A single pain can affect a whole system. The human body system works this way, although it is composed of different unit that independently performing different function, once a single division is smashed, the entire organization probably get inflicted. Being unaware of this, people often neglect to include the entire element functioning in their body. One of this component is the system involve in eliminating waste from our body which is the kidney. Disregarding it can cause problem to our health, therefore affecting our living.
Among the disorder that related to the kidney is nephritic syndrome. “The nephritic syndrome (NS) is a clinical entity characterized by massive loss of urinary protein (primarily albuminuria) leading to its result, edema” (Travis MD, 2005).all substance that passes in our body is filter in the kidney by its functional unit the nephron. One of these nutrients that pass the organ is protein which is the major components of our body, moreover when the level of protein drop, liquid seeps out in the capillaries and settles in the tissue resulting to the fluid retention and swelling. The fluid formed is what we termed as edema, which is the major manifestation of the syndrome. Other symptoms are hypertension due to its effect in the flow of blood; tract infection can also be a sign as well as abdominal tenderness. The syndrome is prone to all ages, male and female.
Nephrotic syndrome is cause by inflammation in the nephrons, and some environmental factors and other disorder such as drugs, cancers and diabetes and for this reason this syndrome can cause complications in the organization within the body.
It is diagnosed through, urine and blood test both examining the protein level in the blood and the laboratory test called biopsy where the small portion of kidney is taken for analysis.
“Glucocorticoid a steroid therapy” (Travis MD, 2005) is the known treatment for this disorder, a medication done to preserve the level of protein in the body. However restricted diet should be observe a low fat and salt but high protein food is advice. As an end, careful activity plan is suggested to prevent this syndrome thus achieving a healthy living.
SOURCE:
Author: Luther Travis, MD, William W Glauser Professor of Pediatrics and Pediatric Nephrology, Department of Pediatrics, Divisions of Nephrology and Diabetes, University of Texas Medical Branch and Children’s Hospital
Last Updated: April 14, 2005
Background: The term nephrosis, or nephrotic syndrome, had its origin in the early 20th century and was introduced primarily to distinguish it from nephritis, a label used to denote a clinical state associated with hematuria, proteinuria, and a cellular proliferation of the glomerulus. It describes a clinical condition of edema and proteinuria in which the renal histology (light microscopy) demonstrates fatty degeneration of the tubules associated with normal appearing glomeruli. Briefly, the name was modified to lipoid nephrosis after the routine finding of lipid droplets in the urine of affected patients.
The nephrotic syndrome (NS) is a clinical entity characterized by massive loss of urinary protein (primarily albuminuria) leading to hypoproteinemia (hypoalbuminemia) and its result, edema. Hyperlipidemia, hypercholesterolemia, and increased lipiduria are usually associated. Although not commonly thought of as part of the syndrome, hypertension, hematuria, and azotemia may occur. NS generally has a glomerular cause and is currently categorized into primary and secondary forms. The name primary NS (PNS) has replaced, in some circles, the older designation of idiopathic NS (INS), but both terms denote a similar same vagueness as to cause. Included are a variety of clinical as well as pathologic states. The term secondary NS relates to the clinical state associated with other, more clearly defined diseases such as anaphylactoid purpura, systemic lupus erythematosus, diabetes mellitus, sickle cell disease, syphilis, and others. In the following sections, the majority of attention will be devoted to PNS or INS because of the relative frequency in children.
The subcategories of PNS are based on histologic descriptions, but clinical-pathological correlations have been made. Even though knowledge of specific causes of NS are too limited for more precise classification, the variants of PNS/INS will be considered as clinical disease states with well-defined histopathologic processes. The histologic type at onset makes it possible to generalize about such things as response to therapy and ultimate prognosis. When possible, the authors use the definitions, descriptions, and nomenclature developed by the International Study of Kidney Diseases in Children (ISKDC). Most attention will be devoted to minimal change nephrotic syndrome (MCNS) and focal segmental glomerulosclerosis (FSGS) with only modest attention to familial or congenital nephrosis, membranoproliferative glomerulonephritis (MPGN), and membranous glomerulonephritis (MGN).
