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Background: Cash transfers are key interventions in the World Health Organisation's post-2015 global TB policy. However, evidence guiding TB-specific cash transfer implementation is limited. We designed, implemented and refined a novel TB-specific socioeconomic intervention that included cash transfers, which aimed to support TB prevention and cure in resource-constrained shantytowns in Lima, Peru for: the Community Randomized Evaluation of a Socioeconomic Intervention to Prevent TB (CRESIPT) project., Methods: Newly-diagnosed TB patients from study-site healthposts were eligible to receive the intervention consisting of economic and social support. Economic support was provided to patient households through cash transfers on meeting the following conditions: screening for TB in household contacts and MDR TB in patients; adhering to TB treatment and chemoprophylaxis; and engaging with CRESIPT social support (household visits and community meetings). To evaluate project acceptability, quantitative and qualitative feedback was collected using a mixed-methods approach during formative activities. Formative activities included consultations, focus group discussions and questionnaires conducted with the project team, project participants, civil society and stakeholders., Results: Over 7 months, 135 randomly-selected patients and their 647 household contacts were recruited from 32 impoverished shantytown communities. Of 1299 potential cash transfers, 964 (74 %) were achieved, 259 (19 %) were not achieved, and 76 (7 %) were yet to be achieved. Of those achieved, 885/964 (92 %) were achieved optimally and 79/964 (8 %) sub-optimally. Key project successes were identified during 135 formative activities and included: strong multi-sectorial collaboration; generation of new evidence for TB-specific cash transfer; and the project being perceived as patient-centred and empowering. Challenges included: participant confidence being eroded through cash transfer delays, hidden account-charges and stigma; access to the initial bank-provider being limited; and conditions requiring participation of all TB-affected household members (e.g. community meetings) being hard to achieve. Refinements were made to improve project acceptability and future impact: the initial bank-provider was changed; conditional and unconditional cash transfers were combined; cash transfer sums were increased to a locally-appropriate, evidence-based amount; and cash transfer size varied according to patient household size to maximally reduce mitigation of TB-related costs and be more responsive to household needs., Conclusions: A novel TB-specific socioeconomic intervention including conditional cash transfers has been designed, implemented, refined and is ready for impact assessment, including by the CRESIPT project. The lessons learnt during this research will inform policy-makers and decision-makers for future implementation of related interventions.

Disorganization (Ds) is a mouse mutant in which heterozygotes manifest defects in various organ systems including cranioschisis, hamartomas, limb abnormalities, body wall defects, eye defects, and craniopharyngeal defects. We report four patients with developmental anomalies that are seen in amniotic band sequence (ABS) with additional anomalies that could not be explained by amniotic bands alone. The anomalies seen in our patients included facial malformation and clefting, brain anomalies (encephalocele, agenesis of the corpus callosum, holoprosencephaly), eye anomalies (anophthalmia, microphthalmia, and microcornea), and extremity and digit anomalies (talipes equinovarus, absent/hypoplastic phalanges, amputation of an extremity, syndactyly, polydactyly, and anomalous attachment of an extremity). Other systems involved include genitourinary anomalies (hydronephrosis and abnormal testes, epidydimis and seminal vesicles), and skin appendages similar to those seen in Ds mice. Presence of amniotic bands in addition to malformations not attributed to amniotic bands, were seen in three of four patients. The four patients in this report have involvement of at least four organ systems including the skin appendages that have been reported in the Ds mouse and have clinical findings that overlap the spectrum of Ds and ABS., (2009 Wiley-Liss, Inc.)

Using a large consortium of undergraduate students in an organized program at the University of California, Los Angeles (UCLA), we have undertaken a functional genomic screen in the Drosophila eye. In addition to the educational value of discovery-based learning, this article presents the first comprehensive genomewide analysis of essential genes involved in eye development. The data reveal the surprising result that the X chromosome has almost twice the frequency of essential genes involved in eye development as that found on the autosomes.

The classic clinical features in the 22q11.2 deletion syndrome are congenital heart defects, hypocalcemia, immunodeficiency, learning, speech, and behavioral difficulties. The phenotype is highly variable and continues to expand. We present two cases of absent uterus and unilateral renal agenesis in females with the 22q11.2 deletion. Clinicians caring for these adolescents should be aware of the possibility of renal anomalies and Mullerian agenesis. The diagnosis of 22q11.2 deletion may be considered in a female with Mullerian agenesis, particularly, in association with a history of learning difficulties and speech delay., (Copyright 2007 Wiley-Liss, Inc.)

