nephrostomy is inappropriate as it carries a greater burden of morbidity versus ureteroscopy. Continued rehydration is not recommended as it does not address the symptoms of infection and obstruction. Lithotripsy is not suitable as it may worsen sepsis in the short term by leading to disseminated bacteraemia. Ureteric stenting is suboptimal as stents may become obstructed or dislodged, leading to subsequent re-presentation with symptoms of ureteric colic.

Granulomatosis with Polyangiitis: Symptoms and Treatment

Granulomatosis with polyangiitis is a condition that affects the small vessels of the kidney and other organs, particularly the lungs. The disease progresses rapidly, causing renal failure within three months, proteinuria, and microscopic haematuria. Patients also experience upper airway involvement, such as nasal ulceration, crusting, rhinorrhoea, and epistaxis, and often have granulomatous lung disease accompanied by alveolar capillaritis.

When a patient presents with symptoms of granulomatosis with polyangiitis, urgent treatment is necessary. Dialysis is often required due to acidosis, hyperkalaemia, and uraemia. Although pulmonary involvement is common, it does not require specific intervention at this stage. Without treatment, the disease is fatal, but with immunosuppression, 70% of patients with severe focal necrotising glomerulonephritis regain independent renal function.

The first step in treatment is to administer methylprednisolone, followed by haemodialysis and then cyclophosphamide. Plasma exchange may also be beneficial for patients already requiring dialysis. Early intervention is crucial in managing granulomatosis with polyangiitis and improving patient outcomes.

Treatment Options for Xanthogranulomatous Pyelonephritis

Xanthogranulomatous pyelonephritis (XGP) is a condition that can be difficult to distinguish from renal cell carcinoma. It typically presents with symptoms such as fever, weight loss, and loin pain. XGP is more common in diabetics, the immunocompromised, and patients with obstructive uropathy. The most common organism responsible for XGP is Proteus mirabilis. Diagnosis is confirmed through histology combined with clinical and radiological evidence.

The definitive treatment for XGP is nephrectomy, as medical therapy alone is insufficient. Antituberculous treatment is not appropriate for XGP, as this condition is not caused by renal tuberculosis. Intravenous antibiotics alone are also not enough to treat XGP completely. Lithotripsy, an ultrasound treatment for renal stones, is not utilized in the treatment of XGP.

Radiotherapy is also not helpful in treating XGP, as it is an infection that requires antibiotics and nephrectomy for treatment. Therefore, the most effective treatment option for XGP is nephrectomy combined with appropriate antibiotic therapy.

If a patient has an increased PSA level, they should not receive testosterone replacement therapy even if they have a deficiency. For instance, a patient with panhypopituitarism and low testosterone levels would typically require replacement therapy due to osteoporosis, regardless of sexual function and erectile dysfunction issues. However, if the patient has a history of prostate cancer, treatment is not recommended. Other contraindications include a PSA level exceeding 4ng/ml, male breast cancer, severe sleep apnea, and severe lower urinary tract symptoms caused by benign prostatic hyperplasia.

Erectile dysfunction (ED) is a condition where a man is unable to achieve or maintain an erection that is sufficient for sexual performance. It is not a disease but a symptom that can be caused by organic, psychogenic, or mixed factors. It is important to differentiate between the causes of ED, with factors such as a gradual onset of symptoms and lack of tumescence favoring an organic cause, while sudden onset of symptoms and decreased libido favoring a psychogenic cause. Risk factors for ED include cardiovascular disease, alcohol use, and certain medications.

To assess for ED, it is recommended to measure lipid and fasting glucose serum levels to calculate cardiovascular risk. Free testosterone should also be measured in the morning, and if low or borderline, further assessment may be needed. PDE-5 inhibitors, such as sildenafil, are the first-line treatment for ED and should be prescribed to all patients regardless of the cause. Vacuum erection devices can be used as an alternative for those who cannot or will not take PDE-5 inhibitors. Referral to urology may be appropriate for young men who have always had difficulty achieving an erection, and those who cycle for more than three hours per week should be advised to stop.

Treatment for Goodpasture’s Syndrome

Goodpasture’s syndrome is a serious medical condition that can lead to life-threatening pulmonary hemorrhage and severe renal impairment. The initial treatment for this condition is plasmapheresis, which aims to remove the circulating antiglomerular basement antibody disease (anti-GBM) antibodies. This is followed by high-dose pulsed methylprednisolone, which is given alongside or soon after plasma exchange has been initiated. Cyclophosphamide therapy and high-dose oral prednisolone are then administered, with the latter being gradually reduced to a lower maintenance dose.

Once remission has been induced, methotrexate and azathioprine may be used as substitutes for cyclophosphamide. This is because the toxicity of cyclophosphamide makes its long-term use undesirable. Overall, the treatment for Goodpasture’s syndrome involves a combination of plasmapheresis, high-dose steroids, and immunosuppressive drugs. It is important to note that this treatment plan should be closely monitored by a healthcare professional to ensure its effectiveness and safety.

