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complications newbrn
misc. comp. of new born: pku, down syndrome, hemolytic, vent. hem., diabetic mo
Question | Answer |
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PKU | A genetic disorder that causes central nervous system damage from toxic levels of the amino acid phenylalanine in the blood. |
how often does it occur in infants and how often is it screened? | All newborns are screened for this condition. Incidence: the U.S. is 1 in 10,000 to 25,000 live births. |
what causes PKU? | A deficiency of the liver enzyme phenylalanine hydrolase, which is needed to convert phenylalanine to tyrosine. It is an autosomal recessive disorder. |
S/S of Down Syndrome are what? | Feeding difficulties. Vomiting. Hypertonia. Irritability. Infant has eczema and musty odor of urine. |
S/S in older children are what? | Older Children: Eczema. Hypertonia. Hyperactive behavior. Mental retardation. Seizures. Hypopigmentation of the hair, skin, and irises. |
what is therapeutic mgm for PKU? | Further evaluations. Low-phenylalanine diet. Special formula for infants. Diet is primarily fruits, vegetables, and starches with a phenylalanine-free protein supplement. Small amounts of phenylalanine are allowed. |
Nursing considerations for PKU are mainly prevention. Name two: | Ensure newborns are screened for PKU at the appropriate time. Assist parents in regulating the diet to meet infant’s changing phenylalanine needs. |
Question: What foods must be avoided in a child with PKU? | PKU can lead to brain damage |
Question: Why is early detection of PKU crucial? | food high in phenyl alinine: hamburgers... |
Chromosomal significance | Most common chromosomal abnormality of a generalized syndrome. |
how often does it occur | 1 in every 800 to 1000 live births. Occurs slightly more often in white than blacks. |
Age as a risk factor for D.S. | Statistically greater risk when maternal age > 35 or paternal age > 55. |
Down Syndrome Etiology | There are three phenotypes: Trisomy 21 (nonfamilial trisomy 21). Translocation of chromosomes 15 and 21 or 22. Mosaicism. |
Trisomy 21 | Attributable to an extra chromosome 21. Occurs in about 95% of all cases. |
Translocation of chromosomes 15 and 21 or 22 | This type of genetic aberration is usually hereditary. Not associated with advance parental age. Occurs in about 3% to 4% of cases. |
Mosaicism | Refers to cells with both normal and abnormal chromosomes., Occurs in 1% to 2% of cases., degree of phys./cogn. impairment is r/t the % of cells w/ abnormal chromosome makeup |
Diagnostic Evaluation | Alpha-fetoprotein (AFP) screening. Multiple Marker screening. Chorionic villus sampling. Amniocentesis. Nuchal translucency. |
clinical manifestations in the head: | Separated sagittal suture. Brachycephaly. Skull rounded and small. Flat occiput. Enlarged anterior fontanel.Face: flat profile. |
clinical manifestations in the eyes & nose: | Eyes: Oblique palpebral fissures (upward, outward slant). Innerepicanthal folds. Speckling of iris. Short, sparse eyelashes.Nose: small, depressed nasal bridge (saddle nose). |
clinical manifestations of the mouth | Mouth: High, arched, narrow palate. Protruding tongue that may be fissured at lip and furrowed on surface. Hypoplastic mandible. Downward curve (especially when crying). Mouth kept open. |
heads and chest: | Hands: Broad, short stubby fingers. Incurved little finger. Transverse palmar crease. Increased ulnar loops on fingers. Chest: Shortened rib cage. Twelfth rib anomaly. |
feet and musculoskeletal | Feet: Wide space between big and second toes. Plantar crease between big and second toes. Broad, stubby, and short. Musculoskeletal: Short stature. Hyperflexibility, muscle weakness. Hypotonia. |
Other manifestations | Abdomen: Protruding, muscles lax and flabby. Diastasis recti. Umbilical hernia Congenital heart malformation (septal) Respiratory tract infections. Dysfunction of immune system, congenital hypothyroidism Increased incidence of leukemia. |
Therapeutic Management | No cure exists. Regular medical care and evaluations. |
Nursing Considerations | Family support and education. Allow parents to express concerns. Involve parents in infant care to promote bonding. Involve siblings in infant care and include them in discussions. Refer parents to others for help or advice. |
What is Trisomy 21? | |
Causes: Incompatability between blood of mother and fetus. Rh incompatibility. ABO incompatibility causes | Hemolytic Disease of the Newborn |
Therapeutic Management of hemolytic disease | Focus is prevention of Kernicterus. Diagnosis is determined by history and diagnostic tests. Indirect Coombs test in mother. Direct Coombs test in infant. Serum bilirubin levels. Transcutaneous bilirubin. |
Periventricular-Intraventricular Hemorrhage | Defined as bleeding around and into the ventricles of the brain. |
patho of Peri/Intrventricular Hem.- | Occurs most often in infants of less than 32 weeks or weight less than 1500g. First few days are the most common time for hemorrhage to occur. Results from rupture of the fragile blood vessels around the ventricles. |
periventricular-inntraventricular Hemorrhage is associated with what disease or disorders? | Hypoxic injury to the vessels. Increased or decreased blood pressure. Increased or fluctuating cerebral blood blow. Rapid blood volume expansion. Hypercarbia. Anemia. Hypoglycemia. |
PIVH Manifestations | Lethargy. Poor muscle tone. Deterioration of respiratory status with cyanosis or apnea. Drop in hematocrit levels. Decreased reflexes. Full or bulging fontanelles. Seizures. |
PIVH Therapeutic Management | Ultrasonography on preterm infants. Treatment is supportive. Hydrocephalus may develop. |
The nurse should consider: | Head circumference. Observation. Minimal handling. Reduced environmental stressors. Parental support and teaching. Care may increase cerebral blood flow and blood pressure. Be alert for early signs of PIVH. |
Congenital anomalies are 3x more likely in infants with diabetic moms. what are the most frequent anomalies in infants with diabetic mothers? | Cardiac. Urinary tract. Gastrointestinal. Neural tube defects. Caudal regression syndrome. Cardiomegaly is common and may lead to heart failure. |
what thought is treatment based on? | The incidence of anomalies is less if blood glucose levels remain within normal limits, especially before conception and in the early weeks of gestation. |
DM Manifestations | Infant may be SGA. One-third are macrosomic. Fetal pancreas secretes large amounts of insulin. Hypoglycemia. Risk for injury during birth. Higher risk of asphyxia and RDS. |
Other complications: | Hypocalcemia. Low magnesium levels. Polycythemia. Increased likelihood of premature delivery. |
IDM Therapeutic Management | Includes controlling the mother’s diabetes throughout the pregnancy to decrease complications. Be prepared for shoulder dystocia or cephalopelvic disproportion and C- Section. |
IDM Nursing Considerations | Assessment: Signs of complications, trauma, and congenital anomalies at delivery and during transition. Respiratory problems. Hypoglycemia. Rapid respirations, low temp and poor muscle tone. |
What are some nursing interventions | Interventions: Monitor glucose levels. Feed infant early. Parental support. |
Why is the infant of a diabetic mother often hypoglycemic after delivery? |