Alterations in cord blood leukocyte subsets of patients with severe hemolytic disease after intrauterine transfusion therapy

OBJECTIVES: The aim of this study was to compare, at delivery, the cord blood mononuclear cells of infants with severe hemolytic disease who received intrauterine transfusion (IUT) therapy with the cord blood mononuclear cells of healthy nonimmunized control neonates. STUDY DESIGN: The expression of leukocyte markers on CBMNC of 14 IUT-treated and 18 control neonates was analyzed by means of a panel of well-defined monoclonal antibodies and flow cytometry. RESULTS: Patients with severe hemolytic disease requiring IUT treatment displayed significant altered expression of some leukocyte markers when compared with control subjects. The circulating CD34+ progenitor cells were significantly increased in comparison with cord blood of nonimmunized neonates. IUT-treated patients also showed a statistically significant decrease in natural killer (NK) cell associated markers (CD16, CD57, and CD69), which correlated with a lower expression of CD56. In these patients an increased expression of CD3/CD45RO and CD3/CD5 was also noted. Although these latter alterations were statistically significant in a single-parameter analysis, the significance disappeared after multi-parameter analysis because of a loss of statistical power. CONCLUSIONS: Compared with nonimmunized healthy newborn infants, patients who underwent IUT also exhibited a down-regulation of NK cells and NK cell associated markers, as well as increased numbers of CD34+ progenitor cells.     

A critical review of intrauterine fetal transfusion

A critical review of 142 intrauterine fetal transfusions performed in 99 patients during 107 pregnancies raised doubts about the absolute benefit of the procedure. Two main problems emerged: the diagnostic criteria upon which decisions were based were inadequate and the fetal mortality associated with transfusion was high, especially when this was performed before 28 weeks. We measured amniotic fluid bilirubin concentration by a biochemical method but the results following intrauterine fetal transfusion were similar to those observed by others using spectrophotometric examination of amniotic fluid.     

Long-term outcome after sagittal synostosis operations

PURPOSE: Optimization of image quality by use of digital luminescence radiography (DLR) in voiding cystoureterography (VCU) instead of a conventional screen film system. PATIENTS AND METHODS: 53 patients with a suspected vesicouretral reflux were examined with a VCU in analogous and digital technique. The X-rays were taken in a sitting position while simultaneously measuring the bladder pressure. An intraindividual comparison was omitted for X-ray saving reasons. In the follow-up 3 patients were examined with the other system. Two radiologists and two urologists compared 60 VCU of 53 patients--30 VCU in conventional screen film and 30 in DLR technique presented in standard and edge-enhanced images--with regard to image quality according to 3-step score (A--good image quality, B--sufficient image quality, C--insufficient image quality). RESULTS: The 59 conventional films were scored 72 times with an A, 76 times with a B, and 72 times with a C, whereas the DLR films were scored 116 times with an A, 72 times with a B, and only 8 times with a C. Hereby the edge-enhanced images were the most useful for distinguishing the bladder shadow from the soft tissue of the thigh and for detecting reflux. Furthermore, the exposure dose when applying DLR could be decreased to 75% in grown-ups and to 57% in children without any significant loss of information. CONCLUSION: The DLR is highly superior to the conventional film screen system because of its relatively high tolerance towards wrong exposures, especially with respect of the sitting position during MCU. With the DLR we obtained 96% and with the conventional system only 63% films usable for diagnosis.     

Neurodevelopmental outcome after open fetal surgery

PURPOSE: This study examined the neurological and age-appropriate developmental outcomes of 11 children who underwent open fetal surgery. RESULTS: Nine children have normal neurological outcomes, and nine have normal development. CONCLUSION: Intensive postnatal care, intracranial hemorrhage, and requirement for prolonged respiratory support were associated with a worse neurological and developmental prognosis.     

Long-term outcome after marrow transplantation for severe aplastic anemia

We reviewed the records and reevaluated 212 patients with aplastic anemia transplanted at the Fred Hutchinson Cancer Research Center (FHCRC) between 1970 and 1993 who survived >/=2 years and who have been followed for up to 26 years. Parameters analyzed included hematopoietic function, chronic graft-versus-host disease (GVHD), skin disease, cataracts, lung disease, skeletal problems, posttransplant malignancy, depression, pregnancy/fatherhood, and the return to work or school, as well as patient self-assessment of physical and psychosocial health, social interactions, memory and concentration, and overall severity of symptoms. Survival probabilities at 20 years were 89% for patients without (n = 125) and 69% for patients with chronic GVHD (n = 86) (the status was uncertain in 1 surviving patient). All patients had normal hematopoietic parameters. Skin problems occurred in 14%, cataracts in 12%, lung disease in 24%, and bone and joint problems in 18% of patients. Eleven patients (12%) developed a solid tumor malignancy and 19% of patients experienced depression. Chronic GVHD was the dominant risk factor for late complications. Seventeen patients died at 2.5 to 20.4 years posttransplant; 13 of these had chronic GVHD and related complications. At 2 years, 83% of patients had returned to school or work; the proportion increased to 90% by 20 years. At least half of the patients preserved or regained the ability to become pregnant or father children. Patients rated their quality of life as excellent and symptoms as minimal or mild. In conclusion, marrow transplantation in patients with aplastic anemia established long-term normal hematopoiesis. No new hematologic disorders occurred. The major cause of morbidity and mortality was chronic GVHD. However, the majority of patients who survived beyond 2 years returned to a fully functional life.     

