1

CHAPTER 1
INTRODUCTION
1.1. Background
Systemic lupus erythematosus (SLE) is a chronic, often life-long,
autoimmune disease. SLE may affect various parts of the body, but it most often
manifests in the skin, joints, blood, and kidneys. Women are more commonly
affected than men and the disease is primarily diagnosed in patients aged 15 to 45
years. The name describes the disease where Systemic is used because the disease
can affect organs and tissue throughout the body. Lupus is Latin for wolf. It refers
to the rash that extends across the bridge of the nose and upper cheekbones and
was thought to resemble a wolf bite. Erythematosus is from the Greek word for
red and refers to the color of the rash.
1
There is varying epidemiologic information regarding systemic lupus
erythematosus among countries in Asia. Prevalence rates usually fall within 30-
50/100,000 population.
2
The clinical classification for systemic lupus
erythmatosus is based on the American College of Rheumatology 1997 revised
criteria for classification of systemic lupus erythematosus. The classification is
based on 11 criteria. For the purpose of identifying patients in clinical studies, a
person is defined as having SLE if any 4 or more of the 11 criteria are present,
serially or simultaneously, during any interval of observation.
3
Treatment of SLE is tailored to the individual, being based on specific
disease manifestations and tolerability. For all patients, sunscreen and avoidance
of prolonged direct sun exposure and other ultraviolet light may help control
disease. Hydroxychloroquine (5-7 mg/kg/day) is recommended for all individuals
with SLE if tolerated. Corticosteroids are a mainstay for treatment of significant
manifestations of SLE.
4
The prognosis has improved with earlier recognition and improved manag
ement. The five-year survival rate is over 90%.
5
2


1.2. Objective
The aim of this study is to explore more about the theoretical aspects on
systemic lupus erythematosus, and to integrate the theory and application of
systemic lupus erythematosus cases in daily life.


























3

CHAPTER 2
LITERATURE REVIEW

2.1. Definition
Systemic lupus erythematosus (SLE) is a clinically heterogeneous disease
which is autoimmune in origin, and characterized by the presence of
autoantibodies directed against nuclear antigens. It is, by definition, a multi-
system disease, and patients can present in vastly different ways.
6

2.2 Epidemiology
There is varying epidemiologic information regarding systemic lupus
erythematosus among countries in Asia. Prevalence rates usually fall within 30-
50/100,000 population. Incidence rates, as reported from three countries, varied
from 0.9/100,000 to 3.1% per annum.
2
More than 90% of cases of SLE occur in
women, frequently starting at childbearing age.
7,8
The use of exogenous hormones
has been associated with lupus onset and flares, suggesting a role for hormonal
factors in the pathogenesis of the disease.
9
The female-to-male ratio peaks at 11:1
during the childbearing years.
10
Common manifestations include mucocutaneous
lesions (seen in 52-98% of patients) and arthritis/musculoskeletal complaints (36-
95%). Antinuclear antibodies were generally positive in 89-100% of patients,
except for two studies. Renal involvement ranged from 18% to 100% with most
articles reporting this in >50% of their patients. Discoid lesions, serositis, and
neurologic involvement were the least frequently seen symptoms.
2


2.3. Risk Factors
2.3.1 Genetics
Genetic susceptibility to lupus is inherited as a complex trait and studies
have suggested that several genes may be important. In particular an interval on
the long arm of chromosome 1, 1q2324, is linked with SLE in multiple
4

populations. Clinically it is widely accepted that active SLE is characterised by
elevated erythrocyte sedimentation rates but normal C-reactive protein (CRP)
levels. Both CRP and complement as well as serum amyloid P protein are
important in clearing apoptotic cell debris and the genes for CRP have been
mapped to chromosome 1, 1q2324, the so-called pentraxin locus. Russell and
colleagues examined the inheritance of polymorphisms at the pentraxin locus in a
family-based association study of SLE and found strong linkage disequilibrium
within each of the CRP and serum amyloid P genes.
11
They demonstrated that an
allele of CRP 4 was associated with SLE. Furthermore there were two haplotypes
that were significantly associated with reduced basal CRP expression: CRP 2 and
CRP 4 and an allele of CRP 4 was associated with ANA production. Thus the
authors proposed a genetic explanation of the link between low CRP levels,
antinuclear autoantibody production and the contribution of these to the
development of human SLE. Another large study of individuals and multi-case
families with SLE suggested that a single nucleotide polymorphism (SNP) within
the programmed cell death 1 gene (PDCD1) is associated with the development of
lupus in both European and Mexican populations. The authors showed that the
associated allele of this SNP alters a binding site for a transcription factor located
in an intronic enhancer, suggesting a mechanism through which it can contribute
to the development of SLE.
12


2.3.1 Gender
About 90% of lupus patients are women, most diagnosed when they are in their
childbearing years. Hormones may be an explanation. After menopause, women
are only 2.5 times as likely as men to contract SLE. Flares also become somewhat
less common after menopause in women who have chronic SLE.
1

2.3.2 Age
Most people develop SLE between the ages of 15 - 44. About 15% of patients
experience the onset of symptoms before age 18.
1
5


2.3.4 Environmental Triggers
In genetically susceptible people, there are various external factors that can trigger
symptoms (flares). Possible SLE triggers include:
Viruses. Epstein Barr virus (EBV) has also been identified as a possible factor in
the development of lupus. EBV may reside in and interact with B cells. Gross et
al
13
found a high frequency of EBV infected B cells in lupus patients compared
to controls and these infected cells are predominantly memory B cells. There was
no relationship with immunosuppressive therapy and furthermore patients with
active lupus flares had more infected cells than patients with quiescent disease.
Although other studies have suggested a causative role for EBV in SLE, these
authors are more cautious and despite their findings of increased frequencies of
infected cells, increased viral loads, and viral gene expression, they have not
interpreted this as directly implicating EBV in the development of SLE and argue
that it is also possible that the immune dysregulation of SLE may result in
aberrant EBV expression. In contrast, studies in a mouse model found that direct
introduction of the whole EBV nuclear antigen 1 protein can elicit IgG antibodies
to Sm and to double-stranded DNA (dsDNA) thus supporting a putative role for
EBV in the development of lupus. The paradox remains that although 90% of the
adult population are infected by EBV, the prevalence of SLE remains low
emphasising the multi-factorial nature of the pathogenesis of SLE.

