medical lipoprotein glomerulopathy

1. The Fatty Kidney
Dr P Pragna
SR, Department of nephrology
NIMS, Hyderabad

2. Case 1
Mrs H M
55 years
Female
r/o Asifnagar, Hyderabad
Date of admission- 30-04-2019

3. History
• Presenting complaints:
Swelling of both legs since 4mths
Shortness of breath on exertion since
2mths
Fever since 1 week
Cough with expectoration since 1wk

4. • History of present illness-
1. Swelling of both limbs since 4 months- initially dependant
increasing since 2 months
2. Shortness of breath since 2 months- on exertion. No orthopnea, no chest pain, no
palpitations
3. Cough with mucoid expectoration since 1 week
4. Fever since 1 week- high grade associated with chills and rigors
No history of decreased urine output, LUTS
Loss of appetite – present
No nausea, vomitings, diarrhea
No headache, altered sensorium, seizures
• Past history-
Hypertension since 4 years on regular treatment

5. • Family history-
• On examination- patient c/c/c
PR- 110/min
RR- 30/min
BP- 140/90 mm Hg
SpO2- 99% on 6 lit/min O2
Febrile
Pallor+, Icterus-, Cyanosis-, Clubbing-, PE+, JVP-
• Systemic Examination-
• Respiratory system: B/L AE present with
reduced air entry in lower areas
occasional rhonchi+
Rest- NAD

6. Investigations
Hemogram result
Hemoglobin 6.5 gm/dl
PCV 19.1
WBC 4100
Platelets 150,000
Peripheral smear Microcytic
Hypochromic RBC, rest
NAD
PT 11.9
INR 1.084
aPTT 24.7
LDH (125-220) 227 U/L
CPK ( ≤ 145) 45 U/L
Viral markers Negative
Biochemical parameters Results
Urea 47 mg/dl
Creatinine 2.76 mg/dl
Na+/K+/Cl (mmol/l) 142/4.7/111
Uric acid 7.4 mg/dl
Calcium 8.1 mg/dl
Phosphorus 4.6 mg/dl
Magnesium 0.77 mmol/l
SGOT/SGPT 8/5 U/L
ALP 82 U/L
T.Bil/D.Bil 0.6/0.15 mg/dl
Total protein 6.1 g/dl
Albumin 2 g/dl
hsCRP 10.27 mg/l

7. Urine Examination Results
CUE: pH 5
Specific gravity 1.010
U.Alb 2+
Sug Nil
M/E trace EC, 0-2 RBC, 6-10
PC
Spot PCR 4.17
Urine Culture Sterile
24Hr Urine Volume- 1100 ml,
Protein- 1034 mg/day
Autoimmune work up Results
ANA 4+ Speckled
dsDNA Negative
ANCA Negative
C3 (90-180) 116 mg/dl
C4 (10-40) 47 mg/dl
FLC ratio K- 112.5, L- 67.5, K/L- 1.67
IgG (700-1600) 1117 mg/dl
IgA (70-400) 187 mg/dl
IgM (40-230) 95 mg/dl

8. Misc Results
S. Iron (35-145) 26 µg/dl
% saturation (14-55) 11%
Ferritin (10-120) 227 ng/ml
TIBC (250-400) 231 µg/dl
B12 (200-680) 227 pg/ml
Folate (3-12) 16.38 ng/ml
Vitamin D3 10 ng/ml
iPTH (10-65) 194 pg/ml
T3 (1.1-3.1) 1.28 nmol/l
T4 (4.5-11.7) 8 µg/dl
TSH (0.2-4) 0.81 µIU/ml
S. Procalcitonin (<0.5) 0.03 ng/ml
B2 Microglobulin (1.2-2.7) 18.36 mg/l
USG- Right Kidney
Left Kidney
HRCT Chest-
Lipid Profile Results
Total Cholesterol
(130-200)
190 mg/dl
HDL ( >40) 28 mg/dl
LDL (≤ 130) 112 mg/dl
VLDL (≤ 30) 50 mg/dl
Triglycerides (≤ 150) 248 mg/dl
TG/HDL (<3.5) 7

