HEALTHY LIFE STYLE

Causes:
Drugs and malignancies most often are implicated as the etiology in adults and the elderly.
Pediatric cases are related more often to infections than to malignancy or a reaction to a drug.
A medication such as sulfa, phenytoin, or penicillin had previously been prescribed to more than two thirds of all patients with SJS.
More than half of the patients with SJS report a recent upper respiratory tract infection.
The 4 etiologic categories are (1) infectious, (2) drug-induced, (3) malignancy-related, and (4) idiopathic.
Infectious diseases that have been reported include herpes simplex virus (HSV), influenza, mumps, cat-scratch fever, mycoplasmal infection, lymphogranuloma venereum (LGV), histoplasmosis, and cholera.
In children, Epstein-Barr virus and enteroviruses have been identified.
Drug etiologies include penicillins, sulfas, phenytoin (and related anticonvulsants), carbamazepine, and barbiturates. In late 2002, the US Food and Drug Administration (FDA) and the manufacturer Pharmacia noted that SJS had been reported in patients taking the cyclooxygenase-2 (COX-2) inhibitor valdecoxib.
Various carcinomas and lymphomas have been associated.
SJS is idiopathic in 25-50% of cases.

DIFFERENTIALS :
Burns, Chemical
Burns, Ocular
Burns, Thermal
Dermatitis, Exfoliative
Erythema Multiforme
Staphylococcal Scalded Skin Syndrome
Toxic Epidermal Necrolysis
Toxic Shock Syndrome
Other Problems to be Considered:
Acute generalized exanthematic pustulosis
Pemphigus

WORKUP :
Lab Studies:
No laboratory studies (other than biopsy) exist that can aid the physician in establishing the diagnosis.
A complete blood count (CBC) may reveal a normal white blood cell (WBC) count or a nonspecific leukocytosis. A severely elevated WBC count indicates the possibility of a superimposed bacterial infection.
Determine renal function and evaluate urine for blood.
Electrolytes and other chemistries may be needed to help manage related problems.
Cultures of blood, urine, and wounds are indicated when an infection is clinically suspected.

Imaging Studies:
Chest radiograph may indicate the existence of a pneumonitis when clinically suspected. Otherwise, routine plain films are not indicated.

Other Tests:
Skin biopsy is the definitive diagnostic study but is not an ED procedure.
Skin biopsy demonstrates that the bullae are subepidermal.
Epidermal cell necrosis may be noted.
Perivascular areas are infiltrated with lymphocytes.

TREATMENT :
Prehospital Care: Paramedics should recognize the presence of severe fluid loss and should treat patients with SJS as they would patients with thermal burns.
Emergency Department Care: Most cases present early and prior to obvious signs of hemodynamic compromise. Perhaps the single most important role for the ED physician is to detect SJS early and initiate the appropriate ED and inpatient management.
Care in the ED must be directed to fluid replacement and electrolyte correction.
Skin lesions are treated as burns.
Patients with SJS then should be treated with special attention to airway and hemodynamic stability, fluid status, wound/burn care, and pain control.
Treatment of SJS is primarily supportive and symptomatic.
Manage oral lesions with mouthwashes.
Topical anesthetics are useful in reducing pain and allowing the patient to take in fluids.
Areas of denuded skin must be covered with compresses of saline or Burow solution.
Underlying diseases and secondary infections must be identified and treated. Offending drugs must be stopped.
The use of systemic steroids is controversial. Some authors believe that they are contraindicated. Treatment with systemic steroids has been associated with an increased incidence of complications.
Address tetanus prophylaxis.

Consultations:
Consultants may help establish the diagnosis and direct inpatient care. A dermatologist is the most likely clinician to establish the diagnosis, with or without biopsy.
Severe cases may require the involvement of a burn specialist or plastic surgery specialist.
Internal medicine, critical care, or pediatrics consultants direct inpatient care.
Ophthalmology consultation is mandatory for those with ocular involvement.
Depending on organ system involvement, consultations with gastroenterology, pulmonary, and nephrology may be helpful.

MEDICATION :
No specific drug treatment exists for SJS. The choice of antibiotic depends on the associated infection. The use of systemic corticosteroids is controversial. They are useful in high doses early in the reaction, but morbidity and mortality actually may increase in association with corticosteroid use.

FOLLOW-UP :
Further Inpatient Care:
Saline compresses may be applied to the eyelids, lips, and nose.
Careful daily inspection is necessary to monitor for secondary superinfections.
Prophylactic systemic antibiotics are not useful, especially in the current era of multiple-drug resistance.
Antimicrobials are indicated in cases of urinary tract or cutaneous infections, either of which may lead to bacteremia.

