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February 1992, Vol 146, No. 2 >
Article | February 1992

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Walter W. Tunnessen, MD
[+] Author Affiliations

Accepted for publication April 15, 1991.

Reprint requests to The Children's Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, PA 19104 (Dr Tunnessen).


Am J Dis Child. 1992;146(2):253-254. doi:10.1001/archpedi.1992.02160140119032.
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Denouement and Discussion 

Tuberous Sclerosis 

Manifestations  Tuberous sclerosis (TS) was first described by von Reclding-hausen in 1862. In 1880, Bourneville used the term tuberous to describe the potatolike findings in the cerebral gyri of affected individuals and the triad of mental retardation, seizures, and adenoma sebaceum (angiofibromas) associated with this disease. This neurocutaneous disorder is much more common than previously thought, has wide variability in its presentation, and affects many body systems.The criteria for diagnosis give some indication of the variability of this genetic disorder. Criteria for the definite diagnosis of TS include one or more of the following conditions:1 facial angiofibroma; ungal fibroma; retinal hamartoma; cortical tuber; subependymal glial nodule; and renal angiomyolipoma. Criteria for a presumptive diagnosis of TS include two or more of the following conditions: hypomelanotic macule; multicystic kidney; shagreen patch; cardiac rhabdomyoma; gingival fibroma; pulmonary lymphangiomyomatosis; dental enamel pits; peripapillary retinal hamartoma; infantile spasms;

REFERENCES

1 +
Gomez MR.  Tuberous sclerosis . In: Gomez MR, ed. Neurocutaneous Diseases: A Practical Approach . Stoneham, Mass: Butterworths; 1987;:30-52.
2 +
Oppenheimer EY, Rosman NP, Dooling EC.  The late appearance of hypopigmented maculae in tuberous sclerosis . AJDC . 1985;;139:408-409.
3 +
Smith HC, Watson GH, Patel RG, Super M.  Cardiac rhabdomyomata in tuberous sclerosis: their course and diagnostic value . Arch Dis Child . 1989;;64:196-200.
4 +
Stapleton FB, Johnson D, Kaplan GW, Griswold W.  The cystic renal lesion in tuberous sclerosis . J Pediatr . 1980;;97:574-579.

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Country-Specific Mortality and Growth Failure in Infancy and Yound Children and Association With Material Stature

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References

1 +
Gomez MR.  Tuberous sclerosis . In: Gomez MR, ed. Neurocutaneous Diseases: A Practical Approach . Stoneham, Mass: Butterworths; 1987;:30-52.
2 +
Oppenheimer EY, Rosman NP, Dooling EC.  The late appearance of hypopigmented maculae in tuberous sclerosis . AJDC . 1985;;139:408-409.
3 +
Smith HC, Watson GH, Patel RG, Super M.  Cardiac rhabdomyomata in tuberous sclerosis: their course and diagnostic value . Arch Dis Child . 1989;;64:196-200.
4 +
Stapleton FB, Johnson D, Kaplan GW, Griswold W.  The cystic renal lesion in tuberous sclerosis . J Pediatr . 1980;;97:574-579.

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