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Hydranencephaly

Hydranencephaly is a type of Cephalic disorder. This is a rare condition in which the cerebral hemispheres are absent and replaced by sacs filled with cerebrospinal fluid. Usually the cerebellum and brainstem are formed normally. An infant with hydranencephaly may appear normal at birth. The infant's head size and spontaneous reflexes such as sucking, swallowing, crying, and moving the arms and legs may all seem normal. However, after a few weeks the infant usually becomes irritable and has increased muscle tone (hypertonia). After several months of life, seizures and hydrocephalus may develop. more...

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Other symptoms may include visual impairment, lack of growth, deafness, blindness, spastic quadriparesis (paralysis), and intellectual deficits.

Hydranencephaly is an extreme form of porencephaly, which is characterized by a cyst or cavity in the cerebral hemispheres, and may be caused by vascular insult or injuries, infections, or traumatic disorders after the 12th week of pregnancy.

Diagnosis may be delayed for several months because the infant's early behavior appears to be relatively normal. Transillumination, an examination in which light is passed through body tissues, usually confirms the diagnosis. Some infants may have additional abnormalities at birth including seizures, myoclonus (involuntary sudden, rapid jerks), and respiratory problems.

There is no standard treatment for hydranencephaly. Treatment is symptomatic and supportive. Hydrocephalus may be treated with a shunt.

The outlook for children with hydranencephaly is poor. Death generally occurs before age 1.

Read more at Wikipedia.org


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Illustrated Textbook of Pediatrics. - book reviews
From Journal of Family Practice, 9/1/98 by Allan J. Wilke

Illustrated Textbook of Pediatrics. Tom Lissauer and Graham Clayden. Mosby/Times Mirror International Publishers, St Louis, Mo, 1997, 330 pages. ISBN 0-7234-1657-5.

The first thing I noticed about this book was that the title on the cover, Illustrated Textbook of Pediatrics, is not the title on the title page (Illustrated Textbook of Paediatrics). Further examination solved the mystery. Dr Lissauer is a consultant paediatrician and Dr Clayden is a reader in paediatrics, as they both hail from London. Mosby has wrapped a British paediatrics text in a more Yank-friendly cover.

What I like about this first edition is stated in its title (both of them): it is illustrated. All the graphs and charts and almost all the photographs are in color. The illustrations of rashes and other physical findings are striking. The text is current with therapies, mentioning surfactants for respiratory distress syndrome and gene therapy for cystic fibrosis. Growth and development receive adequate discussion. There is also a section dealing with HIV infection.

What don't I like? The Illustrated Textbook of Pediatrics is slim, only 330 pages, compared with 2245 pages in the 15th edition of the Nelson Textbook of Pediatrics. It is most appropriate for medical students on their first general pediatric rotation. The detail is scanty. For instance, in the section "Common Genital Disorders in Female Children," we learn that vulvovaginitis or vaginal discharge in young girls sometimes "... results from sexual abuse, and swabs should be taken to identify any pathogens." That is it. Anencephaly rates a three-sentence paragraph, without an illustration. Hydranencephaly merits no mention at all. When therapy was included, I had to hunt for drug selection and dosage. Hepatitis B immunization shows up in a chart, but with no schedule. Bicycle accidents are noted to be "common," but there is no recommendation for helmet use. Some information is wrong; in the section on immunizations, MMR is recommended for children with HIV because it is safe.

Is it very troublesome to read a text written by two Brits? Probably not, but you must not wince at each "whilst." And you must tolerate colloquialisms such as "pen torch" for "pen light" and "napkin rash" for "diaper rash." And you must remember that the British spelling is sometimes different from ours; for example, placing an "a" before an "e." This may not seem too onerous, but when I searched the index for "hemolytic-uremic syndrome," it was not there. "Haemolytic uraemic syndrome," however, was halfway up the page. Additionally, the growth charts in the Appendix are normed for the United Kingdom and include curves for the 0.4th and 99.6th percentiles. The laboratory (with the accent on the second syllable) values are listed with normals in mmol/L. I know that technically we in the United States should be doing the same, but at least our textbooks reference values in mg/dL alongside the international units. Parochial references to the 1981 and 1993 Education Acts in England and Wales (acts to protect the rights of children with disabilities and learning difficulties) and others, while interesting in themselves, do not pertain to US children. And then there are the drug names, some of which we use (paracetamol for acetaminophen, for example) and others, like monosulfiram, that are not available here.

Would I purchase this text? Probably not. Will I refer to it now that it sits on my bookshelf? Maybe for the photographs, and occasionally for the text.

Allan J. Wilke, MD St Cloud, Minnesota

COPYRIGHT 1998 Dowden Health Media, Inc.
COPYRIGHT 2004 Gale Group

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