ADULT NEPHROTIC SYNDROME

S Thamarai Selvi

Physician Assistant, Kauvery Cantonment Hospital, Trichy

Abstract

Nephrotic syndrome is characterised by albuminuria and hypoalbuminaemia and accompanied by oedema, hyperlipidaemia. It can be primary (idiopathic) or secondary. The etiology of nephrotic syndrome in adults is complex. Here we report a 31-year aged female presenting with features of nephrotic syndrome.

Case presentation

A 31-years-aged female was admitted with history of bilateral lower limb swelling, facial puffiness, periorbital edema, dry cough and breathlessness on mild exertion. Patient had a medication history of taking thyroxine hormone 25mcg during pregnancy. Patient had no known past medical/surgical history.

Clinical examination

Conscious, oriented, afebrile

Facial puffiness (+), Periorbital oedema(+), Bilateral pedal oedema (+)

Cardiovascular system – S1 S2(+), Respiratory system Bilateral Air Entry (+), Per Abdomen – Soft

Central nervous system – No focal neurological deficit

Lab findings

Investigation	08/01/2024	13/01/2024	20/01/2024	29/01/2024
Urine routine Albumin	+++	+++	Nil	Nil
Urine protein	672			25.8
Urine Creatinine	66.9			155
Urine PCR	10.04			0.16
Serum Urea	30			21.4
Serum Creatinine	0.416			0.57
Serum Albumin	2.22
Coagulation profile PT	11
INR	0.95
APTT	25.3
Lipid profile Triglycerides	201
HDL Cholesterol	59
LDL Cholesterol	210
Antinuclear Antibody	NEGATIVE
Anti-DS DNA	NEGATIVE
PLA2R Ab IgG	NEGATIVE

Evolution in the hospital

Based on the clinical features and urine sample, the patient was diagnosed with adult nephrotic syndrome.

Hence the patient was managed with fluid restriction 1.5 L/D, salt restricted diet, high protein diet, anti edema measures, Inj. Albumin infusion, diuretics (torsemide 20 mg once a day) and lipid lowering agents. Corticosteroids (Prednisolone 50mg once a day) was given and the patient was relieved of the symptoms.

Discussion

Nephrotic syndrome can be caused by diseases that affect only the kidneys, which are the primary causes of nephrotic syndrome. They are Idiopathic glomerular diseases like minimal change disease, membranous glomerulonephritis, mesangial proliferative glomerulonephritis, focal and segmental glomerulosclerosis (FSGS), and mesangio capillary glomerulonephritis.
Systemic diseases that affect the kidney are called secondary causes of nephrotic syndrome. The secondary causes of nephrotic syndrome are Bacterial endocarditis, malaria, HIV infection, SLE, rheumatoid arthritis Hodgkins lymphoma and amyloidosis. Penicillamine and captopril can also cause the syndrome.
The etiology of nephrotic syndrome in adults is complex and ranges from primary glomerulo nephritis to secondary forms.
Investigations in nephrotic syndrome include a 24 hr urinary protein estimation, and estimation of serum albumin and serum cholesterol concentrations. Renal biopsy may be required to make a histological diagnosis.

Management of nephrotic syndrome involves three steps

Measures to reduce proteinuria
Measures to control complications of nephrotic syndrome. Vitamin D supplementation in patients with biochemical evidence of vitamin D deficiency. Hyperlipidaemia should be controlled with dietary restrictions and lipid-lowering drugs. Anticoagulants are indicated in patients with deep venous thrombosis or arterial thrombosis.
Treatment of underlying cause.

Minimal Change Disease

Patients who respond within the first 4 weeks of steroid treatment are termed steroid responsive. Those who relapse on withdrawal of steroid are termed steroid-dependent. Such relapses are retreated with the same initial regimen, but with more gradual withdrawal of prednisolone. Some patients may require low maintenance doses (510 mg/day) for 36 months. Other options include mycophenolate mofetil, calcineurin inhibitors such as cyclosporin and tacrolimus, and rituximab. Prognosis of patients with minimal change disease is excellent, though it has a characteristic remitting and relapsing course.

Focal and Segmental Glomerulosclerosis

Steroids may be beneficial in only 2030% cases. Cyclophospharnide, tacrolimus and cyclosporine may be of some benefit in steroid-resistant cases.

Membranous Glomerulonephritis

Cyclophospharnide, cyclosporin and chlorambucil in combination with steroids may retard the progression in this subset of patients.

Conclusion

Progress in identifying the cause and pathogenesis of each of these conditions has been slow. Response to steroid therapy is the best prognostic indicator and immune suppressants for steroid resistant or dependent. Dialysis and transplantation have improved the long-term prognosis of patients who reach end-stage renal disease.

S. Thamarai Selvi

Physician Assistant

ADULT NEPHROTIC SYNDROME