Kidney stones can form when there is an excess of calcium, oxalate, or uric acid in the urine, a lack of citrate, or insufficient water in the kidneys to dissolve waste products. Dehydration can lead to the formation of crystals that gradually build up into kidney stones. Urine typically contains chemicals that prevent crystal formation, but low levels of these inhibitors can contribute to stone formation. Most kidney stones pass on their own, but some patients may require hospitalization due to severe pain, inability to retain fluids, infection, or inability to pass the stone. Treatment involves hydration and pain relief, with methods of stone removal considered for those who do not pass the stone naturally. Intravenous furosemide and broad-spectrum antibiotics are not recommended unless there is a specific indication.

Management of Obstructive Uropathy

Patients with obstructive uropathy caused by an enlarged prostate can experience relief with a urinary catheter. After the procedure, patients may produce a large volume of dilute urine, but over time, their tubules will recover their function, and their urine will begin to concentrate appropriately. Adequate hydration is crucial during this recovery period, and fluid restriction should be avoided to prevent severe dehydration. Psychiatric review is unnecessary as this condition is not a psychiatric illness. Desmopressin, a medication used in cranial diabetes insipidus, is not indicated in this context. Overall, the main approach to managing obstructive uropathy is to ensure proper hydration while the kidneys recover their ability to concentrate urine and manage fluid balance.

Post-streptococcal glomerulonephritis is a condition that typically occurs 7-14 days after an infection caused by group A beta-haemolytic Streptococcus, usually Streptococcus pyogenes. It is more common in young children and is caused by the deposition of immune complexes (IgG, IgM, and C3) in the glomeruli. Symptoms include headache, malaise, visible haematuria, proteinuria, oedema, hypertension, and oliguria. Blood tests may show a raised anti-streptolysin O titre and low C3, which confirms a recent streptococcal infection.

It is important to note that IgA nephropathy and post-streptococcal glomerulonephritis are often confused as they both can cause renal disease following an upper respiratory tract infection. Renal biopsy features of post-streptococcal glomerulonephritis include acute, diffuse proliferative glomerulonephritis with endothelial proliferation and neutrophils. Electron microscopy may show subepithelial ‘humps’ caused by lumpy immune complex deposits, while immunofluorescence may show a granular or ‘starry sky’ appearance.

Despite its severity, post-streptococcal glomerulonephritis carries a good prognosis.

This man has been diagnosed with nephrotic syndrome with a membranous pattern on histology, which can have various underlying causes.

Despite reporting a previous throat infection, his ASOT titre is within normal range, indicating that post-infectious GN is unlikely.

Penicillin typically causes acute interstitial nephritis, which does not present with nephrotic syndrome.

Idiopathic membranous glomerulonephritis is highly specific to phospholipase A2.

While membranous GN can be caused by systemic lupus and anti-phospholipid syndrome, this man does not exhibit typical symptoms associated with either condition. His arthralgia and myalgia are likely related to his recent throat infection. Low complement levels are typically associated with systemic lupus, while anti-cardiolipin antibodies are associated with anti-phospholipid syndrome.

Membranous glomerulonephritis is the most common type of glomerulonephritis in adults and is the third leading cause of end-stage renal failure. It typically presents with proteinuria or nephrotic syndrome. A renal biopsy will show a thickened basement membrane with subepithelial electron dense deposits, creating a spike and dome appearance. The condition can be caused by various factors, including infections, malignancy, drugs, autoimmune diseases, and idiopathic reasons.

Management of membranous glomerulonephritis involves the use of ACE inhibitors or ARBs to reduce proteinuria and improve prognosis. Immunosuppression may be necessary for patients with severe or progressive disease, but many patients spontaneously improve. Corticosteroids alone are not effective, and a combination of corticosteroid and another agent such as cyclophosphamide is often used. Anticoagulation may be considered for high-risk patients.

The prognosis for membranous glomerulonephritis follows the rule of thirds: one-third of patients experience spontaneous remission, one-third remain proteinuric, and one-third develop end-stage renal failure. Good prognostic factors include female sex, young age at presentation, and asymptomatic proteinuria of a modest degree at the time of diagnosis.

Vascular Access for Dialysis in Stage 4 Kidney Disease

Planning for vascular access is crucial for patients with stage 4 kidney disease to avoid the need for emergency dialysis. According to the current Renal Association guidelines, an arterio-venous fistula is the preferred choice for vascular access in dialysis patients. This is due to its longevity and lower risk of infection compared to other options. Arterio-venous grafts, which use prosthetic material, are the second choice preference for vascular access. However, they have a reduced lifespan compared to arterio-venous fistulas.