9. Renal Biopsy
• Light Microscopy-
29 glomeruli
10/29- segmental sclerosis
Glomerular foam cells+
patchy tubular atrophy
70% IFTA
• Impression- FSGS
• Immunoflorescence-
IgM- neg
IgG- neg
IgA- neg
C3C- neg
C1q- neg
K- neg
L-neg

13. Electron Microscopy

14. Course and Management
Outside
hospital
• 55 year female with previous h/o HTN presented with b/l pedal edema since 4 months
• SOB on exertion, Fever with cough and expectoration since 1 week
• On evaluation was found to have deranged renal functions and anasarca
• HD was given via right IJV for 1 session and referred to NIMS
NIMS
• Creatinine was 2.76, iron deficiency anemia with subnephrotic proteinuria and no active
sediment in urine
• ANA was 4+ with rest of autoimmune work up negative

15. Course and Management
NIMS
• CXR was s/o b/l pleural effusion and on evaluation was had plenty of polymorphs and
was exudative s/o infective etiology
• BAL was done and it also showed infective etiology
• Given empirical antibiotics, oxygen support and other supportive treatment
• She also had CO2 retention for which BiPAP support was given
NIMS
• In view of RPRF picture and subnephrotc proteinuria with ANA 4+, Renal biopsy was
done which was s/o lipoprotein nephropathy with FSGS and 70% IFTA

16. Diagnosis
• Hypertension
• Community acquired pneumonia with b/l pleural effusion
• Type 2 Respiratory failure
• RPRF with subnephrotic proteinuria
• Lipoprotein glomerulopathy
• FSGS with 70% IFTA

17. Case 2
Mrs K V
25 years
Female
r/o Suryapet, TS
Date of admission- 01-10-2020

18. History • Presenting complaints:
Swelling of both legs since 10mths
Seizures 2episodes on 2/8/2020
Shortness of breath on exertion since
7 mths

19. • History of present illness-
1. P2L2
2. 7 months of gestation during second pregnancy started developing swelling of
both lower limbs, followed by abdominal distention and puffiness of face
3. No history of elevated blood pressure/ Diabetes/ infections
4. Delivered a healthy male baby in July 2020 via LSCS with no perinatal complications
5. h/o vomitings and nausea+, Frothuria+
6. No h/o decreased urine output, hematuria, dysuria, LUTS
7. Loss of appetite – present
8. h/o 2 episodes of Generalised tonic clonic movements on 2/8/2020- was found to
have de novo hypertension
• Past history-
No h/o previous comorbidities

20. • Family history-
• On examination- patient c/c/c
PR- 90/min
RR- 22/min
BP- 138/110 mm Hg
SpO2- 99% on room air
aFebrile
Pallor+, Icterus-, Cyanosis-, Clubbing-, PE+
anasarca+, JVP-
• Systemic Examination-
• Respiratory system: B/L AE present with
reduced air entry in lower areas
no added sounds
• Per Abdomen- Free Fluid+
Rest- NAD
3 years 10 months

21. Hemogram result
Hemoglobin 9 gm/dl
PCV 26.3
WBC 9800
Platelets 200,000
Peripheral smear Microcytic
Hypochromic RBC, rest
NAD
PT 11.6
INR 0.98
aPTT 39.4
LDH (125-220) 423 U/L
CPK ( ≤ 145) 53 U/L
Viral markers Negative
Biochemical parameters Results
Urea 64 mg/dl
Creatinine 2.86 mg/dl
Na+/K+/Cl (mmol/l) 144/4.2/112
Uric acid 8.3 mg/dl
Calcium 7.3 mg/dl
Phosphorus 4.8 mg/dl
Magnesium 0.85 mmol/l
SGOT/SGPT 18/8 U/L
ALP 72 U/L
T.Bil/D.Bil 0.2/- mg/dl
Total protein 4.7 g/dl
Albumin 1.6 g/dl

