Lysosomal and Glycogen Storage Disorders
- Previous year questions on Lysosomal and Glycogen Storage Disorders for NEET PG (National Eligibility cum Entrance Test - Postgraduate), from the Pathology section of the exam.
- Most questions here come from previous-year papers of Indian postgraduate medical entrance and licensing exams; where a paper and year are recorded the question carries them. The image-based questions are original vignettes written around an openly licensed figure, and were never asked in any exam.
- 19 live questions in Lysosomal and Glycogen Storage Disorders.
- 2 of 19 questions carry a verified exam year: 2018.
- Pathology is worth about 25 of 300 questions on the exam. See every subject’s weightage.
- Question set last updated 6 Sept 2026.
Q1.Maroteaux Lamy syndrome characterized by deficiency of
- ABeta Galactosidase
- BBeta Glucuronidase
- CArylsulfatase B✓ Correct answer
- DIduronate sulfatase
Q2.'I' cells disease is due to defect in:-
- APeroxisome
- BMitochondria
- CLysosome
- DGolgi apparatus✓ Correct answer
Q3.Mental retardation is seen in all except
- AHurler
- BSanfilippo
- CHunter
- DMorquio✓ Correct answer
Q4.Mental retardation is not a feature of one of the following Mucopolysaccharidosis?
- AHurler MPS-I
- BHunter MPS -II
- CSanfillipo MPS -III
- DMorquio MPS --IV✓ Correct answer
Q5.What is the use of migalastat approved by FDA in 2018 August?
- AFabry's disease✓ Correct answer
- BBreast cancer
- CGlycogen storage disorder
- DHIV
Q6.A 3- year old child has hepatosplenomegaly. On examination of the bone marrow, large cells are seen with crumpled paper appearance. Which of the following must have accumulated in these cells?
- ASphingomyelins
- BGlucocerebrosides✓ Correct answer
- CCeramides
- DSulphatides
Q7.A one year old child, who is earlier normal, is suffering from copious nasal discharge, coarse facial features, large tongue, bulging head and flattened nose. He is also having enlarged spleen and liver. What may be the cause?
- AHypothyroidism
- BBeckwith widman syndrome
- CProteus syndrome
- DHurler syndrome✓ Correct answer
Q8.A male child presented with coarse facies, protuberant abdomen , frontal head enlargement, thickening of cardiac valve, hepatosplenomegaly, hearing impairement. What is the most probable diagnosis?
- AHurler's disease
- BHunter's disease✓ Correct answer
- CFragile X syndrome
- DTay Sach's disease
Q9.A 48-year old lady presented with bony pain and hepatosplenomegaly. On examination of biopsy specimen from spleen, crumpled tissue paper appearance is seen. Which of the following product is likely to have accumulated?
- AGanglioside
- BSulfatide
- CSphingomyelin
- DGlucocerebroside✓ Correct answer
Q10.A short statured patient brought with X-ray showing flattening of vertebrae with beak
- AAchondroplasia✓ Correct answer
- BProtein Energy malnutrition
- CRickets
- DDown's syndrome
Answer key
Q1: C. Arylsulfatase B; Q2: D. Golgi apparatus; Q3: D. Morquio; Q4: D. Morquio MPS --IV; Q5: A. Fabry's disease; Q6: B. Glucocerebrosides; Q7: D. Hurler syndrome; Q8: B. Hunter's disease; Q9: D. Glucocerebroside; Q10: A. Achondroplasia.
Other topics in Pathology
- Cell Genetics, Adaptation and Cell Injury
- Necrosis and Apoptosis
- Intracellular Accumulations, Pathological Calcification and Cell Ageing
- The Acute Inflammatory Response
- Chemical Mediators and Granulomatous Inflammation
- Wound Healing and Tissue Repair
- Hemodynamic and Hemostatic Disorders
- Patterns of Inheritance
- Chromosomal and Other Genetic Disorders
- Features and Epidemiology of Neoplasia
- Molecular Basis of Cancer and Tumour Immunity
- Carcinogenesis, Paraneoplastic Syndromes and Tumour Markers
- Cells and Organs of the Immune System
- Hypersensitivity Reactions and Autoimmune Disease
- Immunodeficiency Disorders
- Amyloidosis and Transplant Rejection
- Histopathology Techniques and Special Stains
- Microcytic Anemias
- Normocytic and Macrocytic Anemias
- Hemolysis Overview and Intravascular Hemolysis
- Extravascular Hemolytic Anemias
- G6PD Deficiency and Autoimmune Hemolysis
- Disorders of Platelets
- Disorders of the Coagulation Cascade
- Blood Components and Transfusion Reactions
- Acute Lymphoblastic Leukemia (ALL)
- Acute Myeloid Leukemia (AML)
- Hodgkin Lymphoma
- Non-Hodgkin Lymphoma: Overview
- Non-Hodgkin Lymphoma: Low Grade
- Non-Hodgkin Lymphoma: High Grade
- Multiple Myeloma and Other Plasma Cell Neoplasms
- Myelodysplastic and Myeloproliferative Neoplasms and Histiocytosis
- White Cell Disorders and Lymphadenitis
- Atherosclerosis and Hypertensive Vascular Disease
- Aneurysms and Arterial Dissection
- Vasculitis
- Vascular Tumours
- Heart Failure and Ischemic Heart Disease
- Cardiomyopathies, Pericardial Disease and Cardiac Tumours
- Valvular and Congenital Heart Disease
- Rheumatic Fever and Endocardial Disease
- Diseases of the Glomerulus
- Tubulointerstitial, Vascular and Cystic Renal Disease
- Renal Tumours
- Pathology of the Lower Urinary Tract
- Pathology of the Female Genital Tract
- Pathology of the Male Genital Tract
- Infectious and Alcohol-Related Liver Disease
- Metabolic and Autoimmune Liver Disease
- Tumours of the Liver and Biliary Tract
- Gallbladder and Pancreatic Disease
- Esophageal Pathology
- Gastric Pathology
- Small Bowel Pathology
- Large Bowel: Non-Neoplastic Disease
- Large Bowel: Neoplastic Disease
- Congenital Anomalies, ARDS and Lung Infections
- Obstructive and Restrictive Lung Disease
- Lung Tumours
- Pituitary, Parathyroid and Endocrine Pancreas
- Thyroid Gland Pathology
- Adrenal Gland Pathology
- Breast Pathology
- Bone: Developmental Disorders, Infections and Tumours
- Joint and Soft Tissue Pathology
- Dermatopathology
- CNS Infections and Cerebrovascular Disease
- Degenerative, Toxic and Metabolic CNS Disease
- Tumours of the CNS
- Peripheral Nerve and Neuromuscular Pathology