Nonbacterial nonfungal interstitial pneumonitis following autologous bone marrow transplantation in children treated with high-dose chemotherapy without total-body irradiation

Between February 1979 and May 1986, 165 children were treated with autologous bone marrow transplantation after high-dose chemotherapy without total-body irradiation for a hematologic malignancy or a solid tumor. Nonbacterial nonfungal interstitial pneumonitis was observed in 24 children and was the cause of death in 11 cases. Of the 24 cases of interstitial pneumonitis, 14 were considered to be idiopathic. Cytomegalovirus was the major pathogenic agent detected in the 10 other cases of interstitial pneumonitis (n = 5), followed by Herpes zoster (n = 2), Pneumocystis carinii (n = 1), tumor (n = 1), and adenovirus (n = 1). The only factor found to correlate significantly with the increased rate of interstitial pneumonitis was the use of high-dose 1-3 bis chloroethyl-1 nitrosourea (BCNU) (600 mg/m2), whereas BCNU at a dose of 300 mg/m2 did not affect this rate. These data, when compared with the literature, show a lower incidence of interstitial pneumonitis than in allogeneic transplantations, and an incidence similar to that observed in syngeneic transplantations, although there was no radiation toxicity in this series.     

Late-onset interstitial pneumonia following allogeneic bone marrow transplantation

Although most episodes of interstitial pneumonia (IP) following allogeneic bone marrow transplantation occur during the second or third month, occasional cases occur later. The records of 139 patients transplanted for leukemia over 6 years were reviewed in this study to examine cases of IP occurring beyond 100 days following marrow transplantation. Ten episodes of IP occurred among the 67 patients who survived 100 days (an actuarial probability of 18%). All cases occurred within the first year. An infectious cause was established in 80%. The case fatality rate was 60%. Although most infectious cases of IP occurring before 100 days are caused by cytomegalovirus, the late-onset cases in this study were caused by a heterogeneous group of pathogens, some of which were amenable to specific therapies. Thus, an aggressive approach to establishing a specific diagnosis should be made in cases of IP occurring more than 100 days after marrow transplantation.     

Renal transplantation between identical twins: A review

The experience of transplantation between identical twins, occurring primarily at the Peter Bent Brigham Hospital, is reviewed. The remarkable success of this human experiment beginning in 1954 and ending, at least for the present in our hospital, in 1976, has confirmed early hopes that clinical renal transplantation may be an extremely effective treatment for end-stage renal failure. Based largely on this seminal experience, investigations into experimental aspects of transplantation immunology, autoimmunity, and the general host responses, as well as developments in the field of nephrology and overall care of patients with endstage renal disease, have burgeoned. Indeed, during recent years, transplantation of several organs including heart, heart-lung, liver, and kidney have proven remarkably successful. As knowledge and application in this field continues to expand, increasing numbers of patients with organ failure can expect to return to relatively normal lives.

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Resumen Se revisa la experiencia con el transplante entre gemelos idénticos obtenida primordialmente en el Hospital Peter Bent Brigham de Boston. El resonante éxito de este experimento humano iniciado en 1954 y culminado, por lo menos en cuanto se refiere al presente en nuestro hospital, en 1976, ha confirmado las esperanzas iniciales de que el transplante cl/'inico puede ser un tratamiento extremadamente efectivo para la falla renal terminal. Con base en esta experiencia seminal, han florecido las investigaciones sobre los aspectos inmunológicos de los transplantes, la autoinmunidad y la respuesta general del huesped, así como los desarrollos en el campo de la nefrología y el cuidado general de los pacientes con enfermedad renal terminal. En realidad en los últimos años el transplante de various órganos, incluyendo el corazón, el corazón-pulmón y el riñón, ha probado ser notoriamente exitoso. En la medida que el conocimiento y la aplicación clínica en este campo continúan en expansión, un creciente número de pacientes con falla orgánica pueden aspirar a retornar a un estado de vida relativamente normal.