Further Outpatient Care:
Although patients with erythema multiforme minor may be treated as outpatients with topical steroids, those with erythema multiforme major (ie, SJS) must be hospitalized.
Cases of erythema multiforme minor must be followed closely. Some authors recommend daily follow-up.

Transfer:
Patients with SJS are often critically ill; therefore, they must be admitted to hospitals capable of delivering critical care.
Some patients may require the services of a burn unit.
Transfer criteria would be the same as for patients with thermal burns.

Deterrence/Prevention:
Patients must avoid any future exposure to agent(s) implicated in the occurrence of SJS. Recurrences are possible.

Complications:
Ophthalmologic - Corneal ulceration, anterior uveitis, panophthalmitis, blindness
Gastroenterologic - Esophageal strictures
Genitourinary - Renal tubular necrosis, renal failure, penile scarring, vaginal stenosis
Pulmonary - Tracheobronchial shedding with resultant respiratory failure
Cutaneous - Scarring and cosmetic deformity, recurrences of infection through slow-healing ulcerations

Prognosis:
Individual lesions typically should heal within 1-2 weeks, unless secondary infection occurs. The majority of patients recover without sequelae.
Development of serious sequelae, such as respiratory failure, renal failure, and blindness, determines prognosis in those affected.
Up to 15% of all patients with SJS die as a result of the condition.

MISCELLANEOUS:
Medical/Legal Pitfalls:
The gravity of the diagnosis must be recognized. Because patients with SJS who present early in the development of the disease may not yet be critically ill, the clinician may misdiagnose and discharge. SJS should be considered in all patients with target lesions and mucous membrane involvement.
Provide close follow-up and clear instructions.
When discharging a patient home, clearly document the degree (%) of skin involvement, the absence of mucous membrane lesions, and any clinical signs of toxicity.

BIBLIOGRAPHY:
Ball R, Ball LK, Wise RP, et al: Stevens-Johnson syndrome and toxic epidermal necrolysis after vaccination: reports to the vaccine adverse event reporting system. Pediatr Infect Dis J 2001 Feb; 20(2): 219-23[Medline].
Bianchine JR, Macaraeg PV Jr, Lasagna L, et al: Drugs as etiologic factors in the Stevens-Johnson syndrome. Am J Med 1968 Mar; 44(3): 390-405[Medline].
Brett AS, Philips D, Lynn AW: Intravenous immunoglobulin therapy for Stevens-Johnson syndrome. South Med J 2001 Mar; 94(3): 342-3[Medline].
Cohen B: The many faces of erythema multiforme. Contemp Pediatr 1994; 11: 19-39.
Cunha BA: Antibiotic side effects. Med Clin North Am 2001 Jan; 85(1): 149-85[Medline].
Darmstadt GL, Lane A: Vesiculobullous disorders. In Nelson WE, et al, eds. Nelson Textbook Of Pediatrics. 15th ed. Philadelphia: WB Saunders; 1996:1850-2.
Frieden IJ: Hypersensitivity reactions. In Rudolph AM et al. Rudolph's Pediatrics. 20th ed. Stamford, Conn: Appleton & Lange; 1996:906-8.
Ginsburg CM: Stevens-Johnson syndrome in children. Pediatr Infect Dis 1982 May-Jun; 1(3): 155-8[Medline].
Hofbauer GF, Burg G, Nestle FO: Cocaine-related Stevens-Johnson syndrome. Dermatology 2000; 201(3): 258-60[Medline].
Hurwitz S: Erythema multiforme: a review of its characteristics, diagnostic criteria, and management. Pediatr Rev 1990 Jan; 11(7): 217-22[Medline].
Metry DW, Lahart CJ, Farmer KL, Hebert AA: Stevens-Johnson syndrome caused by the antiretroviral drug nevirapine. J Am Acad Dermatol 2001 Feb; 44(2 Suppl): 354-7[Medline].
Prais D, Grisuru-Soen G, Barzilai A, Amir J: Varicella zoster virus infection associated with erythema multiforme in children. Infection 2001 Jan-Feb; 29(1): 37-9[Medline].
Sane SP, Bhatt AD: Stevens-Johnson syndrome and toxic epidermal necrolysis-challenges of recognition and management. J Assoc Physicians India 2000 Oct; 48(10): 999-1003[Medline]
reference : web.infeksi.com

Stevens-Johnson Syndrome

Background:
Stevens-Johnson syndrome (SJS) is an immune-complex?mediated hypersensitivity complex that is a severe expression of erythema multiforme. It is now known also as erythema multiforme major. SJS typically involves the skin and the mucous membranes. While minor presentations may occur, significant involvement of oral, nasal, eye, vaginal, urethral, GI, and lower respiratory tract mucous membranes may develop in the course of the illness. GI and respiratory involvement may progress to necrosis. SJS is a serious systemic disorder with the potential for severe morbidity and even death.

