Primary malnutrition - can we always tell?

OBJECTIVE: To determine if there was a decrease in admissions for primary malnutrition and an increase in those for initially undiagnosed secondary malnutrition over the period January 1, 1990 to October 31, 1999 at the Tropical Metabolism Research Unit (TMRU). DESIGN AND METHODS: A retrospective review of all admissions, for treatment of malnutrition, to TMRU using admission books and patient records was done. Children were classified with secondary malnutrition if diagnosed after admission with illnesses known to cause malnutrition. Those known to have such conditions on admission or who were over 10 years old were eliminated. Nutritional diagnosis according to the Wellcome classification, age at presentation, sex and birth weight were reviewed. RESULLTS: A total of 411 patients were admitted to the TMRU during this 10-year period and 23 of these had secondary malnutrition have increased, especially since 1998. Children with secondary malnutrition were usually marasmic (78 percent), had low normal birth weight (2.8kg) and presented at a later age (15.3 months). The most common secondary cause of malnutrition was HIV infection (56 percent). CONCLUSION: There has been an increase in initially undiagnosed secondary malnutrition seen at TMRU over the last ten years even with a decrease in admissions for primary malnutrition. This may be due to subtlety of presentation and indicates a need for increased vigilance in assessment by health professionals in order to optimize management.(Au)

The changing face of paediatrics in the English-speaking Caribbean

Childhood mortality and morbidity patterns in the English-speaking Caribbean have changed significantly over the past 40 years. Acute respiratory illness, physical injury and conditions originating in the perinatal period have replaced malnutrition, gastroenteritis and other infectious diseases as major causes of illness and death in Caribbean children. Although population growth has slowed down, about one-third of the population of the English-speaking Caribbean remains under the age of 15 years. Infant mortality rates have also fallen but the major contributor to this decline has been a reduction in post-neonatal deaths. The decrease in mortality and morbidity from infectious diseases has led to a prominence of disorders originating in the perinatal period, psychosocial problems and chronic childhood disorders. Adverse economic conditions are held culpable for the re-emergence of protein energy malnutrition (PEM) and pulmonary tubercolosis in some territories. There is an urgent need to focus attention on the areas of perinatal and adolescent health, childhood disability, accidental and non-accidental injury, sexual abuse and human immunodeficiency virus (HIV) infection. Immunization programmes also require continuing support and expansion. These tasks cannot be accomplished without meaningful long term investment of financial and human resources in the health and educational services of the region (AU)

Tropical spastic paraparesis ocurring in HLTV-1 associated infective dermatitis: report of two cases

An association between HTLV-1 infection and infective dermatitis(ID), a relapsing eczematous condition of Jamaican children, was reported in 1990. These patients are at a risk of developing other known HTLV-1 related diseases. We have observed the development of HTLV-1 associated myelopathy/tropical spastic paraparesis im two patients, ages 14 and 35 years, who were diagnosed with ID at ages 2 and 10 years, respectively. Infective dermatitis of children serves as an early marker of HTLV-1 infection and may predict later development of either the malignant outcome, adult T-cell leukaemia/lymphoma or the neurologic manifestation HAM/TSP among adult carriers of HTLV-1 infection(AU)

Parkinsonian-like tremors in the recovery phase of kwashiorkor

Kwashi shakes is described in a 17-month-old Jamaican male infant. This is the first reported case seen at the Tropical Metabolism Research Unit at the University of the West Indies, Mona, Jamaica an the first documented case in the West Indian literature (AU)

Myoclonic encephalopathy in Jamaican children

Four children between the ages two and five years presented at the University Hospital of the West Indies between December 1979 and June 1983 and were diagnosed as having acute myoclonic encephalopathy. All had the typical clinical picture of acute onset myoclonus simulating cerebellar ataxia, and opsoclomus. Neuroblastoma was not demonstrated in any of these patients. One had a protracted illness with a relapsing course. Remission was not induced by dexamethasone in two patients. Complete recovery was seen in only one patient. This is the first known report of acute myoclonic encephalopathy in the Caribbean. The condition, although rare, is important to recognize as remisssion may be induced by adrenocoticotrophic hormone or corticosteroids (AU)

Subacute sclerosing panencephalitis in Jamaica

The clinical features, outcome, electroencephalographic (EEG) and laboratory findings in 12 children with subacute sclerosing panencephalitis are described. The diagnosis was made on the clinical features, the characteristic EEG pattern and the detection of measles complement-fization antibodies in the spinal fluid and serum. The autopsy findings in 2 of 6 patients who died were typical of subacute sclerosing panenecephalitis. This report suggests that the incidence of the condition is high in Jamaica. It is recommended that strenuous efforts be made to improve the measles immunisation status of children and that a registry for the disease be established in the island (AU)

Cerebro-vascular complications of sickle cell disease in Jamaica - abstract

Homozygous sickle cell disease is an uncommon but important cause of hemiparesis and subarachnoid haemorrhage. A 24-year review showed that 42 cases of hemiparesis and 9 cases of subarachnoid haemorrhage had been seen at the University Hospital of the West Indies. All living patients (32) were examined in 1984 for residual defects. Twenty-two males and 20 females, with a median age of 7 years (range 8 months to 36 years) had had a stroke. Most (69 percent) were under 10 years of age at the time of the first stroke. No haematological or clinical predictor for stroke was identified, although transient ischaemic episodes prior to the onset of a complete stroke were seen in one 13-year-old. Recurrence clustered within the first 30 months with 10 of the 17 cases occurring in the first year. Contralateral occurrences were commonest (14) and resulted in death in 13. Subarachnoid haemorrhage was seen in 5 males and 4 females, aged 6 to 57 years (median 19 years), and 3 patients died. At re-evaluation of 32 patients, 8 had complete recovery, 20 residual hemiplegia and 4 quadriplegia. It is concluded that cerebro-vascular accident, though uncommon in sickle cell disease, affects a young age group and is associated with devastating consequences (AU)