Long-term follow-up of immunosuppressive treatment for obstructive airways disease after allogeneic bone marrow transplantation

BACKGROUND: Locally advanced thyroid cancer invading the tracheal cartilage represents a difficult treatment dilemma during thyroidectomy. METHODS: A retrospective chart review was performed to determine the results of laryngotracheal resection or tracheal cartilage shave with adjuvant radiotherapy in patients with locally advanced thyroid cancer invading the upper airway. RESULTS: Of 597 patients undergoing thyroidectomy for thyroid cancer, 40 were found to have laryngotracheal invasion. Thirty-five patients with superficial invasion underwent cartilage shave procedures with adjuvant radiotherapy; five with full-thickness invasion underwent radical resection, including tracheal sleeve resection (n = 3) or total laryngectomy (n = 2). Histologic subtypes included papillary (n = 32), follicular (n = 2), Hurthle cell (n = 1), medullary (n = 3), and anaplastic (n = 2). Of the cartilage shave group, 25 are currently alive with no evidence of disease at a mean follow-up of 81 months (range 1-290). Six developed isolated local/regional recurrence and were managed with total laryngectomy (n = 1), tracheal resection (n = 1), cervical lymphadenectomy (n = 1), or repeat radiotherapy (n = 3). All six patients remain free of disease at a mean follow-up of 5 years. Of those who underwent initial laryngotracheal resection, four remain free of disease at a mean follow-up of 5 years. The rates of 10-year disease-free survival and overall survival for all patients were 47.9% (95% confidence interval [CI] 24.8, 71.0) and 83.9% (95% CI 70.3, 97.5), respectively. CONCLUSIONS: These data suggest that adequate management of thyroid cancer with laryngotracheal invasion can be achieved with a more conservative surgical approach and adjuvant radiotherapy, reserving more radical resections for extensive primary lesions or locally recurrent disease.     

Long-term detection of microchimaerism in peripheral blood after pretransplantation blood transfusion

Renal allograft survival is prolonged after pretransplantation blood transfusion. The aim of this study was to test retrospectively the development and persistence of microchimaerism after pretransplantation blood transfusion and to assess whether the type of blood transfusion (partially matched [= sharing of at least one HLA-B and one HLA-DR antigen between blood donor and recipient] versus mismatched) influences the (continued) presence of donor-type cells. A sensitive nested PCR technique based on HLA-DRB1 allele-specific amplification using sequence-specific primers (detection level: one donor cell among 10(5) recipient cells) for detection of donor cells was implemented in our laboratory. We studied 21 patients for microchimaerism in the peripheral blood compartment, following blood transfusion. Our preliminary data show that microchimaerism was detectable up to 8 weeks after blood transfusion. In all patients receiving a partially matched blood transfusion, donor-type cells were detected in the first week after transfusion, in 7/8 patients 2-4 weeks after transfusion, and in some patients up to 8 weeks after transfusion. After mismatched transfusion a tendency to shorter duration of microchimaerism was observed.     

Long-term neuro-endocrine sequelae after treatment for childhood medulloblastoma

The occurrence of neuro-endocrine deficiencies following craniospinal irradiation for medulloblastoma is well known, but data concerning the spectrum and prevalence of endocrine abnormalities in adulthood are scarce. We studied endocrine function in 20 (median age 25 years) adult subjects, 8-25 years (median 16 years) after therapy. The radiation dose to the whole cranium and spinal axis was 35 +/- 2.6 Gray (mean +/- standard deviation) with a boost to the posterior fossa of 18 +/- 3.7 Gray. 13 subjects had received additional chemotherapy. In 15 of 20 (75%) subjects, endocrine abnormalities were observed. In 14 (70%), growth hormone (GH) secretion was impaired; 7 (35%) subjects had an absolute GH deficiency, while 7 (35%) showed subnormal responses to insulin-induced hypoglycaemia. In contrast, only 20% (4) of these subjects showed impairment of the hypothalamus-pituitary-thyroid (HPT) axis, while 15% (3) showed central impairment of hypothalamus-pituitary-gonadal (HPG) function. Central impairment of the HPG axis was associated with impaired GH secretion in all cases. Central adrenal insufficiency was not observed. Basal levels of prolactin were normal in all subjects. Young age at treatment was a determinant of GH deficiency in adulthood (P = 0.014). Neither post-treatment interval, nor the use of chemotherapy were determinants of central endocrine impairment in adulthood. In long-term survivors of medulloblastoma, GH deficiency has a high prevalence. In contrast, impairment of the HPG and HPT axis is less common, while central adrenal insufficiency was not observed.     