Sunlight. Ultraviolet (UV) rays found in sunlight are important SLE triggers. UV
light is categorized as UVB or UVA depending on the length of the wave. Shorter
UVB wavelengths cause the most harm.
1

Smoking. Smoking may be a risk factor for triggering SLE and can increase the
risk for skin and kidney problems in women who have the disease.
1

6

Chemicals. While no chemical has been definitively linked to SLE, occupational
exposure to crystalline silica has been studied as a possible trigger. (Silicone
breast implants have been investigated as a possible trigger of autoimmune
diseases, including SLE. The weight of evidence to date, however, finds no
support for this concern.) Some prescription medications are associated with a
temporary lupus syndrome (drug-induced lupus), which resolves after these drugs
are stopped.
1

Hormone Replacement Therapy. Premature menopause, and its accompanying
symptoms (such as hot flashes), is common in women with SLE. Hormone
replacement therapy (HRT), which is used to relieve these symptoms, increases
the risk for blood clots and heart problems as well as breast cancer. It is not clear
whether HRT triggers SLE flares. Women should discuss with their doctors
whether HRT is an appropriate and safe choice. Guidelines recommend that
women who take HRT use the lowest possible dose for the shortest possible time.
Women with SLE who have active disease, antiphospholipid antibodies, or a
history of blood clots or heart disease should not use HRT.
1

Oral Contraceptives. Female patients with lupus used to be cautioned against
taking oral contraceptives (OCs) due to the possibility that estrogen could trigger
lupus flare-ups. However, recent evidence indicates that OCs are safe, at least for
women with inactive or stable lupus. Women who have been newly diagnosed
with lupus should avoid OCs. Lupus can cause complications in its early stages.
For this reason, women should wait until the disease reaches a stable state before
taking OCs. In addition, women who have a history of, or who are at high risk for,
blood clots (particularly women with antiphospholipid syndrome) should not use
OCs. The estrogen in OCs increases the risk for blood clots.
1





7


2.4. Classification
1997 Update of the 1982 American College of Rheumatology revised criteria
for classification of systemic lupus erythematosus
1. Malar Rash Fixed erythema, flat or raised, over the malar eminences,
tending to spare the nasolabial folds
2. Discoid rash Erythematous raised patches with adherent keratotic scaling
and follicular plugging; atrophic scarring may occur in older
lesions
3. Photosensitivity Skin rash as a result of unusual reaction to sunlight, by
patient history or physician observation
4. Oral ulcers Oral or nasopharyngeal ulceration, usually painless, observed
by physician
5.Nonerosive
arthritis
Involving 2 or more peripheral joints, characterized by
tenderness, swelling, or effusion
6.Pleuritis or
pericarditis
1. Pleuritis--convincing history of pleuritic pain or
rubbing heard by a physician or evidence of pleural
effusion
OR
2. Pericarditis--documented by electrocardigram or rub
or evidence of pericardial effusion
7. Renal disorder
1. Persistent proteinuria > 0.5 grams per day or > than
3+ if quantitation not performed
OR
2. Cellular casts--may be red cell, hemoglobin, granular,
8

tubular, or mixed
8.Neurologic
disorder
1. Seizures--in the absence of offending drugs or known
metabolic derangements; e.g., uremia, ketoacidosis, or
electrolyte imbalance
OR
2. Psychosis--in the absence of offending drugs or
known metabolic derangements, e.g., uremia,
ketoacidosis, or electrolyte imbalance
9.Hematologic
disorder
1. Hemolytic anemia--with reticulocytosis
OR
2. Leukopenia--< 4,000/mm
3
on 2 occasions
OR
3. Lyphopenia--< 1,500/ mm
3
on 2 occasions
OR
4. Thrombocytopenia--<100,000/ mm
3
in the absence of
offending drugs
10. Immunologic
disorder
1. Anti-DNA: antibody to native DNA in abnormal titer
OR
2. Anti-Sm: presence of antibody to Sm nuclear antigen
OR
3. Positive finding of antiphospholipid antibodies on:
1. an abnormal serum level of IgG or IgM
9

anticardiolipin antibodies,
2. a positive test result for lupus anticoagulant
using a standard method, or
3. a false-positive test result for at least 6 months
confirmed by Treponema pallidum
immobilization or fluorescent treponemal
antibody absorption test
11.Positive
antinuclear
antibody
An abnormal titer of antinuclear antibody by
immunofluorescence or an equivalent assay at any point in
time and in the absence of drugs
The classification is based on 11 criteria. For the purpose of identifying patients in
clinical studies, a person is defined as having SLE if any 4 or more of the 11
criteria are present, serially or simultaneously, during any interval of observation.
3


2.3. Pathophysiology

Developing a model of SLE pathogenesis is challenging, given the need to
account for tremendous heterogeneity in disease expression and fluctuations of
disease activity over time. It is clear that autoantibodies, cytokines, and aberrant
lymphocyte function have important roles in SLE pathogenesis. A hallmark of
SLE is the generation of autoantibodies directed against self-antigens, particularly
nucleic acids. These intracellular antigens are ubiquitously expressed but are
usually inaccessible and cloistered within the cell. During cell necrosis or
apoptosis , the antigens are released. SLE skin cells are highly susceptible to
damage from ultraviolet light, and the resulting cell demise results in release of
cell contents, including nucleic antigens. Individuals with SLE may have
markedly increased levels of apoptosis or signifi cantly impaired ability to clear
cell debris, causing prolonged exposure to these nucleic antigens in the
bloodstream and ample opportunity for their recognition by immune cells, leading
10

to production of autoantibodies by B cells. Circulating autoantibodies may form
immune complexes and deposit in tissues, leading to local complement activation,
initiation of a proinflammatory cascade, and, ultimately, tissue damage.
Antibodies to double-stranded DNA can form immune complexes, deposit in
glomeruli, and initiate infl ammation leading to glomerulonephritis. Many
individuals with SLE have circulating antibodies to double-stranded DNA yet do
not have nephritis, suggesting that autoantibodies alone are not suffi cient to cause
disease. Individuals with SLE frequently demonstrate abnormal cytokine levels.
In particular, peripheral blood mononuclear cells from patients with SLE exhibit
patterns of gene expression suggestive of stimulation by interferon- (IFN- ).
IFN- production by dendritic cells can be stimulated in vivo by immune
complexes. Excess levels of interferon can promote expression of other
proinflammatory cytokines and chemokines, maturation of monocytes into
dendritic dells, promotion of autoreactive B and T cells, and loss of self-tolerance.
Many, but not all, patients with SLE exhibit this interferon signature. Other
cytokines with increased expression in SLE include interleukin-2 (IL)-2, IL-6, IL-
10, IL-12, B-lymphocyte stimulator (BlyS), and anti tumor necrosis factor-
(TNF- ). Both B and T cells demonstrate functional impairments in SLE. In
active SLE, B-cell populations have impaired tolerance and increased
autoreactivity, enhancing B cells ability to produce autoantibodies following
exposure to self-antigen. In addition, cytokines such as BlyS may promote
abnormal B-cell number and function. T-cell abnormalities in SLE include
increased numbers of memory T cells and decreased number and function of
Tregulatory cells. SLE T cells display aberrant signaling and increased
autoreactivity. As a result, they are resistant to attrition by normal apoptosis
pathways.
14

2.4 Diagnosis
In one study
18
that used patients with connective tissue diseases as the control
group, the revised ACR diagnostic criteria for systemic lupus erythematosus were
11

found to have an overall sensitivity of 96 percent and a specificity of 96 percent.
Other studies
15,16,17
have reported sensitivities ranging from 78 to 96 percent and
specificities ranging from 89 to 100 percent. The ACR criteria may be less
accurate in patients with mild disease.
15

