Single-center prospective study of Plummer-Vinson syndrome.

Post-cricoid web is an uncommon cause for dysphagia and is most frequently reported in middle-aged women. Triad of web, iron deficiency anemia (IDA), and dysphagia is known as Plummer-Vinson syndrome (PVS). Literature on PVS is very limited. Here we report the first prospective study of PVS with predefined diagnostic criteria and management plan. Adults with dysphagia or those incidentally found to have esophageal web were prospectively enrolled between July 2011 and June 2013. Participants were evaluated with hemogram, barium swallow, and esophagogastroduodenoscopy. PVS was diagnosed if a person had IDA and a post-cricoid web in barium swallow and/or endoscopy. Patients were managed with dilation using through-the-scope controlled radial expansion balloon followed by oral iron and folic acid supplementation. Thirty-seven patients (age, median [range] 40 [19-65] years; 32 [86%] women) were enrolled. Thirty-one symptomatic patients had dysphagia grade 1 (n = 12, 39%), 2 (n = 13, 42%), and 3 (n = 6, 19%) for a median (range) duration of 24 (4-324) months. Barium swallow, done in 29, showed web in 25 which were either circumferential or anterior in position. Twenty-nine (29/31, 94%) patients had complete and two had partial response after the first session of endoscopic dilatation without any complication. Dysphagia recurred in three (10%) of the 30 patients who were followed for a median (range) of 10 (1-24) months. Esophageal-web related dysphagia in patients with PVS responds favorably after single session of endoscopic dilation.

Aggressive renal angiomyolipoma extending into the renal vein and inferior vena cava - an uncommon entity.

Renal angiomyolipoma is recognised as a benign hamartomatous lesion with no obvious malignant potential. However, the tumour may show extrarenal/perinephric extension at times. Rarely, the lesion may extend into the renal vein and inferior vena cava (IVC) indicating aggressive behaviour. We present a case of an angiomyolipoma of the kidney with sonographic, CT and MRI evidence of extension into the renal vein and IVC.

Trichinoechinococcosis T-5. Case report.

Echinococcosis affecting the spine is rare. The authors present the case of a 23-year-old man in whom features of thoracic spinal cord compression were demonstrated. Magnetic resonance imaging of the thoracic spine revealed an extradural cystic lesion involving the spine and paraspinal musculature. At surgery echinococcosis was confirmed, and subsequent histopathological examination also revealed Trichinella infestation of the paraspinal musculature. After undergoing decompressive surgery and albendazole therapy, the patient made a remarkable improvement. Although primary echinococcosis can affect the spine, concomitant infestation with Trichinella has not been reported. Surgery has to be followed by albendazole therapy.

Distribution of CCR5delta32 in human immunodeficiency virus-infected children and its relationship to disease course.

Homozygosity for a 32-bp deletion in the CCR5 gene (CCR5delta32) has been shown to confer resistance to infection with the macrophage-tropic strain of human immunodeficiency virus (HIV) type 1. We examined the distribution of CCR5delta32 in 47 children (age range, 1.5 to 19 years), of whom 43 were infected with HIV, by the perinatal route (n = 41) or by the intravenous route (n = 2). The infected patients were classified as rapid progressors (RP) (n = 7) (CDC category C3 or death by 2 years of age), non-rapid progressors (NRP) (n = 17) (survival for > or =8 years after infection), or intermediate (n = 19). CCR5delta32 heterozygosity was found in two HIV-infected children, both NRP. None of the subjects were homozygous for CCR5delta32, and the remaining children had no evidence of CCR5delta32. The presence of CCR5delta32 heterozygosity in 4.8% of this, predominantly non-Caucasian population is consistent with the published distribution of the mutation. The finding that CCR5delta32 was present only in NRP and not in any RP is in agreement with previous reports suggesting that heterozygosity for CCR5delta32 may confer limited protection from disease progression.

Evaluation of pharmacokinetics, safety, tolerance, and activity of combination of zalcitabine and zidovudine in stable, zidovudine-treated pediatric patients with human immunodeficiency virus infection. AIDS Clinical Trials Group Protocol 190 Team.

A double-blind phase II trial compared zalcitabine (0.03 mg/kg/day) in combination with zidovudine (720 mg/m2/day) and zidovudine monotherapy in 250 clinically stable, previously zidovudine-treated, human immunodeficiency virus-infected children. The combination was well-tolerated except for an increased incidence of neutropenia (14%) compared with that in children receiving monotherapy (5%). No differences were noted for time to first AIDS-defining illness or death, neuropsychologic status, or weight Z scores. In patients in the combination arm, the CD4 cell count decline was slower (13% per year) than in patients receiving monotherapy (25% per year) (P = .03), and quantitative peripheral blood mononuclear cell virus load remained lower at all time points (P = .08). Deaths were fewer in patients receiving combination therapy (4) compared with those in patients receiving monotherapy (10) (P = .083). Thus, administration of zidovudine with zalcitabine to children with prior zidovudine treatment did not result in a significant increase in toxicity compared with that resulting from zidovudine monotherapy and demonstrated improvement in immunologic and virologic surrogate markers.

