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Pancreatite tropical
ORPHA:103918CID-10 · K86.1CID-11 · DC32.5OMIM 608189DOENÇA RARA

A pancreatite tropical é uma doença rara do pâncreas que começa na juventude e ocorre principalmente em países tropicais em desenvolvimento. Ela é caracterizada por uma inflamação crônica (de longa duração) do pâncreas que não é causada por álcool, manifestando-se com dor na barriga, fezes gordurosas e uma condição do pâncreas que envolve fibrose e formação de pedras. Também é comumente associada ao surgimento de pedras no pâncreas e câncer de pâncreas, com uma frequência muito maior do que a observada na pancreatite crônica comum.

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Introdução

O que você precisa saber de cara

📋

A pancreatite tropical é uma doença rara do pâncreas que começa na juventude e ocorre principalmente em países tropicais em desenvolvimento. Ela é caracterizada por uma inflamação crônica (de longa duração) do pâncreas que não é causada por álcool, manifestando-se com dor na barriga, fezes gordurosas e uma condição do pâncreas que envolve fibrose e formação de pedras. Também é comumente associada ao surgimento de pedras no pâncreas e câncer de pâncreas, com uma frequência muito maior do que a observada na pancreatite crônica comum.

Publicações científicas
98 artigos
Último publicado: 2025 Jan 1

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
Unknown
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Worldwide
Início
Childhood
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: K86.1
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🫃
Digestivo
9 sintomas
📏
Crescimento
3 sintomas

+ 5 sintomas em outras categorias

Características mais comuns

100%prev.
Calcificação pancreática
Muito frequente (99-80%)
90%prev.
Dor epigástrica
Muito frequente (99-80%)
90%prev.
Pancreatite crônica calcificante
Muito frequente (99-80%)
55%prev.
Náusea
Frequente (79-30%)
55%prev.
Morfologia anormal do ducto pancreático
Frequente (79-30%)
55%prev.
Diabetes insulino-dependente, mas resistente à cetose
Frequente (79-30%)
17sintomas
Muito frequente (3)
Frequente (3)
Ocasional (6)
Sem dados (5)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 17 características clínicas mais associadas, ordenadas por frequência.

Calcificação pancreáticaPancreatic calcification
Muito frequente (99-80%)100%
Dor epigástricaEpigastric pain
Muito frequente (99-80%)90%
Pancreatite crônica calcificanteChronic calcifying pancreatitis
Muito frequente (99-80%)90%
NáuseaNausea
Frequente (79-30%)55%
Morfologia anormal do ducto pancreáticoAbnormal pancreatic duct morphology
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2025
Total histórico98PubMed
Últimos 10 anos11publicações
Pico20163 papers
Linha do tempo
2025Hoje · 2026🧪 2009Primeiro ensaio clínico📈 2016Ano de pico
Publicações por ano (últimos 10 anos)

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Genética e causas

O que está alterado no DNA e como passa nas famílias

Genes associados

2 genes identificados com associação a esta condição.

CTRCChymotrypsin-CMajor susceptibility factor inTolerante
FUNÇÃO

Regulates activation and degradation of trypsinogens and procarboxypeptidases by targeting specific cleavage sites within their zymogen precursors. Has chymotrypsin-type protease activity and hypocalcemic activity

LOCALIZAÇÃO

VIAS BIOLÓGICAS (1)
Developmental Lineage of Pancreatic Acinar Cells
MECANISMO DE DOENÇA

Pancreatitis, hereditary

A disease characterized by pancreas inflammation, permanent destruction of the pancreatic parenchyma, maldigestion, and severe abdominal pain attacks.

