Commonest enzymatic defect for development of congenital adrenal hyperplasia is:

Correct Answer: A

Rationale: C-21 hydroxylase deficiency is the most common enzymatic defect leading to the development of congenital adrenal hyperplasia (CAH). This enzyme is necessary for the synthesis of cortisol and aldosterone in the adrenal glands. When there is a deficiency in C-21 hydroxylase, the pathway is shifted towards the production of androgens instead of cortisol and aldosterone, leading to excess androgen production. This results in the characteristic features of CAH, such as ambiguous genitalia in females and virilization in both males and females. Other enzyme deficiencies listed in the choices can also lead to different types of CAH but are less common compared to C-21 hydroxylase deficiency.

Correct Answer: A

Rationale: Acromegaly is a disorder caused by excessive growth hormone production by the pituitary gland in adults, leading to an increase in muscle mass (hypertrophy). Hoffman syndrome, characterized by increased muscle mass with slowness of activity, is a clinical feature often seen in individuals with acromegaly. The excessive growth hormone levels in acromegaly can cause acral growth, soft tissue swelling, and muscle hypertrophy, contributing to the characteristic features of the condition, including Hoffman syndrome. Therefore, the correct answer is Acromegaly.

Correct Answer: B

Rationale: Hyperbilirubinemia is not a common effect of diabetes on the fetus. The correct effects of diabetes on the fetus include:

Correct Answer: A

Rationale: The most common cause of Addison's disease is autoimmune adrenalitis, also known as autoimmune adrenal insufficiency. In autoimmune adrenalitis, the adrenal glands are damaged by immune system-mediated processes, leading to their dysfunction. This autoimmune destruction typically presents as bilateral adrenal cortex atrophy due to the formation of granulomas. Therefore, the commonest cause of Addison's disease is the presence of granulomas in the adrenal glands, making option A, Granuloma, the correct choice.

Correct Answer: D

Rationale: Charcot joint, also known as neuropathic arthropathy, is a serious complication of diabetes mellitus that affects the joints. It commonly affects the foot, especially the midfoot and hindfoot. The condition is characterized by joint deformities, fractures, and dislocations due to nerve damage and loss of sensation in the foot. The repetitive stress on the foot from walking or weight-bearing activities can lead to progressive joint destruction and deformity. It is important for individuals with diabetes to monitor their foot health closely and seek prompt medical attention if they notice any changes or deformities in their feet.

Correct Answer: D

Rationale: Growth hormone is primarily released during sleep, particularly during the first few hours of deep sleep. This release is pulsatile in nature, with the majority occurring at night. Growth hormone plays a critical role in growth, repair, and development of body tissues during periods of rest and sleep. Additionally, the secretion of growth hormone is also influenced by factors such as stress, exercise, and nutritional status.

Correct Answer: D

Rationale: In dwarfism conditions, the upper body segment being greater than the lower body segment is a characteristic feature seen in Achondroplasia. This condition results in a disproportionately short stature with short limbs but a trunk of normal length.

Correct Answer: C

Rationale: Tetany is a condition characterized by involuntary muscle spasms and contractions due to low levels of calcium in the blood. The signs associated with tetany include Trousseau's sign, which is the carpal spasm elicited by inflating a blood pressure cuff above systolic pressure, and Chvostek's sign, which is the facial muscle spasm elicited by tapping on the facial nerve. Peroneal sign is not specifically associated with tetany but can be seen in conditions like peroneal nerve injury. Erb's sign, on the other hand, is not a recognized sign of tetany. It is associated with Erb's palsy, a condition resulting from injury to the brachial plexus nerves, typically during childbirth. Tinel's sign, which is a tingling sensation elicited by tapping over a compressed nerve, is also not a characteristic sign of tetany.

Correct Answer: B

Rationale: Myxoedema refers to severe hypothyroidism which can lead to neurological manifestations. The correct features associated with myxoedema include delayed relaxation of ankle jerk (Choice A), hypertonia (Choice C), and bradylalia (Choice D). Cerebellar ataxia, which involves incoordination and balance issues due to dysfunction of the cerebellum, is not a typical neurological feature seen in myxoedema.