Pathology- MCQ's in Kids

1) After four days, a neonate has not passed meconium, and begins vomiting. Physical examination reveals abdominal distension. Which one of the following is the correct diagnosis?

A. Hirchsprung's disease
B. Meckel's diverticulum
C. Omphalocele
D. Renal agenesis
E. Tracheoesophageal fistula


The correct answer is A. Hirchsprung's disease is caused by an absence of ganglion cells in both the submucosal and inter-myenteric plexus of a segment of bowel. The aganglionic bowel segment is narrowed because the lack of peristalsis keeps stool from moving into that segment. The distal rectum is always involved with the the lesion extending proximally. The bowel proximal to the lesion is usually dilated. Treatment is by surgical removal of the segment.

Meckel's diverticulum (choice B) is a small, usually asymptomatic pouch located near the ileocecal vale.

An omphalocele (choice C) would present with and obvious sac filled with intestines at the site of a defect in the ventral abdominal wall.

Reneal Agenesis (choice D) would present with failure to pass urine.

Tracheoesophageal fistula (choice E) would present with aspiration during feeding.




2) A 3 year old child develops headaches and is brought to the family doctor. Fundoscopic examination reveals papiledema; one retina also shows a very vascular tumor. CT of the head demonstrates a cystic tumor in the cerebellum. This child has a high likelihood of later developing which of the following?

A. Berry aneurysm of the basilar system
B. Bilateral renal cell carcinoma
C. Cancer of peripheral nerve
D. Choreiform movement related to decreased GABA and acetylcholine
E. Serum Cholesterol of greater than 700 mg/dL



The correct answer is B. The disease con Hippel-Landau disease, which is associated with a deletion involving the VHL gene on chromosome 3 (3p). Affected individuals develop vascular tumors (hemangioblastomas) of the retina, cerebellum, and/or medulla. Roughly half of the affected individuals later develop multiple, bilateral renal cell carcinomas.

Berry aneurysms (choice A) are unrelated to hemangioblastomas, but are instead associated with adult polycystic disease.

Peripheral nerve cancers (choice C) are a feature of von Recklinghausen's disease.

Choreiform movement, related to decreased GABA and acetylchline (choice D), are a feature of Huntigton's disease.

Extremely high serum cholesterol (choice E) suggests the homozygous form of familial hypercholesterolemia.




3) A 2 year old boy is brought to the pediatrician for a fever. The physical exam confirms a fever, but no source is identified. Generalized lymphadenopathy is also noted. She is admitted to a local hospital for observation. One day later she begins to have swelling of her knees and elbows bilaterally with rubor but no rash. Her liver and spleen are found to be slightly enlarged. Which of the following is the most likely diagnosis.

A. Felty's syndrome
B. Henoch-Schonlein purpura
C. Kawasaki's disease
D. Septic arthritis
E. Still's disease



The correct answer is E. Still's disease, or juvenile rheumatoid arthritis, affects children younger than 16 years old, usually 1-3 years of age. There is a female predominance. Both small and large joints are affected. The systemic onset of disease usually includes high fever, splenomegaly, general lymphadenopathy, and neutrophilic leukocytosis. A rash may or may not be present. Rheumatoid factor is usually negative, but if positive, it is associated with a poorer prognosis.

Felty's syndrome (choice A) is a polyarticular rheumatoid arthritis associated with splenomegaly, leg ulcers, and leukopenia (rather than leukocytosis). It affects adults with known rheumatoid arthritis or SLE.

Henoch-Schonlein purpura (Choice B) is a disease preceded by a respiratory infection and is characterized by vasculitis that affects the joints, skin, GI tract and kidney.

Kawasaki's disease (choice C), or mucocutaneous lymph node syndrome, is a vasculitis that occurs in children and causes fever, conjunctivitis, erosion of the oral mucosa, lymphadenopathy, and a rash. The most serious sequelae are due to coronary and aortic vasculitis.

Septic arthritis (choice D) is usually caused by hematogenous seeding of a joint during bacteremia. It usually affects children. Clinically, the joint is tender and swollen. Lymphadenopathy and fever are common. The joint affected is usually single rather than bilateral and there is no hepatosplenomegaly.




4) A 3 year old boy with an enlarging, left-sided, abdominal mass undergoes diagnostic biopsy. The tumor reveals a variety of cellular patterns: dense immature islands of epithelial cells, ribbons of spindled fibroblast-like stroma cells, and poorly formed tubular structures. This triphasic hisology is most suggestive of which of the following childhood neoplasms?

A. Embryonal Rhabdomyosarcoma
B. Ewing's Sarcoma
C. Hodgkin's disease
D. Neuroblastoma
E. Wilms' tumor



The correct answer is E. Wilms' tumor, a neoplasm of embryonic renal blastoma, contains both a neoplastic epithelial and stromal components.

Rhabdomyosarcoma (choice A) is a malignancy of skeletal muscle origin, and the cells are recognized by their cytoplasmic and eosinophilic muscle proteins (thin and thick filaments).

Ewing's sarcoma (choice B) appears as a monotonous mass of primitive, small, round cells with a thin rim of clear cytoplasm.

Hodgkin's disease (choice C) is a lymphoid malignancy that presents with lymphadenopathy, typically in the cervical or mediastinal nodes. The cellular components contains varying amounts of inflammatory cells and Reed-Sternberg cells.

Neuroblastoma (choice D) frequently presents as an abdominal mass in children; however, it does not have an epithelial component. The cell population of neuroblastoma ranges from primitive, small, blue, round cells to well-differentiated neuritic cells resembling ganglion cells.




5) A 6 month old child is evaluated by a pediatrician for growth failure and particularly foul-smelling stools. Studies demonstrate and elevated sweat chloride concentration. The child is placed on oral enzyme supplementation and begins to grow. Damage to which of the following organs would be most likely to lead to this child's death during early adulthood?

A. Heart
B. Large intestine
C. Liver
D. Lungs
E. Small intestine



The correct answer is D. Formerly, patients with cystic fibrosis usually dies of malnutrition. The use of oral pancreatic enzyme supplements have markedly changed the course of this disease, and death in the first few years of life is now uncommon. Unfortunately, damage to the the lungs in the form of multiple episodes of pneumonia leading to bronchiectasis and pulmonary fibrosis remains the major cause of mortality in the second and third decades.

The heart (choice A) is not damaged directly by cystic fibrosis, but pulmonary hypertension secondary to pulmonary fibrosis can cause right-sided heart failure.

Life-threatening disease of the large (choice B) or small (choice E) intestines does not usually occur in cystic fibrosis.

The liver (choice D) is also affected by cystic fibrosis, with intrahepatic bile plugs and extrahepatic biliary structures, leading in some cases to portal hypertension and biliary cirrhosis.