Acute Gastroenteritis in Sickle Cell Disease

Fever

• Fever is a temperature above 100.4°F (38°C).
• It's a normal response to various conditions, with infection being the most common.

Rash

• A rash is a skin change affecting color, appearance, or texture.
• It can be localized to one body part or affect the entire skin.

Rash Presentation

• Features of the rash: Characteristic features of the lesions, distribution, progression, timing of onset (relative to fever), and morphological changes (e.g., papules to vesicles).

Common Skin Lesions

• Macule: Non-palpable, circumscribed, flat lesion (<1 cm in diameter).
• Papule: Palpable, elevated lesion (<1 cm in diameter).
• Maculopapular: Combination of macular and papular lesions.
• Purpura: Non-blanching papules or macules.
• Vesicle: Fluid-filled, elevated skin lesion (<1 cm in diameter).
• Bulla: Fluid-filled, elevated skin lesion (>1 cm in diameter).
• Pustule: Pus-containing vesicle.

Other Skin Lesions

• Cyst
• Fissure
• Macule
• Nodule
• Papule
• Polyp
• Pustule
• Vesicle
• Wheal

History

• Important considerations include the patient's age, current season, travel history, geographic location, exposure to ill individuals or animals/insects, medications, and immunization history.

Physical Examination

• Distribution pattern: symmetrical versus asymmetrical rashes.
• Morphology: monomorphic versus pleomorphic.
• Configuration: linear, annular, grouped, and Koebner phenomenon (eruption in an area of local trauma).

Differential Diagnosis of Fever with Rash

• Maculopapular rash: Pathogens can include viral, bacterial, or other conditions.
• Diffuse erythroderma: Bacterial (scarlet fever, Toxic Shock Syndrome, Staph SSS), fungal (Candida albicans), other (Kawasaki syndrome).
• Urticarial rash: Viral (EBV, Hep B, HIV), bacterial (M. pneumoniae, Group A strep), other (drug reaction).
• Vesicular, pustular, bullous: Viral (HSV, VZV, Coxsackievirus), bacterial (Staph SSS, Staph bullous impetigo, Impetigo, strep-crusted impetigo), other (Toxic epidermal necrolysis, Steven-Johnson Syndrome, Rickettsial pox).
• Petechial-purpuric: Viral (atypical measles, congenital rubella, CMV, enterovirus, HIV, HF), bacterial (Sepsis, meningococcal, gonococcal, pneumococcal, Hib), or IE.
• Erythema Nodosum: Viral (EBV, HBV), bacterial (Group A Streptococcus, TB, yersinia), fungal (Coccidiomycosis, histoplasmosis), or other (Sarcoidosis).

Infectious Causes

• Measles: Blanching erythematous maculopapular rash, symptoms include fever, cough, coryza, conjunctivitis, Koplik spots. Diagnosis: measles IgM, detectable for 1 month after illness.
• Chickenpox: Small red papules progress to oval, non-umbilicated, teardrop vesicles on erythematous base, new crops for 3-4 days, starting on the trunk, followed by the head, face, and sometimes extremities. Diagnosis: PCR is current diagnostic method.
• Rubella: Rash resembles measles but patient is not ill looking, prominent postauricular, posterior cervical, and suboccipital adenopathy, Forschheimer spots on soft palate (in 20% of patients). Diagnosis: IgM antibodies diagnostic for congenital rubella syndrome.
• Scarlet fever: Exotoxin-mediated diffuse erythematous rash, pharyngitis due to group A Streptococcus, coarse, sandpaper-like rash, desquamation, circumoral pallor, and strawberry tongue. Diagnosis: Streptococcus present in throat swab.
• Dengue Fever: Tropical, mosquito-borne disease, symptoms include high fever, skin rash, eye and joint aches, backaches, headaches, loss of appetite, nausea, vomiting, and bleeding (nose, gums).
• Typhoid fever: Bacterial infection from Salmonella, common in unsanitary areas, persistent fever, rash with pink blanching spots on stomach or chest, headache, sore throat, tiredness, stomach pain, constipation, and diarrhea.
• Typhus: Rickettsial bacterial infection transmitted by arthropods, severe headache, high fever, rash, confusion, stupor, hypotension, sensitivity to bright lights, and severe muscle pain.

