Long-term follow up of 69 patients treated for optic pathway tumours before the chemotherapy era

AIM: To analyse the long-term results of conservative management with radiotherapy in patients with optic pathway tumours. DESIGN: All 69 patients were symptomatic at diagnosis and most neoplasms involved the optic chiasm and hypothalamus. RESULTS: At 10 years, overall survival and progression free survival were 83% and 65.5%, respectively. After radiotherapy, vision improved in 18 patients and remained stable in 29 other patients. Cerebrovascular complications occurred in nine of 53 patients treated with radiotherapy after a median interval of two and a half years. These complications were five times more frequent in patients with neurofibromatosis type 1 (NF1). Severe intellectual disabilities were present in 18 children, most of whom underwent irradiation at a very young age (median age, 4 years). IMPLICATIONS: Radiotherapy is a valuable treatment in terms of tumour response, visual outcome, and progression free survival. However, in young children and in patients with NF1, major sequelae are encountered and new treatment strategies should be proposed for these patients.     

Long term follow up of patients with early onset anorexia nervosa.

A long term follow up (mean 7.2 years) of 30 children with anorexia nervosa (mean age at onset 11.7 years) was carried out. The outcome was good in only 18 (60%), 10 children remaining moderately to severely impaired and two died. Poor prognostic factors included early age at onset (less than 11 years), depression during the illness, disturbed family life and one parent families, and those in which one or both parents had been married before.     

Long term follow up after inhalation of foreign bodies.

The long term results of treatment of inhalation of foreign bodies in a district children''s hospital and in a tertiary referral centre were reviewed by clinical assessment, chest radiography, and standard four view 81mKr ventilation/99mTc macroaggregated albumin perfusion imaging (V/Q lung scan). The overall incidence in the population served by the district hospital was roughly one in 14,000/year. Of the 12 children reviewed there, three had abnormal chest radiographs and four had abnormal V/Q scans as a result of inhalation of the foreign bodies. Of 21 children treated and reviewed at the referral centre, eight had abnormal chest radiographs, and 14 had abnormal V/Q lung scans. Three factors were assessed for prognostic importance: site of impaction, initial radiographic appearance, and time before removal. A child who had inhaled a foreign body into the left lung and who had collapse/consolidation on the initial chest radiograph was at greatest risk of long term complications. These children merit close follow up.     

Long term follow up of topical mustine treatment for cutaneous langerhans cell histiocytosis

BACKGROUND AND OBJECTIVES—Skin lesions in Langerhans cell histiocytosis (LCH) are often painful and difficult to treat. Topical application of nitrogen mustard (0.02% mechlorethamine hydrochloride, mustine), an alkylating cytostatic agent, has been shown to be effective. There is, however, concern about potentially harmful long term side effects.
STUDY DESIGN—In a retrospective study 20 children with LCH (average extent of initial skin involvement: 16.4% body surface) were followed up for an average of 8.3 years after completion of topical mustine therapy. They had received a total of 34 courses (mean duration 14.2 weeks) of topical mustine. Disease status on follow up was assessed according to the Histiocyte Society classification.
RESULTS—After mustine was introduced, 16 patients were able to discontinue systemic steroids and/or chemotherapy. Topical mustine was well tolerated in 18 patients, but two developed irritant dermatitis. On follow up, the disease was inactive in 10 patients. Among the children with active disease, six had mild skin disease and four had progressive disease, two of them with skin lesions unresponsive to mustine treatment. Scars confined to areas of formerly active skin disease were found in six patients. There was no evidence of premalignant or malignant skin disease in the treated areas.
CONCLUSION—Topical mustine is an effective and safe treatment for skin disease in most children with LCH. Residual scarring was probably a result of the disease itself rather than to mustine. Although no evidence of skin cancer was found in this study, continued long term follow up is advisable.

     

Long term follow up of newborns tested with the auditory response cradle.