The overall prevalence of NS in childhood is approximately 2-5 cases per 100,000 children. The cumulative prevalence rate is approximately 15.5/100,000. MCNS is the most common form in children, and its prevalence is inversely proportional to the age at onset (ie, the younger the child, the more likely the histology will show minimal abnormalities on light microscopic evaluation of glomerular histology). Histologic variations exist within this category in which some patients demonstrate only fusion and smudging of the epithelial cell podocytes while others may demonstrate mild changes within the glomerular mesangium consisting of either proliferation or sclerosis. Since patients with MCNS have the highest rate of responsiveness to standard therapy and the best long-term prognosis, the separation of MCNS from others is important.
IgM mesangial nephropathy (IgM nephropathy) may be a separate entity from MCNS. Assignment of type of NS by histologic criteria is based predominately on light microscopic findings. Most patients with isolated IgM mesangial immunofluorescent staining present clinical characteristics similar to those with MCNS. Whether the finding of immune deposits of IgM alters either response to therapy or subsequent course is controversial.
Focal segmental glomerulosclerosis (FSGS) is the second most common histologic subtype seen in children and appears to be increasing in frequency. It is not a single disease entity, and attempts to portray it as a uniform entity has led to some confusion in the literature with regard to natural history. FSGS is always a histopathologic diagnosis, and its clinical presentation will vary according to the etiology or cause of the histologic lesion. FSGS may manifest in a fashion that is indistinguishable from MCNS, but it may also be found only after years of clinical nephrotic syndrome when earlier biopsies have been interpreted as MCNS. FSGS is a known consequence of hyperfiltration and is regularly seen in patients with reflux nephropathy and in some patients with a single kidney whose other has been lost because of conditions such as multicystic dysplastic kidney disease.
Membranoproliferative glomerulonephritis (MPGN) may manifest as nephrotic syndrome, particularly in older children and adolescents. Its clinical picture is more closely associated with a nephritic picture, but on occasion it may appear similar to MCNS or FSGS. Membranous glomerulonephritis (MGN) accounts for less than 1% of the cases of NS in childhood and adolescence and is often associated with hepatitis or other viral disease. Congenital nephrotic syndrome becomes a consideration when nephrosis appears during the first year of life and particularly in those instances in which the clinical syndrome starts in the first few months.
Pathophysiology: Heavy proteinuria (albuminuria) is the hallmark of this condition and the primary abnormality in NS. The degree of proteinuria varies considerably from one child to another. Some children will excrete as much as 15 g/m2/24 hours, and the minimal excretion compatible with the diagnosis is around 1 g/m2/24 hours (approximately 40 mg/m2/hour).
The initiating event that produces proteinuria remains unknown. PNS is believed to have an immune pathogenesis, but the precise nature of the process has yet to be defined. That PNS is related to lymphocyte dysfunction has been suggested, and various studies lend credence to this hypothesis. A highly cationic plasma protein that may neutralize the anionic charge on the glomerular capillary wall has been described in nephrotic children. Other investigators have noted a decrease in immune responsiveness and related this to alterations in either T-lymphocyte number and/or function. The presence of suppresser cytokines or lymphokines has been postulated, and various investigators have shown changes in interleukin-8, interferon-g, IGF-1, TGF-a, and vascular permeability factor (VPF). The role of the kinin system is also under investigation, because urinary excretion of kinins is increased during exacerbations of the disease. More recently, alterations in ceratin molecules expressed in the epithelial cell podocyte,especially nephrin, podocin, and a-actin, have been shown to have a role in the pathogenesis of the proteinuria. Other researchers have not felt nephrin to be involved in children with MCNS. The rate of apoptosis in circulating T-lymphocytes has been found to be increased, and a role for reduced antioxidant defense has been postulated. Despite the regular finding of elevated levels of IgE and an association with atopy in steroid-responsive NS, current data merely suggest a common immune activation rather than a direct association. Leptin is now being investigated for its role in the pathogenesis since, in MCNS, serum levels are low at onset of the disease and are associated with elevated serum levels of TGF-b1. Additionally, some evidence still exists that genetic factors may be involved in the pathogenesis.
