Case Report

Department of Pediatrics

LABANIEGO, DONNA H.
Clinical Clerk
I. General Data
Name: PGR Religion: Roman Catholic
Age/Sex: 12 year-old/Female Nationality: Filipino
Address: Igbaras Iloilo Occupation: Student
Birthdate: March 10, 2004 # of Admissions: 1
Birthplace: Igbaras Iloilo
II. Chief Complaint: facial puffiness

III. History of Present Illness:

Two weeks prior to consult, patient experienced fever of about 39 C. At the same time, she
also had joint swelling which was painful but nonerythematous in her elbows and knees. Loss of
appetite, easy fatigability, occasional chest tightness and occasional difficulty of breathing were
also experienced. She also noticed her urine to be darker than the usual with decreased frequency
of urination. However, no painful urination, hypogastric pain, low back pain, sore throat, cough, or
any other symptoms of upper respiratory tract infenction, or skin infection experienced. She was
brought to a private physician where she the following medications were prescribed: Paracetamol
250mg/tab, 1 tab every 4 hours for fever, Mefenamic Acid 500mg/tab, 1 tab for joint pains and
Cefixime 200mg/tab, 1 tab 2x a day for 7 days. And there was resolution of symptoms.
One week prior to consult, she and the folks started to notice her face and periorbital area to
be swollen upon waking up which spontaneously resolved in the afternoon. Occasional chest
tightness recurred. The said symptoms were persistent until 3 days prior to consult, she was again
brought back to a private physician who requested the following laboratory tests: complete blood
count, platelet count, and urinalysis, to which they complied. They were then advised to follow up
after one week to get the results.
Facial puffiness and periorbital swelling persisted every morning, that is why on the day of
consult, folks decided to bring the patient to a hospital in the city for consult. Upon assessment, it
was found out that the patients blood pressure was elevated at 160/80. They were advised for
further workup and possible admission however they opted to transfer to this hospital for financial
reasons, thus this consult.

IV. Past personal history

The patient has never been hospitalized before. She has no chilhood illnesses, no bronchial
asthma and no food and drug allergies.
She is a grade 6 student with average performance. She is friendly and sociable in school and
in the neighborhood. She has never been called by the teachers for disciplinary actions. Her diet
consists of rice, vegetables and fish. She is fond of eating junk foods and sometimes drink
softdrinks.

V. Immunization history
The mother of the patient cannot recall all the vaccines given to the patient but she claimed it
to be complete and on schedule.

VI. Family History

The patient is the youngest of 5 siblings. She has 2 older brothers and 2 older sisters. There is
a family history of bronchial asthma on the mother side (some aunts/uncle/cousins). No family
history of hypertension and diabetes mellitus. Other family members are apparantly well.

VII. Socioeconomic history

The father of the patient is a farmer and the mother is a housewife. Everyday food
consumption is from the crops her father harvests and other expenses from the income of her four
other siblings and the allowance her family receives from a government program (4Ps).

VIII. Environmental history

The patient lives together with her parents in a 1 storey house made of mixed materials. Her
four other siblings are working outside their town and they only come home occasionally. The
house is within a compound with their relatives as their neighbors. Water for drinking and
everyday use is from water pump. The comfort room is located outside the house with pur-flush
type toilet.
Garbage are sometimes burned or dumped in a pit. There are no pets in the house. Pests such
as cockroach and mosquitos are sometimes observed.

IX.
X. Physical examination
a. General Survery
The patient was awake, ambulatory, conversant and responsive, not in cardipulmonary
distress or pain and well groomed.
b. Vital Signs
Blood pressure: 160/100 -> 140/90
Temperature: 36.8C
Cardiac rate: 72 bpm
Respiratory Rate: 26 cpm
Oxygen sat: 98%

c. Anthropometric Data
Weight: 33kgs
Height: 141.5 cm
BMI: 16.48
BSA: 1.13m2

d. Nutritional Status
The patients BMI and height for age are within normal range according to the WHO growth
charts.
e. Skin
Skin is light brown, no pallor or cyanosis, anicteric skin,not cold and clammy, not diaphoretic.

