Black urine due to urobilinogen in a patient with alcoholic pellagra.

OBJECTIVES: Systemic exposure to drugs, chemicals and foods can cause abnormally colored urine. Food exposures are typically benign, but urine discoloration due to chemicals or drugs may indicate a potentially dangerous condition. Discolored urine can also be caused by medical problems. This brief report reviews the laboratory findings leading to lactic acidosis and elevated urine urobilinogen in an alcoholic patient with pellagra. DESIGN AND METHODS: A 66-year-old male, found unconscious in his hotel room, was brought to the emergency department (ED). Upon arrival he had hypothermia, a diffuse rash and altered mental status. During ED evaluation, a urinary catheter was placed and demonstrated black urine. Medical history noted chronic alcoholism, malnutrition, and poor self-care. RESULTS: Evaluation in the hospital suggested that his rash and neurologic changes were a result of malnutrition and vitamin deficiency. A thorough biochemical workup demonstrated that elevated urobilinogen was likely causing the patient's black urine. Serum niacin concentration was undetectable. His dermatitis improved with multivitamins, thiamine, and niacin as well as topical steroids. His mental status returned to baseline and he was discharged to a skilled nursing facility following a brief hospital stay. CONCLUSIONS: The patient's abnormal laboratory results were explained by his alcoholism and poor nutrition. Furthermore, urine color returned to normal with decreased concentration of urobilinogen, after vitamin supplementation and supportive medical care.

Whole blood NAD and NADP concentrations are not depressed in subjects with clinical pellagra.

Population surveys for niacin deficiency are normally based on clinical signs or on biochemical measurements of urinary niacin metabolites. Status may also be determined by measurement of whole blood NAD and NADP concentrations. To compare these methods, whole blood samples and spot urine samples were collected from healthy subjects (n = 2) consuming a western diet, from patients (n = 34) diagnosed with pellagra and attending a pellagra clinic in Kuito (central Angola, where niacin deficiency is endemic), and from female community control subjects (n = 107) who had no clinical signs of pellagra. Whole blood NAD and NADP concentrations were measured by microtiter plate-based enzymatic assays and the niacin urinary metabolites 1-methyl-2-pyridone-5-carboxamide (2-PYR) and 1-methylnicotinamide (1-MN) by HPLC. In healthy volunteers, inter- and intra-day variations for NAD and NADP concentrations were much lower than for the urinary metabolites, suggesting a more stable measure of status. However, whole blood concentrations of NAD and NADP or the NAD:NADP ratio were not significantly depressed in clinical pellagra. In contrast, the concentrations of 2-PYR and 1-MN, expressed relative to either creatinine or osmolality, were lower in pellagra patients and markedly higher following treatment. The use of the combined cut-offs (2-PYR <3.0 micromol/mmol creatinine and 1-MN <1.3 micromol/mmol creatinine) gave a sensitivity of 91% and specificity of 72%. In conclusion, whole blood NAD and NADP concentrations gave an erroneously low estimate of niacin deficiency. In contrast, spot urine sample 2-PYR and 1-MN concentrations, relative to creatinine, were a sensitive and specific measure of deficiency.

Low and deficient niacin status and pellagra are endemic in postwar Angola.

BACKGROUND: Outbreaks of pellagra were documented during the civil war in Angola, but no contemporary data on the incidence of pellagra or the prevalence of niacin deficiency were available. OBJECTIVE: The objective was to investigate the incidence of pellagra and the prevalence of niacin deficiency in postwar Angola and their relation with dietary intake, poverty, and anthropometric status. DESIGN: Admissions data from 1999 to 2004 from the pellagra treatment clinic in Kuito, Angola, were analyzed. New patients admitted over 1 wk were examined, and urine and blood samples were collected. A multistage cluster population survey collected data on anthropometric measures, household dietary intakes, socioeconomic status, and clinical signs of pellagra for women and children. Urinary excretion of 1-methylnicotinamide, 1-methyl-2-pyridone-5-carboxymide, and creatinine was measured and hemoglobin concentrations were measured with a portable photometer. RESULTS: The incidence of clinical pellagra has not decreased since the end of the civil war in 2002. Low excretion of niacin metabolites was confirmed in 10 of 11 new clinic patients. Survey data were collected for 723 women aged 15-49 y and for 690 children aged 6-59 mo. Excretion of niacin metabolites was low in 29.4% of the women and 6.0% of the children, and the creatinine-adjusted concentrations were significantly lower in the women than in the children (P < 0.001, t test). In children, niacin status was positively correlated with the household consumption of peanuts (r = 0.374, P = 0.001) and eggs (r = 0.290, P = 0.012) but negatively correlated with socioeconomic status (r = -0.228, P = 0.037). CONCLUSIONS: The expected decrease in pellagra incidence after the end of the civil war has not occurred. The identification of niacin deficiency as a public health problem should refocus attention on this nutritional deficiency in Angola and other areas of Africa where maize is the staple.

Niacin metabolite excretion in alcoholic pellagra and AIDS patients with and without diarrhea.

