Nephrotic syndrome

CASE REPORT
NEPHROTIC SYNDROME
DEPARTEMENT OF PEDIATRICS
H. ADAM MALIK GENERAL HOSPITAL
FACULTY OF MEDICINE
SUMATERA UTARA UNIVERSITY
MEDAN
2011
1

PREFACE
First of all, we praise and gratitude to God Almighty that due to God graceand
blessing the assignment entitled “Nephrotic Syndrome“ was able to be completed in time.
This assignment was written to fulfillduties of the Senior Clinical Assistances, Department of
peadiatric, Haji Adam malik General Hospital/ Univercity of Sumatera Utara, and to
trainourselves in treating the patients correctly with this disorder.
Words of graduate to Prof. dr. S, Sp. A (K) for his advices and guidance contributing
to the completionof this assignment.
We are aware of the imperfection of this assignment, thus, we accept encouraging
critics and constructional advices fo the common good.
2

TABLE OF CONTENTS
Preface .......................................................................................... i
Table of Contents ............................................................................ ii
Chapter 1: Introduction ........................................................................ 1
1.1. Background .............................................................................. 1
1.2. Objective .............................................................................. 2
Chapter 2: Literature Review ...................................................... 3
2.1. Definition .............................................................................. 3
2.2. Epidemiology .............................................................................. 3
2.3. Classification and Clinical Findings .......................................... 4
2.4. Pathophysiology .................................................................. 7
2.5. Diagnosis and Work Up ...................................................... 12
2.6. Management .............................................................................. 17
2.7. Complication .............................................................................. 29
2.8. Follow Up and Prognosis ...................................................... 30
Chapter 3: Case Report .................................................................. 32
Chapter 4: Discussion and Summary .......................................... 43
4.1. Discussion .............................................................................. 43
4.2. Summary .............................................................................. 46
References
3

CHAPTER I
INTRODUCTION
1.1. Background
1.2. Objective
The aim of this study is to explore more about the theoritical aspects on Nephrotic
Syndrome, and to integrate the theory and application of Nephrotic Syndrome case in
daily life
4

CHAPTER II
LITERATURE REVIEW
2.1. Nephrotic Syndrome
2.1.1. Definition
2.1.2. Epidemiology
2.1.3. Classification and Clinical Findings
2.1.4. Patophysiology
2.1.5. Diagnosis and Work Up
2.1.6. Management
2.1.7. Complication
2.1.8. Follow Up and Prognosis
CHAPTER 3
5

CASE REPORT
Name : C F A
Age : 9 years 6 months
Sex : Female
Date of Admission : July, 6th 2011
Chief Complaint : Swollen of entire body
History :
This problem has already been occuring to this patient as long as 3 weeks before
admission. The swelling started from head, stomach, hands, and legs. History of swollen
found in the year 2009 and already in medication with furosemid and prednisone, but patient
fail to control to hospital.
Nausea (+), vomit (+) found in patient past 3 weeks. Vomit contains food whatever
patiens consume. Volume ± 50cc per times vomit. Frequency ± 3times per day.
History of fever (+) found in patient past 3 weeks. the temperature is not stable. The
temperature in never been to high.
Cough (+) found past 1 week, flame (-), flu (-).
Stomach pain (+), hip pain (+), history of joins pain (-), history of pain in swalloing (-
ss)
BAK (+), volume sedikit, warna kuning pekat, frekuensi ± 5 kali/hari dialami sejak 1
minggu ini. Defecation was within the normal limit, and there was no diarrhea.
History of previous illness : This patient is a regular-controlled patient from
Nephrology Division of Pediatric Ward Haji Adam Malik General Hospital with diagnosed as
nephrotic syndrome since 2009, dan telah didiagnosis dengan sindroma nefrotik. Os tidak
teratur kontrol ke rumah sakit.
History of previous medications : Prednisone and Furosemide since 2009. Os tidak
teratur minum obat.
Physical Examination
Generalized status
Body weight: 31 kg, Body length: 124 cm,
6

