In 1939, Dr. Nora Wattie Principal Medical Officer (Maternity and Child Welfare) introduced immunisation clinics across Glasgow, and promoted mother and child health education, resulting in virtual eradication of the infection in the city.Widespread vaccination pushed cases in the United States down from 4.4 per 100,000 inhabitants in 1932 to 2.0 in 1937. In Nazi Germany, where authorities preferred treatment and isolation over vaccination (until about 1939–41), cases rose over the same period from 6.1 to 9.6 per 100,000 inhabitants.Between June 1942 and February 1943, 714 cases of diphtheria were recorded at Sham Shui Po Barracks resulting in 112 deaths because the Imperial Japanese Army did not release supplies of anti-diphtheria serum.In 1943, diphtheria outbreaks accompanied war and disruption in Europe. The 1 million cases in Europe resulted in 50,000 deaths.In Kyoto during 1948, 68 of 606 children died after diphtheria immunization due to improper manufacture of aluminum phosphate toxoid.In 1974, the World Health Organization included DPT vaccine in their Expanded Programme on Immunization for developing countries.In 1975, an outbreak of cutaneous diphtheria in Seattle, Washington, was reported .After the breakup of the former Soviet Union in 1991, vaccination rates in its constituent countries fell so low that an explosion of diphtheria cases occurred. In 1991, 2,000 cases of diphtheria occurred in the USSR. Between 1991 and 1998 as many as 200,000 cases in the Commonwealth of Independent States were reported, with 5,000 deaths. In 1994, the Russian Federation had 39,703 diphtheria cases.
Pathophysiology
The GNAS1 gene involved in both Pseudohypoparathyroidism type 1a and Pseudopseudohypoparathyroidism is greatly affected by imprinting. When a father who has Pseudohypoparathyroidism undergoes spermatogenesis, imprinting of the GNAS1 gene inactivates both copies of his genes: one will be Functional and the other will be defective. Tissues in the body will re-activate different copies of the GNAS1 gene selectively; the kidneys will selectively activate the (functional) maternal copy while keeping the (defective) paternally-derived gene imprinted and inactive, even in normal individuals. Since the maternally-derived GNAS1 gene is functional, renal handling of calcium and phosphate is normal, and homeostasis is maintained in Pseudopseudohypoparathyroidism. However, the rest of the tissues will instead selectively display the defective gene, resulting in haploinsufficiency of the GNAS1 product in most tissues, and giving the phenotype of Pseudohypoparathyroidism type 1a. As a result, there is also a normal response of urinary cAMP to PTH, and normal serum PTH. Diagnosis
The diagnosis is based on the presence of the Albright hereditary osteodystrophy pseudotype but without the PTH resistance. Blood tests including calcium, phosphate, and PTH will exclude other forms of Pseudohypoparathyroidism. X-rays may reveal a short fourth metacarpal. Genetic testing can confirm the diagnosis by showing GNAS gene mutation. Treatment
Treatments focuses on symptoms, with genetic counseling recommended. History
It was characterized in 1952 by Fuller Albright as "pseudo-pseudohypoparathyroidism" (with hyphen). See also
Antidisestablishmentarianism
Floccinaucinihilipilification
GNAS complex locus
Honorificabilitudinitatibus
Longest word in English
Longest words
Parathyroid hormone
Pneumonoultramicroscopicsilicovolcanoconiosis
Supercalifragilisticexpialidocious

References


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