UNIVERSIDAD PRIVADA SAN JUAN

BAUTISTA
ESCUELA DE MEDICINA HUMANA

Tema

The Clinical History

Curso

Ingles Tcnico 1

Profesora

Condori Ataucuri, Carmen Eva

Integrantes

Del Villar Zegarra, Karol

Espinoza Baldeon, Joselyn

Perez Durand, Patricia

Ciclo

VI

Sede

Hospital Hiplito Unanue

 2017
NEOPHOTIC SYNDROME
INTRODUCTION

The term nephrotic syndrome (NS) is applicable to any clinical condition with massive
proteinuria, hypoproteinemia, hyperlipidemia and edema. It consists of a selective
permeability disorder that may be primary (85-90%) or secondary (10-15%) in the
context of a systemic disease. In children the most frequent SN variety is characterized
by minimal histological changes in the glomerulus with the light microscope: nephrotic
syndrome at minimal changes (SNCM) and with response to corticosteroids or
corticosteroids. Children with steroid-resistant SN may have different histologic
patterns including focal and segmental glomerulosclerosis (GEFS) and the greatest
difference is seen in the progression of FSGS to end-stage renal disease, which rarely
occurs in the SNCM. However, there is consensus to include both pathologies within
idiopathic nephrotic syndrome (NIS) and to classify patients according to steroid
response and histological pattern.

DIAGNOSIS

Clinic

The onset is determined by the development of progressive edema and selective

proteinuria. Occasionally arterial hypertension (AHT) and nitrogen retention.
Precipitating events are unknown except for association with allergic reactions.

Vital signs:

A. Normal or Increased Blood Pressure: 20% of patients with mild and transient
hypertension are described in the SN. Orthostatic hypotension is reported.

B. Temperature: Normal, unless an infection coexists.

C. Heart rate: Tachycardia secondary to decreased intravascular volume due to

decreased oncotic pressure or congestive heart failure.

D. Respiratory rate: Increased if there is massive edema with pleural effusion,

pulmonary embolism, ascites, decreased cardiac output or congestive heart failure.

Physical exam:

E. Edema: Edema occurs two to four weeks after the onset of proteinuria and is initially
soft and morning with a progressive and generalized tendency (bloated fascicles).

F. Cardiac Clinic: Decreased cardiac sounds may mean pericardial effusion and, if
increased, may be related to pulmonary edema. Peripheral pulses may be difficult to
locate due to edema.
G. Respiratory Clinic: Respiratory difficulty if it occurs is associated with severe edema
or pulmonary edema. It can also indicate pneumonia. Thromboembolism has been
reported in 30% of patients with NS and a third of them develop pulmonary embolism.

H. Gastrointestinal Clinic: Diarrhea secondary to edema of the intestinal mucosa or

intercurrent infectious process may be observed. Abdominal pain reflects edema of the
intestinal wall or irritation of the mesenteric plexus. The stiffness of the abdominal wall
could mean peritonitis. Ackerman reported hepatomegaly in approximately 50% of
children hospitalized for NS and splenomegaly in four of eleven patients in that series.

I. Genitourinary Clinic: There may be scrotal edema, vulvar and in some cases
lymphedema. Occasionally hydrocele can be observed.

Laboratory.

A. Hematology: Leukocytosis secondary to infection and erythropoietin deficiency

anemia may occur. The prolongation of bleeding time is seen in the states of
hypercoagulability. The mean glomerular sedimentation rate (ESR) is reported at 67
mm / hr.

B. Immunoglobulins: The plasma concentration of IgG and IgA is decreased and

elevated IgM. There are 25% of patients with elevated levels of IgE.

