PedAM

Pediatric Disease Annotations & Medicines


	poems syndrome

Disease ID	575
Disease	poems syndrome
Definition	A multisystemic disorder characterized by a sensorimotor polyneuropathy (POLYNEUROPATHIES), organomegaly, endocrinopathy, monoclonal gammopathy, and pigmentary skin changes. Other clinical features which may be present include EDEMA; CACHEXIA; microangiopathic glomerulopathy; pulmonary hypertension (HYPERTENSION, PULMONARY); cutaneous necrosis; THROMBOCYTOSIS; and POLYCYTHEMIA. This disorder is frequently associated with osteosclerotic myeloma. (From Adams et al., Principles of Neurology, 6th ed, p1335; Rev Med Interne 1997;18(7):553-62)
Synonym	crow fukase syndrome crow-fukase syndrome organomegalies, polyneuropathy organomegaly, polyneuropathy poem syndrome poems poems - polyneuropathy organomegaly endocrinopathy monoclonal and skin changes poems syndrome (disorder) poems syndrome [disease/finding] polyneuropathy organomegalies polyneuropathy organomegaly polyneuropathy organomegaly endocrinopathy monoclonal gammopathy and skin changes polyneuropathy organomegaly endocrinopathy monoclonal gammopathy and skin changes (disorder) polyneuropathy, organomegaly, endocrinopathy, m protein, and skin changes syndrome polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (poems) syndrome polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (poems) syndrome (disorder) polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome (disorder) syndrome, crow-fukase syndrome, poems syndrome, takatsuki's takatsuki syndrome takatsuki's syndrome
Orphanet	ORPHANET:2905
DOID	DOID:14039
UMLS	C0085404
MeSH	D016878
SNOMED-CT	SNOMEDCT_US_2016_09_01:79268002
Comorbidity	UMLS \| Disease \| Sentences' Count(Total Sentences:40) C0152025 \| polyneuropathy \| 6 C0442874 \| neuropathy \| 5 C0032131 \| plasmacytoma \| 4 C0018916 \| hemangioma \| 4 C0031117 \| peripheral neuropathy \| 2 C0024299 \| lymphoma \| 2 C0024419 \| macroglobulinemia \| 2 C0020538 \| hypertension \| 2 C0024419 \| waldenstrom macroglobulinemia \| 2 C0020542 \| pulmonary hypertension \| 2 C0034063 \| pulmonary edema \| 2 C0018916 \| hemangiomas \| 2 C0006666 \| calciphylaxis \| 2 C0040997 \| trigeminal neuralgia \| 1 C0026764 \| myeloma \| 1 C0026764 \| plasma cell myeloma \| 1 C0019163 \| hepatitis b \| 1 C0002726 \| amyloidosis \| 1 C1510415 \| osteosclerotic myeloma \| 1 C0270922 \| demyelinating polyneuropathy \| 1 C0019158 \| hepatitis \| 1 C0035078 \| renal failure \| 1 C0020541 \| portal hypertension \| 1 C0022660 \| acute renal failure \| 1 C0018916 \| haemangioma \| 1 C0270922 \| demyelinating neuropathy \| 1 C0018801 \| heart failure \| 1 C0042373 \| vascular disease \| 1 C0011570 \| depression \| 1 C0018802 \| congestive heart failure \| 1 C0033687 \| proteinuria \| 1 C0079744 \| diffuse large b-cell lymphoma \| 1 C0032131 \| solitary plasmacytoma \| 1 C0032131 \| plasmocytoma \| 1 C0024419 \| waldenstrom's macroglobulinemia \| 1 C0007787 \| transient ischemic attack \| 1 C0007787 \| transient ischemic attacks \| 1 C1136084 \| plasma cell dyscrasia \| 1 C0020255 \| hydrocephalus \| 1 C0079731 \| b-cell lymphoma \| 1
Curated Gene	(Waiting for update.)
Inferring Gene	(Waiting for update.)
Text Mined Gene	Entrez_id \| Symbol \| Score \| Resource(Total Genes:19) 973 \| CD79A \| DISEASES 1440 \| CSF3 \| DISEASES 3569 \| IL6 \| DISEASES 9172 \| MYOM2 \| DISEASES 3553 \| IL1B \| DISEASES 1001 \| CDH3 \| DISEASES 5921 \| RASA1 \| DISEASES 909 \| CD1A \| DISEASES 10158 \| PDZK1IP1 \| DISEASES 947 \| CD34 \| DISEASES 3039 \| HBA1 \| DISEASES 23583 \| SMUG1 \| DISEASES 959 \| CD40LG \| DISEASES 7422 \| VEGFA \| DISEASES 4099 \| MAG \| DISEASES 7124 \| TNF \| DISEASES 56034 \| PDGFC \| DISEASES 100423062 \| IGLL5 \| DISEASES 84000 \| TMPRSS13 \| DISEASES
Locus	(Waiting for update.)