Objective: To determine the pharmacokinetics of marbofloxacin after oral administration in juvenile harbor seals (Phoca vitulina) at a dose of 5 mg/kg (2.3 mg/lb) and to compare pharmacokinetic variables after pharmacokinetic analysis by naïve averaged, naïve pooled, and nonlinear mixed-effects modeling., Design: Original study. Animals-33 male and 22 female juvenile seals being treated for various conditions., Procedures: Blood collection was limited to < or = 3 samples/seal. Plasma marbofloxacin concentrations were measured via high-pressure liquid chromatography with UV detection., Results: Mean +/- SE dose of marbofloxacin administered was 5.3 +/- 0.1 mg/kg (2.4 +/- 0.05 mg/lb). The terminal half-life, volume of distribution (per bioavailability), and clearance (per bioavailability) were approximately 5 hours, approximately 1.4 L/kg, and approximately 3 mL/min/kg, respectively (values varied slightly with the method of calculation). Maximum plasma concentration and area under the plasma-time concentration curve were approximately 3 microg/mL and 30 h x microg/mL, respectively. Naïve averaged and naïve pooled analysis appeared to yield a better fit to the population, but nonlinear mixed-effects modeling yielded a better fit for individual seals., Conclusions and Clinical Relevance: Values of pharmacokinetic variables were similar regardless of the analytic method used. Pharmacokinetic variability can be assessed with nonlinear mixed-effects modeling, but not with naïve averaged or naïve pooled analysis. Visual observation by experienced trainers revealed no adverse effects in treated seals. Plasma concentrations attained with a dosage of 5 mg/kg every 24 hours would be expected to be efficacious for treatment of infections caused by susceptible bacteria (excluding Pseudomonas aeruginosa).

Lead poisoning is a preventable environmental disease. Children and developing fetuses are especially vulnerable; even low blood lead levels (BLLs) are linked with learning and behavioral problems. We assessed children's and their caregivers' BLLs and risk factors for lead exposure in Chuuk State, Federated States of Micronesia. Children aged 2-6 years were randomly selected within 20 randomly selected villages. Children and caregivers provided venous blood, and caregivers offered information about possible risk factors for lead exposure. Mean BLLs were 39 microg/l for children and 16 microg/l for caregivers. Children with BLLs of > or = 100 microg/l (elevated) were 22.9 (95% CI: 4.5-116.0) times more likely to have a caregiver with an elevated BLL, 6.2 (95% CI: 1.4-27.3) times more likely to live on an outer island, and 3.4 (95% CI: 1.7-6.9) times more likely to have a family member who made lead fishing weights than did other children even after controlling for age and sex. For children, 61% of elevated BLLs could be attributed to making fishing weights. Caregivers with elevated BLLs were 5.9 (95% CI: 1.5-23.7) times more likely to live in a household that melted batteries than other caregivers even after controlling for age and education. For caregivers, 37% of the elevated BLLs could be attributed to melting batteries. The association of elevated BLLs in children and their caregiver suggests a common environmental exposure. Melting batteries to make fishing sinkers is a preventable source of lead exposure for children and their caregivers in Chuuk. Published by Elsevier GmbH.

Background: Severe anomalies of the forebrain together with radial limb anomalies have been reported in Steinfeld syndrome, XK aprosencephaly, and partial monosomy 13q. Steinfeld syndrome is an extremely variable autosomal dominant condition that, in severe cases, is characterized by holoprosencephaly, radial limb defects, and renal and/or cardiac defects. In mild cases there may be only thumb hypoplasia, ocular coloboma, or oral clefts. XK aprosencephaly, also called Garcia-Lurie syndrome (GLS), is a usually sporadic disorder with radial limb defects and aprosencephaly/atelencephaly. Based on two atypical sibships, autosomal recessive inheritance has been suggested. Two patients with variations of monosomy 13q have been described with atelencephaly but, generally, Steinfeld and XK aprosencephaly patients are chromosomally normal. Holoprosencephaly in 13q deletion patients appears to be due to ZIC2 mutations, but ZIC2 has not been previously tested in Steinfeld syndrome or GLS patients., Cases: We report three sporadic cases with clinical features intermediate between Steinfeld and GLS, including severe forebrain malformations and radial limb defects. All had normal karyotypes, and mutations in ZIC2 were absent in the two cases tested., Conclusions: In our cases and in the literature there is significant clinical overlap between Steinfeld syndrome and GLS. We propose these conditions may not be nosologically or etiologically distinct. The spectrum of severe forebrain anomalies in these conditions is broader than previously thought and may include some neural tube defects. Mild cases are difficult to identify and the full range of expression remains unknown. Autosomal dominant inheritance with incomplete penetrance and frequent new mutations is postulated. Thorough clinical evaluation is recommended for children with severe forebrain and radial limb defects.