In PNS, the glomerular capillary permeability to albumin is selectively increased, and this increase in filtered load overcomes the modest ability of the tubules to reabsorb protein. This selective proteinuria (as seen in MCNS) is quite different than the more unselective proteinuria observed in cases of glomerulonephritis. Part of this increase in albumin excretion may be because of the smaller size of the albumin molecule, but since the excretion of some even smaller weight plasma proteins is not proportionally increased, the presence of other factors is obvious. At least 2 hypotheses are proposed to account for this increased permeability. The traditional hypothesis relates to changes in the anionic composition of the glomerular basement membrane (GBM). In the normal state, the endothelial side of the glomerular capillary is negatively charged because of the presence of a variety of polyanions along this surface. Thus, the negatively-charged protein, albumin, is less likely to be filtered.
In experimental nephrosis and in some children with primary NS, studies have demonstrated a decrease in the normal content of sialic acid (a polyanion) from the basement membrane. While such has not been confirmed by all investigators, this deficiency may allow for an increased transport of anionic plasma components. In such a state, permeability of the glomerular basement membrane would be selectively altered, increasing capillary transport of anionically charged particles such as albumin.
An alternative proposal to explain the heavy proteinuria invokes a primary role for the epithelial cell podocytes. Flattening, retraction, and effacement of the podocyte foot processes is a constant feature of heavy proteinuria. In the traditional viewpoint, these changes are considered as consequences of the proteinuria. Other investigators believe that primary distortions of the slit diaphragm filaments are present and that a redistribution of nephrin from the podocyte slit pores into the cytoplasm.
Hypoalbuminemia is the result of the increased urinary loss of protein. Other factors, however, may contribute to the hypoalbuminemia, among them decreased synthesis, increased catabolism, and increased gastrointestinal losses. Even though most studies have shown that the albumin synthesis rate is not decreased, the capacity to increase hepatic production appears insufficient to compensate for the large urinary losses.
Edema appears to be the natural consequence of the hypoalbuminemia. The classic explanation for edema formation is a decrease in plasma oncotic pressure (as a consequence of low serum albumin) causing an extravasation of plasma water into the interstitial space. The resulting contraction in plasma volume would theoretically lead to a decrease in renal perfusion and hence to stimulation of the renin-angiotensin system. This hormonal effect coupled with an increase in the synthesis and secretion of antidiuretic hormone (related to the decrease in effective plasma volume) would lead to an increase in renal tubular reabsorption of sodium and water. The net result of the combination of Starling forces, reduction in renal perfusion (GFR), and increased hormonal activity would be avid reabsorption of both sodium and water, leading to either maintenance or furthering of the edema.
While the above hypothesis on the pathogenesis of edema is attractive, certain experimental data do not completely support this traditional concept. First, the plasma volume (PV) has not always been found to be decreased and, in fact, in most adults, measurements of PV have shown it to be increased. Only in young children with MCNS have most (but not all) studies demonstrated a reduced PV. Additionally, most studies have failed to document elevated levels of renin, angiotensin, or aldosterone, even during times of avid sodium retention. Active sodium reabsorption also continues despite actions that should suppress renin effects (ie, albumin infusion, ACEI administration). Coupled with these discrepancies is the fact that, in the steroid-responsive nephrotic, diuresis usually begins before plasma albumin has significantly increased and before plasma oncotic pressure has changed. Some investigators have demonstrated a blunted responsiveness to atrial natriuretic peptide (ANP) despite higher than normal circulating plasma levels of ANP.
Thus, that the precise cause of the edema and its persistence is uncertain should be apparent. A complex interplay of a variety of physiologic factors (ie, decreased oncotic pressure, increased activity of aldosterone and vasopressin, diminished atrial natriuretic hormone, activities of various cytokines and physical factors within the vasa recti) probably contribute to the accumulation and maintenance of edema.