Disease ID	575
Disease	poems syndrome
Integrated Phenotype	HPO \| Name(Total Integrated Phenotypes:46) HP:0100639 \| Erectile abnormalities HP:0001085 \| Papilledema HP:0001824 \| Weight loss HP:0004420 \| Arterial thrombosis HP:0000953 \| Hyperpigmentation of the skin HP:0001324 \| Muscle weakness HP:0002092 \| Pulmonary arterial hypertension HP:0004054 \| Sclerosis of hand bone HP:0001901 \| Polycythemia HP:0000135 \| Hypogonadism HP:0002202 \| Pleural effusion HP:0002716 \| Lymphadenopathy HP:0000771 \| Gynecomastia HP:0005523 \| Lymphoproliferative disorder HP:0004936 \| Venous thrombosis HP:0010702 \| Increased antibody level in blood HP:0001284 \| Areflexia HP:0000819 \| Diabetes mellitus HP:0000969 \| Edema HP:0001894 \| Thrombocytosis HP:0100925 \| Sclerosis of foot bone HP:0001820 \| Leukonychia HP:0000818 \| Abnormality of the endocrine system HP:0001072 \| Thickened skin HP:0001063 \| Acrocyanosis HP:0012531 \| Pain HP:0002111 \| Restrictive deficit on pulmonary function testing HP:0000998 \| Hypertrichosis HP:0012378 \| Fatigue HP:0002694 \| Sclerosis of skull base HP:0001271 \| Polyneuropathy HP:0004576 \| Sclerotic vertebral endplates HP:0000821 \| Hypothyroidism HP:0001541 \| Ascites HP:0011122 \| Abnormality of skin physiology HP:0001698 \| Pericardial effusion HP:0008207 \| Primary adrenal insufficiency HP:0001028 \| Hemangioma HP:0002747 \| Respiratory insufficiency due to muscle weakness HP:0100759 \| Clubbing of fingers HP:0003271 \| Visceromegaly HP:0100963 \| Hyperesthesia HP:0003401 \| Paresthesia HP:0004979 \| Metaphyseal sclerosis HP:0000870 \| Prolactin excess HP:0009125 \| Lipodystrophy
Text Mined Phenotype	HPO \| Name \| Sentences' Count(Total Phenotypes:30) HP:0000969 \| Dropsy \| 7 HP:0001271 \| Polyneuropathy \| 6 HP:0011857 \| Plasmacytoma \| 4 HP:0001028 \| Strawberry mark \| 4 HP:0000822 \| Hypertension \| 2 HP:0005508 \| Waldenstrom macroglobulinemia \| 2 HP:0100598 \| Pulmonary oedema \| 2 HP:0002665 \| Lymphoma \| 2 HP:0009830 \| Peripheral neuritis \| 2 HP:0002092 \| Pulmonary artery hypertension \| 2 HP:0001541 \| Ascites \| 2 HP:0001709 \| Complete heart block \| 1 HP:0012190 \| T cell lymphoma \| 1 HP:0000093 \| Proteinuria \| 1 HP:0000083 \| Renal insufficiency \| 1 HP:0010310 \| Chylothorax \| 1 HP:0002202 \| Pleural effusion \| 1 HP:0012115 \| Liver inflammation \| 1 HP:0002326 \| TIA \| 1 HP:0000238 \| Nonsyndromal hydrocephalus \| 1 HP:0012191 \| B-cell lymphoma \| 1 HP:0000716 \| Depression \| 1 HP:0030005 \| Capillary leak \| 1 HP:0100661 \| Trigeminal neuralgia \| 1 HP:0012722 \| Heart block \| 1 HP:0011034 \| Amyloid disease \| 1 HP:0001409 \| Portal hypertension \| 1 HP:0001919 \| Acute renal failure \| 1 HP:0007131 \| Acute demyelinating polyneuropathy \| 1 HP:0007133 \| Progressive peripheral neuropathy \| 1

Disease ID	575
Disease	poems syndrome
Manually Symptom	UMLS \| Name(Total Manually Symptoms:38) C1963220 \| pulmonary hypertension C1963154 \| renal failure C1963059 \| adrenal insufficiency C1512508 \| human herpesvirus 8 infection C1402315 \| vascular lesions C1384672 \| hypoparathyroidism C1334815 \| multicentric castleman's disease C1304511 \| glomeruloid hemangioma C1304506 \| microvenular hemangioma C1145670 \| respiratory failure C1136084 \| plasma cell dyscrasia C0878544 \| myocardiopathy C0442874 \| neuropathy C0346308 \| pituitary macroadenoma C0271051 \| macular edema C0220989 \| acquired partial lipodystrophy C0178703 \| hypertrophic osteoarthropathy C0152025 \| polyneuropathy C0038454 \| strokes C0038454 \| stroke C0038454 \| cerebral infarction C0037286 \| cutaneous tumor C0037284 \| skin lesions C0031117 \| peripheral neuropathy C0029464 \| osteosclerosis C0027121 \| inflammatory myopathy C0022658 \| renal disease C0022658 \| nephropathy C0018939 \| hematologic disorders C0018916 \| hemangiomas C0018916 \| angiomas C0018852 \| heavy chain disease C0017531 \| castleman's disease C0017531 \| castleman disease C0014130 \| endocrinopathy C0014130 \| endocrine diseases C0006666 \| calciphylaxis C0001206 \| acromegaly
Text Mined Symptom	UMLS \| Name \| Sentences' Count(Total Symptoms:11) C0152025 \| polyneuropathy \| 6 C0442874 \| neuropathy \| 5 C1304511 \| glomeruloid hemangioma \| 3 C0017531 \| castleman's disease \| 2 C0006666 \| calciphylaxis \| 2 C0020542 \| pulmonary hypertension \| 2 C0018916 \| hemangiomas \| 2 C0031117 \| peripheral neuropathy \| 2 C0035078 \| renal failure \| 1 C1136084 \| plasma cell dyscrasia \| 1 C0346308 \| pituitary macroadenoma \| 1

Manually Genotype(Total Text Mining Genotypes:0)
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All Snps(Total Genotypes:0)
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GWASdb Annotation(Total Genotypes:0)
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GWASdb Snp Trait(Total Genotypes:0)
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Mapped by lexical matching(Total Items:0)
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Mapped by homologous gene(Total Items:0)
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Chemical(Total Drugs:0)
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FDA approved drug and dosage information(Total Drugs:0)
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FDA labeling changes(Total Drugs:0)
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