Volume 87 Number 2
Letters to the Editor
previous exposure to the rubella virus and past immunization practices in the Cincinnati area. A survey conducted at the Walter Reed Army Medical Center further demonstrates the necessity to determine the level of protection afforded the adolescent girl today by present immunization practices. Two hundred and eleven healthy teen-age girls voluntarily participated in the study. Each was questioned with regard to past rubella infection or immunization and a HAl titer was determined. The individual patient was randomly selected from military dependents presenting to the adolescent medicine service of the medical center. Fifty-five (26.1%) of the participants gave a history of past rubella immunization. A protective titer was found in all but 7.3% of this group. Forty (18.5%) girls responded with a past history of clinical disease. Seventeen and one-half percent of patients with a past history of rubella were susceptible to infection by HAI titer. The remainder of the sample population gave no history of disease or immunization or were ignorant of their immune status. Over 27.3% of these individuals were susceptible to rubella. These figures demonstrate medical history to be a tenuous basis for clinical decisions in the adolescent population. The level of protection in this controlled population suggests the inability of present immunization practices to provide a true "herd immunity." Our efforts to ensure protection to the nulliparous female before conception must be equal to our efforts to immunize the preschool child. To this end laboratory documentation of rubellaimmune status must become a routine medical practice. HA1 screening at pubarche and periodically in high-risk populations will permit an accurate assessment of need for immunization and prudent vaccine administration. Peter C. Freis, M.D. Major, M C Chief Pediatric Service U.S. Army Medical Department Activity West Point, N. Y. 10996
REFERENCE 1. Rauh JL, Sehiff GM, and Johnston LB: Follow-up studies of rubella vaccinees at adolescence, J PVDIATR 86:138, 1975.
Death from Torulopsis in chronic granulomatous disease To the Editor: The article by Lazarus and Neu 1 listing microorganisms which have been responsible for morbidity and mortality rates in chronic granulomatous disease (CGD) will be most helpful. We wish to add Torulopsis to the types of fungi causing death in this disease,
CASE REPORT A 2-month-old boy developed diarrhea and fever. He was treated with oral tetracycline, but fever persisted and he was
333
hospitalized, Because of increasing fever and abdominal distention he was then transferred to our hospital. He presented as an irritable, febrile, pale infant with marked abdominal distention. Hemoglobin was 6.6 gm/dl; urinalysis was normal. Multiple cultures of the blood, urine, pharynx, stool, and spinal fluid were negative. A nitroblue tetrazolium dye reduction test revealed very low spontaneous activity in the face of severe septic morphology. Endotoxin did not stimulate the patient's cells. (Subsequently, his mother and maternal grandmother were documented to be carriers of CGD.) Laparotomy revealed much ascitic fluid. There were a number of yellow masses along the ileum extending to the root of the mesentery. Microscopic examination of the masses revealed granulomatous and necrotizing lymphadenitis consistent with CGD. The infant died two days following surgery. Autopsy revealed similar nodules in the lungs, spleen, kidney, alimentary tract, liver, and lymph nodes. Antemortem culture of the ascitic fluid and postmortem culture of the lungs, spleen, and liver grew Torulopsis species. DISCUSSION Torulopsis is a yeast ordinarily considered to be a saprophyte of limited pathogenicity. It has been cultured from the oral cavity, respiratory tract, intestine, urine, and vagina of apparently healthy individuals. It has been reported to cause urinary tract infections and bronchopneumonia. Fungemia has been described. ~ Usually systemic infections have occurred in patients who were debilitated; many were immunosuppressed. One wonders whether the use of tetracycline contributed to infectivity of this usually saprophytic yeast. Jay 34. Orson, M.D. 293 Governor St. Providence, R. L 02906 Richard G. Greco, M.D. Department of Pediatrics Rhode Island Hospital Section on Human Growth and Development Brown University Providence, R. L 02902
REFERENCES 1. Lazarus GM, and Neu HC: Agents responsible for infection in chronic granulomatous disease of childhood, J PEDIATR 86: 415, 1975. 2. Pankey GA, and Daloviso JR: Fungemia caused by Torulopsis glabrata, Medicine 52: 395, 1973.
