Paediatrics Marrow Pg 221-230 (Peadiatric Rheumatology)
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Questions and Answers

What is the main characteristic of Stage 2 tumors in children?

  • Involves lymph nodes outside the abdomen.
  • Extends outside the kidney margins but is completely resectable. (correct)
  • Limited to the kidney and completely resectable.
  • Bilateral involvement of the kidneys.

    • Stage 4 treatment includes radical nephrectomy as the sole treatment option.

    False

    What is the primary treatment for Stage 5 solid tumors in children?

    Surgery, chemotherapy, and radiotherapy

    Stage 3 tumors may exhibit __________ lymph node metastasis.

    <p>regional</p> Signup and view all the answers

    Match the treatment stages with their corresponding treatments:

    <p>Stages 1-2 = Surgery + Chemotherapy: (Vincristine + Actinomycin D + Doxorubicin) Stage 3 = Radical nephrectomy + Chemotherapy: (Vincristine + Actinomycin D) for 18 weeks Stages 1-4 = Partial nephrectomy Stage 5 = Surgery + Chemotherapy + Radiotherapy</p> Signup and view all the answers

    What is the most common solid kidney tumor in children?

    <p>Nephroblastoma</p> Signup and view all the answers

    Wilms' tumor is always bilateral in children.

    <p>False</p> Signup and view all the answers

    What is the age range most commonly affected by Wilms' tumor?

    <p>2 to 5 years</p> Signup and view all the answers

    One of the genetic syndromes associated with Wilms' tumor is _____ syndrome, which includes Wilms tumor, aniridia, genitourinary anomalies, and mental retardation.

    <p>WAGR</p> Signup and view all the answers

    Match the following clinical features of Wilms' tumor with their descriptions:

    <p>Increased renin = Leads to Hypertension in children Hematuria = Presence of blood in urine Asymptomatic abdominal mass = Enlargement of the kidney Site of Metastasis = Lung &gt; lymph node &gt; liver</p> Signup and view all the answers

    What is the most common cause of inflammatory myopathy in children?

    <p>Juvenile Dermatomyositis</p> Signup and view all the answers

    Juvenile Dermatomyositis primarily affects males more than females.

    <p>False</p> Signup and view all the answers

    What age group is most commonly affected by Juvenile Dermatomyositis?

    <p>5-14 years</p> Signup and view all the answers

    The sign used to describe a child's difficulty getting up from a chair due to muscle weakness is called Gower's _____?

    <p>sign</p> Signup and view all the answers

    Match the following clinical features of Juvenile Dermatomyositis with their descriptions:

    <p>Heliotrope rash = Violaceous discoloration around the eyes Gottron papules = Red, scaly lesions over the joints Shawl sign = Rash on the back resembling a shawl Periungual erythema = Erythema surrounding the nail beds</p> Signup and view all the answers

    What is a common feature of Enthesitis-related Arthritis?

    <p>Inflammation of tendons/ligaments</p> Signup and view all the answers

    Henoch-Schönlein Purpura primarily affects older adults.

    <p>False</p> Signup and view all the answers

    What does an increased ESR indicate in the context of rheumatic disorders?

    <p>It indicates inflammation.</p> Signup and view all the answers

    In cases of polyarticular arthritis, the first-line treatment is _____ along with disease-modifying antirheumatic drugs.

    <p>NSAIDs</p> Signup and view all the answers

    Match the type of arthritis to its characteristic treatment:

    <p>Oligoarticular = NSAIDs, intra-articular triamcinolone if unresponsive Polyarticular = NSAIDs + DMARDs such as methotrexate Systemic = Biological agents like adalimumab Enthesitis-related Arthritis = HLA-B27 association</p> Signup and view all the answers

    Which of the following are essential criteria for diagnosing rheumatic disorders in childhood?

    <p>Rash: Heliotrope rash &amp; Gottron's papules</p> Signup and view all the answers

    Juvenile Idiopathic Arthritis (JIA) is only diagnosed when there is involvement of two or more joints.

    <p>False</p> Signup and view all the answers

    What is the most common type of Juvenile Idiopathic Arthritis?

    <p>Oligoarticular JIA</p> Signup and view all the answers

    The initial treatment for rheumatic disorders in childhood includes ______.

    <p>steroids</p> Signup and view all the answers

    Match the types of Juvenile Idiopathic Arthritis with their characteristics:

    <p>Oligoarticular JIA = ≤ 4 joints involvement, large joints affected Polyarticular JIA = Typically involves &gt; 5 joints Systemic JIA = Characterized by fever and rash Enthesitis-related JIA = Involves inflammation of the entheses</p> Signup and view all the answers

    What is a characteristic symptom of VIP hypersecretion in children?

    <p>Intractable watery diarrhea</p> Signup and view all the answers

    Stage 3 neuroblastoma is characterized by tumors that do not cross the midline.

    <p>False</p> Signup and view all the answers

    What is the most common imaging study used in diagnosing solid tumors in children?