f. Head
Head is normocephalic; hair is black, straight and long, no bald areas; No lesions.

g. Face
Face is symmetrical, there is sight puffiness of the face, no lesions upon inspection and
palpation.

h. Eyes
Eyes are symmetrical in size, no discharges or hemorrhages. No periorbital swelling. Sclerae is
white, conjuctive is pinkish. Pupils measured 4mm and constricting to 2mm, equally round and
reactive to light and accomodation.

i. Ears
Ears are symmetrical. No lesions. Tympanic membranes are intact and not inflamed. Cerumen
present in both ears but not impacted. Good hearing to whispered voice. Ear lobes are form and
readily recoil. Weber midline. Air conduction greater than bone conduction.

j. Nose
Nasal mucosa is pink, septum is at midline, no discharges or lesions in the nostrils, no sinus
tenderness.

k. Mouth and throat, tonsils

Oral mucosa is pink, dental carries are present in upper and lower molars, tonsils are
nonhyperthrophic and nonhyperemic without exudates. No lesions noted.

l. Neck
 No lesions seen, trachea is at midline, neck is supple, thyroid are palpable but not enlarged, no
other masses palpated.

m. Chest and lungs

Thorax is symmetric with good expansion, no tenderness in the chest, lungs are resonant and
breath sounds are clear.

n. Heart and blood vessels

Adynamic precordium, point of maximal impulse located on the midclavicular line between the 4 th
th
to 5 intercostal spaces. Cardiac rate is normal and rhythm is regular with no murmurs hearsd.

o. Abdomen
Abdomen is flat, with normoactive bowel sounds, soft, nontender, no palpable masses, spleen and
kidneys are not palpable, liver span is about 8-10cm in right midclavicular line and all four quadrants
are tympanitic.

p. Inguinal region, Genitalia, Anus and rectum

*Patient refused to be examined in these areas*

q. Extremities
All four extremities are grossly normal without swelling or any deformities. Joints and other areas
are non tender. Pulses are full. Capillary refill time is less than 2 seconds.

r. Spine
The spine is at midline, no lesions seen, no tenderness and masses palpated.

XI. Assessment
Acute Glumerulonephritis

XII. Course in the ER and Wards

Hospital Day 0 (11/16/16)
At the ER, patient was awake, ambulatory, coherent, not in cardiopulmonary distress. She had
anicteric sclerae, pinkish conjunctivae, with periorbital edema, and facial puffiness, PERRLA, moist
buccal mucosa, symmetric chest expansion clear breath sounds, adynamic precordium, normal
cardiac rate regular in rhythm, flat soft abdomen, grossly normal extremities, capillary refill time
<2s and full pulses.
Heplock was inserted and patient was given Furosemide 30mg IVTT. Laboratories requested
were CBC plt, urinalysis, fecalysis, BUN, Crea, ASOT and C3. Patient was then admitted. Additional
laboratories requested: Na, K, Ca. Medication weres: Furosemide 30 mg slow IVTT q6h and Penicillin
G 1,000,000 units q6h ANST (121,212mkday) and Amlodipine 10mg/tab 1 tab now then OD.

Hospital Day 1 (11/17/16)

Urine output was 1.4cc/hr. BSA:1.13. BP: 120/70. Decreased periorbital edema and facial
puffiness, she had clear breath sounds, adynamic precordium, normal cardiac rate and regular
rhythm. Patient was reweighed daily pre-breakfast upon waking up. Medications were continued.
Fluid intake was limited to 800mL in 24 hours: 350cc AM; 350cc PM; 100cc night.

Hospital Day 2 (11/18/16)

Urine output: 1.3cc/hr BSA: 1.09. BP: 110/60. No periorbital edema and decreased facial
puffines.She had clear breath sounds, adynamic precordium, normal cardiac rate and regular
rhythm, soft and nontender abdomen, grossly normal extremities with full pulses. Fluid intake was
limited to 800mL in 24 hours: 350cc AM; 350cc PM; 100cc night.