Amikacin, an aminoglycoside antimicrobial, was administered to a killer whale (Orcinus orca) and a beluga whale (Delphinapterus leucas) for the treatment of clinical signs consistent with gram-negative aerobic bacterial infections. Dosage regimens were designed to target a maximal plasma concentration 8-10 times the minimum inhibitory concentrations of the pathogen and to reduce the risk of aminoglycoside toxicity. Allometric analysis of published pharmacokinetic parameters in mature animals yielded a relationship for amikacin's volume of distribution, in milliliters, given by the equation Vd = 151.058(BW)1.043. An initial dose for amikacin was estimated by calculating the volume of distribution and targeted maximal concentration. With this information, dosage regimens for i.m. administration were designed for a killer whale and a beluga whale. Therapeutic drug monitoring was performed on each whale to assess the individual pharmacokinetic parameters. The elimination half-life (5.99 hr), volume of distribution per bioavailability (319 ml/kg). and clearance per bioavailability (0.61 ml/min/kg) were calculated for the killer whale. The elimination half-life (5.03 hr), volume of distribution per bioavailability (229 ml/kg). and clearance per bioavailability (0.53 ml/min/kg) were calculated for the beluga whale. The volume of distribution predicted from the allometric equation for both whales was similar to the calculated pharmacokinetic parameter. Both whales exhibited a prolonged elimination half-life and decreased clearance when compared with other animal species despite normal renal parameters on biochemistry panels. Allometric principles and therapeutic drug monitoring were used to accurately determine the doses in these cases and to avoid toxicity.

We present three families with infantile myofibromatosis (IM; OMIM no. 228550) inherited in an autosomal dominant (AD) manner. These three pedigrees prompted re-assessment of pedigrees available within the genetic, oncologic, surgical, and pathologic literature, which suggest autosomal recessive (AR) inheritance. All familial IM may be interpreted as AD or, alternatively, there may be genetic heterogeneity for IM. As most nodules tend to regress spontaneously, familial history may be difficult to obtain and/or confirm. Clinical diagnosis and establishment of inheritance pattern can be important for prognosis and the recognition that other family members may be affected., (Copyright 2004 Wiley-Liss, Inc.)

Biventricular hypertrophy was noted at 24 weeks' gestation in a fetus with isolated cytochrome-c oxidase (COX) deficiency. Shock, caused by hypertrophic cardiomyopathy and severe pulmonary hypertension, led to the patient's death on day 6. His phenotype defines a new lethal variant of COX deficiency characterized by prenatal-onset cardiopulmonary pathophysiology.

The constellation of features in Crane-Heise syndrome (CHS) includes 1) poorly mineralized calvarium, 2) characteristic facial anomalies, and 3) extracranial skeletal anomalies that involve both vertebral anomalies and absent clavicles. Since the original report of Crane and Heise [1981: Pediatrics 68:235-237] describing three affected siblings, there have been few isolated cases published. We present two siblings from a second pedigree with features strikingly similar to those presented in the original publication, and distinct clinical differences, including distal phalangeal hypoplasia and mild cardiac and GI abnormalities that may represent familial variation. Despite extensive cytogenetic, molecular, and biochemical analyses, we could not elucidate a molecular mechanism for this rare phenotype., (Copyright 2003 Wiley-Liss, Inc.)