Upper arm circumference: 14 cm, Head circumference: 57 cm
BW/BL: 25/32 x 100% = 78,13% (moderate malnutrition)
BW/age: 25/51 x 100% = 49,02% (severe malnutrition)
BL/age : 144/162 x 100% = 88,9% (normoheight)
Presens status
Consciousness: Alert, Body temperature: 37,0o
C.
Anemic (-). Icteric (-). Cyanosis (-). Edema (+). Dyspnea (+).
Localized status
Head :
Hair was black, hair fall easily was not found. Inferior palpebra conjunctiva pale (-). Icteric
sclera (-). Light reflex (+/+). Isochoric pupil.
Neck :
Lymph node enlargement (-)
Thorax:
Symmetrical fusiformis. Chest retraction (-). HR: 120 bpm, reguler, murmur (-). RR: 28x/i,
reguler. Breath sound: vesicular. Additional sound (-).
Abdomen:
Soepel. Peristaltic (+) normal. Liver/Spleen/Renal were not palpable.
Shifting dullness (+). Undulation (+).
waist circumference while sitting: 80 cm
waist circumference while lying down: 84 cm
Extremities:
Pulse 120x/i, regular, adequate pressure and volume, warm, CRT < 3”. BP: 120/80 mmHg
(normal: 111-128 / 63-82).
Urogenital:
Female, with swollen at labia major.
7

Laboratory Findings:
Parameters Value Normal Value
Complete Blood Count
Hemoglobin 11,4 gr% 12,0 – 14,4 gr%
Hematocrite 33,6 % 38 – 44%
Erithrocyte 4,21 x 106
/mm3
4,2 – 4,87 x 106
/mm3
Leucocyte 10100 /mm3
4500 – 11000 /mm3
Platelet 556.000 /mm3
150000 – 450000 /mm3
MCV 79,90 fl 85 – 95 fl
MCH 27,2 pg 28 – 32 pg
MCHC 34,10 gr% 33 – 35 gr%
RDW 13,5 % 11,6 – 14,8 %
Diftel 0 / 0 / 78 / 9 / 13
LiverFunction Test
Total Bilirubine 0,20 mg/dL 12,0 – 14,4 gr%
Direct Bilirubine 0,04 mg/dL 38 – 44%
Alcali Fosfatase 63 U/L 12,0 – 14,4 gr%
AST/SGOT 19 U/L 38 – 44%
ALT/SGPT 12 U/L 4,2 – 4,87 x 106 /mm3
Albumine 1,2 g/dL 4500 – 11000 /mm3
Renal Function Test
Ureum 46,10 mg/dL 12,0 – 14,4 gr%
Kreatinin 0,83 mg/dL 38 – 44%
Uric Acid 7,1 mg/dL 4,2 – 4,87 x 106
/mm3
Platelet 556.000 /mm3
150000 – 450000 /mm3
Differential Diagnosis:
-
Working Diagnosis:
Relaps nephrotic syndrome
Management:
- Bedrest, threeway and urinary catheter inserted
- IVFD D5% NaCl 0,45% 20 gtt/i micro
- Regular food less salt 1720 kkal with 62 gr protein
- inj. ceftiaxone 1 gr/ 12hours
8

- Prednisone tab 4-4-4
Diagnostic Planning:
- Fluid Balance per 6 hours
- Morning urine dipstick
FOLLOW UP
July 7th
, 2011
S Swelling of entire body (+)
O Sens: CM, Temp: 37,1o
C. Anemic (-). Icteric (-). Edema (+). Cyanosis (-).
Body weight: 31 kg, Body length: 124 cm. BSA: 1,02
Head Eye : Light reflexes (+/+), isochoric pupil, pale conjunctiva palpebra
inferior (-/-).
Ear/ Nose/Mouth: within normal limit
Neck Jugular vein pressure R-2 cmH2O. Lymph node enlargement (-).
Thorax Simetris fusiformis. Retraction (-). HR: 120 bpm, reguler.
Murmur (-). RR: 24 x/i, regular. Breath sound: vesicular. Additional
sound: (-).
Abdomen Soepel. Liver/Spleen/Renal: undeterminate. Ascites (+)
WSSit: 84 cm. WSSup: 82 cm
Extremities Pulse 120 x/i, regular, adequate p/v, warm acral, CRT < 3”. BP: 120/80
mmHg. Pitting edema (+) pretibia.
Genital Female, with swollen at major labia
A Relaps Nephrotic Syndrome
P Management:
- IVFD D5% NaCl 0,45% 20 gtt/i micro
July 8th
, 2011
S Face and legs swollen (+)
O Sens: CM, Temp: 37o
Body weight: 30 kg, Body length: 124 cm. BSA: 1,004
9

inferior (-/-).
Ear/ Nose/Mouth: within normal limit.
sound: (-).
Abdomen Soepel. Liver/Spleen/Renal: undeterminate. Ascites (+)
Genital Female, with swelling of major labia
Dipstick urine:
Leu / Nit / Uro / Protein / pH / Blood / SG / Ket / Bil / Glu
± / - / 0,2 / +3 / 6 / ± / 1,01/ - / + / -
A Relaps nephrotic syndrome
P Management:
July 9th
, 2011
S Swelling of entire body (+), diarrhea (+)
Body weight: 31 kg, Body length: 124 cm. GFR: 84,1. BSA= 1,02 m2
.
inferior (-/-).
sound: (-).
Abdomen Soepel. Liver/Spleen/Renal: undeterminate. Ascites (+).
10