Follow-up of children after fetal treatment for obstructive uropathy

We report a 6-year-old girl with Japanese B encephalitis. The initial symptoms were high fever, headache and vomiting. On the second day of illness, she developed hemiconvulsion and was admitted to our hospital. Physical examination demonstrated a stiff neck. C-reactive protein elevated to 22.7 mg/dl. CSF examination showed a marked increase in the cell count (10,896/3 mm3). During the course of the treatment, she showed transient hemiparesis and dysphagia, followed by akinetic mutism lasting for about a month. The patient was left with severe cognitive and memory impairment and complex partial seizures but no motor dysfunction. Japanese B encephalitis was diagnosed by means of serological examination. Magnetic resonance imaging revealed cystic lesions in the medial and posterior thalamus and substantia nigra and severe atrophy of the hippocampus. Despite the involvement of substantia nigra, the patient had no parkinsonism. The cognitive impairment may in part be explained by the lesions in the medical and posterior thalamus.     

Long-term follow-up after bowel resection for necrotizing enterocolitis: factors affecting outcome

BACKGROUND: Necrotizing enterocolitis (NEC) is the most common surgical emergency among newborns and is associated with a high morbidity and mortality. This study evaluates the long-term survival of infants requiring surgical intervention for NEC and factors affecting outcome. METHODS: A retrospective review of infants requiring surgery for complications of NEC at a tertiary care, pediatric hospital over a 16-year period was performed. Patients were evaluated for early and late morbidity and mortality, length of intestinal resection, presence of the ileocecal valve (ICV), days of parenteral nutrition (PN), and growth. RESULTS: Two hundred forty-nine patients were included, with an average gestational age of 30 +/- 5 (+/- SD) weeks and birth weight of 1.50 +/- 0.89 kg. The surgical mortality rate was 45%, with survivors (137) being larger (P < .001) and older (P < .001) at time of birth than nonsurvivors. Mortality rates varied inversely with gestational age and birth weight. Surgical survivors had an average of 21 +/- 26 cm of intestinal length resected. The ileocecal valve was preserved in 45% of infants. Growth was similar between infants with or without an ICV. Stratification of length of intestine resected showed that infants with larger resections had greater requirements for parenteral nutrition, but this had no influence on long-term growth at follow-up. CONCLUSIONS: Survivors of NEC are characterized by greater gestational age, greater birth weight, and older postgestational age at surgery. Infants who underwent greater intestinal resections required longer periods of PN. The length of intestine resected or presence of the ileocecal valve had no overall bearing on long-term outcome.     

Long-term outcome after discharge from a stroke rehabilitation unit

The evidence is compelling that stroke units are effective when compared to management of patients on general medical wards. However, the evidence remains equivocal that better outcome is sustained in the longer term. This paper reports an investigation of cognitive and emotional outcome, environmental consequences, social activities, and physical outcome in 57 consecutive one-year survivors of a stroke discharged from a stroke and neurological rehabilitation unit. Satisfaction with inpatient and outpatient services was also investigated. Results were compared with previously reported studies of long-term outcome after stroke. Mean Barthel activities of daily living score at one year or more post-stroke was 16.8. Arm function was impaired in 43% of the participants in the study. Nearly half had cognitive and emotional problems, 19% communication problems, and 25% problems with access both inside and outside their house. All but four of the one-year survivors were less active after their stroke than before. More than 40% were dissatisfied with at least one aspect of inpatient and/or outpatient services. The long-term consequences of stroke in all areas investigated were considerable and in line with previous reports. Some suggestions for reducing these effects are made, including better information for patients about stroke and rehabilitation, improved access to psychology services, detailed assessment prior to hospital discharge of the patient's living environment and effective coordination with social services to improve access to their living environment.     

Diagnoses commonly missed in childhood. Long-term outcome and implications for treatment

Psychiatric and developmental disorders with onset in early childhood are often missed and commonly overlooked by adult psychiatrists. These disorders have important continuities into adulthood and are powerful predictors of chronicity, comorbidity, and severity. It is essential that they are recognized and taken into account in the assessment and treatment of the adult patient.     