Proposed readjustment of eponyms for achondrogensis To the Editor: The form of lethal osteochondrodystrophy known as achondrogenesis has been classified into two types on the basis of radiographic 1 and pathologic findings.2 The differentiation,
334
Letters to the Editor
which was originally based on radiographic studies, has been confirmed by the observation of notable histopathologic differences in enchondral ossification. The terminology in both the book by Spranger and associates I and the paper by Yang and associates ~ is the same: type 1 was designated as FraccaroHouston-Harris and type 2 as Langer-Saldino. We have since had the opportunity to review Fraccaro's paper S and to examine the illustrations as originally published. The reproductions of the roentgenograms resemble the type 1 abnormality, but they are small and cannot be evaluated adequately. The illustrations of bone histology are somewhat better, and they clearly and undoubtedly correspond to the type 2 lesion, showing deficient cartilaginous matrix with large, mesenchymatous, poorly differentiated chondrocytes. We are convinced, therefore, that his case should be reclassified as type 2. We have also reviewed Parenti's paper,' the first report and description of achondrogenesis. The reproductions of the roentgenograms are inadequate for evaluation, but the histologic changes are identifiable as type 1, showing abundant cartilaginous matrix with better differentiated chondrocytes. We propose then to readjust the eponymic designations of these two types of achondrogenesis as follows: type 1 lethal achondrogenesis (Parenti-Houston-Harris), type 2 lethal achondrogenesis (Fraccaro-Langer-Saldino). This readjustment seems appropriate since the differentiation of two types follows from the earliest descriptions of the disease. The validity of this separation on the basis of apparent chondrocytie differentiation receives support also from the recent finding of additional cytologic differences between the two; in type 1 lethal achondro-
The Journal of Pediatrics August 1975
genesis the resting chondrocytes contain intracytoplasmic inclusions, a feature not seen in type 2.5 Parenthetically, it should be noted that a third type of achondrogenesis, described and designated by Grebe, ~ is nonlethal and represents a different disease. S.-S. Yang, M.D. Jay Bernstein, M.D. William Beaumont Hospital Royal Oak, Mich. 48072 Leonard O. Langer, Jr., M.D. Minneapolis, Minn.
REFERENCES 1. Spranger JW, Langer LO Jr, and Wiedemann H-R: Bone dysplasias, Philadelphia, 1974, W B Saunders Company, pp 24-27. 2. Yang S-S, Brough AJ, Garewal GS, and Bernstein J: Two types of heritable lethal achondrogenesis, J PEDIATR85:796, 1974. 3. Fraccaro M: Contributo allo studio delle malattie del mesenchima osteopoietico l'acondrogenesi, Folia Hered Pathol (Milano) 1:190, 1952. 4. Parenti GC: La anosteogenesi (una varietA delia osteogenesi imperfetta), Pathologica 28:447, 1936. 5. Yang S-S, Heidelberger KP, and Bernstein J: Intracytoplasmic inclusion bodies in the chondrocytes of type I achondrogenesis (submitted for publication). 6. Grebe H: Die Achondrogenesis. Ein einfach rezessives Erbmerkmal, Folia Hered Pathol (Milano) 2:23, 1952.
Letters to the Editor
previous exposure to the rubella virus and past immunization practices in the Cincinnati area. A survey conducted at the Walter Reed Army Medical Center further demonstrates the necessity to determine the level of protection afforded the adolescent girl today by present immunization practices. Two hundred and eleven healthy teen-age girls voluntarily participated in the study. Each was questioned with regard to past rubella infection or immunization and a HAl titer was determined. The individual patient was randomly selected from military dependents presenting to the adolescent medicine service of the medical center. Fifty-five (26.1%) of the participants gave a history of past rubella immunization. A protective titer was found in all but 7.3% of this group. Forty (18.5%) girls responded with a past history of clinical disease. Seventeen and one-half percent of patients with a past history of rubella were susceptible to infection by HAI titer. The remainder of the sample population gave no history of disease or immunization or were ignorant of their immune status. Over 27.3% of these individuals were susceptible to rubella. These figures demonstrate medical history to be a tenuous basis for clinical decisions in the adolescent population. The level of protection in this controlled population suggests the inability of present immunization practices to provide a true "herd immunity." Our efforts to ensure protection to the nulliparous female before conception must be equal to our efforts to immunize the preschool child. To this end laboratory documentation of rubellaimmune status must become a routine medical practice. HA1 screening at pubarche and periodically in high-risk populations will permit an accurate assessment of need for immunization and prudent vaccine administration. Peter C. Freis, M.D. Major, M C Chief Pediatric Service U.S. Army Medical Department Activity West Point, N. Y. 10996
REFERENCE 1. Rauh JL, Sehiff GM, and Johnston LB: Follow-up studies of rubella vaccinees at adolescence, J PVDIATR 86:138, 1975.
