A Qualitative Study Exploring Parental Perceptions of Telehealth in Early Intervention.

Using telehealth as a mode of service delivery has the potential to address some long-standing challenges in early intervention (EI) services such as waiting lists to access services. Yet, little is known about parent perceptions of telehealth in EI based on their lived experiences partnering with EI practitioners. The purpose of this study was to explore parent perceptions on using telehealth, especially on family-professional partnerships and coaching. Interviews were conducted with 15 parents of children receiving EI services via telehealth from June to August of 2021. Almost half of the participants reflected under-represented racial and ethnic backgrounds. Constant comparative analysis and emergent coding were used for data analysis. The findings showed that the advantages outnumbered the disadvantages regarding telehealth. Participants reported that telehealth provided a safe and flexible option and eliminated the wait to access EI services. However, participants identified some disadvantages to telehealth including telehealth precluded substantive interactions with therapists and limited access to technology. The findings also indicated that telehealth enhanced family-professional partnerships. Nearly all participants valued coaching during telehealth. Participants suggested initial supports to facilitate EI via telehealth, including stable internet access, telehealth training, and an initial in-person visit. Implications for research and practice are discussed. Supplementary Information: The online version contains supplementary material available at 10.1007/s10882-022-09853-w.

A Longitudinal Analysis of the Relationships Between Social, Communication, and Motor Skills Among Students with Autism.

Many students with autism have difficulties engaging with their classroom environments and forming friendships, which are mostly affected by deficits in social, communication, and motor skills. The Special Education Elementary Longitudinal Study (SEELS, 2000) data set was used, focusing on elementary age students with autism, to explore the longitudinal relationships between social, communication, and motor skills and the mediating role of motor skills in between communication and social skills by using structural equational modeling analyses. Results show that (a) motor skills mediate the relationship between communication and social skills in elementary school, (b) there are significant longitudinal relationships among these skills in elementary school. Increased motor skills may improve social skills for students with autism in elementary school.

Hyperbilirubinemia and cholelithiasis in Chinese patients with hemoglobin H disease.

Hemoglobin H disease (HbH) is a hemoglobinopathy peculiar to parts of the world with high incidence alpha-thalassemia mutations. Among 90 HbH cases, 50 cases suffered from clinically significant jaundice (bilirubin >30 mmol/l), including 14 with severe jaundice (bilirubin >60 mmol/l). Cholelithiasis was found in 38 cases. The incidence is roughly eight times higher than that in background control population but 50% lower than that in beta-thalassemia. The risk of gallstones was related to higher bilirubin levels but not alpha-globin genotype, sex, ferritin, and hemoglobin levels. Homozygotes or double heterozygotes for Gilbert alleles (17.2%), but not heterozgyotes (42.2%), were found to have a significantly increased risk of gallstones and jaundice. However, common Chinese Gilbert syndrome alleles do not completely explain the variable risks.

Two novel factor X gene mutations in a Chinese family with factor X deficiency.

We report a factor X (FX)-deficient Chinese family with two novel FX gene (F10) mutations. Two sibling probands had a bleeding tendency since childhood. Both had very low FX:C (<0.01 IU/ml) and FX:Ag (5-6%) levels and were heterozygous for two novel F10 mutations, a 2-bp GC deletion involving nucleotides 33 and 34, leading to premature chain termination at residue 45, and a T237-->C mutation, leading to Phe71-->Ser. A family study confirmed that the mother had the 2-bp GC deletion and a type I FX deficiency. The father had the Phe71-->Ser mutation. Interestingly, a type I FX deficiency was also observed, suggesting that Phe71-->Ser, occurring at a site sandwiched between two Gla residues, might perturb FX protein stability.

Risk factors for development of paradoxical response during antituberculosis therapy in HIV-negative patients.