Elevation of the antinuclear antibody (ANA) titer to 1:40 or higher is the most
sensitive of the ACR diagnostic criteria. More than 99 percent of patients with
systemic lupus erythematosus have an elevated ANA titer at some
point,
16,18
although a significant proportion of patients may have a negative ANA
titer early in the disease.
16
However, the ANA test is not specific for systemic
lupus erythematosus. A study
4
involving 15 international laboratories found that
ANA tests in the general population were positive in 32 percent of persons at a
1:40 dilution and in 5 percent of persons at a 1:160 dilution. Rates of positive
ANA tests were not affected by age up to 60 years (the upper age limit of the
study).
18

In the absence of systemic lupus erythematosus, the most common reason for a
positive ANA test is the presence of another connective tissue disease. Diseases
that often are associated with a positive ANA test include Sjgren's syndrome (68
percent of affected patients), scleroderma (40 to 75 percent), rheumatoid arthritis
(25 to 50 percent), and juvenile rheumatoid arthritis (16 percent).
15
An ANA test
also can be positive in patients with fibromyalgia. In patients with diseases other
than systemic lupus erythematosus, ANA titers usually are lower, and the
immunofluorescent pattern is different.
15

Rates of positive ANA tests are affected by the prevalence of systemic lupus
erythematosus in the population. Specifically, false-positive rates will be higher in
populations with a low prevalence of the disease, such as primary care patients.
Because of the high false-positive rates at 1:40 dilution, ANA titers should be
obtained only in patients who meet specific clinical criteria (discussed in the
clinical recommendations section of this article). When ANA titers are measured,
laboratories should report ANA levels at both 1:40 and 1:160 dilutions and should
12

supply information on the percentage of normal persons who are positive at each
dilution.
18

Interpretation of ANA titers is similar in children. An ANA titer of less than 1:40
is useful for ruling out systemic lupus erythematosus (sensitivity of 98 percent).
However, an ANA titer of 1:40 or higher has a positive predictive value of only
10 percent because of the common occurrence of high ANA titers in children.
20




Routine Blood Tests
The complete blood count measures the levels of each. In cases of lupus, these
blood tests may reveal low numbers.
Red blood cells carry oxygen to all parts of the body.
13

White blood cells (lymphocytes and others) help the immune system protect
the body against foreign invaders. The white blood cells called T cells and
macrophages are directly involved in this protection. Other white blood cells
called B cells are indirectly involved by producing antibodies to the foreign
substances.
Platelets form in bone marrow; they go to the site of a wound to begin the
blood-clotting process.
Blood serum is the fluid portion of whole blood from which certain
substances in the clotting of blood have been removed.

Other Blood Tests
Some blood tests measure levels of proteins that are not antibodies. The levels of
these proteins can alert your doctor that there is inflammation somewhere in your
body.
Complement
The name of a group of proteins that protect the body against infections. They
work by strengthening the bodys immune reactions. Complement proteins are
used up by the inflammation caused by lupus, which is why people with
inflammation due to active lupus often have low complement levels. There are
nine protein groups of complement, so complement is identified by the letter C
and the numbers 1 through 9. The most common complement tests for lupus are
CH50, C3 and C4. CH50 measures the overall function of complement in the
blood. Low levels of C3 or C4 may indicate active lupus. A new combination
blood test is using a subset of the C4, called C4d, to help physicians rule in
lupus and rule out other diseases and conditions.

C-reactive protein (CRP)
14

A protein produced by the liver, and high levels of CRP in your blood may mean
you have inflammation due to lupus.

Erythrocyte sedimentation rate
Another test for inflammation. It measures the amount of a protein that makes the
red blood cells clump together. The sed rate is usually high in people with active
lupus, but can also be high due to other reasons such as an infection

Urine Tests
Urine tests are very important because lupus can attack the kidneys -- often
without warning signs. The kidneys process your bodys waste materials. Testing
a sample of urine (called a spot urine test) can reveal problems with the way
your kidneys are functioning. Lupus can attack the kidneys without any warning
signs so urine tests are very important. The most common urine tests look for cell
casts (bits of cells that normally would be removed when your blood is filtered
through your kidneys) and proteinuria (protein being spilled into your body
because your kidneys are not filtering the waste properly). A collection of your
urine over a 24-hour period can also give important information

Tissue Biopsies
A biopsy procedure involves removal of a small bit of tissue that the doctor then
examines under a microscope. Almost any tissue can be biopsied.
The skin and kidney are the most common sites biopsied in someone who
may have lupus.
The results of the biopsy can show the amount of inflammation and any
damage being done to the tissue.
15

Further tests on the tissue sample can detect autoimmune antibodies and
determine whether lupus or another factor such as infection or medication is
responsible.


2.5. Treatment
Over 90 percent of patients with systemic lupus erythematosus eventually have a
cutaneous manifestation of the disease, including malar rash, discoid lupus
erythematosus, alopecia or aphthous stomatitis. The usual therapy for cutaneous
lupus erythematosus is strict use of sun block, judicious use of topical steroids
(although fluorinated topical steroids should not be used on the face) and
antimalarial therapy. Some patients with very severe cases of discoid lupus
erythematosus may not respond adequately to the usual dosage of
hydroxychloroquine, which is 400 mg per day for a normal-sized adult.
Quinacrine, in a dosage of 100 mg per day, can be added without increasing the
risk of retinopathy, or the patient can be switched to chloroquine HCl (Aralen), in
a dosage of 250 mg per day.
19
Because chloroquine therapy carries an increased risk of retinopathy compared
with hydroxychloroquine therapy, patients taking it should undergo
ophthalmologic monitoring every three months. In patients who cannot tolerate
antimalarials, dapsone or retinoids are additional therapeutic choices. It is
important that glucose-6-phosphate dehydrogenase status be checked in black
patients before they begin dapsone therapy. Retinoids should not be used in
patients who may become pregnant.
20
Patients with very severe cutaneous lupus
erythematosus, including lupus vasculitis, may require high dosages of
corticosteroids. If the maintenance dosage of corticosteroids is greater than 10 mg
per day, the addition of steroid-sparing agents, such as methotrexate or
azathioprine, should be considered. One of the most effective drugs in the
treatment of cutaneous lupus erythematosus, including discoid lupus
16

erythematosus, is thalidomide (Synovir); however, because of its teratogenic
effects and the increased risk of peripheral neuropathy in patients taking it, this
agent will probably never have widespread use.
21
Treatment of Systemic Lupus Erythematosus: An Update
American Academy of Family Physicians
TREATMENT
AGENT COMMENT
Sun block Blocks both UVA and UVB radiation
Antimalarials Use of combination antimalarial therapy
(hydroxychloroquine [Plaquenil] and quinacrine) or
chloroquine (Aralen), which has more risk of retinopathy,
is sometimes necessary
Dapsone G6PD status should be checked
Retinoids Avoid using in pregnant women
Corticosteroids Use of corticosteroids may be necessary as part of initial
therapy for severe discoid lupus or for lupus vasculitis;
intradermal corticosteroids are helpful for individual
discoid lesions, especially in the scalp
Immunosuppressive
drugs
Methotrexate (Rheumatrex) or azathioprine (Imuran) is
used as steroid-sparing drug
Thalidomide
(Synovir)
One of the most effective drugs for treatment of discoid
lupus, but teratogenicity and neuropathy will limit its
acceptance and use