Iatrogenic secondary infertility caused by residual intrauterine fetal bone after midtrimester abortion

Eleven women with secondary infertility had ultrasonographic findings of intrauterine calcification. Infertility developed in all women after operative termination of midtrimester pregnancy. Dilation and curettage or hysteroscopic removal confirmed residual fetal bony fragments. The removal of these bony fragments was associated with therapeutic success for infertility.     

Early predictors of neurodevelopmental outcome in high risk infants

The purpose of this study was to investigate mortality and morbidity rates and long-term outcome of patients who underwent emergency treatment of abdominal aortic prosthetic graft infection. Between January 1984 and December 1993, 18 men aged fifty-nine +/- sixteen years were operated on as an emergency for an acute life-threatening complication of aortic prosthetic graft infection. The grafts had been implanted for abdominal aortic aneurysm in 9 patients and aortoiliac occlusive disease in 9, from one to one hundred seventy months previously. Five (28%) patients presented with a hemorrhagic shock due to a fistula between the vascular reconstruction and the small bowel (4 patients) or the right ureter (1 patient) and 13 (72%) had generalized sepsis. The grafts were always radically explanted. Extraanatomic revascularization procedures included 6 axillopopliteal and 12 axillofemoral bypass grafts. Operative mortality was 39% (7 patients), and 3 (9%) limbs were amputated within thirty days. Two (11%) patients died after seven and twelve months, respectively, of septic complications, and 1 (5%) patient died after six months from an unrelated cause. Eight (73%) patients are still alive at a mean follow-up of fifty +/- thirty-four months, but in 3 the extraanatomic bypass was removed for infection and 5 major amputations were performed. Two-year survival and limb salvage rates were 44% and 50%, respectively. Aortic prosthetic graft infections that require emergent treatment continue to demonstrate high early and late mortality and limb loss rates despite aggressive intervention and limb salvage procedures. Newer methods of managing these complications should continue to be investigated.     

Toxic and hemolytic properties of serum in the massive transfusion syndrome

Toxic and hemolytic properties of the sera of dogs which received massive homologus blood transfusions were studied in experiments on 20 dogs and 432 mice. The toxicity of the recipient's sera was evaluated in vivo on mice with blocked reticulo-endothelial system and by the method of hemocultures. Red cells labeled with radioactive chromium were used to study the hemolytic activity. Investigations carried out demonstrated the acute blood loss wilth the subsequent massive transfusion of the homologous blood to result in the production in the recipient's serum of toxigenic products. These toxigenic properties were determined by two biotesting methods. The maximal toxigenic action was noted in 2 hours and in the course of the 1st day after the substitution. No toxic effect of the sera was found on the 17th day. A parallel study of hemolytic properties of the sera revealed a marked increase of its activity; it was the greatest during the first 24 hours. In three days the hemolytic properties of the serum were no different from the initial one.     

Long-term outcome after the mustard repair for simple transposition of the great arteries. 28-year follow-up

The effects of feeding different amounts of colostrum or only milk replacer and the effects of Long-R3-IGF-I (administered s.c. or orally; 50 microg/[kg BW x d] for 7 d), and of s.c. injected recombinant bovine GH (rbGH; 1 mg/[kg BW x d] for 7 d) on small intestinal mucosal morphology in newborn calves were studied by histomorphometry. Neonatal calves fed colostrum six times exhibited greater (P < .01) villus circumferences, areas, and heights in total small intestine and especially in the duodenum than calves fed only milk replacer. Furthermore, villus circumferences and areas in total small intestine were greater (P < .05) in calves fed colostrum once than in calves fed no colostrum. Villus size in total small intestine was smaller (P < .05) in rbGH-treated than in control calves; jejunum villus circumferences and heights were especially reduced (P < .05). Crypt depths in ileum were greater (P < .05) in rbGH-treated calves. In conclusion, prolonged colostrum supply significantly enhanced small intestinal villus size in neonatal calves. In contrast, Long-R3-IGF-I had no significant influence on small intestinal morphology, and rbGH in supraphysiological amounts even reduced small intestinal mucosal variables after 1 wk of treatment. The study demonstrated enhanced postnatal development of the gastrointestinal tract by prolonged colostrum feeding, but not by Long-R3-IGF-I or GH.     

Morphological change of lipids in the vaginal epithelium after intrauterine fetal death (lipid test)

The authors studied the lipid content of the vaginal epithelium of gravida in whom intrauterine fetal death had occurred. Fifteen gravida in the 6th to 10th month of gestation were examined and an unusual, inconsistent with the respective gestation term increase in the intra- and extra-cellular lipid content of the vaginal epithelium demonstrated. This is related to a decrease in the amount of estrogen hormones secreted by the placenta. A cytochemical 'lipid test' based on the above findings has been developed by the authors for the diagnosis of intrauterine fetal death during the second half of pregnancy.