The risk factors for development of paradoxical response were studied in a cohort of 104 patients with culture-documented Mycobacterium tuberculosis infection. Paradoxical deterioration occurred in 16 (15.4%) patients (case group) during antituberculosis therapy, involving lungs and pleura (n=4), spine and paraspinal tissue (n=5), intracranium (n=3), peritoneum (n=2), bone and joint (n=1), and lymph node (n=1). The median time from commencement of treatment to paradoxical deterioration was 56 days (range, 20-109 days). Compared with 53 patients without clinical deterioration after antituberculosis therapy (control group), patients with paradoxical response were more likely to have extrapulmonary involvement (62.5% vs. 17.0%; P<0.05) at initial diagnosis, to have lower baseline lymphocyte counts (672+/-315 cells/microl vs. 1,328+/-467 cells/microl; P<0.001), and to exhibit a greater surge in lymphocyte counts (627+/-465 cells/microl vs. 225+/-216 cells/microl; P<0.05) during paradoxical response. Further studies on lymphocyte subsets and cytokine levels would be useful in understanding the exact immunological mechanisms involved in immunorestitution.

Anti-thymocyte globulin treatment of marrow aplasia associated with paroxysmal nocturnal haemoglobinuria (PNH) resulted in haematological improvement due to an expansion of the PNH clone.

A patient with aplastic anaemia developed paroxysmal nocturnal haemoglobinuria (PNH) 4 years after diagnosis, with an ensuing haematopoietic improvement. The PNH clone subsequently declined, leading to pancytopenia again. Anti-thymocyte globulin had to be administered 14 years later, which resulted in haematopoietic improvement once more. Flow cytometric analysis showed that this was attributable to expansion of the PNH clone, owing probably to alleviation of its suppression by immune-mediated mechanisms. PIG-A gene analysis showed that the same PNH clone had waned and waxed in the clinical course. Our results suggest that the PNH clone might rarely be an immune target as well.

Distinct IL-6 signal transduction leads to growth arrest and death in B cells or growth promotion and cell survival in myeloma cells.

In B cell development, interleukin-6 (IL-6) induces terminal maturation of B lymphocytes into antibody producing plasma cells. Terminal differentiated B cells cell cycle arrest and death follows. In contrast, IL-6 acts as a growth factor for malignant myeloma plasma cells and in some cases protects them from therapeutic treatment. In this study, we examined two cell lines that show different responses to IL-6. Lymphoblastoid CESS cells respond to IL-6 by terminally differentiating into antibody producing plasma cells, cell cycle arrest, and undergo cell death. Continuous addition of IL-6 to these cells induces transient activation of STAT3, SHP-2 phosphorylation, and does not alter bcl-X(L) and c-myc expression. In contrast, the myeloma line ANBL6 proliferates when stimulated with IL-6 and this correlates with prolonged STAT3 activation and up-regulation of bcl-X(L) and c-myc. Interestingly, gp130-associated SHP-2 phosphorylation was detected in the IL-6-induced CESS cells but not myeloma cell lines. The data show a very distinct IL-6 signal transduction and kinetics in these cell lines and the distinct molecular events correlate closely to the cell fate of the lymphoblast and myeloma cell lines.

The bone marrow stromal microenvironment influences myeloma therapeutic response in vitro.