2.6 Prognosis
17

The prognosis has improved with earlier recognition and improved management.
The five-year survival rate is over 90%. Morbidity and mortality are usually
higher in patients with extensive multisystem disease and multiple autoantibodies.
Patients who develop renal involvement, particularly focal and diffuse
proliferative glomerulonephritis, have a poorer prognosis. Drug-induced lupus
usually subsides when the responsible drug is discontinued.
22

Definition
A condition of extreme malnutrition and emaciation, occurring chiefly in young
children. It is characterized by progressive wasting of subcutaneous tissue and
muscle. Marasmus results from a lack of adequate calories and proteins and is
seen in children with failure to thrive and in individuals in a state of starvation.
Less commonly it results from an inability to assimilate or use protein because of
a defect in metabolism.
23

Etiology

Marasmus is a form of malnutrition in which inadequate amounts of both protein
and calories are consumed, resulting in an energy deficit in the body. Causes of
marasmus:
23

1. Inadequate caloric intake, as a result of deficiencies in the arrangement of
food.
2. Food habits are not feasible, as contained in the parent-child relationship is
disrupted or as a result of metabolic disorders or congenital malformations.
3. Each body system disorder that can lead to severe malnutrition.
4. Caused by the negative influence of socioeconomic factors and cultural
events that contribute to general malnutrition, negative nitrogen balance can
18

be caused by chronic diarrhea, malabsorption of protein, urine protein loss
(neprofit syndrome), chronic infections, burns and heart disease

Complications
Finally these infants are subject to various complications. CEdema is one of the
most frequent, and attention is often called to its presence in the first place by an
unexpected gain in weight. It starts in the legs and often becomes generalised.
They are particularly prone to infection of all kinds -e.g., in the skin, mouth, and
lungs. Otitis media is of common occurrence and is less a complication than a
feature of the disease. Pyelitis is also frequent. Purpuric eruptions on the skin are
seen in severe cases and are of bad omen. Marantic thrombosis of cerebral sinuses
is sometimes observed post mortem, though clinical recognition of this is
difficult.
24
It also causes other consequences of malnutrition include:
25

Impaired immune response and increased risk of infection
Reduced muscle strength
Impaired wound healing
Impaired psycho-social function, including poor cognition and increased
dependency
Impaired recovery from illness and surgery
Poorer health outcomes
Poor quality of life
26



Treatment
Step 1. Treat/prevent hypoglycaemia
Hypoglycaemia and hypothermia usually occur together and are signs of
infection. Frequent feeding is important inpreventing both conditions.
19

If the child is conscious and dextrostix shows <3mmol/l or 54mg/dl give:
50 ml bolus of 10% glucose or 10% sucrose solution (1 rounded teaspoon of
sugar in 3.5 tablespoons water), orally or by nasogastric (NG) tube. Then feed
starter F-75 every 30 minutes for two hours (giving one quarter of the two-hourly
feed each time)
antibiotics
two-hourly feeds, day and night
If the child is unconscious, lethargic or convulsing give:
IV sterile 10% glucose (5ml/kg), followed by 50ml of 10% glucose or sucrose
by Ng tube. Then give starter F-75 as above
antibiotics
two-hourly feeds, day and night

Step 2. Treat/prevent hypothermia
feed straightaway (or start rehydration if needed)
rewarm the child: either clothe the child (including head), cover with a warmed
blanket and place a heater or lamp nearby (do not use a hot water bottle), or put
the child on the mothers bare chest (skin to skin) and cover them
give antibiotics

Step 3. Treat/prevent dehydration
The standard oral rehydration salts solution (90 mmol sodium/l) contains too
much sodium and too little potassium for severely malnourished children. Instead
give special Rehydration Solution for Malnutrition (ReSoMal). It is difficult to
20

estimate dehydration status in a severely malnourished child using clinical signs
alone. So assume all children with watery diarrhoea may have dehydration and
give:
ReSoMal 5 ml/kg every 30 min. for two hours, orally or by nasogastric tube,
then
5-10 ml/kg/h for next 4-10 hours: the exact amount to be given should be
determined by how much the child wants, and stool loss and vomiting. Replace
the ReSoMal doses at 4, 6, 8 and 10 hours with F-75 if rehydration is continuing
at these times, then
continue feeding starter F-75

Step 4. Correct electrolyte imbalance
All severely malnourished children have excess body sodium even though plasma
sodium may be low (giving high sodium loads will kill). Deficiencies of
potassium and magnesium are also present and may take at least two weeks to
correct. Oedema is partly due to these imbalances. Do NOT treat oedema with a
diuretic.
Give:
extra potassium 3-4 mmol/kg/d
extra magnesium 0.4-0.6 mmol/kg/d
when rehydrating, give low sodium rehydration fluid (e.g. ReSoMal)
prepare food without salt

Step 5. Treat/prevent infection
21

In severe malnutrition the usual signs of infection, such as fever, are often absent,
and infections are often hidden. Therefore give routinely on admission:
broad-spectrum antibiotic(s) AND
measles vaccine if child is > 6m and not immunised (delay if the child is in
shock)

Step 6. Correct micronutrient deficiencies
All severely malnourished children have vitamin and mineral deficiencies.
Although anaemia is common, do NOT give iron initially but wait until the child
has a good appetite and starts gaining weight (usually by the second week), as
giving iron can make infections worse.
Give:
Vitamin A orally on Day 1 (for age >12 months, give 200,000 IU; forage 6-12
months, give 100,000 IU; for age 0-5 months, give 50,000 IU) unless there is
definite evidence that a dose has been given in the last month
Give daily for at least 2 weeks:
Multivitamin supplement
Folic acid 1 mg/d (give 5 mg on Day 1)
Zinc 2 mg/kg/d
Copper 0.3 mg/kg/d
Iron 3 mg/kg/d but only when gaining weight

Step 7. Start cautious feeding
22

In the stabilisation phase a cautious approach is required because of the childs
fragile physiological state and reduced homeostatic capacity. Feeding should be
started as soon as possible after admission and should be designed to provide just
sufficient energy and protein to maintain basic physiological processes. The
essential features of feeding in the stabilisation phase are:
small, frequent feeds of low osmolarity and low lactose
oral or nasogastric (NG) feeds (never parenteral preparations)
100 kcal/kg/d
1-1.5 g protein/kg/d
130 ml/kg/d of fluid (100 ml/kg/d if the child has severe oedema)
if the child is breastfed, encourage to continue breastfeeding but give the
prescribed amounts of starter formula to make sure the childs needs are met.