C. Biochemistry: Hyponatremia is secondary to increased total body water by secretion

of antidiuretic hormone (ADH) in response to decreased oncotic pressure and
intravascular volume. An increase in urea and creatinine can be noted if there is
hypovolemia. There is a decrease in serum proteins with albumin of less than 2.5 g / dL
with edema, hypovolemia, orthostatic hypotension, hyperlipidemia and increased
toxicity of albumin-bound drugs. Hyperlipidemia appears to be the result of both
overproduction and decreased catabolism of b-lipoproteins. Values for
hypercholesterolemia are 300 mg / dL. Low-density lipoproteins and triglycerides are
precociously elevated with varying increases in VLDL, LDL, and intermediate density
lipoprotein fractions. HDL values are usually normal. Total plasma calcium is decreased
although ionized calcium is normal.

D. Urine test: Proteinuria in the nephrotic range is the presence of more than 1 g / l in a
24-hour urine sample, which correlates with proteinuria greater than 40 mg / m2 SC /
hour or protein / creatinine ratio in Urine at random> 2. The presence of albumin (more
than 85%) and weak traces of beta globulins, alpha 1 and 2 are described almost
exclusively. Microscopic hematuria is present in 23% of patients with uncomplicated NS
but may be indicative of other glomerular diseases , Together with the presence of
hematic cylinders. A level of 1384 nmol creatinuria has a sensitivity of 45% and a
specificity of 92% in the diagnosis of SNCM.

E. Serology: Secondary schooling should be ruled out for C3, LE cells, ANA, ANCA,
HBsAg, VDRL. Children with NIS usually have ASO titers <100. A titre> 100 leads to
consideration of another kidney disease.

Study by images.
 Renal echography: It allows to determine the structure and size of the kidneys
and to rule out obstruction. It also determines the presence of two kidneys prior
to the biopsy.

Chest x-ray: Congestive heart failure or infection such as pneumonia should be

ordered in patients with symptoms of pulmonary edema. Cardiomegaly, diffuse
or paraillary infiltrates, or pleural effusion with congestive heart failure may be
observed. Pulmonary TB should also be ruled out.

Differential diagnosis.

It is aimed at secondary schooling such as collagenopathies (systemic lupus

erythematosus (SLE), etc.), vasculitis (Henoch-Schnlein purpura), Wegener's disease,
panarteritis nodosa, etc., amyloidosis, hemolytic uremic syndrome, HIV nephropathy,
diabetes Mellitus, congenital lus among others.

Complications.

The mortality range for SN is 1 to 2%. They are identified as complications:

A. Infections: NS during relapses has an increased susceptibility to severe bacterial

infections by 8% due to decreased immunoglobulins, protein deficiency,
immunosuppressive therapy, reduced splenic perfusion by hypovolemia, and loss of a
component Of complement (factor B-properdin) that opsonizes certain bacteria.
Primary peritonitis is the most frequent infection without neglecting pulmonary,
cutaneous, urinary, meningoencephalitis and sepsis infections. Streptococcus
pneumoniae is the most common causal agent but gram-negative and Haemophilus
bacteria may also be found. Between 15 and 50% of cultures of peritoneal fluid are
negative. The main symptoms of this complication are abdominal pain, fever, peritoneal
irritability, nausea and vomiting. The treatment regimens vary according to the
specialized hospital centers recommending penicillin associated with a aminoglycoside
or cephalosporin of third generation. Gorensek reported in a series of 214 nephrotic
cases, 62 episodes of peritonitis in 37 patients (17%) and in another series of 351
children followed for 10 years, 24 episodes of peritonitis were found in 19 patients (6%
of cases). In a third group of 399 children, 5.5% of cases of primary peritonitis were
reported. A retrospective study of 34 patients in the ISN showed that cellulitis,
peritonitis, UTI, pansinusitis, among others, showed that malnutrition played an
important role because the complications were strikingly more frequent in those with
untreated activity than those with Corticoterapia of which supposedly its immunological
state would be more compromised.

