Mullerian duct remnant fibroids: a case report

Uterine fibroids are the most common benign tumours in women and are particularly common in women of African descent. Mullerian dysgenesis is uncommon and the association of a pelvic mass rare. Only three cases of the association of this syndrome with fibroids have been described and none of these has been in a woman of African descent.(Au)

Significance of fever in Jamaican patients with homozygous sickle cell disease

OBJECTIVE: To investigate the cause and outcome of high fever in Jamaican children with homozygous sickle cell disease. DESIGN: Retrospective review of febrile episodes in a three year period (1 September 1993 to 31 August 1996). SETTING: Sickle Cell Clinic, an outpatient clinic in Kingston run by the Medical Research Council Laboratories (Jamaica). PATIENTS: Patients with homozygous sickle cell disease under 17 years of age presenting with an anxillary temperature o 39.0§c (102.4§F). MAIN OUTCOME MEASURES: Diagnosis, death. RESULTS: There were 165 event in 144 patients (66 (45.8 percent) boys) with a median age of 6.1 years. Bacteraemia was found in 10 (6.1 percent) events (three Streptococcus pneumoniae, two Haemophilus influenzae type b, two Salmonella sp, one Escherichia coli, one Enterobactor sp, and one Acinetobacter sp), and urinary tract infections in four (2.4 percent). All cultures of cerebrospinal fluid were sterile. Acute chest syndrome occured in 36 (21.8 percent) events. A painful crisis was associated with 45 (27.3 percent) events and was the only pathology identified in 20 events (12.1 percent). Hospital admission was necessary in 66 cases including all those with bacteraemia and 31 with acute chest syndrome. There were two deaths: a 5 year old boy with septic shock associated with H influenzae septicaemia, and a 3 year old boy with the acute chest syndrome. CONCLUSIONS: Painful crisis and acute chest syndrome were the most common complications associated with high fever, but other important associated features included bacteraemia and urinary tract infection. Enteric Gram negative organisms accounted for 50 percent of positive blood cultures. (AU)

CT appearance of the rapunzel syndrome: an unusual form of bezoar and gastrointestinal obstruction

The CT appearance oa an unusual type of trichobezoar which cause the Rapunzel syndrome are described. This syndrome is characterized by intestinal obstruction secondary to a bezoar possessing a tail which extends to or beyond the ileocecal valve. Most cases of bezoar are diagnosed with plain films or barium meal, but CT may be requested in patients who present with abdominal masses. The Rapunzel syndrome may be diagnosed with a combination of the CT findings and the history.(AU)

Treatment effects in trichuris dysentery syndrome

Heavy infection with geohelminth trichuris trichiura causes the Trichuris dysentery syndrome (TDS). Growth retardation and anaemia are charcteristic of TDS and both are associated with poor development. We have examined the growth and developmental responses to treatment in 19 children aged 27-84 months with TDS. Development levels (DQ) were measured with the Griffiths mental development scales. Compared with a control group matched for age, gender and neighbourhood, the TDS children initially had serious deficits in DQ (24 points, p < 0.001). After a year of anthelmintic treatment, the TDS children showed improvement in locomotor development (p<0.001) compared with the controls. The TDS children also had initial deficits in height-for-age, weight-for-height, mid upper arm circumference and haemoglobin levels. They caught up rapidly in indices of wasting (weight-for-height and mid-upper arm circumference) and showed steady improvement in height-for-age and haemoglobin levels. Catch-up in height was comparable to that of children recovering from coeliac disease. The importance of continuing prevention after initial treatment is highlighted (AU)

Pulmonary artery pressure and the acute chest syndrome in homozygous sickle cell disease