Step 8. Achieve catch-up growth
In the rehabilitation phase a vigorous approach to feeding is required to achieve
very high intakes and rapid weight gain of >10 g gain/kg/d. The recommended
milk-based F-100 contains 100 kcal and 2.9 g protein/100 ml. Modified porridges
or modified family food can be used provided they have comparable energy and
protein concentrations.
To change from starter to catch-up formula:
replace starter F-75 with the same amount of catch-up formula F-100 for 48
hours then,
increase each successive feed by 10 ml until some food remains uneaten. The
point when some remains unconsumed is likely to occur when intakes reach about
30 ml/kg/feed (200 ml/kg/d).
23


Step 9. Provide sensory stimulation and emotional support
In severe malnutrition there is delayed mental and behavioural development.
Provide:
tender loving care
a cheerful, stimulating environment
structured play therapy 15-30 min/d (Appendix 10 provides examples)
physical activity as soon as the child is well enough
maternal involvement when possible (e.g. comforting, feeding, bathing, play)

Step 10. Prepare for follow-up after recovery
A child who is 90% weight-for-length (equivalent to -1SD) can be considered to
have recovered. The child is still likely to have a low weight-for-age because of
stunting. Good feeding practices and sensory stimulation should be continued at
home. Show parent or carer how to:
feed frequently with energy- and nutrient-dense foods
give structured play therapy
Advise parent or carer to:
bring child back for regular follow-up checks
ensure booster immunizations are given
ensure vitamin A is given every six months
27

24

























CHAPTER III
CASE REPORT
Name : KKS
Age : 13 years
Sex : Female
25

Date of Admission : March, 28
th
2014

Main Complaint: Both legs cannot be straightened. This is experienced by the
patient for past six months. Walking ability (-).
History: This is experienced by the patient for past six months. Walking ability
(-). Firstly, the patient has complained about pain in the right and left knee and
elbow joints, especially in the morning. Eruption of red squama in the body (+)
for four months. With time, the red coloured squama changed into brown coloured
squama. Fever (-), history of fever (+) for 6 months, fever reduced by antipyretic,
shiver(-).
Productive cough (+), history of contact with cough patients(-), paleness (+)
experienced by patient four months ago and during that time, the patients level of
Hb is 3gr%. History of spontaneous bleeding (-). History of blood transfusion (+).
Has transfused 12 bags. Shortness of breath (+) experienced by patient for 3
months and worsened 3 days ago. History of weight loss (+). Weight loss
occurred slowly. Body weight before illness: 45kg (6 months ago). Highest body
weight: 45kg. Loss of appetite (-). Urination and defecation (+) normal.

History of previous illness : Patient was referred from RSU Abdul Manam
Kisaran by SpA with malnutrition and cardiomegaly.
History of previous medications : Progenic 3 x 1 tsp. Cefixime 3 x 1 tsp.
History of labor : normal delivery, handled by her father, cried
as soon as baby was born, no cyanosis, the patient is the 3
rd
child.
History of feeding : Breast milk until 2 years old
History of immunization : BCG


Presens status
Sensorium : Compos Mentis Blood pressure : 100/70mmHg
Body temperature: 37,4
o
C Pulse : 130 bpm
26

Respiratory Rate: 44 x/minute

Physical Examination
BB : 28 kg BW/Age: 59.6%
TB : 131 cm BH/Age: 88.6%
Upper Arm Circumference (UAC): 16 cm BW/BH: 85%
1. Head: Eye: light reflexes (+/+), isocor pupil, pale conjunctiva palpebra
inferior (+/+), Ear : normal, Nose: nasal canule, Mouth: normal
2. Neck: Lymph node enlargement (-)
3. Thorax : Symmetrical fusiformis, retraction (-), intercostals space visible
HR: 130 bpm, regular, murmur (-)
RR: 44 x/minute, regular, rhonchi (-/-).
4. Abdomen: soepel, peristaltic (+) normal,
Spleen palpable 8cm
Liver: not palpapable
5. Extremities: Pulse 130 bpm, regular, adequate vascular pressure and
volume, warm acral, CRT < 3, pitting edema (+), baggy
pants (+), thinning of subcutaneous fat (+). Skin on leg: dry
(+), squama (+).
Work up: DL, KGD, Elektrolit, RFT, Chest X-Ray, ACRP, ANA Test, Anti DS-
DNA

Working Diagnosis
ARJ + Malnutrition stage V + anemia e.c DD chronic disease / Fe deficiency

Differential Diagnosis
DD 1. ARJ + Malnutrition stage V + anemia e.c DD -chronic disease
2. SLE -Fe deficiency


Treatment
27

Oxygen 1-2 L/I nasal canule
D 10% 50cc/oral
Three way

Laboratory result
28
th
of March 2014
COMPLETE BLOOD COUNT (CBC)
Hemoglobin (HGB) g% 6.80
Erythrocyte (RBC) 10
6
/ mm
3
2.45
Leukocyte (WBC) 10
3
/ mm
3
5.02
Hematocryte % 21.50
Trombocyte (PLT) 10
3
/ mm
3
185
MCV Fl 87.80
MCH Pg 27.80
MCHC g% 31.60
RDW % 13.80
MPV Fl 8.80
PCT % 0.16
PDW fL 9.9
Neutrophil % 74.50
Lymphocyte % 19.70
Monocyte % 4.20
Eosinophil % 1.40
Basophil % 0.200
Absolute Neutrophil 10
3
/L 3.74
Absolute Lymphocyte 10
3
/L 0.99
Absolute Monocyte 10
3
/L 0.21
Absolute Eosinophil 10
3
/L 0.07
Absolute Basophil 10
3
/L 0.01

28

CLINICAL CHEMISTRY
Blood Chemical Analysis
pH 7.453
pCO2 mmHg 22.9
pO2 mmHg 173.3
Bicarbonate (HCO3) mmol/L 15.7
Total CO2 mmol/L 16.4
Excess base mmol/L -6.0
O2 Saturation % 98.9
Carbohydrate Metabolism
Blood Glucose level at
random
mg/dL 90.90
GINJAL
Ureum mg/dL 46.80
Creatinin mg/dL 0.68
ELECTROLYTE
Natrium mEq/L 130
Kalium mEq/L 3.8
Chlorida mEq/L 113


Follow up (29
th
March 2014)
S : Body erythema (+). Both legs cannot be straightened. Batuk (+).
O: Sens: CM, HR: 130 bpm, RR: 42 x/minute, Temp: 36.8C
BB : 28 kg BW/Age: 59.6%
TB : 131 cm BH/Age: 88.6%
Upper Arm Circumference (UAC): 16 cm BW/BH: 85%
29

1. Head: Hair thinning, sparsely growing
Eye: light reflexes (+/+), isocor pupil, pale conjunctiva palpebra inferior
(+/+), Ear : normal, Nose: nasal canule, Mouth: normal
2. Neck: Lymph node enlargement (-)
3. Thorax : Symmetrical fusiformis, retraction (-), intercostals space visible
HR: 130 bpm, pansystolic murmur gr IV/6 LMCS ICR III-IV
RR: 42x/minute, regular, rhonchi (-/-).
4. Abdomen: soepel, peristaltic (+) normal,
Spleen palpable 8cm
Liver: not palpapable
5. Extremities: Pulse 130 bpm, regular, adequate vascular pressure and
volume, warm acral, CRT < 3, pitting edema (+), baggy
pants (+), thinning of subcutaneous fat (+). Skin on leg: dry
(+), squama (+).