EXPRESSÃO TECIDUAL(Tecido-específico)
Pâncreas
3340.0 TPM
Baço
0.9 TPM
Testículo
0.7 TPM
Cervix Ectocervix
0.7 TPM
Sangue
0.7 TPM
OUTRAS DOENÇAS (2)
hereditary chronic pancreatitistropical pancreatitis
HGNC:2523UniProt:Q99895
SPINK1Serine protease inhibitor Kazal-type 1Major susceptibility factor inModerado
FUNÇÃO

Serine protease inhibitor which exhibits anti-trypsin activity (PubMed:7142173). In the pancreas, protects against trypsin-catalyzed premature activation of zymogens (By similarity) In the male reproductive tract, binds to sperm heads where it modulates sperm capacitance by inhibiting calcium uptake and nitrogen oxide (NO) production

LOCALIZAÇÃO

Secreted

VIAS BIOLÓGICAS (1)
Developmental Lineage of Pancreatic Acinar Cells
MECANISMO DE DOENÇA

Pancreatitis, hereditary

A disease characterized by pancreas inflammation, permanent destruction of the pancreatic parenchyma, maldigestion, and severe abdominal pain attacks.

EXPRESSÃO TECIDUAL(Tecido-específico)
Pâncreas
4245.9 TPM
Estômago
256.0 TPM
Intestino delgado
62.6 TPM
Cólon transverso
37.4 TPM
Rim - Córtex
25.2 TPM
OUTRAS DOENÇAS (2)
hereditary chronic pancreatitistropical pancreatitis
HGNC:11244UniProt:P00995

Variantes genéticas (ClinVar)

122 variantes patogênicas registradas no ClinVar.

🧬 SPINK1: NM_001379610.1(SPINK1):c.123G>C (p.Lys41Asn) ()
🧬 SPINK1: NM_001379610.1(SPINK1):c.195-1G>A ()
🧬 SPINK1: NC_000005.9:g.(?_147204224)_(147516554_?)del ()
🧬 SPINK1: GRCh37/hg19 5q31.3-32(chr5:141566629-147240595)x1 ()
🧬 SPINK1: GRCh37/hg19 5q32(chr5:147165102-147600692)x1 ()
Ver todas no ClinVar

Classificação de variantes (ClinVar)

Distribuição de 17 variantes classificadas pelo ClinVar.

6
9
2
Patogênica (35.3%)
VUS (52.9%)
Benigna (11.8%)
VARIANTES MAIS SIGNIFICATIVAS
SPINK1: NM_001379610.1(SPINK1):c.126A>G (p.Ile42Met) [Conflicting classifications of pathogenicity]
SPINK1: NM_003122.5(SPINK1):c.-147A>G [Conflicting classifications of pathogenicity]
SPINK1: NM_001379610.1(SPINK1):c.194+2T>C [Pathogenic/Likely pathogenic]
SPINK1: NM_003122.5(SPINK1):c.-191-24G>T [Pathogenic]
SPINK1: NM_003122.5(SPINK1):c.-191-24G>A [Conflicting classifications of pathogenicity]

Vias biológicas (Reactome)

1 via biológica associada aos genes desta condição.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
Aprovado1
·Pré-clínico2
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 3 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Pancreatite tropical

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

Pesquisa e ensaios clínicos

0 ensaios clínicos encontrados.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

Timeline de publicações
11 papers (10 anos)
#1

Laparoscopic lateral pancreaticojejunostomy in the current era: A narrative review.

Journal of minimal access surgery2025 Jan 01

Chronic pancreatitis is a benign disease which causes recurrent abdominal pain and loss of pancreatic function. Lateral pancreaticojejunostomy (LPJ) is a commonly performed drainage procedure for this condition. While usually performed through an open approach, there have been few cases of laparoscopic approaches for this condition. A literature review was conducted to understand the current status of laparoscopic longitudinal pancreatojejunostomy (LLPJ). We conducted a comprehensive literature search using PubMed, Embase and Cochrane Library to find the articles published until 1 st October 2023. We excluded studies involving paediatric patients or robotic assisted surgeries. Our evaluation focussed on pain relief scores, morbidity, hospital stay length, mortality rates and the development of endocrine and exocrine deficiencies in the patients. The patients in the analysis had a mean age of 36.5 and a male to female ratio of 1.4:1. The mean main pancreatic duct diameter was 11.5 mm. Tropical pancreatitis was identified as the primary cause. The surgical procedure was performed using 4 ports with minimal bleeding and a 13% morbidity rate. The conversion rate was 15%. The average operative time was 260 min and the mean hospital stay was 5.7 days. The results for pain control were excellent, as 90% of patients did not report pain in most series at the end of 3 years. The laparoscopic surgical management of chronic calcific pancreatitis with LPJ offers a safe and effective solution for pain relief in carefully chosen patients. However, further comprehensive studies with large sample sizes are essential to establish a more conclusive comparison between LLPJ and open surgery.