Outcome and survival in HIV-infected infants with Pneumocystis carinii pneumonia and respiratory failure.

A retrospective chart review (January 1987-December 1994) of cases of histologically proven Pneumocystis carinii pneumonia (PCP) in 9 infants (ages 1.1-7 months) who had perinatally acquired human immunodeficiency-1 virus (HIV) infection was performed. None of the children was suspected of having HIV or had received PCP prophylaxis. Respiratory failure requiring mechanical ventilation developed in all 9 children. Comparison of survivors (5) with nonsurvivors (4) showed no significant differences in the age of onset, weight for length, hemoglobin level, total protein/albumin, lactic dehydrogenase (LDH), liver function tests, lymphocyte numbers and functions, time on mechanical ventilation, treatment received (including the use of steroids), and other complications occurring during the acute phase of pneumonia. The survivors had significantly higher platelet counts than nonsurvivors (mean 516 K versus 237 K, p = 0.02), a trend toward lower arterial-alveolar (A-a) gradient (mean 415 versus 218, p = 0.07), and earlier use of steroids after the onset of illness (2.5 versus 1 day, p = 0.06). Four of 5 children treated after December 1989 survived compared to 1 of 4 prior to that. Four survivors followed for a median length of 29 months (range 28-32 months) had stable physical and neurocognitive development, improvement in CD4+ T cell counts [mean 27% (range 23-36%), absolute count-mean 1631 (range 1427-1631)] and immunologic functions, and decrease in p24 Ag in 3 of 4. The cellular proviral load measured by DNA quantitative polymerase chain reaction (QC-PCR) decreased (40 K to 17.3 K copies) in one of two patients studied at two time points. PCP continues to be a serious complication of HIV infection in infancy and aggressive preventive approaches seem warranted. In our institution no single factor was responsible for improved survival following PCP after 1989. Four of 5 survivors continued to do well 28-32 months after the acute episode.

Repeatedly positive human immunodeficiency virus type 1 DNA polymerase chain reaction in human immunodeficiency virus-exposed seroreverting infants.

Three human immunodeficiency virus type 1 (HIV-1)-exposed children who had repeatedly positive DNA polymerase chain reaction (PCR) tests for HIV in > or = 5 samples before seroreversion to HIV-negative status are reported. The children belong to a cohort of 210 infants who were born to HIV-infected mothers and were tested at intervals of 1 to 3 months by HIV viral culture, PCR, and p24 antigen; only the PCR was positive in > or = 5 samples in the children reported here. Their clinical features were indistinguishable from other seroreverters. All three children had a transient drop in CD4:CD8 ratio to < 1.0. The transiently positive DNA PCR in HIV-exposed infants may indicate either that HIV infection was eliminated by a strong host immune response or that infection was caused by an attenuated/defective strain of virus.

Tuberculosis in human immunodeficiency virus-infected children. A family infection.

OBJECTIVE: To study the epidemiologic and clinical features of infection with Mycobacterium tuberculosis in human immunodeficiency virus (HIV)-infected children and their families. PATIENTS AND CLINICAL SETTING: Sixty families of children with HIV infection, children of HIV indeterminate status, and seroreverters underwent follow-up in a comprehensive multidisciplinary program for children and families. METHODS: Infection with M tuberculosis was diagnosed based on a positive Mantoux test result or a positive culture. RESULTS: Mycobacterium tuberculosis infection was diagnosed in seven children (three infected with HIV, three seroreverters, and one uninfected sibling of an infected child) from four families (6%). All infections were detected in the period from March 1990 through January 1992. Six of seven children had a history of exposure to M tuberculosis in an HIV-infected adult (parent) who was an intravenous drug user, homeless, and/or noncompliant with the medical regimen. All HIV-infected children and one seroreverter had pulmonary tuberculosis. One child died of complications of tuberculosis and HIV infection. The M tuberculosis isolated from this child was resistant to isoniazid, rifampin, and streptomycin sulfate. CONCLUSIONS: Tuberculosis is a growing problem among inner-city children born to HIV-infected parents. Children infected with HIV in this study had symptomatic and severe disease with tuberculosis, which reflected the drug susceptibility pattern of M tuberculosis seen in our community.