History of previous illness : Patient was referred from RSU Abdul Manam
Kisaran by SpA with malnutrition and cardiomegaly.
History of previous medications : Progenic 3 x 1 tsp. Cefixime 3 x 1 tsp.
History of previous lab reports :
26.12.13 : Hb/Ht/L/Tr : 4.9/14.6/15.890/250.000
30.12.13 : Hb/Ht/L/Tr : 7.5/21.3/9.380/231.000
02.01.14 : Hb/Ht/L/Tr : 9.5/26.3/10.940/345.000
A: DD 1. ARJ + Malnutrition stage V + anemia e.c DD -chronic disease
2. SLE -Fe deficiency
P: Oxygen 1-2 L/I nasal canule
D 10% 50cc/oral
Three way
Paracetamol 3 x400 mg (k/p)
Diet MB 1660 kcal with 60g of protein

30

CLINICAL CHEMISTRY
LIVER
Albumine g/dL 1,8
IMMUNOSEROLOGY
Procalcitonine ng/dL 56.91.52


Follow up (30
th
March 2014)
S : Body erythema (+). Both extremities cannot be straightened. cough (+).
O: Sens: CM, HR: 128 bpm, RR: 42x/minute, Temp: 37.8C
BB : 28 kg BW/Age: 59.6%
TB : 131cm BH/Age: 88.6%
Upper Arm Circumference (UAC): 16 cm BW/BH: 85%
1. Head: Hair thinning, sparsely growing
Eye: light reflexes (+/+), isocor pupil, pale conjunctiva palpebra inferior
(+/+), Ear : normal, Nose: nasal canule, Mouth: normal
2. Neck: Lymph node enlargement (-)
3. Thorax : Symmetrical fusiformis, retraction (-), intercostals space visible
HR: 128 bpm, pansystolic murmur gr IV/6 LMCS ICR III-IV
RR: 42 x/minute, regular, rhonchi (-/-).
4. Abdomen: soepel, peristaltic (+) normal,
Spleen palpable 8cm
Liver: not palpapable
5. Extremities: Pulse 128 bpm, regular, adequate vascular pressure and
volume, warm acral, CRT < 3, pitting edema (+), baggy
pants (+), thinning of subcutaneous fat (+). Skin on leg: dry
(+), squama (+)

A: DD 1. ARJ + Malnutrition stage V + anemia e.c DD -chronic disease
2. SLE -Fe deficiency
P: Oxygen 1-2 L/I nasal canule
31

Three way
Paracetamol 3 x400 mg (k/p)
Diet MB 1660 kcal with 60g of protein


Follow up (31
st
of March 2014)
S : Fever (+). Body erythema (+). Both extremities cannot be straightened.
O: Sens: CM, HR: 130 bpm, RR: 42 x/minute, Temp: 37.0C
BB : 28 kg BW/Age: 59.6%
TB : 131 cm BH/Age: 88.6%
Upper Arm Circumference (UAC): 16 cm BW/BH: 85%
1. Head: Hair thinning, sparsely growing
Eye: light reflexes (+/+), isocor pupil, pale conjunctiva palpebra inferior
(+/+), Ear : normal, Nose: nasal canule, Mouth: normal
2. Neck: Lymph node enlargement (-)
3. Thorax : Symmetrical fusiformis, retraction (-), intercostals space visible
HR: 130 bpm, regular, pansystolic murmur gr IV/6 LMCS ICR
III-IV
RR: 42 x/minute, regular, rhonchi (-/-).
4. Abdomen: soepel, peristaltic (+) normal,
Spleen palpable 8cm
Liver: not palpapable
5. Extremities: Pulse 130 bpm, regular, adequate vascular pressure and
volume, warm acral, CRT < 3, pitting edema (+), baggy
pants (+), thinning of subcutaneous fat (+). Skin on leg: dry
(+), squama (+).

A: DD 1. SLE + Malnutrition stage V + anemia e.c DD -chronic disease
-Fe deficiency
P: Oxygen 1-2 L/I nasal canule
32

Three way
Paracetamol 3 x400 mg (k/p)
Diet MB 1660 kcal with 60g of protein

Follow up (1
st
April 2014)
S : Body erythema (+). Both extremities cannot be straightened.
O: Sens: CM, HR: 126 bpm, RR: 42 x/minute, Temp: 37.4C
TB : 131 cm BW/Age: 59.6%
BH/Age: 88.6%
Upper Arm Circumference (UAC): 16 cm BW/BH: 85%
1. Head: Hair thinning, sparsely growing
Eye: light reflexes (+/+), isocor pupil, pale conjunctiva palpebra inferior
(+/+), Ear : normal, Nose: nasal canule, Mouth: normal
2. Neck: Lymph node enlargement (-)
3. Thorax : Symmetrical fusiformis, retraction (-), intercostals space visible
HR: 126 bpm, regular, pansystolic murmur gr IV/6 LMCS ICR
III-IV
RR: 42 x/minute, regular, rhonchi (-/-).
4. Abdomen: soepel, peristaltic (+) normal,
Spleen palpable 8cm
Liver: not palpapable
5. Extremities: Pulse 130 bpm, regular, adequate vascular pressure and
volume, warm acral, CRT < 3, pitting edema (+), baggy
pants (+), thinning of subcutaneous fat (+). Skin on leg: dry
(+), squama (+).

