Cyclic vomiting syndrome
Is an idiopathic disorder that typically presents in children 4-7 years old.
Repeated, intermittent, stereotypical bouts of severe nausea, vomiting, and lethargy, which are self-limited and have no apparent cause.
It is associated with a family (or personal) history of migraines. Supportive therapy may be required during acute episodes, and some evidence suggests the use of antimigraine medications may be beneficial as prophylaxis.
Intestinal malrotation
presents in newborns with bilious emesis, an upper gastrointestinal series demonstrating a misplaced duodenum, and a “corkscrew” appearance of the distal duodenum and proximal jejunum.
What is the best management to minimize the progression to amblyopia in the evidence of strabismus?
The unaffected eye should be occluded or use Cycloplegic and mydriatic eye drops to “recruit,” and encourage use of the affected eye.
Granulomatous infantiseptica
Listeria monocytogenes infection
the neonatal presentation is severe and fatal in 20 to 30% of cases.
Common manifestations in the newborn include sepsis, pneumonia, meningitis, and a pathognomonic finding, widespread microabscesses, and granulomas.
Patient presents at one minute of life with a pulse of < 100/min and an Apgar score of 5. The best next step in management is?
Positive-pressure ventilation and reassessment of his Apgar score at 5 minutes.
Intussusception
Acute abdominal distress and jelly stools “target sign” on ultrasonography.
Pneumatic enema is very effective at diagnosing and reducing intussuscepted sections of bowel.
Neonates with meconium ileus should undergo?
Sweat testing to evaluate for underlying cystic fibrosis.
Signs of duodenal atresia occur?
Immediately after birth with bilious emesis and no abdominal distention.
Neonatal malrotation with volvulus
Occurs due to an incomplete malrotation of the intestines during the 7-12th weeks of development. Patients will feed well until 3-7 days of life when bilious vomiting, abdominal distention, and rapid decompensation may occur.
Complications: intestinal ischemia, infection, necrosis, perforation of bowel, and ultimately shock.
A corkscrew pattern in an upper gastrointestinal series. This is a common finding of midgut volvulus.
Pediatric patient postprandial non-bilious vomiting, persistent appetite, and “olive-like” mass is highly suggestive of?
Pyloric stenosis, is due to hypertrophy of the pylorus muscle.
symptoms appearing around 3-to-5 weeks of life
Nonbilious projectile vomiting immediately after feeding
Often followed by eagerness to eat again (“hungry vomiter”)
Risk factor: exposure to macrolide antibiotics
Ebstein anomaly
is a congenital heart defect characterized by a malformed tricuspid valve displaced toward the apex of the right ventricle (RV), leading to an abnormally large right atrium (RA) and small RV.
Associated to mother’s lithium consumption during pregnancy.
pharmacology treatment of Infantile capillary hemangiomas
If the hemangioma is ulcerated or blocking vital organs, then urgent treatment with a beta-blocker (either topical timolol or oral propranolol) is recommended.
Meconium ileus
90% of cases have cystic fibrosis
Patients typically present in the first three days of life with bilious vomiting and failure to pass meconium in the first 48 hours of life, dilated proximal bowel on abdominal radiograph with visualization of a microcolon on contrast enema.
Delayed puberty
Absence of breast enlargement by age 13 in girls and the lack of testicular enlargement by age 14 in boys.
Neonatal gonococcal conjunctivitis treatment and prophylaxis?
Prophylaxis with erythromycin ointment
Treatment is with IM ceftriaxone
Most common congenital heart defect in Down syndrome?
Complete atrioventricular septal defect (CAVSD), caused by failure of endocardial cushions to merge, leading to both ventricular septal defect (VSD) and atrial septal defect (ASD).
Henoch-Schönlein purpura IG complications
It increases the risk of ileoileal intussusception due to intestinal edema and bleeding.
Neonatal polycythemia
Hematocrit >65% in term infants.
Causes:
Increased erythropoiesis due to intrauterine hypoxia (maternal diabetes, hypertension, smoking, intrauterine growth restriction)
Erythrocyte transfusion (delayed cord clamping, twin-twin transfusion)
Genetic/metabolic diseases (hypothyroidism/hyperthyroidism, genetic trisomy)
Asymptomatic (most common)
Ruddy skin
Hypoglycemia, hyperbilirubinemia
Respiratory distress, cyanosis, apnea
Irritability, jitteriness
Abdominal distension
Treat: Intravenous fluids, glucose and partial exchange transfusion
Galactosemia
AR due to galactose-1-P uridylyltransferase (GALT) deficiency.
Symptoms: poor feeding and vomiting, typically arise within days of life after galactose introduction via breast milk or formula, jaundice, hepatomegaly, and potential cataract formation.
Increased risk of Escherichia coli sepsis.
Hypoglycemia, metabolic acidosis, elevated transaminases, and conjugated hyperbilirubinemia, with unconjugated hyperbilirubinemia likely caused by galactose 1-phosphate accumulation in RBCs, leading to hemolytic anemia.
Congenital cytomegalovirus infection
The most common congenital infection
Hepatosplenomegaly, jaundice, periventricular calcifications, microcephaly, and thrombocytopenia.
About 10% of affected infants are symptomatic. The primary long-term effect is sensorineural hearing loss.
Breastfeeding contraindications
Maternal: active untreated tuberculosis, HIV infection, herpetic breast lesions, active varicella infection, chemotherapy or radiation therapy, active substance use disorder.
Infant: galactosemia
Neonatal conjunctivitis
Gonococcal: 2-5 days, profuse purulent discharge, marked conjunctival injection & eyelid edema, treated with IM/IV cefotaxime (single dose).
Chlamydial: 5-14 days, watery, serosanguineous, or mucopurulent eye discharge, mild eyelid swelling, treated with oral erythromycin.
A new born with a lower postductal oxygen saturation than preductal saturation suggests?
A lower postductal (PDA) oxygen saturation (right foot) than preductal saturation (right hand) suggests persistent pulmonary hypertension, for example in meconium aspiration syndrome.
Klumpke palsy
A complication of shoulder dystocia resulting from injury to the eighth cervical and first thoracic nerves, leading to hand paralysis (clawhand) and ipsilateral Horner syndrome.