22. Urine Examination Results
CUE: pH 5
Specific gravity 1.010
U.Alb 3+
Sug Nil
M/E EC-nil, 0-2 RBC, 6-10 PC
Spot PCR -
Urine Culture Sterile
24Hr Urine – inadequate
collection
Volume- 350 ml,
Protein- 24.5 mg/day
Autoimmune work up Results
ANA Negative
Anti SM Negative
Anti Ro, La, Scl-70 Negative
dsDNA Negative
APLA Negative
ANCA Negative
C3 (90-180) 71 mg/dl
C4 (10-40) 32 mg/dl

23. Misc Results
T3 (1.1-3.1) 1.11 nmol/l
T4 (4.5-11.7) 8.12 µg/dl
TSH (0.2-4) 0.905 µIU/ml
S. Procalcitonin (<0.5) 0.03 ng/ml
USG- Right Kidney- 13.6 × 6.8 cm Grade 1 RP changes
Left Kidney- 13.1 × 5.7 cm Grade 1 RP changes
Moderate to massive ascites
b/l pleural effuison
Lipid Profile Results
Total Cholesterol
(130-200)
265 mg/dl
HDL ( >40) 38 mg/dl
LDL (≤ 130) 201 mg/dl
VLDL (≤ 30) 26 mg/dl
Triglycerides (≤ 150) 130 mg/dl
TG/HDL (<3.5) 7

24. Renal Biopsy
• Light Microscopy-
6 glomeruli
Expanded mesangial matrix
Capillary loops are occluded by
lipid thrombi- PAS +, Silver- Neg, Oil Red O- +
GBM is thickened with splitting
Interstitium shows minimalfibrosis
No interstitial foam cells
Impression- MPGN with Lipoprotein
glomerulopathy
• Immunoflorescence-
IgM- 2+
IgG- neg
IgA- neg
C3C- 2+
C1q- neg
K- neg
L-neg

27. Immunoflorescence
IgM-
Negative
IgG-
Negative
IgA-
Negative
C3C-
Negative
C1q-
Negative
K-
Negative
L-
Negative

28. Course and Management
Outside
hospital
• 24 year female P2L2 with no h/o previous comorbidities presented with b/l pedal edema since 7 months of
gestation and was found to have proteinuria.
• No h/o hypertension or Diabetes during gestation and uneventful peripartum period
• h/o 2 episodes of GTCS in August 202- on evaluation was found to have PRESS and denovo detected
hypertension
• She underwent renal biopsy at outside hospital in August 2020
• LM 11 glomeruli with 2/11 glomeruli sclerosed, mesangial widening with irregularly thickened GBM and
segmental hyalinosis
• Capillary lumen show foamy lipid thrombi and mesangiolysis+
• IF- Negative
• She was started on steroids- T. Omnacortil 60 mg which she took for 1 month and discontinued
treatment on own
• Came to NIMS for further management

29. Course and Management
NIMS
• On evaluation in NIMS- She had Hb of 9, Creatinine of 2.8, S.albumin 1.6, Urine albumin 3+, S.cholesterol 265
• Diagnosis- Nephritic syndrome with ?nephrotic range proteinuria
• Given IV diuretics with albumin infusion and fluids and other supportive treatment
• Underwent repeat biopsy s/o MPGN with lipid thrombi s/o lipoprotein glomerulopathy
NIMS
• Lipid electrophoresis and EM reports awaited
• Discharged on supportive care

30. Diagnosis
• Hypertension
• Adult onset nephrotic syndrome - Lipoprotein glomerulopathy

31. Discussion
• Lipid metabolism in body
• Cholesterol homeostasis in podocytes
• Altered lipid metabolism in renal dysfunction
• Lipids as a causative agent for development of renal
dysfunction

36. Cholesterol
homeostasis in
Podocyte

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