Semilobar Holoprosencephaly with Neurogenic Hypernatraemia Two new cases
Journal Title: Sultan Qaboos University Medical Journal - Year 2013, Vol 13, Issue 3
Abstract
Holoprosencephaly (HPE) is a developmental defect of the embryonic forebrain and midface. It is due to the non-cleavage of the embryonic forebrain into two cerebral hemispheres and the incomplete development of the paramedian structures. Te overall prevalence is 1.31 per 10,000 births. Te aetiology could be genetic, environmental, or both. HPE is classifed into alobar, semilobar, and lobar subtypes based on the degree of separation of the cerebral hemispheres. We report two new cases of semilobar HPE with neurogenic hypernatraemia. Lack of thirst and hypodypsia associated with chronic hypernatraemia in patients with HPE is highly suggestive of neurogenic hypernatraemia. Early identifcation of neurogenic hypernatraemia is important as it improves with forced fluid therapy and does not require any medication.
Authors and Affiliations
Hashim Javad| Departments of Child Health, Sultan Qaboos University Hospital, Muscat, Oman, Saif Al-Yarubi| Departments of Child Health, Sultan Qaboos University Hospital, Muscat, Oman, Alexander P. Chacko| Departments of Child Health, Sultan Qaboos University Hospital, Muscat, Oman, Dilip Sankhla| Departments of Radiology & Molecular Imaging, Sultan Qaboos University Hospital, Muscat, Oman, Amna Al-Futasi| Departments of Child Health, Sultan Qaboos University Hospital, Muscat, Oman, Anas A. Abdelmogheth| Departments of Child Health, Sultan Qaboos University Hospital, Muscat, Oman, Mohamed El-Naggari| Departments of Child Health, Sultan Qaboos University Hospital, Muscat, Oman
Prevalence of Rabies in Various Species in Yemen and Risk Factors Contributing to the Spread of the Disease
Objectives: Tis study aimed to describe for the frst time the prevalence of the passively-reported rabies virus among different domestic and wild animals submitted to the Central Veterinary Laboratory from various are...
Outcome of pregnancy in patients possessing anticardiolipin antibodies
Objective – To analyse the outcome of pregnancy in a sample of patients with a history of fetal loss, and possessing anticardiolipin antibodies (ACAs), and to assess the effectiveness of therapy with aspirin and predni...
Re: Acute Coronary Syndrome, Diabetes and Hypertension Oman must pay more attention to chronic non-communicable diseases
Sentinel Lymph Node Biopsy A new approach in the management of head and neck cancers
Cervical lymph node metastasis affects the prognosis and overall survival rate of and therapeutic planning for patients with head and neck squamous cell carcinomas (HNSCCs). However, advanced diagnostic modalities sti...
Notice of Duplicate Publication Perceived benefts of pre-clinical simulation-based training on clinical learning outcomes among Omani undergraduate nursing students