Articole semnate de acelasi autor in Revista Romana de Pediatrie:

HAIR-AN syndrome – favorable outcome after one year treatment

Revista Romana de PEDIATRIE | Volumul LX, Nr. 3, An 2011

ISSN 1454-0398 | e-ISSN 2069-6175
ISSN-L 1454-0398
DOI: 10.37897/RJP

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Adela Emandi Chiriţă, Corina Duncescu, Ramona Stroescu and Ioana Micle

REZUMAT

Introduction: HAIR-AN syndrome is a subphenotype of polycystic ovary syndrome and is characterized by hyperandrogenism (HA), insulinresistance (IR) and acanthosis nigricans (AN) associated with obesity. It is not a rare disease. The annual incidence worldwide is estimated at around 5% of adolescent girls. Undiagnosed and untreated insulin resistance is linked to long-term complications, such as coronary artery disease, dyslipidemia, and diabetes.

Aim: We report a case of HAIR-AN syndrome in an adolescent girl with favorable outcome after one year of treatment.

Methods: The patient presented at 14 years 10 months with obesity, acanthosis nigricans, hirsutism, acne and irregular menses. She received recommendations for weight loss and treatment with metformin, progesterone, cyproterone acetate and topic agents for acne. She came regularly to follow-up every 3 months.

Results: Her blood pressure was normal for age. Her blood work revealed insulin resitance (HOMA-IR = 6,38) and elevated DHEAS. Abdominal ultrasound showed polycystic ovary. After 3 month of treatment the insulin resistance was substantially reduced, while her hirsutism, testosterone and DHEAS increased dramatically, with decrease of SHBG. At that moment we performed abdominal MRI, that infirmed the presence of adrenal tumor or adrenal rest tumors in abdomen. We increased metformin dosage (2 g/day) and continued to offer counseling to improve weight loss (psychologist involvement). After one year, her BMI decreased by 6.6 kg/m2 , along with her insulin resitance and androgen levels.

Conclusions: Early diagnosis and treatment of HAIR-AN syndrome may improve the quality of life of patients and decrease risk of future complications. Weight loss has a major impact on metabolic parameters.

Key words: HAIR-AN syndrome, adolescent, metformin, polycystic ovaries syndrome

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Adela Emandi Chiriţă

SINDROMUL HAIR-AN – EVOLUTIE FAVORABILA DUPA UN AN DE TRATAMENT

ISSN 1454-0398 | e-ISSN 2069-6175
ISSN-L 1454-0398
DOI: 10.37897/RJP

National Awards “Science and Research”

NEW! RJP has announced the annually National Award for "Science and Research" for the best scientific articles published throughout the year in the official journal.

ICMJE- Recommendations

Read the Recommendations for the Conduct, Reporting, Editing, and Publication of Scholarly work in Medical Journals.

Promoting Global Health

Adela Emandi Chiriţă, Corina Duncescu, Ramona Stroescu and Ioana Micle

REZUMAT

Introducere: Sindromul HAIR-AN este subfenotip al sindromului ovarelor polichistice şi este caracterizat prin hiperandrogenism (HA), insulinorezistenţă (IR) şi acantozis nigricans (AN), asociate cu obezitate. Nu este o boală rară. Incidenţa anuală la nivel mondial este estimată la aproximativ 5% dintre adolescente. Insulinorezistenţa nediagnosticată şi netratată presupune riscuri metabolice pe termen lung, cum ar fi boala coronariană, dislipidemia şi diabetul zaharat.

Scop: Prezentăm un caz de sindrom HAIR-AN la o adolescentă, cu evoluţie favorabilă, după un an de tratament.

Metodă: Pacienta s-a prezentat la spital la 14 ani şi 10 luni pentru obezitate, acantozis nigricans, hirsutism, acnee şi menstre neregulate. A primit recomandări pentru obezitate şi tratamentul cu metformin, progesteron, ciproteron acetat şi tratament topic local pentru acnee. Trimestrial s-a prezentat la control.

Rezultate: Tensiunea arterială a fost normală pentru vârstă. Analizele de laborator au arătat insulinorezistenţă (HOMA-IR = 6,38) şi DHEAS crescut. Ecografia abdominală a relevat ovare polichistice. După 3 luni de tratament, insulinorezistenţa s-a redus substanţial, în timp ce hirsutismul s-a accentuat, testosteronul şi DHEAS au crescut dramatic, alături de scăderea SHBG. A efectuat RMN-ul abdominal, care a infirmat prezenţa unei tumori suprarenale sau a unor incidentaloame în abdomen. Am crescut doza metformin (2 g/zi) şi a continuat consilierea pentru a îmbunătăţi pierderea în greutate. După un an, IMC a scăzut cu 6,6 kg/m2 . Insulinorezistenţa şi nivelurile de androgeni au scăzut până aproape de nivelurile de referinţă.

Concluzii: Diagnosticul precoce şi tratamentul sindromului HAIR-AN poate îmbunătăţi calitatea vieţii pacientelor şi scade riscul de complicaţii. Pierderea în greutate are un impact major asupra parametrilor metabolici.

Cuvinte cheie: sindrom HAIR-AN, adolescentă, metformin, sindromul ovarelor polichistice

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Adela Emandi Chiriţă