Hospital Day 3 (11/19/16)

Urine output: 1.09cc/hh. BP: 90/60. Facial puffiness further decreased, clear breath sounds,
normal cardiac rate regular rhythm, soft and nontender abdomen, grossly normal extremities with
full pulses.
Furosemide was decreased to every 8 hours. Amlodipine decreased to 5mg/tab OD PO. Fluid
intake was limited to 850mL in 24 hours: 400cc AM; 400cc PM; 50cc night. Repeat urinalysis was
requested.
 Hospital Day 4 (11/20/16)
Urine output: 2.1cc/hr. BP: 100/60. Facial puffiness resolved. She had clear breath sounds,
normal cardiac rate regular rhythm, soft and nontender abdomen, grossly normal extremities with
full pulses.
Fluid intake was limited to 900mL in 24 hours: 400cc AM; 400cc PM; 100cc night. Medications
were continued.

Hospital Day 5 (11/21/16)

Urine output: 1.3cc/hr, BP: 90-100/60-70. BSA 1.08. No facial puffiness, clear breath sounds,
normal cardiac rate regular rhythm, soft and nontender abdomen, grossly normal extremities with
full pulses. She had a febrile episode of 39.3 oC to which she was given Paracetamol 500mg/tab 1
tab q4h PRN for temp >/= 37.8oC.

Fluid intake was limited to 1,000mL in 24 hours: 500cc AM; 400cc PM; 100cc night.
Medications were continued. Amlodipine was discontinued. IV Furosemide was shifted to PO
40mg/tab 1 tab BID once IV stocks were consumed. Patient is for repeat CBC and platelet.

Hospital Day 6 (11/22/16)

Urine output: 1.3cc/hr, BP of 100/60. No facial puffiness, clear breath sounds, normal cardiac
rate regular rhythm,soft and nontender abdomen, grossly normal extremities with full pulses. No
recurrence of fever.
Furosemide was decreased to OD. Medications were continued. Fluid intake was limited to
1,000mL in 24 hours: 500cc AM; 400cc PM; 100cc night.

Hospital Day 7 (11/23/16)

Urine output: 1cc/hr, stable vital signs, afebrile, no facial puffiness, clear breath sounds,
normal cardiac rate regular rhythm,soft and nontender abdomen, grossly normal extremities with
full pulses.
Patient was given may go home orders and discharged with the following medications: Co-
amoxiclav 500mg/tab 1 tab BID x 4days, Furosemide 40mg/tab 1 tab OD x 2 days, and
Multivitamins + Iron syrup, 10mL OD PO. She was then advised for followup after 1 week with
repeat BUN and creatinine.

XIII. Differential Diagnosis

1. Acute postreptococcal glumerulonephritis
*see case discussion at later part*

2. IgA Nephropathy
IgA nephropathy is an immune-complex mediated glumerulonephritis characterized bu the
presence of diffuse IgA deposiits in the mesangium associated with mesangial hypercellularity. The
two most common presentation are: 1) recurrent episodes of macroscopic hematuria often
accompanied with proteinuria after an upper respiratory tract infection or, 2) persistent asymptomatic
microscopic hematuria. These symptoms do not usually appear in the early stages but usually
manifest when the kidney function is already impaired. Other signs and symptoms include high blood
pressure, swelling of the hands and feet, and flank pains.
In our case, the patient only presented with dark yellow urine but there is microscopic hematuria
as evidenced by the presence of red blood cells in the urinalysis. She also had edema but on the face
and periorbital area, not on the hands and feet. Her blood pressure was also elevated at 160/80.
However in IgA nephropathy, serum complement C3 is normal and ASO titer is not elevated, which are
the opposite of the laboratory results of the patient. This could point out other disease that presents
with elevated ASO titer and decreased C3 level which is postreptococcal glumerulonephritis

