Combination of radiotherapy and double blockade HER2 with pertuzumab and trastuzumab for HER2-positive metastatic or locally recurrent unresectable and/or metastatic breast cancer: Assessment of early toxicity.

BACKGROUND AND PURPOSES: We evaluate the early toxicity of concurrent use of radiotherapy, pertuzumab and trastuzumab in patients with HER2-positive metastatic or locally recurrent unresectable breast cancer. PATIENTS AND METHODS: A retrospective study was performed in a population of 23 consecutive patients between 2013 and 2015. Radiotherapy was performed on the chest area or metastatic sites during maintenance with pertuzumab and trastuzumab after six cycles of pertuzumab, trastuzumab and docetaxel. Toxicity was assessed according to the National Cancer Institute Common Toxicity Criteria version 4. RESULTS: Irradiation volumes were whole breast (8 patients) and chest wall (9 patients) at 50Gy in 25 fractions, the supraclavicular nodes (16 patients), the axillary area (nine patients) and the internal mammary nodes (9 patients) at 46Gy in 23 fractions. For five patients, radiotherapy was palliative: bone irradiation (4 patients), whole brain radiotherapy (one patient). Median follow-up was 12.6 months (range: 6.1-21.6 months) since the start of pertuzumab and trastuzumab. One patient presented an asymptomatic decrease of left ventricular ejection fraction below 50%. No symptomatic cardiac events were reported. Two patients presented asymptomatic grade I radiation pneumonitis. Acute skin toxicity was grade III (one patient), grade II (6 patients), and grade I (5 patients). There were two grade II esophagitis. CONCLUSION: Combination of pertuzumab, trastuzumab and radiotherapy was well tolerated, which should be confirmed by the results of larger studies.

One-stage combined surgery with or without preoperative traction for developmental dislocation of the hip in older children.

PURPOSE: To compare one-stage combined surgery with and without preoperative traction, in older children with developmental dislocation of the hip (DDH). METHODS: Records of 9 children who underwent combined surgery for DDH with preoperative traction in 12 hips (group 1) and 12 undergoing the same procedure without preoperative traction in 16 hips (group 2) were retrospectively reviewed. The surgery consisted of open reduction, Salter's innominate osteotomy and femoral shortening with derotation varus osteotomy. The mean age of the patients at the time of operation was 5.8 years. The mean follow-up period was 5.9 years. RESULTS: At final follow-up, clinical outcome in group 1 was worse than that in group 2, though radiographic assessment demonstrated no significant difference between the groups. CONCLUSION: One-stage combined surgery without preoperative traction is effective in the treatment of DDH in older children, and has a lower complication rate, but radiographically the groups did not differ.

Simultaneous bilateral trochanteric fractures during squatting in a patient with multiple myeloma.

Pathologic bone fractures are usually confined to the trunk in multiple myeloma (MM). But bilateral trochanteric fractures have not been reported in patients with MM before. Radiographic, histological and immunoelectrophoresis revealed typical features of MM. This report points out the importance of physical rehabilitation of patients in addition to chemotherapy in adaptation to the activities of daily living.

A case with spondylo-metaphyseal dysplasia type A4.

We present a case with spondylo-metaphyseal dysplasia type A4 characterized by ovoid vertebral bodies with anterior tongue-like deformities, widened irregular and sclerotic metaphyseal changes, short iliac wings, slightly short long bones and short tubular bones of the hands with irregular metaphyses. She also had bipartite trochlea and irregular patellar margins, which have not been described in spondylo-metaphyseal dysplasia types to date.

Two unusual types of syndactyly in the same family; Cenani-Lenz type and "new" type versus severe type I syndactyly?

Cenani-Lenz syndactyly is a very rare syndrome where the syndactyly is totally disorganized with abnormal development of pattern formation of the hand. We report here an additional case of Cenani-Lenz syndactylism in a woman who has congenital cataract and an unusual type of duplication of big toes not described so far. She had a half cousin who had an unusual new type or severe type I syndactyly. It is not clear whether these two types of syndactyly present in this family may be coincidental or not.

A case with Pyle type metaphyseal dysplasia: clinical, radiological and histological evaluation.