OBJECTIVE; to investigate whether attacks of acute chest syndrome affected pulmonary artery pressure in patients homozygous for sickle cell disease. MAIN OUTCOME MEASURES: Pulmonary artery pressure, assessed by non-invasive echocardiographic techniques. PATIENTS; 20 patients with homozygous sickle cell disease with a history of at least six episodes of acute chest syndrome and in 20 age, sex, and height matched controls with homozygous sickle cell disease without a history of acute chest syndrome. RESULTS: There was no difference in any of the echocardiographic or Doppler indices between these two groups. CONCLUSIONS: Repeated attacks of acute chest syndrome by the mean age of 12 ( range eight to 16 ) years have not had a discernible effect upon pulmonary artery pressure (AU)

Solitary rectal ulcer syndrome

Solitary rectal ulcer syndrome (Rectal prolapse syndrome) is an uncommon and imperfectly understood entity which, however, should be included in the differential diagnosis of ano-rectal symptoms. This article reports on the nine cases so far diagnosed at the University Hospital of the West Indies (UHWI), and offers a brief review of the condition (AU)

J type diabetes revisited

J type diabetis is grouped as a subtype of type III or malnutrition-related diabetes, known as protein deficient pancreatic diabetis, (PDPD). J type diabetes has not been reported recently, but a clinical picture called phasic insulin-dependent diabetes mellitus (PIDDM) has been elaborated in Jamaica, the same home country of PDRD and appears to be a "formes frustes" syndrome. The following comparative studies were performed on a group of diabetic patients and normal controls: insulin receptor binding; renal, hepatic, and pancreatic function; and abdominal ultrasonography. The results show a considerbaly decreased white and red blood cell binding to insulin (P<0.05), extensive kidney damage (P<0.05), and increased pancreatic echogenicity in PIDDM, supporting a separate identity of this latter syndrome from types I and II diabetes mellitus. Also, the features of relative insulin resistance, absence of ketosis even in the presence of severe hyperglycemia, and intermittent insulin requirement suggests that PIDDM, J type diabetes, and PDPD are one and the same syndrome. (AU)

The POEMS syndrome

The POEMS syndrome is an unusual disorder manifesting primary as peripheral sensorimotor neuropathy in association with a monoclonal gammopathy. The importance of its recognition is that significant clinical improvement may result from localization and treatment of the underlying plasma cell tumour. AU)

Histopathology and immunohistochemistry of the caecum in children with the Trichuris dysentery syndrome

Caecal biopsy specimens from Jamaican children with the Trichuris dysentery syndrome (TDS) and age matched Jamaican controls were investigated by immunohistochemistry and by light microscopy. Biopsy specimens from all children (with TDS and controls) showed a mild to moderate increase in inflamatory cells. Except in the vicinity of the worm, where the epithlium was flattened, there was no other epithelial abnormality. Compared with controls, children with TDS had increased IgM lamina propria plasma cells and decreased intaepithelial T cells. There was also an increase in crypt epithelial cells proliferation. Lamina propia T cells (both activated and non-activated) were no more common in children with the Trichuris syndrome than controls. Epithelial cell HLA-DR and VLA-1 expression (which are increased in other colitides) were the same in both groups. Despite the presence of large worm burdens and chronic dysentery, therefore, only minor changes were seen in the caecal mucosa of children with TDS. (AU)

Toxic hypoglycemic syndrome - Jamaica, 1989-1991

In January and February 1991, the health officer in the parish of St. Ann, Jamaica, received reports of eight persons with toxic hypoglycemic syndrome (THS), an illness associated with consumption of unripe ackee fruit and, possibly, renta yam; two cases were fatal. On July 25, the Jamaican Ministry of Health (JMH) contacted CDC for assistance in investigating the continued occurrence of THS: the collaborative JMH and CDC epidemiologic investigation focused on characterizing the epidemiology of THS in Jamaica and assessing the role of ackee fruit, renta yams, and other factors. (AU)

Multiple lentigines syndrome in Dominica

Multiple Lentigines Syndrome with variable penetrance is described in a Dominican family mild skeletal manifestations, including arachnodactyly, were present with low intellect in the propositus. The syndrome is of importance because of the association with various cardiac abnormalities and deafness. It occurs in black people though the lentigines may be difficult to recognise, and relatives without lentigines may have cardiac abnormalities (AU)