Glucose 6 phosphate dehydrogenase deficiency and neonatal jaundice in Jamaica

Glucose-6- phosphate dehydrogenase (G6PD) deficiency was detected in 16 (60.6 percent) of a group of23 neonates who had unexplained moderate or severe jaundice. This proportion is significantly more than the 9.4 percent expected in Jamaican neonates who are not moderately or severely jaundiced (P<0.003), and significantly more than the 12.6 percent observed or the 21.0 percent expected in older Jamaican children and adults (P<0.003). Phenobarbitone therapy and phototherapy reduced the need for exchange transfusion but this was necessary in eight patients. Two babies developed kernicterus and one died. On the other hand, only two of 21 neonates who were identified as G6PD deficient at birth subsequently became moderately or severely jaundiced, and this could be attributed to other causes in both cases. These findings indicate that apparently spontaneous neonatal juandice is important in infants who have the G6PD A-enzyme. However, the jaundice is probably precipitated by unknown factors to which the G6PD deficient neonate is more susceptible than the infant who is not G6PD deficient. There is also a slightly increased incidence of G6PD deficiency in neonates who develop jaundice because of ABO or Rh(D) iso-immune disease, infection or prematurity (AU)

Ocular findings in children with sickle cell haemoglobin C disease in Jamaica

The ophthalmological findings in 54 Jamaican children with SC disease are reported. Evidence of peripheral retinal vessel disease was present in 94 percent and retinitis proliferans in 11 percent. Retinitis proliferans was noted as early as 7 years of age and was more common in patients with high haemoglobin levels. There was an unequivocal progression in severity of retinopathy in eight out of eleven children examined 2 years previously. The pathological processes leading to sickle cell proliferative retinopathy are well established in childhood and attempts at prophylactic therapy should be instituted at an early age. (Summary)

Glucose-6-phosphate dehydrogenase (G6PD) deficiency and neonatal jaundice in Jamaica - abstract

The relationship between G6PD deficiency and neonatal jaundice in Jamaica was examined in two ways. A survey of 287 neonates born at the University hospital included clininical examination, and laboratory investigations designed to detect G6PD deficiency (methylene blue screening test and spectrophotometric assay). ABO and Rh hemolytic disease of the newborn, and other causes of neonatal jaundice. Hemoglobin concentrations, hematocrutsm reticulocytes and plasma bilirubin concentrations were also estimated. The mean plasma bilirubin concentration was higher on day 3 in G6PD deficiency neonates (4.2 mgm percent) than in normal neonates (3.6 mgm percent) and was markedly increased in those in whom heterospecificity was associated with G6PD deficiency (9.4 mgm percent). The mean hemoglobin concentration was also lower (17.8 gm percent) in the latter group than in normal neonates (19.2 gm percent). Secondly, 23 moderately or severly jaundiced babies were investigated to determine the cause of their jaundice. In 15, this was due to ABO or Rh hemolytic disease, prematurity or infection. The remainderr were G6PD deficient but 2 were also premature. This left 6 neonates with unexplained moderate or severe jaundice, all of whom were G6PD deficient. The findings therefore suggest that G6PD deficiency may be an important cause of neonatal jaundice in Jamaica (AU)

Clinical features of homozygous SS disease in Jamaican children

The clinical features in the first 12 years of life of 100 patients with homozygous SS anaemia are described. The majority of patients presented before age 2 years. The earliest diagnosis was made at 3 months of age. The hand foot syndrome, painful crisis and megaloblastic change were early manifestations of disease, whereas leg ulceration did not occur until beyond the age of 6 years. No examples of haemolytic crises were seen. Growth did not appear to be retarded by Jamaican standards. Death occurred in 2 cases (AU)

Sickle cell Hb oarab disease - abstract

An infant presented with megaloblastic anaemia of folate deficiency complicating a haemolytic anaemia with hepatosplenomegaly. At first, the laboratory investigations indicated Hb SC disease but, as it is very unusual to see such severe clinical effects in this haemoglobinopathy, especially in infancy, further investigations were carried out which demonstrated a haemoglobin electrophoretically similar to Hb C but having certain distinguishing characteristics. This Hb was compared with Hb C and Hb E on various electrophoretic media. The tryptic peptide maps (prepared at the Institute for Anthropology, Leiden, Netherlands, by W. de Jong) showed an abnormality in beta Tp XIII and amino acid analysis further revealed that lysine had replaced the normal glutamic acid at the 121st residue in the beta chain. This substitution has been previously described for Hb O Arab. In the helical notation of Perutz this position, in the peptide chain, lies between the G and H helices, GH4. The glutamyl residue is invariant at this site in all human haemoglobin chains. Four mutations have been described at this site. One in the gamma chain, one in the alpha chain and two in the beta chain. The other one in the B chain is Hb D Punjab, and this also causes a more severe type of haemoglobinopathy when inherited together with Hb S. Another infant, presenting in a similar way, was found to have the same defect, Hb S with Hb O Arab. The mother of this second case has Hb O-Thalassaemia. A search was then carried out in patients who had previously been diagnosed as SC disease. Two further cases of Hb SO disease were found, making four families in all. The clinical features of Hb SO disease appear to be more like those of homozygous sickle cell desease than SC disease. There are more sickled cells in the blood, a lower haematocrit and haemoglobin level, a greater shift in oxygen dissociation curve and a shorter red cell survival. Hb O Arab is apparently not uncommon in Jamaica and all cases of suspected Hb SC disease who have a clinical course more like SS disease should be further investigated (AU)