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A newborn male is brought to you in the neonatal intensive care unit (NICU). On physical examination, you notice that the infant has deficient abdominal musculature and undescended testes. Your suspicion is high for a certain condition.

You presumptively diagnose the child with which of the following?

  1. VATER association
  2. Cushing's triad
  3. Potter's syndrome
  4. Jones criteria
  5. Eagle-Barrett syndrome

Answer(s): E

Explanation:

The constellation of cryptorchidism, posterior urethral valves, and abnormal abdominal musculature is called Eagle-Barrett syndrome. Another name is prune belly syndrome. The greatest morbidity comes from the poor amniotic fluid production, due to bladder outlet obstruction, with a resulting pulmonary hypoplasia. Cushing's triad are hypertension, bradycardia, and widened pulse pressure. This is seen as terminal findings associated with increased intracranial pressure. VATER association has multiple anomalies, none of which are the three mentioned. VATER is a mnemonic which stands for Vertebral anomalies, Anal atresia (imperforate), Tracheo-Esophageal fistula, and Renal anomalies (the R also indicated Radial anomalies). It is sometimes referred to as VACTERL association in which the C indicates Cardiac anomalies with the L indicating Limb anomalies. Potter's syndrome is bilateral renal agenesis. This condition is fatal, due to marked pulmonary hypoplasia. The Jones criteria are used in the diagnosis of ARF.



A newborn male is brought to you in the neonatal intensive care unit (NICU). On physical examination, you notice that the infant has deficient abdominal musculature and undescended testes. Your suspicion is high for a certain condition.

Upon further imaging, what associated finding would be expected?

  1. posterior urethral valves on a voiding cystourethrogram (VCUG)
  2. hydrocephalus on head ultrasound
  3. cardiomegaly on chest x-ray (CXR)
  4. bilateral adrenal enlargement on abdominal ultrasound
  5. tracheoesophageal fistula on an upper gastrointestinal (UGI) series

Answer(s): A

Explanation:

The constellation of cryptorchidism, posterior urethral valves, and abnormal abdominal musculature is called Eagle-Barrett syndrome. Another name is prune belly syndrome. The greatest morbidity comes from the poor amniotic fluid production, due to bladder outlet obstruction, with a resulting pulmonary hypoplasia. Cushing's triad are hypertension, bradycardia, and widened pulse pressure. This is seen as terminal findings associated with increased intracranial pressure. VATER association has multiple anomalies, none of which are the three mentioned. VATER is a mnemonic which stands for Vertebral anomalies, Anal atresia (imperforate), Tracheo-Esophageal fistula, and Renal anomalies (the R also indicated Radial anomalies). It is sometimes referred to as VACTERL association in which the C indicates Cardiac anomalies with the L indicating Limb anomalies. Potter's syndrome is bilateral renal agenesis. This condition is fatal, due to marked pulmonary hypoplasia. The Jones criteria are used in the diagnosis of ARF.



What is the most sensitive indicator of pneumonia in a child?

  1. tachycardia
  2. tachypnea
  3. hypotonia
  4. vomiting
  5. coughing

Answer(s): B

Explanation:

Tachypnea is the most sensitive clinical parameter for diagnosing a lower respiratory tract infection. The child may have tachycardia from a fever or anxiety but, typically, not simply from pneumonia. Coughing is common in children with pneumonia but it is not specific for lower respiratory tract infections, as it can also be seen in upper and lower respiratory tract infections



A 12-month-old male child is brought to your office for a well-child examination and immunizations. You have been following the child since delivery and are aware that he has acquired immune deficiency syndrome (AIDS) and a markedly reduced T-cell count. Which of the following vaccinations should he not receive?

  1. diphtheria, tetanus, and acellular pertussis (DTaP)
  2. diphtheria tetanus (dT)
  3. hepatitis B
  4. injectable polio vaccine (IPV)
  5. varicella

Answer(s): E

Explanation:

All of the vaccines mentioned, except varicella, are killed or synthetic vaccines. Varivax is an attenuated varicella strain (OKA) from Japan. Live virus vaccines are contraindicated in the case of AIDS with a markedly reduced T-cell count. The IPV is the inactivated version of the live polio vaccine, oral polio vaccine (OPV).






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