Transplacental carcinogenesis (adrenocortical carcinoma) associated with hydroxyprogesterone hexanoate.
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17 alpha-Hydroxyprogesterone Caproate↗
Biomedical subjects
K J Shah
Publications and source records attributed to K J Shah.
Beckwith-Wiedemann syndrome: role of ultrasound in its management.
There is a well-recognised association between Beckwith-Wiedemann syndrome (BWS), hemi-hypertrophy of a limb and childhood malignancies. Ten children with BWS were seen over a period of 14 years. Their ages ranged from newborn to 14 years and the maximum follow-up extended to 14 years. Three children also had hemi-hypertrophy of the lower limb. In one child Wilms' tumour was diagnosed at the age of 4 years; she was successfully treated with surgery and chemotherapy and has been disease-free for the past 7 years. One patient died in infancy, while the remaining nine children were regularly followed up, including 3-monthly ultrasound scans of the kidneys and upper abdomen. Ultrasonic features of nephromegaly, lobulated margin, corticomedullary cysts and nodules of mixed echogeneity and pelvi-calyceal distension are illustrated. Ultrasound is a non-invasive, non-ionising and safe modality with very little discomfort to the patient and is ideal for regular and frequent follow-up of infants and children with conditions predisposed to malignancy.
Abdominal Neoplasms↗
Unusual sites of acute osteomyelitis in childhood.
In children osteomyelitis is common in the long hours of femur, tibia and humerus. This study reports 16 children, aged 2-13 years, with osteomyelitis at unusual sites: in the bones of the thoracic cage including three involving the clavicle; in the spine, foot and elsewhere. In one case, multifocal involvement of the vertebral body and the knee occurred. In two large series reported previously, the incidence of osteomyelitis was 1-3% in the clavicle, 3-8% in the calcaneus and less than 1% in the ribs. Four out of 16 cases (two involving clavicles, one rib and one with multifocal sites of the lesion) required open biopsies and histological examination to achieve the final diagnosis of osteomyelitis; in three of these patients the causative agent was not identified on culture. Staphylococcus aureus was the infective organism in 50% of cases where cultures were obtained. In five cases there was no growth on culture and specific search for less common organisms, including mycobacteria tuberculosis (AAFB), proved negative. It is suggested that in such situations diagnostic problems may present as the clinical and radiological findings may not be specific or conclusive. In such cases early biopsy is mandatory.
Acute Disease↗
4-hydroxyquinoline-3-carboxylic acids as inhibitors of cell respiration. 2. Quantitative structure-activity relationship of dehydrogenase enzyme and Ehrlich ascites tumor cell inhibitions.
Studies on dehydrogenase enzyme inhibition have been extended with the design, synthesis, and correlation analysis of 7-[(substituted-benzyl)oxy]-, 7-[(substituted-phenethyl)oxy]-, and 7([substituted-phenoxy)ethoxy]-4-hydroxyquinoline-3-carboxylic acids. Sixteen new congeners and the fifteen molecules previously synthesized have been tested against cytoplasmic malate dehydrogenase and lactate dehydrogenase, as well as against mitochondrial malate dehydrogenase. The lipophilic congeners show a clear specificity for inhibition of the mitochondrial enzyme. Correlation analysis of the data on the three enzymes allows a comparison of the binding sites in quantitative terms, while examination of the data on inhibition of ascites tumor cell respiration affords an indication of membrane transport. A newly developed high-pressure liquid chromatography based retention index is compared to the octanol-water pi constant as a model for hydrophobic interactions.
Animals↗
Orbital tuberculosis in childhood.
An 11-year-old Indian girl living in England developed proptosis due to tuberculosis of the orbit. The proptosis regressed and she recovered fully after chemotherapy. While malignancy, developmental anomalies, and nontuberculous infections are commoner causes, a tuberculin test should be included among the investigations of children with proptosis.
Child↗
Busulphan lung in childhood.
A child receiving busulphan treatment for adult-type chronic myeloid leukaemia responded well for four years before the onset of complications leading to "busulphan lung'. Pulmonary function tests (PFT) were useful in monitoring the development of this condition and we recommended regular PFT for patient receiving busulphan treatment.