The auditory response cradle is being used in a mass hearing screening project. Babies are assessed in the first week after birth by the fully automatic, microprocessor controlled cradle. The test, lasting from two to 10 minutes, compares physiological auditory responses to natural behaviour measured in control trials. More than 5000 babies have been tested and full follow up information at the age of 7 to 9 months is available from over two thirds of these. Less detailed information is available for 71% and 64% of those babies who have been followed up at 18 months and three years of age respectively. A total of 439 of 5553 neonates tested failed the first screening test. Eighty eight (1 X 6%) failed a second screening test while still in the maternity unit but 61 of these were subsequently shown to be normal, giving a false positive rate of 1 X 1%. The babies who failed the screening tests included 9 with sensorineural hearing loss, three with secretory otitis media, and three with abnormal auditory brain stem response tests. One child who passed the initial screening tests was found to have a moderately severe hearing loss at the age of 18 months.     

Long term follow up of topical mustine treatment for cutaneous langerhans cell histiocytosis.

BACKGROUND AND OBJECTIVES: Skin lesions in Langerhans cell histiocytosis (LCH) are often painful and difficult to treat. Topical application of nitrogen mustard (0.02% mechlorethamine hydrochloride, mustine), an alkylating cytostatic agent, has been shown to be effective. There is, however, concern about potentially harmful long term side effects. STUDY DESIGN: In a retrospective study 20 children with LCH (average extent of initial skin involvement: 16.4% body surface) were followed up for an average of 8.3 years after completion of topical mustine therapy. They had received a total of 34 courses (mean duration 14.2 weeks) of topical mustine. Disease status on follow up was assessed according to the Histiocyte Society classification. RESULTS: After mustine was introduced, 16 patients were able to discontinue systemic steroids and/or chemotherapy. Topical mustine was well tolerated in 18 patients, but two developed irritant dermatitis. On follow up, the disease was inactive in 10 patients. Among the children with active disease, six had mild skin disease and four had progressive disease, two of them with skin lesions unresponsive to mustine treatment. Scars confined to areas of formerly active skin disease were found in six patients. There was no evidence of premalignant or malignant skin disease in the treated areas. CONCLUSION: Topical mustine is an effective and safe treatment for skin disease in most children with LCH. Residual scarring was probably a result of the disease itself rather than to mustine. Although no evidence of skin cancer was found in this study, continued long term follow up is advisable.     

Long term follow up after meningitis in infancy: behaviour of teenagers

Aims: To determine the effects of meningitis in infancy on subsequent teenage behaviour. Methods: A national postal survey of parents and teachers using an established standard behavioural questionnaire. Subjects were 739 of the surviving children from the national incidence study of infantile meningitis in England and Wales carried out between 1985 and 1987, together with a group of 606 matched controls that had been recruited when the index cases were 5 years old. Results: 46% of parents of children who had had meningitis with complications in infancy, compared with 21% of parents of control children rated their children as having behavioural problems. When the children were rated by their teachers, 37% and 23% respectively, were scored as having behavioural problems. There was no significant difference in behaviour between the 103 children who had had meningitis during the first month of life and the 634 who had had postneonatal meningitis. Eight of the index children had been excluded from school compared to none from the control group. Conclusions: The behaviour of teenage children who had meningitis in infancy is worse than that of control children who did not have infantile meningitis when rated by parents and teachers.     

Long term follow up of serostatus after maternofetal parvovirus B19 infection

Background: Maternofetal parvovirus B19 infection may result in fetal hydrops or abortion. Chronic infection has been associated with long term complications (polyarthritis, persistent aplastic anaemia, hepatitis). In pregnancy maternal immunosuppression caused by a TH2 dominant response to viral antigens has been observed. There is little information on long term reactivity to intrauterine infection. Aims: To assess the serological status in children and their mothers after maternofetal parvovirus B19 infection and development of fetal hydrops. Methods: A total of 18 children and their mothers, and 54 age matched control infants were studied. Main outcome measures were parvovirus B19 DNA, specific IgM and IgG against the virus proteins VP1/VP2, and NS-1 in venous blood. Results: Parvovirus B19 DNA and antiparvovirus B19 (IgM) were undetectable in all sera. A significant larger proportion of maternal sera compared to study children''s sera contained IgG against the non-structural protein NS-1. Mean levels of VP1/VP2 IgG antibodies were significantly lower in the children than in their mothers (48 (36) v 197 (95) IU/ml). There was no history of chronic arthritis in mothers and children. Five women had subsequent acute but transient arthritis postpartum, which was not correlated with antibodies against NS-1. Conclusions: Serological evidence of persistent infection after maternofetal parvovirus B19 disease could not be detected. Increased maternal prevalence of anti NS-1 (IgG) and increased levels of antiparvovirus B19 (IgG) may reflect prolonged viraemia compared to fetal disease.     

