We present the case of a 28 year old lady with refractory Crohn's Disease treated with infliximab throughout her pregnancy. Her baby was born healthy and received a Bacillus Calmette-Guérin (BCG) vaccine aged 3 months. Soon after this the infant became unwell and died aged 4.5 months. At post-mortem the cause of death was attributed to an unusual complication of the BCG vaccine, known as disseminated BCG. BCG vaccination is contraindicated in individuals who are receiving immunosuppressive drugs. We recommend physicians should exercise caution before such vaccines are used in infants born to mothers taking anti-TNF therapies or other potentially immunosuppressive IgG1 antibodies.

Routine post-mortem paediatric radiography in foetuses and neonates is neither diagnostically useful nor cost-effective. A more evidence-based, selective protocol should yield significant cost savings.

It is well documented that distal 9p monosomy can be associated with XY sex reversal. Recently, the possibility of DMRT1 and/or DMRT2 (the genes for doublesex and mab-3 related transcription factor 1 and 2) being the sex determining genes(s) at 9p has been raised. DMRT1 and DMRT2 map near the 9p telomere, distal of marker D9S1779. We describe here three unrelated females with distal 9p monosomy, one with XY complement and two with XX complements. In each individual, fluorescent in situ hybridization predicted the loss of the DMRT genes. Patient 1, an XY individual with monosomy 9pter --> p24.1 approximately 24.2 and trisomy 7q32 --> qter had normal female external genitalia, a blind ending vagina, no uterus, a Fallopian tube on the right, and bilateral ovotestes with primitive ovarian tissue. She also had extreme growth retardation. Around 80 cases of distal 9p monosomy have been reported previously, but there has been no report of ovotestes or gonads comprising ovarian tissue in a XY patient with 9p deletion. Findings in Patient 1 suggest that the phenotypic spectrum of the heterozygous DMRT deletion may include true hermaphroditism. Patients 2 and 3 were 12- and 14-year-old females with XX complements, normal external genitalia, and normal pubertal development. Patient 2 had pure monosomy 9pter --> p23 and Patient 3 had monosomy 9pter --> p22.3 approximately 23 combined with trisomy 3pter --> p23 approximately 24. To date, detailed reports on the gonadal status of XX 9p-females have been limited to prepubertal girls. Patients 2 and 3 are the first females reported to have distal 9p monosomy and a normal puberty.

In this article, we report a case of maternal pancreatic carcinoma metastatic to the placenta. The baby is alive and well, with no evidence of tumor metastasis at the age of 1 year. We also present a comprehensive review of all maternal cancers with metastasis to the placenta and/or baby published in the English language.

A 42-year-old previously well man collapsed while holding his throat. Resuscitation was unsuccessful, and at autopsy, the most significant findings were limited to the larynx, pharynx, and anterior mediastinum where there were multinodular dark blue tumors. Within the larynx, the lower border of the tumor was well demarcated, not extending beyond the vocal cords. A separate large polypoidal tumor mass was attached to the right aryepiglottic fold by a thin fibrous stalk. Histologic examination revealed numerous large, thin-walled cavernous-type vascular spaces typical of a multifocal cavernous hemangioma. Death was due to asphyxiation from obstruction of the upper airways by a cavernous hemangioma of the larynx. Adult laryngeal hemangiomas are rare and are usually supraglottic. This case demonstrates that pedunculated laryngeal cavernous hemangiomas may remain occult until the initiation of an obstructive episode with sudden collapse and death. In such instances, the diagnosis must rely upon an autopsy examination.

Immature ganglion cells on rectal biopsy may be an indicator of transient functional immaturity of the intestine.

We present a case of recurrent massive perivillous fibrin deposition (MPVFD) in a patient with polymyositis (PM). Massive perivillous fibrin deposition occurred in 2 consecutive pregnancies: the 1st ended in an intrauterine death at 34 weeks of gestation, while the 2nd pregnancy resulted in a live-born baby at 32 weeks of gestation. The association between MPVFD and PM has been rarely reported in the literature in the form of case reports of single pregnancies.

BackgroundInfancy is the most common period for childhood death, including both neonatal deaths from obstetric or medical complications and sudden unexpected infant deaths. The weighing of organs at autopsy is an established process and is recommended in current protocols. However, minimal contemporary data is available regarding reference ranges for organ weights of infants.MethodsOrgan weight data for consecutive infant autopsies over a 14 year period performed at a single tertiary centre, including >1,000 cases, were examined in order to provide up to date reference ranges across this age range, using linear regression modelling and the standard LMS method.Results1,525 infant autopsies were analysed, of which 1,190 were subsequently used in the creation of linear regression models prior to performance of the LMS method. Organ weight charts were produced for the 5th, 25th, 50th, 75th and 95th centiles for the heart, lungs, liver, spleen, kidneys, pancreas, thymus gland and adrenal glands.ConclusionThis study provides the largest single centre contemporary dataset of infant autopsies allowing provision of up-to-date ‘normal’ ranges for all major organ weights across this age range.