Genital Female, wirh swollen of major labia
Dipstick urine cannot be done because urine mix with diarrhea
A Relaps nephritic syndrome
P Management:
- Cotrimoxazole 2 x 240 mg
- Furosemide 2 x 30 mg
July 10th
, 2011
S Swollen of entire body (+), diarrhea (+)
.
inferior (-/-).
sound: (-).
Abdomen Soepel. Liver/Spleen/Renal: undeterminate. Ascites (+).
Genital Female, with swelling of major labia
Dipstick urine
- / - / - / +3 / 5 / ± / 1,015/ - / + / -
P Management:
11

July 11th
, 2011
S Swollen of entire body (+), diarrhea (-)
.
inferior (-/-).
sound: (-).
Abdomen Soepel. Liver/Spleen/Renal: were not palpable.
Genital Female, swollen of major labia (-)
P Management:
July 12th
, 2011
S Swollen of entire body (+)↓
.
inferior (-/-).
12

sound: (-).
Genital Female, swollen of major labia (-)
- / - / - / +2 / 5,6 / ± / 1,015/ - / - / -
P Management:
July 13th
, 2011
S Swollen of entire body (+) ↓
C. Anemic (-). Icteric (-). Edema (-). Cyanosis (-).
Body weight: 26 kg, Body length: 124 cm. GFR: . BSA= 0,94 m2
.
inferior (-/-).
sound: (-).
Ascites (+) . Shifting dullness (+). Undulation (+).
mmHg. Pitting edema (-) pretibia.
Genital Female, swollen at major labia (-)
Dipstick urine
13

- / - / 0,2 / +2 / 5,6 / ± / 1,025/ - / - / -
P Management:
CHAPTER 4
DISCUSSION AND SUMMARY
4.1. Discussion
A 14-year-old female was admitted to RSUP HAM diagnosed with severe malnutrition
marasmic-khwarsiorkor type. This diagnosis was made based on clinical findings found in
the patient such old man face, thinning of subcutaneous tissue, crazy pavement dermatosis,
and edema in the lower extremities. The antropometry measurement of this patient reveals
14

that body weight according to age was below 60% and thus this patient could be diagnosed
as severe malnutrition.
The treatment conducted to this patient had already been in line with the guideline of
malnutrition management from Department of Health Republic of Indonesia. This patient
was firstly treated in the stabilization phase in which hypoglycemia, hypothermia and
dehydration were assesed and treated subsequently. Analysis of serum electrolyte was
conducted as well to identify whether electrolyte imbalance had been present or not. Since
the serum Na was 131 mEq/l and the patient did not show any symptomps of hyponatremia,
then this patient was given maintenance fluid with Dextrose 5% NaCl 0,45% solution.The
requirement of the fluid was adjusted to the stabilization phase requirements.
Prophylaxis of infection was given to this patient, applying the use of Cotrimoxazole
(Trimetophrim 5 mg with Sulfamethoxazole 20 mg) and thus the patient was given 2 x 480
mg. Citrimoxazole was given for as long as 5 days, since the patient came without any
evidence of infection first.
Micronutrien deficiency was corrected with admission of folic acid 5 mg along with vitamin
A 200.000IU in the first day. The patient was also receiving multivitamin without iron in the
stabilization phase. Iron supplementation shouldnot be given to a severly malnourished child
because iron deposition will give a chance to bacteria to grow maximally in a child with low
immunity state. Thus, iron supplementation could be given only after the patient completed
the stabilization and transitional phase, and was assumed to have a much better
immunological state.
The patient was then immediately given initial refeeding using WHO Formula 75. Since she
presented with edema in the lower extremities, the fluid requirements needed in the
stabilization phase was 100/day. As known that F75 contains 75 kcal each 100ml, then the
requirements was:
Fluid requirements: 100 ml x 25 kg = 2500 ml
Feeding formula F75 (75 kcal / 100ml)
F 75 = 75 x 2500/ 100 per 24 hours
F 75 = 1875 ml / 24 hours
For the stabilization phase, F 75 should be given every 2 hours, then the patient needed:
F 75 = 1875 / 12 times
F 75 = 157 cc (rounded to be 160 cc / 2hours)
15