Long term follow up after meningitis in infancy: behaviour of teenagers.

AIMS: To determine the effects of meningitis in infancy on subsequent teenage behaviour. METHODS: A national postal survey of parents and teachers using an established standard behavioural questionnaire. Subjects were 739 of the surviving children from the national incidence study of infantile meningitis in England and Wales carried out between 1985 and 1987, together with a group of 606 matched controls that had been recruited when the index cases were 5 years old. RESULTS: 46% of parents of children who had had meningitis with complications in infancy, compared with 21% of parents of control children rated their children as having behavioural problems. When the children were rated by their teachers, 37% and 23% respectively, were scored as having behavioural problems. There was no significant difference in behaviour between the 103 children who had had meningitis during the first month of life and the 634 who had had postneonatal meningitis. Eight of the index children had been excluded from school compared to none from the control group. CONCLUSIONS: The behaviour of teenage children who had meningitis in infancy is worse than that of control children who did not have infantile meningitis when rated by parents and teachers.     

Long term follow up of serostatus after maternofetal parvovirus B19 infection.

BACKGROUND: Maternofetal parvovirus B19 infection may result in fetal hydrops or abortion. Chronic infection has been associated with long term complications (polyarthritis, persistent aplastic anaemia, hepatitis). In pregnancy maternal immunosuppression caused by a TH2 dominant response to viral antigens has been observed. There is little information on long term reactivity to intrauterine infection. AIMS: To assess the serological status in children and their mothers after maternofetal parvovirus B19 infection and development of fetal hydrops. METHODS: A total of 18 children and their mothers, and 54 age matched control infants were studied. Main outcome measures were parvovirus B19 DNA, specific IgM and IgG against the virus proteins VP1/VP2, and NS-1 in venous blood. RESULTS: Parvovirus B19 DNA and antiparvovirus B19 (IgM) were undetectable in all sera. A significant larger proportion of maternal sera compared to study children's sera contained IgG against the non-structural protein NS-1. Mean levels of VP1/VP2 IgG antibodies were significantly lower in the children than in their mothers (48 (36) v 197 (95) IU/ml). There was no history of chronic arthritis in mothers and children. Five women had subsequent acute but transient arthritis postpartum, which was not correlated with antibodies against NS-1. CONCLUSIONS: Serological evidence of persistent infection after maternofetal parvovirus B19 disease could not be detected. Increased maternal prevalence of anti NS-1 (IgG) and increased levels of antiparvovirus B19 (IgG) may reflect prolonged viraemia compared to fetal disease.     

Three-year follow up of term and near-term infants treated with inhaled nitric oxide

BACKGROUND: The present study describes the outcome at 3 years in term and near-term infants treated with inhaled nitric oxide (iNO) for persistent pulmonary hypertension of the newborn (PPHN). METHODS: The study population consisted of 18 infants delivered at 34 weeks by best obstetric estimate who were admitted to the neonatal intensive care units with a diagnosis of PPHN. RESULTS: Eighteen infants (mean gestational age 38.5 +/- 2.6 weeks, mean birthweight 3015 +/- 587 g) were treated with iNO. The mean oxygenation index before iNO was 27.2 +/- 15.2. Responses to iNO were classified into three groups: (i) early response in eight infants; (ii) late response in two; and (iii) poor response in eight infants. Three infants died within seven postnatal days. Fifteen surviving infants were followed up to 3 years. The mean developmental scale was 98.4 +/- 9.0. One infant was diagnosed with severe neurodevelopmental disability due to cerebral palsy. Another infant was diagnosed with mild neurodevelopmental disability because of a low developmental scale. No infant showed significant hearing loss. Five infants had reactive airway disease (RAD) at 18 months, these infants required a significantly longer duration of mechanical ventilation in their neonatal period than non-RAD infants (P = 0.02). The frequency of survival with normal neurodevelopmental outcome was significantly higher in the early response group than the late or poor response groups (P = 0.03). CONCLUSION: In iNO-treated PPHN, mortality and neurodevelopmental outcome were associated with response to iNO, and pulmonary outcome was associated with duration of mechanical ventilation.     