Pathophysiology:
SJS is an immune-complex?mediated hypersensitivity disorder that may be caused by many drugs, viral infections, and malignancies. Cocaine recently has been added to the list of drugs capable of producing the syndrome. In up to half of cases, no specific etiology has been identified.
Frequency:
In the US: Cases tend to have a propensity for the early spring and winter.
Internationally: SJS occurs with a worldwide distribution similar in etiology and occurrence to that in the US.

Mortality/Morbidity:
Patients with severe SJS die in 3-15% of cases.
Lesions may continue to erupt in crops for as long as 2-3 weeks. Mucosal pseudomembrane formation may lead to mucosal scarring and loss of function of the involved organ system. Esophageal strictures may occur when extensive involvement of the esophagus exists. Mucosal shedding in the tracheobronchial tree may lead to respiratory failure.
Ocular sequelae may include corneal ulceration and anterior uveitis. Blindness may develop secondary to severe keratitis or panophthalmitis in 3-10% of patients. Vaginal stenosis and penile scarring have been reported. Renal complications are rare.
Race: A Caucasian predominance has been reported.
Sex: Male-to-female ratio is 2:1.
Age: Most patients are in the second to fourth decade of their lives; however, cases have been reported in children as young as 3 months.

CLINICAL :
History:
Typically, the disease process begins with a nonspecific upper respiratory tract infection.
This usually is part of a 1- to 14-day prodrome during which fever, sore throat, chills, headache, and malaise may be present.
Vomiting and diarrhea occasionally are noted as part of the prodrome.
Mucocutaneous lesions develop abruptly. Clusters of outbreaks last from 2-4 weeks. The lesions typically are nonpruritic.
A history of fever or localized worsening should suggest a superimposed infection; however, fever has been reported to occur in up to 85% of cases.
Involvement of oral and/or mucous membranes may be severe enough that patients may not be able to eat or drink.
Patients with genitourinary involvement may complain of dysuria or an inability to void.
A history of a previous outbreak of SJS or of erythema multiforme may be elicited. Recurrences may occur if the responsible agent is not eliminated or if the patient is reexposed.
Typical symptoms are as follows:
Cough productive of a thick purulent sputum
Headache
Malaise
Arthralgia

Physical:
The rash can begin as macules that develop into papules, vesicles, bullae, urticarial plaques, or confluent erythema.
The center of these lesions may be vesicular, purpuric, or necrotic.
The typical lesion has the appearance of a target. The target is considered pathognomonic.
Lesions may become bullous and later rupture, leaving denuded skin. The skin becomes susceptible to secondary infection.
Urticarial lesions typically are not pruritic.
Infection may be responsible for the scarring associated with morbidity.
Although lesions may occur anywhere, the palms, soles, dorsum of hands, and extensor surfaces are most commonly affected.
The rash may be confined to any one area of the body, most often the trunk.
Mucosal involvement may include erythema, edema, sloughing, blistering, ulceration, and necrosis.
The following signs may be noted on examination:
Fever
Orthostasis
Tachycardia
Hypotension
Altered level of consciousness
Epistaxis
Conjunctivitis
Corneal ulcerations
Erosive vulvovaginitis or balanitis
Seizures
Coma

reference : web.infeksi.com

From Wikipedia, the free encyclopedia

Hernia Classification and external resources
Frontal chest X-ray showing a hernia of Morgagni
ICD-10	K40-K46
ICD-9	550-553
MedlinePlus	000960
eMedicine	emerg/251 ped/2559
MeSH	D006547

A hernia is a protrusion of a tissue, structure, or part of an organ through the muscle tissue or the membrane by which it is normally contained. The hernia has three parts: the orifice through which it herniates, the hernial sac, and its contents.

Contents [hide] 1 Pathophysiology 2 Characteristics 3 Treatment 4 Individual hernias 4.1 Inguinal hernia 4.2 Femoral hernia 4.3 Umbilical hernia 4.4 Incisional hernia 4.5 Diaphragmatic hernia 4.6 Other abdominal/inguinal hernias 4.7 Other hernias 5 Complications 6 References 7 External links 7.1 Pictures

[edit] Pathophysiology

By far the most common hernias develop in the abdomen, when a weakness in the abdominal wall evolves into a localized hole, or "defect", through which adipose tissue, or abdominal organs covered with peritoneum, may protrude. Another common hernia involves the spinal discs and causes sciatica.

Hernias may or may not present either with pain at the site, a visible or palpable lump, or in some cases by more vague symptoms resulting from pressure on an organ which has become "stuck" in the hernia, sometimes leading to organ dysfunction. Fatty tissue usually enters a hernia first, but it may be followed by or accompanied by an organ.

Most of the time, hernias develop when pressure in the compartment of the residing organ is increased, and the boundary is weak or weakened.