Death from Torulopsis in chronic granulomatous disease To the Editor: The article by Lazarus and Neu 1 listing microorganisms which have been responsible for morbidity and mortality rates in chronic granulomatous disease (CGD) will be most helpful. We wish to add Torulopsis to the types of fungi causing death in this disease,
CASE REPORT A 2-month-old boy developed diarrhea and fever. He was treated with oral tetracycline, but fever persisted and he was
333
hospitalized, Because of increasing fever and abdominal distention he was then transferred to our hospital. He presented as an irritable, febrile, pale infant with marked abdominal distention. Hemoglobin was 6.6 gm/dl; urinalysis was normal. Multiple cultures of the blood, urine, pharynx, stool, and spinal fluid were negative. A nitroblue tetrazolium dye reduction test revealed very low spontaneous activity in the face of severe septic morphology. Endotoxin did not stimulate the patient's cells. (Subsequently, his mother and maternal grandmother were documented to be carriers of CGD.) Laparotomy revealed much ascitic fluid. There were a number of yellow masses along the ileum extending to the root of the mesentery. Microscopic examination of the masses revealed granulomatous and necrotizing lymphadenitis consistent with CGD. The infant died two days following surgery. Autopsy revealed similar nodules in the lungs, spleen, kidney, alimentary tract, liver, and lymph nodes. Antemortem culture of the ascitic fluid and postmortem culture of the lungs, spleen, and liver grew Torulopsis species. DISCUSSION Torulopsis is a yeast ordinarily considered to be a saprophyte of limited pathogenicity. It has been cultured from the oral cavity, respiratory tract, intestine, urine, and vagina of apparently healthy individuals. It has been reported to cause urinary tract infections and bronchopneumonia. Fungemia has been described. ~ Usually systemic infections have occurred in patients who were debilitated; many were immunosuppressed. One wonders whether the use of tetracycline contributed to infectivity of this usually saprophytic yeast. Jay 34. Orson, M.D. 293 Governor St. Providence, R. L 02906 Richard G. Greco, M.D. Department of Pediatrics Rhode Island Hospital Section on Human Growth and Development Brown University Providence, R. L 02902
REFERENCES 1. Lazarus GM, and Neu HC: Agents responsible for infection in chronic granulomatous disease of childhood, J PEDIATR 86: 415, 1975. 2. Pankey GA, and Daloviso JR: Fungemia caused by Torulopsis glabrata, Medicine 52: 395, 1973.
Proposed readjustment of eponyms for achondrogensis To the Editor: The form of lethal osteochondrodystrophy known as achondrogenesis has been classified into two types on the basis of radiographic 1 and pathologic findings.2 The differentiation,
334
Letters to the Editor
which was originally based on radiographic studies, has been confirmed by the observation of notable histopathologic differences in enchondral ossification. The terminology in both the book by Spranger and associates I and the paper by Yang and associates ~ is the same: type 1 was designated as FraccaroHouston-Harris and type 2 as Langer-Saldino. We have since had the opportunity to review Fraccaro's paper S and to examine the illustrations as originally published. The reproductions of the roentgenograms resemble the type 1 abnormality, but they are small and cannot be evaluated adequately. The illustrations of bone histology are somewhat better, and they clearly and undoubtedly correspond to the type 2 lesion, showing deficient cartilaginous matrix with large, mesenchymatous, poorly differentiated chondrocytes. We are convinced, therefore, that his case should be reclassified as type 2. We have also reviewed Parenti's paper,' the first report and description of achondrogenesis. The reproductions of the roentgenograms are inadequate for evaluation, but the histologic changes are identifiable as type 1, showing abundant cartilaginous matrix with better differentiated chondrocytes. We propose then to readjust the eponymic designations of these two types of achondrogenesis as follows: type 1 lethal achondrogenesis (Parenti-Houston-Harris), type 2 lethal achondrogenesis (Fraccaro-Langer-Saldino). This readjustment seems appropriate since the differentiation of two types follows from the earliest descriptions of the disease. The validity of this separation on the basis of apparent chondrocytie differentiation receives support also from the recent finding of additional cytologic differences between the two; in type 1 lethal achondro-
The Journal of Pediatrics August 1975
genesis the resting chondrocytes contain intracytoplasmic inclusions, a feature not seen in type 2.5 Parenthetically, it should be noted that a third type of achondrogenesis, described and designated by Grebe, ~ is nonlethal and represents a different disease. S.-S. Yang, M.D. Jay Bernstein, M.D. William Beaumont Hospital Royal Oak, Mich. 48072 Leonard O. Langer, Jr., M.D. Minneapolis, Minn.
REFERENCES 1. Spranger JW, Langer LO Jr, and Wiedemann H-R: Bone dysplasias, Philadelphia, 1974, W B Saunders Company, pp 24-27. 2. Yang S-S, Brough AJ, Garewal GS, and Bernstein J: Two types of heritable lethal achondrogenesis, J PEDIATR85:796, 1974. 3. Fraccaro M: Contributo allo studio delle malattie del mesenchima osteopoietico l'acondrogenesi, Folia Hered Pathol (Milano) 1:190, 1952. 4. Parenti GC: La anosteogenesi (una varietA delia osteogenesi imperfetta), Pathologica 28:447, 1936. 5. Yang S-S, Heidelberger KP, and Bernstein J: Intracytoplasmic inclusion bodies in the chondrocytes of type I achondrogenesis (submitted for publication). 6. Grebe H: Die Achondrogenesis. Ein einfach rezessives Erbmerkmal, Folia Hered Pathol (Milano) 2:23, 1952.
