A Rare Case of Congenital Partial Hypopituitarism Presenting with Portal Hypertension and Complete Heart Block

Program: Abstracts - Orals, Featured Poster Presentations, and Posters
Session: SAT 164-196-Pituitary
Basic/Clinical
Saturday, June 15, 2013: 1:45 PM-3:45 PM
Expo Halls ABC (Moscone Center)

Poster Board SAT-164
Shilpa Abhay Chaudhari1, Shardul Gadhia*2, Ashvin Butala1 and Alan Scott Sacerdote3
1Woodhull Medical Center, Brooklyn, NY, 2Woodhull Medical Center, Brooklyn, 3NYU School of Medicine, New York, NY
A 28 year old male with  a history of cogenital panhypopituitarism ( not currently on any hormonal replacement), and  who is an ex- alcohol abuser  presented to the Emergency Department complaining of hematemesis x 1 day associated with lightheadedness and weakness. Stool and nasogastric lavage were  positive for occult blood. On upper GI endoscopy the patient was found to have multiple varices on the distal esophagus and  stomach fundus. Immediate banding was done to control bleeding and patient was transferred to ICU. In ICU, EKG showed 3rd degree complete heart block ( previous EKGs are unavailable). A pacemaker was placed.  CT abdomen showed nodular liver contour compatible with  cirrhosis, and a  markedly enlarged spleen. Further work  up for panhypopituitarism yielded the following results: FSH by chemiluminescence=0.94 mIU/L (1.6-8.0), LH by chemiluminescence<0.07 mIU/L (1.5-9.3), TSH by chemiluminescence 0.21 mIU/L (0.4-4.5), ACTH  by chemiluminescence 52 pg/ml (6-50), Total Testosterone by liquid chromatography tandem mass spectrometry (LCMS/MS) 9 ng/dl (250-1100)’, IGF-1 by liquid chromatography mass spectrometry LC/MS 33 ng/ml (114-492) which is suggestive of partial hypopituitarism.

Discussion:

The mechanism of liver injury in congenital hypopituitarism is still unknown.  It is hypothesized that cortisol and/or growth hormone deficiency both play a role in delayed maturation of bile acid synthesis and transport, and this leads to cholestasis in infancy.  The liver disease often resolves following endocrine replacement therapy, and the cholestasis resolves within 6 weeks of starting treatment with thyroxine, cortisol and GH.  However, delay in diagnosis and treatment of the endocrinopathy may lead to persistently abnormal liver function tests, and in some cases to irreversible liver cirrhosis / portal hypertension. In our case patient did not take hormonal therapy and might have developed portal hypertension leading to esophageal varices and bleeding. It is likely that his former ethanol abuse contributed to his liver disease. Complete heart block  occurs in  hypothyroidism and is reported to be reversible with l-thyroxine replacement.

Conclusion:

 Untreated congenital hypopituitarism can present with heart block and portal hypertension with bleeding. Clinicians should be aware of associated complications and start hormonal therapy early.

Nothing to Disclose: SAC, SG, AB, ASS

*Please take note of The Endocrine Society's News Embargo Policy at http://www.endo-society.org/endo2013/media.cfm

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