Busulfan↗
Clinical, haematological, and radiological features of children presenting with lymphoblastic mediastinal masses.
During 1968-1978, 68 children presented in six centres in the United Kingdom with lymphoblastic mediastinal masses. The disease was classified as acute lymphoblastic leukaemia (ALL) in 49 children whose bone marrow aspirates contained > 20% lymphoblasts, and as lymphoma (Sternberg lymphosarcoma - LS) in 19 with < 20% marrow infiltration. Male predominated in both groups, and children with ALL had more visceromegaly and lymphadenopathy, lower haemoglobin levels and platelet counts, and higher white cell counts. The most common chest x-ray finding in both groups was a nonspecific anterior mediastinal mass, but the appearances varied considerably and could be classified into three categories, which are illustrated. Pleural effusions were present in 44%. Cell surface-marker studies showed T cell characteristics in 14 of the 17 patients tested. Response to treatment and complications, such as central nervous system (CNS) and testicular relapse, were similar in ALL and LS, and were related to the size of the initial tumour load. Median remission lengths were 37 weeks for ALL and 89 weeks for LS patients. Leukaemic transformation occurred in 47% of LS children. Compared with the outcome in ALL children without mediastinal mass, the results of treatment were poor, regardless of the protocol used, and prophylactic therapy to the CNS reduced the frequency of, but did not eliminate, CNS disease.
Adolescent↗
Allergic bronchopulmonary aspergillosis complicating cystic fibrosis in childhood.
Allergic bronchopulmonary aspergillosis, known to be associated with cystic fibrosis in older patients, occurred in 7 young atopic children with cystic fibrosis. The diagnosis was suggested by the onset of, or the increase in, asthmatic symptoms accompanied by major chest x-ray changes ranging from total collapse of a lung or lobe to extensive but changing areas of consolidation. Each of the children had a blood eosinophilia, positive type I skin tests to Aspergillus fumigatus, and reversible airways obstruction. Most had a positive type III skin test and circulating precipitins to A. fumigatus, with raised IgE levels which contained specific antibodies to the fungus on radioallergosorbent (RAST) test. None had advanced suppurative chest disease of cystic fibrosis. None was given specific antifungal agents; two received systemic treatment with corticosteroids, the other received additional drugs for their asthma. Two developed total collapse of one lung, one child being only 2 years old. Five have had recurrences of pulmonary shadowing typical of allergic aspergillosis but are not showing significant progression of their cystic fibrosis lung disease. Our experience suggests that there should be an increased awareness of this condition, particularly its association with extensive pulmonary collapse or consolidation in children with cystic fibrosis who are atopic.
Aspergillosis, Allergic Bronchopulmonary↗
Renal lesion in Jeune's syndrome.
In three children, presenting in chronic renal failure at the ages of six, four and three years radiology suggsested the diagnosis of Jeune's syndrome (asphyxiating thoracic dysplasia). In each case the skeletal survey showed widespread diagnostic features of this condition. The syndrome is characterized by varying degrees of repiratory distress in infancy and early childhood, with osseous dl three children in this study died from juvenile nephronopthisis. The correct diagnosis is essential for patient management and prognosis and also for genetic counselling. This report emphasizes the importance of the renal lesions in older children with Jeune's syndrome.
Bone Diseases, Developmental↗
Bladder diverticulum: an uncommon cause of acute retention of urine in a male child.
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Acute Disease↗
Giant-cell pneumonia caused by measles and methotrexate in childhood leukaemia in remission.
Four children who had acurate lymphoblastic leukaemia in remission and developed pneumonia were studied. Investigations including electron microscopy and immunofluorescence of lung biopsy material disclosed measles, although there was no clinical evidence of the disease. Despite an identical presentation, two types of illness developed: two children died of giant-cell pneumonia, while the other two developed pneumonia indistinguishable from that associated with methotrexate treatment, recovering when treated with steroids and gammaglobulin. Measles infection is easily overlooked in the absence of rash. The diagnosis may be suggested by clinical and radiological features and confirmed by specific immunofluorescence staining of lung biopsy tissue.
Child↗
Necrotising entero-colitis following umbilical vein catheterisation.