The syndrome of insulin resistance, acanthosis nigricans and an autoimmune disorder in diabetes mellitus treated with immunosuppressants

Insulin resistance in diabetes mellitus associated with acanthosis nigricans and an underlying autoimmune disorder has, so far, been described in the world literature only in 25 patients. This is another such patient to be described, and antibodies were demonstrated against the insulin receptor sites in monocytes. The insulin refractoriness was treated successfully by immunosuppressant therapy and, after one year, the autoimmune condition subsided. The patient continues to do well and achieved euglycaemic control on a diabetic diet alone (AU)

Hypohidrotic ectodermal dysplasia (Christ-Siemans-Touraine Syndrome) in siblings

Hypohidrotic ectodermal dysplasia (Christ-Siemans-Touraine Syndrome) is reported in three male siblings. The patients gave a history of repeated hospital admissions from the age of 4 months for hyperpyrexia and seizures. They were being followed up for "febrile convulsions". Since the condition is familial, each member of the family should be carefully investigated for hair, teeth and sweating abnormalities in order to detect both homozygous and heterozygous forms of the condition (AU)

Conn's syndrome: case report and a review of the syndrome of primary aldosteronism (SOPA)

This report describes the investigation and treatment of a 30-year-old woman with Conn's Syndrome caused by an aldosterone-producing right adrenocortical adenoma associated with the classic triad of hypertension, hypokalaemia, and metabolic alkalosis. Removal of the tumour resulted in the reversal of these abnormalities. To the best of our knowledge, this is the first case of Conn's Syndrome in the West Indies. It is hoped that, with the presently improved diagnostic facilities in the region, this report will stimulate interest in finding other cases of this rare but important and treatable cause of secondary hypertension (AU)

Retinal changes in sickle cell/hereditary persistence of fetal haemoglobin syndrome

We describe for the first time retinal changes in sickle cell/hereditary persistence of fetal haemoglobin syndrome, which is a rare and benign disorder. The changes are qualitively similar to retinal disease seen with sickle haemoglobin and sickle C haemoglobin, but are mild (AU)

Rastafarianism and the vegans syndrome

Low serum vitamin B12 concentrations are common in vegans. We report the first account of the syndrome in members of the Rastafarian cult, which started in Jamaica more than 40 years ago (AU)

Sickle cell-Hb Lepore Boston syndrome: uncommon differential diagnosis to homozygous sickle cell disease

Two siblings assumed on the basis of clinical and hematological evidence to have homozygous sickle cell (SS) disease were found to have a mother without sickle hemoglobin. Subsequent investigation and hemoglobin structural studies indicated the diagnosis to be sickle cell-Hb Lepore Boston syndrome. This syndrome generally manifests clinically significant sickle cell disease, and this genotype should be borne in mind in apparent SS disease where a parent without sickle hemoglobin is discovered.(AU)

West Indian amblyopia

A series of 21 patients admitted to St. Thomas' Hospital, Medical Ophthalmology Unit, with a diagnosis of West Indian or West African amblyopia is reported. Patients were investigated for haematological, biochemical, serological, and radiological abnormalities and particular attention was paid to dietary history. Patients admitted in recent years also underwent neurophysiological investigations. No definite correlation between visual loss and dietary or family history was found, and there was no evidence that the improvement in vision which occured in just under half the patients on follow-up was related to treatment with hydroxocobalamin or multivitamins. Visual-evoked response in 4 patients showed a prolonged latency suggesting optic nerve demyelination, while in only one case was the electroculogram definitely subnormal. These findings contrast with those in "toxic" amblyopias and suggest that the syndrome of West Indian amblyopia may be due to bilateral optic nerve demyelination of unknown aetiology rather than the effect of toxic substances or nutritional deficiency on the retina. (AU)