Glucose and insulin infusion, calcium gluconate, sodium bicarbonate and nebulized salbutamol were tapered and stopped and potassium controlled with high dose of ion exchange resin. This occurs probably due to impaired ability to absorb liquid from airway surfaces. It remains uncertain if there is genotype—phenotype correlation, due to the rarity of the disease. PHA 1 can be differentiated clinically from CAH by an earlier onset and by no response to steroid therapy, given the delay in obtaining results for many hormonal assays. Ha, Y. Ranjith, S.
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CAH is the most frequent cause of severe dehydration, hyponatremia and hyperkalemia in the newborn period, and therefore this was our first diagnosis, supported by a initial high level of hydroxyprogesterone.
Print Send to a friend Export reference Mendeley Statistics. The most intriguing and interesting aspect of this association is represented not so much by the co-existence of the two diseases, often not easy to ascertain, as by the possibility that they can develop in the same patient over a lifetime.
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The PHA 1 phenotype of our patient was particularly severe, making normalization of electrolytes a challenging task, only possible with unusually high salt intake as well as continuous ion exchange resin. Therefore, the diagnosis of Marine-Lenhart syndrome remains difficult to be determined, and the simultaneous occurrence of the two diseases is still matter of debate 21 Clinicians should be aware of such a possibility, especially in those patients who are candidate to radioiodine treatment.
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c GDPN Doutor Sérgio Castedo, Porto, Portugal. This item has. de Endocrinologia da Faculdade de Medicina da Universidade de São Paulo ( FMUSP).
An uncommon case of MarineLenhart syndrome
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He was born at full term with a birth weight of g 10—25th percentile and there were no perinatal problems.
Hofmann, Z. Corresponding author. Tachibana, Y. Ranjith, S. The two diseases may coexist or may be present at different moments in the same patient.
Indian J Pediatr, 73pp.
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|Si continua navegando, consideramos que acepta su uso. The patient is currently 8. Marthinsen, A.
Since then he has had recurrences of fluid and electrolyte imbalances necessitating repeated short-term hospitalizations. Figure 4.