Fifteen cases of neonatal necrotising entero-colitis (NEC) following umbilical vein catheterisation are reported. Their clinical and radiological features are briefly described and the proper course of a catheter through the umbilical vein--ductus venosus--inferior vena cava segment is demonstrated. The significance of mal-position of the catheter and its association with NEC is discussed. It is suggested that the abdominal radiographs be taken in the antero-posterior and lateral projections with a portable X-ray machine to check the position of the radio-opaque catheter prior to the commencement of transfusion of infusion.
Catheterization↗
Renal scarring and vesicoureteric reflux.
A review of 105 children with urinary tract infection showed an increasing prevalence of grades II-III vesicoureteric reflux with diminishing age. During infancy reflux was almost always severe, and affected boys as often as girls. Radiologically scarred kidneys were drained by refluxing ureters in 98% of cases. The prevalence of scars also rose significantly with increasinglyly severe reflux. Deterioration of existing scars or new scar formation was seen in 15 children; 18 out of 20 affected kidneys (90%) were associated with grade III vesicoureteric reflux. 2 out of 5 children who developed new scars did so after 5 years of age. Because severe reflux may occasionally be seen in the presence of a normal intravenous urogram, and since the finding of grades II-III vesicoureteric reflux is an indication for chemoprophylaxis, we consider cystourethrography essential in children of all ages with recurrent urinary tract infection. In children under 5 years the increased prevalence of both severe reflux and renal scarring are arguments for regarding cystourethrography as a necessary initial investigation.
Child↗
Clinical applications of serum carcinoembryonic antigen and alpha-fetoprotein levels in children with solid tumours.
A study was carried out on serum carcinoembryonic antigen (CEA) and alpha-feto-protein (AFP) levels, both measured by radioimmunoassay, in 88 children with malignant solid tumours and in 26 children with nonmalignant disorders, who presented during the years 1973-77. Slightly or moderately raised CEA levels were found at presentation in 11 of 66 children with malignant tumours, in 2 others with recurrent tumours, and in 4 children with nonmalignant disorders. Raised CEA levels generally indicated advanced malignant disease, often affecting the liver, or other hepatic disorders, but were not associated with a specific tumour type. Except in the first months of life, significantly raised AFP levels were detected only in 11 patients with yolk sac-derived tumours, or hepatomas, and in one child with tyrosinosis who later developed a malignant hepatoma. Serial measurements of AFP accurately reflected the clinical response to treatment and in 2 patients indicated recurrence before this could be detected clinically.
Adolescent↗
Ascending uretero-pyelography in renal failure.
Ascending uretero-pyelography has been carried out over a period of 13 years in 97 consecutive patients with undiagnosed renal failure. Sixty-nine were in a non-obstructive uropathy group while 26 had ureteric obstructions. There were two failures. Over 60% of examinations were performed under local anaesthesia, each examination taking an average of 20 min. There has been no mortality and two anaesthetic complications have been the only significant morbidity. Ureteric injury, urinary infection and renal function have all been investigated and recorded. Five per cent of patients developed urinary infection following AUP but without any major consequences. No significant ureteric injury occurred and no late sequelae were noted. Neither any reaction to contrast medium nor any further deterioration in renal function was observed; AUP was diagnostic in 46% of patients. In the remainder it ruled out obstructive uropathy and gave useful information about the kidneys, ureters and bladder. In experienced hands and with proper facilities AUP is safe and can be helpful in the diagnosis and management of patients in renal failure.
Adolescent↗
Design, synthesis, and correlation analysis of 7-substituted 4-hydroxyquinoline-3-carboxylic acids as inhibitors of cellular respiration.
Fifteen 7-substituted 4-hydroxyquinoline-3-carboxylic acids have been designed to minimize covariance between the physicochemical substituent parameters: pi, MR, and sigmap. The molecules have been synthesized and evaluated for their ability to inhibit the respiration of Ehrlich ascites cells as a whole cell model and for their ability to inhibit malate dehydrogenase as an intracellular target enzyme model. Correlation analysis indicates that ascites cell inhibition is linearly related to pi and that malate dehydrogenase inhibition is linearly related to MR.
Animals↗
Giant cell pneumonia complicating acute lymphoblastic leukemia in remission.
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Child↗