Dialysis catheters, both tunnelled and non-tunnelled, carry a risk of infection and are not recommended as the first line of vascular access. They can be used in emergency situations or as an interim measure while waiting for more permanent dialysis access. On the other hand, a Hickman line is a permanent single lumen line inserted into the subclavian vein. It is commonly used to deliver antibiotics or chemotherapeutic agents but is not suitable for dialysis.

In summary, planning for vascular access is essential for patients with stage 4 kidney disease to avoid the need for emergency dialysis. Arterio-venous fistulas are the preferred choice for vascular access due to their longevity and lower risk of infection. Arterio-venous grafts are the second choice preference, while dialysis catheters and Hickman lines are not recommended as the first line of vascular access.

All the options provided could potentially cause the observed clinical presentations. Diabetic kidney disease may lead to fluid overload and result in pedal edema, as well as explain the proteinuria, but it cannot account for the hypoalbuminemia. Congestive cardiac failure and alcoholic cirrhosis can both cause edema, but they do not explain the proteinuria. However, considering the clinical findings and medical history, there is a strong suspicion of malignancy, and the most probable diagnosis is membranous glomerulonephritis secondary to colorectal cancer.

Membranous glomerulonephritis is the most common type of glomerulonephritis in adults and is the third leading cause of end-stage renal failure. It typically presents with proteinuria or nephrotic syndrome. A renal biopsy will show a thickened basement membrane with subepithelial electron dense deposits, creating a spike and dome appearance. The condition can be caused by various factors, including infections, malignancy, drugs, autoimmune diseases, and idiopathic reasons.

Management of membranous glomerulonephritis involves the use of ACE inhibitors or ARBs to reduce proteinuria and improve prognosis. Immunosuppression may be necessary for patients with severe or progressive disease, but many patients spontaneously improve. Corticosteroids alone are not effective, and a combination of corticosteroid and another agent such as cyclophosphamide is often used. Anticoagulation may be considered for high-risk patients.

The prognosis for membranous glomerulonephritis follows the rule of thirds: one-third of patients experience spontaneous remission, one-third remain proteinuric, and one-third develop end-stage renal failure. Good prognostic factors include female sex, young age at presentation, and asymptomatic proteinuria of a modest degree at the time of diagnosis.

ACE Inhibitor Induced Cough

When starting ACE inhibitors, cough is a frequent side effect that occurs due to the accumulation of bradykinin. This cough can be bothersome and uncomfortable for patients. To alleviate this symptom, a switch to an angiotensin II receptor blocker is typically recommended. Other substances are not believed to contribute to ACE inhibitor induced cough, making them incorrect. Moxonidine, an anti-hypertensive agent, should not be chosen as a replacement for ACE inhibitors. Proper management of ACE inhibitor induced cough can improve patient comfort and adherence to treatment.

Plasma exchange is recommended for ANCA-associated vasculitis patients with rapidly progressive renal failure or pulmonary hemorrhage. This includes individuals with severe active renal disease, indicated by a serum creatinine level above 354 micromol/L or those requiring dialysis.

Plasma Exchange: Indications and Complications

Plasma exchange, also known as plasmapheresis, is a medical procedure that involves removing plasma from the blood and replacing it with a substitute solution. This procedure is used to treat various medical conditions, including Guillain-Barre syndrome, myasthenia gravis, Goodpasture’s syndrome, ANCA positive vasculitis, TTP/HUS, cryoglobulinemia, and hyperviscosity syndrome. Plasma exchange is particularly useful in cases where the patient’s immune system is attacking their own body, as it removes the antibodies responsible for the attack.

However, like any medical procedure, plasma exchange is not without its risks. Complications can include hypocalcemia, metabolic alkalosis, removal of systemic medications, coagulation factor depletion, and immunoglobulin depletion. Hypocalcemia is caused by the presence of citrate, which is used as an anticoagulant for the extracorporeal system. Metabolic alkalosis can occur due to the loss of acid in the plasma. Removal of systemic medications can be a concern, as plasma exchange can remove medications from the bloodstream. Coagulation factor depletion can lead to bleeding, while immunoglobulin depletion can increase the risk of infection. It is important for healthcare providers to carefully monitor patients undergoing plasma exchange to minimize the risk of complications.

Based on the patient’s history of bladder cancer, it is highly probable that the cause of their bilateral hydronephrosis is malignant infiltration of the ureters. Urothelial cell cancer of the bladder has the ability to spread to the cells lining the ureters and nearby lymph nodes, which can lead to urine blockage and subsequent hydronephrosis. Renal tract calculi and ureteric blood clot are improbable causes of bilateral hydronephrosis, while urinary tract infection is highly unlikely but can complicate acute hydronephrosis.

