pediatrics specific disorders

7/28/2019 Pediatrics Specific Disorders

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Pediatrics

Specific disorders

1. Infants of diabetic mothers Mother is diabetic so has low insulin level in her circulation and blood sugar is high. But the

fetus has no problem in producing insulin. Actually they have high level of insulin whichincrease their calorie intake. As we know insulin is also a growth hormone. So when the

baby is born and the cord is clumped then the circulating insulin level in baby is high in

relative to level of glucose which predisposes the baby to hypoglycemic state.

Clinical signs and symptoms in newborns:

1. Macrosomia= 5pounds, 8 ounces or 8 pounds 5ounces2. Ruddy and Plethoric = inc. red cells and inc. hemoglobin3.

Jitteriness, seizures

4. Electrolyte imbalance esp. calcium and magnesium5. Inc. bilirubin which increases risk of jaundice

- Due to hypoglycemeia the enzymes needed for the bilirubin is decreased so incamount of bilirubin

6. Increase risk of RDS7. Cardiac problem esp hypertrophic cardiomyopathy which resolves by 6 months of age8. GI problem

- Lazy left colon syndrome i.e. baby donot pass meconium Treatment:

- Mother most be treated for hyperglycemia- Blood sugar of baby most be monitored and if necessary IV glucose solution such as

Dextrose must be given


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2. Respiratory distress syndrome Etiology: lack of surfactant which is made by type 2 pneumocytes

Preterm baby don not have enough surfactant

Initial APGAR score can be normal but after few hours the baby begin to show respiratorydistress

Best clinical symptom: GRUNTING Best initial test: CHEST X-RAY

- Ground-glass appearance due to collapsing of alveoli and lung looks solid so lunglooks wider

- Heart border not clear- Airbronchograms can be seen

Best initial treatment- supportive- If necessary intubate the baby- Give surfactant and antibiotics if infection is suspected

Prevention- Try to make the baby to term by giving the steroids to mother before labor.- Steroids help in maturing the lungs

3. Transient tachypnea of the newborn(TTN) Due to retained fluid in the lungs and baby tries to breathe faster Infant is term, usually in 2nd stage of labor or C/S Treatment: oxygen and after that supportive This resolves within couple of days itself X-ray looks normal

Airbronchograms


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4. Meconium aspiration syndrome(MAS) Etiology: stress for baby due to asphyxia or hypoxia which results in passing of stool by fetus

in the amniotic fluid

Complications:- Aspiration pneumonia- due to breathing o meconium by baby- Persistent fetal circulation- due to shunting of the blood- Primary pulmonary hypertension of newborn due to hypoxia and vasoconstriction

during stress.

Diagnosis: meconium seen when amniotic fluid is ruptured Treatment: suction and supportive X-ray: uniform distribution of infiltrates in contrast to pneumonia

5. Diaphragmatic hernia Displacement of abdominal content into the thoracic cavity through a defect in the

diaphragm>

Pathophysiology:- Ipsilateral pulmonary hypoplasia results from compression of affected lung by the

displaced GI organs. A shift of mediastinal structures, resulting in compression of

contralateral lung may cause hypoplasia of that lung to a lesser degree.

Clinical features:- Severe respiratory distress with cyanosis and dyspnea is seen shortly after the birth- Breath sounds are heard in the chest- Scaphoid abdomen is seen due to distention of the bowel contents

Diagnosis: chest X-ray can demonstrate the air-filled bowel in left hemithorax.


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Gastrointestinal/ hepatobiliary

1. Meconium ileus- cystic fibrosis if otherwise proven, dilated loops of bowel in X-ray

2. Imperforated anus- can be seen grossly3. Hirschsprung disease: no ganglion cells in tissue biopsy(definitive)

Megacolon in X-ray

4. Traceho-esophageal fistulas: obstruction in esophagus, air in stomach, should considerfor other problems such as cardiac problems. Baby presents with choking, coughing,

gagging, vomiting because the food is aspirated to lung.


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5. Duodenal atresia: double bubble in X-ray, no distal air is present after the site ofobstruction, bilious vomiting after 1

stfeed

6. Necrotizing enterocolitis: preterm, low APGAR, bloody stool, abdominal distention,shocky baby, air can be seen in side wall of bowel

7. Jaundice:i. Physiologic jaundice: not seen in 1st24 hours of life, bilirubin level doesnt go

above 12.9-13, peak at 5-6th

day and goes away at 7-10th

day or upto 2weeks.

Caused due to indirect hyperbilirubinemia i.e. due to high circulating RBCs and

Hb. Inc. turnover of RBC-Inc. bilirubin. Immature liver cant metabolize the

bilirubin so jaundice


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ii. Pathologic jaundice

iii.

pediatrics specific disorders

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