Sex: In children younger than 8 years at onset, the ratio of males to females varies from 2:1 to 3:2 in various studies. In older children, adolescents and adults, the male to female prevalence is approximately equal. ISKDC data indicate that 66% of patients with either MCNS or FSGS are male, whereas, for MPGN, 65% are female.
History:
Regardless of the type of NS (the histopathologic type), the major clinical manifestation is edema, which is the presenting symptom in about 95% of children. The edema in the early phase is intermittent and insidious; even its very presence may not be appreciated. It usually appears first in areas of low tissue resistance (ie, periorbital, scrotal, and labial regions) and may progress either rapidly or quite slowly. Ultimately, it becomes generalized and can be massive (anasarca). It is typically dependent in nature, more noticeable in the face in the morning (upon arising) and predominately in lower extremities later in the day. It is pitting in nature. In cases with marked edema, the skin may ooze clear fluid and appear thinner than usual.
An occasional child with NS will present with gross hematuria. The frequency of macrohematuria depends on the histologic subtype of NS. It is more common in those patients with MPGN than in other causes, but its frequency in MCNS has been reported to be as high as 3-4% of cases. Statistically, a higher percentage of patients with FSGS have microhematuria than those with MCNS, but this is not helpful in differentiating between types of NS in the individual patient. Oliguria is a common occurrence whatever the etiology.
Regardless of the type of NS, anorexia, irritability, fatigue, abdominal discomfort, and diarrhea are common. If ascites is marked, respiratory distress is not uncommon. An occasional child will present with fever and septic picture; the peritoneum is often the site of the infection. Streptococcus pneumoniae is the most frequent organism responsible for peritonitis in this population, but Staphylococcus aureus and Escherichia coli are commonly recovered. Symptoms of a urinary tract infection are occasionally present.
A history of a respiratory tract infection immediately preceding the first clinical signs of the disease is frequent, but the relevance to causation is uncertain. A history of prior allergic events is common, and atopy has been reported in approximately 40-50% of children with MCNS. A hypersensitivity event (ie, insect sting, ant bites, poison ivy, immunizations, etc.) has clearly preceded the onset in some cases and may be considered etiologically significant. The peak incidence of NS appears to have a seasonal variation. A few children have been reported with major food allergies, and, in some, ultimate remission has been associated only with dietary elimination programs.
Hematuria
Physical: The most common clinical finding of edema is present in more than 95% of cases. The degree is usually greatest in those patients with MCNS. It may be mild and localized only to those areas where tissue resistance is low (eg, periorbital area, scrotum, labia). Generalized edema is common and is dependent and pitting in character. Ascites is common, and anasarca may be present. In those children with marked ascites, there may be mechanical restriction to breathing and the child may manifest compensatory tachypnea. The child usually demonstrates a pallor that is greater than laboratory evidence of anemia would suggest.
Hypertension may be present; ISKDC studies demonstrated that approximately 30% of patients with MCNS have both systolic and diastolic pressures above the 90th percentile for age. When values above the 98th percentile were used to denote an abnormality, then approximately 20% had systolic pressures that were elevated and about 13% of the diastolic pressures were aberrant. The percentage of children with nephrosis who exhibit hypertension is higher with other subsets such as children with FSGS and, particularly so, in patients with MPGN in which hypertension may be severe.
Other consistent abnormalities of the physical state are unusual. Signs of a concurrent upper respiratory tract infection may be present, and some children will have overt evidence of an atopic state with varying degrees of eczema. An occasional child will show evidence of an insect sting or bite. Abdominal tenderness is unusual in the absence of a peritoneal infection.
On rare occasions, the child with nephrosis may demonstrate signs of either an arterial or venous thrombosis near the onset of disease. These thromboses, which may involve either the extremities or internal vessels, occur secondary to the hypercoagulable state of such patients. Early recognition is essential if the organ involved is to be salvaged.
Causes:
Edema, the predominant clinical feature, is ultimately the result of the urinary loss of large amounts of albumin from the serum with a consequent lowering of the serum albumin concentration.
The cause for the maintenance and progression of the edema is less certain (see Pathophysiology).
The GFR is often reduced by a mild-to-moderate degree, and the ability of the renal tubules to aggressively reabsorb sodium and water is enhanced. Oliguria and edema ensue.
