::INDO AMERICAN JOURNAL OF PHARMACEUTICAL SCIENCES::

CODEN : IAJPBB
issn 2349-7750

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TITLE:

ANALYSIS OF CONGENITAL ADRENAL HYPERPLASIA WITH CHOLESTATIC JAUNDICE IN PAKISTAN

AUTHORS:

Dr Zarka Batool, Dr Imama Jamal, Dr Qamar Abbas

ABSTRACT:

Introduction: The term Congenital Adrenal Hyperplasia (CAH) describes a group of autosomal recessive disorders with a defect in the biosynthesis of Cortisol, and presents with consequent over production of adrenocorticotropic hormone (ACTH) and secondary adrenal hyperplasia. Aims and objectives: The basic aim of the study is to analyze the cases of congenital adrenal hyperplasia with cholestatic jaundice in Pakistan. Material and methods: This case study analysis was conducted in DHQ hospital, Layyah during October 2018 to December 2018. The data were collected from 10 infants from the hospital. All were full-term babies of consanguineous parents with normal birth weights. The postnatal period was uneventful apart from jaundice that started during the first week. The neonate was an outcome of consanguineous marriage, born at term through a normal vaginal delivery, with a birth weight of 3.15 kg. Results: The data was collected from 10 infants from which 4 males and 6 females. On examination, child was noticed to have a dark complexion in comparison to the parents and was icteric. Weight at the time of presentation was 2800 gm, length 54 cm and Occipital Frontal Circumference of 36 cm; blood pressure was 68/46mmHg (90th centile). Systemic examination showed some dehydration, but was otherwise normal with no visceromegaly appreciated. External genitalia were darkly pigmented, with normal phallus (3cm in length) and bilaterally descended testis. Conclusion: It is concluded that AH may present itself with persistent conjugated hyper-bilirubinaemia leading to difficulty in its diagnosis, with an extensive workup or even surgical intervention required.