jean robert
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Ultrasound examination in utero reveals bilateral absence of the kidneys in fetus at 34 weeks of gestation. The baby is born alive at term but dies 12 hours after birth. Which of the following is the most likely cause of death?
A.-Lung immaturity
B.-Anuria
C.-Cardiomyopathy
D.-Hydrocephalus
E.-Internal bleeding
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| new_n_lost
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A.-Lung immaturity <== Potter's Syndrome
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| keepgoing
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??I thought anuria....
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| Ms.Apara
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[quote=Ms.Apara]Development of the mature kidney begins between weeks 5 and 7 of gestation. Fetal urine production begins in early gestation and comprises the majority of the amniotic fluid in the second and third trimesters of pregnancy. The fetus continuously swallows amniotic fluid, which is reabsorbed by the gastrointestinal tract and then reintroduced into the amniotic cavity by the kidneys via urination. Oligohydramnios occurs if the volume of amniotic fluid is less than normal for the corresponding period of gestation. The fetal urine is critical to the proper development of the lungs by aiding in the expansion of the airways - alveoli, by means of hydrodynamic pressure and by also supplying proline which is a critical amino acid for lung development. Alveoli are the small sacs in the lungs that exchange oxygen with the blood. If the alveoli, and thereby the lungs, are underdeveloped at the time of birth the infant will not be able to breathe air properly and will go into respiratory distress shortly after birth due to pulmonary hypoplasia (underdeveloped lungs). This is the primary cause of death to Potter syndrome infants secondary to renal failure. The fetal urine also serves to cushion the fetus from being compressed by the mother's uterus as it grows.
?????????
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| usmle4me
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Agree with A. Lung immaturity.
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| new_n_lost
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Ms.Apara wrote:
[quote=Ms.Apara]Development of the mature kidney begins between weeks 5 and 7 of gestation. Fetal urine production begins in early gestation and comprises the majority of the amniotic fluid in the second and third trimesters of pregnancy. The fetus continuously swallows amniotic fluid, which is reabsorbed by the gastrointestinal tract and then reintroduced into the amniotic cavity by the kidneys via urination. Oligohydramnios occurs if the volume of amniotic fluid is less than normal for the corresponding period of gestation. The fetal urine is critical to the proper development of the lungs by aiding in the expansion of the airways - alveoli, by means of hydrodynamic pressure and by also supplying proline which is a critical amino acid for lung development. Alveoli are the small sacs in the lungs that exchange oxygen with the blood. If the alveoli, and thereby the lungs, are underdeveloped at the time of birth the infant will not be able to breathe air properly and will go into respiratory distress shortly after birth due to pulmonary hypoplasia (underdeveloped lungs). This is the primary cause of death to Potter syndrome infants secondary to renal failure. The fetal urine also serves to cushion the fetus from being compressed by the mother's uterus as it grows.
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Ur resources plz cos i have couple of them that say otherwise
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| Ms.Apara
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http://en.wikipedia.org/wiki/Potter_syndrome
Edited by Ms.Apara on 04/27/07 - 11:18 AM
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| keepgoing
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| jean robert
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A  you re right nnl.
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| new_n_lost
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Jean................ Plz Post the Explanations Plz it will help everyone
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| new_n_lost
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Potter syndrome is usually fatal in the first few days of the patient's life; most often, the cause is pulmonary failure. Neonates with the Potter sequence have an increased morbidity rate because of respiratory failure, pneumothorax, and acute renal failure during the neonatal period. During early childhood, patients may have chronic lung disease or chronic renal failure.
http://www.emedicine.com/ped/topic1878.htm
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| Ms.Apara
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My post was to share some extra info.. which compelled me to give a second thought..otherwise i shall choose A
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| jean robert
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EXPLANATION
The patient described in the question stem suffers bilateral renal agenesis,which invariably leads to oligohydramnios or anhydramnios and results in the POTTER SYNDROME.
THE SIGNS OF POTTER SYNDROME CAN RESULT FROM ANY CAUSE OF PERSISTENT OLIGOHYDRAMNIOS.
Theses signs include: 1.-Clubfeet 2.-Classic facies consisting of nasal,ocular,ear,jaw deformities and pulmonary hypoplasia.By definition,true Potter syndrome results from bilateral renal aplasia and is a very rare condition;a similar presentation caused by other etilogies is called the POTTER SEQUENCE.
The amniotic fluid volume is maintained primarily by fetus. The fetus swallows amniotic fluid,which is then absorbed by the fetal GI tract and released by the fetal urinary system back into amniotic fluid space. Swallowed amniotic fluid also contributes to the development and maturation of the fetal lungs.Infants with prolonged oligohydramnios often suffer from respiratory distress due to pulmonary hypoplasia.
Respiratory failure and renal failure cause death within hours of birth in 100% of infants born with bilateral agenesis.
The take-home message: RESPIRATORY FAILURE is the mcc of Death of children with POTTER syndrome.They typically pass away within hours of birth.
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