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Résumé L'expérience de la transplantation rénal entre des jumeaux identiques qui a été conduite en premier lieu au Peter Bent Brigham Hospital est passée en revue. Le remarquable succès de cette expérience de la transplantation chez l'être humain qui a commencé en 1954 pour se poursuivre jusqu'en 1976 dans cette formation a confirmé tous les espoirs: la transplantation rénale réprésente un traitement extrêmement efficace de l'insuffisance rénale arrivée à son stade ultime. Basée sur cette expérience initiale les enquêtes conduites à propos de l'immunologie de l'autoimmunité, des réponses de l'hôte à la greffe ainsi que la meilleure connaissance de la néphrologie et du traitement de l'insuffisance rénale terminale se sont multipliées. C'est à partir de l'acquisition de ces données qu'au cours des dernières années la transplantation de différents organes: coeur, coeur-poumon, foie, et rein tend à donner lieu à de multiples succès. Comme les connaissances dans le domaine de la transplantation s'approfondissent et que leurs applications ne cessent de s'étendre, de nombreux malades peuvent nourrir l'espoir de mener une vie relativement normale grâce à la greffe.

     

Cerebral aneurysms in identical twins

The hypothesis that genetic factors play a role in the genesis of cerebral aneurysms may be supported by clinical analysis of cerebral aneurysms in identical twins. In addition to reporting the present case, we review the literature on clinical features and genetical considerations in this matter. A forty-two-year-old male presented with subarachnoid hemorrhage (SAH) due to a left MCA aneurysm. At the age of 39, his identical twin brother also had SAH due to a ruptured left MCA aneurysm. This identical twin brother and his father's sisters died of SAH. Venous blood of the patient and his parents was collected to determine apolipoprotein E, angiotensinogen, angiotensin-converting enzyme, and very low density lipoprotein receptor genes. This analysis suggested a strong genetic association of apolipoprotein E epsilon4 with the pathogenesis. Including our cases, 13 cases of cerebral aneurysms in identical twins have been reported. There was a total of 37 aneurysms. Six twins had the same sites of aneurysm. These aneurysms show a tendency to be small in size, and to cause a high frequency of SAH occurring at a similar age. In young adults, cerebral aneurysms in identical twins tend to rupture. A screening diagnosis should be carried out as early as possible on an asymptomatic twin when the other has been discovered to have a ruptured cerebral aneurysm. Preventive treatment and systemic genome-wide linkage study should be conducted.     

Cloacal exstrophy in identical twins

Cloacal exstrophy occurring in identical twins is reported.     

Klinefelter syndrome in identical twins

A rare case of Klinefelter syndrome in identical twins is reported. Salient features and clinical recognition of the syndrome are discussed.     

Tethered cord syndrome in identical twins

Summary  The diagnosis of tethered cord syndrome (TCS) without typical conus medullaris symptoms and the radiological features such as a low set conus medullaris or dysraphic malformation is difficult. We report 11 year old identical twin brothers with TCS associated with the conus at the normal level. Their presenting symptom was progressive leg pain and both patients underwent surgical interruption of the filum terminale. The pain recurred in one patient treated surgically only after symptom became worse but resolved immediately in the other sibling treated promptly. We indicate the importance of early diagnosis and treatment of TCS to obtain excellent long-term outcome despite the absence of a low set conus or specific symptoms. Furthermore, when a twin or sibling of an affected person has neurological symptoms and the cutaneous signature of spinal dysraphism, radiological examination should be performed to establish the cause.     

Malignant testicular tumors in identical twins

Two cases of testicular malignancy in a pair of identical twins are reported. The literature is also reviewed. The tumors appeared within 7 years of each other. The histology was different in both cases.     

Dose-response to pancuronium in identical twins

The dose-response to pancuronium was determined in identical twins within an hour with the same anesthetic technique. The dose-response curves did not differ from parallelism, but one infant was more sensitive to pancuronium than the other. The recovery rates were also different. The variation in the dose response to pancuronium seems to extend to identical twins.