#2

Precut papillotomy on a minor papilla followed by balloon papilloplasty to provide a complete pancreatic-duct drainage in tropical pancreatitis with reverse pancreas divisum.

VideoGIE : an official video journal of the American Society for Gastrointestinal Endoscopy2023 May

Video 1Demonstration of the precut on minor papilla and papilloplasty with pancreatoscopy.

#3

Tropical pancreatitis: an unusual presentation in Western Australia.

ANZ journal of surgery2020 Jan
#4

Early outcome of Frey's procedure for chronic pancreatitis: Nepalese tertiary center experience.

BMC surgery2019 Sep 18

Chronic pancreatitis is a progressive and persistent inflammatory disease resulting in pancreatic insufficiency leading to diabetes and steatorrhea. Abdominal pain is the most debilitating feature and is often refractory to treatment. Medical management with adequate analgesia and replacement of pancreatic enzyme supplements is the first line in management of chronic pancreatitis. Surgery is reserved for those who fail medical management. The choice of surgical procedure and timing of surgery is a topic of debate. The objective of this study was to analyze surgical safety along with short- and long- term outcomes of Frey's procedure for patients suffering from chronic pancreatitis. This was a retrospective review of cases of chronic pancreatitis who underwent Frey's procedure from 2016 January to 2019 February at Tribhuvan University Teaching Hospital. Demographics, intraoperative findings, perioperative outcomes, and short- and long-time outcomes were analyzed. Total of 26 patients (age ranged 17-52, male - 14) underwent Frey's procedure in the study period. Alcohol was etiology in six patients while the majority (76.9%) were nonalcoholic. Half of the patients had tropical pancreatitis. Intractable pain was present in all cases along with pseudocyst in three and pseudoaneurysm in one case. The mean preoperative Izbicki scores were 53.4 ± 17.6. Six patients had diabetes and two patients had steatorrhea. Major complications were seen in 11.5% of cases while mortality was in one patient. The median duration of the hospital stay was seven days. Over a median follow up of 17 months (range, 3-38), there were significantly lower pain scores postoperatively and 92% were pain-free. Only one new case of diabetes developed postoperatively. Our early experiences suggests that Frey's procedure can be a safe option for patients with chronic pancreatitis, with acceptable perioperative morbidity with adequate pain relief without worsening of pancreatic endocrine and exocrine function.

#5

Laparoscopic lateral pancreaticojejunostomy: an evolution to endostapled technique.

Surgical endoscopy2019 Jun

Chronic pancreatitis (CP) is a debilitating condition resulting in severe pain with progressive deterioration of pancreatic function. "Tropical" pancreatitis represents a variant of the disease with widely dilated ducts, numerous calculi, and few strictures. Traditionally, modified Puestow's procedure has been the treatment of choice for a dilated pancreatic ductal system. However, it has only recently been adapted to laparoscopic approach which is a technically demanding procedure primarily due to need for extensive intra-corporeal suturing. Symptomatic cases of CP presenting at our center with minimum 8 mm mean ductal diameter at body and head were selected for laparoscopic modified Puestow's procedure. Those with prior pancreatic surgery, pancreatic head masses, endoscopic pancreatic stenting, and portal hypertension were excluded. Twenty-eight cases meeting selection criteria underwent a laparoscopic procedure. Seven patients (25%) underwent a stapled pancreaticojejunal anastomosis, 17 (60.7%) received a sutured anastomosis. Four patients (14.3%) were converted to open surgery due to failure to localize the pancreatic duct with percutaneous needle aspiration. Of those patients who underwent a successful laparoscopic procedure, a single patient developed a pancreatic fistula which resolved spontaneously; another patient had a difficult post-operative course with prolonged intensive care. We suffered no mortality within the series and no patient had any long-term disability. Anastomotic patency rates of 100% were achieved by the third post-operative month. Lateral pancreaticojejunostomy is an effective surgical management for CP with a dilated ductal system. Its laparoscopic adoption is the rational next surgical step. It allows effective duct decompression with low mortality and morbidity. The procedure demands an advanced surgical skill set with an emphasis on intra-corporeal suturing. Those patients suffering from tropical CP with wide ductal dilatation greater than 12 mm are suited to an endostapled anastomosis which helps significantly reduce operative time without any corrosion of outcomes.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC42 artigos no totalmostrando 11

