Histology of the upper pole in complete urinary duplication--does it affect surgical management?

Objective: To review the histological change in the upper pole of excised duplex kidneys and assess whether ante-natal diagnosis might predispose to more conservative surgical management of this abnormality.

Patients And Methods: Fifty consecutive patients undergoing upper pole hemi-nephroureterectomy for ectopic ureter or ectopic ureterocele between 1980 and 1992 had their histology reviewed and assessed for dysplastic, inflammatory and obstructive change.

Results: Segmental scarring and chronic and acute inflammatory change occurred consistently and the degree of inflammation seemed unaffected by antenatal diagnosis.

Autologous gastro-intestinal reconstruction: the composite ileo-colic loop.

A reproducible technique was developed experimentally in pigs for construction of a 'composite bowel tube' (CBT) made up of ileal mucosa that was grafted to a mucosally denuded colonic muscle surface vascularised by the colonic mesentery. Macroscopic and microscopic studies at terminal laparotomy revealed a viable, mucosally lined, patent peristaltic loop of bowel in six of the eight animals. Two animals died after sloughing the grafted mucosa.

Childhood steroid-sensitive nephrotic syndrome: does the histology matter?

Renal biopsy specimens from 51 children with steroid-sensitive nephrotic syndrome who were following a frequently relapsing or steroid-dependent course were reviewed by two histopathologists. In all cases the biopsy was performed prior to the commencement of an 8-week course of cyclophosphamide. The clinical courses of these patients both prebiopsy and for a minimum of 2 years after completion of cyclophosphamide therapy were analyzed using retrospective case note analyses.

Simultaneous choriocarcinoma in mother and newborn infant.

A male infant, born following an uncomplicated pregnancy, was severely anaemic at birth following significant foeto-maternal haemorrhage. At three weeks of age a tumour was found in the liver with evidence of metastatic disease in the lungs. The infant died before treatment could be started.

Oligohydramnios sequence: the spectrum of renal malformations.

Objective: To assess the value of the autopsy findings on a series of infants dying with features of the oligohydramnios sequence, with particular reference to anomalies of the renal tract.

Design: Retrospective review.

Setting: Pathology departments serving three maternity units in Manchester.

Choristoma of the submandibular gland: a rare cause of cervical swelling.

A case of choristoma of the submandibular gland excised from a 4-week-old female infant is reported. The differential diagnosis is discussed and suggestions are made for the possible etiology of the lesion.

Pseudo-Gaucher cells.

A case of acute lymphoblastic leukaemia, associated with cells resembling Gaucher cells in the bone marrow, is reported. The patient had no evidence of inherited Gaucher's disease and the ultrastructural appearance of the cells was consistent with pseudo-Gaucher cells described in other haematological diseases. This is the first report of these cells in association with acute lymphoblastic leukaemia.

Inflammatory pseudotumor of the liver masquerading as a metastasis in a child treated for nephroblastoma.

A 3 1/2-year-old boy presented with a palpable hepatic tumor thought on clinical and radiological grounds to be a metastasis but which was found to be an inflammatory pseudotumor on histological examination. Eighteen months previously he had received chemotherapy and radiotherapy for a stage IV Wilms' tumor, which had been surgically excised 4 months after commencing treatment. This case illustrates the importance of obtaining a histological diagnosis in the management of patients with malignant tumors.

Male fertility in long-term survivors of childhood acute lymphoblastic leukaemia.

To study long-term testicular function following the treatment of acute lymphoblastic leukaemia (ALL) in childhood, 37 young adult males were assessed at two separate time points. The initial assessment was made by a wedge testicular biopsy after completion of treatment (median 9.7 years; range 4.

Ultrastructure of the human placenta in metabolic storage disease.

Eleven placentae and one fibroblast cell culture from pregnancies complicated by various inherited metabolic disorders, together with five chorionic villus biopsies from pregnancies at risk, were examined for ultrastructural evidence of accumulation of metabolites. Abnormal ultrastructural features were present as early as 10 weeks gestation. Myelin bodies were found in all placental cell types in a case of Niemann-Pick disorder and stromal cells showed marked vacuolation in Hurler's disease.