Background/purpose: Epithelial tumors of the ovary account for about 15% of pediatric ovarian masses. The authors reviewed a 14-year experience with ovarian masses to understand the spectrum of pathology, presentation, and outcome of children with epithelial lesions., Methods: All ovarian masses resected or biopsied at the authors' institution from 1988 to the present were reviewed retrospectively. Patient age, presenting symptoms, operative procedures, postoperative treatment, and outcome were obtained from the medical record., Results: A total of 240 cases were identified. A total of 123 (51.2%) were nonneoplastic in nature. Of the 117 neoplastic masses, 79 (67.5%) were germ cell tumors and nineteen (16.2%) were epithelial-derived tumors. Patients with epithelial tumors had a mean age of 13.9 +/- 4 years. Ten patients (52.6%) presented with abdominal pain, and 9 (47.4%) presented with an asymptomatic mass. Eleven underwent oophorectomy, 6 underwent cystectomy, and 2 had biopsy alone. Four patients (21%) had ascites, and 1 (5.3%) had a pleural effusion. The histopathologic diagnoses for the epithelial tumors included 9 serous cystadenomas (47%) and 3 mucinous cystadenomas (16%), 3 mucinous cystadenocarcinomas (16%), and 4 serous tumors of borderline malignancy (21%). Two patients (11%) had bilateral disease. Four patients (21%) underwent a subsequent laparotomy for either staging or recurrence, and 2 patients (11%) required chemotherapy. One patient (5.3%) died of ovarian adenocarcinoma., Conclusions: Epithelial tumors comprise a small but significant proportion of pediatric ovarian masses. The pediatric surgeon must understand the biologic characteristics, operative management, and follow-up treatment of these tumors, and how these differ from germ cell lesions., (Copyright 2003, Elsevier Science (USA). All rights reserved.)

The WT1 gene encodes a transcription factor implicated in normal and neoplastic development. The purpose of this study was to evaluate the diagnostic utility of a commercial WT1 antibody on a variety of pediatric small round blue cell tumors (SRBCT). A mouse monoclonal antibody (clone: 6F-H2, DAKO) raised against the N-terminal amino acids 1-181 of the human WT1 protein was tested. Microscopic sections from 66 specimens were stained using an antigen retrieval protocol with trypsin. The tumors included peripheral neuroectodermal tumors (PNET/Ewing's), neuroblastomas, desmoplastic small round cell tumors (DSRCT), lymphomas, Wilms' tumors, and rhabdomyosarcomas (RMS). One RMS case was investigated by Western blot analysis and RT-PCR to confirm the antibody specificity. A strong cytoplasmic staining was demonstrated in all RMS (11/11). The Western blot analysis confirmed the WT1 protein in the tissue, and the RT-PCR confirmed the presence of WT1 mRNA in the peripheral blood and tissue of one RMS patient. The Wilms' tumors had a variable nuclear and/or cytoplasmic positivity in most (17/24) cases. All PNET/Ewing's were negative. The nuclei of two lymphoblastic lymphomas stained strongly. A weak nuclear or cytoplasmic staining was reported in a few DSRCT (3/5), lymphomas (2/10), and neuroblastomas (2/8). This is a useful antibody in the differentiation of RMS from other SRBCTs. A strong cytoplasmic staining favors an RMS, and a strong nuclear staining is suggestive of a Wilms' tumor. A role for WT1 in the pathogenesis of rhabdomyosarcomas is raised. The limited sampling precludes any conclusions regarding the value of tissue or peripheral blood analysis for WT1 mRNA in patients with rhabdomyosarcoma.

A potential consequence of systemic administration of viral vectors is the inadvertent introduction of foreign DNA into recipient germ cells. To evaluate the safety of in vivo recombinant adeno-associated virus (rAAV) mediated gene transfer approaches for hemophilia B, we explored the risk of germline transmission of vector sequences following intramuscular (IM) injection of rAAV in four species of male animals (mouse, rat, rabbit and dog). In vector biodistribution studies in mice and rats, there is a dose-dependent increase in the likelihood that vector sequences can be detected in gonadal DNA using a sensitive PCR technique. However, in dogs DNA extracted from semen is negative for vector sequences. To address this discrepancy, studies were done in rabbits, and both semen and testicular DNAs were analyzed for the presence of vector sequences. These studies showed that no AAV vector sequences were detected in DNA extracted from rabbit semen samples collected at time points ranging from 7 to 90 days following IM injection of 1 x 10(13) vector genomes rAAV (vg) per kg. In contrast, DNA extracted from gonadal tissue was positive for vector sequences, but the positive signals diminished in number and strength with time. By FISH analysis, AAV signals were localized to the testis basement membrane and the interstitial space; no intracellular signal was observed. We observed similar findings following hepatic artery administration of rAAV in rats and dogs, suggesting that our findings are independent of the route of administration of vector. Attempts to transduce isolated murine spermatogonia directly with AAV-lacZ were unsuccessful. In clinical studies human subjects injected IM with an AAV vector at doses up to 2 x 10(12) vg/kg have shown no evidence of vector sequences in semen. Together, these studies suggest that rAAV introduced into skeletal muscle or the hepatic artery does not transduce male germ cells efficiently. We conclude that the risk of inadvertent germline transmission of vector sequences following IM or hepatic artery injection of AAV-2 vectors is extremely low.

