Aphallia or complete absence of penis is a rare disorder, often associated with other congenital anomalies of the genitourinary and gastrointestinal systems. This disorder is difficult to manage and has a very significant psychosocial impact on the child as well as the family. Individualized assessment and formulation of management plan is important.
There is a need for high index of suspicion for diagnosis of LCH; misdiagnosis is frequent. Pulmonary involvement in children with LCH appears common. It is possibly still underdiagnosed. Nail changes are uncommon, but may act as a marker for multisystem disease. In addition to survival data and analysis of prognostic factors, the prospective collection of data on diverse presentations is essential, along with a high index of suspicion for the diagnosis of LCH.
On multivariate analysis, the variables found to be significantly associated with adverse outcome in febrile neutropenic episodes were three or more previous episodes of febrile neutropenia, child being already on oral antibiotics and Chest Radioraph abnormality at presentation.
A major advance for the use of tobramycin has occurred with its use by the inhalational route, in children with CF. The inhalation route provides the advantage of ease of administration for prolonged periods at home and allows use of very high doses. Systematic reviews suggest that tobramycin inhalation improves outcome, decreases the need for hospitalization and decreases the need for use of frequent systemic antibiotics in CF patients colonized with pseudomonas. Data on the efficacy of inhaled tobramycin in non-CF bronchiectasis are scarce, as are data on the prevention and treatment of ventilator-associated pneumonia, and on the role of combining inhaled tobramycin with systemic tobramycin. Despite limitations, this drug has the potential to be used in various conditions other than CF.
This is a report of a rare case of frontonasal dysplasia (FND) in a full-term girl with birth weight of 2.750 kg. The baby had the classical features of FND. There were no other associated anomalies. There was no history of consanguinity and no family history of similar conditions. So inheritance of this case could be considered sporadic. Maxillofacial surgery should be considered for all patients for whom improvement is possible. However, in developing countries where there are considerable limitations in provision of social services, with economic and educational constraints, correction of such major defects remains a challenging task.
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