Pediatric intracranial aneurysms—clinical characteristics and outcome of surgical treatment

Object Intracranial aneurysms are rare in children and have different epidemiological, clinical, and morphological characteristics and outcome from those in adults. Materials and methods We analyzed demographic, clinical, radiologic features, treatment, and outcome in 55 patients <18 years of age, treated from Jan 1995 through December 2005. Results Intracranial aneurysms in children below 18 years constituted 4% of all intracranial aneurysms. Internal Carotid artery (ICA) bifurcation was the commonest location. About half of the aneurysms were complex. Three-fourth of the patients required surgical treatment. Two patients died, constituting 5% mortality. Two patients (5%) had poor outcome, whereas the majority (90%) had a favorable outcome. Conclusions Pediatric aneurysms have male predominance, higher incidence of clinical features of mass effect or seizures, high incidence of large, traumatic/mycotic aneurysms, associated illnesses and ICH/IVH and hydrocephalus, better Hunt and Hess grades at presentation, ICA bifurcation as the commonest site, and better outcome than their adult counterparts.     

High dose cyclophosphamide, BCNU, and VP-16 (CBV) as a conditioning regimen for allogeneic bone marrow transplantation for patients with acute leukemia

A high dose combination chemotherapy regimen (CBV) consisting of cyclophosphamide (1.5 gm/m2 day 1 to day 4); BCNU (300 mg/m2 day 1) and etoposide (100 mg/m2 every 12 hours for 6 doses), followed by bone marrow transplant from human leukocyte antigen (HLA) identical sibling donors, was evaluated in 29 patients in whom acute leukemia was in relapse or remission. Engraftment of donor cell type occurred in all but one of 21 patients, in whom marker differences between donor and recipient were established. Two of 11 patients transplanted during relapse of the disease, lived beyond 1 year after bone marrow transplantation. One patient died free of leukemia, 41 months after transplantation of meningitis. Two of seven patients transplanted during the second remission of the disease, are alive and free of leukemia at 42+, and 8+ months. All patients transplanted during the third or fourth remission of the disease have died from either a further relapse, or transplant related causes. The low incidence of organ toxicity with CBV allows for further dose escalation of its drug components.     

The relevance of a staging laparotomy for Hodgkin's disease in India.

A retrospective analysis of 328 cases of Hodgkin's Disease (HD) subjected to a staging laparotomy at the Tata Memorial Hospital, Bombay, India, from 1974 to 1986 was undertaken to assess its relevance to our setup. Eighty percent of the patients were from clinical stages (CS) I and II, 38% with lymphocyte predominance (LP), and 41% with mixed cellularity (MC) histologies. Staging laparotomy was positive in 60% cases overall, including 50% from CS IA and IIA, 68% from CS IB and IIB, and 53% and 67%, respectively, from LP and MC histologies. Splenic involvement was seen in 54% cases. Operative complications were encountered in 2% of cases and deaths in two cases only. In view of the high propensity for abdominal spread, only selected CS IA and IIA cases would merit a staging laparotomy within which, nearly 50% cases with a negative yield could be offered radical segmental irradiation alone for cure. The majority of our patients would, however, require combination therapy.     

Microcystic serous cystadenoma of the pancreas: a report of two cases with one of diffuse presentation.

Microcystic adenoma or serous cystadenoma is an uncommon tumor and accounts for 1-2% of the exocrine neoplasms of the pancreas. Usually unifocal, they present as single, large, well-demarcated multiloculated cystic tumors, ranging in size from 1 to 25 cm. Multifocal variants or diffuse serous cystadenomas are extremely rare. We present 2 cases of which 1 is a diffuse variant affecting the body, tail and part of the neck of the pancreas. In both the patients the tumors were detected incidentally. We highlight on the diffuse variant in view of its rarity and present a review of literature. In this case the entire body and tail of the pancreas was spongy replaced by multicystic lobules and hyalinized fibrocollagenous stroma. The cysts were lined by low cuboidal glycogen containing bland cells. Such a unique presentation wherein the entire body and tail of the pancreas is replaced with multiple cysts is a diffuse presentation of microcystic adenoma and a search through literature revealed only 7 such cases among the 15 cases with multifocal presentation reported.     

Decreased infectivity despite unaltered C3 binding by a DeltahbhA mutant of Mycobacterium tuberculosis

HbhA of Mycobacterium tuberculosis is a multifunctional binding protein, binding to both sulfated sugars such as heparin and to human complement component C3. HbhA may therefore interact with host molecules and/or host cells during M. tuberculosis infection and play a role in the pathogenesis of this bacterium. The purpose of this study was to use allelic exchange to create an M. tuberculosis strain deficient in expression of HbhA to determine whether this protein's C3-binding activity plays a role in the pathogenesis of M. tuberculosis. An in-frame, 576-bp unmarked deletion in the hbhA gene was created using sacB as a counterselectable marker. Southern blotting and PCR analyses confirmed deletion of hbhA in the DeltahbhA mutant. The DeltahbhA mutant strain grew at a rate similar to that of the parent in broth culture and in J774.A1 murine macrophage-like cells but was deficient in growth compared to the parent strain in the lungs, liver, and spleen of infected mice. In addition, the DeltahbhA mutation did not reduce binding of M. tuberculosis to human C3 or to J774.A1 cells in the presence or absence of serum, suggesting that in the absence of HbhA, other molecules serve as C3-binding molecules on the M. tuberculosis surface. Taken together, these data indicate that HbhA is important in the infectivity of M. tuberculosis, but its ability to bind C3 is not required for mycobacterial adherence to macrophage-like cells. Using the DeltahbhA mutant strain, a second M. tuberculosis C3-binding protein similar in size to HbhA was identified as HupB, but the role of HupB as a C3-binding protein in intact organisms remains to be determined.