(MCQS 298-300) :A 5 month old infant with recurrent flexor rapid sudden flexion and extension of the extremities

Correct Answer: E
Hypothyroidism often indirectly causes anemia. Iron deficiency occurs in association with myxedema in part because menorrhagia is a common manifestation of the illness, in part because the achlorhydria found in myxedema subjects may lead to impaired absorption of food iron, and in part because thyroid hormone itself may be essential for normal iron absorption Heavy, more frequent mentral flow is a major (but often overlooked) symptom of thyroid disease. This in turn causes anemia and even more hair loss. Anemia can cause you to be tired and cold, but generally not gain weight Three morphologic types of anemia have been described in adult patients . The relative incidence of the three types differs considerably in three large series reported since 1960 . The reasons for the differences are not clear, but they may have been related to factors not directly concerned with the thyroid disease as well as to its degree and duration. When hypochromic, microcytic anemia is found in association with myxedema, it almost always is attributable to iron deficiency. Serum iron values are subnormal , and the anemia responds to iron therapy, even if thyroid hormone is not administered; it is not relieved by hormone therapy if iron is withheld. Often, iron deficiency in myxedema produces normocytic anemia. Therefore, an important step is to determine the serum iron concentration, regardless of the morphologic picture. Distinctly, macrocytic anemia usually results from complicating deficiency of vitamin B12 or folate. The increased incidence of pernicious anemia in thyroid disease possibly is the result of an autoimmune mechanism, as discussed in. Folate deficiency may develop because of an inadequate diet, but this correlation has not been established with certainty. If the patients with iron, folate, or vitamin B12 deficiency are excluded, a significant population of hypothyroid patients with anemia remains. This type of anemia constitutes the so-called uncomplicated anemia of hypothyroidism , and is a manifestation of the hormone deficiency itself. All or nearly all children with anemia and hypothyroidism have the uncomplicated form of the disease . The anemia usually affects children whose height is below the third percentile.
Correct Answer: D
West's syndrome is also called infantile spasms and usually presents during the first year of life with a peak incidence of onset between 4 and 7 months. It consists of a triad of arrested psychomotor development, infantile spasms, and a characteristic EEG pattern called hypsarrhythmia. Hypsarrhythmia is an EEG pattern with high-voltage slow waves, irregularly interspersed with spikes and sharp waves that occur randomly in all cortical areas. The seizures usually consist of flexor spasms, extensor spasms, or both. They occur with rapid onset, are bilateral and symmetric, and appear in clusters. They may involve the head, trunk, and extremities. The intensity of the contractions and the number of muscle groups involved vary both among individuals and in the same individual during repeated attacks. Any repetitive phenomena (e.g., head nodding, eye elevation or deviation, or movement of one limb) should suggest infantile spasms. Initially, the attacks appear to be a normal startle response but then go on to develop into the typical salaam, jackknife seizures with sudden flexion and extension of the extremities. The majority of children (greater than two thirds) have an underlying CNS disorder (e.g., congenital malformation or dysgenesis, hypoxic-ischemic encephalopathy, tuberous sclerosis, metabolic disorder, or congenital infection), and the remaining cases are thought to be cryptogenic in origin. It is thought that the pons is involved in the spasms because it is essential for the maintenance of extensor and flexor tone and is part of the reticular activating system that controls attention and consciousness.The outcome is poor because this syndrome is usually refractory to anticonvulsant medications, and 90% to 95% of the children are developmentally delayed. Current medications for this disorder include the following: adrenocorticotropic hormone (ACTH), steroids, nitrazepam, and valproic acid. Steroids and ACTH may have a selective effect on neurotransmitters in the CNS.
Correct Answer: G
The cause of amyoplasia is unknown, but children with this disorder have a decreased number of anterior horn cells in the spinal cord, suggesting a neuropathic cause. Other studies have shown that the disorder may be myopathic in origin. In the latter instance, diminution of in utero movement may be the final common pathway leading to contractures. Every child presenting with multiple joint contractures should have a complete musculoskeletal evaluation and genetics consultation. The distribution of involvement of multiple joint contractures is variable. The classic presentation involves the upper and lower extremities. The lower extremities are typically more involved than the upper extremities. Involvement of all four extremities is quadrimelic involvement. It is also possible that only the lower extremities or upper extremities (bimelic) may be involved. It is unusual to see only one extremity or a portion of one extremity (monomelic) involved


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