B. Thromboembolic disorders: Addis in 1948 described the association of

hypercoagulability and nephrotic syndrome. Kendall and colleagues found elevated
levels of fibrinogen, factors V, VII VIII and X, moderate thrombocytosis and accelerated
generation of thromboplastin in the nephrotic adult. Almagro and others, on the other
hand, studied 20 nephrotic children in relapse and with the exception of one case all
presented alterations of coagulation and platelet function. Hyperfibrinogenemia was the
most frequent finding and in 22% showed thrombocytosis. Thrombosis of the renal vein
is the most frequent thromboembolic complication in the nephrotic. Thrombosis of the
renal artery is very rare although there are reports of pulmonary thrombus in addition to
the femoral, mesenteric, axillary, ophthalmic, carotid and cerebral. 80% of nephrotic
patients have low levels of antithrombin III associated with the higher frequency of
thromboembolism. It also describes decreased alpha-2 antiplasmin and free S protein
in 50% of cases.

C. Hypovolemia: Classically it was suggested that NS is accompanied by hypovolemia,

however 50% of patients are euvolemic, 35% hypovolemic and 15% hypervolemic.
Nephrotic patients can also be classified into two groups: one vasoconstrictor with high
plasma renin activity, hypovolemia and elevated hematocrit, and the other with
hypervolemia, low hematocrit and suppression of renin activity.

D. Reversible Idiopathic Renal Impairment: A consequence of a significant decrease in

blood volume, bilateral renal vein thrombosis, pyelonephritis, severe HTA, or drug-
induced dysfunction. This clinical condition of renal failure seen in SNCM has been
evidenced in anasarca, massive proteinuria, and oliguria.

TREATMENT

General principles.

Diet: It is recommended rest, water restriction and a decortified, normoproteic and

hypercaloric diet. There are reports that an additional dietary protein intake does not
significantly increase plasma albumin concentration. Low-protein diets decrease
albuminuria but have a high risk of malnutrition. In case of hypocalcaemia add calcium
carbonate at doses of 50-100 mg / kg / d.

Antibiotics: Any potential or obvious infectious site needs to be investigated. Consider

peritonitis, sepsis and cellulitis due to streptococcal or staphylococcal infections.
Prophylactic use of oral penicillin is recommended in all children with severe ascites.
The possibility of otorhinolaryngological, dermal problems, dental caries, UTI and
intestinal parasitosis should also be considered.

Diuretics: Diuretics may be used in addition to salt restriction. Furosemide (1-5 mg /

kg / d) or hydrochlorothiazide (2-5 mg / kg / d) possibly combined with a potassium
saver such as spironolactone (1-2 mg / kg / d) or amiloride (0.3 -0.5 mg / kg / d). There
is experience of the use of mannitol in refractory edema.

Albumin infusions: Management includes human albumin 0.5 to 1 g / kg or plasma 10

mL / kg in 2 hours in conjunction with furosemide at 3 mg / kg / d. Caution is related to
volume overload and precipitating acute pulmonary edema.

Blood pressure: The child with NS is usually normotensive. Hypertension may reflect
hypervolemia or excessive vasoconstrictor response in response to hypovolemia. HBP
may also be suggestive of glomerulonephritis.

Pharmacological Treatment (Protocol Service Nephrology ISN) .-

It is typified according to the response to steroids and the histological pattern

FORECAST
Follow-up in children with NS has been extensively studied. It is recommended at high
weekly controls that includes weight, height, BP and determination of proteinuria in 24-
hour urine, protein / creatinine ratio or sulfosalicylic acid -ASS-, and can be performed
every 15 days. Follow-up should be completed with biochemical parameters such as
urea, creatinine, calcium, albumin, and cholesterol. In the case of cyclophosphamide
and chlorambucil, a periodic leukocyte count should be performed. Consider all
infectious intercurrences and treat them. In many children, complete remission after
steroid therapy is also seen in addition to multiple exacerbations of the disease. More
than 70% of children have had one or more episodes of nephrotic syndrome. Changes
in podocytes rapidly revert to a normal appearance after cortico- therapy coinciding
with the disappearance of proteinuria.