Long term follow up after parenteral specific hyposensitization in children (author's transl)]

The long term follow up of 55 children with allergy treated by specific hyposensitization therapy is reported. Hyposensitization therapy had been terminated from 2--7 years previously. The success of treatment was subjectively judged to be satisfactory of good in 62% of the cases. In the group of asthmatic children, asthma attacks had decreased or ceased in 74% of the cases: in children with hay fever only 24% continued to have as many or more symptoms as before treatment. In children with asthma and pollen allergy, the success rate was slightly higher with aqueous extracts (68%) than with pyridine-extracted and alum-precipitated extract administration (43%). Also in children with hay fever symptoms alone the use of aqueous extracts seemed to bring somewhat better results (57% with good or satisfactory success) in comparison with pyridine-extracted and alum-precipitated extracts (45%). Side effects of hyposensitization therapy occured in 29 children (53%).     

Negative results of antiendomysial antibodies: long term follow up

Fifteen patients with intestinal villous atrophy, but simultaneously negative results of antiendomysial antibodies (EMA) were studied. Two patients were finally diagnosed as having coeliac disease. The predictive value of negative results of EMA assessment in children suspected of coeliac disease is high, approaching 86.7%.     

Long-term follow up of surgically treated primary vesicorenal reflux

It is well known that the most serious complication of primary vesicoureteric reflux is chronic atrophic pyelonephritis or reflux nephropathy. The main goal in the management of vesicorenal reflux (VRR) is the prevention of progressive renal damage.The elimination of reflux and the eradication of infection are fundamental in order to achieve this goal. The authors performed a retrospective study in 146 pediatric patients operated upon by ureteral reimplantation for primary VRR; 78 operations were unilateral and 68 bilateral, for a total of 214 reimplanted ureters. The mean age at the time of operation was 4.8 years; the mean age at the time of the study was 15.8 years, and the average postoperative observation period was 13.5 years.The study included pre- and postoperative urine analysis and determinations of blood pressure, renal scarring and parenchymal growth, and evolution of renal function.The study showed that: (1) urinary concentrating ability in bilateral high-degree VRR (64 patients) remained reduced in a relatively high percentage of patients; (2) there was significant improvement (from 40.7% to 17.9%) in proteinuria that was related to favorable evolution of renal function (z = 4.152; P <0.01); (3) the frequency of acute pyelonephritis decreased from 41.4 to 3.4%, and only 20% of the patients had episodes of afebrile pathologic bacteriuria in the postoperative period (z = 7.647; P <0.01); (4) more than 15% of the patients were hypertensive (10.3%) or at risk for hypertension, having a high peripheral plasma renin activity (5.2%); (5) some renal scarring developed despite successful antireflux surgery, and usually parenchymal growth resumed; in about 6% of the renal units a further decrease in renal size occurred; and (6) renal function generally improved after successful surgery (z = 4.115; P <0.01). This favorable evolution was much more evident in patients operated upon in the first 2–3 years of life (z = 3.885; P <0.01). However, when the renal function was already severely compromised, an antireflux operation had little or no effect on the inexorable decline in renal function. Correspondence to: G. Belloli     

Long term effects of periconceptional multivitamin supplements for prevention of neural tube defects: a seven to 10 year follow up.

Periconceptional supplementation with Pregnavite Forte F was offered to women who presented consecutively to the Oxford genetic counselling service in the early 1980s who had previously had one or more pregnancies complicated by a neural tube defect. The first 100 children born alive to these women are the subject of this study. Birth weight, gestation, and congenital abnormalities were recorded. At age 2-5 years all 96 children remaining in the United Kingdom were assessed clinically and developmentally and behavioural information was obtained by questionnaire. At age 7-10 years, follow up of 91 children by telephone and postal questionnaire yielded further information about growth, general health, vision, hearing, and educational and behavioural status. Entry criteria excluded single mothers but the social class distribution of the sample was otherwise representative of the Oxfordshire population. There were no recurrences of neural tube defects. One child had radiological evidence of spina bifida occulta affecting only the fifth lumbar vertebra. One had an autosomal recessive disorder. Eight had random minor congenital anomalies. Birth weight for gestational age was significantly greater than for the local population and at age 7-10 years the girls were considerably taller than expected. Health, auditory, visual, and developmental status were no different from the general population. None of the children had special educational needs. None showed a major behaviour disorder but worries, fussiness, and fearfulness were highly significantly over represented.