Mesenteric fibromatosis presenting with ascites in childhood.

A 7-year-old boy presented with acute ascites due to mesenteric fibromatosis. The pathophysiological mechanisms for formation of ascitic fluid and some aspects of the aetiology of intra-abdominal fibromatosis and its association with Gardener's syndrome are discussed.

Cutaneous presentation of sarcoidosis in an infant.

A 4-month-old infant developed a symptomless erythematous maculopapular rash on her abdomen spreading to involve other areas of her trunk and limbs. Skin biopsy showed the features of sarcoidosis and she later developed sarcoid uveitis and arthritis requiring systemic steroid therapy.

Increased concentration of aluminium in the brain of a parenterally fed preterm infant.

Parenteral feeding solutions currently used for preterm infants are contaminated with aluminium. We report the case of an infant who was fed parenterally for 45 days, who died aged 3 months, and who had a considerably increased concentration of aluminium in his brain tissue at necropsy.

Haemolytic uraemic syndrome and the Thomsen Friedenreich antigen.

In three children with haemolytic uraemic syndrome (HUS), evidence of red cell polyagglutinability due to Thomsen Friedenreich antigen (T-antigen) exposure was demonstrated. This was suspected after difficulties in ABO typing and was confirmed using specific antisera. Further supportive evidence included elevation of plasma sialic acid, alteration in red cell surface charge and evidence of T-antigen exposure in the renal biopsy specimen of one patient.

Vertebro-basilar thrombosis and involuntary movements.

There are a number of causes of thrombosis in the vertebro-basilar system, and among these are trauma and abnormal movements. This is particularly likely to occur if there are anomalies or abnormalities of the spine or arteries. A case is reported which suggests that involuntary movements should be included in the possible aetiologies.

Long term prognosis of recurrent haematuria.

A long term follow up study of 100 children referred with recurrent haematuria for at least one year to two regional paediatric nephrology units is described. The mean duration of follow up was 8.2 years.

Testicular invasion and relapse and meningeal involvement in a rat T-cell leukaemia.

During the haematogenous dissemination of this acute rat T-cell (Roser) leukaemia, infiltration of both epididymal and testicular interstitial tissue has now been demonstrated, probably as an invariable occurrence. The gonadal duct system itself was not invaded. In contrast to an earlier histopathological study with this leukaemia, meningeal invasion has also been encountered during routine passage.

Testicular relapse in acute lymphoblastic leukaemia: studies with an experimental mouse model.

Neither testis nor epididymis was found to be invaded by L1210 leukaemic cells in spite of widespread dissemination through other organs and tissues. The same applied to animals in relapse after protracted remissions induced by cyclophosphamide. Prior damage to the gonadal vascular endothelium by Cd++ did enable leukaemic cells to enter the testicular interstitium, but not the depleted seminiferous tubules.

Aneurysm of the coronary sinus.

Two patients, an adult and a baby, with an aneurysm of the coronary sinus are described. This unusual anomaly was detected in the baby during angiographic investigations for congenital heart disease. The aneurysm was an unexpected postmortem finding in the adult.

The testis. A protected environment for leukaemic cells against cyclophosphamide in a mouse model.

Groups of BDF1 mice, inoculated either IM or by the intratesticular (IT) route with comparable numbers of L1210 cells, died within the same time range from the disseminated disease. Cyclophosphamide (100 mg/kg IP) given on day 6 after inoculation, when the disease was advanced, increased the lifespan by about 100%, but all the mice died. The same dose on day 3 effectively cured all mice inoculated IM, whereas those injected with cells into the testicular lymphatic sinusoidal system died with only a short prolongation of lifespan.

Renal pseudotumours caused by xanthogranulomatous pyelonephritis.

Five cases of xanthogranulomatous pyelonephritis are described. Wilms's tumour was suspected preoperatively in 2 of the cases and a renal tumour appeared to be confirmed at laparotomy in three. Pathological examination is diagnostic and the condition appears to be always unilateral.