2025

Laparoscopic lateral pancreaticojejunostomy in the current era: A narrative review.

Journal of minimal access surgery
2023

Precut papillotomy on a minor papilla followed by balloon papilloplasty to provide a complete pancreatic-duct drainage in tropical pancreatitis with reverse pancreas divisum.

VideoGIE : an official video journal of the American Society for Gastrointestinal Endoscopy
2019

Early outcome of Frey's procedure for chronic pancreatitis: Nepalese tertiary center experience.

BMC surgery
2020

Tropical pancreatitis: an unusual presentation in Western Australia.

ANZ journal of surgery
2019

Laparoscopic lateral pancreaticojejunostomy: an evolution to endostapled technique.

Surgical endoscopy
2018

Chronic pancreatitis. Some important historical aspects.

Gastroenterologia y hepatologia
2016

Progression of recurrent acute and chronic pancreatitis: A short-term follow up study from a southern Indian centre.

Indian journal of gastroenterology : official journal of the Indian Society of Gastroenterology
2016

Changing phenotype and disease behaviour of chronic pancreatitis in India: evidence for gene-environment interactions.

Global health, epidemiology and genomics
2016

Quality of Life after Frey's Procedure in Patients with Chronic Pancreatitis.

Journal of clinical and diagnostic research : JCDR
2015

Demographic and clinicopathological profile of patients with chronic pancreatitis in a tertiary referral teaching hospital of West Bengal: Personal experience.

Indian journal of gastroenterology : official journal of the Indian Society of Gastroenterology
2015

Pancreatic cancer in chronic pancreatitis.

Indian journal of surgical oncology
Ver todos os 42 no EuropePMC

Associações

Organizações que acompanham esta doença — pra ter apoio e orientação

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Comunidades

Grupos ativos de quem convive com esta doença aqui no Raras

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Doenças relacionadas

Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico

Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Laparoscopic lateral pancreaticojejunostomy in the current era: A narrative review.
    Journal of minimal access surgery· 2025· PMID 39611599mais citado
  2. Precut papillotomy on a minor papilla followed by balloon papilloplasty to provide a complete pancreatic-duct drainage in tropical pancreatitis with reverse pancreas divisum.
    VideoGIE : an official video journal of the American Society for Gastrointestinal Endoscopy· 2023· PMID 37197165mais citado
  3. Tropical pancreatitis: an unusual presentation in Western Australia.
    ANZ journal of surgery· 2020· PMID 30836443mais citado
  4. Early outcome of Frey's procedure for chronic pancreatitis: Nepalese tertiary center experience.
    BMC surgery· 2019· PMID 31533694mais citado
  5. Laparoscopic lateral pancreaticojejunostomy: an evolution to endostapled technique.
    Surgical endoscopy· 2019· PMID 30194645mais citado

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:103918(Orphanet)
  2. OMIM OMIM:608189(OMIM)
  3. MONDO:0011986(MONDO)
  4. GARD:16946(GARD (NIH))
  5. Variantes catalogadas(ClinVar)
  6. Busca completa no PubMed(PubMed)
  7. Q55783556(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Pancreatite tropical
Compêndio · Raras BR

Pancreatite tropical

ORPHA:103918 · MONDO:0011986
Prevalência
Unknown
CID-10
K86.1 · Outras pancreatites crônicas
CID-11
Início
Childhood
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C1842402
Repurposing
3 candidatos
camostat-mesilateprotease inhibitor
gabexateserine protease inhibitor
methanthelineacetylcholine receptor antagonist
EuropePMC
Wikidata
Papers 10a
DiscussaoAtiva

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