Human parvovirus B19 can cause congenital infection with variable morbidity and mortality in the fetus and neonate. Although much information exists on the B19-specific antibody response in pregnant women, little information is available describing the cell-mediated immune (CMI) response at the maternal-fetal interface. The focus of this study was to characterize the CMI response within placentas from women who seroconverted to B19 during their pregnancies and compare it to controls. Immunohistochemical techniques were used to identify the various immune cells and the inflammatory cytokine present within placental tissue sections. Group 1 consisted of placentas from 25 women whose pregnancies were complicated by B19 infection; 6 women with good outcome (near-term or term delivery), and 19 with poor outcome (spontaneous abortion, nonimmune hydrops fetalis, or fetal death). Group 2 consisted of placentas from 20 women whose pregnancies were complicated with nonimmune hydrops fetalis of known, noninfectious etiology. Group 3 consisted of placentas from eight women whose pregnancies ended in either term delivery or elective abortion. The results of the study revealed a statistically significant increase in the number of CD3-positive T cells present within placentas from group 1 compared to group 2 or 3 (13.3 versus 2 and 1, respectively) (P < 0.001). In addition, the inflammatory cytokine interleukin 2 was detected in every placenta within group 1 but was absent from all placentas evaluated from groups 2 and 3. Together, these findings demonstrate evidence for an inflammation-mediated cellular immune response within placentas from women whose pregnancies are complicated with B19 infection.

In order to study the pathogenesis of prenatal deformities, we reviewed maternal histories, delivery records, pathology reports, radiographs, and photographs of 90 fetuses with prenatally documented oligohydramnios at gestational ages from 14 weeks to term. The causes of oligohydramnios included premature rupture of membranes (44 cases), fetal renal insufficiency (25 cases), idiopathic (15 cases), and twin-twin transfusion (6 cases). The fetuses were grouped according to gestational age at delivery and duration of oligohydramnios. Sixty-three fetuses (70%) had documented contractures. As expected, contractures were more frequent with earlier onset and longer duration of oligohydramnios. During the 2nd trimester, the frequency of contractures in fetuses with oligohydramnios was 77% compared to 52% in the 3rd trimester (chi(2) = 5.33, 1 df, P =.02). Considering all gestational ages together, 57% of fetuses had contractures after less than 2 weeks of oligohydramnios compared to 81% of fetuses with a longer duration of oligohydramnios (chi2 = 6.23, 1 df, P <.02). The type of contracture varied with gestational age. Clubfoot was the most frequent at all ages, but hand contractures such as camptodactyly were common only in the 2nd trimester while the broad flat hand originally described in Potter sequence was found almost exclusively in the fetuses with oligohydramnios in the 3rd trimester. Of the 63 fetuses with oligohydramnios and contractures, 25 (40%) had either additional malformations or family history that could explain contractures independent of oligohydramnios., (Copyright 1999 Wiley-Liss, Inc.)