The edema first collects in those sites where tissue resistance is low, such as the periorbital area and in the scrotum.
Later, the edema becomes generalized.
The etiology of the hypertension is probably multifactorial.
In some patients with elevated blood pressure (BP), particularly in small children with MCNS, the PV is low, and the associated tachycardia suggests an increase in sympathetic nervous system activity. In such patients, the BP falls following an infusion of albumin.
In most older patients with NS, the PV is either normal or increased. In some of these patients, BP returns to normal with diuresis.
Plasma renin levels have been reported variously as either normal or slightly increased; however, the response to blockade of the renin-angiotensin system does not support this as the primary cause of the hypertension.
Various cytokines known to have pressor effects are increased and may be the primary cause of hypertension.
Other Problems to be Considered:
An approach to the differential diagnosis should be looked upon as a 2-step process. First is the question of what other conditions are likely to manifest similarly (ie, edema). Allergic reactions are probably the most commonly confused condition, and how often the child with new onset nephrosis is initially felt to have some form of allergic reaction as a cause for the periorbital edema is striking. Other conditions that may produce facial or generalized edema in children of this age group include congenital heart disease and congestive heart failure, severe hepatic disease associated with hypoalbuminemia, protein-losing enteropathy (infants with cystic fibrosis, particularly), and congenital defects in albumin synthesis.
The key to determining that renal disease is responsible for the initial clinical presentation is an examination of the urine for protein and cellular elements. Most patients with MCNS have proteinuria without hematuria, but the presence of microhematuria does not eliminate this diagnosis from consideration. The proteinuria is predominately selective with a high ratio of albumin to globulins. The proteinuria from patients with FSGS is less selective but is still more so than that from patients with MPGN. In the latter disease, hematuria is routinely present.
Lab Studies:
Procedures:
A renal biopsy is usually not performed until after a therapeutic trial of glucocorticoids has proved to be unsuccessful. Exceptions to this general rule may be made in the following situations:
A child older than 10 years at onset of NS
Coexistence of significant hematuria, hypertension, and azotemia at the onset of NS
Any child in whom the levels of serum complement (or C3) are depressed
Histologic Findings: If a histologic assessment is made, the findings discovered are, obviously, dependent on the subtype of NS. These will not be discussed in any detail here, and the interested reader is referred to other sources for this. Briefly, the findings are summarized below.
Minimal-change nephrotic syndrome (MCNS) indicates glomerular morphology that on light microscopic examination is little different from normal. There may be minimal mesangial alterations, but immunoglobulins are usually absent, and no deposits are observed on electron microscopy. The only significant change seen on electron microscopic examination is flattening and fusion of the epithelial cell podocytes.
Focal global glomerulosclerosis (FGGS) describes a globally sclerotic glomerulus occurring in focal areas with remaining glomeruli being normal. The precise meaning of such a lesion is not always certain since normal glomerular attrition occurs by global sclerosis. For this reason, a normal variant is usually considered to be if <5% of the glomeruli are globally sclerotic.
Focal segmental glomerulosclerosis (FSGS) describes a lesion in which some glomeruli are involved with segmental sclerosis (one lobule or section within a glomerulus), with the remaining glomeruli being normal. Because this lesion is focal and is often confined to the juxtamedullary nephrons, it may be overlooked on renal biopsy examination. Immunofluorescent microscopy yields a variable picture. In some patients, all classes of immunoglobulins and complement appear to be trapped in the sclerotic area; in others, distinct immune-complex-type, particularly IgM, deposits are found.
Mesangial proliferative glomerulonephritis (MPN) has only recently been distinguished from MCNS, and some examples of MCNS with mesangial alterations may be inappropriate. Light microscopy reveals minimal to moderate proliferation of the mesangial cells, with some mesangial expansion, but the most striking change is observed with immunofluorescent microscopy, in which IgM, IgG, and C3 are often seen.
Membranoproliferative glomerulonephritis (MPGN), or mesangiocapillary glomerulonephritis, has a distinctive histologic picture; all glomeruli are involved. Three varieties of MPGN can be described, both with proliferation of cells and extensive immune deposits as demonstrated by immunofluorescent and electron microscopy.