3. Lupus Nephritis
Systemic lupus erythematosus or SLE is a systemc disease characterized by fever, weight loss,
dermatitis, arthritis, hematologic abnormailities the involvement of various organs such as the central
nervous system, heart, lungs and kidneys. Lupus nephritis happens when there is already involvement
of the kidneys, and occasionally it is the only presenting clinical manifestation of the disease. It is the
most important cause of morbidity and mortality in patients affected with SLE. Up to 80% of pediatric
patients with SLE present with lupus nephritis and it known to be more active than that in adult
patients. Depending on the severity of the disease, the patient can present with different
 manifesations. Milder forms of lupus nephritis (class I-II and some class III) can present with
hematuria, normal renal function, and proteinuria of <1g/24hrs. In some cases of class III and all clas
IV presen with hematuria, proteinuria, hypertension, reduced renal function, acute renal failure or
nephrotic syndrome. In severe cases (class V), patients commonly present with nephrotic syndrome.
Lupus nephritis can be considered since in this disease, the C3 and C4 levels are usually
decreased, and the patient also had decreased C3 level. She also presented with fever and joint pains
that could be some of the systemic manifestations of SLE. However, the elevated ASO titer of the
patient and the periorbital edema and facial swelling points more to the another diagnosis which is
postreptococcal glumerulonephritis.

XIV. Final Diagnosis

Acute postreptococcal glumerulonephritis

XV.
CASE REPORT

This is a case of a 12 year-old female who came in due to facial puffiness. History of present illness revealed she had fever (39
C) and joint pains and swelling on elbows and knees with associated loss of appetite, easy fatigability, occasional chest tightness
and occasional difficulty of breathing two weeks ago. Her urine became darker han the usual with associated decrease in urination
frequency. Facial puffiness and periorbital swelling were experienced one week prior to consult and just resolved spontaneously in
the afternoon. Upon consult at the city hospital, her blood pressure was elevated at 160/80, thus was advised for further workup and
subsequent admission but opted to transfer in this hospital for financial reasons.

There were no painful urination, hypogastric pain, low back pain, sore throat, cough, or any other symptoms of upper
respiratory tract infenction, or skin infection experienced.

Vital signs were 160/100mmHg -> 140/90mmHg, 36.8C, 72 bpm, 26 cpm and 98% oxygen saturation. Pertinent PE findings
were periorbital edema and facial puffiness.

The patient was managed in the emergency room as acute glumerulonephritis and was subsequently admitted to the wards.

Glomerular diseases come in various forms with different pathogenesis depending on the cause, such as genetic mutations,
infections, autoimmunity, toxin exposure, hypertension, emboli, thrmbus, or diabetes mellitus. Glumerulonephritis is the result of an
immunologic injury to the glomerulus. It is used as a generic term for several diseases but in a strict manner, it is a histopathologic
term for inflammation of the glomerular capillaries. In this case, in infectious cause can be highly considered due to history of fever.
Poststreptococcal glumerulonephritis can be considered as it is most common in children ages 5 to 12 years old. Typically, an acute
nephritic syndrome develops 1-2 wk after a streptococcal pharyngitis infection or 3-6wks after a streptococcal pyoderma. Hoewever,
if there is no history of previous pharyngitis or skin infection, like in the patients case, postreptococcal GN can not be readliy ruled
out, as symptoms of previous infection may have been mild or have been already resolved before the patient seek consult or
receives specific treatment.

Acute nephritic syndrome is characterized by sudden onset of gross hematuria, edema, hypertension and renal insufficiency.
And one of its classic example is the acute poststreptoccal glomerulonephritis (APSGN). The severity of symptoms such as
hypertension, edema, and oliguria which were manifested by the patient depends on the extent of renal involvement. The edema
(facial puffiness and periorbital area swelling) presented by the patient can occur abruptly, first involving the periorbital area then
may become generalized. It typically results from salt and water retention due to renal damage and it is the most frequent and
sometimes in other patients can be the only clinical finding during physical examination. Hypertension is reported in up to 90% of the
cases and its effects deoends on its severity and increase from the patients baseline blood pressure. In patients presenting with
severe headaches, blurring of vision, seizures, or changes in mental status, hypertensive encephalopathy must be considered.
Hematuria can vary from asymptomatic microscopic hematuria to gross hematuria depending on the glomerular damage. It is also
common for the patients to present nonspecific signs and symptoms such as abdominal pain, malaise, flank pain, and lethargy.

