Clinical course of patients with cystic fibrosis presenting with meconium ileus.
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Biomedical subjects
Publications and source records attributed to R Carachi.
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Mesenchymoma of the chest wall is rare in infancy and can be easily mistaken for a malignant tumor. It is a distinct pathological entity that presents at birth or during infancy as self-limiting expansile intraosseous lesions involving the body of one or more ribs. Histologically, it is a benign focal overgrowth of normal skeletal elements. Two such cases are presented.
OBJECTIVE: To assess whether pre-operative chemotherapy reduces operative morbidity in children with intravascular extension of renal tumours. PATIENTS AND METHODS: Thirty children with intravascular extension of their renal tumour, treated in 10 different centres in the UK, were reviewed retrospectively. RESULTS: Twenty-nine patients had nephroblastoma and one child had clear cell sarcoma (favourable histology in 23, unfavourable histology in six). Patients were classified into stage II (17 patients), stage III (three patients) and stage IV (10 patients). Ultrasonography had been performed in 29 patients and had correctly diagnosed intravascular extension in 11 (40%); computed tomography (CT) was accurate in 93% of patients. A pre-operative diagnosis was made accurately in 24 patients, with caval extension in 18 and atrial extension in six. Nine patients underwent primary surgery, whilst 21 had pre-operative chemotherapy followed by delayed nephrectomy. In the latter group, the intravascular thrombus diminished in 16 patients. Five patients died, one from tumour rupture and four from extensive or progressive tumour disease; the overall 2-year survival was 83%. Unfavourable histology did not adversely affect survival, and patients having pre-operative chemotherapy appeared to have a better outcome. CONCLUSION: CT remains the best imaging modality to assess intravascular tumour extension. Pre-operative chemotherapy is recommended for patients with intra-caval extension of tumour. Those with intra-atrial extension or with hepatic vein obstruction (Budd-Chiari syndrome) may require a cardiopulmonary bypass and primary surgery.
Two siblings with extensive aplasia cutis congenita of the extremities are described. These lesions healed spontaneously and in the older sibling at four years follow-up there was minimal scar tissue at the affected sites.
Collagen-coated Vicryl mesh (C.C.V.M.) was used in 28 children who needed repair of thoracic and abdominal wall defects. Herein the authors report the clinical experience, surgical technique, and outcome in these cases.
Sonographic and operative findings in both groins of 23 infants presenting with a unilateral inguinal hernia were compared in a prospective study. Ultrasound accurately confirmed the presence of a hernia on the affected side in all infants but provided no useful additional diagnostic information. Ultrasound examination of the unaffected side was less accurate. Surgical exploration revealed the presence of a patent processus vaginalis (PPV) in 18 cases and its absence in five. Ultrasound was found to be accurate in only 15 out of the 23 cases with four false positive and four false negative results. This yielded an accuracy of 65%, a sensitivity of 78% and a specificity of only 20%. It is concluded that ultrasound cannot at present be used alone to plan the management of the contralateral groin, especially as the majority of children who do have a PPV will never develop an inguinal hernia.
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The case history is described of an infant, with a thoracic foregut duplication cyst containing a perforated peptic ulcer, who presented with haemoptysis and respiratory distress. This presentation is discussed within the context of thoracic foregut duplications.
In a prospective study, 25 children with nephroblastoma (Wilms' tumor) had preoperative plasma prorenin and renin concentrations measured. The mean plasma renin and prorenin concentrations in the patients were raised compared with a control group of patients without nephroblastoma. Four children had recurrence of tumor and, in three, this was associated with an increase in plasma prorenin concentration. Nephroblastoma tissue contained immunoreactive renin and renin messenger RNA, and the renin protein was immunologically and biochemically similar to normal human renin. We conclude that prorenin concentrations in plasma are an indicator of nephroblastoma.
The differentiation of the causes of acute scrotal pathology by clinical means alone is prone to inaccuracy, resulting in inappropriate surgical exploration. Colour Doppler imaging (CDI) provides the facility to assess blood flow. Torsion of the testis impedes blood flow, whereas it is enhanced or normal in inflammatory pathology. We have investigated 40 cases of acute scrotal disease by means of CDI in which the radiologist performing the test was unaware of the preliminary clinical diagnosis. The clinical diagnostic accuracy was 60%, whereas the diagnostic accuracy of CDI was 82%. CDI provides an effective means of enhancing diagnostic accuracy.
A collagen-coated Vicryl mesh bioprosthesis was used to repair a 4 x 4 cm full-thickness abdominal wall defect, created in experimental rats. The tensile strength of the repair at 6 months reached 70% of the original abdominal wall. Implant collagen could not be differentiated from host collagen after 2 weeks. The increase in collagen content of the repair was responsible for the increasing tensile strength of the wound with time. All histological sections showed good tolerance of the implant. These results support the use of collagen Vicryl membrane to repair large abdominal wall defects. Clinical trials are indicated.
We describe a technique used to provide urinary continence to a group of teenagers with neuropathic bladders. The technique gives continence to a urinary reservoir by utilising the appendix as a continent catheterisable conduit. Of the first five patients to have this procedure performed in this institute, four became fully continent. We feel that this procedure can be of great benefit to both children and adults with urinary incontinence.
Fifteen children with urinary incontinence underwent continent urinary diversion using Mitrofanoff principle between April 1990 and March 1993, at a mean age of 14 years. Neurogenic bladder was the main underlying cause of incontinence and spina bifida accounted for the majority of cases (12 patients). Reversed vascularised appendix was used as a cutaneous stoma for intermittent self-catheterisation Fourteen out of the fifteen children treated, achieved urinary continence with 3-4 hourly catheterisation.
Serum alpha-interferon levels were analysed in 50 consecutive children admitted with right iliac fossa pain. Serum alpha-interferon levels were significantly raised in 33% of children without acute appendicitis when compared to 5.7% of children with histologically proven acute appendicitis and 9% of normal controls. This interesting phenomenon needs further investigation. A larger series may show it to be of clinical use in non-specific abdominal pain in childhood.
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This article reviews the management and mortality of 53 neonates with meconium ileus and cystic fibrosis treated in one paediatric surgical centre from 1972 to 1990. Forty-four patients (83 per cent) presented with simple meconium ileus and 26 of 40 patients in this group were successfully treated with Gastrografin enemas. Four patients suffered a perforation as a result of the enema (perforation rate 5 per cent). The 1-year survival rate for simple meconium ileus was 81 per cent and for complicated meconium ileus 75 per cent.
Collagen/vicryl composite membrane has been previously shown to be of use in an experimental model as a patch graft in the bladder and oesophagus. Attempts to use it as a circumferential tube graft have been unsuccessful due to the development of strictures at the site of anastomosis. We inserted a polytetrafluoroethylene (PTFE) stented collagen/vicryl bioprosthetic tube into rat omentum, which served as a vascular pedicle. Subsequent histology of the graft showed serial replacement of the bioprosthesis with host collagen and the development of a blood supply. In a second stage procedure, the bioprosthesis was anastomosed to the distal ileum and brought out on the skin to form a fistula. Squamous epithelium was subsequently identified growing the full length of the graft. Therefore, we have developed a vascularised bioprosthetic tube graft which will support the growth of unspecialised epithelium. This model could be of use in bridging congenital atresias or pathological strictures.