Inflammatory Causes – Kawasaki Vasculitis

• Usually in kids <4 years old.
• Symptoms include fever >5 days, bilateral conjunctival injection, injected, or fissured lips, injected pharynx or strawberry tongue, erythema of palms or soles, edema of hands or feet, generalized or periungual desquamation, rash, and cervical lymphadenopathy.

Systemic Lupus Erythematosus

• Autoimmune disease where the body's immune system attacks healthy tissues in many body parts.
• Symptoms include: pleuritis or pericarditis, discoid rash, malar rash, photosensitive rash, oral ulcers, arthritis, anemia, leukopenia, lymphopenia, thrombocytopenia, and renal nephritis.

Infectious Mononucleosis

• Caused by Epstein-Barr virus (EBV).
• Symptoms include fever, malaise, Tonsillopharyngitis, lymphadenopathy, petechiae on soft palate, and splenomegaly/hepatomegaly/maculopapular rash.

Dehydration

• Symptoms include abnormally sleepy, lethargic behavior, sunken eyes, irritability, no tears when crying, thirst, dry mouth and skin.
• Severe dehydration requires admission to a hospital for intravenous fluids.
• Mild to moderate dehydration can be managed with oral rehydration solutions (ORS).
• Evaluating skin turgor to determine degrees of dehydration.
• ORS should be given for hypernatremia cases within 12 hours and not 4.

Acute Gastroenteritis (AGE)

• Inflammation of the Gastrointestinal tract.
• Symptoms include diarrhea, fever, and vomiting.

Diarrhea

• Watery stools are more consistent with viral gastroenteritis.
• Stools with blood or mucous indicate a bacterial pathogen.
• A long duration of diarrhea (>14 days) might suggest a parasitic or non-infectious cause.

Diagnostic Considerations

• Lab studies (serum electrolytes, bicarbonate, glucose, blood urea and creatinine, urine specific gravity, and stool examination) are helpful with severe dehydration, not otherwise.
• Stool examination for pus, RBC or gross blood helps determine potential bacterial causes. Viral detection in stool is also considered, as is evidence of systemic infection (CBC, blood cultures, urine cultures, chest radiography, and/or LP).

Complications

• Dehydration
• Electrolyte Imbalance (including sodium)
• Seizures
• Secondary carbohydrate malabsorption
• Hemolytic uremic syndrome

Prevention

• Vaccination (RotaTeq & Rotarix)
• Probiotics
• Hand washing
• Clean food preparation and preservation

Anti-microbials

• Generally not recommended.
• Exceptions apply to C. difficile, Cholera, Giardia, and Cryptosporidium.

Anti-diarrheals

• Generally not recommended.
• Ondasetron is an exception and can be useful for children >6 months.

Probiotics

• Live microbial feeding supplements.
• Mechanisms include antimicrobial substance synthesis, nutrient competition, toxin modification, and nonspecific immune stimulation.
• Showed to be effective in reducing diarrhea duration, especially Lactobacillus GG, and preventing C. difficile-associated diarrhea in patients on antibiotics.

Zinc

• Zinc supplementation may reduce diarrhea duration in children older than 6 months in areas with zinc deficiency prevalent.
• WHO recommends zinc supplementation for children <5 years old with acute gastroenteritis.

Differential Diagnoses (Gastroenteritis)

• Infections outside the Gastrointestinal tract (eg, AOM, URI)
• Chronic nonspecific diarrhea of childhood (toddler diarrhea)
• Malabsorption syndromes
• Inflammatory Bowel Disease
• Pediatric Lactose Intolerance