Understanding Hydronephrosis: Causes, Investigation, and Management

Hydronephrosis is a condition characterized by the swelling of the kidney due to urine buildup. It can be caused by various factors, including pelvic-ureteric obstruction, aberrant renal vessels, calculi, tumors of the renal pelvis, stenosis of the urethra, urethral valve, prostatic enlargement, extensive bladder tumor, and retroperitoneal fibrosis. To diagnose hydronephrosis, ultrasound is the first-line investigation, while IVU can assess the position of the obstruction. Antegrade or retrograde pyelography is also used to allow treatment. In cases of suspected renal colic, a CT scan is the preferred method of detection.

The management of hydronephrosis involves removing the obstruction and draining urine. For acute upper urinary tract obstruction, a nephrostomy tube is used, while a ureteric stent or pyeloplasty is used for chronic upper urinary tract obstruction. It is important to address hydronephrosis promptly to prevent further complications and ensure proper kidney function.

IgA Nephropathy

IgA nephropathy, also known as IgA nephritis or Berger’s disease, is a common kidney disease that is characterized by mesangial widening, inflammation, and mesangial cell proliferation. The disease is often associated with the onset of haematuria and sore throat virtually simultaneously, which is sometimes referred to as synpharyngitic nephritis. The hallmark of IgA nephropathy is the presence of granular IgA deposits on immunofluorescence, which gives the disease its name.

In contrast to post-streptococcal glomerulonephritis, which usually occurs weeks after an upper respiratory illness, IgA nephropathy presents with renal disease almost immediately after the onset of symptoms. While IgA nephropathy may occasionally present as a crescentic glomerulonephritis, this is rare and is more often associated with ANCA positive vasculitides or anti-GBM disease. In such cases, renal function is unlikely to be relatively preserved, as it is in IgA nephropathy.

Other kidney diseases have different characteristics. For example, diffuse membrane thickening is found in membranous glomerulonephritis, podocyte fusion in minimal change disease, and focal segmental glomerulosclerosis is found in the disease of the same name. the characteristics of each kidney disease is important for accurate diagnosis and treatment.

Diagnosis of Nephrotic Syndrome in Young Adults

In young adults without haematuria, the most probable cause of nephrotic syndrome is minimal change nephropathy. Steroid treatment is highly effective in minimal change nephropathy, with a response rate of almost 100%. However, in focal segmental glomerulosclerosis, the response rate is only about 40%. The protein selectivity index, which is the ratio of serum and urine IgG and albumin, is a highly selective indicator of minimal change disease when it is less than 10%. However, this indicator is less reliable in adults. Therefore, in young adults with nephrotic syndrome, minimal change nephropathy is the most likely diagnosis, and steroid treatment is highly effective.

Diagnosis and Treatment of Lower Urinary Tract Obstruction

Lower urinary tract obstruction is a condition that can be caused by various factors, including prostate cancer, urethral stricture, bladder stones, and benign prostatic hypertrophy. In this case, the patient’s symptoms suggest that the most likely cause of the obstruction is benign prostatic hypertrophy. However, it is important to consider referral to a urologist for further evaluation to rule out other potential causes.

To relieve the urinary obstruction, the patient requires catheterisation. This procedure involves inserting a thin tube through the urethra and into the bladder to allow urine to drain out. While catheterisation can provide immediate relief, it is not a long-term solution and further treatment may be necessary depending on the underlying cause of the obstruction.

In summary, lower urinary tract obstruction can be caused by various factors, and a proper diagnosis is essential for effective treatment. In this case, benign prostatic hypertrophy is the most likely cause, and catheterisation is necessary to relieve the obstruction. However, referral to a urologist is recommended for further evaluation and management.

Understanding Nephrogenic Diabetes Insipidus and its Causes

Nephrogenic diabetes insipidus (NDI) is a condition where the kidneys are unable to concentrate urine due to the lack of response to antidiuretic hormone (ADH). This results in the excretion of a large amount of dilute urine. The water deprivation test is used to assess the kidneys’ ability to concentrate urine and response to ADH. In NDI, the result shows abnormally low urine osmolality, which increases only slightly after exogenous ADH. NDI is X-linked recessive, and acquired NDI can occur in disorders that disrupt the medulla or distal nephron and impair concentrating ability.

Lithium-induced nephrotoxicity is a condition where lithium induces acute kidney disease, resulting in severe dehydration, natriuresis, and water diuresis. Nephrogenic DI is also associated with lithium treatment. Analgesic nephropathy can cause renal papillary necrosis and chronic interstitial nephritis, but the water deprivation test is normal in these conditions.

Cranial diabetes insipidus is characterized by polyuria and polydipsia, but the water deprivation test is normal, and serum ADH levels are low. Psychogenic polydipsia is characterized by excessive fluid intake despite no physiological stimuli to drink, resulting in low plasma sodium and urinary osmolality, but the water deprivation test is normal.