The perception of face gender was examined in the context of extending "face space" models of human face representations to include the perceptual categories defined by male and female faces. We collected data on the recognizability, gender classifiability (reaction time to classify a face as male/female), attractiveness, and masculinity/femininity of individual male and female faces. Factor analyses applied separately to the data for male and female faces yielded the following results. First, for both male and female faces, the recognizability and gender classifiability of faces were independent--a result inconsistent with the hypothesis that both recognizability and gender classifiability depend on a face's "distance" from the subcategory gender prototype. Instead, caricatured aspects of gender (femininity/masculinity ratings) related to the gender classifiability of the faces. Second, facial attractiveness related inversely to face recognizability for male, but not for female, faces--a result that resolves inconsistencies in previous studies. Third, attractiveness and femininity for female faces were nearly equivalent, but attractiveness and masculinity for male faces were not equivalent. Finally, we applied principal component analysis to the pixel-coded face images with the aim of extracting measures related to the gender classifiability and recognizability of individual faces. We incorporated these model-derived measures into the factor analysis with the human rating and performance measures. This combined analysis indicated that face recognizability is related to the distinctiveness of a face with respect to its gender subcategory prototype. Additionally, the gender classifiability of faces related to at least one caricatured aspect of face gender.

We describe a reaction tray for a heterogeneous chemiluminescence (CL) immunoassay having the following features: separate sample incubation and signal detection wells; a design that allows for noncontact transfer of the reaction mixture from incubation wells to detection wells; surface features to mate with a detector and create a light-tight seal for CL detection; and self-contained means for liquid removal. The reaction mixture is transferred by injecting a wash solution from a group of nozzles into the incubation well. Quantitative transfer of microparticles (transfer efficiencies greater than 95% and CV less than 5%) is achieved by injecting two 300-microL pulses of transfer solution at a rate of 2.1 m/s. The performance of the tray and method of transfer is tested by determining the precision of CL signal for a sample containing a concentration of anti-hepatitis B core antigen (anti-HBc) or hepatitis B surface antigen (HBsAg) close to the cutoff value for the assay.

This study sought to determine if lottery play along with other possible causes engenders problem gambling. Problem gambling was defined as a progression and measured by three scales: Gambling behavior, loss of control over gambling and gambling consequences. Possible causes of problem gambling included lottery play, personality traits, exposure to gambling, leisure pursuits, marital status, residence, and other background characteristics of respondents. Respondents are a stratified random sample of adult residents of Iowa contacted by telephone in May-June, 1989. It was found that lottery play is a predictor of gambling behavior, as well as loss of control and gambling consequences when previous stages of problem gambling were deleted from the analysis as predictor variables. Other predictors of the latter stages of problem gambling include its earlier stages, as well as personality traits and various background characteristics of respondents. The relevance of the findings for theory and future research on gambling are discussed.

Here we describe a fetus in whom a cystic hygroma was detected by ultrasound during the second trimester. Autopsy demonstrated a female fetus with manifestations of Ullrich-Turner syndrome, including gonadal dysgenesis, generalized lymphedema, and preductal aortic coarctation. Surprisingly, the karyotype was 46,XY, with no evidence of mosaicism for a 45,X cell line. Y-DNA hybridization studies demonstrated a deletion of the sex-determining segment of the short arm of the Y chromosome. This is the first report, in a fetus, of XY Ullrich-Turner syndrome due to a Y chromosome deletion.

In a collaborative study, 48 prepubertal boys with undescended testes ranging in age from 15 months to 11 years were treated with low-dose intranasal buserelin following an every-other-day programme for a period of 6 months. Urinary LH, FSH, and testosterone were not altered during the treatment period. Boys over 7 years of age experienced a slight but significant rise in testosterone at the end of treatment. Testicular descent was achieved in only 17% of boys. In the remainder, bilateral testicular biopsies were obtained during orchiopexy. Grouped analysis showed a significant increase in the number of germ cells per tubule in both unilateral and bilateral cryptorchid boys, suggesting that buserelin treatment of the testis in a cryptorchid position is capable of improving fertility potential. If time-matched controls are compared to treated boys of the same age, again a significant difference is observed indicating that buserelin treatment does increase the germ cell count.

All pediatric autopsies of patients with hypoplastic left heart syndrome seen during an 11-year interval were reviewed to determine the frequency of underlying chromosomal and single-gene defects and idiopathic major extracardiac anomalies associated with this common, lethal congenital heart abnormality. Of 83 patients identified, nine had underlying chromosomal abnormalities, four had single-gene defects, ten had one or more major extracardiac anomalies without an identifiable chromosomal or mendelian disorder, and two were infants of insulin-dependent diabetic mothers. Overall, 23 patients (28%) had a genetic disorder and/or major extracardiac anomaly. The substantial prevalence of genetic causes of and major extracardiac anomalies associated with hypoplastic left heart syndrome underscores the need for a detailed genetic evaluation for all patients with hypoplastic left heart syndrome.