A: DD 1. ARJ + Malnutrition stage V + anemia e.c DD -chronic disease
2. SLE -Fe deficiency
P: Oxygen 1-2 L/I nasal canule
Three way
33

Paracetamol 3 x400 mg (k/p)
Diet MB 1660 kcal with 60g of protein


COMPLETE BLOOD COUNT (CBC)
Hemoglobin (HGB) g% 6.80
Eritrocyte (RBC) 10
6
/ mm
3
2.44
Leukocyte (WBC) 10
3
/ mm
3
5.51
Hematocryte % 21.70
Trombocyte (PLT) 10
3
/ mm
3
178
MCV fL 88.90
MCH Pg 27.90
MCHC g% 31.30
RDW % 14.50
MPV fL 8.70
PCT % 0.15
PDW fL 10.3
Neutrophil % 79.10
Lymphocyte % 15.60
Monocyte % 4.20
Eosinophil % 0.90
Basophil % 0.200
Absolute Neutrophil 10
3
/L 4.36
Absolute Lymphocyte 10
3
/L 0.86
Absolute Monocyte 10
3
/L 0.23
Absolute Eosinophil 10
3
/L 0.05
Absolute Basophil 10
3
/L 0.01

34

FAAL HEMOSTASIS
Ferritine ng/mL >2000.00
Ferrum (Fe/iron) mg/dL 34
TIBC ug/dL 96
Clinical Chemistry
Liver
Total Bilirubin mg/dL 0.73
Direct Bilirubin mg/dL 0.62
Alkali
phosphatase
U/L 994
AST/SGOPT U/L 114
ALT/SGPT U/L 43
Kidney
Ureum mg/dL 50.70
Creatinine mg/dL 0.71
Uric Acid mg/dL 7.6
IMMUNOSEROLOGY
AUTOIMUMUNE
ANA test 112
Anti ds-DNA 530.0
Qualitative CRP Positif
Other Test
Procalcitonin mg/dL 0.77







35

Follow up (2
nd
of April 2014)
S : Body erythema (+). Both extremities cannot be straightened.
O: Sens: CM, HR: 128 bpm, RR: 40 x/minute, Temp: 37.4C
TB : 131 cm BW/Age: 59.6%
BH/Age: 88.6%
Upper Arm Circumference (UAC): 16 cm BW/BH: 85%
1. Head: Hair thinning, sparsely growing
Eye: light reflexes (+/+), isocor pupil, pale conjunctiva palpebra inferior
(+/+), Ear : normal, Nose: nasal canule, Mouth: normal
2. Neck: Lymph node enlargement (-)
3. Thorax : Symmetrical fusiformis, retraction (-), intercostals space visible
HR: 128 bpm, regular, pansystolic murmur gr IV/6 LMCS ICR
III-IV
RR: 40 x/minute, regular, rhonchi (-/-).
4. Abdomen: soepel, peristaltic (+) normal,
Spleen palpable 8cm
Liver: not palpapable
5. Extremities: Pulse 130 bpm, regular, adequate vascular pressure and
volume, warm acral, CRT < 3, pitting edema (+), baggy
pants (+), thinning of subcutaneous fat (+). Skin on leg: dry
(+), squama (+).

A: DD 1. ARJ + Malnutrition stage V + anemia e.c DD -chronic disease
2. SLE -Fe deficiency
P: Oxygen 1-2 L/I nasal canule
Three way
Paracetamol 3 x400 mg (k/p)
Diet MB 1660 kcal with 60g of protein



36

Follow up (3
rd
of April 2014)
S : Body erythema (+). Both extremities cannot be straightened.
O: Sens: CM, HR: 128 bpm, RR: 40 x/minute, Temp: 37.5C
TB : 131 cm BW/Age: 59.6%
BH/Age: 88.6%
Upper Arm Circumference (UAC): 16 cm BW/BH: 85%
1. Head: Hair thinning, sparsely growing
Eye: light reflexes (+/+), isocor pupil, pale conjunctiva palpebra inferior
(+/+), Ear : normal, Nose: nasal canule, Mouth: normal
2. Neck: Lymph node enlargement (-)
3. Thorax : Symmetrical fusiformis, retraction (-), intercostals space visible
HR: 128 bpm, regular, pansystolic murmur gr IV/6 LMCS ICR
III-IV
RR: 40 x/minute, regular, rhonchi (-/-).
4. Abdomen: soepel, peristaltic (+) normal,
Spleen palpable 8cm
Liver: not palpapable
5. Extremities: Pulse 130 bpm, regular, adequate vascular pressure and
volume, warm acral, CRT < 3, pitting edema (+), baggy
pants (+), thinning of subcutaneous fat (+). Skin on leg: dry
(+), squama (+).

A: DD 1. ARJ + Malnutrition stage V + anemia e.c DD -chronic disease
2. SLE -Fe deficiency
P: Oxygen 1-2 L/I nasal canule
Three way
Paracetamol 3 x400 mg (k/p)
Diet MB 1660 kcal with 60g of protein



37

URINANALYSIS RESULTS
Colour Clear yellow
Glucose Negative
Bilirubin Negative
Ketone Negative
Specific Gravity 1.015
pH 5.0
Protein +2
Urobilinogen Negative
Nitrite Negative
Blood Negative
Urine Sediment
Erytrocyte LPB 0-2
Leucocyte LPB 1-2
Epithel LPB 0-1
Casts LPB Negative
Crystal LPB Negative

Follow up (4
th
of April 2014)
S : Body erythema (+). Both extremites cannot be straightened.
O: Sens: CM, BP: 100/70mmHg, HR: 120 bpm, RR: 40 x/minute, Temp: 36.8C
BB : 28 kg BW/Age: 59.6%
TB : 131 cm BH/Age: 88.6%
Upper Arm Circumference (UAC): 16 cm BW/BH: 85%
1. Head: Hair thinning, sparsely growing
Eye: light reflexes (+/+), isocor pupil, pale conjunctiva palpebra inferior
(+/+), Ear : normal, Nose: nasal canule, Mouth: normal
2. Neck: Lymph node enlargement (-)
3. Thorax : Symmetrical fusiformis, retraction (-), intercostals space visible
38

HR: 130 bpm, , regular, pansystolic murmur gr IV/6 LMCS ICR
III-IV
RR: 44 x/minute, regular, rhonchi (-/-).
4. Abdomen: soepel, peristaltic (+) normal,
Spleen palpable 8cm
Liver: not palpapable
5. Extremities: Pulse 120 bpm, regular, adequate vascular pressure and
volume, warm acral, CRT < 3, pitting edema (+), baggy
pants (+), thinning of subcutaneous fat (+). Skin on leg: dry
(+), squama (+).

A: SLE + Malnutrition stage V + anemia e.c DD -chronic disease
-Fe deficiency
P: Oxygen 1-2 L/I nasal canule
Furosemide 2x20mg
Spironolactone 2x12.5mg
Digoxin 2x1/2 tab
Three way
Paracetamol 3 x400 mg (k/p)
Diet MB 1660 kcal with 60g of protein

Follow up (5
th
of April 2014)
S : Body erythema (+). Both extremites cannot be straightened.
O: Sens: CM Temp: 37.2C
1. Head: Hair thinning, sparsely growing
Eye: light reflexes (+/+), isocor pupil, pale conjunctiva palpebra inferior
(+/+), Ear : normal, Nose: nasal canule, Mouth: normal
2. Neck: Lymph node enlargement (-)
3. Thorax : Symmetrical fusiformis, retraction (-), intercostals space visible
39

HR: 125 bpm, , regular, pansystolic murmur gr IV/6 LMCS ICR
III-IV
RR: 40x/minute, regular, rhonchi (-/-).
4. Abdomen: soepel, peristaltic (+) normal,
Spleen palpable 8cm
Liver: not palpapable
5. Extremities: Pulse 125 bpm, regular, adequate vascular pressure and
volume, warm acral, CRT < 3, pitting edema (+), baggy
pants (+), thinning of subcutaneous fat (+). Skin on leg: dry
(+), squama (+).