The bone marrow microenvironment supports growth and differentiation of normal hematopoietic cells and can contribute to malignant growth. Since myeloma cells localize and accumulate in bone marrow, it is important to understand the influence of the bone marrow microenvironment not only on the growth of the malignant cells, but also on the therapeutic response of myeloma cells. Growth factors such as interleukin-6 (IL-6) produced by bone marrow stromal cells can protect myeloma cells from glucocorticoid-induced apoptosis. We examined the effect of myeloma cells-bone marrow stromal cells interaction in vitro on several therapeutic treatments. An interleukin-6-dependent myeloma cell line ANBL6 was used and treated with dexamethasone, doxorubicin, and melphalan in the presence of bone marrow stromal cells. Stromal cells were able to protect ANBL6 from dexamethasone, but significantly enhanced the effect of doxorubicin and melphalan. IL-6-induced bcl-XL and cyclin D2 expression in ANBL6 cells, but dexamethasone was able to suppress both bcl-XL and cyclin D2 expression in ANBL6. Doxorubicin and melphalan were able to suppress bcl-XL expression only in the presence of IL-6. We also looked at the effect of activating mutations of N-ras in myeloma cells interacting with stromal cells on therapeutic responses. Surprisingly, ANBL6 N-ras shows significant resistance to all drugs used. Notably, the presence of stromal cells did not alter ANBL6 Nras cells' drug resistance. These results suggest both the bone marrow microenvironment and genetic alterations of myeloma cells can independently impact on therapeutic responses.

Substance P-induced enhanced permeability of dura mater microvessels is accompanied by pronounced ultrastructural changes, but is not dependent on the density of endothelial cell anionic sites.

Experimental data point to a determinant role for endothelial cell (EC) anionic sites in the regulation of vascular permeability. Previous studies have shown that EC anionic sites density is reduced in conditions of enhanced permeability. The pathophysiology of migraine and vascular headache encompasses dilatation of dural vessels and extravasation of plasma proteins. The current study was carried out to determine if the density of EC anionic sites is reduced in enhanced permeability of dural vessels. Enhanced permeability was chemically induced in rats by intravenous injection of substance P and was tested by assessing leakage of horseradish peroxidase (HRP). Anionic sites were labelled with cationic colloidal gold and their density was quantified from electron microscopy negatives. Experimental animals showed increased leakage of HRP from dural vessels. However, anionic sites in EC membranes (luminal and abluminal) showed no statistical differences when their mean densities in experimental and control animals were compared. The results indicate that in this model, factors other than the density of anionic sites may be important determinants in the permeability of dural vessels. Such factors may include structural alteration of ECs consistent with an increased permeability. In this study pronounced ultrastructural changes in ECs were noted in experimental animals including widening of intercellular junctions and an increase in the number of EC gaps and vesicles.

Delayed recurrent thromboembolism of the allograft kidney.

Renal artery thromboembolism is a rare event in native kidneys and has never been reported to occur in allograft kidney. We report a case of allograft kidney infarction secondary to embolisation from thrombus in the hypogastric artery supplying the allograft on two separate occasions 1 and 2 years after transplant. Anticoagulation therapy alone was given and the patient responded well with partial recovery of renal function.

The requirement of low calcium dialysate in patients on continuous ambulatory peritoneal dialysis receiving calcium carbonate as a phosphate binder.

In the present study we investigated the requirement of low calcium dialysate in 35 patients on continuous ambulatory peritoneal dialysis (CAPD) receiving calcium carbonate as the sole phosphate binder over a 12-month period. Patients with corrected serum calcium > or = 2.85 mmol/L after switching to oral calcium carbonate were given 1 to 3 2-litre exchanges of 2.5 mEq/L calcium dialysate. Serum phosphate level dropped from the pretreatment value of 2.95 +/- 0.62 to a level of between 1.70 +/- 0.41 to 2.03 +/- 0.44 mmol/L 2 weeks after therapy. Corrected serum calcium level increased significantly from 2 weeks onwards. Serum alkaline phosphatase rose initially at 2 and 6 weeks and decreased from 3 months onwards. Serum parathyroid hormone level dropped significantly from a mean pretreatment level of 569 to 320 pg/ml after 12 months (p < 0.001). Serum aluminum decreased significantly from a mean of 1.04 to 0.65 umol/L (p < 0.01). Daily calcium carbonate requirement fluctuated but tended to increase till 8 months and plateaued and ranged from 2.61 +/- 0.57 to 3.98 +/- 2.11 gm. The daily requirement of low calcium dialysate followed a similar trend with approximately three-quarters of patients ultimately requiring at least 1 bag of low calcium dialysate. Eight patients did not require low calcium dialysate. Patients who required low calcium dialysate were significantly older, had a significantly lower pretreatment serum parathyroid hormone and higher serum aluminum levels than those who did not.(ABSTRACT TRUNCATED AT 250 WORDS)

Isoniazid induced encephalopathy in dialysis patients.