OBJECTIVE: Malnourished patients with the acquired immunodeficiency syndrome (AIDS) can develop pellagra-like manifestations such as dermatitis, diarrhea, and dementia; therefore, we tested the hypothesis that patients with AIDS and diarrhea would have niacin depletion. This study compared 24-h urine excretion of N1-methyl-nicotinamide (N1MN) among patients with pellagra and patients with AIDS who did and did not have diarrhea. METHODS: Three groups were studied: G1 (patients with AIDS and diarrhea, n = 5); G2 (patients with AIDS and no diarrhea, n = 7), and G3 (patients with alcoholic pellagra and without the human immunodeficiency virus, n = 8). Diarrhea was defined as the production of at least three liquid stools per day over 3 to 5 d. Studies included mucosal intestinal biopsy, malabsorption tests, detection of parasites in stool, and serum albumin measurements. Semiquantitative food-frequency questionnaire, anthropometry, and daily urinary N1MN excretion were also determined. Groups were matched in relation to age, sex, presence of parasites in stool, and intestinal absorption results. RESULTS: G1 had normal intestinal examination by light microscopy and no parasites in stools. G2 group showed lower levels of serum albumin (2.6 +/- 0.3 g/dL) when compared with G1 (3.4 +/- 0.3 g/dL) and G3 (3.1 +/- 0.7 g/dL). Except for patients with pellagra, groups met their energy requirements. Patients in G3 (0.013, 0.01-0.081 mg/dL) and G1 (0.062, 0.001-0.33 mg/dL) excreted smaller amounts of N1MN in urine than did those in G2 (0.63, 0.02-2.9 mg/dL). CONCLUSIONS: Patients with AIDS and diarrhea excreted less N1MN in urine than did those without diarrhea. These patients may have an impaired niacin nutritional status, possibly associated with increased metabolic needs.

[The urinary metabolites of niacin during the course of pellagra]. / Les métabolites urinaires de la niacine au cours de la pellagre.

The three main metabolites of niacin have been measured in urine of 10 Mozambican women living in refugee camps in Malawi and displaying clinical symptoms of pellagra. This study, in which a control group was included, showed that the ratio 6PYR/N1MN is well correlated to the occurrence of clinical symptoms of niacin deficiency and constitutes a reliable indicator of vitamin PP status in subjects at risk of this deficiency.

Pellagra in a patient with an eating disorder.

A case of pellagra is described that occurred in a patient with an eating disorder and who presented with marked photosensitivity and diarrhoea. We found urinary 5-hydroxy-indole-acetic acid to be low and suggest that this may be a useful screening test. To our knowledge this is only the second reported case of pellagra associated with an eating disorder.

Plasma amino acid patterns in alcoholic pellagra patients.

Plasma amino acid concentrations in 33 male alcoholic patients with pellagra (age 20-68 years) were compared with those in 17 healthy male subjects (age 20-45 years). Pellagra diagnosis was made on the basis of the typical clinical skin picture, and low urinary excretion of N'methylnicotinamide and N'methyl-2-pyridone-5-carboxamide (reduced by 70 and 80%, respectively, compared with controls). There were significant differences in body mass index, creatinine/high index and serum albumin between the two groups, indicating that besides pellagra the alcoholic patients had some degree of malnutrition. Of 17 plasma amino acids measured, the following had significantly lower concentrations in the pellagrins: tryptophan (3.65 vs 5.93 mumol/dl, pellagrin vs control), isoleucine (6.31 vs 11.13), leucine (11.54 vs 24.19), lysine (16.25 vs 34.47), methionine (2.61 vs 4.22), phenylalanine (5.71 vs 9.23), threonine (13.29 vs 26.81), valine (17.60 vs 41.06), alanine (42.54 vs 70.87), arginine (5.87 vs 10.09), tyrosine (5.57 vs 9.30). Glutamic acid was significantly higher in the pellagrins (18.45 vs 9.49). There was no difference between the groups of aspartic acid, glycine, histidine, proline and serine concentrations. It is concluded that pellagra is an important factor influencing the amino acid profiles in these patients. This finding should be taken into account when using plasma amino acid levels to assess the clinical status of the pellagrin.

Quantificaçäo de uro e coproporfirinas urinárias em fotodermatoses / Quantification of urinary uro coproporphyrins in photodermatitis

Conhecendo que as porfirinas (uroporfirina), quando acumuladas na pele agem como cromóforos, que interagindo com a energia luminosa produzem lesöes fotoquímicas que se manifestam por eritema, edema e bolhas em áreas expostas a luz, e ainda que o mecanismo de formaçäo destas lesöes fotossensíveis näo está bem esclarecido, foram quantificadas as uro e coproporfirinas urinárias em fotodermatoses, excluindo as porfirias, especialmente, porfiria cutânea tardia, na qual a manifestaçäo cutânea está relacionada a níveis elevados de porfirinas, principalmente de uroporfirina. Estudou-se os níveis destas porfirinas em 58 pacientes subdivididos em grupos, segundo a fotodermatose: 1. 21 com pelagra; 2. 15 com forodermatite medicamentosa; 3. 14 transplantados renais (pseudoporfiria?); 4. cinco com lúpus eritematoso cutâneo/sistêmico e 5. três com lucite. Observou-se aumento de porfirinas em 17% do total dos doentes, sendo encontrado níveis aumentados de uroporfirinas urinárias nos grupos 1 e 2. Na pelagra houve aumento em 33% (7/2) dos doentes e em pacientes com fotodermatite medicamentosa 20% (3/15). Os resultados sugerem que as porfirinas urinárias, principalmente as uroporfirinas, podem estar envolvidas na patogênese da pelagra e da fotodermatite medicamentosa, talvez por acometimento da funçäo hepática provocado pelo etilismo crônico, hábito comum em doentes de pelagra, ou uso de medicamentos