Block 15 Pediatric Pathology

An Apgar score of 8 indicates excellent chances to survive. The Apgar score is a quick assessment tool used to evaluate the physical condition of a newborn immediately after birth. It assesses the baby's heart rate, respiratory effort, muscle tone, reflex irritability, and color. A score of 8 out of 10 is considered very good and suggests that the baby is in good health and has a high likelihood of survival.

During weeks 3-8 of pregnancy, the embryo is in the early stages of development and is particularly vulnerable to the influence of teratogens. This is because this is the period when the major organs and body systems are forming. Exposure to teratogens during this time can lead to structural abnormalities or functional defects in the developing embryo. Therefore, it is crucial for pregnant women to avoid exposure to harmful substances or environmental factors that could potentially harm the developing embryo during these critical weeks.

A combination of microcephaly, hydrocephaly, cataract, chorioretinitis, and cardiac septal defect is characteristic for TORCH infection. TORCH infections are caused by a group of pathogens including Toxoplasma, Other (such as syphilis, varicella-zoster, parvovirus B19, etc.), Rubella, Cytomegalovirus, and Herpes simplex virus. These infections can be transmitted from mother to fetus during pregnancy and can lead to various congenital abnormalities. The presence of multiple symptoms like microcephaly, hydrocephaly, cataract, chorioretinitis, and cardiac septal defect suggests a TORCH infection as the cause.

Subserosal petechiae are small red or purple spots that occur on the surface of organs, such as the heart, liver, or intestines, due to bleeding from tiny blood vessels. These findings are commonly seen in cases of Sudden Infant Death Syndrome (SIDS). SIDS is the sudden and unexplained death of an apparently healthy infant, usually during sleep. The presence of subserosal petechiae in SIDS cases suggests that there may have been some degree of asphyxia or suffocation, leading to increased pressure within the chest and rupturing of small blood vessels.

Rubella, also known as German measles, is a viral infection that can cause various complications during pregnancy. If a pregnant woman contracts rubella, it can lead to congenital rubella syndrome in the newborn. One of the potential complications of congenital rubella syndrome is cataract formation in the newborn. Therefore, cataract in a newborn is associated with rubella.

The correct answer is A - control; B - necrotizing enterocolitis. This is indicated by the pathology depicted in the images. Image A shows a normal, healthy tissue (control), while Image B shows signs of necrotizing enterocolitis, which is characterized by inflammation and necrosis of the intestinal tissue.

Maternal alcoholism is known to cause a variety of birth defects, including fetal alcohol syndrome (FAS). FAS is characterized by growth retardation, mental retardation, microcephaly, narrow upper lip, short palpebral fissures, and smooth filtrum. While alcohol exposure during pregnancy can potentially affect the development of the heart, atrial septal defect and ventricular septal defect are more commonly associated with maternal alcoholism than cataracts. Therefore, cataracts are the least likely congenital defect to be associated with maternal alcoholism.

Necrotizing EnteroColitis

The condition is typically seen in premature infants, and the timing of its onset is generally INVERSELY PROPORTIONAL TO THE GESTATIONAL AGE OF THE BABY AT BIRTH, i.e. the earlier a baby is born, the later signs of NEC are typically seen. Initial symptoms include feeding intolerance, increased gastric residuals, abdominal distension and bloody stools. Symptoms may progress rapidly to abdominal discoloration with intestinal perforation and peritonitis and systemic hypotension requiring intensive medical support.

The terms applied to this baby are Prematurity and LGA (Large for Gestational Age). Prematurity refers to the baby being born before the full 37 weeks of gestation. LGA refers to the baby being larger than expected for their gestational age.

Kernicterus — Kernicterus is the chronic and permanent sequelae of BIND. The following major post-icteric features of kernicterus in preterm infants are usually similar to that observed in term infants, because the basal ganglia remain the primary target of brain injury.
Choreoathetoid cerebral palsy (CP, chorea, ballismus, tremor, and dystonia).
Sensorineural hearing loss commonly manifesting as auditory neuropathy (abnormal ABR with normal otoacoustic emissions). Observational studies suggest that isolated sensorineural hearing abnormalities may be the dominant or the sole manifestations of BIND.
Gaze abnormalities, especially limitation of upward gaze

Spina bifida occulta is a condition characterized by a defect in the vertebral arch only. This means that there is an incomplete closure of the bones that make up the spinal column, specifically the vertebral arch. Unlike other forms of spina bifida, there is no skin outpouching or herniation of the spinal cord or meninges in spina bifida occulta. It is usually asymptomatic and may only be detected incidentally on imaging studies.

CAPUT SUCCEDANEUM is an edematous swelling of the scalp above the periosteum, which is occasionally hemorrhagic (figure 1). It presents at birth after prolonged engagement of the fetal head in the birth canal or after vacuum extraction. Unlike cephalohematoma, it EXTENDS ACROSS THE SUTURE LINES. Caput succedaneum is generally a benign condition, and it usually resolves within a few days and requires no treatment.

There are reported complications in infants with caput succedaneum that include necrotic lesions resulting in long-term scarring and alopecia. Halo scalp ring is an annular alopecic ring that occurs in infants after a prolonged or difficult labor due to compression from the bony prominence of the maternal pelvis. Rarely, systemic infection may occur as a complication of an infected caput succedaneum

Homer-Wright rosettes are characteristic structures found in certain types of tumors, such as neuroblastomas and medulloblastomas. These rosettes consist of a central core surrounded by tumor cells arranged in a circular pattern. The central core of Homer-Wright rosettes is filled with axons and dendrites, which are the extensions of nerve cells responsible for transmitting signals. This arrangement is a key feature of Homer-Wright rosettes and helps in the identification and diagnosis of these tumors.

Potter sequence is a condition characterized by a group of physical abnormalities that occur in a baby as a result of low amniotic fluid levels during pregnancy. The primary cause of Potter sequence is usually renal agenesis or renal hypoplasia, which refers to the absence or underdevelopment of one or both kidneys. These conditions lead to a decrease in the production of amniotic fluid, resulting in compression of the baby's body and causing the characteristic physical features associated with Potter sequence. Other factors such as lung aplasia, oligohydramnios, anal atresia, and esophageal atresia can also contribute to the development of Potter sequence, but renal agenesis and renal hypoplasia are considered the primary causes.

Cesarean section and maternal diabetes are factors that favor RDS development. Cesarean section can increase the risk of RDS because it prevents the natural squeezing of the baby's chest during vaginal delivery, which helps remove fluid from the lungs. Maternal diabetes can also increase the risk of RDS as it can lead to higher levels of insulin in the baby's bloodstream, which can inhibit the production of surfactant, a substance that helps keep the lungs inflated.

The hyaline membrane is located in the neonate's lungs with RDS in the alveolar ducts, respiratory bronchioli, and terminal bronchioli. These structures are part of the lower respiratory tract and are involved in the exchange of oxygen and carbon dioxide. The presence of hyaline membrane in these areas indicates inflammation and damage to the lining of the airways, leading to respiratory distress syndrome.

Sturge-Weber syndrome is a rare neurological disorder that is characterized by the presence of certain physical abnormalities. Leptomeningeal angiomas are one of the components of this syndrome, referring to abnormal blood vessels in the meninges, the membranes that cover the brain and spinal cord. Another characteristic feature of Sturge-Weber syndrome is the presence of a port wine stain, which is a birthmark that typically appears on the face. Therefore, the correct answer includes these two components that are commonly associated with Sturge-Weber syndrome.