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P196 Acute abdominal pain in a child with intersexuality
  1. Andries Camelia
  1. No affiliatons

Abstract

Backgrounds and aims Intersexuality is defined as a condition in which people are born with sexual traits that are not specific to either male or female organisms. Sex is usualy defined by factors like: chromosomes, gonads, sex hormones, genitals (internal and external) and psycological traits. Two notions are mentioned: hermaphrodite (when a person has sexual organs common to male and female sex) and pseudohermaphrodite (when a person is born with primary sexual characteristics of a sex, but develops second sexual characteristics of the other sex).

From the many causes of intersexuality, the most common in the infant period is Congenital Adrenal Hyperplasia, by which the most frequent form is 21-Hidroxilase Defficiency (90%). There are two forms: simple virilization and salt-wasting form. The latter leads to defficency of both cortisol and aldosterone and excess of testosterone. In newborn girls it manifests with masculinization of the genital organs present at birth and with weight loss, anorexia, vomiting, hypothension, hypoglycemia, hyponatremia and hyperkalemia, manifested in the first 2 weeks of life. The treatment is glucocorticoid and mineralocorticoid replacement (for life), together with surgical management of ambiguous genitals (controversial).

Methods A female sex child aged 10 years is admitted to Grigore Alexandrescu Hospital for fever, vomiting, abdominal pain. The simptomathology started 1 week ago with abdominal pain. During the day she presented fever and persistant vomiting.

Personal pathological history born with female pseudohermaphroditism, diagnosed with 21-OH-defficiency at 2 months, salt-wasting-form, treated with Hidrocortisone and Fludrocortisone dayly, was performed Feminization Reconstructive Genitoplasty at 1 year age.

Clincally pale teguments, dry lips, 9 episodes of vomiting in 12 hours, diffuse pain in the abdominal area, spontaneous and at palpation. Paraclinically: leucocitosis, inflammatory syndrome, respiratory alkalosis, hypokalemia. Abdominal imaging and surgical consult exclude an acute abdomen.

Despite reequilibration with endovenous perfusion, antiemetic, analgesic and antibiotherapy treatment, the evolution is unfavourable (vomiting and abdominal pain persists).

Results An Addisonian crisis is considered and corticotherapy with intravenous Hidrocortisone therapy is initiated, with rapid improvement both clinically (vomiting and abdominal pain stop) and paraclinically (normalising of blood gas and electrolytes).

The case particularity is represented by the hypokalemia and alkalosis, not typical in an Addisonian crysys

Addisonian crysis is manifested with malaise, hypothension, hypoglycemia, hyperkalemia, hyponatremia. The treatment is a medical emergency and is represented by glucose and electrolytes replacement and intravenous corticotherapy with Hidrocortisone (it has both corticosteroid and mineralocorticoid effects). This is a case when corticotherapy proves to be life-saving.

Conclusions Even though abdominal pain is a frequent symptom, its causes are varriable. The treatment should be considered individually, particularly if the patient presents a less frequent disorder. The important thing for the outcome of the patient is the efficient collaboration between different medical specialities.

  • Intersexuality
  • 21-OH-defficiency
  • abdominal pain
  • corticotherapy

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