• Weakening of containing membranes or muscles is usually congenital (which explains part of the tendency of hernias to run in families), and increases with age (for example, degeneration of the annulus fibrosus of the intervertebral disc), but it may be on the basis of other illnesses, such as Ehlers-Danlos syndrome or Marfan syndrome, stretching of muscles during pregnancy, losing weight in obese people, etc., or because of scars from previous surgery.
• Many conditions chronically increase intra-abdominal pressure, (pregnancy, ascites, COPD, dyschezia, benign prostatic hypertrophy) and hence abdominal hernias are very frequent. Increased intracranial pressure can cause parts of the brain to herniate through narrowed portions of the cranial cavity or through the foramen magnum. Increased pressure on the intervertebral discs, as produced by heavy lifting or lifting with improper technique, increases the risk of herniation.

[edit] Characteristics

Hernias can be classified according to their anatomical location:

Examples include:

• abdominal hernias
• diaphragmatic hernias and hiatal hernias (for example, paraesophageal hernia of the stomach)
• pelvic hernias, for example, obturator hernia
• anal hernias
• hernias of the nucleus pulposus of the intervertebral discs
• intracranial hernias
• Spigelian hernias ^[1]

Each of the above hernias may be characterised by several aspects:

• congenital or acquired: congenital hernias occur prenatally or in the first year(s) of life, and are caused by a congenital defect, whereas acquired hernias develop later on in life. However, this may be on the basis of a locus minoris resistentiae (Lat. place of least resistance) that is congenital, but only becomes symptomatic later in life, when degeneration and increased stress (for example, increased abdominal pressure from coughing in COPD) provoke the hernia.
• complete or incomplete: for example, the stomach may partially or completely herniate into the chest.
• internal or external: external ones herniate to the outside world, whereas internal hernias protrude from their normal compartment to another (for example, mesenteric hernias).
• intraparietal hernia: hernia that does not reach all the way to the subcutis, but only to the musculoaponeurotic layer. An example is a Spigelian hernia. Intraparietal hernias may produce less obvious bulging, and may be less easily detected on clinical examination.
• bilateral: in this case, simultaneous repair may be considered, sometimes even with a giant prosthetic reinforcement.
• irreducible (also known as incarcerated): the hernial contents cannot be returned to their normal site with simple manipulation.

If irreducible, hernias can develop several complications (hence, they can be complicated or uncomplicated):

• strangulation: pressure on the hernial contents may compromise blood supply (especially veins, with their low pressure, are sensitive, and venous congestion often results) and cause ischemia, and later necrosis and gangrene, which may become fatal.
• obstruction: for example, when a part of the bowel herniates, bowel contents can no longer pass the obstruction. This results in cramps, and later on vomiting, ileus, absence of flatus and absence of defecation.
• dysfunction: another complication arises when the herniated organ itself, or surrounding organs start dysfunctioning (for example, sliding hernia of the stomach causing heartburn, lumbar disc hernia causing sciatic nerve pain, etc.).

[edit] Treatment

It is generally advisable to repair hernias quickly in order to prevent complications such as organ dysfunction, gangrene, multiple organ dysfunction syndrome, and death. Most abdominal hernias can be surgically repaired, and recovery rarely requires long-term changes in lifestyle. Uncomplicated hernias are principally repaired by pushing back, or "reducing", the herniated tissue, and then mending the weakness in muscle tissue (an operation called herniorrhaphy). If complications have occurred, the surgeon will check the viability of the herniated organ, and resect it if necessary. Modern muscle reinforcement techniques involve synthetic materials (a mesh prosthesis) that avoid over-stretching of already weakened tissue (as in older, but still useful methods). The mesh is either placed over the defect (anterior repair) or more preferably under the defect (posterior repair). At times staples are used to keep the mesh in place. These mesh repair methods are often called "Tension Free" repairs because, unlike older traditional methods, muscle is not pulled together under tension. Evidence suggests that these Tension Free methods have the lowest percentage of recurrences and the fastest recovery period compared to older suture repair methods. Increasingly, some repairs are performed through laparoscopes.

Many patients are managed through day surgery centers, and are able to return to work within a week or two, while heavy (heavy Lifting? Running?) activities are prohibited for a longer period. Patients who have their hernias repaired with mesh often recover in a number of days. Surgical complications have been estimated to be up to 10%, but most of them can be easily addressed. They include surgical site infections, nerve and blood vessel injuries, injury to nearby organs, and hernia recurrence.

Generally, the use of external devices to maintain reduction of the hernia without repairing the underlying defect (such as hernia trusses, trunks, belts, etc.), is not advised. Exceptions are uncomplicated incisional hernias that arise shortly after the operation (should only be operated after a few months), or inoperable patients.

It is essential that the hernia not be further irritated by carrying out strenuous labour.