In membranous glomerulonephritis (MGN), with well-developed lesions, findings on light microscopic examination are typical, but detection of early lesions requires the detail afforded by immunofluorescent and electron microscopy.
Other lesions, including proliferative glomerulonephritis and chronic glomerulonephritis, occur in <5% of children with NS, and the histologic picture varies with the specific etiology
Medical Care: The following discussion assumes that all attempts have been made to exclude MPGN and secondary causes of NS. An underlying assumption is that the most likely diagnosis is MCNS or a similar subtype in which reasonable expectations exist that the disease will be responsive to glucocorticoid therapy. Treatment that appears specific (steroids) and treatment that is nonspecific (observation for infections, diet, diuretics, antihypertensives) is discussed here. In addition, only initial treatment is considered here; treatment of nonresponders and those who relapse is discussed later.
Specific therapy
Initial management
Glucocorticoid (steroid) therapy has so changed the morbidity and mortality of NS as to make it almost specific and is considered standard. Even though differences in the beliefs among pediatric nephrologists about the specific details of steroid management exist, the feelings are uniform that this is the primary agent of choice.
After contraindications to the use of high-dose glucocorticoid drugs are eliminated (ie, usually considered to be exclusion of an active infection), oral prednisone or prednisolone is started in a dosage of 2 mg/kg/day (60 mg/m2/d).The total daily dose is usually split into two doses and given daily for 4-8 weeks. Higher dosages or longer courses of daily steroids do not significantly change the response rate in MCNS, but they do increase steroid toxicity. Approximately 90% of patients with MCNS respond to this therapy with complete clearing of proteinuria, but only about 20% of children with FSGS and <5% of those with MPGN experience a clinical remission (defined as a diuresis without complete clearing of proteinuria). The majority of children with MCNS will respond between the 10th and 14th days of such therapy, but a full course of at least 4 weeks of daily therapy is still recommended. Children who do not respond (ie, complete clearing of proteinuria) should be referred to a pediatric nephrologistfor percutaneous renal biopsy and consideration be given to an alternative plan of treatment.
Maintenance therapy
Maintenance glucocorticoid therapy is still controversial, not about the need for some period of continuing prednisone but about the duration that such should be given. Following the 4-8 weeks of daily therapy, the standard recommendation has been for the dose (of either prednisone or prednisolone) to be reduced to ~1.5 mg/kg/day (40 mg/m2/d) given as a single dose every other morning for a 4-week period. Based on the author’s unpublished experience and other data, such a brief period of maintenance therapy is associated with a higher rate of early recurrence than in those patients in whom the maintenance therapy is continued for between 3-6 months.
Some investigators have accentuated the steroid effectiveness without increasing the side effects by giving intravenous pulse doses of methylprednisolone. A recent assessment of the Cochrane Database comparing 2 months of steroid therapy with 3 or more months concluded that “the longer duration significantly reduced the risk of relapse at 12-24 months without an increase in adverse events.” They subsequently concluded that “…daily prednisone for 4 weeks followed by alternate day therapy for 6 months would be expected to reduce the number of children experiencing a relapse by about 33%.” A recent meta-analysis of several controlled studies tends to support this contention.
Thus, the author’s recommendation for management is as follows:
4 weeks: intensive (daily) treatment-as above
8 weeks: 1.5 mg/kg/d (one dose every other morning)
8 weeks: 1.0 mg/kg/d (one dose every other morning)
8 weeks: 0.5 mg/kg/d (one dose every other morning)
Therapy stopped
Nonspecific therapy
Observation for infection: The child with nephrotic syndrome is a prime candidate for infection, and the potential for dissemination is increased if steroids are administered indiscriminately. Thus, the child who is febrile or has evidence of infection should be observed closely for a brief period while appropriate studies are performed. A child from an environment conducive to tuberculosis should be tested. Documented infections should be actively treated, but prophylactic therapy is not usually indicated. Pneumococcal vaccine is indicated in all patients after remission is obtained.