A: SLE + Malnutrition stage V + anemia e.c DD -chronic disease + bilateral genu
Contraction -Fe deficiency
P: Oxygen 1-2 L/I nasal canule
Furosemide 2x20mg
Spironolactone 2x12.5mg
Digoxin 2x1/2 tab
Three way
Paracetamol 3 x400 mg (k/p)
Diet MB 1660 kcal with 60g of protein

Follow up (6
th
of April 2014)
S : Body erythema (+). Both extremites cannot be straightened.
O: Sens: CM, Temp: 36.8C
1. Head: Hair thinning, sparsely growing
Eye: light reflexes (+/+), isocor pupil, pale conjunctiva palpebra inferior
(+/+), Ear : normal, Nose: nasal canule, Mouth: normal
2. Neck: Lymph node enlargement (-)
3. Thorax : Symmetrical fusiformis, retraction (-), intercostals space clearly
visible
40

HR: 120 bpm, , regular, pansystolic murmur gr IV/6 LMCS ICR
III-IV
RR: 30x/minute, regular, rhonchi (-/-).
4. Abdomen: soepel, peristaltic (+) normal,
Spleen palpable 8cm
Liver: not palpapable
5. Extremities: Pulse 120 bpm, regular, adequate vascular pressure and
volume, warm acral, CRT < 3, pitting edema (+), baggy
pants (+), thinning of subcutaneous fat (+). Skin on leg: dry
(+), squama (+).

A: SLE + Malnutrition stage V + anemia e.c DD -chronic disease + bilateral genu
-Fe deficiency
contraction + mild moderate PE + moderate TR + mild AR + pleural effusion

P: Oxygen 1-2 L/I nasal canule
Furosemide 2x20mg
Spironolactone 2x12.5mg
Digoxin 2x1/2 tab
Three way
Paracetamol 3 x400 mg (k/p)
Diet MB 1660 kcal with 60g of protein

Follow Up (7
th
of April 2014)
S : Body erythema. Both extremites cannot be straightened.
O: Sens: CM, Temp: 37.0C
1. Head: Hair thinning, sparsely growing
Eye: light reflexes (+/+), isocor pupil, pale conjunctiva palpebra inferior
(+/+), Ear : normal, Nose: nasal canule, Mouth: normal
2. Neck: Lymph node enlargement (-)
41

3. Thorax : Symmetrical fusiformis, retraction (-), intercostals space clearly
visible
HR: 120 bpm, , regular, pansystolic murmur gr IV/6 LMCS ICR
III-IV
RR: 30x/minute, regular, rhonchi (-/-).
4. Abdomen: soepel, peristaltic (+) normal,
Spleen palpable 8cm
Liver: not palpapable
5. Extremities: Pulse 120 bpm, regular, adequate vascular pressure and
volume, warm acral, CRT < 3, pitting edema (+), baggy
pants (+), thinning of subcutaneous fat (+). Skin on leg: dry
(+), squama (+).

A: SLE + Malnutrition stage V + anemia e.c DD -chronic disease + bilateral genu
-Fe deficiency
contraction + mild moderate PE + moderate TR + mild AR + pleural effusion

P: Oxygen 1-2 L/I nasal canule
Furosemide 2x20mg
Spironolactone 2x12.5mg
Digoxin 2x1/2 tab
Paracetamol 3 x400 mg (k/p)
Diet MB 1660 kcal with 60g of protein

Follow Up (8
th
of April 2014)
S : Both extremites cannot be straightened. Edema of right hand (+)
O: Sens: CM, Temp: 37.7C
42

1. Head: Hair thinning, sparsely growing
Eye: light reflexes (+/+), isocor pupil, pale conjunctiva palpebra inferior
(+/+), Ear : normal, Nose: nasal canule, Mouth: normal
2. Neck: Lymph node enlargement (-)
3. Thorax : Symmetrical fusiformis, retraction (-), intercostals space clearly
visible
HR: 124 bpm, , regular, pansystolic murmur gr IV/6 LMCS ICR
III-IV
RR: 30x/minute, regular, rhonchi (-/-).
4. Abdomen: soepel, peristaltic (+) normal,
Spleen palpable 8cm BAC (D)
Liver: not palpapable
5. Extremities: Pulse 124 bpm, regular, adequate vascular pressure and
volume, warm acral, CRT < 3, pitting edema (+), baggy
pants (+), thinning of subcutaneous fat (+). Skin on leg: dry
(+), squama (+).

A: SLE + Malnutrition stage V + anemia e.c DD -chronic disease + bilateral genu
-Fe deficiency
contraction + mild moderate PE + moderate TR + mild AR + pleural effusion

P: Oxygen 1-2 L/I nasal canule
Furosemide 2x20mg
Spironolactone 2x12.5mg
Pabanox cream
Digoxin 2x1/2 tab
Paracetamol 3 x400 mg (k/p)
Diet MB 1660 kcal with 60g of protein


43


CHAPTER IV
DISCUSSION AND SUMMARY
Systemic lupus erythematosus is an autoimmune disorder characterized by
the production of autoantibodies and polyclonal activation of B lymphocytes. The
child with lupus nephritis presented with systemic and often severe manifestations
in the form of arthritis (60-80%), skin rash (60-78%), malar rash (20-70%),
central nervous system manifestations (5-30%), and cardiopulmonary
manifestations (10-30%) at the time of diagnosis. It shows immunogenetic
association with HLA-A1, B8, DR3, DR2, C
4a
null and inherited defects of the
complement component C
2
, C
4
, C
1
esterase inhibitors.
In our case, the 13-year-old girl initially presented with features such as
juvenile arthritis where both her legs couldnt be straightened and eruption of red
squamous in the body. Later on, a detailed investigation such as complete blood
count, blood glucose level, electrolyte, RFT, Chest X-Ray, ANA test, Anti DS-
DNA, Renal Ultrasonography and urinalysis confirmed the diagnosis of lupus
erythematosus. Complete blood count was suggestive of anemia. Electrolyte test
suggested hyponatremia. Urinalysis was leading towards proteinuria. Renal
ultrasonography was suggestive of a possibility of a chronic parenchymal kidney
disease with hydronephrosis bilateral accompanied by ascites. The ANA test was
positive and the Anti DS-DNA was also positive. Diagnosis of systemic lupus
erythematosus was confirmed by a combination of clinical and laboratory
manifestations. As per American Rheumatism Association's 1997 revised
classification criteria for systemic lupus erythematosus, 4 of 11 criteria are
required. In our case, 6 of 11 criteria were fulfilled, and so diagnosis of systemic
lupus erythematosus was confirmed. The patient was given furosemide 20mg
twice daily. She was also started on spironolactone 12.5mg twice daily. The
patient was also given digoxin a tablet, twice daily.

44


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