    <p>CT Abdomen</p> Signup and view all the answers

    Opsoclonus-myoclonus ataxia is associated with _______ antibodies.

    <p>anti-neuronal</p> Signup and view all the answers

    Match the stages of neuroblastoma with their descriptions:

    <p>Stage 1 = Completely resectable tumors Stage 2a = Incompletely resectable tumors; no lymph node involvement Stage 3 = Tumors that cross the midline Stage 4 = Disseminated tumors</p> Signup and view all the answers

    Which disease is characterized by skull defects and exophthalmos?

    <p>Hand-Schüller-Christian disease</p> Signup and view all the answers

    Birbeck granules are typically observed in patients with Eosinophilic granuloma.

    <p>False</p> Signup and view all the answers

    What is the common treatment used for multiple lesions in LCH?

    <p>Vinblastine</p> Signup and view all the answers

    The primary diagnostic method for LCH involves tissue sample examination through _______.

    <p>biopsy</p> Signup and view all the answers

    Match the following LCH clinical features with their corresponding age groups:

    <p>Eosinophilic granuloma = Older children Hand-Schüller-Christian disease = 2-6 years Letterer-Siwe disease = &lt; 2 years</p> Signup and view all the answers

    Which of the following joints are typically affected in polyarticular JIA?

    <p>Metacarpophalangeal joint</p> Signup and view all the answers

    Systemic JIA is more commonly found in females than in males.

    <p>False</p> Signup and view all the answers

    What is a severe complication of systemic JIA that involves hyperinflammation?

    <p>macrophage activation syndrome (MAS)</p> Signup and view all the answers

    In psoriatic arthritis, nail _____ is a characteristic symptom.

    <p>pitting</p> Signup and view all the answers

    Match the type of JIA to its typical characteristic:

    <p>Polyarticular JIA = Asymmetrical joint involvement Systemic JIA = Quotidian fever and salmon rash Psoriatic arthritis = Nail pitting or dactylitis</p> Signup and view all the answers

    What is the most common primary site of neuroblastoma in children?

    <p>Adrenal gland</p> Signup and view all the answers

    Neuroblastoma is the most common extra-cranial solid tumor in children.

    <p>True</p> Signup and view all the answers

    Name one common constitutional symptom of neuroblastoma.

    <p>Fever</p> Signup and view all the answers

    Neuroblasts are derived from __________ cells.

    <p>neural crest</p> Signup and view all the answers

    Match the following clinical features of neuroblastoma:

    <p>Horner's syndrome = Pupil constriction and ptosis Blueberry muffin lesions = Purplish/bluish subcutaneous nodules Bony pain = Most common site of metastasis Raccoon eyes = Peri-orbital swelling and ecchymosis</p> Signup and view all the answers

    What is the most common and characteristic feature of paediatric rheumatology conditions discussed?

    <p>Palpable purpura</p> Signup and view all the answers

    Renal involvement is not a common feature in paediatric rheumatology conditions.

    <p>False</p> Signup and view all the answers

    What type of biopsies are usually required for diagnosis in most cases?

    <p>Skin or kidney biopsy</p> Signup and view all the answers

    In children with paediatric rheumatology conditions, the presence of ______ is essential for diagnosis.

    <p>raised, palpable, hemorrhagic skin lesions</p> Signup and view all the answers

    Match the complications with their description:

    <p>Acute = GI involvement such as intussusception and GI bleed Chronic = Long-term renal problems requiring regular monitoring Both = Can lead to nephritis and progressive kidney disease None = Related to platelet count abnormalities</p> Signup and view all the answers

    Study Notes

    Solid Tumors in Children - COG Staging

    • Stage 1: Tumor limited to kidney, completely resectable.
    • Stage 2: Tumor extends beyond kidney, completely resectable, no lymph node involvement. Residual tumor confined to abdomen.
    • Stage 3: Tumor metastasized to regional lymph nodes, extending into IVC.
    • Stage 4: Tumor metastasized to lymph nodes outside abdomen and distant organs like lungs and liver.
    • Stage 5: Bilateral kidney involvement.

    Solid Tumors in Children - Treatment

    • Stages 1-4: Radical or partial nephrectomy.
    • Stage 3/4: Chemotherapy (Vincristine + Actinomycin D) for 18 weeks, surgery + chemotherapy + radiotherapy.
    • Stages 1/2: Chemotherapy (Vincristine + Actinomycin D + Doxorubicin) for 24 weeks and radiotherapy.
    • Stage 5: Surgery + chemotherapy + radiotherapy.

    Juvenile Dermatomyositis (JDM)

    • A multisystem disorder involving skin and muscles, possibly with systemic vasculopathy, affecting children aged 5-14 (females more than males).
    • Proximal, symmetrical muscle weakness affecting shoulders and hips, causing difficulties with getting up and climbing stairs.
    • Skin features include violaceous discoloration, red scaly lesions over extensor aspects of joints, periorbital edema, heliotrope rash, Gottron papules, and shawl sign.
    • Nail findings include periungual erythema and dilated nail fold capillaries on nail fold capillaroscopy.