Diuretic therapy may be beneficial, particularly in children with symptomatic edema. The loop diuretics (furosemide) given orally in usual amounts (~1-2 mg/kg/d) are safe and moderately effective; their administration, however, should be handled with care because plasma volume contraction may already be present, and hypovolemic shock has been observed with overly aggressive therapy. If the edema is sufficiently intense that intravenous diuretic therapy seems indicated, then salt-poor albumin should be infused (usually at 1 gram/kg body weight given IV over 2-4 hours) simultaneously. Diuretics other than loop diuretics (eg, thiazides, spironolactone, metolazone) are generally not potent enough alone to effect diuresis but may give an added effect when combined with furosemide. Metolazone (with or without spironolactone) may be beneficial in combination with furosemide for resistant edema. Patients must be monitored carefully on this regimen. If the child is sent home on diuretic therapy, the
family must have clear guidelines about discontinuing therapy when edema is no longer present.
Antihypertensive therapy should be given when hypertension is present and particularly if it persists, but caution should be exercised. In some patients the hypertension will respond to diuretics. Angiotensin-converting enzyme inhibitors (ACEI) or angiotensin II receptor antagonist (A2RA) agents are the preferred therapy even though calcium channel blocking agents have been used effectively in short term therapy. In those patients in whom significant proteinuria persists, therapy with ACEI or A2RA are preferred for their antiproteinuric effect.
Ambulatory monitoring of the child’s condition and response to management is a very important aspect of overall management. We have found that the best outcomes occur in children in whom the parents keep an ongoing log (journal, diary, ledger) of their child’s treatment and progress. It seems best to initiate this at the beginning of outpatient care. The child’s clinical state (ie, presence, absence, degree of edema; blood pressure; illnesses), urine protein results, and treatments can be entered in the log.
Home monitoring of urine protein/albumin is an important aspect of management. All patients and/or parents should be trained to monitor random urine proteins at home by the most simple and inexpensive test available. This is most often accomplished with urine dipsticks but the use of turbidity tests (ie, sulfosalicylic acid) may also be used. The author recommends that the urine be tested once each morning and the results recorded in the log. This becomes particularly helpful after the urine has become free of protein (during maintenance therapy and beyond) since it may give clues about recurrences of the disease before edema occurs, thus allowing earlier initiation of treatment.
Surgical Care: No routine surgical care exists for this condition. On occasion, a patient with NS either presents with or develops clinical signs of an acute surgical abdomen, which is frequently due to peritonitis. The diagnosis can usually be made clinically and confirmed by bacteriologic examination of the peritoneal fluid aspirate. The organism most often responsible for the peritonitis is pneumococcus; however, enteric bacteria may also cause peritonitis. Treatment is medical.
Consultations: In most instances, NS is a chronic problem that requires understanding of the pathophysiology and knowledge of treatment options. For these reasons, consultation with a pediatric nephrologist is appropriate for all patients with NS. Referral to a pediatric nephrologist is mandatory for all children with NS whose symptoms fail to respond to initial therapy (ie, complete clearing of proteinuria); in most of these patients, a percutaneous renal biopsy is indicated, and an alternative treatment plan may be desirable.
Diet: Diet & fluids: The kidneys of children with active NS exhibit the usual tubular mechanisms for sodium conservation and total body sodium is uniformly increased. With the addition of prednisone therapy, renal sodium excretion is further curtailed. Because free access to salt is known to increase edema, dietary salt intake should be restricted. Limitation of water or fluid intake has not been a part of the usual therapeutic plan, even though, on occasion, a child’s thirst may be so stimulated that intake is excessive; if so, moderate restriction may be beneficial. The remainder of the diet should be normal. Alterations in protein intake are not indicated.
Activity: A normal activity plan is recommended. Since viral respiratory illnesses are usually responsible for initiating exacerbations of NS, it may be beneficial, if possible, to keep the child away from those who have obvious respiratory tract infections. Limiting exposure to large groups of children with potential infections seems to decrease the number of exacerbations. Do not restrict activity unless the child is severely edematous.
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