The purpose of this study was to review the literature pertaining to non-surgical cure rates for strabismus published since 1958 and compare it to Flom's prognostic model. However, no studies were found that could be compared directly to Flom's model. One reason for this was due to the use of different definitions of a cure by different clinicians. Another reason was the failure to categorize the data according to the effect that retinal correspondence, frequency, and direction of the deviation had on the cure rates. From the studies which specified Flom's functional cure or its equivalent, it was determined that strabismic cure rates could be broken down as follows: Constant esotropia-29%; Intermittent esotropia-73% Constant exotropia-53%; Intermittent exotropia-62% Suggestions were made for the reporting of data to make future research more comparable and useful to the practitioner.

We studied 48 prepubertal boys with cryptorchidism between 1 year 3 months and 11 years old who were treated with buserelin every other day for 6 months. Urinary luteinizing and follicle-stimulating hormones, and testosterone remained unchanged during the entire treatment period. In boys older than 7 years a slight but significant increase in testosterone was noted in the first morning voided urine at the end of treatment. Testicular biopsies were obtained at orchiopexy in all patients in whom testicular descent was not complete (83 per cent). A significant increase in the number of germ cells was observed in patients with unilateral and those with bilateral cryptorchidism, indicating that 6 months of buserelin therapy improved the fertility status even when testes were in an undescended position during treatment.

This interlaboratory study was conducted to examine four erythrocyte protoporphyrin control materials from Aviv Biomedical, Helena Laboratories, Kaulson Laboratories, and the New York State Department of Health for use with hematofluorometers. Our principal aims were to monitor the stability of these materials at three different storage temperatures (room, refrigerator, freezer) and, where appropriate, to validate the manufacturer's target values. Measurements for the study were generated in three reference laboratories that used a total of five hematofluorometers, three from Environmental Science Associates and two from Aviv Biomedical. Each instrument was calibrated against a consensus acetic acid-ethyl acetate extraction procedure. We found the materials from Aviv to be the most stable, followed by the New York State material. However, the target values assigned by Aviv were not within the acceptable range determined by consensus. The target values assigned by Kaulson Laboratories for their materials did fall within the acceptable consensus range, but they were the least stable of the materials evaluated. The materials from Helena Laboratories were originally designed for use as calibrators with Helena's "ProtoFluor Z" hematofluorometer, which reports in different units. They were deemed unsuitable for use as control materials with the Aviv or Environmental Science Associates hematofluorometers because of the narrow range of values and the wide scatter of results.

Nineteen male infants died with a large omphalocele and 52% had associated cryptorchidism. However, two different groups with both omphalocele and cryptorchidism were recognized: (1) Eleven patients with omphalocele without brain malformation and an incidence of undescended testes not significantly different from the normal population; (2) Eight patients with omphalocele and brain malformation all having cryptorchidism. A comparison of the groups indicated that intact intraabdominal pressure during intrauterine life is not a main driving force of testicular descent, whereas normal testicular descent may occur only when the brain is normally developed. Whenever a child with omphalocele and cryptorchidism is examined, careful evaluation of the central nervous system is indicated. This triad of malformations may have prognostic and therapeutic implications.

In 3 male patients, chronic pulmonary sequelae followed influenza virus infection at 5, 24, and 42 months of age. Varying degrees of interstitial fibrosis, bronchial and bronchiolar erosions and metaplasia, obliterative bronchiolitis, and interstitial chronic inflammatory infiltrates were found on lung biopsy. Influenza A/Hong Kong/68 (H3N2) virus was isolated from the lung tissue of one patient 8 weeks after the onset of illness. This is the longest persistence of infectious virus in lung tissue yet reported. Persistent radiographic abnormalities included peribronchial thickening, interstitial densities, bronchiectasis, obliterative bronchiolitis, and segmental atelectasis. Pulmonary function tests showed an obstructive restrictive pattern, with mild improvement after bronchodilation and with deterioration after exercise. These observations suggest that influenza virus infection may be more serious in infants and young children than has been previously recognized and may contribute to the pathogenesis of unexplained interstitial pneumonitis, pulmonary fibrosis, obliterative bronchiolitis, and bronchiectasis.