A rare case of congenital generalized fibromatosis with predominant bone involvement in a Chinese newborn is reported. Radiological examination revealed multiple osteolytic lesions in all the long limb bones and the skull. Pathology showed a mixture of fibroblasts and smooth muscle-like cells. The spontaneous regression of most of the bone lesions after 18 months is highlighted.

Bone Neoplasms↗

PubMedDate not supplied

Ultrastructural observations on Penicillium marneffei in natural human infection.

The ultrastructure of Penicillium marneffei and the host response to the infection were studied in two patients. One was immunocompetent and the other an immunosuppressed renal graft recipient. In the immunocompetent patient it was observed that all the yeast cells were phagocytosed and were found either within membrane-bound vacuoles or lying freely within the cytoplasm of the macrophages. It was postulated that continuous lysosomal fusion with the phagolysosomes and multiplication of the fungi within the phagocytic vacuoles might eventually lead to the rupture of the vacuoles with release of the organisms into the cytoplasm of the macrophages. In the second patient, the immunosuppressive effects of corticosteroids might account for the large number of nonphagocytosed fungi in the tissue space, and the failure to form large phagocytic vacuoles.

Adult↗

PubMedDate not supplied

Aggressive angiomyxoma of the vulva in an 11-year-old girl.

A rare case of aggressive angiomyxoma involving the vulva of an 11-year-old girl is reported. The pathologic features that distinguish this lesion from other myxoid tumors or tumorlike conditions of the perineum and vulva in children are discussed.

Child↗

PubMedDate not supplied

Subcutaneous T-cell lymphoma presenting as panniculitis in children: report of two cases.

Two cases of peripheral T-cell lymphoma with primarily subcutaneous involvement and clinically presenting as panniculitis were reported in two children. One child developed florid hemophagocytic syndrome and was treated by combination chemotherapy but died 14 months later of disseminated fungal infection. The other child had mild systemic symptoms but no histological evidence of hemophagocytosis. Despite a more florid lymphomatous infiltrate, he attained a complete remission after a short course of prednisone and remains symptom-free at 3 years. Our report confirms that subcutaneous T-cell lymphoma is a distinct clinicopathological entity and highlights the two modes of clinical presentation. The development of florid hemophagocytic syndrome indicates a bad prognosis. This entity must be distinguished from other causes of panniculitis and immunophenotypic analysis of the atypical cells is essential in the diagnosis. Combination chemotherapy is the treatment of choice, but in patients who pursue an indolent course the administration of aggressive chemotherapy may be deferred.

Antineoplastic Combined Chemotherapy Protocols↗

PubMedDate not supplied

Intestinal spirochetosis in children: report of two cases.

Two cases of intestinal spirochetosis in two children are reported. The first patient, a 7 1/2-year-old boy, presented with diarrhea and rectal bleeding. After the diagnosis was made by a rectal biopsy, he was given metronidazole and neomycin. Symptoms persisted despite a further rectal biopsy that showed clearance of the organisms. Appendiceal spirochetosis was an incidental finding in our second patient, an 8-year-old girl who presented with acute abdominal pain and in whom mesenteric adenitis was diagnosed clinically at laparotomy. The possible pathogenic mechanisms causing clinical symptoms are discussed.

Abdominal Pain↗

PubMedDate not supplied

Focal and segmental glomerulosclerosis in children with reflux nephropathy.

A histological review of 86 pediatric nephrectomy specimens from patients with vesicoureteric reflux (with or without apparent obstruction at the vesicoureteric junction) investigated the relationship between the presence and extent of focal and segmental glomerulosclerosis (FSGS) and coexisting renal hypoplasia and postnatally acquired cortical damage. FSGS was found in 18 patients, 9 of whom were less than 5 years old. There was no significant association between the presence (or grade) or absence of FSGS and age at nephrectomy, gender, presence or absence of obstruction, and severity of hypoplasia and/or postnatally acquired cortical loss. FSGS was absent from 18 hypoplastic kidneys without vesicoureteric reflux (although of relatively young age), 40 normally developed kidneys age-matched with the index population, and 72 nephrectomy specimens without vesicoureteric reflux (except in 2 known cases of focal segmental glomerulonephritis). Within the index population FSGS was significantly (P < .01) associated with hypertension, and hypertension was significantly associated with proteinuria (P < .001) but not with an abnormal contralateral kidney. There was no significant association between FSGS, proteinuria, and an abnormal contralateral kidney. Our results were unexpected when interpreted within a pathogenesis for FSGS of glomerular "hyperfiltration." They may, at least in the pediatric age group, indicate a possible role for other mechanisms in the development of FSGS.

Adolescent↗

PubMedDate not supplied

Metachronous pulmonary and cerebral inflammatory pseudotumor in a child.

An 8-year-old boy had a right pneumonectomy performed for a large inflammatory pseudotumor. Over the subsequent 8 years he developed multiple similar lesions in the meninges and bilateral cerebral hemispheres as well as new growths within the ipsilateral pleural cavity and contralateral lung. Metachronous pulmonary and intracranial inflammatory pseudotumor has been reported only once. These lesions probably represent a multifocal, exaggerated inflammatory response to some as yet unidentified stimuli.

Adolescent↗

PubMedDate not supplied

Factors affecting the incidence of polyploidy in a human in vitro fertilization program.

OBJECTIVE: To study the factors affecting the incidence of polyploidy in an assisted reproduction program. DESIGN: Retrospective analysis. SETTING: University-based subfertility clinic. PATIENTS AND METHODS: Subfertile patients were treated in 163 treatment cycles of in vitro fertilization (IVF) or pronuclear stage tubal transfer (PROST). The relationship between the incidence of polyspermy and the indication of subfertility, the stimulation protocol, the oocyte, and the semen parameters were analyzed. RESULTS: Eighty-nine of the 1,149 oocytes were polyploid (7.8%). The incidence of polyploidy was not affected by the indication for IVF, the age of the patients, the stimulation protocol, the maturity of oocytes as assessed by the appearance of the cumulus, the sperm concentration, the number of sperm inseminated, the serum estradiol level, and the number of oocytes retrieved. The incidence was increased when: (1) the serum estradiol fell before the administration of human chorionic gonadotrophin; (2) the oocyte retrieval-insemination interval was 6 hours or more; (3) the sperm motility was 70% or more; (4) the percentage of sperm with normal morphology was 50% or more. CONCLUSION: The incidence of polyploidy was affected by the serum estradiol pattern, the preinsemination interval, sperm motility, and percentage of morphologically normal sperm.

Adult↗