Pyle type metaphyseal dysplasia is a rare autosomal recessive disease that is primarily affect metaphyses. Here we present a case with Pyle type metaphyseal dysplasia. The characteristic features of the case were metapyhseal broadening with undertubulation and Erlenmeyer flask sign at distal femoral and proximal tibial metaphyses. There were also platyspondyly with biconcave lens appearance of the vertebral bodies, congenital hip dislocation and normal cranium. Bone histopathology showed decreased number of osteoclasts. To the best of our knowledge, this is the first reported case of Pyle type metaphyseal dysplasia from Turkey.

First-trimester diagnosis of Robinow syndrome.

We present 2 cases with Robinow syndrome in a nonconsanguineous Turkish couple. The first case, second living child of the family, has all of the cardinal features of this syndrome including short stature, mesomelic shortening of forearms, frontal bossing, hypertelorism, anteverted nares, triangular mouth, hypoplastic genitalia and vertebral and costal anomalies. The second case was diagnosed with first-trimester ultrasonographic findings such as shortening of extremities and increased nuchal translucency thickness at 12 + 4 weeks of gestation, and the family wished to terminate this pregnancy. After abortion, we obtained findings such as typical face features, shortening of forearms, ambiguous genitalia suggesting Robinow syndrome with autopsy examination.

Surgical risk factors in Larsen's syndrome.

The authors report on a child with typical Larsen's syndrome with some rare findings such as mixed-type hearing loss and with some potentially fatal operative risks including laryngomalacia and cervical instability. A few deaths with Larsen's syndrome have been reported associated with various fatal risks such as spinal instability. Therefore, laryngomalacia and several other potentially fatal risks are presented in this report as awareness may prove essential to orthopedic surgeons.

Mesoaxial complete syndactyly and synostosis with hypoplastic thumbs: an unusual combination or homozygous expression of syndactyly type I?

Syndactyly type I is an autosomal dominant condition with complete or partial webbing between the third and fourth fingers or the second and third toes or both. We report here a previously undescribed phenotype of severe mesoaxial syndactyly and synostosis in patients born to affected parents. The characteristic features of these severe cases are (1) complete syndactyly and synostosis of the third and fourth fingers; (2) severe bone reduction in the proximal phalanges of the same fingers; (3) hypoplasia of the thumbs and halluces; (4) aplasia/hypoplasia of the middle phalanges of the second and fifth fingers; and (5) complete or partial soft tissue syndactyly of the toes. We report on three offspring with this phenotype from two different branches of a syndactyly type I family, suggesting that they may be homozygous for this condition. SSCP and linkage analysis indicated that neither HOXD13 nor other relevant genes in the chromosome 2q31 region was responsible for this phenotype.

Larsen's syndrome with mixed-type hearing loss.

Mixed-type hearing loss is an extremely rare feature of Larsen's syndrome, a complex of symptoms characterized by multiple joint dislocation and "flat" faces. In this report, a patient with bilateral mixed-type hearing loss and Larsen's syndrome is presented.

A single preoperative dose of cefazolin versus postoperative amoxicillin and gentamycin combination in prophylaxis of orthopaedic and traumatologic surgery.

The use of antibiotic prophylaxis was evaluated in 228 surgical patients. The patients were given either cefazolin preoperatively or amoxicillin + gentamycin postoperatively in a randomized fashion to determine whether cefazolin would demonstrate an advantage over amoxicillin + gentamycin with respect to protection against postoperative wound infection, systemic infection and cost. We also tried to find out if the inflation of a tourniquet would interfere in the incidence of wound infection. Major postoperative wound infections were reduced in the cefazolin treated group (2.56 per cent versus 4.50 per cent). There also was a reduction in minor postoperative wound infections in the cefazolin group when it was compared with the amoxicillin + gentamycin group (6.83 per cent, 13.51 per cent, respectively). No systemic infection was seen in either group. There was no difference between the patients on whom were used tourniquets or not. Analysis of patients' charges suggested that 68.75% of the cost of preoperative antibiotic prophylaxis might be saved by limiting the duration of prophylactic drug administration by cefazolin.

Simultaneous bilateral fractures of the femoral neck and superior pubis ramus following renal failure-induced hypocalcaemic convulsions.

There have only been a few reports of bilateral hip fractures resulting from hypocalcaemic convulsions. We report a case of simultaneous bilateral fractures of the femoral neck and superior pubic ramus caused by convulsions secondary to renal failure-induced hypocalcaemia. To our knowledge, such an occurrence has not previously been described.