Osteoporosis in severe congenital neutropenia treated with granulocyte colony-stimulating factor

Recombinant human granulocyte colony-stimulating factor (G-CSF) has substantially improved life expectancy for children with severe congenital neutropenia (SCN). Severe osteoporosis, reported in this population, may relate to the disease process, or be a therapeutic side-effect. This report details bone loss, quantitated absorptiometrically and histomorphometrically, in a child with SCN and vertebral collapse, and the positive response to anabolic steroid and bisphosphonate therapy.     

Minor,moderate and severe head injury

Summary The future role of the neurosurgeon in the management of head injury is reviewed in terms of the care of patients with minor, moderate and severe head injuries. In minor head injury it is predicted that there will be increasing pressure on the neurosurgeon to undertake the management and follow-up of all patients who have sustained head injury, and this will place a considerable additional load on each neurosurgical unit. This is based on a survey of 1919 head injuries admitted in one calendar year (1981), consisting of 93 severe injuries (GCS < 8), 210 moderate injuries (GCS 8–12), and 1616 minor injuries (GCS 13/14). In moderate injuries CT will assume a major role in detecting hematoma early and identifying contusions. There may yet be a role for steroids in these cases and there should be a greater use of neurorehabilitation, instead of the current overemphasis on the severely injured. In severe injury future efforts will be to prevent early secondary insults and to find better methods of controlling raised intracranial pressure.     

Clinical efficacy of intravenous immunoglobulin in patients with severe inflammatory chest disease and IgG3 subclass deficiency

To investigate the efficacy of i.v. IgG treatment in pediatric patients with inflammatory lung disease, a prospective, controlled clinical trial was carried out over a 2-year study period. Patients were enrolled on the basis of severe clinical symptomatology. After 1 year of conventional treatment, the patients received 400 mg/kg per month of an i.v. IgG product containing only trace amounts of IgG3 in addition to their regular treatment throughout the second year. Significant clinical improvement, as documented by duration of hospital stay (first year 27.8 days, second year 4.9 days), use of antibiotics (132.8 versus 30.9 days) and use of steroids (21.4 versus 0.7 days) could be observed. Data obtained on a subgroup of patients with IgG3 deficiency were analysed separately. These results indicate that patients with severe chest disease who have IgG3 deficiency will also benefit from i.v. IgG treatment. The mode of action cannot be attributed to replacement of the respective isotypes, but is probably due to the effect of i.v. IgG in preventing repeated viral infections.     

The potential of mild testicular heating as a safe, effective and reversible contraceptive method for men

A preliminary assessment of the contraceptive efficacy of a daily mild increase (1–2°c) in testicular temperature during waking hours is reported in nine couples using two techniques of non-surgical fixation of the testes close to the inguinal canal. With technique 1, immobilization was achieved by passing the penis and the empty scrotum through a hole made in close-fitting underwear; there was one pregnancy, hm a man who stopped the heating after 7 weeks, for 42 cycles of exposure in three couples. With technique 2, immobilization was achieved by adding a ring of soft material surrounding the hole in the underwear; there was no pregnancy for 117 cycles of exposure in six couples. Reversibility and safety were assessed. These preliminary results suggest that a daily mild increase in testicular temperature could be a potential contraceptive method for men.     

河南省传染性非典型肺炎患者胸部X线表现

目的 :探讨传染性非典型肺炎的X线表现及变化规律。方法 :对河南省临床诊断的 1 5例患者发病后不同时间的系列胸片和CT进行回顾性分析。结果 :1 5例胸部X线病灶初始形态为斑片状 1 1例 (73.3% ) ,大片状 4例 (2 6 .7% ) ;双侧 9例 (6 0 .0 % ) ,单侧 6例 (4 0 .0 % ) ,均为中、下肺野 (1 0 0 % )。动态观察发现病变进展快 ,病变发展到高峰期时间为 4～ 1 4d , x±s(7.7± 3.2 )d ,双侧 1 3例 (86 .7% ) ,单侧 2例 (1 3.3% ) ,两肺叶及两肺叶以上病变者 1 4例 (93.3% )。病变开始吸收时间为 6～ 2 1d , x±s(1 1 .3± 4 .1 )d ;完全或基本吸收时间 8～ 2 6d , x±s(1 6 .4± 5 .0 )d。1 5例经治疗均痊愈出院。住院时间 1 8～ 4 2d , x±s(31 .9± 7.9)d。 结论 :传染性非典型肺炎的胸部X线特点为急性双侧或单侧多叶炎性浸润阴影 ,以中、下野常见 ,进展迅速 ,及时复查胸部X线对临床诊断及判断病情具有重要价值。     

Paroxysmal nocturnal haemoglobinuria: Nature's gene therapy?

The development of paroxysmal nocturnal haemoglobinuria (PNH) requires two coincident factors: somatic mutation of the PIG-A gene in one or more haemopoietic stem cells and an abnormal, hypoplastic bone marrow environment. When both of these conditions are met, the fledgling PNH clone may flourish. This review will discuss the pathophysiology of this disease, which has recently been elucidated in some detail.     

Chronic anemia as a complication of parvovirus B19 infection in a pediatric kidney transplant patient

. This is a report of unexplained anemia that persisted for 4 months in an adolescent renal transplant patient receiving immunosuppression that included prednisone, tacrolimus, and mycophenolate mofetil. This patient required monthly blood transfusions for fatigue, palpitations, and hematocrit levels between 15% and 17%. In addition, his posttransplant course was notable for the development of insulin-dependent diabetes mellitus. While receiving low-dose prednisone, he was switched from tacrolimus to cyclosporin and tapered off insulin injections over the next 2 months. At 4.5 months post-transplantation, further diagnostic evaluation was suggestive of parvovirus B19 infection as the cause for our patient’s chronic anemia. After testing negative for serum-specific parvovirus B19 IgM and IgG antibodies, parvovirus B19 infection was detected in blood by the polymerase chain reaction. Treatment with intravenous immunoglobulin (1 g/kg per day × 2 days) resulted in normalization of both his reticulocyte count and hematocrit within 6 weeks. At 4 months after receiving the immunoglobulin infusion, he has maintained a normal hematocrit level and stable renal function without requiring further blood transfusions. Received August 23, 1996; received in revised form and accepted November 20, 1996     

Mono- or Polypharmacotherapy in Institutionalized Epileptic Children with Severe Mental Retardation?

ABSTRACT. Nine severely mentally retarded patients with severe epilepsy who were living in an institution were studied during a three-year period by a multi-disciplinary team. A seizure rate serum level chart was made for each patient. It served as the basis for monthly discussions in the team about medication changes. Drug plasma concentrations were monitored monthly. Statistical comparisons between seizure frequency on different drug regimens were made by χ² test. Withdrawal of all medication was possible in one case and reduction to monotherapy in two cases. In the remainder of the patients a combination of two or three anticonvulsants gave the best clinical effect. We thus found polypharmacotherapy necessary for some severely retarded patients with epilepsy. Our multidisciplinary approach and chart monitoring system has many advantages and is valuable for the medical care and drug treatment of this patient category.