Very sick kid

[letmesleep]

Well, if we are at the hospital, then get your butt inside and transfer care to an ER doc, hang out and get the answer to our question......."WHAT THE HELL IS WRONG WITH THE BABY?"

Then write your report, jump through all your billing hoops, signature from the nurse, and hang out till your next partner and truck show up.

[Just Plain Ruff]

Here is what happened to the baby in the ER after you dropped off the baby into the arms of the new pediatric resident

Upon physical examination, he was dusky and floppy and had a temperature of 36.3° C, a heart rate of 170, a respiratory rate of 45, and a blood pressure of 64/45. He had not received any paralytic agents. His oxygen saturation levels were unobtainable. A subconjunctival hemorrhage was noted in the left eye, and his fontanelle was soft and flat. There were coarse breath sounds bilaterally, and his heart had a regular rhythm with no obvious murmur. His abdomen was firm and full, without bowel sounds. His liver edge was not palpable given his firm abdomen. His extremities were cool with thready pulses and a delayed capillary refill time of 5 to 6 seconds. In the ED, umbilical lines were placed for intravenous access, laboratory tests were made, and the baby was given doses of ampicillin and cefotaxime (50 mg/kg of each).

Initial laboratory evaluation demonstrated a pH of 6.73, base deficit of -30 mmol/L, glucose of

175 mg/dL, sodium of 133 mmol/L, potassium of 7.5 mmol/L, and hemoglobin of 14.2 gm/dL. Additional laboratory tests included a complete metabolic panel, lactate, ammonia, and blood and urine cultures. He was given a saline bolus of 10 mL/kg and sodium bicarbonate for hyperkalemia and his base deficit. A chest x-ray showed cardiomegaly (Figure 1). The patient's ammonia level was elevated at 578 micromole per liter (mcmol/L) with a lactate level of 24.5 mmol/L. Blood urea nitrogen level was 24 mg/dL with an elevated creatine level of 2.2 mg/dL.

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References

From Medscape Emergency Medicine

Cases in Pediatric Emergency Medicine

A Newborn Presenting in Extremis CME/CE

Posted 01/25/2008

Christy A. Meade, MD; Ghazala Q. Sharieff, MD

Disclosures

Introduction

Managing a toxic, decompensating newborn can be a stressful and difficult task. There are significant physiologic changes associated with birth, and multiple congenital abnormalities that can present shortly thereafter. The differential diagnosis can be immense and overwhelming. The mnemonic THE MISFITS can aid in the vast differential diagnosis (Table 1). This case discusses a 5-day-old who was brought to the emergency department (ED) with the chief complaint of difficulty breathing.

Table 1. THE MISFITS. Causes of Shock/Severe Illness in the Newborn

T

Trauma/nonaccidental trauma (NAT)

H

Heart disease - congenital

E

Electrolyte disturbances

M

Metabolic disturbances (congenital adrenal hyperplasia)

I

Inborn errors of metabolism

S

Sepsis

F

Formula dilution or over concentration

I

Intestinal catastrophes

T

Toxins (home remedies)

S

Seizures/central nervous system (CNS) abnormalities

Case Report

A 5 day-old-male is brought to the ED with tachypnea and increasingly labored breathing. He was born at full term and had no difficulties after birth. Prenatal care and ultrasound were unremarkable. He was with his parents in Mexico when they noticed that he did not awaken for his 6 AM feeding and that he had slept for 7 to 8 hours in the afternoon without urinating (no wet diapers during that time period). At about 8 PM, the parents noted that he was laboring to breathe by breathing rapidly and making gasping noises. The baby was taken to the closest Mexican clinic, where he was found to have an undetectable blood glucose level and to be in respiratory distress. An intravenous line was inserted, bolus doses of dextrose 10% in water were given, and he was intubated. He was then transported across the border to the closest US children's hospital.

Upon physical examination, he was dusky and floppy and had a temperature of 36.3° C, a heart rate of 170, a respiratory rate of 45, and a blood pressure of 64/45. He had not received any paralytic agents. His oxygen saturation levels were unobtainable. A subconjunctival hemorrhage was noted in the left eye, and his fontanelle was soft and flat. There were coarse breath sounds bilaterally, and his heart had a regular rhythm with no obvious murmur. His abdomen was firm and full, without bowel sounds. His liver edge was not palpable given his firm abdomen. His extremities were cool with thready pulses and a delayed capillary refill time of 5 to 6 seconds. In the ED, umbilical lines were placed for intravenous access, laboratory tests were made, and the baby was given doses of ampicillin and cefotaxime (50 mg/kg of each).

Initial laboratory evaluation demonstrated a pH of 6.73, base deficit of -30 mmol/L, glucose of

175 mg/dL, sodium of 133 mmol/L, potassium of 7.5 mmol/L, and hemoglobin of 14.2 gm/dL. Additional laboratory tests included a complete metabolic panel, lactate, ammonia, and blood and urine cultures. He was given a saline bolus of 10 mL/kg and sodium bicarbonate for hyperkalemia and his base deficit. A chest x-ray showed cardiomegaly (Figure 1). The patient's ammonia level was elevated at 578 micromole per liter (mcmol/L) with a lactate level of 24.5 mmol/L. Blood urea nitrogen level was 24 mg/dL with an elevated creatine level of 2.2 mg/dL.

Figure 1. Chest x-ray showing cardiomegaly.

