Breast specimen radiography: evaluation of a compression device

The irregular shape and uneven tissue thickness of excised breast specimens makes radiographic evaluation difficult, especially when calcifications are not present. Xeroradiographs before and after compression of 20 separate excised breast specimens were compared, and 17 of the same specimens were compared after compression combined with immersion in water. Specimen compression improved visibility of the lesion on average in 88% of cases, and visibility was equal in 12%. Combined compression/immersion further improved visibility of the lesion on average in 37% of cases. More significantly, evaluation of the compressed specimen led to a change in interpretation of the radiographs in 45% of cases. Compression of the specimen in specimen radiography is recommended in all cases in which pre-biopsy localization is performed.     

Acute demyelinating sensorimotor polyneuropathy in B‐cell lymphoma with IGM autoantibodies against glycolipid GD1B

A 20‐year‐old man developed weakness without sensory complaints ten days after rubella. Examination showed limb weakness and brisk tendon reflexes but no sensory abnormalities. Laboratory investigations revealed IgG and IgM anti‐Rubella and increased CSF protein content (0,8 g/L). Electrophysiological examination showed partial motor conduction blocks in eight nerves and normal sensory conductions even across the sites of CB. Brain and spinal cord MRI and SEPs were normal. The patient was treated with four plasmaphereses and fully recovered in six months. Conduction blocks gradually improved with increasing duration and abnormal temporal dispersion in proximal CMAPs. GBS has been rarely reported after rubella. Anti‐myelin basic protein antibodies have been found in a patient with a relapsing motor neuropathy following rubella vaccination. As antibodies cross‐reacted with a viral protein, molecular mimicry has been proposed as a pathophysiological mechanism. In our patient we did not find anti‐MBP antibodies and antibodies to‐glycolipids (GM1, GM2, GA1, GD1a, GD1b, GQ1b, sulfatides, galactocerebroside) were also negative. Indirect immunofluorescence after incubation of patient's serum on rabbit sciatic nerve and human sural nerve and roots was negative. Our patient confirms the occurrence of GBS following Rubella and shows some uncommon features: 1) hyperactive deep tendon reflexes; 2) demyelination selectively involving motor fibres; and 3) widespread early conduction blocks in intermediate nerve segments.     

Remission following an elemental diet or prednisolone in Crohn's disease

The short- and long-term effects of an elemental diet in children with acute Crohn's disease were compared with those of prednisolone in historical controls. Clinical remission was induced in 25 of 30 and in 18 of 28 episodes treated for six weeks with an elemental diet and prednisolone. Patients with proximal disease had longer remission after treatment with an elemental diet (p < 0.05) than did patients with colonic disease after treatment with prednisolone (p < 0.01). Disease activity index score improved in both groups compared with the pretreatment scores (p < 0.05). However, the improvement in the elemental diet group was significantly better than in the prednisolone group (p < 0.001). Changes in linear growth were better after treatment with an elemental diet compared with steroids (p < 0.001). Serum albumin and haematocrit concentrations all improved significantly in the children treated with an elemental diet (p < 0.001) but not in those treated with steroids. Thus an elemental diet was better than prednisolone in proximal disease and confirmed improved growth and nutritional status.     

Renal and cerebral necrosis in survivor after in utero death of co-twin

A newborn with bilateral renal cortical necrosis and severe cerebral damage in association with a macerated stillborn twin is reported. The alterations in the kidneys and brain of the twin born alive suggest that the primary event took place before birth. Thromboplastic material and embolizing particles derived from the dead fetus may have passed the monoamnionic-monochorionic twin placenta and caused disseminated intravascular coagulation in the living twin, followed by infarction in other organ systems.     

Renal Fanconi syndrome: first sign of partial respiratory chain complex IV deficiency

A 2-year-old boy who developed hypophosphatemic rickets without signs of muscular weakness or neurological disturbances is presented. Biochemical findings included hypophosphatemia, metabolic acidosis, hypouricemia, hyperphosphaturia, severe glucosuria, generalized hyperaminoaciduria, hypercalciuria, proteinuria with elevated excretion of IgG, transferrin, albumin and high levels of α-1-microglobulin. Urine concentration capacity and creatinine clearance were normal. Lactaturia without elevated levels of plasma lactate and a high urinary excretion of β-hydroxybutyrate were suggestive for mitochondriopathy. Partial deficiency of cytochrome c oxidase (complex IV of the respiratory chain) was found in skeletal muscle. A renal biopsy specimen demonstrated enlarged mitochondria with abnormal arborization and disorientation of the cristae in the proximal tubular cells. Reduced activity of mitochondrial cytochrome c oxidase in tubular cells could be demonstrated by ultracytochemistry. In conclusion, rickets due to the renal Fanconi syndrome can be the first clinical sign of mitochondrial cytopathies without extrarenal symptoms. Elevated excretion of lactate and ketone bodies in urine may serve as a diagnostic marker. Received: 6 October 1998 / Revised: 17 August 1999 / Accepted: 18 August 1999     

Vertical transmission of Kaposi's sarcoma

AIDS related Kaposi's sarcoma is commonly seen in homosexual men, only occasionally in men and women with heterosexually acquired HIV, and extremely rarely in children. The case of an HIV infected mother and her vertically infected child who both developed visceral Kaposi's sarcoma is reported. It is proposed that the putative Kaposi's sarcoma agent may also be transmitted vertically.     

Extracorporeal life support in paediatrics

Extracorporeal membrane oxygenation (ECMO) is a life support technique based on modifications of heart-lung bypass technology. It is used to support severe but potentially reversible pulmonary or cardiopulmonary failure. There is increasing use of the technique for neonates and a return of interest in its use for adults. The number of non-neonatal paediatric patients receiving pulmonary support with ECMO worldwide is, however, small, and survival rates average less than 50%. Initial experience in 15 patients aged 3 months to 5 years with a high survival and low morbidity is reported.     

Pathological complications of non-survivors of newborn extracorporeal membrane oxygenation

The pathology was reviewed of the early deaths identified from the first 50 neonates treated with extracorporeal membrane oxygenation (ECMO) during its introduction to the UK. Fifteen neonates died during or shortly after ECMO between August 1989 and June 1992. Data on 12 are presented (three did not have a postmortem examination). The clinical diagnoses at referral for ECMO were as follows: persistent pulmonary hypertension of the newborn (six infants), primary congenital pneumonia (one infant), community acquired pneumonia (two infants), birth asphyxia (one infant), respiratory distress syndrome (one infant), and meconium aspiration syndrome (one infant). In our group, at necropsy, five had significant haemorrhage (three intracranial, one pulmonary, one pericardial and intraventricular). Three of five infants with evidence of haemorrhage also had signs of sepsis. Six infants had evidence at necropsy of systemic sepsis, five showed evidence of severe anoxic brain injury, and four infants had cerebellar haemorrhages. Three infants had evidence of myocardial ischaemia. It is difficult to discriminate between the relative influence of the primary diagnosis, the mode of treatment, and the severity of presentation in the genesis of this pathology. It is likely that the extent and severity of some of the findings represent a pathological progression that would have been interrupted by the death of the patient, had ECMO not been instituted.