On the 5th day, the edema was no longer present, and thus the daily fluid requirement was
then adjusted to 130 ml/kg body weight/day. The estimated calory needed then was
recounted to be as the following:
Fluid requirements: 130 ml x 25 kg = 3250 ml
Feeding formula F75 (75 kcal / 100ml)
F 75 = 75 x 3250/ 100 per 24 hours
F 75 = 2437,5 ml / 24 hours
For the stabilization phase, F 75 should be given every 2 hours, then the patient needed:
F 75 = 2437,5 / 12 times
F 75 = 203,12 cc (rounded to be 210 cc / 2hours)
This formula was given for as long as 7 days during the stabilization phase, then folowed by
Formula 100 WHO which contains more calories then the previous formula.
Daily evaluation of treatment was conducted to this patient assesing the weight gain
achieved each day. Since, the weight gain was below the expected value (less than 5 gr/kg
body weight/ day), she was then classified as poor response. According to the guideline
published, patient with poor response should be evaluated for any comorbid or any infection
that could probably present.
A more detail anamnesis was conducted to this patient in order to identify the cause of this
poor response. According to her mother, the patient ate the whole diet prepared for her
during the treatment. Neither vomitting nor diarrhea occured to this patient. Since the patient
complained about pain while urinating on admission, then a suspicion of urinary tract
infection was then raised. Urinalysis by daily dipstick was then conducted, then the result
revealed that leukosituria and high levels of nitrites were present, thus another investigation
was conducted. Urine culture was done and the result revealed the growth of Pseudomonas
aeruginosa infection. Urinary tract infection was then raised to be comorbid diagnosis.
For the suitable treatment, sensitivity and resistancy test of antibiotics were done and it’s
been proved that the only sensitive antibiotic was Meropenem. Antibiotic switch was then
done to injection of meropenem 250 mg every 8 hours.
Another important problem in this case is the fact that the patient complained about
menstrual irregularity. Since she had not been getting any menstruation for the last 3 months,
then a suspicion of amenorrhea was raised. This condition is assumed secondary to the
severe malnutrition problem happened earlier.
16

Malnutrition and stress causes hypothalamic hypogonadism. The hypothalamic-pituitary-
gonadal axis shuts down as the body struggles to survive, directing finite energy resources to
support more vital functions. Both males and females experience decreased libido and
interruption of pubertal development,depending on the timing of the illness.
The hormones that cause pubertal development and reproduction emanate from the
hypothalamus, the pituitary gland, the gonads, and the adrenal glands. Hypothalamic
gonadotropin-releasing hormone (GnRH) is a 10-amino-acid peptide (coded by a gene on
chromosome 8) secreted from the median eminence into the hypophyseal portal system in a
pulsatile manner to reach the pituitary gland. The GnRH pulse generator is affected by
biogenic amine neurotransmitters, peptidergic neuromodulators, neuroexcitatory amino
acids, and neural pathways. Aminobutyric acid exerts a suppressive effect on GnRH
secretion and can be an important factor in the relative quiescence of gonadotropin secretion
that characterizes the "juvenile pause" that occurs after infancy and ends before the
endocrine activity of puberty resumes. Sex steroids, mainly testosterone, estrogen, and
progesterone, also inhibit GnRH pulse frequency in a negative feedback inhibition, whereas
estrogen has the additional ability to exert positive feedback on gonadotropin secretion at
midpuberty.
Gonadotropin-releasing hormone acts on the gonadotropes of the pituitary gland to increase
intracellular calcium concentration and cause phosphorylation of protein kinase, which
stimulates secretion of luteinizing hormone (LH) and follicle-stimulating hormone (FSH), in
a pulsatile manner owing to the pulsatile nature of GnRH secretion. Gonadotropes exposed
to continuous rather than episodic LH and FSH decrease the number of GnRH receptors,
decrease the action of the occupied receptors, and decrease gonadotropin secretion (down-
regulation).
In girls, FSH binds to cell-surface receptors on ovarian follicular cells to stimulate secretion
of estrogen. Luteinizing hormone becomes important later in pubertal development in
completing the menstrual cycle of girls when it affects the theca cell after the onset of
ovulation. Because there is damage on GnRH secretion in malnutrition, this phase (secretion
of estrogen that stimulates by FSH and GnRH) is not complete yet and cause amenorrea in
this case.
17

4.2. Summary
This paper reports a case of a 14-year-old female diagnosed with severe malnutrition
marasmic-khwarsiorkor type. A comprehensive treatment including ten principal steps to
manage severely malnourished chid has been conducted to this patient. She has been
stabilized during the first week of treatment, and now she has been in the transitional phase.
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