Three dialysis patients with extrapulmonary tuberculosis developed confusion 4-14 days after commencement of treatment with anti-tuberculosis drugs, despite the use of prophylactic pyridoxine. Full recovery of conscious state resulted within 1 week in all patients after stopping isoniazid. In 2 patients confusion recurred on rechallenge of the drug. The risk factors of isoniazid induced encephalopathy and the dosage of isoniazid in uraemic patients were discussed.

A randomized prospective comparison of oral versus intraperitoneal ciprofloxacin as the primary treatment of peritonitis complicating continuous ambulatory peritoneal dialysis.

The present study compared oral versus intraperitoneal (ip) ciprofloxacin (ciproxin) as primary treatment of bacterial peritonitis in patients receiving continuous ambulatory peritoneal dialysis (CAPD) in a randomized, prospective trial. A total of 54 episodes in 46 patients were recruited for study. After excluding nonbacterial episodes and those not treated according to protocol, 48 episodes evenly divided between the two treatment arms were eligible for analysis. The primary cure rate was 41.7% and 66.7%, respectively, in the oral and ip treatment group. Half of those who failed or relapsed were due to infection with resistant, mostly gram-positive bacteria, which accounted for 79% of culture-positive episodes. Of the gram-positive isolates 42.3% were either resistant or intermediately susceptible to ciproxin compared to 16.7% of gram-negative isolates. The high level of bacterial resistance to ciproxin and treatment failure rate were related to the previous exposure to fluoroquinolones. Inadequate trough peritoneal drug levels also accounted for the failures in the ip but not the oral treatment group. We conclude that oral ciproxin is ineffective as a primary treatment of CAPD peritonitis in patients previously exposed to fluoroquinolones and that when administered ip, a dose of 50 mg/L instead of 25 mg/L of ciproxin should be used as maintenance in order to achieve adequate trough peritoneal drug levels.

An outbreak of Candida tropicalis peritonitis in patients on intermittent peritoneal dialysis.

An outbreak of Candida tropicalis peritonitis in intermittent peritoneal dialysis patients during a 6-week period is reported from a general hospital. Five patients were involved and there were three deaths. The strains recovered from affected patients were identical to those recovered from the water baths on the basis of biotyping, antimicrobial susceptibility pattern and chromosomal DNA fingerprints. No further cases were identified on subsequent surveillance after the prohibition of wet-warming of peritoneal dialysate in the hospital.

Disseminated intravascular coagulopathy associated with rifampicin.

A case of subclinical disseminated intravascular coagulopathy due to antituberculosis drugs, probably rifampicin, is described. The patient also developed marked leucocytosis, a 'flu-like illness, intravascular haemolysis, and acute renal failure as part of the drug reaction.

Herpes zoster associated encephalitis in dialysis patients.

Two patients on dialysis because of chronic renal failure who developed herpes zoster associated encephalitis are reported. Both developed overt encephalopathy despite treatment with oral acyclovir for the preceding herpes zoster eruption. The encephalopathy responded rapidly to intravenous acyclovir.

T-prolymphocytic leukemia with circulating carrot-like cells.

A case of T-prolymphocytic leukemia leading to rapid demise of a 67-year-old man is reported. He presented with multiple skin lesions and splenomegaly. A unique feature was that a proportion of circulating leukemic cells assumed bizarre shapes, resembling carrots. The leukemic cells expressed the T-cell markers T11, T8 and Dako-T2, and the natural killer cell markers NKH1, Leu7 and Leu 11b.