Primordial Dwarfism

Primordial dwarfism is a form of dwarfism that results in a smaller body size in all stages of life beginning from before birth. More specifically, primordial dwarfism is a diagnostic category including specific types of profoundly proportionate dwarfism, in which individuals are extremely small for their age, even as a fetus. Most individuals with primordial dwarfism are not diagnosed until they are about 3 years of age.

Those who have Microcephalic osteodysplastic primordial dwarfism type II often have further medical issues than the other types such as a squeaky voice, microdontia, widely spaced primary teeth, poor sleep patterns (in early years), delayed mental development, frequent sickness, breathing problems, eating problems, hyperactivity, farsightedness, brain aneurysms, and do not respond to hormone therapy because primordial dwarfism is not caused by a lack of any growth hormone. After reviewing x-rays it is also found that many have dislocated joints, scoliosis, and delayed bone age as well as microcephaly. They will not reach the size of an average newborn until they are between the ages of 3-5. (from wikipedia)

There are as yet no effective treatments for primordial dwarfism. It is known that PD is caused by inheriting a mutant gene from each parent.  The lack of normal growth in the disorder is not due to a deficiency of growth hormone, as in hypopituitary dwarfism.  Administering growth hormone, therefore, has little or no effect on the growth of the individual with primordial dwarfism. (wikipedia)

image from Rare Book Room flickr  additional image of reverse of photograph by J Wood


(Photo caption) Phineas Taylor Barnum’s smallest dwarf:  Lucia Zarate (1864 in San Carlos, Mexico – 1890) is the first person to have been identified with Majewski Osteodysplastic Primordial Dwarfism Type II.  She was entered into the Guinness World Records as the “lightest recorded adult”, weighing 4.7 pounds (2.1 kg) at the age of 17. According to an 1894 article in Strand Magazine, Zarate achieved her full growth by the age of one year.

More pictures and infromation about Lucia Zarate at ShowHistory :Lucia Zarate “The Mexican Lilliputian”

Her home Casa Grande is now a museum.  More pictures and information can be found here, Casa Grande link

Other similar posts
This entry was posted in Disease, Endocrine, Historical and tagged , , , , , , , .