Post streptococcal glumerulonephritis has three stages following the initial streptoccal infection: latent, acute and recovery
phases. The latent phase is the inactive period wherein the patient is asymptomatic, occuring between the initial infection and the
onset of secondary symptoms. This corresponds to 1-2 wk period after a streptococcal pharyngitis infection and 3-6wks after a
streptococcal pyoderma. The acute phase which lasts for about a week is the nephritic phase wherein patients experience secondary
symptoms such as hypertension, hemauria, oliguria and edema. The recovery stage is the final stage where the mentioned symtoms
start to subside. It could take several weeks or months depending on the severity of the disease.

Aside from thorough history and physical examination, some diagnostic tests are requested to strongly support the diagnosis of
APSGN. Urinalysis will reveal red blood cells with associated red blood cell casts, proteinuria, and polymorphonuclear cells most of
the time. In more than 90% of the patient in acute phase, C3 level is significantly reduced and it normalizes 6-8 week after the onset
of the disease. CH50 level is also usually depressed however, C4 level is normal or just slighty depressed. The patient might have
positve throat culture if there is still throat infection or the antibody titer to streptococcal antigen will be elevated confirming a recent
streptococcal infection. After a pharyngeal infection, the antistreptolysin O is usually elevated, but rarely in streptococcal pyoderma.
To document a streptococcal skin infection, the antideoxyribonuclease B level is measured if availabe. The streptozyme screen is also
a valuable diagnostic tool as it measures multiple antibodies to various streptococcal antigens. Imaging studies such as magnetic
resonance imaging og the brain is used for those who present with severe neurologic involvement. For those who present signs of
heart failure or respiratory distress/failure or other heart and lungs abnormalities such as heart gallop, rales or decreased breath
sounds, chest radiograph is requested.

The following laboratory tests were requested for the patient: CBC, platelet, urinalysis, fecalysis, BUN, creatinine, serum sodium,
serum potassium, serum calcium, ASO titer, and C3. The patient did not present with gross hematuria but her urinalysis showed
RBCs of 10-15 and repeat urinalysis revealed 15-25 RBCs. And as claimed, there was no recent history of sore throat or any
symptoms of upper respiratory tract infection but her ASO titer was elevated at >200 IU/ml. Her C3 was also decreased at <138
mg/dl.

Possible mechanisms of immunologic injury in acute postreptococcal glumerulonephritis include molecular mimicry in which the
streptococcal antigen elicit circuating antibodies that react with the normal glomerular antigens, in situ complex formation of
antistreptoccocal antibodies with glomerular deposited antigen, and activation of complement by the directly deposited streptococcal
antigen. Over the years, many candidate antigens have been proposed to play a role in the disease. At present, the most promising
candidates are the streptococcal pyrogenic exotoxin B (SPEB), a cationic cysteine proteinase that is generated by the proteolysis of a
zymogen receptor and the nephritis-associated plasmin receptor (NAPlr). These two antigents are known to have biochemical affinity
for plasmin and bind as complexes, and subsequently activate the alternative complement pathway. In renal biopsy of patient with
postrepotococcal glumerulonephritis, there are hypercellularity of mesangial and endothelial cells, glomerular infiltrates of
polymorphonuclear leukocytes, granular subendothelial deposits of IgG, IgM, C 3, C4 and C5-9, and subepithelial deposits that appear as
humps. Inside these subepithelial humps, the nephritogenic SPEB can be demonstrated.

The management of APSGN involves treating the acute effect of hypertension and renal insufficiency. A 10-day course of
Penicillin G is the recomended antibiotic but it does not alter the natural history of the disease, it only limits the spread of the
nephritogenic organisms. The standard managment to treat hypertension are sodium restriction, diuresis with the use of furosemide,
and calcium channel antagonists, ACE-inhibitors, or vasodilators.

Among patients with APSGN, >95% goes into complete recovery. It is extremely rare for the disease to recur. Appropriate
management of renal insuffiency, cardiac failure and hypertension avoids patient mortality in the acute stage of the disease.