Given his decompensated state, with cardiomegaly and elevated ammonia level, ED staff called for cardiology and genetics consultation. Repeat arterial blood gas prior to transfer to the newborn intensive care unit (NICU) showed a pH of 7.16 with a base deficit of -19 mmol/L.

An echocardiogram was performed upon arrival to the NICU that was consistent with hypoplastic left heart syndrome with aortic and mitral atresia. The ascending and transverse aorta were severely hypoplastic and there was severely reduced right ventricular function. An atrial septal defect was present with left to right flow and there was a moderate patent ductus arteriosis. The patient was started on prostaglandin E1 (PGE1) and dopamine for inotropic support, and the cardiothoracic surgery department was consulted.

Further discussion

The differential diagnosis of a decompensating newborn can be extensive and includes infectious causes, hypoglycemia or electrolyte disturbances, metabolic disorders, congenital adrenal hyperplasia, renal failure, hematologic disorders, gastrointestinal disorders, neurologic disease, and trauma. A few common tests may narrow the differential diagnosis, including glucose, electrolytes, complete blood count, ammonia, lactate, and chest and abdominal x-rays. This patient's major findings in the ED included hypoglycemia, acidosis, hyperammonemia, and cardiomegaly. These findings trigger concerns for cardiac or metabolic disease. However, there is always a concern for sepsis in a toxic newborn, and antibiotics should be given promptly.

Hypoglycemia in an infant is usually transient and secondary to hyperinsulinism or glycogen depletion. More persistent hypoglycemia can be derived from more significant illnesses, including congenital adrenal hyperplasia, growth hormone deficiency, panhypopituitarism, congenital hyperinsulinism, inborn errors of metabolism, hepatic disease, and sepsis. Hypoglycemia in a newborn should be treated with dextrose (0.1-0.2 g/kg/dose or 1-2 mL/kg/dose of 10% dextrose solution) immediately and if needed, a continuous infusion can be started (4-6 mg/kg/minute).

Hyperammonemia is the hallmark of an inborn error of the urea cycle. The most common presentation is a 2- to 5-day-old male who has poor feeding, emesis, and decreased mental status. Males are primarily affected by the X-linked disorder ornithine transcarbamylase deficiency. Although our patient did have an elevated ammonia level, acidosis from any cause results in a nonspecific downregulation of the urea cycle, which results in hyperammonemia.

Cardiac malformations account for approximately 10% of infant mortality and almost all pediatric cardiac-related deaths. The incidence of congenital heart disease is 8 to 10 per 1000 live births. The most common lethal heart defect in the neonatal period is hypoplastic left heart syndrome. This patient did have cardiomegaly with acidosis, which led to the concern for heart disease.

At birth, there is a transition from right-sided fetal circulation to left-sided circulation. The decrease in pulmonary vascular resistance results in closure of the ductus arteriosus, ductus venosus, and foramen ovale. Depending on their cardiac defect, infants may be in extremis with cyanosis, have respiratory failure, or be in shock. Left ventricular outflow tract obstructions are the most common subset of ductal-dependent lesions and include hypoplastic left heart syndrome, interrupted aortic arch, coarctation of the aorta, and aortic valve stenosis. Left ventricular outflow tract obstructions account for 12.4% of cases of congenital heart disease in infancy.

Hypoplastic left heart syndrome (HLHS) is a combination of mitral valve atresia, severe aortic valve stenosis, a hypoplastic left ventricle, a hypoplastic ascending aorta, a patent ductus arteriosus, and a patent foramen ovale. When pulmonary vascular resistance decreases after birth, the obstruction of the left ventricular outflow results in a higher percentage of fixed right ventricular output to the lungs instead of to the body. Although increased pulmonary blood flow results in higher oxygen saturation, systemic blood flow is decreased. Perfusion becomes poor and metabolic acidosis and oliguria develop. Coronary artery and cerebral perfusion also are dependent on systemic blood flow through the ductus arteriosus. Therefore, increased pulmonary blood flow results in decreased flow to the coronary arteries and brain, with a risk for myocardial or cerebral ischemia. PGE1 should be started as soon as possible at 0.05 to 0.1 mcg/kg/minute if ductal dependant congenital heart disease is suspected. These patients should be monitored closely as side effects include apnea (10%), hypotension, bradycardia, and seizures. Intubation may be necessary, especially if the patient must be transported. Patients should be transported to a center skilled in pediatric cardiac surgery. Oxygen saturations are expected to be lower (70s or 80s) and 100% oxygen should not be given. Higher oxygen saturations increase pulmonary blood flow, leading to pulmonary edema, and decrease systemic flow, resulting in acidosis.

Without staged reconstructive surgery or orthotopic heart transplantation, HLHS is uniformly fatal, usually within the first 2 weeks of life. Survival for a longer period occurs rarely and only with persistence of the ductus arteriosus. Orthotopic heart transplantation results in early and long-term success similar to that of staged reconstruction. Among low-risk patients who undergo staged reconstruction or transplant, survival at 5 years is approximately 70%. Most studies report neurodevelopmental disabilities in a significant number of patients who survive either staged surgical reconstruction or cardiac transplantation.

Our patient stabilized on PGE1 and underwent the first stage of reconstructive surgery. His course was complicated by 2 episodes of line sepsis and feeding difficulties. He was discharged to home after 4 weeks and is now awaiting further surgeries.