    Wilms' Tumor

    • Unilateral kidney tumor, also known as nephroblastoma, most common solid kidney tumor in children.
    • Second most common intra-abdominal tumor in children, originating from undifferentiated mesenchyme of the kidney.
    • Most common in children aged 2-5 years.
    • Risk factors include malformations like horseshoe kidney, cryptorchidism, and hypospadias.
    • Genetic syndromes associated include WAGR syndrome with WT-1 gene mutation and Beckwith-Wiedemann syndrome with WT-2 gene mutation.
    • Clinical features include asymptomatic abdominal mass in the loin, hypertension, and hematuria.
    • Most common site of metastasis is lung, followed by lymph nodes and liver.
    • Localized tumors are treated with surgical resection, while disseminated tumors require surgery + chemotherapy, and radiotherapy if chemotherapy is ineffective.
    • Autologous hematopoietic stem cell transplant (AHSCT) is an alternative treatment option.
    • More common in males.
    • Characterized by inflammation of tendons and ligaments, asymmetrical arthritis of lower limbs, and HLA-B27 association.
    • Potential feature of sacroiliitis, which is a precursor to ankylosing spondylitis.

    Juvenile Idiopathic Arthritis (JIA)

    • Most common chronic rheumatic arthritis in children, an autoimmune disorder.
    • Affecting children under 16 years with involvement of at least one joint for a duration of at least 6 weeks.
    • Other conditions are excluded.
    • Types include Oligoarticular JIA (most common, affecting≤4 joints, large joints like knee, ankle, wrist), Polyarticular JIA, and Systemic JIA.

    Langerhans Cell Histiocytosis (LCH)

    • Spectrum of LCH varies based on age and clinical features.
    • Eosinophilic granuloma - older children, lytic bone in skull.
    • Hand-Schüller-Christian disease - 2-6 years, skull defect, exophthalmos.
    • Letterer-Siwe disease - <2 years, seborrheic dermatitis, ear discharge, systemic involvement: Hepatosplenomegaly (Abnormal LFT), Lymphadenopathy.
    • Diagnosis is confirmed by Immunohistochemistry (CDla, CD207), biopsy, and electron microscopy.
    • Treatment depends on the severity of the lesion and might include curettage, intralesional steroids, Vinblastine, 6-MP, prednisolone, and Cladribine.

    Neuroblastoma

    • Most common intra-abdominal tumor in children, second most common extracranial solid tumor.
    • Common malignancy in infancy, originating from neuroblasts derived from neural crest cells.
    • Primary sites of involvement include the adrenal gland and paravertebral sympathetic chain.
    • Secondary sites include the posterior mediastinum and pelvis.
    • Grossly, it appears as a solid tumor, microscopically - small, uniform blue cells with scant cytoplasm and hyperchromatic nuclei.
    • Homer-Wright rosettes have a rose petal-like appearance.
    • Clinical features include abdominal mass, constitutional symptoms, Horner's syndrome, heterochromia iridis, and metastasis features such as bone pain, peri-orbital swelling, and blueberry muffin lesions.

    Henoch-Schönlein Purpura (HSP)/IgA Vasculitis

    • A multisystem small vessel vasculitis, common in children (age 4-8 years).
    • Pathogenesis involves leukocytoclastic vasculitis, fibrinoid necrosis, and IgA deposits.
    • Characterized by palpable purpura, non-blanching and non-thrombocytopenic, typically located in dependent areas like lower limbs and genital area.
    • Other features include abdominal pain, joint involvement, and renal involvement including hematuria/nephritis and nephrotic range proteinuria.

    Paediatric Rheumatology: Polyarticular JIA

    • Rheumatoid Factor Positive Polyarticular JIA affects any age up to 16 years, predominantly occurring between 9-11 years.
    • Joints affected include knee, hip, wrist, metacarpophalangeal, proximal interphalangeal, cervical spine, and temporomandibular joint.
    • The disease is symmetrical, associated with rheumatoid nodules and uveitis.

    Paediatric Rheumatology: Systemic JIA

    • More common in males.
    • Characterized by daily fever spikes, arthritis, and any one of the following features: Salmon-colored non-pruritic maculopapular rash, hepatosplenomegaly, lymphadenopathy, serositis (pleuritis/pericarditis), Koebner phenomenon, and hypersensitivity reactions.
    • Complication: macrophage activation syndrome (MAS) - a hyperinflammatory condition with continuous fever, drop in cell counts predominantly thrombocytopenia, elevated ferritin and triglycerides, and hemophagocytosis on bone marrow biopsy.

    Paediatric Rheumatology: Psoriatic Arthritis

    • More common in females.
    • Characterized by arthritis and psoriasis or any two of the following: nail pitting, dactylitis (sausage digits), or a first-degree relative with psoriasis.

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    Description

    This quiz covers staging and treatment options for solid tumors in children, including nephrectomy and chemotherapy regimens for various stages. In addition, it addresses juvenile dermatomyositis, a multisystem disorder affecting young children. Test your knowledge on these critical pediatric conditions.

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