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Comprehensive Pediatrics Glossary

K

Kawasaki Disease

  • Definition: Acute, self-limited vasculitis of medium-sized arteries, predominantly affecting children <5 years.

  • Etiology: Unknown; likely immune-mediated, possibly triggered by infection in genetically susceptible children.

  • Clinical Features:

    • Fever >5 days, non-purulent conjunctivitis, mucosal changes (strawberry tongue, cracked lips).

    • Polymorphous rash, cervical lymphadenopathy, extremity changes (erythema, edema, desquamation).

  • Complications: Coronary artery aneurysms, myocarditis, arrhythmias.

  • Investigations: CBC (leukocytosis), ESR/CRP elevation, echocardiography (for coronary involvement).

  • Management: IV immunoglobulin (2 g/kg single infusion), high-dose aspirin during acute phase, cardiac monitoring.

Klinefelter Syndrome

  • Definition: Genetic disorder in males (47,XXY karyotype) causing hypogonadism and infertility.

  • Clinical Features: Tall stature, gynecomastia, small testes, sparse body hair, learning difficulties.

  • Investigations: Karyotype analysis, testosterone levels, semen analysis.

  • Management: Testosterone replacement therapy, educational support, fertility counseling.

  • Complications: Increased risk of metabolic syndrome, osteoporosis, autoimmune diseases, psychosocial challenges.

Klippel-Feil Syndrome

  • Definition: Congenital fusion of cervical vertebrae.

  • Clinical Features: Short neck, low hairline, limited neck mobility, scoliosis, sometimes associated with congenital heart defects or renal anomalies.

  • Investigations: Cervical spine X-ray, MRI for associated anomalies.

  • Management: Symptomatic: physical therapy, surgical correction for severe scoliosis or neurological compromise.

  • Complications: Cervical instability, neurological deficits, hearing loss.

Krabbe Disease

  • Definition: Rare autosomal recessive lysosomal storage disorder caused by deficiency of galactocerebrosidase.

  • Clinical Features: Early infantile onset: irritability, feeding difficulties, hypotonia, developmental regression, seizures.

  • Investigations: Enzyme assay, genetic testing, MRI showing demyelination.

  • Management: Supportive; hematopoietic stem cell transplantation may slow progression if done early.

  • Prognosis: Poor in untreated infants; progressive neurodegeneration.

Keratitis (Pediatric)

  • Definition: Inflammation of the cornea in children.

  • Etiology:

    • Infectious: bacterial (Pseudomonas), viral (HSV), fungal.

    • Non-infectious: trauma, contact lens-related, autoimmune.

  • Clinical Features: Pain, photophobia, redness, tearing, decreased vision.

  • Investigations: Slit-lamp examination, corneal scraping for culture.

  • Management: Topical antibiotics/antivirals, lubricants, avoid contact lens use, manage underlying cause.

  • Complications: Corneal scarring, vision loss if untreated.

Keratoconjunctivitis Sicca (Dry Eye)

  • Definition: Decreased tear production or excessive tear evaporation causing ocular surface dryness.

  • Etiology: Rare in children; associated with juvenile Sjögren syndrome or congenital lacrimal gland agenesis.

  • Clinical Features: Eye irritation, burning, photophobia, red eyes.

  • Investigations: Schirmer test, ocular surface staining.

  • Management: Artificial tears, lubricating ointments, immunosuppressants for autoimmune cases.

Kidney Diseases (Pediatric)

  • Nephrotic Syndrome: Heavy proteinuria, hypoalbuminemia, edema, hyperlipidemia; minimal change disease most common in children.

  • Nephritic Syndrome: Hematuria, hypertension, oliguria; post-infectious glomerulonephritis common.

  • Congenital Anomalies: CAKUT (renal agenesis, dysplasia, UPJ obstruction).

  • Inherited Disorders: Polycystic kidney disease, Alport syndrome.

  • Investigations: Urinalysis, renal function tests, ultrasound, renal biopsy if indicated.

  • Management: Etiology-specific: steroids for nephrotic syndrome, supportive care, surgery for congenital anomalies.

Keratoderma (Pediatric)

  • Definition: Thickening of the palms and soles.

  • Etiology:

    • Hereditary: epidermolytic, non-epidermolytic forms.

    • Acquired: nutritional deficiencies, infections.

  • Clinical Features: Yellowish, thickened plaques on palms and soles; may be painful or fissured.

  • Investigations: Clinical diagnosis, skin biopsy if unclear.

  • Management: Emollients, keratolytic agents, retinoids in severe cases.

Kyphosis (Pediatric)

  • Definition: Excessive posterior curvature of the thoracic spine.

  • Types:

    • Postural (physiologic, flexible)

    • Scheuermann disease (structural, rigid)

    • Congenital (vertebral malformations)

  • Clinical Features: Hunched back, back pain, reduced spinal mobility.

  • Investigations: Spine X-ray, MRI for congenital/neurological involvement.

  • Management: Postural kyphosis: physiotherapy.

    • Structural: bracing, surgery for severe deformities.

Kwashiorkor

  • Definition: Severe protein-energy malnutrition characterized by edema.

  • Epidemiology: Common in children 1–3 years in resource-limited settings.

  • Clinical Features: Edema (feet, legs), distended abdomen, hypoalbuminemia, hepatomegaly, irritability, hair changes (depigmentation).

  • Investigations: Serum albumin, electrolytes, anthropometric measurements (weight-for-age, weight-for-height).

  • Management:

    • Nutritional rehabilitation: gradual refeeding, high-protein diet, micronutrient supplementation.

    • Treat infections and complications.

  • Complications: High mortality if untreated; susceptibility to infections, hypoglycemia, electrolyte imbalances.

 

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